Today is the first day of family caregiver month. I was thrust into this role almost three years ago in January 2010 when my then 15 year old daughter was diagnosed with a congenital heart defect as well as pulmonary arterial hypertension and Eisenmengers Syndrome. Both of these were caused by the lack of diagnosis of her congenital heart defect.
To say this role has been difficult is an understatement. Our lives were forever turned upside down. For any caregiver, it is painful to watch your loved one be sick and/or watch them get sicker. I can't express the feelings of helplessness I feel every single day.
I had a friend on facebook private message me apologizing that she hadn't wished me a happy birthday. She said that she thought about me every single day and wished with all of her being that she could help... but to no avail. This really got me thinking about my experience with friends and acquaintances. I get those who are just very uncomfortable around me for whatever reason... then there are those who don't want to bother me because I have "too much on my plate" and while this may be true ... it is my decision to make as to whether or not this is true in my eyes.
There are those like my friend who feel that there's nothing that they can do but there are so many things you can do to help a family caregiver. I belong to a wonderful online community of caregivers at www.caregiving.com and every Friday is take a meal to a family caregiver day. I think that this is a wonderful idea. You can call, text, email, private message a caregiver that you are thinking of them and it doesn't cost anything and it will put a smile on their face. In Sept. Nicole had a doctor's appointment and a site change the same day and it was going to be a long day and a friend of mine late in the afternoon when we were still at the doctor's office called me and said she had a meal for me that she wanted to bring by. This was so sweet of her and while she had to bring me the meal the next day it was so much appreciated. A get together for an hour for a cup of coffee, stopping by the house to say "hi", a card in the mail etc.are all little things that mean so much. My next door neighbor will bring me my mail, bring up my garbage can on garbage days and/or just come over to ask how Nicole is doing. It doesn't have to be something big to make an impact on a caregiver's day.
I understand how my friend feels. I can't express how it feels to have a child (whether they are young or an adult) with an incurable illness. The guilt (at least for me) and the helplessness I feel some days overwhelms me. To me there is just a different bond/relationship that a mother has with a child than with any other relationship. This is not to say that caregiving isn't difficult for anyone who who takes care of a parent, spouse, sibling, friend etc. because it is.
I know that I can't make Nicole better which is a very hard pill to swallow and I wallow in this with denial, self-pity etc. but I have to think about Nicole as she is the one with the illness. I would do anything to be able to make her better but since God is the only one that can do this and the only thing I can do is to make her life easier which is my goal every day and I will strive to do this to the best of my ability regardless what people say or how they judge.
What can you do today to make life easier for a family caregiver?
Thursday, November 1, 2012
Thursday, October 18, 2012
Forgiveness...
Earlier this month I was watching a week of shows on forgiveness with Joyce Meyer. She just released a new book in September called "Do Yourself a Favor.... Forgive" . I have not read the book yet but I will get to it soon.
One show had an interview with Sandra, Joyce's daughter. She was talking about her struggle with forgiveness. Her struggle with this was with herself. I also have a very big problem with this. Sometimes I believe more so than with forgiving others. Sandra talked about how she would go to bed every night feeling bad because of all the things she didn't get done on her to do list. She would never be at peace. I feel this same exact way. I feel so bad if I don't get my to do list done for the day. Some of my problem is that I procrastinate and then things pile up and I also goof off... read a book, surf the net, facebook you name it anything to keep me from my reality and to do list. This makes me feel bad because if I didn't put things off I might actually get my to do list done. Sandra said she had to change her attitude to looking at it from the stand point of what she did get done and not from what she didn't get done. My friend, Denise, has told me to do this too but so far this concept hasn't reached through to my stubborn brain.
I have struggled with offense and taking things too personal. I have had a lot of people tell me they are going to do this or that and then they never do. This happens to be a real pet peeve of mine and I am very conscious that I don't do this myself. I consciously try not to do this to others but I will do this to myself and Nicole. Denise also believes that this struggle with offense and taking things personally against me is tied to my parents divorce and the relationship I had/didn't have with my dad when I was a teenager. I can see where there might be a connection but I don't like putting the blame on others unless it is clear the blame goes there. I think it is too easy of a trap that we fall into when we start blaming others.
I have had to forgive a lot since 2010 with friends and family not living up to MY expectations and causing me to be offended, hurt, and to question myself. God says we should be quick to forgive like he forgives us. I think one of the biggest people I have had to forgive is myself and my role in Nicole's illness. I wish I could say with peace that I have forgiven myself but I can't as this is still a work in progress.
I think so many things tie into forgiveness ... our own expectations, offense and pride to name a few.
One show had an interview with Sandra, Joyce's daughter. She was talking about her struggle with forgiveness. Her struggle with this was with herself. I also have a very big problem with this. Sometimes I believe more so than with forgiving others. Sandra talked about how she would go to bed every night feeling bad because of all the things she didn't get done on her to do list. She would never be at peace. I feel this same exact way. I feel so bad if I don't get my to do list done for the day. Some of my problem is that I procrastinate and then things pile up and I also goof off... read a book, surf the net, facebook you name it anything to keep me from my reality and to do list. This makes me feel bad because if I didn't put things off I might actually get my to do list done. Sandra said she had to change her attitude to looking at it from the stand point of what she did get done and not from what she didn't get done. My friend, Denise, has told me to do this too but so far this concept hasn't reached through to my stubborn brain.
I have struggled with offense and taking things too personal. I have had a lot of people tell me they are going to do this or that and then they never do. This happens to be a real pet peeve of mine and I am very conscious that I don't do this myself. I consciously try not to do this to others but I will do this to myself and Nicole. Denise also believes that this struggle with offense and taking things personally against me is tied to my parents divorce and the relationship I had/didn't have with my dad when I was a teenager. I can see where there might be a connection but I don't like putting the blame on others unless it is clear the blame goes there. I think it is too easy of a trap that we fall into when we start blaming others.
I have had to forgive a lot since 2010 with friends and family not living up to MY expectations and causing me to be offended, hurt, and to question myself. God says we should be quick to forgive like he forgives us. I think one of the biggest people I have had to forgive is myself and my role in Nicole's illness. I wish I could say with peace that I have forgiven myself but I can't as this is still a work in progress.
I think so many things tie into forgiveness ... our own expectations, offense and pride to name a few.
Thursday, October 11, 2012
What is the next step?
Nicole started a new continuous drug that is supplied via an infusion pump that is delivered subcutaneously (through the skin) on July 25th. The doctor had high hopes that this would significantly improve Nicole's symptoms and make her feel better.
Between July 25th and August 27th Nicole was slowly being weaned off of the inhaled medicine, Tyvaso, that she had been on since Dec. 2010. After she stopped the Tyvaso I saw a big difference in her symptoms and how she was feeling. It wasn't an improvement. On Sept. 7th she went in to see the PH specialist and he saw how bad she looked and we discussed how she had been feeling. He immediately had her get an echo and then gave us a script for a chest x-ray to rule out any pneumonia or anything since she had been congested. The echo came back with no change since the last one which was three months prior to that. At this time, the doctor didn't have an answer for why she wasn't feeling well and her symptoms had increased. He said to increase her Remodulin every other day with pump changes until the vial we were using was gone and then when we started a higher strength vial we would go back to increases every three days.
We had been told by one of the nurses that if her blood pressure got below 85 (the top number) we were suppose to contact the PH doctor. On Sept. 16th after Nicole finished with rehab they took her blood pressure and it was 80/60 so I had to call the doctor's office. I talked to one of the trauma nurses who told me to have Nicole drink some juice and a bottle of water and re-take her blood pressure in thirty minutes we were not to leave the hospital. If her blood pressure didn't come up we would have to leave Florida Hospital and go to ORMC as this is where her doctor has privileges. Nicole was showing no adverse effects of the low blood pressure and said she felt no different than usual. Thankfully, after thirty minutes her blood pressure did go back up and we were able to come home. The nurse had asked me about side effects and her current rate of Remodulin. She said that she would give all the information to the doctor. Later that afternoon the doctor called me and said that he was disappointed that he hasn't seen more progress with the Remodulin. Nicole was having some major side effects with the dose increases that wasn't helping. At this time he stopped the increases and told me that he thought it was time to talk about the next step which is transplant. I remember that I started to tear up and get emotional. We have known since Nicole's diagnosis that this would be the end result but I didn't think it would be so soon. Since this conversation with the doctor Nicole has been on a steady dose of the Remodulin and she is doing well compared to August and the beginning to middle of September. I haven't heard from the doctor since this conversation but I know Nicole's cardiologist has spoken to him.
Nicole saw her cardiologist on Tuesday for her three month check-up. He had called around to some major hospitals around the country inquiring about transplants and what there procedures were etc. He also contacted two of the transplant hospitals in this area and told me where he thought would be the best place for us to go. He also agrees with the PH specialist that it is time to be evaluated. It should be sooner rather than wait for later. The transplant process is long.
I have mixed feelings about this. I don't think that the Remodulin has been at a high enough dose nor used long enough to see if in fact it will significantly improve her symptoms. I have noticed with Nicole especially in July when she started taking the hormone to regulate her periods that her exercise tolerance at rehab suffered. I think that anytime she starts/stops a medication her body rebels and she doesn't feel well for a period of time. She is just now starting to get back her exercise tolerance at rehab.
I remember on Tuesday sitting in the cardiologist listening to him talking and remembering that January day in 2010 in that same office being told that Nicole had this deadly disease and a heart defect that she had been born with. I have been able to since this time for the most part stick my head in the sand and forget just how sick she is (even though she doesn't look it). When the cardiologist recommended the evaluation also it all hit me in the face. I can deal with the life that we have now with this illness but I don't know if I can watch Nicole go through what she will go through during major heart surgery and a double lung transplant. I was reading a little about lung transplant results and found that the survival rate for lung transplants is 82% but in my mind I think not of the 82% percent who survive but the 18% who don't.
A part of me believes that she needs to be on a higher dose and longer time on the Remodulin before we can say it was a failure but something that the cardiologist said really hit be like a brick in the face. He said " if she had one bout of diarreha where she could keep no fluids down it would be lethal." I can talk myself into believing that even though Nicole is ill she isn't that ill and she does all right for herself even though I know in the back of my mind if she got the flu she would be in the hospital and it could be potentially deadly for her. I understand the doctors don't want to wait to long because of the length of time this process takes but I am just not ready to deal with this and I don't think that I have been able to deal with this since her diagnosis but I just fake it.
In 2009 I believe God put in my heart to "get my house in order". I have quite a bit of clutter and not enough house. I, of course, have put this off thinking that God didn't really speak to me etc. I have been trying to declutter at least 15 minutes a day if it is busy and if I am home for the day I will go for 30 minutes if not longer. I struggle with this because I don't want to do this (just like I don't want Nicole to be sick). I have to make decisions every day about Nicole's medical issues, finances etc. and don't seem to have a hard time with this most of the time but make me look at a piece of clutter and decide what to do with it and I can't. I was already overwhelmed with my duties the past couple of week as Nicole's teacher and getting out her progress reports to the school we use for that along with a ton of paperwork that I have to fill out for Nicole's disability review for SSI as well as some other commitments that I have and my mind has turned into a tornado swirling around.
Sometimes I just feel like I want to stop the world just for a day and not have any problems so I can get my head in order, unfortunately, I know this isn't likely to happen. I always think of myself and what this is like for me but I don't consider that it is Nicole that has to live this life. I feel that life is the hardest for those that are left behind. We are the ones who grieve the loss not the person who passed. I don't know if this is just my warped feelings or if it has some merit. I have been reading this book called "When Bad Things Happen to Good People" . The author was talking about how people respond to tragedy and that the majority (I'm included) assume that God is the cause of the suffering because we deserve punishment or He doesn't care. We are left hating ourselves for deserving the fate or hating God for sending this to us. He asks could it be that God doesn't cause our suffering but He stands ready to help cope with the tragedies? I have been thinking a lot about this lately. I'm still not clear on my feelings about it though.
