We arrive at the cardiologist's office. The nurse does an EKG and then we're told she will be having an echo. The person who did the echo was awesome and put both Nicole and I at ease. I will always be grateful to her.
After the echo was done the doctor comes in. He was very nice. We were told a lot of information this day and this is the day our whole life as we knew it changed.
First we are told that Nicole had been born with a large 2.4 (or so) cm hole in the lower chamber of her heart (ventricular septal defect VSD) as well as several smaller holes (referred to as swiss cheese holes) also in the lower chamber of the heart. I don't remember if the hole/flap in her upper chamber (atrium septal defect ASD) was discovered this day as it is very hard to see on an echo. We were also told that because of the lack of diagnosis she developed Eisenmenger's syndrome which is a rare progressive heart condition that occurs mainly with VSD's that aren't diagnosed which causes reverse shunting of the heart. This causes the oxygen rich blood that should be going to the lungs to go to her extremities instead because of this lack of oxygen in her lungs she has blue/purple fingernails and toenails. We were also told because of these congenital heart defects she also developed pulmonary arterial hypertension (PAH/PH). I had never heard of this but I knew enough medical terms to know it had something to do with lungs, arteries, and high blood pressure which it does. Basically, your lungs have a blood pressure (not associated with your blood pressure that's taken at every doctor's appointment) PH causes the pressure within the lungs to rise which causes the arteries in the lungs to narrow and close.
We were told there's no cure and it's usually fatal. The only "cure" (which isn't really that) is a lung transplant which in Nicole's case would have to be a lung/heart transplant.
We were told that she wouldn't be able to work and hold a full-time job. That the medications available to treat the disease would only slow and maybe stop the progression of the disease but not reverse it.
We were told that Nicole would never be able to get pregnant as the stress on her heart could be fatal. We were also told should would have to get routine monthly blood work, be on oxygen at least at night while she slept and while she's home for the rest of her life.
We were also told that to be "officially" diagnosed she would have to have a right heart catheterization which would mean driving to the children's hospital in Tampa. This will be another blog. The doctor was confident though that this was the diagnosis because the pressures were severely high.
Five years later.... Nicole is twenty years old now. She is getting a Bachelor's of Fine Art in Illustration online and is an incredible artist (yes.... I am bias)!!!!! She is on oxygen now 24/7. She is on a triple therapy regime for the treatment of her PH which includes two different oral meds as well as a continuous medication that she receives through a catheter in her skin through an infusion pump. About once a month she has to change the site usually because of infection and when she does it causes great pain for the first seven to ten days for which she must take narcotics to control that pain.
She has to get a monthly pregnancy test which is a requirement by one of the medications as it is known to cause birth defects. She also requires a whole slew of blood work every three months to keep tabs on her platelet count (which is chronically low), her red blood cells, her potassium levels, her magnesium levels etc.
She sees her PH specialist every three months; her primary care physician every two months; she sees a rheumatologist for benign hypermobility joint syndrome which is also something she has had since birth which was diagnosed in 2011 every six months; she sees her psychiatrist for OCD and generalized anxiety disorder that was diagnosed in 2004 every three months and she is monitored at the University of Florida Congenital Heart Clinic as well as the Lung Transplant program and goes there for pulmonary function tests and other lung function tests as well as a EKG and echo every six months.
I am so very, very grateful that the medications keep her stable, however, the side effects cause their own problems from chronic low platelet count to chronic nausea, diarrhea, joint pain, site pain and dizziness among others.
We always tell Nicole she is one in a million because when she was diagnosed with PH I believe the statistic of children who diagnosed with this disease was 1 in 2 million. Both PH and Eisenmenger's are rare diseases according to the National Organization for Rare Diseases (NORD) which means less than 200,000 Americans a year are diagnosed.
The medications for PH are considered orphan drugs because they are rare they are extremely expensive. Nicole's infusion medication alone costs more in 2 months than my husband makes in a year. Fortunately, since Nicole is on disability the insurance she currently has covers all her meds with no out of pocket costs. I thank God for this everyday. She loses her insurance later this year and will have to switch because her insurance is for chronically ill children up to 21 years old. So I don't know if new insurance will cover it without a co-pay.
While our lives were changed forever this day I am grateful each day for Nicole, the doctor's, the medication etc. I still have trouble understanding how a congenital heart defect wasn't diagnosed for fifteen years. If it had been diagnosed she wouldn't be sick like she is. I guess this is something that God doesn't want me to understand.
Wednesday, January 21, 2015
Tuesday, January 20, 2015
The date is Thursday, January 21st, 2010 .....
I had waited a month to get Nicole into the pediatrician's office under the guise of an annual check-up. Nicole has been with this doctor's office since she was four years old and she's now fifteen years old. The doctor that was with and treated Nicole for pneumonia and her hospitalization when she was a month shy of five years old left the practice shortly after this. I had seen that he had returned. I wanted to specifically see him and would've waited however long it took.
