LAM Support Ireland

LAM or Lymphangioleiomoymatosis is rare lung disease that only affects women especially in child-bearing years.
This web page has been set up in order to highlight the disease LAM and to help Irish women find the different groups in Europe and America that are helping to highlight this disease.

There are different types and stages of LAM. When you are first diagnosed there is a lot of information to take in but over time you will come to know that many women across the world have this rare disease and each are at different stages. Some might need oxygen others might never require it.

We need to highlight LAM in the medical field in Ireland and increase awarenesss of this rare disease amongst doctors.

At present the UK have a patient support group that Irish women are more then welcome to join and also there is a facebook group called lammies. Please see our links page for details.

LAM mainly affects the lungs, where an abnormal type of smooth muscle cell (called a LAM cell) builds up. This build up of LAM cells occurs around the airways (breathing tubes) and also around the blood vessels and the lymph vessels (lymph vessels drain excess fluid from the lungs). The LAM cells lead to cysts developing in the lungs and are  very clear on a CT lung scan. The main effects of LAM are in the lung but quite a few patients also have a tumour in the kidneys and sometimes more than one; they are not malignant but can cause bleeding. Lymph nodes may be enlarged in other parts of the body but they don’t usually cause problems.
The name lymphangioleiomyomatosis reflects the different components of the disease. Lymph and angio refer to the lymph and blood vessels that are involved and leiomyo refers to the smooth muscle. LAM is sometimes called sporadic LAM to distinguish it from LAM that occurs in patients who have tuberous sclerosis (see LAM and Tuberous Sclerosis.)

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Symptoms

What are signs or symptoms of LAM?

For many patients with LAM the first sign of the disorder is when they develop a collapsed lung, known as a pneumothorax. This occurs when one of the cysts bursts and air leaks into the space around the lung. A pneumothorax usually causes sudden breathlessness, often with a sharp pain, and it requires treatment in hospital.  If the pneumothorax recurs a small operation is usually needed; this will prevent a further pneumothorax though some patients continue to be aware of occasional gurgling in their chest after the operation.

 Many symptoms of LAM are similar to those of more common lung diseases such as asthma and for this reason patients may have symptoms for some time before LAM is diagnosed. Symptoms, X-rays and breathing (lung function) tests may suggest LAM but the diagnosis is usually confirmed by a CT scan (a detailed X-ray scan) of the lungs and sometimes with a lung biopsy. In a patient with LAM the CT scan usually shows typical cysts throughout the lungs.

In around one third of cases a lung biopsy may be performed to ensure that the diagnosis is correct. This is usually carried out through a fairly small incision in the chest under a general anaesthetic. With LAM the biopsy will show the typical cysts and LAM cells. The kidney tumours are also seen best on a CT scan although they can also be seen with ultrasound. Patients with kidney tumours or tuberous sclerosis seldom need to have a lung biopsy performed and these features should be looked for before deciding to perform a lung biopsy.

As the LAM cells enlarge there is a fall in lung function but the rate at which this occurs varies markedly between patients. A few patients still have moderately preserved lung function after 20 years whilst others are less fortunate and deteriorate quite rapidly. Most patients are between these extremes. Looking at change in lung function over a period of time gives a good indication of the rate at which the disease is progressing in an individual patient.

Kidney Tumours

Some patients with LAM have a benign tumour in the kidney (called an angiomyolipoma). Most kidney tumours
in LAM are small, do not cause symptoms and do not need treatment as they are benign. Patients with
LAM should have a kidney scan to see if angiomyolipomas are present, and if they are present, they should
be monitored to ensure they are not growing. Occasionally larger tumours cause pain or bleeding and may
need to be treated. This is done either by removing the tumour with an operation or blocking its blood supply
(embolisation) which causes it to shrink. Embolisation is done through a catheter inserted into the artery to the
kidney and it does not normally need a general anaesthetic. Patients recover more quickly from embolisation.
It is not possible to treat all tumours in this way however and the procedure is not performed in all hospitals.
The aim of treatment is to preserve as much normal kidney as possible, and avoid removing a kidney unless
there is no alternative.

Tuberous Sclerosis

Both the lung condition and the kidney tumours seen in LAM can occur in patients with a disease called
tuberous sclerosis. Tuberous sclerosis is generally associated with unusual skin changes, tumours in other
organs and sometimes epilepsy (fits), learning and behavioural problems. There is a very important difference
between tuberous sclerosis and sporadic LAM which is that tuberous sclerosis is inherited whereas LAM is sporadic.

What is LAM?

LAM, or lymphangioleiomyomatosis (lim-FAN-je-o-LI-o-MI-o-ma-TO-sis), is a rare lung disease that mostly affects women of childbearing age.
In LAM, abnormal, muscle-like cells begin to grow out of control in certain organs or tissues, especially the lungs, lymph nodes, and kidneys.
Over time, these LAM cells can destroy the normal lung tissue. As a result, air can’t move freely in and out of the lungs. In some cases, this means the lungs can’t supply the body’s other organs with enough oxygen.


Overview

There are two forms of LAM. Sporadic LAM occurs for unknown reasons. LAM also can occur in women who have a rare disease called tuberous sclerosis complex  (TSC). Women who have TSC often have a milder form of LAM.
About 50 percent of women who have LAM develop pneumothorax (noo-mo-THOR-aks), or collapsed lung. In this condition, air leaks out of the lung and into the space between the lung and chest wall (the pleural space).
A collapsed lung can cause pain and shortness of breath. Sometimes one lung will collapse over and over again. Pneumothorax is a serious condition. It usually requires treatment and might be life threatening.

Figure A shows the location of the lungs and airways in the body. The inset image shows a cross-section of a healthy lung. Figure B shows a view of the lungs with LAM and a collapsed lung (pneumothorax). The inset image shows a cross-section of a lung with LAM.
Many women who have LAM get tumors called angiomyolipomas (AN-je-o-my-o-li-PO-mas), or AMLs, in their kidneys. Women who have LAM also may develop:
•    Growths in other organs, including the liver and brain
•    Large tumors in their lymph nodes
•    A buildup of fluid in their chests, abdomens, or pelvic areas