People with Marfan syndrome can be at increased risk of sudden lung collapse (spontaneous pneumothorax). Sudden lung collapse is a collection of air or gas in the space between the lungs and the chest that “collapses” the lung and prevents it from inflating completely. While this condition is usually not life-threatening, it should be considered an emergency.
Symptoms of sudden lung collapse (pneumothorax)
Symptoms of a sudden lung collapse include chest pain that usually starts very suddenly. The pain is sharp and may lead to the feeling of tightness in the chest. Shortness of breath, rapid heart rate, rapid breathing, cough, and fatigue are other symptoms of pneumothorax. The skin may develop a bluish color (called cyanosis) due to decreases in blood oxygen levels.
What you can do
People with Marfan syndrome who require treatment for pneumothorax should tell the surgeons that they have Marfan syndrome and may need aortic surgery in the future. This will enable the surgeons to make the best treatment recommendation.
Describe the pain completely
Be prepared to answer the following questions. If your doctor does not ask, speak up and tell him/her anyway. Be prepared to answer:
- Where is the pain located?
- How severe is the pain?
- When did it start?
- What does the pain feel like?
- Does the pain radiate (move) to other areas of the body (for example, the back, neck or arms)?
- Is this kind of pain like anything you have ever felt before?
Tell the surgeons that you have Marfan syndrome and may need aortic surgery in the future.
This will enable the surgeons to make the best treatment recommendation among options that may include supplemental oxygen, chest tube insertion or pleurodesis, which involves scarring the lung surface to attach the lung to the chest wall. The best pleurodesis method for people with Marfan syndrome is mechanical rather than chemical pleurodesis, as the former makes cardiac surgery easier.
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