Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare lung disease which affects mainly women. It’s a type of cystic lung disease that is characterised by abnormal growth of smooth muscle cells in the lungs, lymph vessels and abdomen. This leads to the formation of tumours or cysts and causes breathing difficulties. LAM can also affect the lymph vessels, kidneys and other bodily organs.

LAM typically develops during early to mid-adulthood, typically between ages 15 and 50, and is more common among women than men. It’s sometimes linked to the genetic disorder known as tuberous sclerosis complex (TSC). LAM is a progressive condition, and its symptoms can vary widely from person to person.

Symptoms

Shortness of breath during exercise
Coughing, sometimes with blood
Chest pain
Wheezing
Fatigue
Unintelligible speech
Loss of appetite
Swelling of the face, legs, or abdomen

Diagnosis and Treatment

LAM is difficult to diagnose and can be misdiagnosed as asthma, bronchitis, or other respiratory illnesses. Diagnosis is usually established through a combination of physical exam, laboratory tests, imaging studies, and pulmonary function tests. Treatment for LAM is tailored to a person's unique needs and may include a combination of medications, lifestyle changes and other therapies.

Medications, such as corticosteroids and immunosuppressants, can slow the progression of the disease and help to reduce symptoms. Oxygen therapy may also be recommended, and some patients can benefit from surgery. In some rare cases, lung transplantation may be an option. Physical activity is important for people with LAM and can help to improve exercise capacity and quality of life.

Coping and Support

Living with LAM can have a significant emotional effect, and patients may experience fear, stress, and depression due to the uncertainty of the condition. It’s important to seek out support from people who understand what you’re going through. There are local support groups and online forums that can provide a safe and supportive environment, where you can connect with others who are going through similar experiences. Additionally, talking to a counsellor or therapist can be a helpful way to manage emotional distress.