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. 

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.

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.