It has been a busy month for the CRIBBS project and I want to update you on our activities. First, I need to let you know that the grant application to the Patient-Centered Outcomes Research Institute examining weight loss strategies in BBS was not funded. The major criticism by the reviewers was that we have not conducted pilot studies using the weight loss strategies in individuals with BBS. The reviewers were RIGHT! The strategies have been used in many other people but have not been rigorously studied in BBS. So we will be announcing at the June Conference two pilot studies on weight loss in BBS. Stay tuned for some great opportunities. As always we are working with the BBSFA to insure that medically safe and thoughtfully designed therapies are developed. It is often tantalizing to try the "latest and greatest fad" but unfortunately sometimes the fads are ineffective (e.g. Grapefruit diet) or more concerning may be dangerous to a person with BBS (e.g. Adkins diet). I look forward to sharing our efforts in June.
Recently there was a lot of interest expressed on the topic of bone health in BBS. I want to share with you some findings from CRIBBS.
Polydactyly has been identified in 189 of 228 (83%) CRIBBS participants
Blount disease has been diagnosed in 2 of 228 of participants. Both had surgical care.
Club foot has reported in 4 of 228 participants. Three of 4 had surgical care.
Scoliosis was reported in 37 of 228 (16%) of participants. Only 5 were treated with surgery.
Leg length discrepancy (one leg shorter than the other) has been identified in 22 of 228 (9.6%) of participants.
Joint laxity (excessively flexible or mobile joints) is present in 63 of 228 (27.6%)
Back pain is reported by 75 of 228 (32.9%)
Difficulties with balance were reported by 168 of 228 (73%)
Falling more often than others was reported by 101 of 228 (44.2%)
But despite these challenges 116 individuals participate in recreational or competitive sports ranging from bowling to horseback riding to dancing to wrestling to field hockey to swimming. That is amazing. I remain in total awe of your efforts to be active even with the bones and joints don’t always cooperate!
As always thank you for your support of the CRIBBS project. If you are not a member of the registry and would like to join please contact us at 877-594-3499 or look at our website at www.bbs-registry.org or email me at firstname.lastname@example.org.
We need your participation and financial support!
Dr Bob Haws
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