Between July 25th and August 27th Nicole was slowly being weaned off of the inhaled medicine, Tyvaso, that she had been on since Dec. 2010. After she stopped the Tyvaso I saw a big difference in her symptoms and how she was feeling. It wasn't an improvement. On Sept. 7th she went in to see the PH specialist and he saw how bad she looked and we discussed how she had been feeling. He immediately had her get an echo and then gave us a script for a chest x-ray to rule out any pneumonia or anything since she had been congested. The echo came back with no change since the last one which was three months prior to that. At this time, the doctor didn't have an answer for why she wasn't feeling well and her symptoms had increased. He said to increase her Remodulin every other day with pump changes until the vial we were using was gone and then when we started a higher strength vial we would go back to increases every three days.
We had been told by one of the nurses that if her blood pressure got below 85 (the top number) we were suppose to contact the PH doctor. On Sept. 16th after Nicole finished with rehab they took her blood pressure and it was 80/60 so I had to call the doctor's office. I talked to one of the trauma nurses who told me to have Nicole drink some juice and a bottle of water and re-take her blood pressure in thirty minutes we were not to leave the hospital. If her blood pressure didn't come up we would have to leave Florida Hospital and go to ORMC as this is where her doctor has privileges. Nicole was showing no adverse effects of the low blood pressure and said she felt no different than usual. Thankfully, after thirty minutes her blood pressure did go back up and we were able to come home. The nurse had asked me about side effects and her current rate of Remodulin. She said that she would give all the information to the doctor. Later that afternoon the doctor called me and said that he was disappointed that he hasn't seen more progress with the Remodulin. Nicole was having some major side effects with the dose increases that wasn't helping. At this time he stopped the increases and told me that he thought it was time to talk about the next step which is transplant. I remember that I started to tear up and get emotional. We have known since Nicole's diagnosis that this would be the end result but I didn't think it would be so soon. Since this conversation with the doctor Nicole has been on a steady dose of the Remodulin and she is doing well compared to August and the beginning to middle of September. I haven't heard from the doctor since this conversation but I know Nicole's cardiologist has spoken to him.
Nicole saw her cardiologist on Tuesday for her three month check-up. He had called around to some major hospitals around the country inquiring about transplants and what there procedures were etc. He also contacted two of the transplant hospitals in this area and told me where he thought would be the best place for us to go. He also agrees with the PH specialist that it is time to be evaluated. It should be sooner rather than wait for later. The transplant process is long.
I have mixed feelings about this. I don't think that the Remodulin has been at a high enough dose nor used long enough to see if in fact it will significantly improve her symptoms. I have noticed with Nicole especially in July when she started taking the hormone to regulate her periods that her exercise tolerance at rehab suffered. I think that anytime she starts/stops a medication her body rebels and she doesn't feel well for a period of time. She is just now starting to get back her exercise tolerance at rehab.
I remember on Tuesday sitting in the cardiologist listening to him talking and remembering that January day in 2010 in that same office being told that Nicole had this deadly disease and a heart defect that she had been born with. I have been able to since this time for the most part stick my head in the sand and forget just how sick she is (even though she doesn't look it). When the cardiologist recommended the evaluation also it all hit me in the face. I can deal with the life that we have now with this illness but I don't know if I can watch Nicole go through what she will go through during major heart surgery and a double lung transplant. I was reading a little about lung transplant results and found that the survival rate for lung transplants is 82% but in my mind I think not of the 82% percent who survive but the 18% who don't.
A part of me believes that she needs to be on a higher dose and longer time on the Remodulin before we can say it was a failure but something that the cardiologist said really hit be like a brick in the face. He said " if she had one bout of diarreha where she could keep no fluids down it would be lethal." I can talk myself into believing that even though Nicole is ill she isn't that ill and she does all right for herself even though I know in the back of my mind if she got the flu she would be in the hospital and it could be potentially deadly for her. I understand the doctors don't want to wait to long because of the length of time this process takes but I am just not ready to deal with this and I don't think that I have been able to deal with this since her diagnosis but I just fake it.
In 2009 I believe God put in my heart to "get my house in order". I have quite a bit of clutter and not enough house. I, of course, have put this off thinking that God didn't really speak to me etc. I have been trying to declutter at least 15 minutes a day if it is busy and if I am home for the day I will go for 30 minutes if not longer. I struggle with this because I don't want to do this (just like I don't want Nicole to be sick). I have to make decisions every day about Nicole's medical issues, finances etc. and don't seem to have a hard time with this most of the time but make me look at a piece of clutter and decide what to do with it and I can't. I was already overwhelmed with my duties the past couple of week as Nicole's teacher and getting out her progress reports to the school we use for that along with a ton of paperwork that I have to fill out for Nicole's disability review for SSI as well as some other commitments that I have and my mind has turned into a tornado swirling around.
Sometimes I just feel like I want to stop the world just for a day and not have any problems so I can get my head in order, unfortunately, I know this isn't likely to happen. I always think of myself and what this is like for me but I don't consider that it is Nicole that has to live this life. I feel that life is the hardest for those that are left behind. We are the ones who grieve the loss not the person who passed. I don't know if this is just my warped feelings or if it has some merit. I have been reading this book called "When Bad Things Happen to Good People" . The author was talking about how people respond to tragedy and that the majority (I'm included) assume that God is the cause of the suffering because we deserve punishment or He doesn't care. We are left hating ourselves for deserving the fate or hating God for sending this to us. He asks could it be that God doesn't cause our suffering but He stands ready to help cope with the tragedies? I have been thinking a lot about this lately. I'm still not clear on my feelings about it though.
Friday, September 21, 2012
Mixed feelings... I think...
I have a lot of mixed feelings today and I'm sure they will be with me at least through the weekend if not longer. Tomorrow the 22nd will be Nicole's last day as a minor and my last day as her legal guardian. On Sunday, she will be a legal adult.
I wish I knew what it felt like in this situation in a perfect world but I do not and I only know what it feels like with our circumstances and our life. I don't think it would be such a problem for me if Nicole wasn't chronically, terminally ill. All the issues that I deal with on her behalf as her legal guardian/parent no 18 year old should be expected to be able to do themselves. On Monday I will take Nicole to the bank for the notarization giving me power of attorney over her finances and health issues. I struggle with this because of criticism from others that I need to let her go as she is an adult. I would agree with this but I also know my daughter and she isn't capable of this at this point in her life. I don't want to take away her decision-making I just want to be able to take the bulk of the stress away from her as it is detrimental to her health. She is also still in high school.
I am having a hard time getting my thoughts in order so I hope that I am making sense. I feel that I am losing all control and that my job as her mom/legal guardian at least in the eyes of the world, medical community etc. is over. I know logically that I will always be her mom but she will be expected to make her own decisions and I have no say in that other than to Nicole if this is what she wishes. I will always support her in all her hopes, dreams, problems etc. and will do anything I can to help her. I have done my job raising her so now she can move forward with those morals and values and make her mark in the world and I know she has awesome potential to do this but it saddens me that she doesn't see this. I also feel that our relationship will be different but I don't know what this means or looks like.
I don't think that I would have such an emotional struggle with this if Nicole was healthy but she is not so we have to adjust accordingly but I know I will have to battle for this harder than I did as her legal guardian. Social Security doesn't recognize power of attorney so I had to request and was granted representative payee status that gives me authority over Nicole's SSI checks and I am the one accountable to how that money is spent and for what. Nicole and I will come up with a budget and her expenses together but I will handle everything pertaining to bill payment etc. Children's Medical Services doesn't recognize power of attorney only legal guardianship. In order to get legal guardianship I have to go in front of a judge and even if Nicole agrees to it ... she will have no rights and her decision making will be taken away and I don't want to do this.
At the same time as the power of attorney is complete Nicole will also name me as her health care surrogate. I know that she needs to do a living will but I haven't been able to find a way to approach her about this subject. It will be hard for her to deal with because of her anxiety issues and it isn't fun conversation to begin with. In light of the fact that a transplant is down the road at some point this is very important to have.
I know that this is just another day and the world will not end we will just move forward as we have been but a little differently. I will still be an advocate for her and I will do everything I can with her medical issues to take this burden off of her as she carries enough just trying to get through each day so if I have to take calls and talk to doctor's, pharmacies, do her medicine etc this is the least I can do.
I hope that she always knows that no matter what she does I will always love her and this will never change.
I wish I knew what it felt like in this situation in a perfect world but I do not and I only know what it feels like with our circumstances and our life. I don't think it would be such a problem for me if Nicole wasn't chronically, terminally ill. All the issues that I deal with on her behalf as her legal guardian/parent no 18 year old should be expected to be able to do themselves. On Monday I will take Nicole to the bank for the notarization giving me power of attorney over her finances and health issues. I struggle with this because of criticism from others that I need to let her go as she is an adult. I would agree with this but I also know my daughter and she isn't capable of this at this point in her life. I don't want to take away her decision-making I just want to be able to take the bulk of the stress away from her as it is detrimental to her health. She is also still in high school.
I am having a hard time getting my thoughts in order so I hope that I am making sense. I feel that I am losing all control and that my job as her mom/legal guardian at least in the eyes of the world, medical community etc. is over. I know logically that I will always be her mom but she will be expected to make her own decisions and I have no say in that other than to Nicole if this is what she wishes. I will always support her in all her hopes, dreams, problems etc. and will do anything I can to help her. I have done my job raising her so now she can move forward with those morals and values and make her mark in the world and I know she has awesome potential to do this but it saddens me that she doesn't see this. I also feel that our relationship will be different but I don't know what this means or looks like.
I don't think that I would have such an emotional struggle with this if Nicole was healthy but she is not so we have to adjust accordingly but I know I will have to battle for this harder than I did as her legal guardian. Social Security doesn't recognize power of attorney so I had to request and was granted representative payee status that gives me authority over Nicole's SSI checks and I am the one accountable to how that money is spent and for what. Nicole and I will come up with a budget and her expenses together but I will handle everything pertaining to bill payment etc. Children's Medical Services doesn't recognize power of attorney only legal guardianship. In order to get legal guardianship I have to go in front of a judge and even if Nicole agrees to it ... she will have no rights and her decision making will be taken away and I don't want to do this.
At the same time as the power of attorney is complete Nicole will also name me as her health care surrogate. I know that she needs to do a living will but I haven't been able to find a way to approach her about this subject. It will be hard for her to deal with because of her anxiety issues and it isn't fun conversation to begin with. In light of the fact that a transplant is down the road at some point this is very important to have.
I know that this is just another day and the world will not end we will just move forward as we have been but a little differently. I will still be an advocate for her and I will do everything I can with her medical issues to take this burden off of her as she carries enough just trying to get through each day so if I have to take calls and talk to doctor's, pharmacies, do her medicine etc this is the least I can do.
I hope that she always knows that no matter what she does I will always love her and this will never change.
Saturday, September 8, 2012
New Medicine
Nicole started a new medicine, Remodulin, on July 25th which is delivered subcutaneously (SQ) . This means that the medicine is absorbed through the skin through a tiny catheter. She uses a pump that gives her a continuous supply of medicine... like a diabetic.
Nicole seemed to be doing well. On Aug. 27th she took her inhaled medicine for the last time. It is Remodulin in the inhaled form and the doctor wanted her to slowly wean off of it. It seems ever since this time she has had more shortness of breath and more fatigue/tiredness. If she is without oxygen for more than 10 minutes sometimes her lips will start to turn blue. Her ability to exercise at rehab has decreased.
Until we start with a stronger concentration vial of the medicine we have to change Nicole's syringe of medicine and the pump every 2 days a process that takes about 20 minutes if everything goes smooth. Every other pump change came with an increase in the medicine. Nicole seems to be tolerating the medicine well.
