What is Therapy for Pulmonary Alveolar Proteinosis and Its Cost?

Pulmonary Alveolar Proteinosis Therapy in India – Costs and Treatment Options

Pulmonary Alveolar Proteinosis (PAP) is a rare disease characterised by the buildup of protein in the alveoli (air sacs) of the lungs, making breathing difficult. Although there is no definitive cure for the condition, several treatments are available to help manage its symptoms effectively.

What is Pulmonary Alveolar Proteinosis Therapy?

Therapy is needed for the more severe cases where the condition does not improve on its own. However, the exact treatment differs based on the exact type and severity of the condition of PAP. Here are a few of the more common therapies and treatments for PAP for affected individuals:

• Whole Lung Lavage (WLL) - In this procedure, a saline solution is administered repeatedly to a patient to remove excess surfactant from the lungs till they are clear.
• Bronchodilators - They are medicines that help relax the muscles present around the lungs to help a person breathe better.
• Lung Transplantation - People who have severe lung damage require a lung transplant to restore healthy function in the body.
• Oxygen Therapy- It helps a person breathe more easily by providing supplemental oxygen, which can be delivered either through a face mask or soft tubes placed in the nose.

What is the Cost of Pulmonary Alveolar Proteinosis Therapy?

How Common is the Condition of Pulmonary Alveolar Proteinosis?

Pulmonary Alveolar Proteinosis is a very rare condition with a global prevalence estimated at 7–10 cases per million people and in diverse clinical forms. Amongst all of the different clinical forms, Autoimmune PAP is the most common, affecting almost 90% of individuals who are affected with PAP.

How is Pulmonary Alveolar Proteinosis Diagnosed?

The healthcare provider first conducts a medical assessment of the lungs if they suspect that you have PAP. Then ask questions related to lifestyle conditions and family medical history. Here are a few tests that determine the condition of PAP in individuals:

• Blood tests to get the blood oxygen level.
• Pulmonary function testing assesses the condition of the lungs.
• Lung bioscopy that removes lung tissues for examination.
• Imaging tests like MRIs and CT scans.
• Serum biomarker called GM-CSF (granulocyte-macrophage colony-stimulating factor) to define the causes of PAP.

Is  Pulmonary Alveolar Proteinosis Hereditary?

PAP occurs primarily due to autoimmune factors. However, there is also a prevalence of hereditary PAP in children. Hereditary PAP happens due to mutations in the CSF2RA or CSF2RB genes.

What is the Prognosis of People with Pulmonary Alveolar Proteinosis After Treatment?

Patients undergoing whole, lung lavage generally have a 5 year survival rate of approximately 80%. Beyond that, there are fewer chances of a patient surviving the disease. However, if the patient has a mild case of PAP, there are more chances of being cured of the disease to an extent, with minor breathing problems later on in life.

If you have this condition, then it is important to seek prompt medical attention. A timely medical intervention can help prevent PAP from spiralling out of control and cause adverse effects.