We got the results from Riley's recent stool sample test that we hoped would confirm that the PLE (protein-losing enteropathy) was gone. But it is not. The Alpha-1 Antitrypsin test found elevated levels of protein in Riley's stool. A normal result would produce a test results of less than 55. Riley's result was 160. That is the same number he had from the same test just days before he was taken to the ER, and then admitted to UCSF for his eventual Fontan-reversal.
Dr. Tarnoff said that he was disappointed that we haven't seen any improvements, but we shouldn't read too much into it at this point. He said that it may take many more months to see improvements. On the flip side, we may never see improvements. However, Riley is doing well from a clinical perspective, which is good news and he does not have any physical symptoms of PLE (the puffiness, poor profusion, constant upset stomach, diarrhea). Here are some highlights from my conversation with Dr. Tarnoff:
- Obviously, we were all hoping to see his numbers start to head back towards a normal range. It may take many months. It may never reach a normal range. He seems to have an "exceedingly mild" form of PLE, and we'll keep an eye on albumen levels and as long as there is no significant difference in albumen, and he continues to thrive from a clinical perspective, then we do nothing. You can have a disease and be borderline normal. Right now, Riley's PLE is so minor that it isn't having any affect on his organ systems.
- Since there isn't much known about PLE or why some kids get it, it's not clear what could set it off again. The last two times Riley got a cold (Sept. 06; March 07), we ended up at UCSF. That means it could happen again and the effusions could come back and the puffiness could come back. And with cold season just about to start, we are feeling nervous. If we start to see any puffiness, or if he starts to have the diarrhea and frequent upset stomach, then we need to get in touch with Tarnoff right away.
- As for what's next, he will have more tests to check his albumen, globulins, and Alpha-1 Antitrypsin again in six months. If albumen and globulins start to drop, then we could start what Dr. Tarnoff refers to as "voodoo medicine," none of which has much scientific evidence of doing anything.
This is all very discouraging. Yes, it's true that Riley has a good amount of energy and he seems to be feeling better with fewer upset stomachs and less diarrhea, but we were hoping that his Fontan-takedown would cure PLE. That is what we had heard up until after the surgery. Then it was revealed to us that sometimes PLE doesn't go away after the child is reverted to the Glenn circulation. So we are feeling very anxious and worried that this miserable disease might come back and take us back to the hospital. There is just so little known about it, that no one can really tell us what we want to hear: that Riley is going to be hospital-free for a long, long time.
3 comments:
Mr. and Mrs Norton,
My name is Mike Leithren, President of the Rebekah G. Leithren Foundation based in Newark, DE. We lost our daughter to the effects of PLE in 2005. Unfortunately the best we can do is hold PLE at bay. Not much is known about what causes PLE and many of the known treatments are as harmful as the disease itself on a longterm basis. Our foundation has several purposes, one is to be a storehouse of any information currently available on PLE on our website. We are also here to provide encouragement and support to families fighting this disease. Our main goal is to raise the funding necessary to research this disease, to identify causes, safer interim treatments, and ultimately a cure. Because this disease is considered to be rare, the medical community cannot obtain government grants for this research. They rely on private funding and right now there is not a lot out there dedicating funding for this disease. This is where we hope we can make a difference for children like Riley. One thing that did help our daughter to hold PLE at bay was supplementing her diet with a combination of soy and whey protein which together, forma a complex protein like meat but is more easily absorbed by the digestive system than meat protein. The brand we felt worked best was Herbalife. A protein shake made with their Formula 1 protein powder (soy) and Personal Protein Powder (whey) made a tasty treat and helped her maintain decent Protein levels in her blood. It can also be made into protein bars that travel better. Just offering this as a suggestion. Feel free to contact us at rglfoundation@verizon.net and check out our website at www.rglfoundation.org.
Suzanne
I think of you and Riley often and was glad to see an update but also dissapointed that the news you received from Tarnoff was not more uplifting. I am glad to hear though, that Riley has more energy and is enjoying life. I will keep good thoughts coming your way and hope for an uneventful and hospital free winter.
If you ever come this way for any heart events (I don't seem to be getting any information on them again) let me know and maybe we can connect.
Dina
What discouraging news! I am so sorry to hear that the PLE is not gone as we had all hoped. But it is good to hear that Riley is functioning well, feeling good and acting like a normal four-year old. We'll be thinking of you and praying for health this winter.
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