LAM Medical Investigations
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> Chest X-Ray
May appear normal, particularly in early stages of LAM. -
> High-Resolution CT Scan
High-resolution CT scan of the chest is required to detect cysts or abnormal clusters of cells in the lungs. Most women are diagnosed this way. -
> Lung Biopsy
Due to improved accuracy of CT scans, biopsies are no longer considered essential to diagnosis. A biopsy requires a small piece of lung tissue to be removed for microscopic analysis, either during a bronchoscopy or via a keyhole incision in the chest wall, risking pneumothorax. -
> Abdominal CT Scan
Abdominal CT scan may detect AMLs, but these can exist in the absence of LAM. -
> Lung Function Tests (Spirometry)
Lung function tests (spirometry) cannot be used to diagnose LAM and may be misleading as some changes are suggestive of asthma or emphysema. -
> Blood Test
A blood test may prove helpful if current studies prove that VEGF-D a marker in the bloodstream, is an indicator of LAM.
Major Donors
Macquarie Group Foundation
Henry H. Roth Charitable Foundation
Hollick Wines
Acknowledgements
LARA thanks the professionals who work pro bono for this not-for-profit organisation. We highly recommend the services of:
Ben Higham, Webhead
Karen Riethmuller, KGR Design
Allan Hughan, Accountant
Peter Kelso, Bartier Perry Lawyers
Contact Us
LAM Australasia Research Alliance
Nevena 0419 185 491
Janet 0411 816 444
PO Box 636 Bondi Junction NSW 1355
Australia
What is LAM?
Living with LAM
A woman who has been living with LAM for 15 years writes:
"It's understandable that you are anxious, but try not to worry. I know it's easier said than done. I've been anxious too lately. Last night I walked along the beach and felt the lapping waves soothing me. I reminded myself that fear has no place in my life and is just an unpleasant byproduct of my inability to think through things in a calm and rational way. I tried to imagine a peaceful sphere around myself in which I could be totally aware of myself in the present. Anything which wasn't compatible with the space within that sphere, like anxiety about the future or regrets from the past, I put outside. As I walked along holding this sphere of peace around me I felt all my senses harmonise with my surroundings. Most of the time our thoughts are so loud they drown out our experience of the moment, depriving us of clarity. Once I held on to the present for a while it was like being fully aware of every instrument In the orchestra for each second of a wonderful performance."
A rare and progressive lung disease, LAM, or lymphangioleiomyomatosis, usually attacks women in their childbearing years, with a variety of manifestations.
Because it is rare, many GPs and even specialists are unfamiliar with LAM and may misdiagnose symptoms as due to asthma, bronchitis, emphysema or depression.
Breathlessness and AMLs
The first sign of LAM is often breathlessness. This may be accompanied by chest pain, lung collapse, or coughing up blood. Women with LAM may be unaware of changes to their lungs, but have angiomyolipomas (AMLs). These so-called "benign" tumours on the kidneys affect 40% of women with LAM and are asymptomatic unless they burst. Some women develop benign tumours within their abdomen (lymphangioleiomyomas), enlarged lymph nodes, and free fluid within the abdominal cavity (chylous ascites).
Pneumothorax
Pneumothorax or lung collapse occurs when a cyst close to the surface of the lung ruptures, letting air leak into the gap between the lung and the chest wall.
What is known about LAM?
LAM comes in many forms. The two major types are TS/LAM and Sporadic LAM. TS/LAM is linked with tuberous sclerosis and may be hereditary. It is more common (40 per million) and less progressive. Sporadic LAM is not inherited. Caused by a random gene mutation, it occurs in about 10 per million.
Disease process
In some women LAM develops rapidly. Currently the only proven treatment is a lung transplant.
In other/older women the disease progresses more slowly, particularly after menopause.
- LAM is not caused by lifestyle choices.
- LAM is not contagious.
- LAM develops after puberty and appears to be accelerated by the hormone oestrogen.
- Pregnancy and hormonal medication may affect the progress of LAM.
- Average age of diagnosis is 35 years, but most women notice symptoms long before LAM is detected.
Is there a cure for LAM?
Currently there is no cure, but several drugs are being investigated. Rapamycin (Sirolimus) has shown significant promise and is currently being trialled in the USA and Japan. Doxycycline, cholesterol-lowering drugs (Statins) and oestrogen-blocking agents like Arimidex are also being studied.
Until a sure fire therapeutic drug treatment is found, lung transplantation is the ultimate remedy for LAM. Several women in Australia are enjoying the benefits of new LAM-free lungs.
Diagnosing LAM
LAM can be difficult to diagnose. The signs and symptoms of LAM are similar to many other lung diseases like asthma, emphysema and bronchitis. Some women may feel that asthma treatments like Ventolin temporarily improve their breathing.