Living with Duchenne Muscular Dystrophy

Let's talk about helping you continue on your journey with DMD. Living with DMD has its challenges, but when respiratory difficulties arise, there are things you can do to help improve lung function and well-being.

What to Do to Help Improve Well-Being

The Importance of Good Nutrition

As DMD progresses, nutritional issues also change and should be monitored and addressed. During childhood, especially when steroids are taken and wheelchairs are being used, there is a risk for obesity. A change in your child's body weight can affect the strength and activity of respiratory muscles. For example, excess weight can place extra pressure on already weak respiratory muscles and impede breathing. There are things you can do to help:

  • Have a registered dietitian nutritionist (RDN) on your team to help monitor and support good nutritional status
  • Avoid being underweight or overweight; both are harmful to respiratory health
  • Eat well-balanced meals, with the right amounts of fiber and fluid
  • Watch vitamin and mineral levels, especially vitamin D and calcium levels
  • Ask your doctor or RDN before taking supplements to see if it is appropriate

Gastrointestinal and nutritional management for DMD

Boys with DMD should be assessed regularly by a registered dietitian nutritionist to address their unique dietary needs. They often have gastrointestinal or nutritional complications, including weight gain or loss, dietary or nutrient imbalance, fluid imbalance, low bone density, swallowing dysfunction and mandible (jaw) contracture. These nutritional imbalances can negatively affect the respiratory, skeletal muscles and cardiac systems. In the later stages of DMD, there's a greater chance of becoming underweight. Muscle strength weakens which can make it hard to swallow and prolongs the time it takes to eat a meal (longer than 30 minutes), both of which can reduce the amount of food a person eats.

Based on their findings, the registered nutritional dietitian will design a healthy balanced diet, with optimum intake of calories, protein, fluid, and micronutrients, especially calcium and vitamin D for your needs. If weight loss persists, a feeding tube (called a gastrostomy tube or g-tube) might be something to think about. Talk to your care team to see if a tube is a consideration.

Recently the US Centers for Disease Control and Prevention (CDC) in conjunction with the TREAT-NMD network for neuromuscular diseases, the Muscular Dystrophy Association, and Parent Project Muscular Dystrophy (PPMD) has updated the guidelines for DMD care considerations.

The following charts adapted from these guidelines show what you can expect from a registered nutritional dietitian through the disease stages of DMD:

Stage 1:
At diagnosis
Stage 2:
Early ambulatory
Stage 3:
Late ambulatory
Stage 4:
Early non-ambulatory
Stage 5:
Late non-ambulatory
 
 
 
 
 
 
Include assessment by registered dietitian nutritionist at clinic visits (every 6 months); discuss obesity prevention strategies; monitor for changes in weight, especially during critical transition periods
Provide annual assessments of vitamin D and calcium levels
Assess difficulty swallowing, constipation, gastroesophageal reflux disease, (inflammation of the canal that connects the throat to the stomach), and gastroparesis (weakness of the muscles of the stomach) every 6 months
Discuss possibility of gastrostomy tube as part of usual care
 
Stage 1-5:
At diagnosis to Late non-ambulatory
 
Include assessment by registered dietitian nutritionist at clinic visits (every 6 months); discuss obesity prevention strategies; monitor for changes in weight, especially during critical transition periods
Provide annual assessments of vitamin D and calcium levels
 
Stage 2-5:
At Early ambulatory to Late non-ambulatory
 
Assess difficulty swallowing, constipation, gastroesophageal reflux disease, (inflammation of the canal that connects the throat to the stomach), and gastroparesis (weakness of the muscles of the stomach) every 6 months
 
Stage 3-5:
At Late ambulatory to Late non-ambulatory
 
Discuss possibility of gastrostomy tube as part of usual care

Symptoms of DMD and treatment options

Here are some of the things that your registered dietitian nutritionist might be looking for and how they may be treated.

Symptoms of dysphagia (trouble swallowing)
chart arrow
Refer to speech-language pathologist for swallowing assessment, including videofluoroscopic swallowing study
 
Low serum concentrations of 25-hydroxyvitamin D <30-0 ng/mL
chart arrow
Treat for vitamin D deficiency
One or more of the following:
  • Weight loss and dehydration
  • Moderate and severe dysphagia
  • Aspiration
  • Malnutrition
chart arrow
Recommend gastrostomy tube placement
 
Calcium intake less than recommended dietary allowance
chart arrow
Recommend increased dietary intake and supplementation

Using Cough Assist Regularly

Using cough assist on a regular basis, not just when you feel sick, can benefit respiratory health. Your doctor may recommend cough assist be done daily (like physical therapy for the lungs) to help maintain a clear airway and as a way to prevent the airways from getting blocked with mucus and the lungs from becoming restricted. There are two ways to assist a cough:

  1. Manual cough aid
  2. Mechanical cough aid

Managing Medical Complications

Flu Shots

Weakened breathing muscles and being non-ambulatory puts those with DMD at a higher risk for flu-related complications which can be life-threatening. Here are some things to remember:

  • Talk to your medical team before getting any vaccine--typically those with DMD would get the injectable flu vaccine that contains inactivated viruses
  • Standard of care guidance recommends getting the flu vaccine every year to help protect against the flu
  • If you suspect the flu, call your doctor immediately for help obtaining medicine to treat your flu symptoms
  • Antiviral drugs can lessen flu symptoms and shorten the time you're sick by a day or two

Learn more about care for the flu.

