Is this aHUS?

[Kamal D Shah]

I recently met a 3 year old boy who is suspected to have aHUS. The boy was asymptomatic until the beginning of February. He started passing cola colored urine at this time. Investigations revealed that his hemoglobin was 4.4%, platelets were 110,000. LDH was 11,000 and creatinine was around 4.5.

He has been receiving plasma infusions - 150 ml every day pretty much. He has also received packed cells. He has been put on peritoneal dialysis. They cannot do a biopsy apparently because his platelets are low.

He has been given steroids starting yesterday and his hemoglobin has improved. So has his LDH. The docs here are thinking it could be hemolytic anemia and are hoping to treat it with steroids. My question is if it is only hemolytic anemia and not HUS then why has his renal function declined? Also, if it is HUS then why is he responding to steroids?

I am no doctor and am only desperately trying to help this little boy. I am not related to him. He is the first HUS patient I have met in person and so have developed a bond with him. I want to really try to help him not get into chronic kidney failure like I did.

Any information / suggestions would be most welcome!

Thanks

Kamal

[Svetlana Finley]

Hi, Kamal
I am not a doctor, but even my daughters situation, she is diagnosed with AHUS, but never had dialysis. When she first got sick she was very anemic and they give her lots of blood transfusions, sometimes was several a day. It did affected her kidney and her urine was brown, but creatinine is not high enough for her to have dialysis. She had high protein in her urine too and she was on steroids for 6 month to help her with treatments.

[Linda Burke]

Hi Kamal,
There are three resources open to you via this website. A great chart comparing HUS and aHUS (along with supporting medical info) awaits you at www.atypicalhus.50megs.com , the website created by the Biermann family and the official information website of The Foundation for Children with Atypical HUS. There is a Links Box on our Home Page will will take you there directly, allowing you to immediately utilize this detailed medical information.
A diagnosis of atypical HUS is difficult even for the most seasoned physicians, due to the wide variety of symptoms and the complexities involved in aHUS cases/treatment. You could provide the child's doctor with the email addresses of two physicans willing to share their experiences treating aHUS children : Dr. Stuart Goldstein,MD can be reached at '[e-mail]' and Dr. Christoph Licht, MD, FASN at '[e-mail]' .
Unfortunately, aHUS issues tend to be case specific to the particular patient and therefore details of cases are best discussed physician to physician. The '50 megs site' noted above will inform you that with regular HUS, renal function also declines - and hopefully www.atypicalhus.50megs.com will provide you with much of the additional medical information you need.
My heart goes out to the little boy struggling through this, and I can appreciate your desire to help him. Since we are only parents here, we can't give medical advice to assist you......I'm hoping that you can use the info above to help him. With best wishes, Linda Burke

[Cheryl Biermann]

As usual, Linda has given a great place to begin your journey with this little boy. I think another route you can take is to talk with parents of children who have been treated with steroids.The doctors talking to each other is not going to give you any assurances unless his parent's have an understanding of all of this new information being heaped on them all at once, and dealing with the worry, and are willing to let you know what the experts are saying. I would send a message to Jodi Kayler, as her son Coen has been treated for several years with steroids, this way while the doctors are connecting, you can compare stories. Keep us up to date, but it is nice to know you have a little buddy who can benefit from your experiences and concern! So sorry he has to go through this but it is nice to know he's not alone.