Peliminary Results

Monday afternoon we received the peliminary results of James' Lung Biopsy. The doctors took time to sit down with us and explain what was found and what to expect. We did not really learn anything new from these results; as we expected it just confirmed the diagnosis.

The current diagnosis:
1. LPI PAP (Pulmonary Alveolar Proteinosis)
The macrophages in James lungs do not function properly. There job is to get rid of the surfactant in his lungs, and since they cannot do this the surfactant builds up and reduces his ability to breath. Although the fluid in James' lungs is not what they call Classic PAP, it is still PAP since it is a protein based fluid building up in the air sacs of his lungs. What makes it "unclassical" is the excessive lipids mixed in the fluid and hence the reason it is called LPI PAP as they feel these lipids are cause by his LPI
2. Lipoid Pneumonia
Although they mention this as a separate item, it is really one in the same as PAP. This has to do with the excessive lipids in his lungs, which is in line with LPI PAP. Other individuals can also have this type of pneumonia which is most common in aspiration cases (hence the reason we were always fighting the battle of whether he had aspiration), but in James’ case it’s part of his LPI PAP
3. Interstitial Lung Disease/ Pneumenitis
This is inflammation of the actual lung air sacs or lung tissue.

The one good thing they were able to report was that there is no fibrosis or scarring on the tissue they took. Scarring is irreversible, so as long as scarring is not present then if we are able to keep the PAP under control, the lung will be healthy.

What does this diagnosis mean? Really it doesn't tell us anything new and it doesn't help us to treat James. There still remains no treatment, and as long as that remains, James has a terminal illness and it's only a matter of time before his lungs give up.

I must say the doctors are more optimistic then we are as they gave James a prognosis of up to two years ... of course advising that it could be more or less. Given how James was doing before all this, we have a hard time seeing this as possible. We cherish each day, knowing that any day he can leave us for a better place. If the Lord should grant James another year or so we can still pray that through new research an answer may be found during that time.

One thing that may have made him go downhill so fast is the GM-CSF treatment. We really do not know if it did or not, but there is a possibility that is actually did more damage then good. In the labs they studied his macrophages being treated with GM-CSF and without GM-CSF. What they saw was that initially the GM-CSF did help the macrophages to function, but then they quickly pooled together and created a "clump" of lipids. The macrophages that did not receive the treatment did the same thing but at a much slower pace. So they stopped the GM-CSF treatment last week, thinking that it may have been the reason for his quick decrease in lung function as it was making the lipid levels in his lung increase. This is obviously unfortunate, but that is the risk of being a trial. This too is from the Lord.

As we have seen, the WLL's do not work on James and almost seem to have the opposite effect on him. Why? We do not know.

Those are the two main treatments for common PAP, but James does not have common PAP and these treatments are not working for him. This leaves us with no known treatment and nothing we can do to help our boy get better.

There is one new treatment the doctors have implemented in order to help the inflammation of his lung air sacs (Interstitial Lung Disease). This treatment is to give him high doses of a steroid called MethylPrednisolone (10 mg/kg in comparison to the 1 mg/kg he had when on prednisone) for 3 days. This is called a Pulse Dose. For three days they "sock" the inflammation in his lungs in an attempt to shut everything down and reduce what is already there. They started this new treatment last night with the hopes that this may bring him back to his baseline breathing and allow him off the ventilator. If this treatment seems to have some effect (and no major side effects) then they will consider doing this once a month to slow things down and keep the inflammation in check.

Regardless this is not a cure. They knew he had Interstitial Lung Disease but they also know that it is not his main problem. They just feel that this may just give him that little edge he needs to improve a bit and get him out of ICU. They have told us we should see pretty instant results from this Pulse Dose if it works, so the next few days will be the tell-tale.