This may or may not pertain to my situation, I don’t think ultimately it will. Yet, the more successful attempts & changes to level the playing field in scoring for transplant, all the better. So maybe indirectly, rather than directly.

LAM as far as illness is tricky to score to begin with because of its variance person to person.  We have many of the same quality of life issues, symptoms & problems of other diseases (Like COPD, CF ,& IPF for example. These are other lung diseases that may have transplant as an option) but yet evidently, those of us with LAM don’t “die off as quickly”, as oddly as that sounds which complicates the usual one-size-fits-all scoring scenario.  So there’s more to the score & getting a transplant than just the standard matching & scoring; most transplant centers realize this though & that’s why proper evaluation & timing is critical). We often also perform higher than national outcomes & other lung diseases since most of us are otherwise healthy, or at least relative to other lung disease counterparts.

Though I was never directly told, I had a strong sense that in August after my evaluation that my LAS  (lung allocation score) qualified, but most likely at the very bottom listing level (lowest score possible).  Therefore, not much could be done for me immediately, so I was advised to keep stress levels low, not work, continue my disability benefits, &  obtain a few more tests before the next appointment.  I am also continuing to take Rapamune,  (originally developed as a common kidney transplant anti-rejection drug) which they will pull me off in November.  Then unless something radically changes, plan to list me then.

I’ve had more quality of life changes & symptom progression since that they might be able to factor in to the overall listing & scoring as well or at least as additional documentation that explains my needs & timing at the very least.

(Especially since Rapamune is the only FDA approved treatment for LAM at this time. It is clearly not holding my lungs as well as it had in the past).

Since the drug won’t clear my system for a month, I’ll most likely be put on the list in “inactive” status for November & then most likely go active in December. They will give me an anticipated wait time frame at my next appointment.  I agreed with this approach.  To minimize wait time, I’d rather wait when I’m healthier than not.

I’m thankful to be listed for a few reasons.

At my first appointment just to discuss facts & figures in 2011, my kidney function was worse & TSC was still causing a few issues with my kidneys.  This has since resolved & I’ve had shrinkage of my kidney tumors.  The transplant drugs are hard on them; so I know it’s a concern but less so now at least. Staying on the drug because it’s still working there & going strong can only benefit & give them a head start to buffer any changes.

The second reason is that some transplant centers won’t touch you if you have severe GERD (aka acid reflux) or any gastrointestinal issues. I most likely developed GERD from the steroids I take to keep my airways open & other factors.

Luckily my transplant center does not see either the GERD or pleurodeses (pleural – had it done on both lungs) as a disqualification to listing.

In fact, one of the hurdles of my car accident recovery at the trauma center was the inability of the hospital to give me the GERD drug I needed & regularly took. Others don’t work or work as well. They gave me a significantly weaker drug that did not work until they some how told me 3 days in I could bring in my own; which I ended up doing.  I started doing better with other things once this was controlled. But it also made it hard to keep my food & pain medicine in my stomach properly & required two different nausea medications to manage it.

GERD can complicate transplant in several ways, ability to swallow or properly digest food; the risk of uncontrolled acid being backed up from the esophagus & then “aspirated” (or inhaled) into the lungs.

(In fact, another complication of my car accident recovery was pneumonia caused by inhalation of airbag dust which led to aspiration pneumonia.)

I also had a bad run-in where I developed esophagitis & a hiatal hernia  a few years ago. (My gastro doc told me the hernia was a result of the esophagitis. I point this out because it doesn’t always happen this way) from taking doxycycline (a common antibiotic) for an infection. I only took it 3 days & took it properly; yet it burned my esophagus, closed up my throat, & gave me this hernia. (Probably from a weakened diaphragm from all my lung problems over the years).   I know this to be true because of the symptoms I had developed so quickly.  I was shortly writhing in pain suddenly & could barely swallow water or eat without pain & it hit very suddenly after only the second pill.

I was in between primary care doctors myself when it got really bad, so I booked an urgent appointment with my friend’s primary that was recommended to me. I told him what happened. He was an astute listener so I had a diagnosis in 5 minutes & ended up drinking a concoction of lidocaine & pepto for at least 2 weeks before I could eat any meals or drink much of anything but that helped significantly.

In my swallow study, this all showed up. Yet it didn’t come up as a point of concern for my team.  I know for patients who do have severe GI issues who are transplanted they may need some further surgeries post-transplant to address them if they can’t be managed. They can be invasive if warranted.  I am hoping I am spared that fate.  Yet another trade-off.

Most people don’t realize too that if a transplantee has issues prior to surgery outside of the organ they are having transplant, the drug cocktail (to prevent rejection or complications) or other factors, or even the body’s reactions to the magnitude of the surgery itself may kick up a little dust & temporarily exacerbate those issues. The transplant won’t fix problems with other organs & can cause some new issues with others or make existing problems a little worse. Still overall, manageable compared to life prior to transplant.

But outside of these two concerns, I’m a good candidate & have a strong chance at succeeding with my new lungs.

Even if these proposed scoring changes don’t directly affect me or my LAS score, they are the first proactive changes made in 10 years  since the scoring criteria was revised last. They may be more telling & pertinent criteria than the original criteria used.

Whatever progress in this realm, I’ll take it; whether it is of direct benefit to me immediately or not.  For both the good of the process but then also those who transplantees waiting with me to have increased access to organs, so there will be more room for me to wait my turn for my gift once I’m listed.

(As a final aside & reminder to newer followers who may not have seen the archives or fully understand my lung disease; this feature image puts it into context. Please take a minute to check it out as it makes it easier to understand LAM better & what I’ve been living with in the 12 years since diagnosis. In this image one can see the difference between normal lungs & LAM lungs in an easier way & why the lungs are prone to collapse. The only thing that is not illustrated is the proteins secreted by these cysts that overtake the lungs.  They poke holes in the remaining healthy tissue between the cysts. My problems with breathing as a result of this disease are two-fold. I have problems getting air in, but I also have issues with moving air out as efficiently.)