We took Michael to Pittsburgh on March 14th for his follow up from his transplant evaluation and here is what they had to say...Michael's X-rays showed advancement of his disease and more scarring and damage than his last X-ray. He has advanced to the point where the Vertex medications will not help him. We have no hope now except for transplant. There is just too much damage already done. Michael's PFTs have dropped from 45% to 37% this past year. The only idea they had for bringing them back up was to put him on continuous low dose steroids but before they do that, they want him to have a DEXA (bone) scan to ensure that he doesn't have osteoporosis already. If he does,the steroids won't be an option because they can cause bone problems and stunt growth. Also, they are presenting his case to the whole transplant team on Tuesday and Dr. Spahr is going to consult infectious disease about keeping Michael on a long term antibiotic called Zyvox. We have already lost so many options with antibiotics because Michael's bacterias have grown resistant to most everything. If we use this and lose it as an option, it could be catastrophic when he does get a transplant. They gave the time frame for transplant at about a year but said that we are one major event away from having to list him. Michael is at high risk for pneumothorax (collapsed lung) and hemoptysis (coughing up blood) because of how damaged his lungs are. Should either of those things happen, he will be placed in the intensive care unit here and Pittsburgh will go ahead and list him as a priority one which means he would be at the top of the list. They also want Michael to have an EKG which will tell them how his pulmonary hypertension is. If that has advanced as well, it could push them into listing him sooner so his heart doesn't sustain more damage. He would also then be placed on oxygen around the clock. I will hear from the transplant coordinator after the team meets on Tuesday so she can inform me of the decisions they made. Basically, they are not ready to list him quite yet but we are steadily moving towards it.
I had a conversation with Michael about what happened in Pittsburgh and it went like this: "Michael, do you have any questions about Pittsburgh and what the doctors said?" "No, I don't have any questions but I've decided that I don't want the transplant." "What made you come to that decision?" "The doctor told me that recovery is a slow process and I just want to run and play like a normal kid." "Well Michael, you will get there someday it will just take some time. Do you understand what will happen if you don't get the transplant?" "Yeah, I'll be fine. I've been taking my meds." "It's great that you comply and take your meds but your lungs are very sick and at some point they will stop working. Do you understand what that means?" "Does that mean that I will die?" "Yes, honey, it does. Daddy and I have made this decision for you because we don't want to lose you and we want to give you the best possible chance but we aren't going to force you into anything. It's not happening any time soon so you have some time to think about it." Hardest conversation I've ever had to have in my life! I had to walk outside and compose myself because I don't want him to see how heart wrenching this is. What do you do when your 8 year old doesn't want a procedure that will save his life? Do you force him into it because he's a minor and under your care or do you respect his wishes and let him die? I just don't have the answers but I pray every day that we are saying the right things, doing the right things and making the right decisions.