Loss of Ambulation Phase (about ages 10-14)
- In the “tween” and teen years there is a continuous
progression of muscle weakness. By about 12 years of age, most people with
Duchenne are unable to walk and need to use a power wheelchair on a regular
basis. Children usually need help or
mechanical support with activities involving the legs or trunk. Fatigue (feeling tired) is quite common.
- Because they have weak back muscles and are seated much of
the day, scoliosis (too much curving of the spine) may begin to develop.
Scoliosis, as well as muscle cramps, may cause physical discomfort at
times. Be sure to discuss issues of
physical discomfort/pain with your child’s doctors and therapists.
- Weakness in the arms can make activities of daily living more difficult. Most teens, however, are able to use of their arms and fingers through this phase, so they can generally still write and use a computer.
Important information for all stages
Transition to Adult Care
Many children begin their transition from pediatric to adult care during the Loss of Ambulation stage. This transition should be a planned out, multi-year process. You can help your child by encouraging him to play an active role in his own care, and even leaving the room for a few minutes at each visit so he can speak to his providers on his own.
Glucocorticoids (“steroids”) are the only medications approved to treat Duchenne. They may act to slow down the progression of muscle deterioration. Many experts recommend that children who were on steroids continue to take them when they stop walking. Sometimes the doctor will change the dose of steroids after a child stops walking. Steroids help keep the muscles of the upper body stronger, slow down scoliosis, and help with breathing and heart function.
Taking steroids often makes puberty happen late, which may become a concern in children at this stage. Taking steroids may cause problems with gastroesophageal reflux (GERD or heartburn). If your child has stomach pain or burning, talk to your neuromuscular provider about medications that can help. A child who is taking steroids may be more likely to get sick. Make sure your child gets a ﬂu and pneumonia shot.
Ask your physical therapists and doctors which exercises and activities your child should and should not do. Many individuals at this stage benefit from gentle swimming and floating in a pool.
Continue to stretch your child’s muscles and joints at least 4 to 6 times a week. Contractures (joints becoming locked in one position) are a common problem at this stage. It is important to try to minimize tightness in the shoulders, elbows, wrists and fingers. Ask your doctor and physical therapist about helpful stretches, ways to keep your son standing as long as possible, and about using orthoses.
Regular heart screening is very important. Heart problems may begin during this phase, even though no there are no symptoms. Heart tests (EKG, echocardiogram and/or cardiac MRI) may begin to detect heart changes that can be treated with medication. Heart screening should happen at least once a year. The cardiologist may change this schedule based on heart test results.
As he gets older, there are two common problems that may develop with your son’s heart.
- Your son’s heart muscle may become weak and not pump blood properly.
- Your son may have problems with heart rate or rhythm.
Experts don’t yet know the best time to start heart medications. Some start heart medications before any heart problems are seen, while some wait until they see a heart problem on an echocardiogram or cardiac MRI. Your child should start heart medications if any heart problem is seen, even if your child has no symptoms.
Duchenne affects most muscles, including muscles important for breathing. Your child’s muscles that are important for breathing will become weaker as he gets older. The diaphragm is a muscle under the lungs that helps with breathing and taking deep breaths. Some people with Duchenne develop diaphragm weakness at this stage and have difficulty taking deep breaths. After he stops walking, he may have trouble coughing.
Fatigue, morning headaches, and waking up frequently during the night may be a result of breathing problems. Pulmonary function tests, including a “forced vital capacity” test and “a peak cough flow” test, should be done at this stage at least once a year.
If your child has any symptoms of decreased oxygen during sleep, a “sleep study” test will look at oxygen levels during sleep. Everyone has more shallow breathing during sleep, but people with Duchenne may have even more shallow breathing, resulting in decreased oxygen during sleep (“nocturnal hypoventilation”). If the sleep study shows that there is decreased oxygen during sleep, using a BiPap machine can help breathing and increase oxygen during sleep.
A weakened diaphragm can also result in a weak cough, which decreases the child’s ability to clear secretions out of his lungs and may result in more lung infections, and even pneumonias. A “cough peak flow,” which is part of a pulmonary function test, will show if the child’s cough is weak. If his cough is weak, a “cough assist” machine can be used to help to clear the lungs. If your child is taking steroids, he may be more likely to get sick.
Keep a copy of your child’s latest breathing tests to show other healthcare providers. Make sure your child gets all of the recommended childhood shots. If your child gets sick, especially with a cold or chest infection, make sure he receives antibiotics, extra breathing tests, and extra help coughing. Your child’s lung function should always be tested before surgery.
Being able to get from place to place is important for everyone. Mobility comes in many forms—strollers, walking (unassisted or with braces), electric scooters, manual or electric wheelchairs, and more.
As the person with Duchenne starts to have more problems moving around, consider making changes in your home that allow the person with Duchenne to move around the house as independently as possible. Special equipment or additions, such as wider doorways and ramps, can make life easier once the person with Duchenne is using a wheelchair.
For many parents and caregivers, it is painful to accept that a child needs help getting around, but it is better for the child to have mobility using help from braces, scooters, or wheelchairs—and the independence it gives the child—than not to be able to move as freely as possible. Parents and caregivers might find that children, teens, and young adults willingly accept devices and technologies that allow them to get around by themselves.
Children may have more trouble with headaches, mental lapses, or difficulty concentrating or trouble staying awake during the day due to breathing difficulties. These may affect his ability at school. Steroid use may cause your child to have more trouble controlling his emotions. Children at this age may have weak problem solving skills and may not be aware of the effect of his behaviors on other people.
At this stage, your child needs a lot more help than many of his peers. Help your child feel comfortable with having other people care for him. Your child needs to know that his parents are not the only ones who can keep him safe and happy. Work with your child and other caregivers to have open, honest communication about your child’s needs and preferences related to his care.
Mental Health Care
Adolescence may be a difficult time for any child. The physical limitations with Duchenne, as well as delayed physical development and delayed puberty that come with taking steroids, may make adolescence more problematic for your child. Teens at this stage may want to be more independent but feel frustrated because they require more care and assistance from others, such as parents. As muscle weakness progresses, people with Duchenne are at risk for becoming more isolated or socially withdrawn. Parents and teachers should look for signs of chronic sadness, depression, or anxiety. People with Duchenne and their families should take care to remain involved in activities and friendships outside of the home and family. Family and individual counseling may be helpful at this stage and should be considered.
As your child’s muscles weaken, he may have difficulty moving or turning over at night, and he’ll need help to sleep comfortably. People on steroids may also have difficulty sleeping. Physical therapists can recommend certain beds or mattresses to improve comfort and decrease the need for help during sleep times.