Today we went to see Dr Muburak at Shands in Gainesville for a second opinion. Prior to my last visit I was hesitant of going because I thought things were going great so why bother. I'm glad I went!
He is very nice. He is laid back but not afraid to voice his opinion! He doesn't believe in echos. He doesn't think they are accurate and shouldn't be used for making treatment decisions. In his words "they are bullshit"! When he reviewed the cath that I had done at Mayo he was surprised to see that I reacted to the nitric oxide and haven't been started on calcium channel blockers (CCB's). Only a small percent of PH patients are CCB responders. When the CCB works you often don't have to go on any other medications. According to the cath report my cardiac output was low therefore they didn't start me on any CCB. The normal cardiac output is about 5 L/min and the cath showed mine to be about 3 L/min which would mean my heart wasn't functioning well. When there is poor heart function CCB's are not an option because they can cause further damage. He said that he doesn't know how they calculated my cardiac output so he doesn't know how accurate it was. He thinks I should try a CCB challenge which can be done in one of two ways. One would be outpatient where they would give me the meds and see what happens. The other is to be admitted to the ICU for a few days, have a catheter placed to continuously measure my pulmonary pressures and cardiac output and slowly give me CCB's and monitor my reaction. This isn't something that is done very frequently. I asked him how often he has done this test and he said once! Not very reassuring! However if I decide to give CCB's a try this is the route I will take. I figure I will know what my cardiac output is prior to starting and if it's too low then we don't proceed and if it's ok then we try it and I am in a controlled environment where people can react right away if something went wrong. He told me about a doctor in Chicago - Stuart Rich - who is an expert with CCB's and other PH treatments. He suggested I see him for his opinion. I looked him up online and what I found was impressive. He has been treating PH for 30 years and helped lead the way to start using IV meds for treatment. It looks like Eric and I will be taking a trip to Chicago! One more opinion can't hurt and it will be like a tie breaker between the two docs I have already seen! If the CCB's end up not being an option Dr Muburak said he would put me on a second PH medication such as Tracleer or Letaris. He agreed that there aren't any studies to prove two types of meds work better than one but from what he has seen it does. The other good thing that he said is that he classifies me as Stage I not stage II - basically he thinks I am doing well which is nice to hear :)
I have a renewed sense of hope that things are going to be okay. The last month has been a little rough with getting the bad news from my last visit that my heart wasn't as strong and the PH community lost two young women to this stupid disease. One was only 32 and the other 26 - life just isn't fair. It only makes me more determined to fight and to find a way to help find a cure. I have spoken with PHA and with their help I am going to organize a bunco tournament where all proceeds will go to PHA for research and PHA programs. I have a wonderful group of friends who are going to help make it a huge success! I'll post more about it once the details have been ironed out.
Thanks again to everyone for all the good wishes and prayers. They really work so keep them coming :)
