Late Ambulatory (late childhood/adolescent/young adult)

In the late ambulatory stage, walking will become increasingly difficult, with greater struggle during motor activities, such as climbing stairs and getting up from the floor.

Continued evaluation of learning delays or specific learning disabilities is important at this stage. Psychologists and neuropsychologists can help identify the interventions best suited to meet your educational needs. Continued support from professionals will be necessary to help with any learning and behaviour issues, and specific interventions may be necessary to address coping strategies for dealing with the loss of physical strength and functions. At this stage, you should start to set goals for the future so that you and your family can work with your school to organise the education and training needed to meet those goals. For some, a neuropsychology evaluation may be indicated to help identify possible cognitive impairments and provide strategies for home and classroom settings to help you function at your best.

Caregiving requires emotional and physical energy. Carving out time for oneself, as well as your relationships with others, is extremely important. Developing support networks that include extended family and trusted community members can be very helpful in giving caregivers time to rest and recharge.

Physiotherapy input will continue to focus on maintaining range of movement, strength, and function in order to maintain independence. If joint tightness cannot be managed by physical therapy, assessment and input from orthopedic specialists may be necessary. A physiotherapist has many roles in the care team, including overseeing stretching regimens, recommending equipment such as wheelchairs and standers, and promoting continued weight bearing and standing (this will hope with both bone health and digestion). It is important to make sure that mobility aids, such as wheelchairs, are equipped with supportive seating to promote body alignment, proper positioning, and comfort. A daily home stretching program is of continued importance.

Ongoing management of steroid treatment is important at this stage, with attention to the specific regimen and dose used, as well as attention to side effect prevention, surveillance, and management. Twice-yearly assessments to monitor skeletal muscle strength and function are important. Continued attention to weight control needs to take into account any tendency to be under- or overweight with appropriate intervention if there is a concern.

Ongoing surveillance of bone health and risk for fractures is important while on steroids, especially as mobility decreases. It is important for your NMS to monitor the risk for fractures through blood tests that check vitamin D levels (ā€œ25 OH vitamin Dā€), as well as imaging tests such as a DEXA scan or spinal X-rays that can assess bone density/health. Diet should be assessed at each visit to ensure that you are eating adequate amounts of vitamin D and calcium. Height, lengths, and weight should continue to be monitored for signs of growth delays.

Ongoing assessments of the heart and breathing muscles are necessary. ECG, cardiac MRI or echocardiogram and other types of tests should be done at least annually from diagnosis, and more frequently as needed after age 10. The cardiologist will recommend interventions if there are any changes observed on the ECG, cardiac MRI or echocardiogram.