This doctor had always listened to me and I hoped he would again. I told him I was here for a specific reason. I explained Nicole's permanent purple fingernails and toenails, her lips turning blue when she was cold or over-exerted herself. I explained how she couldn't ride a bike to the entrance of our subdivision (up a slight incline) without having to rest and the shortness of breath with just walking.
He checked her vitals. He also put a pulse ox on her finger. That isn't a normal part of a check up and I don't recall having this done at all since her hospitalization for pneumonia ten years prior. It really should be part of every office visit just like height, weight and blood pressure. It would have been a tremendous help in Nicole's case.
The pulse ox read in the 83 range. A pulse ox measures the amount of oxygen in the blood. A normal, healthy person should have a pulse ox of 98-100. The doctor asked his nurse to get another one as he thought it was broken. The second pulse ox said about the same thing. The doctor then had Nicole wear some oxygen for a few minutes. I don't remember if this changed her number or not. I will never forget him looking at me and saying "this isn't good...." They also pricked her finger to check her hemoglobin which I don't remember if it was high, low or normal but I don't believe it was normal. The doctor said she needed to see a cardiologist He asked if I wanted him to set it up and I said yes. Later that afternoon I got a call from the cardiologist with an appointment for the next day...
This doctor had always listened to me and I hoped he would again. I told him I was here for a specific reason. I explained Nicole's permanent purple fingernails and toenails, her lips turning blue when she was cold or over-exerted herself. I explained how she couldn't ride a bike to the entrance of our subdivision (up a slight incline) without having to rest and the shortness of breath with just walking.
He checked her vitals. He also put a pulse ox on her finger. That isn't a normal part of a check up and I don't recall having this done at all since her hospitalization for pneumonia ten years prior. It really should be part of every office visit just like height, weight and blood pressure. It would have been a tremendous help in Nicole's case.
The pulse ox read in the 83 range. A pulse ox measures the amount of oxygen in the blood. A normal, healthy person should have a pulse ox of 98-100. The doctor asked his nurse to get another one as he thought it was broken. The second pulse ox said about the same thing. The doctor then had Nicole wear some oxygen for a few minutes. I don't remember if this changed her number or not. I will never forget him looking at me and saying "this isn't good...." They also pricked her finger to check her hemoglobin which I don't remember if it was high, low or normal but I don't believe it was normal. The doctor said she needed to see a cardiologist He asked if I wanted him to set it up and I said yes. Later that afternoon I got a call from the cardiologist with an appointment for the next day...
Wednesday, January 7, 2015
Appointments, lab results and pump problems
Happy New Year!!!!!!!! I hope 2015 is the best year yet for you.
Nicole saw the hematologist on Dec. 30th. Her last visit prior to that had been Oct. 31st. She had a lot of blood taken at this appointment in Oct. All of this lab work came back normal. I had a feeling going into this appointment on Dec. 30th that there is really no reason to keep seeing him. There is no clear cut reason or answer why she has a chronically low platelet count. We know if she ever needs surgery she will need a platelet transfusion. The doctor did say that her previous diagnosis of idiopathic thromocytopenic purpura (hoping for correct terminology and spelling) was wrong and her immune system isn't attacking her platelets.
The only issue was her IgG was low but not low enough for infusions. He was going to test it again which he did along with some other immunology tests but no follow up appointment was made. I was not surprised. They were suppose to call with the results.
The hematologist believes that her Eisenmenger Syndrome is the cause of her low platelet count. I have never heard this before which I would think a congenital heart defect cardiologist would know and neither one of her CHD doctor's have every mentioned this. The first hematologist Nicole saw said it was her medication and while I know it does contribute it wasn't the reason as her platelet count was low upon diagnosis.
Nicole was having pump issues the week of Christmas and the week of New Year's. The majority of the time, at least for Nicole, these occur while she's sleeping. The first time was Christmas Eve day at 9:00 a.m. but she didn't tell me she obviously unkinked the tubing and the pump was working again. It alarmed again at 1:00 p.m. and wouldn't stop alarming so we had to call Accredo (the specialty pharmacy) and speak with a nurse. The nurse had her unattach the tubing at the site and clean off the tip with alcohol as sometimes the Remodulin builds up and I believe we were told it gets sticky and will gunk up. This seemed to work and got the pump to stop alarming and go back to running.
On Monday, Dec. 29th Nicole's pump malfunctioned with blockage detected twice within a five minute period at 4:20 a.m. and she was able to clear it by running her fingers along the tubing. At 7:35 a.m. it started alarming again and she couldn't get it to stop so we had to call a nurse at Accredo. He had her check for kinks and then had her push some buttons on the screen of the pump and it went back to running. After this Nicole believed she found the kink so we went ahead and changed the tubing. At 10:45 a.m. the pump goes off again for blockage detected and wouldn't stop so called a nurse again. She was able to get the pump to stop by having Nicole check the tubing and hit some buttons but she did say if the pump went off again then she would need to change the pump and if it still alarmed she would have to change her site.
