The past few months have been really hard on Noah.
Noah normally has his annual biopsy at the end of December but since he is now on year-round school we decided to move it to October and get it done while he was off track. I wasn't terribly worried about it. Just the standard anxiety of going in for any cath - they aren't my favorite. But there was also a part of me that was ready, almost as if I knew there would be rejection. And there was.
Even though he had been symptom free he was in moderate rejection C3V3. Both cellular and vascular (antibody). He had the same thing last year but we were able to get it cleared up in January. Yet, here it was again in less than a year. His doctors labeled it Chronic. He continues to have rejection and then it clears and then it comes back ever since his first bout back in 2011 when we started the Cellcept. This time we decided to try an Oral steroid taper and come up on all his rejection meds. We have tried to keep them low to help his body fight off the BK virus but it was obvious now that we can no longer do that.
After the Oral taper he had another biopsy; C2.5V3. Not enough improvement. He also had an increase in pressures (although they were still good) and I started noticing a remarkable increase in ectopy. He had a ton of PVCs and Brady's. We decided to do a round of IV Steroids. He was admitted a week before Christmas. It was also during this particular Cath that the Dr. found a narrowing of his Superior Vena Cava (SVC). This is the main vein where all the blood from the arms and head collect to go back into the heart and go to the lungs. This narrowing was causing the blood to back-up a bit before it could go into the heart. The pressure gradient was about 8. The Doctors wanted to fix it in just a few days but I told them no. He wasn't having symptoms yet and since this was the first time we had seen it, I wanted to wait. I also don't think it's wise to do such a risky surgery when his heart was already sick with rejection. This could wait.
The reason this surgery was so risky is because of his pacer wire. That damn thing that I never wanted in the first place was no putting my son's life in danger a mere 20 months after it was put in. You see, the pacer wire had become attached to his SVC right were the narrowing was. Since you can't put a stent in on top of the wire, the wire would need to be removed first. This same wire was suppose to last 15-20 years! Now they would need to remove it but they could only do that when a Thoracic Surgeon was on hand because removing it could tear the SVC causing him to bleed out quickly. This would lead to Open Heart Surgery as a life saving option. It's been my experience with Noah that what is normally easy always becomes complicated. I've lost track of how many times I've been told that "This is really rare" for something we are dealing with. It wasn't something I was in any rush to address.
So we waited. Noah was in the hospital for a few days getting his IV steroids but was able to come home for Christmas. It was also about this time that he really started to get sick. Throwing up a lot, not eating at all, he lost a ton of weight, got really tired, and looked absolutely awful all the time. He was now doing the second Oral Steroid Taper after the IV doses and still taking all the Prophylactic meds to help keep him healthy while we wiped out his immune system.
He had his 3rd biopsy right after the new year. Results were mixed. The great news was that the IV steroids cleared up his rejection! C1V1 (lowest levels possible)! But the SVC was still there and definitely causing problems. His Doctors were worried about it causing a clot that would travel directly into his lungs. And now that his rejection was gone, it needed to be treated.
We got the hospital early that morning. Everyone was very serious. They were anticipating a 7 hour surgery with the possibility of it being longer if he ended up needing Open Heart Surgery. The Surgeon was at the ready. They took blood before hand so that they could type and cross match in case he needed a transfusion during the operation. I stayed with Noah until he was asleep. I kissed his forehead and left.
The nurse said she would call me every hour to give me an update. I was too worried to eat so I sat in the hallway and tried to clear my thoughts. The first update came. Noah is doing great and we are done. So come on back! I couldn't believe it! I called Shane immediately to tell him and then headed back to talk to the Doctors. The Dr who performed the cath was able to balloon the narrowing without cracking his pacer wire. This meant the narrowing was fixed without even needing a stent. And since the wire wasn't damaged during the process, it didn't need to be replaced either.
**I'm leaving out a lot of the "feelings" and spiritual experiences in this post. I'm saving those for my private record. But I can't write that last paragraph and not call out the amazing Miracle that this was. I KNOW Heavenly Father hears and answers prayers. And I KNOW that Faith can truly move mountains. This was truly a Divine Miracle from God worked through the hands of a skilled surgeon. Of this I have no doubt.**
Noah was able to come home that night.
The biopsy results came back C1V2. No cellular rejection but they did see an antibody immune response. Not enough to classify as rejection but still there. Doctors said he was officially rejection free!
One thing we did decide to do was switch out his Neoral (Cyclosporin) for Prograf (Tacrolimus). He had been on Neoral since his transplant even though it wasn't as strong as the newer Prograf. It was our thought that we would save the other med as a back up option when and if it was needed. Shane and I finally felt like the time was right. To help keep this Chronic Rejection in check we switched to Prograf. This also caused his body to better metabolize the Cellcept. We really feel like this change will help him. But it was kind of freaky not giving him Neoral for the first few days.
The other complication from the increased immune suppression is that the BK virus is increasing with every lab draw. We continue to do monthly IVIG but without the lower med levels, it continues to increase. We went to see an Infectious Disease Doctor who specializes in Transplant patients. She said that BK is one of the trickiest viruses. It's very similar to CMV and EBV in that it becomes part of your DNA. Even if the virus load is gone, it's not really gone. It also is harder to treat. CMV is super tricky and resistant and BK is even worse. She also said that him having had symptoms of BK in the beginning (the thickening of the bladder and the kidney reflux/kidney damage) was super rare. Even rarer that just getting BK Virus in the first place. **Seriously people, I laughed out loud!** After explaining that we know all about "Rare Things" she said she anticipates that even though his virus load is increasing he won't experiences symptoms again. But since he already has once we would need to watch it. As soon as symptoms do appear he will need to take this very toxic antiviral med that is known to kill the kidneys. The problem is that once he starts having symptoms again, the BK virus is killing his kidneys anyways. This antiviral med will take several WEEKS of an IV infusion. Until then, he will need a Urinalysis every 2 weeks to check for blood. Blood at the microscopic level will tell us the virus is starting to cause problems long before it has time to begin the symptoms he had last time (like peeing blood).
This has been a hugely complicated issue. The rejection, the BK virus and the SVC narrowing. The last 3 months have been incredibly stressful. It's been so hard watching Noah go from healthy to sick so quickly. He is so pale and thin and tired all the time. But I think he's finally starting to improve. He hasn't thrown up in a week and a half. And it seems that his appetite is slowly increasing. He needs to put on some weight and get some color back in his face.
For now, the plan is to continue the IVIG every month for the full 6 months. Then we will check his native IGG level and possibly continue the infusions to help ward-off the BK symptoms. He will have another transplant clinic in a month and we'll determine when the next biopsy will be. He will have UA's every 2 weeks until we see blood at which point he will start the new antiviral med. His daily meds are Cellcept and Prograf with a small daily dose of Prednisolone. Not sure how long he will stay on the steroid but for now it's okay. And if the narrowing of the SVC comes back we'll try ballooning it again in hopes that pacer wire can last as long as possible.
There is so much more to this story. Faith, prayers, fasting, service, love, revelation, and more. I want to keep as many of those details private as they are very dear to my heart and also very sacred to me. But in case anyone reads this who participated in the prayers and fasting for my sweet boy: Thank you so very much! I have no doubt that your prayers and sacrifice were seen and heard. Your faith and service contributed to this miracle. I felt your love and I know it helped Noah through this as well. Things could have been so much different. You have my eternal love and gratitude for serving my family in this way. Saying Thank You isn't enough.