Lymphangioleiomyomatosis (LAM)

Trainee Blebber Joined: 21 Sep 2006 Posts: 22

I now know why I had my two SPs. Last week I was diagnosed with lymphangioleiomyomatosis (or LAM for short).

It is a rare lung disease that affects almost exclusively women, as it seems to be releated to estrogen. The disease is characterized by an unusual type of muscle cell that invades the tissue of the lungs, including the airways, and blood and lymph vessels. Over time, these muscle cells grow into the walls of the airways, and blood and lymph vessels, causing them to become obstructed. The muscle cells block the flow of air, blood, and lymph vessels to and from the lungs, preventing the lungs from providing oxygen to the rest of the body.

Symptoms of LAM include shortness of breath and SP.

I was so depressed and scared at first, but I have done reading and have found out that its not uncommon to live 20 to 30 years after diagnosis. There are also several new drug trials going on now. I am trying my best to stay positive because freaking out will do me no good.

If you are a woman who has had a SP you may want to ask your doctor about this. There are estimates that up to 300,000 women have the disease but have not been diagnosed.

You can read more at www.thelamfoundation.org

Trainee Blebber Joined: 21 Mar 2006 Posts: 30

Hi,

Do you mind my asking at what age your SPs started for you?

I've heard of LAM and was concerned that I have it as well...

Thanks,

Connie

Trainee Blebber Joined: 21 Sep 2006 Posts: 22

Hi Connie-

I have had two SPs, both at age 31. I found out I had LAM after the 2nd SP. I had VATS done, the surgeon did a biopsy and it came back positive for LAM.

Good luck and find out as much as you can. If you do have LAM the earlier you get diagnosed the better.

Hugs,

Nikki

Trainee Blebber Joined: 21 Mar 2006 Posts: 30

Hi Nikki -

I did have VATS after my 4th SP and they did do a biopsy. It came back as microscopic blebs and emphysemous changes to lung tissue, but they didn't mention LAM? Would most surgeons/pathologists know to look for LAM?
I've been feeling a bit out of breath lately even though I don't currently have an SP so I'm concerned. It might also be anemia I"m thinking because I've been having unusually heavy menstrual periods (gyno diagnosed uterine fibroids - when it rains, it pours!)

Thanks for the info,

-Connie

Trainee Blebber Joined: 21 Sep 2006 Posts: 22

HI Connie-

If they did a biopsy, and it was clear, then you are probably okay. However, we hear about missed diagnosis all the time so I'd talk to your doctor about it if you are concerned.

Hugs,

Nikki

Trainee Blebber Joined: 13 Feb 2006 Posts: 36 Location: UK

Nikki, I am so sorry to hear that you've been diagnosed with LAM. I take my hat off to you for your positive attitude and sharing your experiences.
All the best
Anna

Trainee Blebber Joined: 21 Sep 2006 Posts: 22

Hey everyone. Thought I'd give you an update on how I'm doing with my lungs. Since my first 2 SPs in the fall of 2006, I have not had any recurrences. So far so good. In fact, last time I got checked, my FEV1 was 106% for a woman of my height. That's better than average. FEV1 is the amount of air one is able to exhale in one second.

I do my best to exercise. Which usually means 30-40 minutes on the elliptical machine at least four times a week. I have no problem doing with this activity; no shortness of breath at all. I also go swing dancing at least twice a week and can dance all night with no problem.

Here's the odd thing though. There are some activities that leave me out of breath and feeling very weak right away, primarily running, climbing stairs, and lifting and carrying heavy objects. For example, on one trip the handle on my heavy rolling carry-on suitcase broke. I had to carry it along the plane's aisle and down the jetway to the gate. By the time I got to the gate, I was painfully short of breath and cussing like a sailor. Wink

So the way I cope is I just try to avoid activities that bother me, and increase the physical activity that I can do with little or no problem.

Here are some other things I've done since I've been diagnosed. I minimize stress as much as possible, at work and in personal relationships. I've re-evaluated what's important in life and sweating a silly deadline or silly relationship is just not worth the effort anymore.

Also, since researchers believe LAM is somehow related to estrogen, I've stopped taking birth control pills and minimized my soy intake (phytoestrogens.)

I've never been a smoker, but now I try to avoid smoke if possible. For example, I won't date someone or hang out with people who have issues with NOT smoking around me.

Anyway, I really want to say this again. If you are a woman who is experiencing shortness of breath or has had a SP, especially more than one SP, talk to your doctor about being screened for LAM. It will not show up in an x-ray. You need a biopsy or a cat scan. Please note that most doctors, even pulmonologists, know little or nothing about LAM. Every single time I've had to got to the ER for a suspected SP, which thankfully were all false alarms after the first two SPs, I had to explain LAM for the ER doctors and nurses.

Point them to www.thelamfoundation.org for more information.

I know it's scary. Believe me, I was scared sh*tless when I was diagnosed. But the earlier you find out, the sooner you can start altering your lifestyle or possibly even enrolling in clinical trials to find a treatment and maybe even a cure. There are trials going on around the US right now. You are eligible if your FEV1 is 70% or lower.

A'ight girls. Stand firm.

Posted: Tue Mar 10, 2009 7:18 pm Post subject:

Hi Nikki,

Firstly can I say I admire you for staying so positive - go girl!

I'm 37 and have had 3 SPs in my left lung and two Pleurodesis Ops. My first SP was at the age of 25, then 26 and more recently at the age of 37. I've just had my second Pleurodesis Op and also had a Bronchoscopy and Biospy at the same time. I hadn't heard of LAM until I came out of hospital (I found info on the internet) - and I'm not sure if the Surgeon thought to test for LAM?? Also by coincidence - a routine health check last May showed blood in my urine - I've had it investigated but no apparent cause. I understand that LAM can also cause kidney problems??

I've asked for a CT scan to check for LAM. I raised LAM with my GP and she seems to be taking it seriously and doing the research. Are you aware of any other tests?

Nic x

Trainee Blebber Joined: 21 Sep 2006 Posts: 22

hi- sorry for the late reply. cat scans and biopsy of the lungs are the main tests for lam.