On the 30th Nicole said her pump went off again at 4:00 a.m. and she unattached and reattached the tubing at the site and it stopped.
On the 31st when we went to change the pump the pump that we were switching too wouldn't display properly and was going into setup which is something the patient shouldn't have to do. Had to call a nurse again and she had to walk Nicole through the set up process which she was relunctant to do. I believe she was the same nurse we had spoke with for one of the times we had called with this batch of pump problems. It didn't work the first time because the nurse told Nicole to program in 0.000 and it should have been her pump rate that went there and this is why it didn't work.
Since this time she has had no problems with the pump. However, Nicole started a new vial of Remodulin on Monday and it leaked and I thought that it was because it was the first draw or the syringe was inserted crooked but yesterday it leaked also because it was on my hands as well as Nicole's. I will need to call a nurse today to see what we should do.
Yesterday she went back to pulmonary rehab for the first time since Dec. 18th. She did complete her full routine but did have some chest pain. On the way home we stopped at the hospital where she gets her blood work to pick up the lab results from Oct. 31st (the hematologist) ; 12/4 (her normal monthly blood work) and 12/30 (the hematologist). I knew what her results from 10/31 were all normal. I also knew what her blood work results were for 12/4 and was a little ticked off that her potassium was low and nobody from the doctor's office told me so her potassium could be adjusted. No real surprises. I was suppose to get a call from the hematologist office with the lab results from 10/31 and surprise no call. I did look at and compare the IgG to her last test in Sept. and it is still low but not as low as in Sept. Her iron was tested as I believe the doctor thought she was getting too much iron (which in my opinion was doubtful). Her ferritin level was suppose to be checked but I only saw iron/plasma and I don't think they are the same thing. Her iron saturation percent was low. I'm not sure what this means. Her platelet count had taken a nose dive between 12/4 and 12/30. Hopefully it will go back up this month. Her reticulocytes which is related to red blood cells were all high just like in Sept. which I was told was no big deal and actually were good. Her IgG was still low but went up a bit since Sept. The IgG subclasses were tested and of the four only subclass 1 was low... not sure what this means either but it looks like it could be related to DTP levels which were tested in Oct. and they were normal.
Nicole sees her PH doctor tomorrow so I need to sit down today after calling Accredo, and rescheduling a doctor's appointment she has next week and write down the list of questions we have for him.
I am also waiting on her last echo which she had done in August 2014 at the University of Florida to be faxed to me as none of the doctors ever send copies of any tests to each other. It seems that I am always the one to have to get these and distribute them accordingly.
Nicole saw the hematologist on Dec. 30th. Her last visit prior to that had been Oct. 31st. She had a lot of blood taken at this appointment in Oct. All of this lab work came back normal. I had a feeling going into this appointment on Dec. 30th that there is really no reason to keep seeing him. There is no clear cut reason or answer why she has a chronically low platelet count. We know if she ever needs surgery she will need a platelet transfusion. The doctor did say that her previous diagnosis of idiopathic thromocytopenic purpura (hoping for correct terminology and spelling) was wrong and her immune system isn't attacking her platelets.
The only issue was her IgG was low but not low enough for infusions. He was going to test it again which he did along with some other immunology tests but no follow up appointment was made. I was not surprised. They were suppose to call with the results.
The hematologist believes that her Eisenmenger Syndrome is the cause of her low platelet count. I have never heard this before which I would think a congenital heart defect cardiologist would know and neither one of her CHD doctor's have every mentioned this. The first hematologist Nicole saw said it was her medication and while I know it does contribute it wasn't the reason as her platelet count was low upon diagnosis.
Nicole was having pump issues the week of Christmas and the week of New Year's. The majority of the time, at least for Nicole, these occur while she's sleeping. The first time was Christmas Eve day at 9:00 a.m. but she didn't tell me she obviously unkinked the tubing and the pump was working again. It alarmed again at 1:00 p.m. and wouldn't stop alarming so we had to call Accredo (the specialty pharmacy) and speak with a nurse. The nurse had her unattach the tubing at the site and clean off the tip with alcohol as sometimes the Remodulin builds up and I believe we were told it gets sticky and will gunk up. This seemed to work and got the pump to stop alarming and go back to running.
On Monday, Dec. 29th Nicole's pump malfunctioned with blockage detected twice within a five minute period at 4:20 a.m. and she was able to clear it by running her fingers along the tubing. At 7:35 a.m. it started alarming again and she couldn't get it to stop so we had to call a nurse at Accredo. He had her check for kinks and then had her push some buttons on the screen of the pump and it went back to running. After this Nicole believed she found the kink so we went ahead and changed the tubing. At 10:45 a.m. the pump goes off again for blockage detected and wouldn't stop so called a nurse again. She was able to get the pump to stop by having Nicole check the tubing and hit some buttons but she did say if the pump went off again then she would need to change the pump and if it still alarmed she would have to change her site.
