Bone & Joint Care

The information on this page represents the recommended standard of care for Duchenne muscular dystrophy. Most of the care recommendations also apply to Becker muscular dystrophy, but at older ages. Most, but not all, people with Duchenne are males—but the care recommendations apply to both males and females with Duchenne.

If you don’t understand any of the medical terms and concepts, ask your healthcare providers. Take notes and ask questions during your clinical visits.

Bone & Joint Facts to Remember

  1. People with Duchenne often have weak bones, especially if they are taking steroids.
  2. It is important to have the right amount of calcium and vitamin D to make bones stronger. Ask your neuromuscular care team about the correct amount.
  3. Your doctor should watch your spine closely.
  4. If you have back pain, you should see your doctor.
  5. Although rare, fat embolism syndrome (FES) should be considered if shortness of breath or neurological symptoms develop after falls, fractures, or other trauma.

Bone Health

People who have Duchenne often have small and weak bones. Steroids (corticosteroids) also affect bone health. Steroids decrease the bone mineral density, causing them to become thinner and weaker. This is known as osteoporosis. There are a few topics to be aware of regarding bone health and Duchenne.

Importance of Calcium and Vitamin D

It is important that people with Duchenne have the correct amount of calcium and vitamin D to optimize their bone health and lessen the risk of fractures from happening. Ask your doctor and dietitian about foods rich in vitamin D and calcium, and whether supplements are needed.

Monitoring Bone Health

Take spinal X-rays

Make sure your doctor checks your spine during every visit for signs of a curve. Have spinal X-rays every year while you are still growing. Your doctor should watch your spine closely after you stop walking to look for scoliosis. If the curve in your back (scoliosis) is severe, you should have X-rays at least every six months. This way your doctor can keep close surveillance on your spine and decide if you need any interventions (i.e. surgery).

Take lateral spine X-rays

Lateral spine X-rays should also be done every 1-2 years after starting steroid to check for vertebral compression fractures that may not be causing symptoms. Lateral spine X-rays can also measure the width of your vertebrae (also known as your Ganat score) to further evaluate your bone health.

Your doctor should also do an X-ray if you have a problem or complaint, such as back pain, to check to make sure there are not vertebral compression fractures.

Have a dual energy X-ray absorptiometry (DEXA or DXA scan)

It is important to keep close surveillance on the status of your bone health. You may need DEXA scans annually after starting steroids. DEXA scans measure your bone density and can tell how high risk your child is of fracturing bones.

If DEXA scans are challenging for cost or logistical reasons, a lateral spine X-ray should be prioritized.

Have blood and urine tests

Blood and urine tests provide information about vitamin D and calcium levels and may help your doctor or dietician recommend doses of vitamin D and calcium supplements, if they are needed. Vitamin D levels (25-hydroxyvitamin D) should be checked annually.

Prevention of fractures

Do weight-bearing exercises and stretches

Weight-bearing exercises recommended by your doctor or physical therapist (such as walking and standing as tolerated; not to be confused with weight-lifting exercises) can help your bones stay strong for a longer period of time. Starting when you are young, your muscles and joints should be stretched 4 to 6 times a week. This can help delay and minimize your joints becoming locked in one position (contractures). Ask your doctor and physical therapist about helpful stretches and exercises to do.

Stay safe

It is important to stay safe and prevent falls. If your child is ambulatory, it is important to keep shoes on most of the time to avoid slips, keep paths in your house clear to avoid tripping, and watch for overexertion. Children with Duchenne may get tired more easily, and it is always a good idea to take a rest or use mobility devices (scooters, etc.) when needed. Keep in mild some falls cannot be prevented despite every effort you make.

If you or your child is not ambulatory, it is still important to take safety precautions. This includes providing safe transfers to chairs, beds, or in the shower. It is also important to always wear seatbelts in wheelchairs, shower chairs and in the car – if there is a seatbelt available on any device, use it!

As an extra precaution during the winter, it is advised ambulatory children, and caregivers who are assisting them, wear “boot grips” (sometimes called “Yaktrax) on the soles of the boots. They can help tremendously to avoid falls on the snow and ice.

Fractures due to osteoporosis

As bones become weak and thin (osteoporosis), there is more chance of broken bones (fractures). In Duchenne, the most common types of fractures are in the legs and spine.

Long bone fractures (i.e. femur)

This type of fracture can happen when people with Duchenne fall or experience trauma. However, most fractures generally happen after ambulation is lost and may occur with very little trauma (a bump, changing position, being lifted, etc.). Learn more about managing leg fractures.

Spinal compression fractures

Some of the bones in the spine (vertebrae) may begin to collapse because they are thin, putting more pressure on other vertebrae and causing them to break. This is called a compression fracture. Steroids may slow down the development of scoliosis, but may increase the risk of compression fractures.

Risk of Fat Embolism Syndrome (FES) following fall/fractures/trauma

Although rare, fat embolism syndrome (FES) develops quickly, and the consequences are serious and life threatening. FES should be considered if shortness of breath or neurological changes occur (confusion, disorientation, and “not acting like themselves”) after a fall or other trauma. Learn more about the symptoms of FES you need to look out for, and what to do if your child falls, is bumped or dropped, and develops FES symptoms.

Orthopedic Issues

The progressive weakening of bones and their surrounding muscles may lead to orthopedic problems such as scoliosis and contractures. Your physical therapist and rehabilitation team can work with you to prevent these from happening using personalized stretching regimens, custom orthotics, and proper positioning (especially in your wheelchair).

Recommended treatment

Fracture Management

If you are diagnosed with a fracture, it is best to see an orthopedic specialist who is familiar with Duchenne. If you have a leg fracture and were able to walk well before the fracture, ask the doctor if it is possible to have surgery (internal fixation) rather than casting (external fixation). Surgery may help preserve muscles so that you can start walking sooner compared to casting. Learn more about managing leg fractures.

Bisphosphonates Therapy

Bisphosphonates are medications given to treat osteoporosis. The current standard of care for treating osteoporosis in children is IV bisphosphonate therapy (pamidronate, zoledronic acid, or neridronate). There are forms of oral bisphosphonate medication, but data from studies in other pediatric diseases have shown them to not be as effective. The goal of bisphosphonate therapy is to increase the density of the bones of the body, including the vertebrae in the spine. Increasing the density of bones puts you at less risk for a fracture.

For adults, additional bone health therapies exist, including those that promote additional bone growth (teriparatide) and prevent the breakdown and resorption of bone (Denosumab).

Other Therapies

In addition to bisphosphonates, other therapies may be appropriate for adults. More information may be found on the medication and supplements page.

Meetings & Publications

Osteoporosis and Bone Health Meeting

In 2016, PPMD convened a meeting of global experts in Duchenne osteoporosis and bone health. A published summary of that meeting can be found here.

Bone Health Supplement Article

A series of supplement articles were released in October 2018 to discuss areas of care in response to the 2018 DMD Care Considerations. “Bone Health and Osteoporosis Management of the Patient With Duchenne Muscular Dystrophy” was written by several specialists to outline the importance of bone health in people living with Duchenne. The published Pediatrics article can be found here.