"What is heel cord lengthening?"
"What are AFO's?"
"How do I know if I am correctly doing stretches with my child?"
"What should I know about scoliosis surgery?"
"My child hates "night boots" – are they really that important?"
These and so many other questions are asked regularly around orthopedic complications in Duchenne. To address the issues of contractures, scoliosis, and fractures, PPMD elicited the assistance of three experts for a webinar discussion on Tuesday, March 31, 2015.
Q: Is the excess fat due to muscle break down?
A: Extra fat may be an effect of the steroids with greater food intake because the medication makes you feel hungry. The large calves that are seen are not actually large muscles. When the muscles break down they are replaced by fat and connective tissue. Connective tissue is also called fibrous tissue or you could think of it as scar tissue.
Q: Do bisphosphonates really strengthen the bone or just make the bone look more solid on a dexascan? There are many DMD boys on these, my son included, who still fractured their femur just by standing up or a quick twist. How long should DMD men use bisphosphonates?
A: In boys with DMD the literature is pretty sparse. There are a couple of studies that show stabilization of the bone density scores. This is actually a good thing because we would expect over time that the scores would get worse with more weakness. There is no study that I know of that shows that fractures stop. This is one of the reasons it’s controversial. In post-menopausal women the fractures do decrease.
Q: Can walking on toes all the time cause scoliosis?
A: No. It is very unusual for boys who are walking to have a scoliosis. The walking pattern (on the toes) is related to the muscle weakness pattern which all boys have. Boys on steroids are much less likely to develop a scoliosis.
Q: At what age should we start using the night splints?
A: I typically think the earlier the better. Compliance is typically better when started early. Some clinics start with any signs of tightness. If you wait until the ankle cannot come to neutral it is much harder to get a good stretch.
Q: Do you recommend proactive use of night splints for young, ambulatory boys or do you recommend that use begin once "needed"?
A: Please see answer above.
Q: Our son has recently started using the toe off AFOs after sustaining a fracture tibia/fibula fracture. An Orthotist came to observe our son during a PT session and noted his knee hyperextends which put him at an increase risk for falls. So, now, after reviewing this information, I am concerned about him using this device. He is currently walking with a walker. The toe-off AFOs are very lightweight. ANy suggestions as to what direction we should go from here?
A: I can understand why this would be recommended after a fracture especially since this brace has an anterior (front) support. I think this will take a group approach with your PT, rehab physician or orthopedist, if available, and possibly orthotist to decide when/if it is appropriate to wean the use of this brace if it is possible. This is one of the cases where he will need to be monitored frequently to make the best choices.
Q: How important are AFOs when the boy is non-ambulatory. My son is 14 and does have AFOs which he refuses to wear. He is claustrophobic.
A: This is not unusual, I have also had the same experience with many boys that age. If they can’t tolerate them all day, I recommend using them when they get home from school or at night to help. Some therapists have suggested that showing they boys pictures of foot/ankle contractures can be helpful. Not being able to wear shoes or the shoes they prefer can also be something to discuss. Please know that sometimes, even with the very best efforts and consistent stretching and use of splints, contractures still develop so don’t make him feel too guilty either. Make sure that they are comfortable, perhaps they don’t fit correctly.
Q: SMO's w full amb child- should continue if adds stability?
A: These are typically well tolerated and do not restrict the ability to move the ankle and do not interfere with transitions off the floor or sit to stand. They should be evaluated for fit frequently by therapist and physicians and to make sure they are still appropriate as your son’s range of motion changes as well.
Q: Is it common for stepping foot/ankle to over-use extensors- my son is stiiffer on plantar flexion?
A: Tightness in the ankles (heel cords/gastrocs) and into plantar flexion is the first and most common limitations early and on and persists throughout. Toe-walking is often one of the first things noticed in young boys and may lead to a diagnosis of DMD.
Q: Can I get the name and the article again for the guidelines for serial casting. Thanks
A: See the "References" section above.
Q: Any advice on how I can force my muscle clinic to give us a DEXA scan?
A: Unfortunately, there is not a consensus amongst the experts, regarding when to do DEXAs or how to use the DEXA data. Many providers do not treat with bisphosphonates based on a DEXA score, but treat with a history of fracture or pain, so see no value in a DEXA. Also, as we mentioned on the webinar, even though the DEXA score goes up after treatment with bisphosphonates, it is unclear if that higher number indicates that the bone is now stronger, or just denser and, if it is just denser, is it at decreased risk of fracture? There is no evidence regarding how long to treat, if bisphosphonates are started. There are many unknowns. This is one area of care ripe for discussion, and we plan to start those discussions this year.
Q: Leslie Becknell Marx: how do i find out if my clinic at Portland OR Shriners is PPMD certified?
A: The PPMD website, under the Care for Duchenne tab, hosts a page for the Certified Duchenne Care Center Program. Certified centers can be found on that page.
Q: Is there any studing programs focusing n treatment patients with DMD?
A: There are many programs investigating potential treatments for Duchenne. Those programs/companies are listed on the PPMD website, under “research.” current trials can also be found on ClinicalTrials.gov.
Q: Dr McCarthy! Is there any conditions when you can't do operative treatment of contracture? Thank you!
A: Yes the decision to perform surgery is highly dependent on the family and patients input and goals. They must be medically healthy enough to undergo surgery and willing to engage in physical therapy after surgery. I would be very careful to avoid surgery in boys who are marginally ambulatory, because even the smallest set back may stop them from walking.
Q: Once their feet are turned in what can we do to straighten it? I was told nothing can be done and my son never was braced.
A: Surgical correction is usually possible, but may or may not be indicated depending on the individual situation. In more severe cases the interventions are much more involved.
Q: Would heal cord surgery have to be repeated to maintain the position?
A: That is possible but we work hard, once we achieve satisfactory foot position to maintain it, but if no therapy or bracing is employed, then the deformity will likely recur.