On the 30th Nicole said her pump went off again at 4:00 a.m. and she unattached and reattached the tubing at the site and it stopped.
On the 31st when we went to change the pump the pump that we were switching too wouldn't display properly and was going into setup which is something the patient shouldn't have to do. Had to call a nurse again and she had to walk Nicole through the set up process which she was relunctant to do. I believe she was the same nurse we had spoke with for one of the times we had called with this batch of pump problems. It didn't work the first time because the nurse told Nicole to program in 0.000 and it should have been her pump rate that went there and this is why it didn't work.
Since this time she has had no problems with the pump. However, Nicole started a new vial of Remodulin on Monday and it leaked and I thought that it was because it was the first draw or the syringe was inserted crooked but yesterday it leaked also because it was on my hands as well as Nicole's. I will need to call a nurse today to see what we should do.
Yesterday she went back to pulmonary rehab for the first time since Dec. 18th. She did complete her full routine but did have some chest pain. On the way home we stopped at the hospital where she gets her blood work to pick up the lab results from Oct. 31st (the hematologist) ; 12/4 (her normal monthly blood work) and 12/30 (the hematologist). I knew what her results from 10/31 were all normal. I also knew what her blood work results were for 12/4 and was a little ticked off that her potassium was low and nobody from the doctor's office told me so her potassium could be adjusted. No real surprises. I was suppose to get a call from the hematologist office with the lab results from 10/31 and surprise no call. I did look at and compare the IgG to her last test in Sept. and it is still low but not as low as in Sept. Her iron was tested as I believe the doctor thought she was getting too much iron (which in my opinion was doubtful). Her ferritin level was suppose to be checked but I only saw iron/plasma and I don't think they are the same thing. Her iron saturation percent was low. I'm not sure what this means. Her platelet count had taken a nose dive between 12/4 and 12/30. Hopefully it will go back up this month. Her reticulocytes which is related to red blood cells were all high just like in Sept. which I was told was no big deal and actually were good. Her IgG was still low but went up a bit since Sept. The IgG subclasses were tested and of the four only subclass 1 was low... not sure what this means either but it looks like it could be related to DTP levels which were tested in Oct. and they were normal.
Nicole sees her PH doctor tomorrow so I need to sit down today after calling Accredo, and rescheduling a doctor's appointment she has next week and write down the list of questions we have for him.
I am also waiting on her last echo which she had done in August 2014 at the University of Florida to be faxed to me as none of the doctors ever send copies of any tests to each other. It seems that I am always the one to have to get these and distribute them accordingly.
Friday, December 26, 2014
Miscellaneous Ramblings
I hope that everyone had a wonderful Christmas. Ours was low key this year but I always enjoy the time with family. I also like presents too but I can live without them :) The one thing that I want more than anything else is a cure for Pulmonary Hypertension and so far that hasn't come but I believe it will.
Every morning I watch Joyce Meyer and this morning was no exception. It was a re-run I believe but I always learn something new or if I am struggling with some area a show pertaining to that will air. It's a little freaky sometimes.
This morning's show was about Trusting God When You Don't Understand. This is something I always have to remind myself. There was a segment with a couple who had lost their young son (under 5 years old) to a rare genetic disorder which at this time is incurable. The mother stated when she found out that she was pregnant with him she prayed that he would not only be a blessing to them but to others as well.
I can so see the blessing that Nicole has had in our life.... I have learned so much from her and she is truly my hero. I also know that she is a blessing to others as well either through her illness and the PH community as well as the way she lives and copes with it. She is also a blessing through the beauty of her artwork.
As I watched this segment (which I have seen before) with tears rolling down my face I thought about Nicole and how I don't understand why not so much that God allowed her to be born with congenital heart defects but more so why were her health issues misdiagnosed for 15 years. I am in awe everyday of the things that could have happened and I am most grateful for God keeping her safe.
Unless you are a parent of a chronically ill child, and it doesn't matter if the child is 2 or 20 they still are your child, it is harder to grasp the struggles that a parent goes through everyday. It is absolutely heart-wrenching to watch your child suffer. The hopelessness and helplessness can be overwhelming at times. There is one particular person that was such an influence after I heard her speak about her struggles with her son less than a month prior to Nicole's diagnosis and I just couldn't wrap my brain around the strength and courage that she has everyday. She was and continues to be an inspiration to me.
I truly believe to the depths of my soul that if it wasn't for Nicole's illness she wouldn't be the artist that she is today. Her artwork is incredible and I am so proud of how she continues to grow and do things that she never would have thought she could do. The first time she drew a face, the first time she drew a figure etc. I see the passion in her eyes when she completes a piece that is she proud of.
