Pulmonary Alveolar Proteinosis: Treatment, Cost and Side Effects

Pulmonary alveolar proteinosis, is a lung disease which is usually caused among adolescents that requires treatment if the condition is critical or serious. Pulmonary alveolar proteinsis can be cured but this may lead to death for twenty percent of the people suffering from this which is usually due to respiratory or breathing problems. The other eighty percent of the people can manage this with a treatment. Such people can lead a normal life after taking the treatment. These people might still suffer from breathing problems and other respiratory related problems even after taking the treatment. This, pulmonary alveolar proteinsis can cause a few lung problems. The treatment for this problem is started only after a few tests. These tests involve a chest scan and CT scan of the lungs, the air capacity of lungs is checked and also blood tests related to Pulmonary alveolar proteinsis is taken before the treatment process begins. Even if these tests are found positive, this is still curable most of the times. There are a very few cases which have caused death either due to excessive respiratory problems or lack of oxygen in the blood. There also a few cases where it may reduce the lung capacity and also cause infections in the lungs.

The treatment is done only when the case is serious or critical. The Therapy involves Whole Lung Lavage(WLL). It is currently the standard therapy for PAP. This therapy Is done In case a patient is asleep. Under this, the surfactant present is washed in and washed out using saltwater and The Other lung is engaged in normal breathing process through the supply of oxygen through the machine. The normal treatment for this is a process of whole lung lavage. Under this, the lung is filled with a sterile Fluid and later the fluid is removed along with the abnormal surfactant material. This generally improves the PAP symptoms for a longer period of time. Often GM-CSF injections are used to cure PAP and often these have brought Success for a longer period of time. In severe cases, lung transplantation can also be a better option. If the person is suffering from mild or minimal pulmonary alveolar proteinsis, then, treatment can be avoided and a basic oxygen therapy can be done to ensure that the person gets cured. If the case is severe, the surface area of the lungs is washed by the doctors with saline or salt solution. There are two stages, primary and secondary levels of treatment. In the process, they will fill the lung area with saline solution and then it is drained out. Sometimes, only one part of the lung might have been affected. In such situations, only the affected area is cleaned with the solution. Or else, the whole lung has to be cleaned with the saline solution. In a few cases, a single wash is sufficient to clear the lungs. Else, they will be suggested to either go for blood stimulating medication or lung transplant based on the intensity of the problem. While one of the lung is being cleaned with the saline solution, the other lung is made sure to be kept ventilated. After one side is completed, the same process is repeated for the other lung. There are different stages of treatment depending on the severity of the problem they are suffering from.

The treatment is done only after it is found the tests like chest scan, CT scan, and other related scanning is done after which, if the results are found positive, then, the treatment process begins. They ensure to check that the blood pressure levels are stable before beginning the treatment. Treatment is recommended only when it is found severe. Else, it can be cured by oxygen therapy. The eligibility for the treatment for the disease can be best diagnosed by the specialist doctor. It is therefore advised that the patient consults the doctor before undergoing any specific treatment. Doctors on examining the conditions shall decide the eligibility for the treatment.

Patients who are not having any symptoms related to the diseases or the conditions needed for the treatment are therefore not eligible for the disease. But the best advice can be given by the specialist doctor and therefore it is advised that the patient consults the doctor before the treatment.

Usually, there are no side effects caused by this treatment for pulmonary alveolar proteinsis. Potential side effects of GM-CSF are normal reactions, fever, myalgia, headache, and flu-like reactions. Larger side effects include anaphylaxis, cardiac failure, and leaky capillary syndrome. More disadvantages are the need for a daily subcutaneous injection and the higher cost of GM-CSF. But, in a few cases it was observed that, even after treating the patient suffering from this lung disease, they had a few respiratory or breathing issues. They are much easily prone to pneumonia even after the patient is treated for the disease is assumed to have been cured permanently. They have also observed that breathing become difficult when they go to regions with high altitude which has to be taken care of even if they lead a normal life after the treatment.

There was no change in the period of actual survival after the treatment. Although the overall mortality of the older people was lower. There was not much difference between the mortality of the two age groups related to infection-related deaths. People were advised to stay indoors as their lungs were recently operated and hence required more diligence with the environment around.

In some situations, when the person is suffering from mild pulmonary alveolar proteinsis and the patient is undergoing oxygen therapy, then it might take a few sitting which might vary from 2 to 5 times. If the person is suffering from severe lung disorder (severe pulmonary alveolar proteinsis) then, it is recommended to undergo lung cleaning with saline solution. This is mostly cleaned in a single attempt (each lung at a time if it is the whole lung or even it is only a part of the lung that is affected).

The basic consultation charges may vary from rupees 700 to rupees 1,500. The tests related to this might cost up to rupees 15,000 on an average. Depending on the severity of the disease the treatment charges vary. They are different depending on the age and the intensity of the problem if it is mild or requires primary treatment or requires secondary treatment.

Yes, in most of the cases the results are permanent. But, if the case is severe, the person has to undergo the treatment for a few years. The life expectancy for such a case is a maximum of 5 years. Beyond that there are very few chances of the person to survive the disease. If the case is mild, the results are permanent but, they may have a few breathing problems later in their life but will definitely not cause too much harm or will not lead to death. Primary cases that are cured with cleaning are mostly permanent but they may require more than one attempt of cleaning with saline solution.

The alternatives for the treatment depend on the various kinds of cases. In case of a person suffering from mild pulmonary alveolar proteinsis, oxygen therapy is the way of curing the disease for them. Recently, methods such as inhaled granulocyte-macrophage colony stimulating factor (GM-CSF) are being used instead of the traditional method or the golden standard of whole lung lavage (WLL).