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Rambam Medical Center in Haifa has spent NIS 50,000 to buy special expandable titanium nails to help a 10-year-old boy to stand - and they hope eventually walk - for the first time in his life.
A., a Galilee resident, suffers from an unidentified variation of osteogenesis imperfecta, a genetic disease in which bone is imperfectly made from the beginning of life. Victims have a faulty gene that tells their bodies to make either too little or poor quality Type-I collagen. The result is bones that break easily.
While there are tens of thousands of people in the world with osteogenesis imperfecta, A's unclassified type is apparently unique, with a variety of additional complications, said Dr. Marc Eidelman, the surgeon who performed the eight hour surgery last week. "I have treated five children with osteogenesis imperfecta deformations, but I have never seen a case like this," he added.
A's bones break easily, often from little or no apparent cause, and he has already suffered 78 fractures since birth. This makes his limbs look like rubber, as the bones beneath them are not solid.
"The only solution is to insert long titanium nails into his bones to hold them together," Eidelman told The Jerusalem Post. "Since A. is a growing child, ordinary nails would have to be changed every year. So the hospital ordered special telescoping nails for his thigh and calf bones, at a total of NIS 50,000, from a Montreal company."
Called Fassier Duval, the nails have 'male' and 'female' parts that fit one into the other and expand as the growth plates pull on them.
After the operation, A., with support, was able to stand up on his bed, and Eidelman hopes he will be able eventually to walk. If the boy shows marked improvement, Eidelman plans on an additional surgery to insert more in his arms.
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