If you have mild PAP, your doctor may choose a period of watchful waiting to observe if the disease improves spontaneously. You may receive oxygen supplementation to improve your shortness of breath.
If treatment is required, the main treatment for pulmonary alveolar proteinosis is washing of the lungs, also called whole lung lavage (WLL). In WLL sterile saline (salt water) is used to wash out the abnormal build-up of lung surfactant material from the lungs. WLL is carried out under general anaesthesia where doctors wash one lung at a time. The other lung is protected from the saline and ventilated with oxygen.
During the procedure, the doctor (anaesthetist) inserts a special double-barrelled breathing tube (called a double-lumen-endobronchial tube) through the mouth and into the windpipe so that the left and right lungs can be isolated from each other. The lung is washed until all the ‘milkyness’ of the lung surfactant is washed out and the fluid is crystal clear. In some cases this may take several hours and may be interrupted by lung massage in order for the surfactant to come loose.
Elisabeth a Danish PAP patient, who has had several whole lung lavages for the treatment of PAP, explains:
When you are lying on the bed, you are tilted up and down, while a physiotherapist beats your back black and blue. After that, you are sore both on the inside and on the outside, but you welcome the beating as it loosens the surfactant, which helps you breathe more easily, says Elisabeth.
After the lung massage the procedure is repeated. Any remaining saline is drained out of the lung and both lungs are ventilated with oxygen. In some cases, both lungs can be treated during the same procedure but often only one lung on the same day. Normally, you will be kept asleep and on controlled ventilation (breathing) for a further hour or so to allow for recovery from the procedure. Then the anaesthesia is stopped, you will wake up and the breathing tube is removed so you can breathe normally again but with the help of oxygen from a facemask.
At first, you may not be able to feel the effect of the treatment. This is probably because of the after effects of anaesthesia and the small amount of saline remaining in the alveoli. However, most people notice that they feel a lot better about 24 hours after the WLL.
Some patients are able to be discharged once they are out of anaesthesia, while others may have to stay for washing of the other lung.
After your treatment (washing of one or two lungs) your progress and improvements or any negative reactions will be assessed. In some cases further treatment with WLL will be needed but it is not possible to predict how many treatments a person will need.