I contacted the PH nurse earlier in the week as I am concerned that Nicole isn't feeling well. She was complaining of site pain again also. The nurse recommended the site be changed. On Friday the Accredo nurse and a local nurse were here to help Nicole and I do the site change. This means that the administration site is changed. Nicole's initial site was on her left side stomach area. A decision had to be made where to put her next site. It was decided that we would try the middle abdomen area. Nicole put in the site which at the time of insertion should have no pain other than a prick when the needle goes it and then when it is pulled out. Nicole kept having burning pain so the nurse said that wasn't normal so the site had to be taken out and was moved over closer to her side. It is almost in the same spot as before except on her opposite side. This worked a lot better.
We had an appointment with the doctor that afternoon. She was suppose to do a six minute walk test to see if there has been improvement from a month ago but she wasn't feeling well and just her walking to the back had her SOB (short of breath). Liz could tell she didn't feel well and her oxygen saturation levels were low, even for her. Liz had said she was going to tell the doctor to see her first and then to tell her to do the six minute walk. He didn't make her do it. She would have done worse than 6 weeks ago and she did pretty bad then. I explained to him her symptoms and how ever since she stop the Tyvaso she has felt bad. She was squeezed in for an echo which he said showed no sign of decline from the last one she had which was earlier this year. He said that he is stumped as to why her symptoms are worsening. He told me to get her a chest x-ray next week just to rule out pneumonia since she has had some congestion. He also said that instead of every other syringe/pump change do an increase of medicine every syringe change.
I started the 2.5 mL vial yesterday so according to the pharmacist this will last 12 days. Since we will be doing an increase every two days this means it will only last for maybe 5 more pump changes. The pharmacy called during her doctor's appointment and I wasn't able to take the call. I was told earlier in the day that the pharmacy had gotten the authorization from Medicaid for the 5 mL strength vial :). I know they will be getting that to me next week once I call them on Monday (which will be another long, busy day more for me than Nicole). I also have to compare the dosing chart that I have that tells me how much medicine to put in the syringe, what to set the pump at and how many nanograms of medicine it is and coordinate it to the 5 mL chart.
I'm hoping that this increase in medicine will start to make her feel better. Nicole tends to downplay everything but I know that something is bothering her when she has been quite grumpy which isn't like Nicole. Today she will start the battle of site pain which will probably last until for about 7- 10 days during which time she will be taking pain medication every six hours so it doesn't get too unbearable. Last night she started walking hunched over because of the pain and that will probably continue through the weekend so sitting is the best option for her. My prayer is that she won't have the pain but if she must it will be tolerable.
Yesterday when we were at the doctors, a lady who was there with her husband came over and asked me about Nicole portable oxygen concentrator. I answered her questions and she told me about her husband. She told Nicole how beautiful she was (which she is) and that she hoped that she would get better. Another lady who worked at the office saw us when Nicole was getting her echo and she said hi to me and wished my luck and blessing and seemed very nice. When we were done with the doctor the office had already closed. The doctor pushed Nicole in a wheelchair out into the waiting room as my husband was coming from work to pick us up. The same lady that I saw earlier outside of the echo room came out and told the doctor that she would make sure we got out of the building when Rick got their. She stayed with us and talked to Nicole and I. She said that she knew the doctor would do everything he could for her and she was in good hands. She asked if we had been there before and I said "yes". She said she recognized Nicole. She also told Nicole how pretty she was.
While we were waiting for the doctor to read the echo a friend called me. This person we only know because of Nicole's illness. She had a dinner for us and wanted to drop it off as she knew we had had a long day. She has done so much for me and especially Nicole and for that I am forever grateful. I am forever grateful for her doctor who has also gone above and beyond to help Nicole.
It is very difficult to watch Nicole be so ill, but I am no different or more special than any other parent who has a seriously ill child. It saddens me to watch Nicole some days only focus on her illness and how bad she feels and how hard her life is instead of all the blessings she does have. Nicole doesn't complain but I can see it through her attitude and anxiety. She is beautiful, sweet, kind, intelligent, a very gifted artist and has people who really care about her. I know she is mad at God for letting her be born sick (her congenital heart defect) and doesn't understand. I try to tell her that in some ways she is more special because not every teenager could go through what she does every day. She is an inspiration and some day I hope that she will see that.
Nicole seemed to be doing well. On Aug. 27th she took her inhaled medicine for the last time. It is Remodulin in the inhaled form and the doctor wanted her to slowly wean off of it. It seems ever since this time she has had more shortness of breath and more fatigue/tiredness. If she is without oxygen for more than 10 minutes sometimes her lips will start to turn blue. Her ability to exercise at rehab has decreased.
Until we start with a stronger concentration vial of the medicine we have to change Nicole's syringe of medicine and the pump every 2 days a process that takes about 20 minutes if everything goes smooth. Every other pump change came with an increase in the medicine. Nicole seems to be tolerating the medicine well.
I contacted the PH nurse earlier in the week as I am concerned that Nicole isn't feeling well. She was complaining of site pain again also. The nurse recommended the site be changed. On Friday the Accredo nurse and a local nurse were here to help Nicole and I do the site change. This means that the administration site is changed. Nicole's initial site was on her left side stomach area. A decision had to be made where to put her next site. It was decided that we would try the middle abdomen area. Nicole put in the site which at the time of insertion should have no pain other than a prick when the needle goes it and then when it is pulled out. Nicole kept having burning pain so the nurse said that wasn't normal so the site had to be taken out and was moved over closer to her side. It is almost in the same spot as before except on her opposite side. This worked a lot better.
We had an appointment with the doctor that afternoon. She was suppose to do a six minute walk test to see if there has been improvement from a month ago but she wasn't feeling well and just her walking to the back had her SOB (short of breath). Liz could tell she didn't feel well and her oxygen saturation levels were low, even for her. Liz had said she was going to tell the doctor to see her first and then to tell her to do the six minute walk. He didn't make her do it. She would have done worse than 6 weeks ago and she did pretty bad then. I explained to him her symptoms and how ever since she stop the Tyvaso she has felt bad. She was squeezed in for an echo which he said showed no sign of decline from the last one she had which was earlier this year. He said that he is stumped as to why her symptoms are worsening. He told me to get her a chest x-ray next week just to rule out pneumonia since she has had some congestion. He also said that instead of every other syringe/pump change do an increase of medicine every syringe change.
I started the 2.5 mL vial yesterday so according to the pharmacist this will last 12 days. Since we will be doing an increase every two days this means it will only last for maybe 5 more pump changes. The pharmacy called during her doctor's appointment and I wasn't able to take the call. I was told earlier in the day that the pharmacy had gotten the authorization from Medicaid for the 5 mL strength vial :). I know they will be getting that to me next week once I call them on Monday (which will be another long, busy day more for me than Nicole). I also have to compare the dosing chart that I have that tells me how much medicine to put in the syringe, what to set the pump at and how many nanograms of medicine it is and coordinate it to the 5 mL chart.
I'm hoping that this increase in medicine will start to make her feel better. Nicole tends to downplay everything but I know that something is bothering her when she has been quite grumpy which isn't like Nicole. Today she will start the battle of site pain which will probably last until for about 7- 10 days during which time she will be taking pain medication every six hours so it doesn't get too unbearable. Last night she started walking hunched over because of the pain and that will probably continue through the weekend so sitting is the best option for her. My prayer is that she won't have the pain but if she must it will be tolerable.
Yesterday when we were at the doctors, a lady who was there with her husband came over and asked me about Nicole portable oxygen concentrator. I answered her questions and she told me about her husband. She told Nicole how beautiful she was (which she is) and that she hoped that she would get better. Another lady who worked at the office saw us when Nicole was getting her echo and she said hi to me and wished my luck and blessing and seemed very nice. When we were done with the doctor the office had already closed. The doctor pushed Nicole in a wheelchair out into the waiting room as my husband was coming from work to pick us up. The same lady that I saw earlier outside of the echo room came out and told the doctor that she would make sure we got out of the building when Rick got their. She stayed with us and talked to Nicole and I. She said that she knew the doctor would do everything he could for her and she was in good hands. She asked if we had been there before and I said "yes". She said she recognized Nicole. She also told Nicole how pretty she was.
While we were waiting for the doctor to read the echo a friend called me. This person we only know because of Nicole's illness. She had a dinner for us and wanted to drop it off as she knew we had had a long day. She has done so much for me and especially Nicole and for that I am forever grateful. I am forever grateful for her doctor who has also gone above and beyond to help Nicole.
It is very difficult to watch Nicole be so ill, but I am no different or more special than any other parent who has a seriously ill child. It saddens me to watch Nicole some days only focus on her illness and how bad she feels and how hard her life is instead of all the blessings she does have. Nicole doesn't complain but I can see it through her attitude and anxiety. She is beautiful, sweet, kind, intelligent, a very gifted artist and has people who really care about her. I know she is mad at God for letting her be born sick (her congenital heart defect) and doesn't understand. I try to tell her that in some ways she is more special because not every teenager could go through what she does every day. She is an inspiration and some day I hope that she will see that.
Monday, July 30, 2012
Week in Review
On Tues., July 24th we had the pharmacy nurse and the local home health nurse come to our house to begin training for the new subcutaneous drug that Nicole is starting in hopes that this will improve her quality of life as the inhaled medication isn't doing the job anymore. The process is very complicated as opposed to the inhaled medication. There are a lot more steps and things must be kept sterile.
On Wed. we went to the doctor's office with the medication and all of the supplies that I had received that morning to do the training again as this time it is for real. I put two video's on facebook of Nicole inserting the catheter and getting started on the medication. In subcutaneous (subq) the medicine is admistered through a small catheter that is attached to her abdomen in which a pump keeps medicine flowing into her body. The pump cannot be turned off for any reason unless the pharmacist or Nicole's doctor tells us too. In case of a malfunction or an emergency we would usually have enough time to get to the hospital that her doctor works out of. Before the pump was started Nicole did a 6 minute walk test which was worse than in Feb. and her oxygen saturation levels got down to 74 at one point. This is a test that doctor's use to track how well the treatment is going as well as judging how the disease is or isn't progressing.
On Thurs., Nicole did very well except for having a hard time walking as she was getting use to a catheter as well as tape being on her belly and adjusting to a new way of life with making sure both the pump and the site is kept dry at all times especially in the shower. She can't go swimming anymore without a special suit called a dry suit which protects both the site and the pump. We had to go get bloodwork as the doctor wanted her to have a BNP test that measures the heart function. She also had her monthly CBC (complete blood count) liver function and pregnancy testing at the same time.
Friday was the hardest day for Nicole. She was in a lot of pain and even taking Advil and using ice, numbing cream, biofreeze etc., she still couldn't get relief. It isn't as painful if she doesn't walk or move around. It broke my heart to see her go through this. I spoke with the nurse at the doctor's office and found out that the BNP was normal which made me very, very happy. I wasn't so happy with her CBC though. Her platelet count had dropped to right above the critical stage again which could be from the new medicine but she hadn't even been on it 24 hours and she had some white blood issues this time as well as the normal red blood cell issues. I will call her cardiologist to see what/if I should be worried about this.
Saturday the local health nurse came out to help us with the pump change. It was evident when we took the tape off that Nicole was having a slight reaction to the tape and her skin was pink but it didn't itch or bother her. The nurse had a different tape and we are trying this to see if her skin will tolerate it better. The nurse also told me that I needed to give her the pain medication every six hours for the time being until such time as the site pain goes away. She will return on Tuesday for another pump change and drug increase.
Every three days the medicine has to be refilled and the pump has to be changed. About every two months the site itself will be changed. This is where the site pain comes into play. Every time the site is changed from about the third to tenth day afterwards there is a good chance of site pain. This is when the ice, gel, pain killers etc. come into play. I have been told that usually after this period this goes away. I can only hope.
Every time the pump is changed and the medicine is increased she has a chance of side effects which includes headaches, joint pain, jaw pain, nausea, diarrhea, dizziness etc., so far this hasn't happened yet but she is still at a very low dose. In addition, she is slowly being weaned off the inhaled medication which she will totally go off of in Sept. unless the doctor states otherwise.