Pneumococcal Vaccines (new-mo-coc-cal)

There are many kinds of pneumococcal infections that can lead to more serious conditions. The Centers for Disease Control (CDC) recommends pneumococcal vaccines including PCV13 and PPSV23 for children who have illnesses, like DMD, which put them at a higher risk for serious diseases.

Protecting with pneumococcal vaccines is one of the CDC’s recommendations for patients with medical conditions like DMD.

Also, keep a record of this and every other vaccine your child gets and share it with your medical team.

Common Colds

Colds are annoying and make people feel miserable. They can also lead to more serious illnesses including respiratory tract infections or bronchitis. So those with DMD should be watched carefully when they catch colds.

Should your child start feeling worse, talk to a doctor about antibiotics or other medications that can fight the cold to avoid complications. If a cold becomes something more serious, a cough assist machine may have to be used more frequently. Talk to your doctor about how frequently to use cough assist devices and how to properly adjust the settings on the device.

The benefits of cough assist techniques and a multidisciplinary team for DMD are an important part of your approach to caring for people with DMD.

Being Prepared

Accidents happen, colds escalate, difficulties can arise when you least expect it. But a little preparation can go a long way to help you manage unexpected health emergencies with DMD. Here's how to help the ER staff help you:

1

Plan Ahead

Learn where your day-to-day doctors have hospital privileges and consider using that emergency room if you can. If you have to use a different emergency room, ask if they can contact your regular doctors.

  • During your regular clinic visits with your neuromuscular team, make a plan regarding how they want to handle respiratory emergencies
  • If you are in an ER where your neuromuscular team is NOT, have a plan in place ahead of time to find out how to contact your neurologist and pulmonologist after hours
  • Speak to your local Emergency Medical Technician (EMT) about your family’s needs in an emergency situation
2

Have Medical Information Handy

  • Keep records of recent doctor visits and test results
  • List any medication being taken
  • List doctors’ names and contact information
  • Include insurance information

You can keep hard copies in an “emergencies” folder, keep digital copies on a thumb drive, or use a service like MedicAlert® to catalog medical information.

3

Bring An Advocate

A family member or friend isn’t just company, they can help:

  • Facilitate conversations with the ER staff
  • Take notes on medical care advice given by the ER staff
  • Provide emotional support and be a sounding board in a time of stress
4

Remember To Bring

  • Any respiratory equipment used at home such as cough assist--not all emergency rooms have this equipment

Things to Remember When Going to the ER or Hospital

Mention the following important information to an ER or hospital physician during a visit or before a procedure:

  • Doctors should be careful when giving patients with DMD oxygen
    • supplemental oxygen can sometimes make his ability to breathe on his own even more difficult and should never be administered without simultaneously monitoring carbon dioxide
  • People with DMD should not be given succinylcholine (sux-innel-ko-leen)
    • succinylcholine is a muscle relaxant sometimes used during surgery or other medical procedures
    • succinylcholine can cause complications such as dangerously high levels of potassium in the blood which can lead to cardiac arrest
  • General anesthesia should be administered intravenously (into the veins) instead of being inhaled because inhaled anesthesia can lead to cardiac complications
  • Talk to your doctor for any specifics you need to remember for your particular case
  • Visit the Parent Project Muscular Dystrophy's emergency care guidelines for more tips and information.

Travel Tips

What to do when traveling.

Traveling with a medical condition means preparing for some potential obstacles along the way. Your doctor can assess your ability to travel as well as offer advice on how to make traveling easier.

Air Quality Can Affect Breathing

The environment and air quality that you'll be heading into can affect how easy or difficult it will be to breathe.

High Humidity

  • A lot of moisture in the air can sometimes affect people with respiratory conditions
  • Consider staying indoors on really humid days
  • Carry a sweater, scarf, or blanket to help when going from heat and humidity outdoors to the air conditioning indoors

High Altitude

  • Breathing at higher altitudes (usually about 5,000 ft and higher) can be challenging for healthy people and even more so for people with respiratory conditions
  • Check with your doctor prior to traveling to higher altitudes
  • Drink plenty of water
  • Stay rested

High Pollen Counts

  • Check local weather newscasts for information about high pollen counts in your area
  • Pollen counts are highest between 5 AM and 10 AM so consider staying indoors during this time
  • Keep windows closed in cars or the home and use air conditioning

Frigid Air Temperatures

  • Cold air is often dry air which can irritate the airways of people with chronic respiratory conditions
  • Consider using a scarf to cover your mouth and nose when going outdoors in the cold
  • Avoid excessive activity outdoors in very cold weather

Other Travel Considerations

  • Know your rights before your travel. The Air Carrier Accessibility Act prohibits discrimination during air travel
  • Create packing and travel lists
  • Ship medical equipment (include: addresses, items, contact numbers) beforehand, if possible
  • Research accessibility of airports, hotels, tourist sites, and any other locations
  • Plan what kind of assistance you will need to get to and from the airport and make arrangements beforehand if needed

More Travel Resources

The websites below can provide you with travel information, tips, and assistance for people traveling with DMD and other medical conditions.

Able2Travel.com
tsa.gov
mda.org
cdc.gov
Disabled-World.com