What precautions should a patient of Marfan's disease take?
Q: I am a 30y unmarried male living in Mumbai. Last month while on a hiking trip, I suddenly developed chest pain and coughing but I still continued hiking. On my return, as these symtoms persisted, I visited a local doctor who advised a X-ray. He said that I had pneumothorax and my left lung was collapsed. I was advised admission to hospital where an intercostal drain (ICD) was inserted in the left side to drain the pleural fluid. Even then my lung did not return to normal, so another ICD was inserted (this time on the top nearly between 2nd and 3rd rib) to drain the air. My condition improved and the lung started expanding. I was in the hospital for 20 days. My lung expanded but not fully so doctors advised removal of both the ICDs one after another within a gap of 3 days. My breathing was (I feel) shallow and I would gasp for breath at times. The latest X-ray shows some air pockets as per the doctors. The also said that it would take a long time to heal and the air that is leaking is due to a rupture in the bullae, which could be one or two. The air present in the pleural space will eventually get absorbed in the body but could take upto 3 months or so. My condition is still uneasy, I take short breaths, and I easily get tired and fatigued if I walk for 10 mins or more. At present I am at home. During my hospital stay, the doctors said that I have something called as Marfans disorder. I am thin and tall. I had also undergone an operation for aneurysm in the brain 5 years ago. I want to know: 1) How will this affect me just now and in the future? 2) What are the precautions I should take? 3) What is the treatment, I mean preventive care I should undergo? 4) Are there any specialised care/treatment centers in and around Mumbai for this type of problem? 5) What are the changes in my food habits/work etc that I should make? 6) How do I lead a normal life?
A:Marfans disease puts people at risk for pneumothorax as well as aneurysms, heart disease, joint complications and other problems which can vary considerably. Pneumothorax usually resolves eventually, but sometimes a sclerosing solution needs to be injected into the chest. In resitant symptomatic cases, chest surgery and sewing up of the leak or removal of part of the lung will be needed. Some chest surgeons are now doing a less extensive procedure called video-assisted thorascopic surgery (VATS), and this is certainly preferable if available. There are no specific dietary or life style changes that help. However, vigorous exercise should be avoided particularly in hazardous situations such as diving or mountaineering. Airline flights also increase the risk of a recurrent pneumothorax.