My deepest desire is that this medication will improve her quality of life. Her doctor who is just absolutely wonderful in so many ways and I have so much gratitute toward him for many reasons told her on Wednesday how awesome she was and that he hoped this medicine would make her feel so much better so she would be able to see how awesome she was. Unfortunately, it isn't hard to see that Nicole doesn't have a lot of self-confidence in herself.
As stressful as this is for me I know how much more stressful it is for Nicole. I want to so much remain positive and hopeful that this will be the magic bullet but at the same time I don't want the hurt and disappointment if this doesn't work and she shows no improvement.
On Wed. we went to the doctor's office with the medication and all of the supplies that I had received that morning to do the training again as this time it is for real. I put two video's on facebook of Nicole inserting the catheter and getting started on the medication. In subcutaneous (subq) the medicine is admistered through a small catheter that is attached to her abdomen in which a pump keeps medicine flowing into her body. The pump cannot be turned off for any reason unless the pharmacist or Nicole's doctor tells us too. In case of a malfunction or an emergency we would usually have enough time to get to the hospital that her doctor works out of. Before the pump was started Nicole did a 6 minute walk test which was worse than in Feb. and her oxygen saturation levels got down to 74 at one point. This is a test that doctor's use to track how well the treatment is going as well as judging how the disease is or isn't progressing.
On Thurs., Nicole did very well except for having a hard time walking as she was getting use to a catheter as well as tape being on her belly and adjusting to a new way of life with making sure both the pump and the site is kept dry at all times especially in the shower. She can't go swimming anymore without a special suit called a dry suit which protects both the site and the pump. We had to go get bloodwork as the doctor wanted her to have a BNP test that measures the heart function. She also had her monthly CBC (complete blood count) liver function and pregnancy testing at the same time.
Friday was the hardest day for Nicole. She was in a lot of pain and even taking Advil and using ice, numbing cream, biofreeze etc., she still couldn't get relief. It isn't as painful if she doesn't walk or move around. It broke my heart to see her go through this. I spoke with the nurse at the doctor's office and found out that the BNP was normal which made me very, very happy. I wasn't so happy with her CBC though. Her platelet count had dropped to right above the critical stage again which could be from the new medicine but she hadn't even been on it 24 hours and she had some white blood issues this time as well as the normal red blood cell issues. I will call her cardiologist to see what/if I should be worried about this.
Saturday the local health nurse came out to help us with the pump change. It was evident when we took the tape off that Nicole was having a slight reaction to the tape and her skin was pink but it didn't itch or bother her. The nurse had a different tape and we are trying this to see if her skin will tolerate it better. The nurse also told me that I needed to give her the pain medication every six hours for the time being until such time as the site pain goes away. She will return on Tuesday for another pump change and drug increase.
Every three days the medicine has to be refilled and the pump has to be changed. About every two months the site itself will be changed. This is where the site pain comes into play. Every time the site is changed from about the third to tenth day afterwards there is a good chance of site pain. This is when the ice, gel, pain killers etc. come into play. I have been told that usually after this period this goes away. I can only hope.
Every time the pump is changed and the medicine is increased she has a chance of side effects which includes headaches, joint pain, jaw pain, nausea, diarrhea, dizziness etc., so far this hasn't happened yet but she is still at a very low dose. In addition, she is slowly being weaned off the inhaled medication which she will totally go off of in Sept. unless the doctor states otherwise.
My deepest desire is that this medication will improve her quality of life. Her doctor who is just absolutely wonderful in so many ways and I have so much gratitute toward him for many reasons told her on Wednesday how awesome she was and that he hoped this medicine would make her feel so much better so she would be able to see how awesome she was. Unfortunately, it isn't hard to see that Nicole doesn't have a lot of self-confidence in herself.
As stressful as this is for me I know how much more stressful it is for Nicole. I want to so much remain positive and hopeful that this will be the magic bullet but at the same time I don't want the hurt and disappointment if this doesn't work and she shows no improvement.
Friday, July 13, 2012
OB-GYN Appointment
I took Nicole for her first visit with a ob-gyn yesterday. Nicole has had anemia issues for almost two years now. On paper her hemoglobin looks normal and falls in normal ranges but for Nicole and her heart/lung issues her hemoglobin should run high and it doesn't. Her platelet count is consistently low and has been for the last two years at least. She takes a daily supplement of iron but it isn't helping to the extent that it should. Nicole tends to have heavy cycles on a regular basis. I see how tired and fatigued she gets, dizzy spells, cramps, headaches and just not feeling well during this week.
I am always very nervous when we have to go to a new doctor. I never know what to expect. Will the doctor listen to me, will the doctor know what PH is etc. It turns out that a resident was shadowing the doctor so we were actually seeing two doctors. She was very nice and very young. I was impressed when she knew that her VSD (ventricular septal defect, a congenital heart defect, a hole in the heart), PAH, and Eisenmenger's were all connected together. As with most doctors they are always surprised that her heart defect went undetected for so long. I explained about her anemia and the reason for the visit. She asked what her hemoglobin numbers were and I showed her the lab results but I had the May results along with the ones from previous months. I had, however, forgotten June's bloodwork. She looked at the numbers and looked at me thinking "what is the problem... these numbers are normal?" She asked me to leave for a minute so she could ask Nicole questions privately which I know they do as some girls are afraid to answer sensitive/private questions in front of their moms.
The resident left and consulted with the doctor. The doctor came in and was very nice. The first thing that impressed me was the fact she had contacted a friend of hers at University of Florida that deals with CHD's (congenital heart defects) to ask about her hemoglobin and this is how she knew that Nicole's hemoglobin although it was "normal" on paper wasn't normal. She explained about how we get our monthly cycles. She explained that Nicole can't take estrogen because of the risk of blood clots and with her health issues this is too big of an risk. She could give her progestin only which comes in severals methods like the Depo shot but the doctor didn't want Nicole taking something she couldn't take back out of her body. She has opted to start with the pill. I said I needed the approval of her cardiologist before I can give her any medication and she called her cardiologist in front of me and spoke to him. She told him that she believed that if she could stop her periods and with talk of possible iron infusions they could get her hemoglobin up and her anemia under control so she wouldn't be so fatigued.
I am very, very hopeful that this will be a godsend for Nicole. In some ways, Nicole taking birth control bothers me but I also know that because of Nicole's health issues combined with the fact that the medicine she takes causes serious birth defects she will never be able to have children of her own. If the pill works at getting her anemia in check this is the end goal.
I was also very impressed with the doctor and the compassion and understanding she showed Nicole. I am saddened that she is pediatric so she will probably only see Nicole until she is 21 since she is under the CMS (children's medical services) program. I will fill the prescription tomorrow and Nicole will start taking the pill. It is so hard to keep track of all these symptoms and issues but I know I must be more vigilant about this. I had a great idea on how to do this and hope that this will be simple so I will follow through. Nicole will go back in Sept. for a follow up. The doctor will start with the minimum dose and adjust it accordingly.
Both the resident and the doctor listened to Nicole's heart beat and agreed she has no murmur which is why the doctor's never suspected a heart defect. Nicole has a large VSD which is why there's no murmur. Even her diagnosing cardiologist would never of suspected without an EKG followed by an echo that she had a heart defect.
I am always very nervous when we have to go to a new doctor. I never know what to expect. Will the doctor listen to me, will the doctor know what PH is etc. It turns out that a resident was shadowing the doctor so we were actually seeing two doctors. She was very nice and very young. I was impressed when she knew that her VSD (ventricular septal defect, a congenital heart defect, a hole in the heart), PAH, and Eisenmenger's were all connected together. As with most doctors they are always surprised that her heart defect went undetected for so long. I explained about her anemia and the reason for the visit. She asked what her hemoglobin numbers were and I showed her the lab results but I had the May results along with the ones from previous months. I had, however, forgotten June's bloodwork. She looked at the numbers and looked at me thinking "what is the problem... these numbers are normal?" She asked me to leave for a minute so she could ask Nicole questions privately which I know they do as some girls are afraid to answer sensitive/private questions in front of their moms.
The resident left and consulted with the doctor. The doctor came in and was very nice. The first thing that impressed me was the fact she had contacted a friend of hers at University of Florida that deals with CHD's (congenital heart defects) to ask about her hemoglobin and this is how she knew that Nicole's hemoglobin although it was "normal" on paper wasn't normal. She explained about how we get our monthly cycles. She explained that Nicole can't take estrogen because of the risk of blood clots and with her health issues this is too big of an risk. She could give her progestin only which comes in severals methods like the Depo shot but the doctor didn't want Nicole taking something she couldn't take back out of her body. She has opted to start with the pill. I said I needed the approval of her cardiologist before I can give her any medication and she called her cardiologist in front of me and spoke to him. She told him that she believed that if she could stop her periods and with talk of possible iron infusions they could get her hemoglobin up and her anemia under control so she wouldn't be so fatigued.
I am very, very hopeful that this will be a godsend for Nicole. In some ways, Nicole taking birth control bothers me but I also know that because of Nicole's health issues combined with the fact that the medicine she takes causes serious birth defects she will never be able to have children of her own. If the pill works at getting her anemia in check this is the end goal.
I was also very impressed with the doctor and the compassion and understanding she showed Nicole. I am saddened that she is pediatric so she will probably only see Nicole until she is 21 since she is under the CMS (children's medical services) program. I will fill the prescription tomorrow and Nicole will start taking the pill. It is so hard to keep track of all these symptoms and issues but I know I must be more vigilant about this. I had a great idea on how to do this and hope that this will be simple so I will follow through. Nicole will go back in Sept. for a follow up. The doctor will start with the minimum dose and adjust it accordingly.
Both the resident and the doctor listened to Nicole's heart beat and agreed she has no murmur which is why the doctor's never suspected a heart defect. Nicole has a large VSD which is why there's no murmur. Even her diagnosing cardiologist would never of suspected without an EKG followed by an echo that she had a heart defect.
Wednesday, July 4, 2012
Cardiology appointment
Nicole had her three month cardiology appointment on July 3rd. Normally, she has an echo done at these appointments but since she had a heart cath on June 15th they didn't do one.
Nicole's doctor had gone to the PHA conference and said he learned a lot. He spoke with several doctor's regarding Nicole's case and her issues with her low platelet count and anemia. He got a lot of good information.
He has wanted a blood transfusion or something that would help out with her fatigue from the anemia and I believe he is now leaning toward an IV iron treatment as opposed to a blood transfusion as this could be detrimental to a lung transplant in the future. He was going to speak with some hematologist in the area and find another one for Nicole to see as the one she has seen several times just doesn't know what to do with her. He is going to consult with her PH specialist to see what he thinks about the IV iron. Nicole does take daily iron but she can only tolerate one pill a day. I asked the doctor if she continued to take two a day would the constipation issue get better like some medications that you are on for awhile the side effects will go away and he didn't know the answer to this.
We also talked about options like coumadin and baby aspirin and whether or not it would be beneficial to Nicole. He doesn't believe that she is at risk for clotting which is why coumadin is given and it only seems to help those who have PH from a blood clotting disorder or have a history of clots. He does, however, want her to start taking one baby aspirin a day. He doesn't know if this will show improvement but he doesn't feel at this time coumadin is right in her case. So when I go shopping on Saturday I will buy some aspirin and add this to her daily medication.
I was surprised to see that the report was in from the cardiologist that performed the cath. I have a copy of it but haven't read it and really don't know if I'm up for it yet. These reports from the doctor's always kick me hard in the gut added to that just seeing the cardiologist/PH specialist and the emotion this involves. I asked him what her PVR pressure was and he said is was 16.2 and her STR was 16.1. I know from the conference and one of the sessions that I went to that normal PVR pressure is 1. When nitrite oxide and O2 were added it went up to 19.2 (PVR) and 20.4 (STR) and with O2 it went to 24 (PVR) and 19.2 (STR).
