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. 

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. 

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. 

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. 

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.

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 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.