My heart goes out to those parents that have lost children and I never, ever want to experience this and I hope that God will spare me this pain but I know if I had to choose to either live my life without Nicole or have her live her life without me I would rather live without her.
As we end 2014 and move into 2015 I would love to see a cure for PH but short of this I would love to see Nicole to continue to grow in her passion of art. I would also pray that she remains stable and that the medication that she takes even with all of it side effects and chronic pain will continue to do this for her. I pray for continued good health for myself and the rest of my family. It is very frustrating and stressful dealing with a chronic illness whether it is for yourself or for a child but I pray that I will continue to provide that support and love for Nicole for as long as she requires me to do so. I would do anything for her and if this is what I need to do I will.
I would love to see others be more compassionate in regards to someone who is disabled either through a chronic illness or through injury especially when their disability may not be "visible" to the naked eye. I would love to see others think about how they would want someone to hold a door open because they are pushing a person in a wheelchair or are struggling carrying a child etc. I struggle everyday with being judgmental because something looks different than what it is .... I can tell you that God has really opened my eyes to this A LOT since 2010 and continues to do so. As Joyce Meyers says "I'm not where I need to be but thank God I'm not where I use to be."
I wish everyone a happy, healthy, safe and prosperous 2015!!!!!!!!!!!!
Every morning I watch Joyce Meyer and this morning was no exception. It was a re-run I believe but I always learn something new or if I am struggling with some area a show pertaining to that will air. It's a little freaky sometimes.
This morning's show was about Trusting God When You Don't Understand. This is something I always have to remind myself. There was a segment with a couple who had lost their young son (under 5 years old) to a rare genetic disorder which at this time is incurable. The mother stated when she found out that she was pregnant with him she prayed that he would not only be a blessing to them but to others as well.
I can so see the blessing that Nicole has had in our life.... I have learned so much from her and she is truly my hero. I also know that she is a blessing to others as well either through her illness and the PH community as well as the way she lives and copes with it. She is also a blessing through the beauty of her artwork.
As I watched this segment (which I have seen before) with tears rolling down my face I thought about Nicole and how I don't understand why not so much that God allowed her to be born with congenital heart defects but more so why were her health issues misdiagnosed for 15 years. I am in awe everyday of the things that could have happened and I am most grateful for God keeping her safe.
Unless you are a parent of a chronically ill child, and it doesn't matter if the child is 2 or 20 they still are your child, it is harder to grasp the struggles that a parent goes through everyday. It is absolutely heart-wrenching to watch your child suffer. The hopelessness and helplessness can be overwhelming at times. There is one particular person that was such an influence after I heard her speak about her struggles with her son less than a month prior to Nicole's diagnosis and I just couldn't wrap my brain around the strength and courage that she has everyday. She was and continues to be an inspiration to me.
I truly believe to the depths of my soul that if it wasn't for Nicole's illness she wouldn't be the artist that she is today. Her artwork is incredible and I am so proud of how she continues to grow and do things that she never would have thought she could do. The first time she drew a face, the first time she drew a figure etc. I see the passion in her eyes when she completes a piece that is she proud of.
My heart goes out to those parents that have lost children and I never, ever want to experience this and I hope that God will spare me this pain but I know if I had to choose to either live my life without Nicole or have her live her life without me I would rather live without her.
As we end 2014 and move into 2015 I would love to see a cure for PH but short of this I would love to see Nicole to continue to grow in her passion of art. I would also pray that she remains stable and that the medication that she takes even with all of it side effects and chronic pain will continue to do this for her. I pray for continued good health for myself and the rest of my family. It is very frustrating and stressful dealing with a chronic illness whether it is for yourself or for a child but I pray that I will continue to provide that support and love for Nicole for as long as she requires me to do so. I would do anything for her and if this is what I need to do I will.
I would love to see others be more compassionate in regards to someone who is disabled either through a chronic illness or through injury especially when their disability may not be "visible" to the naked eye. I would love to see others think about how they would want someone to hold a door open because they are pushing a person in a wheelchair or are struggling carrying a child etc. I struggle everyday with being judgmental because something looks different than what it is .... I can tell you that God has really opened my eyes to this A LOT since 2010 and continues to do so. As Joyce Meyers says "I'm not where I need to be but thank God I'm not where I use to be."
I wish everyone a happy, healthy, safe and prosperous 2015!!!!!!!!!!!!
Monday, December 22, 2014
Frustration with reordering medications......
Just spent 45 minutes on the phone which included being disconnected once in the midst of transferring me.
I have to get Nicole's medication through a specialty pharmacy and they are shipped via UPS monthly. We get three medications through this pharmacy two of which are oral medications and the third is her continuous medication which she receives subcutaneously through her skin via an infusion pump.
