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:

  • Bone Health
  • Bone density testing – when and how frequent
  • Bisphosphonates – when to intervene and with what (oral or IV)
  • Data collection / multiple sites
  • Delayed Growth – discussions around GH (growth hormone) benefits/risks
  • Delayed Puberty – use of testosterone to promote puberty
  • Insulin Metabolism – specific tests and use of Metformin

 

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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Comment by Rebecca Saulsbury on November 10, 2011 at 9:38pm

It is the bravery of moms like Pat and Patty, that keep me getting up everyday focused on LIFE and not death. Brady was loved and will continue to be loved. Thank you Patty for being dedicated to your son and helping to show us all how to do it with happiness and dignity, despite all the rest.

Comment by Sabrina on November 10, 2011 at 5:56pm

hugs your way loss for words

 

 

Comment by Mindy on November 10, 2011 at 5:27pm

Patty - you are an amazing person to posting on here to explain what happened. Brady could not have had better. I wish I could give you peace.

Comment by Chris cate on November 10, 2011 at 10:55am

Patty,  You and your family are in our prayers.  May God provide comfort and love to such a wonderful family.  Thank you God Bless!

Comment by Jo-Anne on November 10, 2011 at 5:14am

Pat, my son Kevin had a fall last week, they said he broke his L1 in his back, the next day we were told that there was no breaks or fractures. We see the neurologist next week, any specific questions I can ask? I read the above and am very sad to hear that Brady died after breaking his leg, this is tragic. There are a couple of questions I will put together because as you say, there are no answers yet, or parents just do not know any better. It is amazing how much you learn when a child is admitted to hospital.

Comment by Chris cate on November 9, 2011 at 7:10pm

Pat keep going and thank you for the update.   Our prayers are with all of the boys! 

Comment by Brian Denger on November 9, 2011 at 6:38pm

There are several studies that published data concerning short stature and DMD as early as 1988 (long before steroids were commonly used in DMD).  One study (http://www.springerlink.com/content/m8m17644t73873q4/) was presented at the annual meeting of the Swiss Paediatric Society 1985 in Zürich. It appears boys begin life at normal heights, yet the acceleration of growth diminishes over time.  The correlation Pat mentions makes sense.

There is no question the use of steroids exacerbates managing low bone density and the other noted side effects, yet one cannot place full responsibility for this aspect on that therapy. There is no easy answer in DMD.

There are times it seems when we learn something it only highlights how much more we need to know.

Comment by JUAN PEDRO ARBULU on November 9, 2011 at 3:54pm

Pat, same doubts as Ofelia, Juan (my son) is above average and fine with bones after 5 months of deflazacort.

Take care.

 

 

Comment by Ofelia Marin on November 9, 2011 at 2:47pm

Is this figure true for most boys? "Boys with Duchenne lose 30% of their height (estimated) because of the muscle weakness in Duchenne." What age does one start to see this? My son (age 4) was always above average height, after 7 months of steroids he is still 75th percentile. His bone density before starting steroids was also normal. So it will be hard for me to say that his bone dentsity will be lower due to Duchenne and not due to steroid treatment. When we see older man/boys who did not take steroids they seem to have "normal" height, they definitely do not look short as the boys on steroids. The list here seems to address the side effects of the steroids.

  • Bone Health
  • Bone density testing – when and how frequent,
  • Bisphosphonates – when to intervene and with what (oral or IV)
  • Data collection / multiple sites
  • Delayed Growth – discussions around GH (growth hormone) benefits/risks
  • Delayed Puberty – use of testosterone to promote puberty
  • Insulin Metabolism – specific tests and use of Metformin
Comment by David Stalling on November 9, 2011 at 2:33pm

Thank you for this informative update.

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