Pulmonary alveolar proteinosis (PAP) is an ultra-rare lung disease with approximately 3,600 diagnosed cases in the United States. Unfortunately, some people are potentially living with the disease without knowing it, as it is often misdiagnosed.
(StatePoint)
Pulmonary alveolar proteinosis (PAP) is an ultra-rare lung disease with approximately 3,600 diagnosed cases in the United States. Unfortunately, some people are potentially living with the disease without knowing it, as it is often misdiagnosed.
That is why the American Lung Association, with support from Savara Inc., is launching a new educational campaign to help healthcare providers and patients better recognize the signs and symptoms of autoimmune pulmonary alveolar proteinosis, (aPAP), the most common form of the disease. As part of the campaign, they are sharing these fast facts:
What is aPAP? This disease is characterized by the abnormal buildup of surfactant in the air sacs of the lungs, which can make breathing difficult. The buildup is due to an inability to clear the surfactant. Occurring in both males and females, aPAP is often diagnosed between the ages of 30 and 60.
What are its symptoms? Some people who are living with aPAP may not show symptoms initially, while others may have progressive shortness of breath. Additional symptoms include chronic cough, fatigue, unintentional weight loss and chest pain.
Why is aPAP commonly misdiagnosed? Since aPAP is so rare, and because symptoms are similar to other more common lung diseases, it is often misdiagnosed. Common misdiagnoses include both acute and chronic lung diseases such as pneumonia and asthma.
How is aPAP diagnosed? If you are diagnosed with another lung disease and the treatment is not effective, your doctor may recommend a chest CT scan. If you have an abnormal chest scan with unresolved lung symptoms, you should also talk to your healthcare provider about getting a free, simple blood test called aPAP ClearPath, which measures the level of the GM-CSF antibodies in your blood to determine if you have the disease.
How is aPAP treated? Currently, there is no cure for aPAP and no FDA-approved therapies; however, symptoms can be managed. The most common treatment is whole lung lavage, (WLL) also called “lung washing.” WLL washes out the built-up surfactant from the lungs, allowing you to breathe more easily. This treatment often needs to be repeated, as it doesn’t address the underlying cause of the disease.
Managing aPAP well means seeing a specialist who is familiar with this rare lung disease and going to all of your regularly scheduled healthcare appointments.
To learn more, visit Lung.org/PAP.
Without treatment, this progressive disease can increase the risk of infection and lead to respiratory failure that may become life threatening. Don’t wait. Talk to your doctor if your respiratory symptoms are not being managed with current treatments.