I am always conscious of what I say because Nicole is in the room and even though she is 17 she already goes through enough pain and anxiety just surviving everyday I don't want to add to that. I did ask out right if there has been any improvement since her initial cath and he said "no". This is why she is being switched from Tyvaso to Remodulin. I didn't have the nerve, especially with Nicole in the room, to ask if she has gotten worse because the disease has progressed. I would like to think that isn't the case but I am also realistic. I am terrified of the prospect of her going on Remodulin either the SubQ or the IV but I know that this must be done as her heart function is okay and is tolerating the high lung pressures so the PH must be fought aggressively but I will ask her PH specialist what are the odds that these will show any improvement as so far we have seen none.
I am so grateful to her cardiologist as he has done a lot of research about remodulin (none of his PH patients take any of the continuous meds) and he has talked to countless doctors regarding Nicole's case to find out what is the best possible treatment for her. This really says a lot to me. Her PH specialist who I spoke with at conference also told me that he had spoken with a doctor at Baylor about Nicole and that he agreed that the best option at this point is switching her to Remodulin.
I am very disappointed with the outcome of the cath to say anything else would be a lie but I must continue to believe for a cure, a new medication, a new procedure, and/or for the remodulin to at least show an improvement. I also can not and will not give up hope that someday Nicole will be healthier even if she still has this disease. Even on those dark days when I go down the road of fear with Nicole being terminally ill and the thought of losing her everyday I must remember to be grateful for the cardiologist, the PH specialist, PHA, UT, PHriends and family for all that they have done and will continue to do for Nicole.
Nicole's doctor had gone to the PHA conference and said he learned a lot. He spoke with several doctor's regarding Nicole's case and her issues with her low platelet count and anemia. He got a lot of good information.
He has wanted a blood transfusion or something that would help out with her fatigue from the anemia and I believe he is now leaning toward an IV iron treatment as opposed to a blood transfusion as this could be detrimental to a lung transplant in the future. He was going to speak with some hematologist in the area and find another one for Nicole to see as the one she has seen several times just doesn't know what to do with her. He is going to consult with her PH specialist to see what he thinks about the IV iron. Nicole does take daily iron but she can only tolerate one pill a day. I asked the doctor if she continued to take two a day would the constipation issue get better like some medications that you are on for awhile the side effects will go away and he didn't know the answer to this.
We also talked about options like coumadin and baby aspirin and whether or not it would be beneficial to Nicole. He doesn't believe that she is at risk for clotting which is why coumadin is given and it only seems to help those who have PH from a blood clotting disorder or have a history of clots. He does, however, want her to start taking one baby aspirin a day. He doesn't know if this will show improvement but he doesn't feel at this time coumadin is right in her case. So when I go shopping on Saturday I will buy some aspirin and add this to her daily medication.
I was surprised to see that the report was in from the cardiologist that performed the cath. I have a copy of it but haven't read it and really don't know if I'm up for it yet. These reports from the doctor's always kick me hard in the gut added to that just seeing the cardiologist/PH specialist and the emotion this involves. I asked him what her PVR pressure was and he said is was 16.2 and her STR was 16.1. I know from the conference and one of the sessions that I went to that normal PVR pressure is 1. When nitrite oxide and O2 were added it went up to 19.2 (PVR) and 20.4 (STR) and with O2 it went to 24 (PVR) and 19.2 (STR).
I am always conscious of what I say because Nicole is in the room and even though she is 17 she already goes through enough pain and anxiety just surviving everyday I don't want to add to that. I did ask out right if there has been any improvement since her initial cath and he said "no". This is why she is being switched from Tyvaso to Remodulin. I didn't have the nerve, especially with Nicole in the room, to ask if she has gotten worse because the disease has progressed. I would like to think that isn't the case but I am also realistic. I am terrified of the prospect of her going on Remodulin either the SubQ or the IV but I know that this must be done as her heart function is okay and is tolerating the high lung pressures so the PH must be fought aggressively but I will ask her PH specialist what are the odds that these will show any improvement as so far we have seen none.
I am so grateful to her cardiologist as he has done a lot of research about remodulin (none of his PH patients take any of the continuous meds) and he has talked to countless doctors regarding Nicole's case to find out what is the best possible treatment for her. This really says a lot to me. Her PH specialist who I spoke with at conference also told me that he had spoken with a doctor at Baylor about Nicole and that he agreed that the best option at this point is switching her to Remodulin.
I am very disappointed with the outcome of the cath to say anything else would be a lie but I must continue to believe for a cure, a new medication, a new procedure, and/or for the remodulin to at least show an improvement. I also can not and will not give up hope that someday Nicole will be healthier even if she still has this disease. Even on those dark days when I go down the road of fear with Nicole being terminally ill and the thought of losing her everyday I must remember to be grateful for the cardiologist, the PH specialist, PHA, UT, PHriends and family for all that they have done and will continue to do for Nicole.
Sunday, June 17, 2012
Nicole's heart cath
I
just wanted to post an update on Nicole's surgery. She had her heart
cath Friday, June 15, 2012 it was scheduled for noon but didn't go in until
about 1:30 p.m. She did very well with the anxiety ... I knew how
scared she was especially when we had to say goodbye as they took her to
the cath lab.
The
procedure took about 1 1/2 - 2 hours so Rick and I went to the
cafeteria for lunch and then waited in the waiting area. They called us
twice to let us know how she was doing. The doctor's came in after
her procedure. The cardiologist who did the procedure said that her
heart looked fine and that her holes were pronounced and everything from
that standpoint looked as expected.
The
other doctor (her PH specialist) talked about her lungs. He said her
pressures in her lungs are the equivalent of her systolic blood pressure
but I'm not sure if he means the normal bp or her bp but they are
extremely high as the pressure in your lungs should be no more than 25.
This is as it was 2 1/2 years ago. He isn't happy with the results of
the inhaled drug (Tyvaso) that she has been taking for the last year and a half
which has the same medicine that she will be switching too but at a much
lower dose. Nicole will be switching medication (Remodulin) and she will be put
on continuous medication via a subcutaneous (SubQ) sight as the IV/PICC
line route has more risks with air getting in the line which is deadly
for someone with a hole in the heart. This medication is the last step
and once this stops working there will be no other options for her
except for a lung transplant unless they come out with some new more
powerful drugs or there's a cure.
I
am nervous about switching as I will have to learn all about a new
medicine and be trained by the drug manufacturer as I did with her
inhaled. I am concerned as the sub cutaneous method which supplies
continuous medicine through the skin is very painful at the site and I
don't want to see Nicole in pain. She is too scared at this point of
the IV. At the hospital her anxiety was extremely high
when they had to put the IV in her hand. I had to hold her other hand
and keep her still. The switch over should happen the end of July or
beginning of August. Fortunately, the SubQ doesn't require a hospital
visit only a doctor's visit to start. The only issue that the doctor is concerned with is the fact that Nicole is so thin.
I
was disappointed with the results but they were what I expected. They
have told us from the beginning that her pressures will not come down
with the hole in the heart but the hole can't be corrected because of
her pressures. It is a catch 22. I am extremely grateful and I don't
think I really realized this until Friday that even though it was
gross negligence on behave of the doctor's etc. that Nicole's congenital
heart defect wasn't diagnosed it is a true blessing because it is
because of that defect that has kept the PH from progressing like it
would normally. If it wasn't for the heart defect we would have probably lost
her already and for that I am extremely grateful.
We
got home about 9:30 a.m. Saturday morning. I spent the night with Nicole
in her room. She didn't get much more than a hours worth of sleep and I
may have gotten a couple more than that. They woke us up at 4:30 a.m.
and kicked me out of the room to take a chest xray in her bed which was
required for discharge. Nicole didn't get back to sleep after this and
was only the first or second time in her life that she has seen the sun
come up.
Going
forward I will pray that while the new medicine may not lower her
pressures I will have to rely on it improving her quality of life which
is a statement that I dislike very much. I will be happy and grateful
if she has more energy and isn't as fatigued all the time. I pray that
she will tolerate the site pain without pain killers and she will
tolerate the medicine with the least amount of side effects. The PH
specialist told Nicole at her initial appointment in Feb. that he
believed that he could make her feel better. He asked her what her top three wishes were in regards to her illness and the top one was her fatigue level. I told him Friday that
Nicole and I were going to hold him to that. He laughed and said "oh
boy, I'm in trouble now".
Thank you for your thoughts, prayers, emails, facebook messages etc. I can't tell you how grateful I am.
Wednesday, February 29, 2012
Today is Rare Disease Day...
The last day of February is Rare Disease Day. A rare disease is one that affects fewer than 200,000 people which is defined by the Orphan Drug Act of 1983.
There's nearly 7,000 rare diseases affecting nearly 30 million Americans. 1 in 10 Americans suffer from a rare disease.
My daughter, Nicole, has more than one rare disease. She has pulmonary arterial hypertension (PAH; PH) which is high blood pressure within the arteries of her lungs that can lead to heart failure. She has Eisenmenger's Syndrome which is a rare progressive heart condition that develops from some congenital heart defects (her defect is one of them) . This disorder is characterized by the increased blood pressure in the main pulmonary artery connecting the heart to the lungs and improper blood flow to the heart. She also has benign hypermobility joint syndrome which in some medical circles is Ehlers-Danlos Syndrome (EDS) which is a group of hereditary connective tissue disorders. The only way to get a true diagnosis of EDS is through genetic testing.
I didn't realize so many of the diseases that I had heard of are rare diseases such as Scleroderma; Cystic Fibrosis, Lupus, Multiple Sclerosis just to name a few.
I have changed my profile picture on Facebook to the perwinkle ribbon which is the symbol for PH in honor of Rare Disease Day and Nicole to hopefully make people more aware of some of these and the need for research so cures can be found for my daughter and for all the others with rare diseases.
There's nearly 7,000 rare diseases affecting nearly 30 million Americans. 1 in 10 Americans suffer from a rare disease.
My daughter, Nicole, has more than one rare disease. She has pulmonary arterial hypertension (PAH; PH) which is high blood pressure within the arteries of her lungs that can lead to heart failure. She has Eisenmenger's Syndrome which is a rare progressive heart condition that develops from some congenital heart defects (her defect is one of them) . This disorder is characterized by the increased blood pressure in the main pulmonary artery connecting the heart to the lungs and improper blood flow to the heart. She also has benign hypermobility joint syndrome which in some medical circles is Ehlers-Danlos Syndrome (EDS) which is a group of hereditary connective tissue disorders. The only way to get a true diagnosis of EDS is through genetic testing.
I didn't realize so many of the diseases that I had heard of are rare diseases such as Scleroderma; Cystic Fibrosis, Lupus, Multiple Sclerosis just to name a few.
I have changed my profile picture on Facebook to the perwinkle ribbon which is the symbol for PH in honor of Rare Disease Day and Nicole to hopefully make people more aware of some of these and the need for research so cures can be found for my daughter and for all the others with rare diseases.
Saturday, February 25, 2012
Nicole's Narrative
Nicole had to write a narrative for school that relates to a real life experiences that teaches or show a lesson. She finally got it done. I think it is very good but also saddens me at the same time. I did get her permission to share it. I do homeschool Nicole and she is enrolled in an accredited Christian homeschooling academy.
1.
I feel invisible sometimes.
It makes me very visible in many ways.
People judge me because of this invisibility, but that's because they don't understand. They of course don't see it.
What am I referring to? My illness.
This illness is very invisible to others, and this makes people judge me.
"Why is she just sitting out when we're all working? She must be very lazy."
"Why does she have a handicap placard? She doesn't need it."
"Why does she have to have special treatment? She looks fine to me."
These thoughts, although understandable, are hurtful.
I know people think these thoughts about me. Why shouldn't they? It's human nature. I'm guilty of doing the same thing sometimes.
However, one shouldn't judge. Just because somebody is different from you doesn't mean that they're necessarily bad. This concept that people have is called prejudice.
Again, it's human nature to have these thoughts. I consider myself prejudiced about some things myself.
However, I shouldn't be. Nobody should be.
I feel invisible sometimes.
It makes me very visible in many ways.
People judge me because of this invisibility, but that's because they don't understand. They of course don't see it.
