Pulmonary vaso-occlusive disease
Treatment
There is currently no known effective medical treatment. However, the following medications may be helpful for some patients:
- Vasodilator drugs (drugs that widen the blood vessels)
- Drugs that control immune system's response (such as Azathioprine or steroids)
A lung transplant may be needed.
Expectations (prognosis)
The outcome is often very poor in infants with a survival rate of just a few weeks. Survival may be months to a few years in adults.
Complications
- Progressive
difficulty breathing - Pulmonary hypertension
- Right sided heart failure (cor pulmonale)
- Coughing up blood
Calling your health care provider
Call your health care provider if you have symptoms of this disorder.
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