I'm not sure that this will be in any random order as my thoughts are so scattered with all the things happening with Nicole and her illness and also just with life in general.
The last time Nicole saw her PH specialist he referred us to an adult hematologist to try and get some answers as to why her platelet count is consistently low and also her anemia issues even though she takes iron. The hematologist did diagnosis her with ITP which is Idiopathic Thrombocytopenia Purpura. Basically, this is a bleeding disorder in which the immune system destroys platelets, which are necessary for normal blood clotting. Usually people with this have too few platelets in the blood. There's either chronic or drug-induced... the doctor seems to think that Nicole's is drug-induced, I disagree, and while I am not a doctor, I do know Nicole better than they do. Her platelet count was low at the date of diagnosis prior to medications which I absolutely believe have also contributed. The doctor feels that having a bone marrow biopsy will help them in seeing how her bone marrow functions and whether the problem lies in the production of platelets or whether the problems lies after the bone marrow has spit them out.
This was a complicated decision to make as to whether to actually put Nicole through this painful test. One she has to be sedated (anxiety mostly) and this is extremely dangerous for a PH patient and secondly I don't want her to go through unnecessary testing and the final reason is one of her doctor's doesn't agree with having this test done.
Earlier this month, Nicole's cardiologist, who specializes in congenital heart defects and whose office diagnosed her CHD and PH in 2010 stepped down as her doctor for a number of reasons from lack of communication between the doctor's to not agreeing with the treatment plan for transplant etc. This was a very stressful situation. I will do whatever I can to make sure doctors have better communication. There is no reason that if a doctor is cc'd that they are not getting it isn't getting it. As one of Nicole's doctor said they would probably have better communication if they were on facebook and did so.
There were a lot of issues that needed to be addressed in regards to the bone marrow which didn't get addressed to my satisfaction so the procedure was cancelled at the last minute. It has been rescheduled for next week and while I do feel better I still worry because most doctors do not have a clue about PH and how they are different when it comes to sedation. I know she can have the procedure without sedation but I don't think her anxiety will allow this. She was/is terrified of having this test which shows up for Nicole with major stomach issues.
After Nicole's doctor stepped down I wrote an email to both her PH doctor and the transplant center basically letting them know of my displeasure over this whole issue. Nicole had an appointment yesterday at the transplant center. I am still trying to process and haven't listened to the recording of the appointment but the jest of it was.... we have to make a decision as to how to proceed. The center has determined that Nicole needs a lung/heart transplant as opposed to a lung/heart repair. If we decide to go to Mayo or University of Florida for a second opinion and they say they can do a lung/heart repair this center will not change their mind. Tampa will not see Nicole because they do not do lung/heart. The question is to we want to travel several hours away to have all the testing done again that she has already gone through to get a second opinion regardless of what that is. We know enough about transplant and the odds of living 10 years after a heart/lung are 35% but the chances that Nicole will be alive in 10 years with her illness now (PH) is less than that.
My hope with the appointment yesterday was that we would get a recommendation for another pediatric cardiologist (she has to see pediatrics because they specialize in congenital heart defects). I didn't get this. Basically, we were told if we decide to stay with this transplant center and proceed with transplant at the appropriate time that we don't need to replace the cardiologist. I'm not sure I am comfortable with this. The doctor basically said "well the PH specialist is a cardiologist... yes, this is true.... but he has no knowledge of CHD's as this isn't his specialty and he is an adult cardiologist. The transplant doctor said that they have a cardiologist there and he wants us to see him (we haven't before). So basically, the bottom line is we would see the doctor's at the transplant center and the PH specialist and that would be it outside of her rheumatologist, hematologist, ob-gyn, primary, and her psychiatrist.
I guess at this point we will see the cardiologist and see what he says. I will wait until after this time to really think about this. My main goal for the next week is the bone marrow biopsy which her PH specialist wants her to have. We spoke on the phone at great length about this. I would just feel a lot better if someone was there who was knowledgeable with PH like the doctor or his nurse etc. but since the specialist is at a different hospital than the procedure this won't happen. I have the name of the doctor (who I have never heard of) doing the procedure but I don't know yet who the anesthesiologist is at this point as on Wednesday when the appointment was made the lady who I have dealt with through this whole ordeal didn't have the schedule. I'm sure aneshesiologist doesn't have a clue. I asked if they had knowledge of PH but she said she could only assume that they did... not... this isn't a good thing (to assume). This is the first time in almost four years since Nicole's diagnosis that she will have a procedure that requires sedation (she gets the same sedation every time to my knowledge) that her PH doctor hasn't done it/been present, his nurse hasn't been present, or the same doctor/anesthesiologist hasn't done the procedure. This makes me so nervous and I would feel much, much better if there was a nurse or somebody present who was knowledgeable.
The stress and anxiety this puts on our family is tremendous when I spend every moment of every day worrying about Nicole and her future as well as our future as a family. It is exhausting with the phone calls, doctor appointments etc. It is exhausting feeling like a total bitch because I have to ask 101 questions for everything and if I forget anything the consequences could be bad. It is exhausting trying to fight a system that clearly doesn't work well when doctors and hospitals can't communicate especially in this day and age with electronic every thing from iPhone's to mini computer's. Does me running my mouth trying to raise awareness about anything pertaining to Nicole and her illness do any good at the end of the day?
I guess my biggest question/concern is why does a 19 year old young lady have to make such life-changing decisions when she should be out having fun with friends, a boyfriend and looking forward to a future? Instead, she has to make a decision as to whether or not she should have a lung/heart transplant, get a second opinion about that, and all the other decisions that need to be made in context to her health. The future for her is so uncertain and while in reality it is for all of us because none of us know if we have a future or what is in store but for Nicole this hits hard. The decisions that Nicole has to process would be hard for a mature adult let alone a teenager. I will help her and support her with any decisions/issues pertaining to her health and I will be with her every step of the way on this journey no matter what her/our future holds.