Chronic rejection, also known as Bronchiolitis Obliterans Syndrome (BOS), is the narrowing of the small airways in the lungs. These small airways help process oxygen to the rest of the body, and get rid of the harmful CO2 from the lungs. Unfortunately, 50% of all lung transplant patients experience chronic rejection within 5 years of transplantation and 80% within 10 years. There’s no known cure for it right now, nor is there a known cause for it. That’s just the reality of lung transplantation.
Anya was diagnosed with chronic rejection about 2.5 years ago; almost 4 years post-transplant. She just celebrated her 6 year transplant anniversary on June 24, 2014. In early 2012 she was running 4 miles per day, and several months later, she had a rough time walking a mile. Her lung function (FEV1) dropped from 100% to roughly 35% in a span of 6 months. Today, after her second lung infection in less than a year, her FEV1 is probably around 20%, maybe less.
Yes, chronic rejection really sucks. She describes it to me as though she’s breathing through a straw. As the chronic rejection gets worse, the straw gets smaller.
While there are treatments like RATG, photopheresis, and Campath to slow down the effects of chronic rejection, there is nothing that will fix the problem. That’s the reality of lung transplantation. Until a cure for chronic rejection is found, odds are, everyone that has a lung transplant will go through what Anya’s going through now. She’s been glad to have those extra 6 years, but still…chronic rejection really sucks.
The only option that we have right now is to prepare Anya for another double lung transplant. The good news is that the success rate for double lung retransplant patients are similar to those of first time double lung transplant patients. The bad news is that the risk of a retransplant is almost double (~20%), which is why transplant centers are highly selective. With that said, ten years ago, retransplants were few and far between; these days, over 100 are performed each year, so we’re just thankful that we still have the option of a retransplant.
Our goal from here on out is pretty clear. Get Anya some new lungs. Put her in the best shape possible to endure the rigors of another transplant.
So, how’s Anya feeling? Well, she just got out of the hospital about a week ago. She spent a total of three weeks there. She’s had several bad days (hard time breathing, sleepless nights, nausea, dry heaving, etc.), but so far, more good days than bad days. Hopefully the good days will continue.
On the transplant front, we’ve heard from all three centers (UPMC, Cleveland Clinic, and Fairfax Inova). Fairfax Inova is going to do their doctor’s evaluation tomorrow. They were the ones who did Anya’s first transplant. I think it’s going to be a long shot to get listed here, since they only do one or two retransplants per year. My guess is that they will most likely try to expedite the process at one of the bigger centers.
Cleveland Clinic called today. They just started the evaluation process. Fortunately, they are going to be able to use most of the test results from Duke, so Anya won’t have to go through the entire set of tests again. It took them over two weeks to get back to us after the initial contact, so who knows how long it’ll take them to get back to us again. My guess is 1-2 weeks, requesting more documents.
University of Pittsburgh Medical Center (UPMC) contacted us several days after the initial contact on July 15th. They went through the basic questions, and told us that they’d get back to us in 2-3 weeks, so hopefully they’ll be contacting us very soon here.
We have several doctor’s appointments scheduled for the next few weeks, mainly with the transplant team and the infectious disease doctors, but we also have several with the endocrinologist, since Anya was officially diagnosed with diabetes in the hospital. Her diabetes is not that bad (A1c level around 8), but not managing it makes it harder to fight off infections, and could lead to other problems. It seems as though the doctors have things in control here. The infectious disease doctor at Fairfax Inova seems pretty confident that the mycobacterium infection will not have any affect on Anya’s chances of getting listed for another transplant. I guess the biggest question now is still the liver. We’ll have to wait to see what the other centers think.
That’s about it for now. Again, the waiting game continues. It really sucks that we were rejected from Duke, but we can’t dwell on that now. We just have to keep moving…and keep moving, we will.