Late Ambulatory (Late childhood/Adolescence/Young Adults)
At this stage, there is often fatigue with walking long distances and difficulty keeping up with peers. A carriage/scooter/manual wheelchair may be needed to help decrease this fatigue.
Important Information for All Stages
If your child is taking steroids, it is imperative they are not stopped suddenly for any reason. This puts them at risk for an adrenal crisis, which is a life-threatening condition.
- During the early/late ambulatory phase of Duchenne, the quadriceps (the muscles in the front of the thighs) get weaker and children have more and more difficulty walking. This causes people with Duchenne to be off-balance as they shift their weight while walking. People with Duchenne may also walk on the balls of their feet or toes to help stay balanced.
- In order to compensate for a weak trunk, people with Duchenne may stick out their bellies and throw their shoulders back as they walk. When asked to stand up, they will put their bottoms up in the air first and use their arms for support by “walking” their arms up their legs with their hands until they are standing (also known as the “Gower Manuever”)
- Most children in this age range have use of their hands and arms, but they may be weakened, causing them to have difficulty carrying books and other school materials (even when using a backpack). Fatigue (feeling tired) is common, and children with Duchenne may need the use of a stroller, lightweight wheelchair, or electric scooter for longer distances. Some children may use a walker to assist them in getting around the classroom.
Steroids (corticosteroids) are the only medications approved to treat Duchenne. They are different from the anabolic steroids that are sometimes misused by athletes who want to become stronger. They may act to slow down the progression of muscle deterioration. It is recommended to start steroids when a child is in the “plateau phase” — when the child’s motor skills are not improving anymore but have not yet gotten worse. This is usually between ages 4-6 years.
Taking steroids may cause many side effects. Side effects should be monitored and addressed at each visit with your neuromuscular provider. A child who is taking steroids may be more likely to get sick. Make sure your child gets a ﬂu and pneumonia shot to avoid these common illnesses.
Muscle and Joint Care
Most muscles of the body are eventually affected by Duchenne, including the heart and breathing muscles, and need to be monitored.
Exercise in children in this age range can be beneficial and keep them active. However, some types of exercises should be avoided in Duchenne. Talk to your physical therapist and neuromuscular team about which exercises and activities are safe for your child.
Continue to stretch your child’s muscles and joints at least 4 to 6 times a week. There are braces and splints (orthoses) that can help keep a child’s hands, feet, knees, and back in the correct position. Stretching and orthoses can help delay and minimize joints becoming locked in one position (contractures). Ask your doctor and physical therapist about helpful stretches to do and about using orthoses.
Regular heart screening is very important. Children in the early and late ambulatory phase of Duchenne usually do not have any heart symptoms, but problems with heart rate, rhythm, and fibrosis may begin to develop.
Your child 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. Heart tests (EKG, echocardiogram, and/or cardiac MRI) may begin to detect heart changes. Cardiac medication can be started with signs of changes.
In Duchenne, the heart is affected before heart symptoms appear (cardiomyopathy). Your child may need to start heart medications even if there are no symptoms of heart problems. Keep a copy of your child’s latest heart tests to show all of the healthcare providers who care for your child. Other treatments for Duchenne, such as steroids, may affect your child’s heart.
Children in this stage usually do not have any problems breathing, but some may. Pulmonary function tests should be done at this stage at least once a year. Keeping a copy of your child’s latest breathing tests to show other healthcare providers is a good idea.
Ensure your child gets all of the recommended childhood shots. If your child gets sick, especially with a cold or chest infection, make sure he/she receives antibiotics, extra breathing tests, and extra help coughing. Your child’s lung function should also be tested before any type of 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.
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.
Learning & Behavior
Some, but not all, children with Duchenne have learning and behavior problems. If you or your child’s teacher suspect any learning delays, your child should be screened by a professional. Children with Duchenne are more likely to have difficulties with language (such as responding to questions or expressing himself) and learning to read.
Behavior problems are quite common in all young children, and children with Duchenne are no different. However, young children with Duchenne may have more difficulty with impulsivity and emotional control than other children their age. They are also more likely to be rigid and inflexible in their thinking, which can result in stubbornness or arguing. They may have difficulty with change or resist new routines. Additionally taking steroids may have an impact on their behavior, such as making them more emotional and active than normal.
As your child gets older, it is likely that he/she will need more help than many of their peers. Helping your child and other family members to become comfortable with your care team early can give them the added support they need.
Mental Health Care
Children with Duchenne tend to become more aware of their differences and limitations during this stage. Although most adjust well to their condition over time, there may be times of emotional distress because of Duchenne. Professional counseling (by a psychologist, neuropsychologist, psychiatrist, social worker or nurse with mental health expertise) during this stage may be helpful and should be considered.