Levi’s apt with the rheumatologist was yesterday here at Children’s Hospital Los Angeles. Even though we recently went to see the 2 doc team in San Diego, I wanted to keep this apt (made 3 months ago) to make sure Levi doesn’t have any issues with arthritis or things with his joints. We are nearing the end of our Physical Therapy services and, though he is all caught up, the PT and I were still concerned about his lack of energy, enthusiasm, and his often downright refusal to walk. He is active at times, when it’s on his time and pace, but even then, not a lot of activity.
The apt started the way they always do- I spend 20 mins reciting Levi’s entire life history, health, services, behaviors, and my pregnancy. I also showed them the documents from other doctors and fever calendar. The rheumatologist, Dr. Reiff, comes in and we talk all about PFAPA for a while (my long post about PFAPA is HERE). I tell him about my concerns with his complaints of joint pain, and after he examines him and we talk, he says there is nothing going on with Levi’s joints other than the inflammation. What a relief!!! He did say some other interesting things:
The Doc said he is not surprised to hear that Levi has struggles with developmental delay. With Periodic Fever Syndromes, the child’s body is in a constant state of inflammation. He explained that all of our bodies have a protein called Amyloid, in small amounts, and our bodies dispose of it easily. But in all Periodic Fever Syndromes, this protein is being constantly made because of the constant inflammation and it’s stored in areas like the kidneys. When the fever syndrome is untreated, the “bucket is full and overflowing with Amyloid” is the analogy he used. Treating the fevers with Prednisone gives the body a chance to dump the Amyloid.
What does Amyloid have to do with developmental delay? Is causes fatigue and inactivity! This issue is highest in the hereditary forms of PfAPA like FMF. Those children, he said, look kinda sickly in general and are on the bottom of growth charts. The good news is that classic PFAPA has the least issue with Amyloid. The rheumatologist mentioned that as it’s treated with prednisone more and more, they notice the children improving in tone, energy, and activity as well as weight gain. He said it’s not solely PFAPA’s fault for Levi’s developmental delay, but that I should start to see more improvement along with PT since using the prednisone.
Lastly, he mentioned that the mouth ulcers are still to come. They don’t show this young, but he was pretty certain they will come but that the prednisone should help them some. He also made it clear that he was against the tonsillectomy. In all of their cases of various PFAPAs, they have NO patients who got the tonsils removed and was cured of PFAPA. He said he cannot understand how the current studies are getting 80% success rate. But, I will say, that this hospital is located in Little Armenia and so he sees many FMF cases which cannot be cured or outgrown, ever. I don’t know what to think yet. But we have decided Levi is too young right now and hopefully there will be more info about it in a few years.
Obviously, seeing this rheumatologist is closer, but both doc teams have a lot of info to offer. And seeing the San Diego docs allows Levi to be part of the research. So we might see both, alternating visits. We only have to go every 6 months. All we can do now is maintenance.
Thanks for following along, and I hope it gives info to other parents seeking answers!
Leasa