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Organizations

• International Federation of Marfan Syndrome Organizations
• National Marfan Foundation
• Canadian Marfan Association
• Marfan Association UK
• Marfan Network Japan
• More National Organizations...

How are the lungs affected?

The lungs need connective tissue to provide stability and elasticity. The abnormal connective tissue decreases the elasticity and stability of the small air sacs within their lungs. The air sacs may be unusually large after becoming swollen or stretching out considerably. This does not cause problems for all individuals with Marfan Syndrome. However, many people will suffer one of the following conditions:

Collapsed Lung

Individuals diagnosed with Marfan Syndrome often experience “pneumothorax” or a collapsed lung. Individuals with the disorder have a 50 times higher chance of having this occur than the general population. In fact, approximately 1 out of every 20 people with MFS will eventually suffer a collapsed lung.

Stiff air-filled sacs or cysts may develop in the lungs. These cysts can rupture bringing air into the space surrounding the lungs. This pocket of air results in a collapsed lung. This can occur after even a minor blow to the chest. However, it can even occur spontaneously while a person is resting! A sudden shortness of breath and chest pain are both symptoms of a collapsed lung.

Sleep Apnea

Sleep-related breathing disorders such as snoring and sleep apnea are often associated with overweight individuals. However, many thin people diagnosed with Marfan Syndrome also suffer from these disorders. The problems are caused by the loose connective tissue in their lungs.

Sleep apnea is a breathing disorder very similar to snoring. The difference is that the person actually stops breathing for short periods of time while they are sleeping!

Emphysema

Individuals with Marfan Syndrome also experience a great risk of developing other lung-related diseases. Many people will contract emphysema. This is a progressive disease that destroys the lung tissues. These tissues are necessary to support the shape and function of the lungs.

 

Once the lung tissues around the smaller airways, called bronchioles, are destroyed, they can’t hold their shape properly when a person exhales. Less air is able to enter and exit the lungs which results in a severe shortness of breath. Individuals may find it difficult to breathe even when they are performing simple tasks such as getting dressed or preparing a meal.

Chronic Obstructive Pulmonary Disease

Marfan Syndrome can also lead to a serious lung disease called chronic obstructive pulmonary disease or COPD. This disease is similar to asthma and it gets worse over time. The stretchy fibers in a person’s lungs eventually become destroyed, making it very difficult for a person to breath. A person may also suffer a persistent cough.

 

 

COPD

COPD is one of the few major diseases that is on the rise, instead of the decline. This disease is currently the fourth leading cause of death in the US and more than 600 million people worldwide are diagnosed with it. Experts claim that it may be become the third most common cause of death in the world by the year 2020.