Chronic Rejection Sucks!

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.

Rejected from Duke

Never in a million years did I think that Duke would reject Anya for a second transplant because she DIDN’T NEED a liver transplant.  If anything, I figured they’d reject us because she NEEDED a liver transplant.  Anyways, here is the official letter that they sent us:

duke-letter

 

 

I think they’re still worried about the liver pressures being outside of their normal range.  Anya’s measured Hepatic Venous Pressure Gradient (HVPG) range is 6-7 mm Hg; normal is 1-5 mm Hg; full blown portal hypertension (indicative of cirrhosis) is anything greater than or equal to 10 mm Hg.  Anya’s liver is normal enough to not need a liver transplant, but not quite good enough to go through a 2nd lung transplant without also a liver transplant.  At least, that’s what the Duke lung surgeon believes, even though the Duke liver surgeon believes the opposite.  Unfortunately, the Duke lung surgeon makes the final decision, since he’s the one doing the procedure, so he won out.

Another thing that scared them is that Anya’s first liver biopsy, performed down at Duke in March 2014, showed that her HVPG was 16 mm Hg.  In addition, in the liver biopsy itself, “the bridging fibrotic bands focally exhibit nodular contour, suspicious for cirrhosis.”  These two things scared them enough that they’re unwilling to do second transplant on Anya without also doing a liver transplant, which she does not need.


 

So, here we are now.  Anya’s still in the hospital getting treated for a lung infection.  We’re going on #Day15 now.  We should have the results of the bronchoscopy culture by tomorrow, which will tell the doctors the type of mycobacterium that they need to treat.  Right now, they’re giving Anya a few antibiotics that treats all of the types, but they’d like to alter the regimen to only treat the specific mycobacterium that she’s growing in her lungs.  We’ll know the results by tomorrow, and then they’ll alter her meds accordingly.

 

Anya’s doing pretty good.  She’s still in isolation because one of the strains of mycobacterium is Tuberculosis (even though there’s a 0.01% chance of it being that strain), they need to follow the proper protocols until they can officially rule out TB.  Isolation just means that she can’t leave the room without a mask, and anybody entering the room needs to wear a mask.  She’s been in isolation since last Friday.


 

So, what’s next?  Well, the good people over at Duke referred us over to Cleveland Clinic and University of Pittsburgh Medical Center (UPMC).  These two hospitals are not as active as Duke in performing lung transplants, but they are a close second.  I don’t know if we’ll have to go through the full evaluation at these two places, but I think at the very least, they will want to meet with Anya before making any decisions.  These places normally work pretty fast with evaluations, so I imagine that we’ll get a call some time over the next week, and maybe schedule a visit within a month.  That’s my hope, at least.

First things first though, I guess…Anya needs to be released from the hospital.  In addition, she’ll probably need to be on IV antibiotics for several more weeks while at home.

 

IVIG Infusion, Another Bronchoscopy, CCU, Duke

Last Tuesday, the doctors decided to give Anya something called an IVIG infusion.  The idea was to boost her immune system to help her fight off this lung infection.  Most people don’t have a problem with this blood product, however, Anya had a really bad reaction to it.

The nurses started the infusion around 2pm.  The infusion is just a slow drip on an IV line.  Around 5pm, Anya started getting really cold.  We wrapped some blankets around her.  The nurse asked us if we wanted to stop the infusion or not.  There was just a little bit left, so we just kept going with it.  By 7pm, after the infusion was finished, Anya’s blood pressure shot up to 193/110, her resting heart rate was 120, and she was having really really hard time breathing.  She was on four liters of oxygen before the infusion, but they had to pump it up to 15 liters with an oxygen mask and she was still gasping for air.

I’m not going to lie, it got a little scary.  Okay, maybe a lot scary.

By morning time, Anya was still gasping for air.  Her vitals had improved slightly, but there was still a lot of concern.  She didn’t get any of sleep, nor had she eaten anything since 2pm the day before.  The doctors ordered another bronchoscopy for 1pm.  This was Wednesday.  I don’t know how smart it was to do another bronchoscopy in her state, but it turned out to be the right decision.

After removing a ton of mucus from her lungs again, with a little bit of twilight medicine, by 9pm Wednesday night, Anya was back.  Even though she was still a little bit dazed from lack of food, breathing in CO2 (lungs unable to process the oxygen), various drugs in her system, we were actually able to have a conversation again.

After the bronchoscopy, the docs decided to move her to the CCU (Critical Care Unit, aka ICU) for round the clock care, just in case.  They wanted to move her out of the ICU on Thursday, but we opted to stay for another day.  On Friday, she was moved to the Acute Pulmonary Unit (APU), which has more devices to deal with her lungs.

Needless to say, Anya will not be getting the IVIG infusion again.  I asked around the Duke Lung Transplantees Facebook group if anyone else had this same reaction, and no one else had this bad of a reaction, so it looks like this was just an anomaly.  BTW…if you’re going through the lung transplantation process and want a great resource of information, the Facebook group above is the place to be.


So, here we are 4 days later.  Anya’s feeling much better now.  The infectious disease doctors determined that she has a “gram negative bacteria” growing in her lungs, along with some sort of mycobacterium.  Not good news, but she’s been through this before.  The mycobacterium is of concern because most transplant centers will not transplant patients with certain strains.  It takes about 3-6 weeks to determine the exact strain of mycobacterium that she has, and another 6-18 months of antibiotics before it’s gone for good.  The good news is that the previous strain of mycobacterium that she’s grown (Mycobacterium Avium Complex, aka MAC) has been easier to treat and Duke is one of the few centers in the world that still transplant patients with this strain.  We just need to figure out if it is indeed this strain or not.


I contacted our transplant coordinator at Duke last week.  It looks like Anya has been cleared of any liver problems by the two liver doctors at Duke, thus making a liver transplant unnecessary.  We’re still waiting for the liver doctors to meet with the lung doctors to fully clear Anya for another transplant.  That meeting should happen next week some time.  However, a lot still hinges on this mycobacterium strain.  My guess is that they probably won’t do anything until they’re certain that mycobacterium is not a factor anymore.  Sigh.

The waiting continues…like usual.  If there’s anything that’s a constant in this process, it’s the waiting.  We’re always waiting to see what’s around that next corner.  And as we make that turn, trying to get a glimpse of what’s next, it’s almost always unpleasant, but nothing that we haven’t been able to get around yet.

If all goes well, then Anya will be sent home with antibiotics in the next few days….

Last night. Trying to get some much needed calories back into Anya.  Nothing better than Mario's for that. #Day11

Last night. Trying to get some much needed calories back into Anya. Nothing better than Mario’s for that. #Day11