I’ll be posting a bit more often this month because I want to support the first WorldWide LAM Awareness Month.
Today, I’m going to talk about a statement that has really worked my nerve for quite some time on my LAM/TSC journey.
I just don’t agree with it.
I know why it’s said, but my experience & the experience of some other women don’t sync up with this statement.
“If you have TSC (tuberous sclerosis complex) the progression of your LAM will be slower than those with sporadic LAM.”
The only reason I was able to go 13 years with LAM until I was transplanted was because for 6 of those years, Rapamune worked for me.
It didn’t hold off my progression entirely, but it did curb it quite a bit.
Prior to me being on that drug, I had at least 10 point drops in my function (annually) for several years.
I started the drug in 2009. I was diagnosed in late 2003. (That’s about a 5 year window).
When I was diagnosed in 2003, I had normal lung function (letter perfect, in fact).
By 2009, I had 47 percent function. I also had 3 pleurodesis surgeries on both lungs (2 on the left) to glue my lungs to my chest wall to keep them from collapsing.
So, in 5 years I lost more than half of my lung function.
Without that drug I invariably would have needed a transplant evaluation within a year or two, as around 30-35 percent or a little higher is when they start testing to see if one qualifies for transplant.
This was only what was transpiring in my lungs.
It did not take into account what was happening in my kidneys & other parts of my body. There was havoc there too.
My progression was not as rapid as some women for sure. But it certainly did concern my doctors & several others I reached out to for help.
Some of them seemed genuinely shocked I was having these steep drops & these continual issues.
In 2009-2014, I felt much better. I was used to compensating for my shortness of breath by then, but I certainly didn’t feel as sick as I had before.
I also managed to stay out of the hospital.
I stopped asking for number updates at my lung checkups at each visit.
In those 5 years of relative calm, I noticed in reviewing past function numbers (in 2015, after my problems started again). I had still lost another 10-15 points of function overall in those 5 years of treatment with Rapamune. This was an improvement, (because it wasn’t 10 points annually anymore) but still not quite good enough, as I was already under 50 percent function when I started the drug.
When I found out I needed oxygen for exercise in April 2015, I was at 33 percent. But things were about to take a steep dive.
By July of 2015, I was on oxygen full-time, not just for exercise.
I had months I was losing 5 points of function per visit, even with the drug.
Just before listing in March of 2016, they pulled me off the drug in preparation for transplant since it affects my body’s ability to heal.
I went from 30 percent down to about 18 or 20 percent in those few short months.
They were concerned about that, since they weren’t sure how long I was going to have to wait on the list, so they put me on a very low dose, despite the small risk.
That time I didn’t rebound either. Still had drops, but of course smaller.
I was at 12 percent function 9 months later when I was transplanted in December of 2016.
At 12 percent function, I was at 8 liters of oxygen to do anything. I was inching to using 10 liters.
I could barely leave the house for rehab or appointments. I’d wake up in the middle of the night gasping for air.
I don’t sugarcoat my experience or say it’s indicative of all women, it’s completely mine & mine alone.
But, this is why I took issue with that statement of TSC women “having it easier” (so to speak), because it was not at all true for me.
(It also wasn’t true of one of my best friends with both diseases who died.)
From my LAM diagnosis to transplant, I only had 6 months of normal lung function, not years.
On top of it, I also had asthma, which made everything harder.
I was a spontaneous mutation (meaning my TSC wasn’t passed down in my family). I had knowledge of this illness as young child which always made me feel a bit different from most children.
Then I grew into an adult who just had reconciled my TSC, then I had to start all over & try to accept LAM.
But LAM was even more insidious it seemed, & for a long time I was really resentful because it was intent on blowing up my life.
The sicker I grew, the more isolated I felt.
Especially as I heard that statement being repeated more & more: “If you have TSC the progression of your LAM will be slower than those with sporadic LAM.”
I silently “called bullshit” on this statement.
I’d sit in forums from time-to-time & address full on the ugliness of having to face my own mortality at an early age.
I didn’t hide the struggle when speaking with people one-on-one. I couldn’t.
I knew better but occasionally I’d ask myself in the beginning (when that statement would cut me & I wasn’t sure how to respond), “Where did I go wrong?” “Why am I progressing like a lot of sisters with sporadic LAM?”
“Why do I feel if I’m honest about this sometimes within communities or with doctors, I fear being looked at as being overdramatic or having 3 heads?”
This is me though. It’s happening to me. LAM is destroying my life & at a rapid pace.
I had wished I never heard that statement uttered at all.
I was already “an exception to the rule” in my circle of peers outside of illness. I didn’t want to be one in it.
Then I decided the only way to counter a statement like this was to speak out against it.
From then on, when I heard that statement, I’d retort back, “That isn’t true in my case or some others.” “It’s not something that can be relied upon as fact or certainty. “
I don’t believe in being lulled into a false sense of security.
I also don’t believe that those of us who the statement doesn’t hold true for should just be overlooked or dismissed, with a “that’s them/one person” or “your case is just the exception, oh well”.
Instead, own the statement is problematic & stop trumpeting it as absolute truth.
If hell bent on using such a statement, these are closer to the truth:
“If you have TSC the progression of your LAM (may be/could be/might be) slower than those with sporadic LAM.”
“If you have TSC the progression of your LAM has the potential to be slower than those with sporadic LAM.”
(Note: pleurodesis is a surgery in which they glue the lung to the chest wall to keep it from collapsing. It can however still happen, but usually the collapses are smaller. I had to have it done twice on my left because the first time it didn’t hold. Sporadic LAM is LAM without TSC aka tuberous sclerosis complex. Normal lung function is 90 percent or above. Mild LAM is considered 80-90 percent, Moderate LAM is considered 70-80 percent, Moderate-Severe LAM is 60-70 percent, & Severe LAM is less than 50 percent. These values have changed since my diagnosis. It used to be 40 percent & lower was considered severe LAM, there was no moderate-severe score. [Thank you LAM Handbook for the update.] )
First – where did this statement come from?
Second, I hate generalizations – that people/doctors/organizations, etc think that a disease can only progress or happen in one way. I know so many people that have experienced years of suffering or misdiagnosis or complications that were treated improperly because of statements like this that make someone think a divergent experience couldn’t be “real” – because they think that diseases have to fit some sort of pre-defined outline.
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I think it started with paraphrasing (incorrectly) this study statement from the NIH “Overall, lung disease tends to be milder in patients with TSC than is seen in the sporadic LAM population…” — in their natural history study. URL: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2883494/ — the paragraph under Tuberous Sclerosis complex. But then it has been published elsewhere in both lay & scientific community in various iterations & presentations. Now “tends” is not as absolute as “will be”. I know that might be parsing words/semantics, but if you read the rest of that paragraph in context the NIH said that was their trend in their study patients at the time, with how their subjects presented & not an absolute.
second – I agree w/you on generalizations 🙂
I also agree as I know at least one lady here in Australia that has TS/Lam and also had to have a lung transplant. Also I’m glad they have finally removed the 10 years to live once diagnosed. I think they have to remember that any disease condition will be different to each person.
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