I know how much medication I have left when I call so I start with her Letairis which she will run out of first on Dec. 29th. The customer service representative is suppose to ask certain questions like did she have her monthly pregnancy test etc. and she didn't. They have their own calculations of when she will run out and most of the time with the orals we are in sync. She says I can have this delivered on the 29th.... I said "she runs out on the 29th and I don't want to receive it on the day she runs out" and she replies "well there is a holiday" and I reply "on Thursday". She then says she will have it delivered on Friday the 26th.... this is much better and I will take this. I realize that I should have called last week to have this set up but they should have called me saying it was time to reorder and to my knowledge they hadn't.
The second medication she was also suppose to ask questions which she didn't. I asked her how many tablets Nicole had based on their records and it was what I had which was enough for 13 days including today. She said do you want it delivered on Friday with the Letairis and I said sure if you can but I don't think you will get insurance approval. She said sure I do this all the time. She then says I will have it delivered on January 2nd. I then asked her to reorder the Remodulin and she says she has to transfer me (usually the same c.s. rep will do it all) while she's transferring me she disconnects me (at least she called me back). During this "hold" time I count out 13 days and find that she will run out on the 3rd.... I am not waiting until the day before she runs out to get her meds.
I get another c.s rep who is going to do the Remodulin but before she does this I tell her I need her Adcirca for a sooner date than the 1/2 shipment. She said the rep is entering it now and puts me on hold. She isn't able to contact the rep so she is going to fix it herself. She changes the date to the 30th which is still a little to close for comfort but I will live with it. She then says she wants to confirm that the Letairis will be delivered on 1/2 and I said "no" she runs out on the 29th. I am so glad that she confirmed this or I would have been speaking to a supervisor on Friday.
She then tells me that it is to early to re-order the Remodulin which means I have to call twice a month to reorder her medications. I will call back on the 31st to reorder. I have re-ordered all her medications even when they are a couple of weeks out for the last 4 years.
When I call back I will also find out if I can get a email or something from UPS stating that I will receive a shipment so I know that it was shipped when it was suppose to be.
This is so frustrating and this is where most of it comes from on a monthly basis is from the pharmacies.
I have to get Nicole's medication through a specialty pharmacy and they are shipped via UPS monthly. We get three medications through this pharmacy two of which are oral medications and the third is her continuous medication which she receives subcutaneously through her skin via an infusion pump.
I know how much medication I have left when I call so I start with her Letairis which she will run out of first on Dec. 29th. The customer service representative is suppose to ask certain questions like did she have her monthly pregnancy test etc. and she didn't. They have their own calculations of when she will run out and most of the time with the orals we are in sync. She says I can have this delivered on the 29th.... I said "she runs out on the 29th and I don't want to receive it on the day she runs out" and she replies "well there is a holiday" and I reply "on Thursday". She then says she will have it delivered on Friday the 26th.... this is much better and I will take this. I realize that I should have called last week to have this set up but they should have called me saying it was time to reorder and to my knowledge they hadn't.
The second medication she was also suppose to ask questions which she didn't. I asked her how many tablets Nicole had based on their records and it was what I had which was enough for 13 days including today. She said do you want it delivered on Friday with the Letairis and I said sure if you can but I don't think you will get insurance approval. She said sure I do this all the time. She then says I will have it delivered on January 2nd. I then asked her to reorder the Remodulin and she says she has to transfer me (usually the same c.s. rep will do it all) while she's transferring me she disconnects me (at least she called me back). During this "hold" time I count out 13 days and find that she will run out on the 3rd.... I am not waiting until the day before she runs out to get her meds.
I get another c.s rep who is going to do the Remodulin but before she does this I tell her I need her Adcirca for a sooner date than the 1/2 shipment. She said the rep is entering it now and puts me on hold. She isn't able to contact the rep so she is going to fix it herself. She changes the date to the 30th which is still a little to close for comfort but I will live with it. She then says she wants to confirm that the Letairis will be delivered on 1/2 and I said "no" she runs out on the 29th. I am so glad that she confirmed this or I would have been speaking to a supervisor on Friday.
She then tells me that it is to early to re-order the Remodulin which means I have to call twice a month to reorder her medications. I will call back on the 31st to reorder. I have re-ordered all her medications even when they are a couple of weeks out for the last 4 years.
When I call back I will also find out if I can get a email or something from UPS stating that I will receive a shipment so I know that it was shipped when it was suppose to be.
This is so frustrating and this is where most of it comes from on a monthly basis is from the pharmacies.
Sunday, December 14, 2014
Update
It has been awhile since I have written on this page. I had created a facebook page called A Day in the Life and was using this page to write about my daughter and our journey with her chronic health issues.
I did like updating the page and did so quite frequently because it was convenient but I have decided that I am going to come back to blogging on here.
I don't know much about how to step up a blog or making it look pretty like some of the blogs that I see but I hope that I will learn as I go.
