Transitional Phase (about ages 6-9)
- During the transitional 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. Children with Duchenne may 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 maneuver”).
- Most children in this age 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). Children in this phase usually will need a
stroller, scooter or manual wheel chair for long distances.
- 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.
Important information for all stages
Glucocorticoids (“steroids”) are the only medications approved to treat Duchenne. 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 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.
Muscle and Joint Care
Exercise can help keep your child active, but some exercises may damage the muscles. Ask your physical therapists and doctors which exercises and activities your child should and should not do.
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. Boys in the transitional phase of Duchenne usually do not have problems with their heart, but problems with rate, rhythm, and fibrosis may begin to develop.
Heart screening should happen at least every 2 years until age 10, and then at least once a year. The cardiologist may change this schedule based on heart test results. Heart tests (EKG, echocardiogram and/or cardiac MRI) may begin to detect heart changes that can be treated with medication.
In Duchenne, the heart is already damaged before symptoms begin. Your child may need to start heart medicines even if he doesn’t have symptoms. 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 steroid use, may affect your child’s heart.
Many boys at this stage do not have breathing problems, but some do. Fatigue 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. 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.
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 and Behavior
Some, but not all, children with Duchenne have learning or behavior problems. Your child should be screened for learning difficulties if you or your child’s doctors or teachers notice learning delays. 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 noncompliance or arguing. They may have difficulty making transitions. They may also be taking steroid medication with strong side effects that can 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 will need more help than many of his peers. Help your child (and your family) by getting your child 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.
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. Counseling during this stage may be helpful and should be considered.