What am I referring to? My illness.
This illness is very invisible to others, and this makes people judge me.
"Why is she just sitting out when we're all working? She must be very lazy."
"Why does she have a handicap placard? She doesn't need it."
"Why does she have to have special treatment? She looks fine to me."
These thoughts, although understandable, are hurtful.
I know people think these thoughts about me. Why shouldn't they? It's human nature. I'm guilty of doing the same thing sometimes.
However, one shouldn't judge. Just because somebody is different from you doesn't mean that they're necessarily bad. This concept that people have is called prejudice.
Again, it's human nature to have these thoughts. I consider myself prejudiced about some things myself.
However, I shouldn't be. Nobody should be.
Sometimes my disease is visible though. The thoughts are completely different then. They express sympathy more than prejudice.
"Why does a teenager have oxygen? It's sad that there's something wrong with her."
Or, they might be prejudice. I don't know.
"Why does a teenager have oxygen? It's sad that there's something wrong with her."
Or, they might be prejudice. I don't know.
2
Doctors are morons.
Why, you ask?
I don't know. I just know they are.
You might be thinking it's very negative of me to be thinking something like that. You might be right, but, no matter how right you are, it doesn't change the fact.
I've been misdiagnosed left and right.
"Oh, you have joint pain? Must be growing pains."
"Oh, you have shortness of breath? Must be asthma."
"Oh, the shortness of breath isn't getting better? Must be a stamina issue. You still have asthma by the way."
I've even been misdiagnosed when the results of a serious illness have been staring the doctors plain in the face.
I had a chest x-ray when I was four. Nobody saw any signs of something being wrong with my cardiopulmonary system.
I was sick all the time as a young child. Everyone thought it was normal when it probably wasn't.
I've been to a pulmonologist who should have seen something wrong. He never did.
I had an EKG when I was seven. The cardiologist who read it signed if off as normal when it would have never been normal.
I've had purple fingernails all my life. Nobody knew it meant something.
Only when my mom got a tiny bit more involved did the doctors realize that something was wrong.
Doctors are morons.
Why, you ask?
I don't know. I just know they are.
You might be thinking it's very negative of me to be thinking something like that. You might be right, but, no matter how right you are, it doesn't change the fact.
I've been misdiagnosed left and right.
"Oh, you have joint pain? Must be growing pains."
"Oh, you have shortness of breath? Must be asthma."
"Oh, the shortness of breath isn't getting better? Must be a stamina issue. You still have asthma by the way."
I've even been misdiagnosed when the results of a serious illness have been staring the doctors plain in the face.
I had a chest x-ray when I was four. Nobody saw any signs of something being wrong with my cardiopulmonary system.
I was sick all the time as a young child. Everyone thought it was normal when it probably wasn't.
I've been to a pulmonologist who should have seen something wrong. He never did.
I had an EKG when I was seven. The cardiologist who read it signed if off as normal when it would have never been normal.
I've had purple fingernails all my life. Nobody knew it meant something.
Only when my mom got a tiny bit more involved did the doctors realize that something was wrong.
Doctors only use you for money.
They don't really listen to patients and caregivers sometimes. Half of the time the doctors think that they are just being paranoid.
They don't really listen to patients and caregivers sometimes. Half of the time the doctors think that they are just being paranoid.
3
Why do people have to ask questions?
They're curious, of course. They want to know more than they already know.
But sometimes questions are very annoying.
And why do people assume?
They're just ignorant. I don't think there's any other explanation to this.
A lot of times assumptions hurt though.
"Are you going to die if you do this?"
"Are you going to die if you do that?"
"You're gonna die if you miss one dose of meds, aren't you?"
"You wouldn't be able to handle doing this, so I'm not going to invite you to do this."
"You're just going to hold us back if you come along with us."
I realize that I am a bit of a burden sometimes with my frequent resting and medicine schedule, but you shouldn't assume that.
Just because I might hold you back doesn't mean that you and I won't have a good time.
I'm sure you just don't want to be seen with a chronically ill person.
Why do people have to ask questions?
They're curious, of course. They want to know more than they already know.
But sometimes questions are very annoying.
And why do people assume?
They're just ignorant. I don't think there's any other explanation to this.
A lot of times assumptions hurt though.
"Are you going to die if you do this?"
"Are you going to die if you do that?"
"You're gonna die if you miss one dose of meds, aren't you?"
"You wouldn't be able to handle doing this, so I'm not going to invite you to do this."
"You're just going to hold us back if you come along with us."
I realize that I am a bit of a burden sometimes with my frequent resting and medicine schedule, but you shouldn't assume that.
Just because I might hold you back doesn't mean that you and I won't have a good time.
I'm sure you just don't want to be seen with a chronically ill person.
4
Sometimes you gotta fight.
Fight for what you need.
A lot of people don't fight. It could be because they're afraid to, or simply because they don't want to.
But if you do fight, you have a good chance of getting more than if you don't fight.
My mom always fights for me. For medication, authorization, insurance, doctors and a lot of other things.
She did hit obstacles along the way, but that didn't stop her.
She just kept going.
And sometimes, we get more than we expect.
Like that one time we got a trip to Hawaii because my mom fought for payment help with a new medication.
She was able to single-handedly change the distributor's policy, and I was honored to be patient of the year. With that, we got to go to the Global Sales Meeting in Honolulu, Hawaii.
That was pretty rewarding. I'm sure it especially was for her.
So sometimes, when you fight, you not only get what you want, you get much, much more.
And plus, somebody's gotta give those stubborn people a challenge.
Sometimes you gotta fight.
Fight for what you need.
A lot of people don't fight. It could be because they're afraid to, or simply because they don't want to.
But if you do fight, you have a good chance of getting more than if you don't fight.
My mom always fights for me. For medication, authorization, insurance, doctors and a lot of other things.
She did hit obstacles along the way, but that didn't stop her.
She just kept going.
And sometimes, we get more than we expect.
Like that one time we got a trip to Hawaii because my mom fought for payment help with a new medication.
She was able to single-handedly change the distributor's policy, and I was honored to be patient of the year. With that, we got to go to the Global Sales Meeting in Honolulu, Hawaii.
That was pretty rewarding. I'm sure it especially was for her.
So sometimes, when you fight, you not only get what you want, you get much, much more.
And plus, somebody's gotta give those stubborn people a challenge.
5
Friends are amazing.
They care about you and love you.
They help you, listen to you, and encourage you.
Or at least that's what they should do.
Friends are amazing.
They care about you and love you.
They help you, listen to you, and encourage you.
Or at least that's what they should do.
Friends that are in similar situations than you are also amazing.
They have more understanding of what you are going through so they can help you better.
They might even be able to fight with you.
They have more understanding of what you are going through so they can help you better.
They might even be able to fight with you.
Lessons
People are prejudiced
Doctors are morons
People are ignorant
Determination is a virtue
Friends are amazing
People are prejudiced
Doctors are morons
People are ignorant
Determination is a virtue
Friends are amazing
I guess the fourth point offsets the first three because fighting can turn all of those people and doctors into smart people.
And the fifth one is special in that it encourages the fourth point.
And the fifth one is special in that it encourages the fourth point.
Of course, I don't speak for everyone on this.
Being prejudice and ignorant is just part of human nature.
Be idiotic might be too. Sometimes it seems that way.
Not all friends are helpful and good listeners.
But I think the fourth point is true in pretty much all situations. That point is probably the best point of the series.
Being prejudice and ignorant is just part of human nature.
Be idiotic might be too. Sometimes it seems that way.
Not all friends are helpful and good listeners.
But I think the fourth point is true in pretty much all situations. That point is probably the best point of the series.
I am so proud of Nicole for writing this paper. I am even more proud that she believes number four outweighed all the negative things. I have tried to impress this upon her. Yes, I know we have been through a lot of negative and bad things but there are good things and good people that we wouldn't have if it wasn't for those bad things.
Saturday, February 11, 2012
A Second Opinion...
I decided that I wanted a second opinion about Nicole's lung disease pulmonary arterial hypertension as I have had reservations about the care she's receiving from her current ph specialist.
I meant one of the local PH specialist at the local support group meeting and he agreed to see Nicole. His office doesn't take Medicaid but he said he would run it through for her. I talked to children's medical services (CMS) which is the Medicaid plan Nicole has. I was told if her cardiologist sent in some letters stating why it was necessary for Nicole to see him they would take it under advisement and consider covering an out-of-network doctor. I had another nurse after her appointment tell me this wasn't true and they don't pay for any out-of-network doctor's.
Nicole has severe PH caused by a large VSD that wasn't diagnosed until 2 years ago when she was also diagnosed with PH. He said he agreed with the diagnosis. He only had the records from the cardiologist and not the PH specialist. He said that from what he sees at the time of diagnosis she was a borderline Stage 4 (this is the worst stage) and he thinks that she has probably dropped back down to a little above a Stage 3 since her drug therapy has started. He said as well as her other drs. have said that the pressures in her pulmonary arteries will not come down until her heart defect is corrected and her defect can't be corrected because of the PH. He said that the PH was the much more serious of the two. He said he tends to be a little more aggressive and he would have her on Remodulin IV pump with the hopes of her taking part in a study with an implantable pump that is inside the abdomen area. She would be a good candidate for this because of her heart defect. She would need to be on a stable dose (basically this means the dose that they want her on long term as they have to start at a low level and work up) for four months before she could participate. The only issue I have with this is that there are some more complications and it isn't as easy as Tyvaso. It also has to be implanted in the hospital whether it is the IV through a cathedar or the implantable. He did mention something about air getting in the line can be bad for a heart defect. He also said because of her heart defect her PH has progressed very slowly and more than likely she has had it most of her life. He was also concerned that she hasn't had a catherization (this is the most accurate way of obtaining accurate pulmonary pressures but also the most invasive) other than her initial one diagnosing her so there's no information to compare after she's been on therapy. He also said that she would need a lung transplant at some point down the road. I probably would have asked more prognosis related things but Nicole was there and I don't want her to be upset.
She also did a 6MW ( a test that is used to measure the exercise ability it is where you walk for 6 minutes and your distance along with you oxygen saturation levels are measured). She was on 6 LPM and still her O2 stats fell at least twice below 78% (normal is 98% which Nicole rarely hits 90% and this is called cynosis which is blueness) and the nurse made her stop until it went back up. So needless to say she didn't do well. They were also surprised when I said when she had her last one in Oct. it was without O2. He said (contrary to her current PH specialist) that her O2 stats should never be below 85% and she needs to be on whatever O2 that keeps her there. The current PH doctor had told her that she didn't need to be on 8 LPM (liters per minute) at rehab and it was alright if the stats dropped.
Since this appointment she has gone to wearing a mask at rehab which is much better at hi-flow oxygen (anything above 6 LPM) than the cannula's. She is using 12 LPM and it is still hard to keep her stats above 85. Her time and intensity has to be backed down.
I'm not sure what to make of the second opinion. I really like the doctor and he is excellent with Nicole and I feel like even though she is very sick there is still hope I don't feel that most of the time with the other doctor. I don't know if she will be able to switch from Tyvaso to Remodulin because her current PH doctor has almost no chance of going along with this.
I am trying to figure out how to proceed. There are not a lot of ph specialist to begin with and then a lot of adult doctors don't take Medicaid. I know of another group of doctor's here locally (they are pulmonary, not cardiac) but they won't see her until she's 18. The cardiologist would like Nicole to have a transfusion due to her hemoglobin issues and also put her on a hormone that the kidney produces (I can't say it and don't know how to spell it off the top of my head as it is very long) that regulates the amount of red blood cells in the blood in hopes of raising her hemoglobin and well as her oxygen staturation levels. I don't know when or if this will happen.
The ph specialist she saw last week and her cardiologist have been playing telephone tag with each other so I am hoping that they will talk soon so I know what we are looking at.
I feel better with a second opinion but it doesn't really change anything about her diagnosis. The doctor asked Nicole what are the top three wishes she had in regards to getting rid of... these were fatigue, shortness of breath and joint pain (not sure how much of this is related to drug side effects and how much is her BHJS/EDS).