I will still forward my postings here over onto facebook and will not take down the page. I will probably share things on there like articles and such that pertain to Pulmonary Hypertension.
I am excited about coming back to this type of forum. I am not going to put pressure on myself by saying I will blog once a week, twice a week, once a month etc. I will just do it when I need to update about my daughter.
I am grateful for the followers that I have on my facebook page and hope that they will follow me over here.
I did like updating the page and did so quite frequently because it was convenient but I have decided that I am going to come back to blogging on here.
I don't know much about how to step up a blog or making it look pretty like some of the blogs that I see but I hope that I will learn as I go.
I will still forward my postings here over onto facebook and will not take down the page. I will probably share things on there like articles and such that pertain to Pulmonary Hypertension.
I am excited about coming back to this type of forum. I am not going to put pressure on myself by saying I will blog once a week, twice a week, once a month etc. I will just do it when I need to update about my daughter.
I am grateful for the followers that I have on my facebook page and hope that they will follow me over here.
Sunday, March 9, 2014
Week of March 3rd 2014
This week started out on Monday with a trip to University of Florida in Gainesville. This time Nicole was being seen by the pulmonologist in the Lung Transplant Program. She was also having a chest x-ray, a pulmonary function test (PFT) and a 6MW (minute walk). Nicole increased her walk distance from 913 ft to 930 ft. This is good. The doctor said that her PFT was better than the one she had when she was sixteen. I was glad to hear this too.
Nothing new happened during the visit with the doctor. He basically said that he was a passenger and the Congenital Heart Center was going to call the shots. The nurse coordinator did tell Nicole that post transplant she wouldn't be able to have any reptiles, cats, or birds as pets. This wasn't taken well by Nicole as she does have a turtle that she adores.
It sounds like she will see the pulmonologist at or around the same time as she sees the cardiologist up there so we don't have to make two trips. She will be seeing the cardiologist there on August 20th and will have another EKG and echo at that time. She is suppose to have a right heart cath at some point between now and then and this will be up at the University of Florida.
Nicole seemed to like the nurse coordinator and the pulmonologist but Nicole does like everybody for the most part unless they are mean to her.
On Thursday, Nicole had her 3 month visit with the PH specialist. I had sent an email to his nurse basically telling her what the cardiologist had suggested in regards to Nicole's medication. The cardiologist at UF wanted Nicole's Revatio to be increased from 20 mg three times a day to 40 mg three times a day for three to six months and then increase it again to 80 mg three times a day. The PH doctor didn't have a problem with this, however, he is going to switch her from Revatio (viagra) to Adcirca (cealis). The reason for this is because Revatio while it is available in 20 / 40 and 80 mg strength it is only approved for 20 mg by the FDA and therefore it is very hard to get the insurance companies to cover one of the other doses. With Adcirca she can get the equivalent of these doses with no problem and Nicole will only take it one time a day as opposed to three. I will be refilling her Revatio for the month as it will probably take a couple of weeks for it to go through and get shipped. I hope that she doesn't have any issues switching over. He said she may have some back pain.
The PH doctor had no problem with increasing her Remodulin but it will be only increased on the same day as she does a site change. He said she might as well have the pain, nausea, diarrhea all at one time and get it over with.
He didn't want to change her pain management because the stronger the narcotic the more side effects such as drowsiness etc. and he just wants to keep it the way it is. He said that the ibuprofen did not lower the platelet count but it does affect the way the platelets are formed or something like that. I have to transcribe the recording of the appointment, hopefully, this week.
He didn't realize that Nicole was changing her site every 30 days because of infection which he said may be because of the very high dose of Remodulin she is on. He also thought it might be good to try an antibacterial nose spray that she takes for 5 days but I am not clear on when she is suppose to take it. I will have to contact the nurse tomorrow to ask about this. He also prescribed Hibiclens in the pump that he wants her to use in the shower like a soap or he wants her to soak in it I'm not clear on this either but I do remember him saying to do it with a site change. This will be a trial and error thing as he doesn't know how often she will need to use this. He is hoping that these things will cut down on her infections.
He was upset with me because his nurse said I was rude to her in an email. I didn't realize I was rude and I am not real sure what email she is referring to but it wasn't my intention. I did send her an email apologizing to her. I need to work on being a little nicer (I am nice but if I am having difficulty getting a medication etc. I can get frustrated and not be nice) and really watch what I say. I am so grateful to the doctor and his nurse for helping Nicole as much as they have with the treatment of her PH.
After the doctor's appointment we went with Jackie, the other support group leader to visit one of our support group members who is in the final stages of PH as well as some other health issues. The PH specialist (the same one Nicole sees) said there was nothing more he could do for her. She was so excited to see Nicole and gave her a hug and told her that she loved her. I don't think Nicole really realizes what that meant to Suzy. We then went to Costco and Nicole drove a motorized cart for the first time she did an awesome job and didn't run over anybody, knock anything over etc.
