I wasn’t able to join that day but I did skim through this & found it to be a good basic primer for people who know a little bit about both diseases but want to learn more.
As many followers of my blog may be aware I have both Tuberous Sclerosis Complex (TSC) & Lymphangioleiomyomatosis (LAM). I developed the TSC form of LAM when I was 26 years old. There are women who develop a sporadic form of LAM (they don’t have a history of TSC).
Because of that fact, & because there’s a high likelihood of women with TSC going on to develop LAM (just like I did), I’m sharing this webinar in hopes of spreading education & awareness on both diseases.
Also primarily because the surveillance is changing.
It used to be just an initial baseline CT was recommended but no further imaging unless there was a problem. I’m glad to see the criteria has changed on that to updated scans every 5-10 years.
I think this is smart.
In my case, I had my first lung collapse before LAM even showed up. It was only after a resident warned me because he saw TSC in my medical history did I even think to get baselines after treatment.
A year & a half later, when I was just diving into volunteering heavy duty, I started noticing changes. I did get screened & then the markings showed up on my CT.
I do credit the early diagnosis with being able to stave off transplant for so long.
In the early days, my journey was not easy because the predominant thinking was that because I had TSC & LAM both that my LAM would take a slower progression. This did not turn out to be the case for me.
The only reason I was able to delay transplant talks until last year was because I did respond to sirolimus (or Rapamune) for a good number of years. However, though it did stop many dramatic drops while on it, I was below 50 percent lung function when I started & continued to have drops while on the drug, albeit less severe and less symptomatic than before I was treated.
In 2009 when I did go on it off-label (since I didn’t qualify for a trial at the time & couldn’t risk placebo if I had) I honestly wasn’t sure if I would respond or if it would slow my progression. Thankfully, it did some. But it also did clear my kidneys to a point where those are less of a risk factor now than they were 5 years ago. It’s possible that the transplant drugs might not be well tolerated by my kidneys, & may fail down the line. That risk seems to be a bit more mitigated now than what it once was which was another plus of the drug.
But it doesn’t work for everyone & unfortunately if one does have TSC, they do have to be vigilant & on the look out for LAM. Especially if they are female. The earlier any of these drugs in trial or approved treatments are started, the better the likelihood of curbing or slowing the decline.
For the first few years of my diagnosis, I really did feel like I was an anomaly amongst my peers, especially when I started progressing & having numerous issues.
Yet, as time wore on I did meet more women across the entire LAM spectrum & that made me feel less lonely, although it was very difficult at first because there was no treatment at that time. Some women weren’t diagnosed with LAM until they were very advanced & little could be done for them.
It’s frustrating to have to live with, but I have always felt that knowing what I’m fighting is better than being in the dark or having limited information.
I’m glad the two organizations are working together to recognize what a hardship life with LAM can be & help people get diagnosed earlier but then also steer research to hopefully more treatment options down the line.