About Hereditary Lymphedema

What is Hereditary Lymphedema?

Hereditary lymphedema is a rare genetic disorder that is characterized by the abnormal accumulation of lymph fluid in the body's tissues, leading to swelling. It is caused by a mutation in the genes responsible for the development and maintenance of the lymphatic system. Symptoms of hereditary lymphedema can include swelling of the arms, legs, and/or trunk, as well as thickening of the skin in the affected areas. Treatment typically involves compression garments, physical therapy, and/or surgery.

What are the symptoms of Hereditary Lymphedema?

The most common symptoms of Hereditary Lymphedema include:

- Swelling of the arms, legs, or other parts of the body
- Aching or heaviness in the affected area
- Tightness of the skin
- Reduced flexibility in the affected area
- Recurring infections in the affected area
- Hardening and thickening of the skin in the affected area
- Abnormal accumulation of fluid in the affected area

What are the causes of Hereditary Lymphedema?

Hereditary lymphedema is caused by a genetic mutation that affects the development of the lymphatic system. This mutation can be passed down from parent to child, and is most commonly seen in families with a history of the condition. Other causes of hereditary lymphedema include Turner syndrome, Noonan syndrome, and congenital malformations of the lymphatic system.

What are the treatments for Hereditary Lymphedema?

1. Compression garments: Compression garments are specialized garments that are designed to apply pressure to the affected area to reduce swelling and improve lymphatic drainage.

2. Exercise: Exercise can help to improve lymphatic drainage and reduce swelling.

3. Manual lymphatic drainage: Manual lymphatic drainage is a type of massage that helps to move lymph fluid through the body and reduce swelling.

4. Surgery: Surgery may be recommended in some cases to remove excess tissue or to reconstruct the lymphatic system.

5. Medications: Medications such as diuretics may be prescribed to reduce swelling.

6. Skin care: Proper skin care is important to prevent infection and reduce swelling.

What are the risk factors for Hereditary Lymphedema?

1. Family history of Hereditary Lymphedema
2. Certain genetic mutations
3. Certain medical conditions, such as Turner Syndrome, Noonan Syndrome, and Proteus Syndrome
4. Certain medications, such as those used to treat cancer
5. Certain infections, such as filariasis
6. Trauma to the lymphatic system, such as surgery or radiation therapy
7. Obesity

Is there a cure/medications for Hereditary Lymphedema?

Yes, there are treatments available for Hereditary Lymphedema. These include compression garments, manual lymphatic drainage, exercise, and medications such as diuretics and anti-inflammatory drugs. Surgery may also be an option in some cases.