This coming week doesn't have any doctor's appointments on tap only her monthly blood work and a return to rehab. This will probably be the last full week before she has to change her site again.
I have several things to do this week in regards to Nicole's medical issues. I have to contact Social Security to find out if she meets the criteria for Medicare. I also have to transcribe the appointment with the PH specialist. I need to re-order her Revatio. I need to call Accredo and ask a question regarding some paperwork that they sent me when Nicole got her first shipment of Revatio through them. I have to call Theralogix which is where I get Nicole's Vitamin D that her rheumatologist has her take because the bottle of vitamins that they sent me in January expired in June 2013 so I want to be reimbursed for that.
Nothing new happened during the visit with the doctor. He basically said that he was a passenger and the Congenital Heart Center was going to call the shots. The nurse coordinator did tell Nicole that post transplant she wouldn't be able to have any reptiles, cats, or birds as pets. This wasn't taken well by Nicole as she does have a turtle that she adores.
It sounds like she will see the pulmonologist at or around the same time as she sees the cardiologist up there so we don't have to make two trips. She will be seeing the cardiologist there on August 20th and will have another EKG and echo at that time. She is suppose to have a right heart cath at some point between now and then and this will be up at the University of Florida.
Nicole seemed to like the nurse coordinator and the pulmonologist but Nicole does like everybody for the most part unless they are mean to her.
On Thursday, Nicole had her 3 month visit with the PH specialist. I had sent an email to his nurse basically telling her what the cardiologist had suggested in regards to Nicole's medication. The cardiologist at UF wanted Nicole's Revatio to be increased from 20 mg three times a day to 40 mg three times a day for three to six months and then increase it again to 80 mg three times a day. The PH doctor didn't have a problem with this, however, he is going to switch her from Revatio (viagra) to Adcirca (cealis). The reason for this is because Revatio while it is available in 20 / 40 and 80 mg strength it is only approved for 20 mg by the FDA and therefore it is very hard to get the insurance companies to cover one of the other doses. With Adcirca she can get the equivalent of these doses with no problem and Nicole will only take it one time a day as opposed to three. I will be refilling her Revatio for the month as it will probably take a couple of weeks for it to go through and get shipped. I hope that she doesn't have any issues switching over. He said she may have some back pain.
The PH doctor had no problem with increasing her Remodulin but it will be only increased on the same day as she does a site change. He said she might as well have the pain, nausea, diarrhea all at one time and get it over with.
He didn't want to change her pain management because the stronger the narcotic the more side effects such as drowsiness etc. and he just wants to keep it the way it is. He said that the ibuprofen did not lower the platelet count but it does affect the way the platelets are formed or something like that. I have to transcribe the recording of the appointment, hopefully, this week.
He didn't realize that Nicole was changing her site every 30 days because of infection which he said may be because of the very high dose of Remodulin she is on. He also thought it might be good to try an antibacterial nose spray that she takes for 5 days but I am not clear on when she is suppose to take it. I will have to contact the nurse tomorrow to ask about this. He also prescribed Hibiclens in the pump that he wants her to use in the shower like a soap or he wants her to soak in it I'm not clear on this either but I do remember him saying to do it with a site change. This will be a trial and error thing as he doesn't know how often she will need to use this. He is hoping that these things will cut down on her infections.
He was upset with me because his nurse said I was rude to her in an email. I didn't realize I was rude and I am not real sure what email she is referring to but it wasn't my intention. I did send her an email apologizing to her. I need to work on being a little nicer (I am nice but if I am having difficulty getting a medication etc. I can get frustrated and not be nice) and really watch what I say. I am so grateful to the doctor and his nurse for helping Nicole as much as they have with the treatment of her PH.
After the doctor's appointment we went with Jackie, the other support group leader to visit one of our support group members who is in the final stages of PH as well as some other health issues. The PH specialist (the same one Nicole sees) said there was nothing more he could do for her. She was so excited to see Nicole and gave her a hug and told her that she loved her. I don't think Nicole really realizes what that meant to Suzy. We then went to Costco and Nicole drove a motorized cart for the first time she did an awesome job and didn't run over anybody, knock anything over etc.
This coming week doesn't have any doctor's appointments on tap only her monthly blood work and a return to rehab. This will probably be the last full week before she has to change her site again.
I have several things to do this week in regards to Nicole's medical issues. I have to contact Social Security to find out if she meets the criteria for Medicare. I also have to transcribe the appointment with the PH specialist. I need to re-order her Revatio. I need to call Accredo and ask a question regarding some paperwork that they sent me when Nicole got her first shipment of Revatio through them. I have to call Theralogix which is where I get Nicole's Vitamin D that her rheumatologist has her take because the bottle of vitamins that they sent me in January expired in June 2013 so I want to be reimbursed for that.
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