One would figure by now that I would know tuberous sclerosis complex (TSC) and lymphangioleiomyomatosis (LAM) inside and out. I know quite a bit, but there’s always something to learn in dealing with these diseases because of their unpredictability. Luckily right now, I’m in good shape with the TSC aspects & it’s just tracking research updates & advances. But there’s a few new aspects that are waiting in the wings for me to dig into.
When I was first diagnosed with LAM in 2003, it took me a good year to wrap my head around what this disease was & what it actually doing to my lungs. My abbreviated “elevator speech” I give when people ask about it is pretty polished & easy t0 follow by now. Or at least I try to make it that way.
But it’s so complex at times to keep up with everything that comes with self-managing this disease in particular. At first it was just a matter of interpreting my own understanding of the test results my doctors would share on both LAM and TSC. Then it was weighting risks and benefits of surgeries & treatments. It was also a lot of tracking numbers & images like MRIs & CTs. I pretty much had it down to a science for awhile.
Now, I have new learning curves to adjust to. Transplantation has so many fascinating aspects, but the learning curve of what goes into my scoring and listing is something I’m trying to come up to speed with and something I’m finding I really have to break down a bit & study over these next few months so I have a better understanding.
What I was trained to monitor for years as far as LAM progression are FEV1 and DCLO. Primarily FEV1. But those don’t factor in to the allocation score at least not as heavily as I had assumed. What actually does is the FVC ratio. But in all my years, I’ve never been aware of that or trained myself to ask for those numbers in addition to my other scores. I only recently learned this when coming up to speed with my evaluation tests & the listing process. Which is funny because not only in LAM progression is FEV1 a factor, it’s a criteria for qualifying for Social Security Disability benefits. So it seems really awkward that all three numbers aren’t weighted the same universally.
In my case right now, all 3 numbers are important for me to understand but for different reasons. The LAM Handbook actually is very helpful in breaking down the information with all 3 scores and why they are meaningful without jargonese. I’m fortunate to have a resource, because these types of nitty-gritty technical aspects of understanding test results are what I struggle with explaining to family, friends, & LAM sisters in an understandable but easy to follow way. I know what they mean to me but articulating that to others clearly & simply is harder.
(I recommend taking a look at that resource for further reading on these numbers & their interpretations if one is interested. Because explaining this in simple terms other than saying they are breathing numbers that tell me whether I’m getting more air trapping in my lungs or having a hard time getting air in or having struggles with both issues is about as simplified as I can make it in layman’s terms. Both of these issues and all 3 numbers are ultimately a measure of worsening lung function which leads to more difficulties breathing.)
I need to remember to print that section & go through my past results to get my updated ratios to see where I’m at (or at least see where on the test print out to find the FVC ratio). However, I was just surprised that it’s not really common knowledge or things I’ve felt have been driven home the importance of this in other spheres of educating us about our disease. So now I feel a bit behind the 8-ball so to speak that I hadn’t tracked this before now. But it’s never too late to start.
I also recommend this session from Lamposium. Dr. Gilbert does do a great job of breaking down the complexities of pulmonary function testing on a general level. Especially for new women with LAM who are looking for a little extra help in sorting out all those numbers & what they mean.
There is an allocation score calculator but I’m missing some data and will have that data after a few more tests. So I know I don’t have to sweat this right now so much since my doctors and I both need more testing information. I just need to remember on my next list of questions to have my transplant doctor re-review some things with me and how they interplay.
I know in November he’ll go through this with me step-by-step as he’s always been great at breaking things down for me even if he suspects I already may have some knowledge of it. He doesn’t just assume I know.
This subject was driven home to me in a few different conversations and ways that even as much “homework” on my diseases that I’ve done as they progress, there’s always more to do. It came up in a discussion of a close friend of mine who is post-transplant when she was following up on my evaluation results & we were tossing some questions around.
Also in just an ordinary conversation on health care issues from a new Twitter friend and healthcare geek, I realized how I had my own knowledge gaps but didn’t know how people who aren’t used to navigating the system or don’t have a lot of health literacy would manage to understand these things on their own (even with assistance) since they take a bit of time to wrap one’s head around, even for me.
The only reason I feel I pick it up faster is that I question other people, observe from other people’s experiences & file it all away in my memory bank for further use down the line. I am one who when it comes to health issues absolutely hates feeling blindsided, naive, dumb, or in the dark. Which I know I’m none of those it’s just I don’t like surprises that trigger those feelings. I’ve also felt the more I know then the more I can find ways to adapt to situations as they come (or even ahead of time), so doing my homework is how I obtain that knowledge. I learn from everyone & everything I can.
The other major learning curve that I’m curious about but is going to be explained to me in detail soon is the matching process (blood typing/antibodies/donor details). That I have to admit makes me feel like I’m starting back at square one with a new diagnosis, which I guess I am & it’s called end-stage lung disease/pre-transplant.
But, I’m lucky that I have a great support group & other information that might help me start digging into that a little deeper. I’ve watched a few good documentaries that have given me at least a good starting ground from liver, pancreatic, and kidney recipients and listees from their perspective & bit of basic insight. But it’s the scientific & medical data aspects & how they factor into the scoring (the real meat and potatoes that the doctors do and how it relates to timing & allocation on the donor end) that is something new to me & I want to get up to speed on.
But I feel in a good place to start my education & then once I learn perhaps someone else down the line might have the same questions but will have an easier time finding ways to come to their own understanding based off sharing my journey. One can hope.
I’m sure this is a common experience for anyone with a rare disease, chronic illness, or going through the transplant journey too & that I’m not alone with these questions. We just manage to deal & reconcile our questions & experiences differently.