Late Non-Ambulatory (Adults)

It is important that adults at this stage receive the care and services they need to stay as healthy, independent, and active as they want to be. It is also important to be engaged in a job or career, maintain an active social life, and stay engaged with the community.

Important Information for All Stages

Remember: If you need surgery or go to the emergency room, tell the doctors not to give inhaled anesthesia or succinylcholine. They also need to use caution when giving oxygen.

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.

Adult Imperatives

The Adult Imperatives for Duchenne MD (download) describes the imperative components of transitions of care across late adolescence and adulthood as described in the Care Considerations. This document is intended as a brief, single page snapshot of care, helpful for medical providers who may not be familiar with caring for adult men living with Duchenne.

Physical Symptoms

Adults with Duchenne have more trouble using their hands and maintaining good posture. Weakness continues during the adult phase. However, many young adults with Duchenne can maintain some function of their fingers well into the adult stage. It is important to plan for an adult life that maintains as many elements of independence as possible, within a supportive care environment.

Transition to Adult Care

Transitional planning from pediatric to adult care should be a multi-year process, starting in adolescence. The individual living with Duchenne or Becker should play an active role in their decision making and overall care. During clinic visits, ask your parents/support people to leave the room for a few minutes so you are able to speak with providers by yourself. The Duchenne Transition Checklist can help you prepare for your future wishes, goals and areas of support. Part of the transition planning includes care coordination & medical care, education,employment, & vocations, housing and assistance with ADLs, transportation, relationships and more.

Steroids

Adults with Duchenne should talk to their doctors about whether to continue using steroids, and if so at what dose. Staying on steroids may slow progression of weakness, but as an adult, the benefits and downsides should be considered on an individual basis.

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.

Stretching of the hands and upper limbs should continue to be a part of your daily routine may help minimize tightness and help with comfort. 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 stretching routines.

Mobility & Environmental Accessibility

You should talk to your physical therapists and occupational therapists about the best equipment to support your independence and participation. The therapists may suggest adaptive equipment to help adults with eating, drinking, toileting, and transferring.

Heart Care

Heart problems are common during this phase. Heart tests (EKG, echocardiogram, and/or cardiac MRI) may begin to detect heart changes that can be treated with medication. Visits to your cardiologist should happen at least once a year, and may be increased to every six months with signs of heart disfunction or fibrosis.

Two problems are common in the hearts of adults with Duchenne:

• Your heart muscle may become weak and not pump blood properly.
• You may have problems with heart rate or rhythm.

You should be started on medication (ACE-Inhibitor) by this stage, even if you are not showing signs of heart problems or changes. Keep a copy of your latest heart test results to show other members of your care team, or if you have a medical emergency requiring a hospital visit.

Lung Care

Duchenne affects most muscles, including muscles important for breathing. Breathing muscles will become weaker with age. The diaphragm is a muscle under the lungs that helps with breathing and supports taking deep breaths. Many people with Duchenne develop diaphragm weakness at this stage and have difficulty taking deep breaths. Adults may also have trouble coughing and require assistance.

Pulmonary function tests should continue to be done at least once a year to monitor the strength of the breathing muscles over time. 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 the child’s ability to clear secretions out of his 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.

Psychosocial and Mental Health Care

The physical limitations and progression of Duchenne and Becker may make you feel angry or sad. Adults living with Duchenne or Becker may also be anxious about their health status and as muscle weakness progresses, they are at risk of becoming more isolated or socially withdrawn. Talk with your caregivers and support people about your feelings. Work with your caregivers and support people 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. 

Palliative Care

Palliative care is a system of care aimed at improving quality of life for patients and their families. Palliative care teams work alongside your other medical teams to target suffering from symptoms and stress of serious illness. Palliative care teams can help you think about what is most important to you and coordinate care with your other teams to ensure that your medical care is in line with your goals and values. Palliative care can be appropriate at any age or any stage of illness. There is no such thing as “too early” for palliative care. If you are an adult with Duchenne or Becker and have not seen a palliative care team, consider asking to see them at your next visit. You can also visit this site to locate a palliative care team nearby. 

Navigating late stage decision making

All adults need a plan for what may happen in case of a healthcare emergency. This is especially important for patients with advanced Duchenne. Who will make decisions for you if you cannot speak for yourself? What decisions would you like for them to make? If you are at the end of your life, where do you want to be? These conversations can seem scary, but it is always easiest to talk about these things when life is calm, before a crisis occurs. Your healthcare team can help with these conversations, and palliative care clinicians are experts in helping with this. 

The Conversation Project is a wonderful resource for anyone thinking about these questions. This website will walk you through:

• Thinking about what is most important to you (The Conversation Starter Guide)
• Choosing a Healthcare Proxy (a person to make decisions for you)
• Talking with your healthcare team about your plan
• Creating an advance directive or MOLST/POLST form, a document that communicates your plan
• And much more

If you make a plan and then someday change your mind about what you want, that’s ok. You can change your mind anytime. You just need to let your healthcare proxy know and ask your healthcare team to help you create new paperwork. A palliative care team has expertise in these conversations, but any healthcare provider should be comfortable in assisting with getting started.

Coping with loss

Loss is a normal part of life, but for people with Duchenne, it can be even more common. Each stage of life can mean physical changes and losses. Friends and family members with Duchenne may die earlier than anyone expected. Grief is the sadness we feel when such losses happen. It is normal for parents to grieve the loss of a “normal” childhood when their child is diagnosed with Duchenne. It is normal for people with Duchenne to grieve the way their body used to be, or to feel sad when they see friends without Duchenne who are able to do things they can no longer do. 

Each person learns to cope with these losses in their own way. Some people find support groups helpful to manage their grief and feelings of loss; some people benefit from therapy or counseling; some ultimately find that their grief and mood changes are related, and medications are helpful. More resources for grief and loss support can be found here:

PPMD’s Adult Advisory Committee (PAAC)

Pathfinders Neuromuscular Alliance

PPMD appreciates  the work of the Pathfinders Neuromuscular Alliance, a user-led charity from the UK who work to promote choice and control and quality of life for teenagers and adults living with Duchenne Muscular Dystrophy.

The Pathfinders Neuromuscular Alliance has developed a series of advice guides for teens and adults living with Duchenne, and have kindly given PPMD permission to share with our community. They have used their knowledge to create downloadable comprehensive guides in the areas of ventilation, nutrition, and technology usage.