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Bardet Biedl Syndrome Foundation

Connecting families and sharing information on research, treatment, and therapies for Bardet Biedl Syndrome.

Dr. Haws Presents on Kidneys in BBS

Dr. Haws provided a terrific overview of how BBS affects the kidneys and what to look out for.

Here are some specific important notes:

1) BBS patients do not show classic symptoms of chronic kidney disease. You must monitor kidney function through metabolic tests at least yearly

2) BBS patients are never “in the clear”. Kidney disease can appear at any time.

3) Many doctors react to the extra amount of urine BBS kids produce by suggesting that parents restrict fluids. THIS IS ABSOLUTELY THE WRONG THING TO DO. IT IS DANGEROUS.

4) Other doctors prescribe DDAVP to help concentrate urine. Again, THIS IS DANGEROUS for BBS kids

5) Every BBS patient should have a full imaging of their urinary system. It is very likely that there are other abnormalities in the urinary tract or bladder in addition to the kidneys.

6) However, kidney imaging is not conclusive. The only way to really tell what is happening is metabolic testing.

7) A large study of children with kidney disease showed there are very positive effects from targeting 50th percentile for blood pressure (rather than just “normal range”)

8) Preferred medications for high blood pressure are ACE inhibitors or ARBs. Avoid Beta blockers, thiazides or calcium channel blockers.

9) Other common sense ways to preserve kidney function: Avoid NSAIDS (like ibuprofen, naproxen), avoid dehydration, treat UTI aggressively.