I have to believe that God will provide the right doctor and treatment that Nicole needs. I hope that he will give me the wisdom to know what is best for her with minimal risks and side effects. I have tried to get quiet about this but it is hard when there doesn't seem to be any options but what we already have at this point.
Saturday, February 4, 2012
February is CHD Awareness Month
February is CHD Awareness month. My daughter, Nicole, was born with a congenital heart defect but we didn't know it until she was 15 years old. CHD awareness week is Feb. 7th - 14th and I will write more about Nicole's story regarding her CHD at this time but in the mean time here are some facts regarding CHD's:
•Congenital heart defects are America’s and every country’s #1 birth defect. Nearly one of every 100 babies is born with a CHD.
•Congenital heart defects are the #1 cause of birth defect related deaths.
•Congenital heart defects are the leading cause of all infant deaths in the United States.
•Each year approximately 40,000 babies are born in the United States with a congenital heart defect. Thousands of them will not reach their first birthday and thousands more die before they reach adulthood.
•Each year over 1,000,000 babies are born worldwide with a congenital heart defect. 100,000 of them will not live to see their first birthday and thousands more die before they reach adulthood.
Lifelong Disease
•Almost half all children and adults with complex congenital heart disease have neurological and developmental disabilities.
•There are an estimated 2,000,000 CHD survivors in the United States.
•For the first time, more than 50% of the CHD survivors are adults.
•10% of all CHD cases evaluated in an Adult CHD clinic are first diagnosed in adulthood.
Economic Factors
•91,000 life years are lost each year in this country due to congenital heart defects.
•The cost for inpatient surgery to repair congenital heart defects exceeds $2.2 billion a year.
General CHD FACTS
•More than 50% of all children born with congenital heart defect will require at least one invasive surgery in their lifetime.
•There are more than 40 different types of congenital heart defects. Little is known about the cause of most of them. There is no known prevention or cure for any of them.
•In the United States, twice as many children die from congenital heart defects each year than from all forms of childhood cancer combined, yet funding for pediatric cancer research is five times higher than funding for CHD.
Research Allocations & Impact
•Congenital heart defects are common and deadly, yet CHD research is grossly under-funded relative to the prevalence of the disease.
•Only one penny of every dollar donated to the American Heart Association goes towards congenital heart defect research.
•Of every dollar the government spends on medical funding only a fraction of a penny is directed toward congenital heart defect research.
•The NHLBI has stated that Congenital Heart Defects are a serious and under appreciated global health problem.
•In the last decade death rates for congenital heart defects have declined by almost 30% due to advances made through research.
•Congenital heart defects are America’s and every country’s #1 birth defect. Nearly one of every 100 babies is born with a CHD.
•Congenital heart defects are the #1 cause of birth defect related deaths.
•Congenital heart defects are the leading cause of all infant deaths in the United States.
•Each year approximately 40,000 babies are born in the United States with a congenital heart defect. Thousands of them will not reach their first birthday and thousands more die before they reach adulthood.
•Each year over 1,000,000 babies are born worldwide with a congenital heart defect. 100,000 of them will not live to see their first birthday and thousands more die before they reach adulthood.
Lifelong Disease
•Almost half all children and adults with complex congenital heart disease have neurological and developmental disabilities.
•There are an estimated 2,000,000 CHD survivors in the United States.
•For the first time, more than 50% of the CHD survivors are adults.
•10% of all CHD cases evaluated in an Adult CHD clinic are first diagnosed in adulthood.
Economic Factors
•91,000 life years are lost each year in this country due to congenital heart defects.
•The cost for inpatient surgery to repair congenital heart defects exceeds $2.2 billion a year.
General CHD FACTS
•More than 50% of all children born with congenital heart defect will require at least one invasive surgery in their lifetime.
•There are more than 40 different types of congenital heart defects. Little is known about the cause of most of them. There is no known prevention or cure for any of them.
•In the United States, twice as many children die from congenital heart defects each year than from all forms of childhood cancer combined, yet funding for pediatric cancer research is five times higher than funding for CHD.
Research Allocations & Impact
•Congenital heart defects are common and deadly, yet CHD research is grossly under-funded relative to the prevalence of the disease.
•Only one penny of every dollar donated to the American Heart Association goes towards congenital heart defect research.
•Of every dollar the government spends on medical funding only a fraction of a penny is directed toward congenital heart defect research.
•The NHLBI has stated that Congenital Heart Defects are a serious and under appreciated global health problem.
•In the last decade death rates for congenital heart defects have declined by almost 30% due to advances made through research.
Sunday, January 22, 2012
January 23, 2010
This is a day I will never forget… just like the day I got married, the day my mom died, the day Nicole was born and many other important days in my life whether good or bad.
It was this day that changed our lives forever. It was this day when we found out that our precious daughter had been born with a congenital heart defect and because it wasn’t diagnosed for 15 years it led to another rare heart condition as well as a rare lung disease. At this point, there is nothing that can be done to correct the heart defect because of her lung disease and unless the heart defect is correct it will not help her lung disease. All they can do is give her a cocktail of medication that will slow down and hopefully stop (for now) the progression of her lung disease but at this time will not cure it. This event started off a domino effect. It is very apparent that Rick lost his job because of Nicole’s health issues but of course we have no proof of this.
The Bible says that all things work for the good for those that love the Lord. I say this over and over and still don’t see how some of the difficulties we have had since this day have worked out for the good. It has been a struggle for me to come to terms with the fact that my daughter isn’t going to get better she remains stable and I am so very grateful for this but she won’t get better which is what I want more than anything else in this world. I am not strong enough to go through what the doctor’s have told me she will go through with this illness.
I have struggled with being content for a long time it just never seems like I am happy with what I have. I was watching Joyce Meyer the other day and she said “Contentment doesn’t come from what you own it comes from your relationship with God.” I never thought of it that way… maybe it isn’t the stuff I am discontented with and it is God because he has done this to my daughter and my family. She also said “contentment comes from no longer needing to impress people” I haven’t arrived there yet but I will strive for that.
I won a book of my choice from Denise Brown from caregiving.com and I chose “Take Time” I was looking at it and I have to rate my level of happiness and I have been thinking about this since Friday when I received the book. I don’t think that I’m that unhappy I just think that I’m discontent and I feel that there must be more. I feel that I finally know my calling which is to be a voice for my daughter, her illnesses, and for caregivers and my vision of these things have not happened the way that I want and/or thought they would. It is so hard to explain.
I hope that I will be able to stay positive on this day and not try to look at it as the day my life (and my family’s) fell apart even though it is. My mom passed away in April 1998 (she was diagnosed, had surgery and died during this month) and I can tell you every year I would say I hate April and then in April of 2009 I found out I had carcinoma in-situ and I thought you know I can’t continue to hate April. I have to let it go and you know in 2010 I was sad on the day of my mom’s passing but I didn’t focus on the other bad days in April.
Thank you for listening to me ramble and I hope that I made some sense.
Hugs:o)
Jane ~ mom to Nicole, 17 yo, VSD, PAH, Eisenmengers, BHJS
"If you don't like something change it, if you can't change it change the way you think about it."
It was this day that changed our lives forever. It was this day when we found out that our precious daughter had been born with a congenital heart defect and because it wasn’t diagnosed for 15 years it led to another rare heart condition as well as a rare lung disease. At this point, there is nothing that can be done to correct the heart defect because of her lung disease and unless the heart defect is correct it will not help her lung disease. All they can do is give her a cocktail of medication that will slow down and hopefully stop (for now) the progression of her lung disease but at this time will not cure it. This event started off a domino effect. It is very apparent that Rick lost his job because of Nicole’s health issues but of course we have no proof of this.
The Bible says that all things work for the good for those that love the Lord. I say this over and over and still don’t see how some of the difficulties we have had since this day have worked out for the good. It has been a struggle for me to come to terms with the fact that my daughter isn’t going to get better she remains stable and I am so very grateful for this but she won’t get better which is what I want more than anything else in this world. I am not strong enough to go through what the doctor’s have told me she will go through with this illness.
I have struggled with being content for a long time it just never seems like I am happy with what I have. I was watching Joyce Meyer the other day and she said “Contentment doesn’t come from what you own it comes from your relationship with God.” I never thought of it that way… maybe it isn’t the stuff I am discontented with and it is God because he has done this to my daughter and my family. She also said “contentment comes from no longer needing to impress people” I haven’t arrived there yet but I will strive for that.
I won a book of my choice from Denise Brown from caregiving.com and I chose “Take Time” I was looking at it and I have to rate my level of happiness and I have been thinking about this since Friday when I received the book. I don’t think that I’m that unhappy I just think that I’m discontent and I feel that there must be more. I feel that I finally know my calling which is to be a voice for my daughter, her illnesses, and for caregivers and my vision of these things have not happened the way that I want and/or thought they would. It is so hard to explain.
I hope that I will be able to stay positive on this day and not try to look at it as the day my life (and my family’s) fell apart even though it is. My mom passed away in April 1998 (she was diagnosed, had surgery and died during this month) and I can tell you every year I would say I hate April and then in April of 2009 I found out I had carcinoma in-situ and I thought you know I can’t continue to hate April. I have to let it go and you know in 2010 I was sad on the day of my mom’s passing but I didn’t focus on the other bad days in April.
Thank you for listening to me ramble and I hope that I made some sense.
Hugs:o)
Jane ~ mom to Nicole, 17 yo, VSD, PAH, Eisenmengers, BHJS
"If you don't like something change it, if you can't change it change the way you think about it."
Sunday, January 15, 2012
2011
Trish inspired me by her post about 2011 on caregiving.com. I can brag and say that I'm later than she is! I meant to get this done earlier but it has been a busy start to 2012.
I don't think I achieved as much as Trish but we had some good times/accomplishments. The year started out in January with finding out that Nicole had been chosen as United Therapeutics patient of the year. They manufacture several of the drugs for pulmonary hypertension. They have an annual marketing meeting each year at a different location. For 2011 it was Honolulu, Hawaii. We spent five days at a resort. It was awesome. I had always, since I was a little kid, dreamed of going to Hawaii. Didn't like the flying part though. As a result of this, someone in the company donated to Nicole a brand new Macbook Pro.
In March I believe I found this wonderful site called caregiving.com and started blogging. I am so blessed to have made so many wonderful friends.
I won the caregifter award on caregiving.com in May and enrolled in Penn Foster's pharmacy technician program. I need to have this completed by May 9th of this year. I hope that I will make the deadline. I was on the radio show that Denise Brown does through caregiving.com shortly after that to talk about the award and a little about my caregiving experience. I really enjoyed the experience.
We filed bankruptcy in August which was discharged in December. It is an embarrassing and humiliating experience especially when it is your second time. I will not get into credit card debt again!
Around September or so, I officially became a peer mentor for United Therapeutics where I help and counsel people who are starting on Tyvaso. In November, I had my first speaking gig in which I spoke as a caregiver. It was the first time a caregiver has ever spoke.
Nicole had her first article published in the Pathlight magazine which is put out by PHA. It was her story of being a teenager with this disease along with the article was her picture and a piece of her artwork. I was very proud. She also had a piece of artwork in another addition that was a puzzle for the children in which you had to find the differences between the two pictures. It is my understanding that she will have another one of these drawings in the newest magazine that comes out later this month.
We started attending the local PH support group here and Nicole was asked to be the official photographer for the group. Nicole is an excellent photographer. I want to download some of her pictures but haven't gotten to it. She is also going to be in charge of all the literature that is at the meetings about PH and PHA.
We had a wonderful holiday and even though the days are long and hard we are truly blessed.
I have decided that at the end of each month I will write a synopsis of that month so next year at this time I will be able to look back and see exactly what I accomplished.
