Early Non-Ambulatory (Adolescence/Young Adults)
Teens living with Duchenne will need to navigate all of the hurdles of normal adolescence, as well as the challenges of Duchenne. During adolescence, you will need to start to become more independent and begin making decisions around your future adult life.
Important Information for All Stages
If you are taking steroids, it is imperative they are not stopped suddenly for any reason. This puts you at risk for an adrenal crisis, which is a life-threatening condition.
- 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. You will need help or mechanical support with activities involving the legs or trunk. Fatigue (feeling tired) is quite common.
- Because you 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 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 you may be able to write, eat somewhat independently, use a computer, and take care of your own daily needs (like brushing their hair and teeth).
Transition to Adult Care
Many children/teens begin their transition from pediatric to adult care during this stage. This transition should be a planned out, multi-year process. Tell your parents you want to play an active role in your own care, and even ask your parents to leave the room for a few minutes at each visit so you can speak to your healthcare providers on your own.
Steroids (corticosteroids) are the only medications approved to treat Duchenne. Many experts recommend that people who are on steroids continue to take them when they stop walking. Sometimes your doctor will change the dose of steroids after you stop walking. Continuing steroids can help keep the muscles of the upper body stronger, slow down scoliosis, and help with heart and breathing function.
Taking steroids may cause many side effects. Side effects should be monitored and addressed at each visit with your neuromuscular provider. Taking steroids often makes puberty happen late, which may become a concern in children/teens at this stage. If you are taking steroids, you may be more likely to get sick. Make sure you get a ﬂu and pneumonia shot to avoid these common illnesses.
Muscle & Joint Care
Talk to your physical therapist or neuromuscular specialist about exercise or activities that are safe in this stage. They will typically suggest non weight-bearing activities such as gentle swimming.
Include stretching of your muscles and joints in your daily routine. 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 neuromuscular specialist and physical therapist about helpful stretches, ways to keep you standing as long as possible, and about using orthoses.
Mobility & Environmental Accessibility
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 you start to have more problems moving around, discuss making changes in your home that allow you to move around the house as independently as possible. Special adaptive equipment or additions, such as wider doorways and ramps, can make life easier once you are using a wheelchair.
As muscles weaken, you may have difficulty moving or turning over at night, and may require assistance to sleep comfortably. Physical therapists can recommend certain beds or mattresses to improve comfort and decrease the need for help during sleep times.
For many parents and caregivers, it is painful to accept that their child needs help getting around, but it is better for you to have mobility using help from braces, scooters, or wheelchairs—and the independence it gives you—than not to be able to move as freely as possible. Your parents or caregiver might find that you willingly accept devices and technologies that allow you to get around by yourself.
Regular heart screening is very important. Heart problems may begin during this phase, even if there are no outward symptoms. Heart tests (EKG, echocardiogram and/or cardiac MRI) may begin to detect heart changes. Heart medications, such as ACE-inhibitors should be started at the first sign of cardiac fibrosis or dysfunction, or preferably by age 10.
You should continue to have yearly visits to their cardiologist. The cardiologist may change this schedule based on heart test results and require exams more frequently.
There are two common problems that may develop in the heart:
- The heart muscle may become weak and not pump blood properly.
- Heart rate and heart rhythm may be affected
Keep a copy of your latest heart tests to show all of the healthcare providers who care for you. Other treatments for Duchenne, such as steroids, may affect your heart.
Duchenne affects most muscles, including muscles important for breathing. Breathing muscles will become weaker as they get older. The diaphragm is a muscle under the lungs that helps with breathing and supports taking deep breaths. Some people with Duchenne develop diaphragm weakness at this stage. This can cause difficulty taking deep breaths and expanding the lungs completely. After a person with Duchenne stops walking, they may have trouble coughing and require assistance.
Pulmonary function tests should continue to be done at least once a year to trend monitor the strength of the breathing muscles. However, it is important to talk to your pulmonologist about how to monitor for symptoms of difficulty breathing at home. Fatigue, morning headaches, and waking up frequently during the night may be mistaken for other problems, but can be a result of breathing problems at night.
If you have any symptoms of decreased oxygen during sleep, your pulmonologist should order a sleep study. 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 non-invasive ventilation can help breathing and increase oxygen during sleep.
A weakened diaphragm can also result in a weak cough, which decreases your ability to clear secretions out of your lungs and may result in more lung infections, and even pneumonias. During pulmonary function testing, the respiratory therapist will also measure the “cough peak flow.” If coughing becomes weak, methods to assist cough should be discussed with your pulmonologist.
Ensure you get all of the recommended childhood shots. If you get sick, especially with a cold or chest infection, make sure you receive antibiotics, extra breathing tests, and extra help coughing. Your lung function should also be tested before any type of surgery.
Learning & Behavior
You may have more trouble with headaches, mental lapses, or difficulty concentrating or trouble staying awake during the day due to difficulty breathing. These may affect you ability at school. Steroid use may also exacerbate behavioral problems such as controlling your emotions. At this age, you may have weak problem solving skills and may not be aware of the effect of your behavior on other people.
At this stage, you may need more help than your peers. With your parents, make sure you find care providers that you are all comfortable with. Take time to make sure you find someone that you are comfortable communicating your needs to.
Mental Health Care
Adolescence may be a difficult time for anyone. The physical limitations with Duchenne, as well as delayed physical development and delayed puberty that come with taking steroids, may make your teen years particularly challenging. You may want to be more independent but feel frustrated because you require more care and assistance from others, such as your parents. As muscle weakness progresses, people with Duchenne are at risk for becoming more isolated or socially withdrawn.
Talk to a parent, doctor, or teacher if you are feeling chronic sadness, depression, or anxiety. It is important 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.