The death of Brady Sherman (14 years old) is heartbreaking and strikes fear in the hearts and minds of every parent of every boy with Duchenne. It is so frightening that the idea of wrapping your son in bubble wrap to protect him or never letting him out of your sight, makes perfect sense.
Today we do not have any concrete answers about Brady. We have some ideas about what happened and have been connecting with experts (pulmonary, cardiology, neurology, orthopedics) in an effort to understand what might have happened and what we, as parents and family, need to do.
In Duchenne, bone density is below normal. Often initial tests for bone density show a negative 1 or 2 standard deviation below normal. The reason this occurs is that muscle activity is required for bone mineralization. Muscles and activity ‘bang’ the bones around as the child grows, stimulating bone turnover and bone growth. Boys with Duchenne lose 30% of their height (estimated) because of the muscle weakness in Duchenne.
Because the bones are not normal, they fracture. The femur, because it is a very long bone, is at considerable risk. Parents talk about a slight fall or tripping and recall hearing the bone snap. I can imagine it is a sound you will never forget. Other bones fracture as well – tibula/fibula (lower leg) and sometimes, though not as frequently, the bones of the arm.
I have discussed this with a number of experts and all thought Brady may have died as a result of a fatty embolism. This has nothing to do with weight, rather is released from the bone after fracture. There was no way to predict it and no way to treat the fatty embolism. There are just more questions.
We know that the low bone density creates a greater risk for fracture. Consensus on testing and intervention is essential. We need to know if the risk for embolism is predictable. We need to understand if there are specific tests that would have the potential to identify if the risk is higher for some boys.
PPMD is sponsoring an Endocrine meeting December 1 and 2. The purpose of the meeting is to form a consensus around issues related to chronic steroid use and:
We have lost too many boys this year. One is too many. This loss makes all of us weep for the one we lost and for our own son, as the risk and loss is so close to home. As a community we need answers. Please know that PPMD will bring the right people, the experts together, and will do what we can to get us all the information we need.
Pat Furlong, Founding President, CEO
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