| |
Also known as arachnodactyly, Marfan's syndrome is a rare hereditary disorder that causes connective tissue to be weaker than normal. Affected individuals are most often unusually tall and slender, with particularly long arms, legs and fingers in comparison to the rest of their body. This condition is thought to affect approximately one person in 10,000 in the US.
While there is no cure, there are effective treatments, and most people with the disorder live full active lives.
Conventional treatment recommendations include such things as:
1. Ocular and cardiovascular surveillance of all people with Marfan syndrome every 6-12 months 2. Activity restrictions: NO Isometric exercise (no weight lifting) No sudden acceleration/deceleration activities (roller coasters, bungy jumping, drag racing) No unpressurized flights; No SCUBA diving No competitive sports No contact sports 3. Medications to minimize the rate of aortic root dilatation (beta blocking agents, calcium channel blocking agents) 4. Adherence to prophylactic antibiotic recommendations as indicated
Close surveillance of the Marfan aorta is recommended during pregnancy. If aortic enlargement is detected in the early stages of pregnancy, aortic imaging studies are recommended every 1 to 3 months until delivery.
|
|