I don't think I achieved as much as Trish but we had some good times/accomplishments. The year started out in January with finding out that Nicole had been chosen as United Therapeutics patient of the year. They manufacture several of the drugs for pulmonary hypertension. They have an annual marketing meeting each year at a different location. For 2011 it was Honolulu, Hawaii. We spent five days at a resort. It was awesome. I had always, since I was a little kid, dreamed of going to Hawaii. Didn't like the flying part though. As a result of this, someone in the company donated to Nicole a brand new Macbook Pro.
In March I believe I found this wonderful site called caregiving.com and started blogging. I am so blessed to have made so many wonderful friends.
I won the caregifter award on caregiving.com in May and enrolled in Penn Foster's pharmacy technician program. I need to have this completed by May 9th of this year. I hope that I will make the deadline. I was on the radio show that Denise Brown does through caregiving.com shortly after that to talk about the award and a little about my caregiving experience. I really enjoyed the experience.
We filed bankruptcy in August which was discharged in December. It is an embarrassing and humiliating experience especially when it is your second time. I will not get into credit card debt again!
Around September or so, I officially became a peer mentor for United Therapeutics where I help and counsel people who are starting on Tyvaso. In November, I had my first speaking gig in which I spoke as a caregiver. It was the first time a caregiver has ever spoke.
Nicole had her first article published in the Pathlight magazine which is put out by PHA. It was her story of being a teenager with this disease along with the article was her picture and a piece of her artwork. I was very proud. She also had a piece of artwork in another addition that was a puzzle for the children in which you had to find the differences between the two pictures. It is my understanding that she will have another one of these drawings in the newest magazine that comes out later this month.
We started attending the local PH support group here and Nicole was asked to be the official photographer for the group. Nicole is an excellent photographer. I want to download some of her pictures but haven't gotten to it. She is also going to be in charge of all the literature that is at the meetings about PH and PHA.
We had a wonderful holiday and even though the days are long and hard we are truly blessed.
I have decided that at the end of each month I will write a synopsis of that month so next year at this time I will be able to look back and see exactly what I accomplished.
Friday, January 6, 2012
Trip to Gainesville
Yesterday we went for our quarterly appointment at Shand's Hospital. One of Rick's customers at work had told us about a different route to take that was quicker and would take 2 hr. 5 min. as opposed to 2 1/2 hours the way we had been going.
We left about 7:35 a.m. as we had a 11:00 a.m. appointment and boy was it cold. We never know what the traffic will be like etc. so we must make sure we have ample time.
We stopped about 8:45 a.m. or so to grab some breakfast at a rest stop that had dunkin donuts :o) . We hit the medical plaza in Gainesville about 10:05 a.m. but once we parked and got to the clinic it was 10:20 a.m. It was very busy and I thought great we will be here all day. I heard a nurse telling another patient that there's actually 12 clinics going on. I was happy to hear that they all weren't for the heart/lung clinic.
Nicole was called for her vitals as I talked to a lady regarding medicaid. She told me to pick a plan ASAP because Medicaid would without any notice stick her in a HMO. I was grateful for this information so this is on my to due list for next week. Medicaid has that we still have Rick's insurance that expired on 10/31/11 and all Medicaid has to do is look this up which takes two minutes.
I was upset because I had received an Ipad for Christmas specifically to keep all my medical notes/reports etc. on for her appointments. I am using evernote for this but what I didn't realize is that you need an internet connection to get any of the notes to come up and without Wifi this isn't possible. I don't know how to remedy this. I didn't know Shand's password and I must have been in a timid mood because I was to scared to ask and I also was too scared to ask a lady in the waiting room where she got her pill box which I really liked. I really regret it as on the trip back one of the pill compartments popped open and I lost her medication at the bottom of her backpack and the pill is very small.
I was also upset because I forgot to bring a copy of Nicole's blood test results from last week. I have it in Evernote but didn't have it in the packet of papers for the doctor. Nor did I have a hard copy with me nor did I find it on my desk this morning so I will have to hunt for it to fax to them.
Nicole had an EKG followed by her echo. I was asked if Nicole would be interested in taking part in a voluntary 3 year observational study to determine if Tyvaso causes more respiratory problems than the other meds. She will be asked questions every three months and will be paid $50. She accepted this and we signed paperwork.
The doctor is so good with Nicole. He loves her artwork and always looks at it on her Itouch every time we are there. He showed her a picture on his phone of two dogs and asked her if she could draw them for him if he sent her the picture. He even said he would pay her. He asked for her email address and I gave it to him.
The doctor said her heart function was great :o) Didn't really address her PH other than to say there was no change in her echo/EKG. I asked about her oxygen intake to make sure that since we are switching oxygen companies she didn't need a high flow concentrator as the company we want to go back to doesn't have them. The RT (respiratory therapist) at rehab feels that she should be on more oxygen then 3 lpm when she's home. He said it doesn't make a difference whether she's on 3 or 8 lpm. He asked Nicole if the 8 lpm that she uses at rehab bothered her and she said yes (it does, she sneezes and gets really stuffed up) and he said she didn't need to be on that high flow. I said her O2 stats will drop and he said that was okay. He believes that her low O2 stats are more to do with her hemoglobin being low. For Nicole when she has this tested we should be seeing a higher number that what it should be according to the labs scale and we are not. The scale is approximately 11 - 15 depending on the lab and she should be above 15 and she isn't. He wants to get to the bottom of why and I believe for some reason her bone marrow isn't producing the right amount of hemoglobin. He had the nurse take blood to do a test that measures a hormone or something in the kidney which will tell them if it is low and her body doesn't make enough if this is the case they can give her medication to raise this level which will raise her hemoglobin. I think she had this test done already by the hemotologist but I didn't have that lab result with me nor did I have it scanned. I should have because I had received this since her last appointment in Gainesville. I know the hematologist tested her for IBP or something to that effect and said it was normal. It was also recommended by her rheumatologist that she have genetic testing to see if in fact her benign hypermobility joint syndrome is indeed Ehler's - Danlos syndrome which can only be diagnosed through genetic testing. I am very scared of this test and I am afraid they will tell us something else is wrong with Nicole. Some rheumatologist and genetist (spelling) believe the BHJS is in fact the same as Ehler's - Danlos. We need to know because if she has it she has to be treated. Right now the only treatment for her joint pain is Advil and Biofreeze. This will be another "thing" that she was born with that the doctor's just poo-pooed away. This test will take 2 months to get the results. I guess I will just wait and see what happens with this before I take her to a rheumatologist because unfortunately I don't think the one she was seeing takes Medicaid. I hope Medicaid pays this.
The social worker also talked to me about Medicaid and my options as well as what to do when Nicole turns 18 and what I need to have in place such as power of attorney so I can speak on her behalf. I will also need to get an advance directive so that whatever wishes she has will be followed.
While I always hope for a report of she cured... I am happy with the appointment. However, I still have some doubts about the doctor. I feel that he is too conservative. I am hoping I can get a second opinion. The doctor I would like to see ( who both Nicole and I have met after a presentation at the support group meeting ) but I don't know what forms of medicaid he takes, nor do I know if he will see a minor. He says he will but it isn't always up to him when he is affliated at a hospital. The cardiologist was suppose to find this information out but obviously they haven't ... just another reason to do things myself :o)
We left about 7:35 a.m. as we had a 11:00 a.m. appointment and boy was it cold. We never know what the traffic will be like etc. so we must make sure we have ample time.
We stopped about 8:45 a.m. or so to grab some breakfast at a rest stop that had dunkin donuts :o) . We hit the medical plaza in Gainesville about 10:05 a.m. but once we parked and got to the clinic it was 10:20 a.m. It was very busy and I thought great we will be here all day. I heard a nurse telling another patient that there's actually 12 clinics going on. I was happy to hear that they all weren't for the heart/lung clinic.
Nicole was called for her vitals as I talked to a lady regarding medicaid. She told me to pick a plan ASAP because Medicaid would without any notice stick her in a HMO. I was grateful for this information so this is on my to due list for next week. Medicaid has that we still have Rick's insurance that expired on 10/31/11 and all Medicaid has to do is look this up which takes two minutes.
I was upset because I had received an Ipad for Christmas specifically to keep all my medical notes/reports etc. on for her appointments. I am using evernote for this but what I didn't realize is that you need an internet connection to get any of the notes to come up and without Wifi this isn't possible. I don't know how to remedy this. I didn't know Shand's password and I must have been in a timid mood because I was to scared to ask and I also was too scared to ask a lady in the waiting room where she got her pill box which I really liked. I really regret it as on the trip back one of the pill compartments popped open and I lost her medication at the bottom of her backpack and the pill is very small.
I was also upset because I forgot to bring a copy of Nicole's blood test results from last week. I have it in Evernote but didn't have it in the packet of papers for the doctor. Nor did I have a hard copy with me nor did I find it on my desk this morning so I will have to hunt for it to fax to them.
Nicole had an EKG followed by her echo. I was asked if Nicole would be interested in taking part in a voluntary 3 year observational study to determine if Tyvaso causes more respiratory problems than the other meds. She will be asked questions every three months and will be paid $50. She accepted this and we signed paperwork.
The doctor is so good with Nicole. He loves her artwork and always looks at it on her Itouch every time we are there. He showed her a picture on his phone of two dogs and asked her if she could draw them for him if he sent her the picture. He even said he would pay her. He asked for her email address and I gave it to him.
The doctor said her heart function was great :o) Didn't really address her PH other than to say there was no change in her echo/EKG. I asked about her oxygen intake to make sure that since we are switching oxygen companies she didn't need a high flow concentrator as the company we want to go back to doesn't have them. The RT (respiratory therapist) at rehab feels that she should be on more oxygen then 3 lpm when she's home. He said it doesn't make a difference whether she's on 3 or 8 lpm. He asked Nicole if the 8 lpm that she uses at rehab bothered her and she said yes (it does, she sneezes and gets really stuffed up) and he said she didn't need to be on that high flow. I said her O2 stats will drop and he said that was okay. He believes that her low O2 stats are more to do with her hemoglobin being low. For Nicole when she has this tested we should be seeing a higher number that what it should be according to the labs scale and we are not. The scale is approximately 11 - 15 depending on the lab and she should be above 15 and she isn't. He wants to get to the bottom of why and I believe for some reason her bone marrow isn't producing the right amount of hemoglobin. He had the nurse take blood to do a test that measures a hormone or something in the kidney which will tell them if it is low and her body doesn't make enough if this is the case they can give her medication to raise this level which will raise her hemoglobin. I think she had this test done already by the hemotologist but I didn't have that lab result with me nor did I have it scanned. I should have because I had received this since her last appointment in Gainesville. I know the hematologist tested her for IBP or something to that effect and said it was normal. It was also recommended by her rheumatologist that she have genetic testing to see if in fact her benign hypermobility joint syndrome is indeed Ehler's - Danlos syndrome which can only be diagnosed through genetic testing. I am very scared of this test and I am afraid they will tell us something else is wrong with Nicole. Some rheumatologist and genetist (spelling) believe the BHJS is in fact the same as Ehler's - Danlos. We need to know because if she has it she has to be treated. Right now the only treatment for her joint pain is Advil and Biofreeze. This will be another "thing" that she was born with that the doctor's just poo-pooed away. This test will take 2 months to get the results. I guess I will just wait and see what happens with this before I take her to a rheumatologist because unfortunately I don't think the one she was seeing takes Medicaid. I hope Medicaid pays this.
The social worker also talked to me about Medicaid and my options as well as what to do when Nicole turns 18 and what I need to have in place such as power of attorney so I can speak on her behalf. I will also need to get an advance directive so that whatever wishes she has will be followed.
While I always hope for a report of she cured... I am happy with the appointment. However, I still have some doubts about the doctor. I feel that he is too conservative. I am hoping I can get a second opinion. The doctor I would like to see ( who both Nicole and I have met after a presentation at the support group meeting ) but I don't know what forms of medicaid he takes, nor do I know if he will see a minor. He says he will but it isn't always up to him when he is affliated at a hospital. The cardiologist was suppose to find this information out but obviously they haven't ... just another reason to do things myself :o)
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