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.