Unfortunately there is no cure for LAM as yet. Treatment may be given to deal with symptoms or complications (supportive treatment) or it may be given in the hope that it will reduce the rate at which LAM progresses.
Supportive treatment
For breathlessness. The treatment for breathlessness depends on the cause of the breathlessness. For example, if there is a pneumothorax or a pleural effusion treating these should help the breathlessness. Some patients benefit from the B-agonist inhalers used for asthma such as Ventolin and Bricanyl.
Fluid on the chest (pleural effusion). If this is large it may be helpful to remove the fluid though in the long term it is better to prevent it accumulating. This may be helped by a low fat diet or by progesterone treatment (see below).If it continues to build up it may need a small operation to stick the outside of the lung to the inside of the ribcage. This is known as a pleurodesis and is carried out with a general anaesthetic.
Pneumothorax. This is usually treated initially by sucking the air out of the space round the lung with a needle or tube inserted under a local anaesthetic. If it recurs it is may also be treated by a pleurodesis so that it can't collapse again.
Oxygen. When breathlessness becomes more troublesome breathing additional oxygen may provide some relief. Oxygen can be given from oxygen cylinders or from a machine called a concentrator which extracts oxygen from air. Having a concentrator is worthwhile if you need oxygen for several hours a day and means you don't need to keep replacing oxygen cylinders which may only last for 6 hours. There are no hard and fast rules as to when oxygen should be started but patients who have to stop after 100 to 200 yards are likely to benefit.
Lung transplant. Lung transplantation is a possibility for patients with severe LAM. More than one hundred patients with LAM have had a lung transplant and the outcome appears to be similar to patients who have had a transplant for other conditions. A lung transplant is a major undertaking, however, and the results for lung transplantation are not yet as good as those for a kidney transplant. It is only considered therefore when LAM has become severe.
General
Smoking. It is clearly not sensible to smoke if you have LAM since the damage from smoking will only add to that of LAM. There is also some evidence that smoking may make LAM worse.
'Flu jab. As with all patients with a significant lung condition it is sensible to have a 'flu jab each winter. It may also be worth having a pneumococcal vaccination to reduce the risk of pneumonia. This should be discussed with your doctor.
Keeping fit. There is good evidence from other types of lung diseases that there are benefits from keeping fit. When LAM becomes more severe a pulmonary rehabilitation course may provide an extra stimulus but the main thing is to walk as much as you can.
Juliet Stevenson