Living with Duchenne Muscular Dystrophy

First, let’s talk about helping you meet the challenges that lie ahead. The difficulties of living with DMD aren’t new to you. You’ve been living with them for years. When respiratory difficulties arise, you should know there are things you can do to help improve lung function and well-being.

What to Do to Help Improve Well-Being

Using Cough Assist Regularly

When a person’s cough isn’t as effective, he needs help to cough deeply and clear the airways. There are two ways to assist a cough:

  1. Manual cough aid
  2. Mechanical cough aid

What’s important to remember though is that once cough assistance starts, it shouldn’t just be saved for when he feels ill. Your doctor may recommend cough assist be done daily 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.

Knowing how to manually or mechanically aid a cough on a regular basis while living with DMD is critical.

Good Nutrition

“Eat well” is good advice for everyone, but it’s especially important to people with DMD. Good nutrition helps maintain an ideal body weight which is key to managing DMD long term. People with DMD should:

  • Avoid being underweight or overweight; both are harmful to respiratory health
  • Watch vitamin and mineral levels, especially vitamin D and calcium levels
  • Ask your doctor before taking supplements to see if it is appropriate

As DMD progresses, there’s a greater chance of becoming underweight. Why? Because muscle strength weakens which can make it hard to swallow and prolong the time it takes to eat a meal (longer than 30 minutes), both of which can reduce the amount of food a person eats.

If weight loss persists, a feeding tube (called a gastrostomy tube or g-tube) might be something to think about. It’s important to talk to your care team to see if a tube is even a consideration.

Breathing Exercises

Since the issue in DMD is weakening muscles, the question many ask is whether there are exercises people with DMD can do to strengthen their respiratory muscles. The answer is: it depends. Some could benefit from muscle training in the early stages of the disease. However, this will generally be for boys who are able to perform the exercises and follow the routine. The benefits of exercise can only be assessed on a case-by-case basis. That’s why it’s best to talk to your doctor or health care team before starting any kind of exercise program.

Managing Medical Complications

Keep a watchful eye on infections.

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. That’s why it’s important to:

  • Talk to your medical team before getting any vaccine—typically boys and men 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 2

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 this vaccine 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 boys with DMD.

Also, keep a record of this and every other vaccine a boy with DMD 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 a boy 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, it’s important to use a cough assist machine while sick. This type of machine may also 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

The more prepared you are for emergencies, the better you can handle them. It’s important that the emergency room (ER) staff understands the unique needs of people with DMD. Here’s how you can help the ER help you:

1

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.

2

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
3

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, you need to 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
4

Remember To Bring

  • A cell phone and charger
  • Any respiratory equipment used at home

Things to Remember When Going to the ER or Hospital

Make sure to 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

Travel Tips

What to do when traveling.

Traveling with a medical condition means facing some obstacles along the way that you’ll need to try and prepare for. Your doctor can assess your ability to travel as well as offer advice on how to make traveling easier.

Whether you’re traveling by air, land, or sea, there are things you’ll want to consider and prepare before planning a trip.

For example, air isn’t the same everywhere. And since air can affect how easy it is to breathe, whenever you travel away from home, be aware of the air you’re heading into.

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
  • Think about how you will get to and from the airport and what kind of assistance you will need (i.e., taxi, help from a friend)

More Travel Resources

Able2Travel.com

DMD Support Groups

The following organizations provide resources and ways to connect with others in the DMD community for information and support.

Cure Duchenne
www.cureduchenne.org

Jett Foundation
www.jettfoundation.org

Muscular Dystrophy Association
www.mda.org

Parent Project Muscular Dystrophy
EndDuchenne.org

DMD Clinical Trial Updates

There’s a great need for new treatment options in DMD.

That’s why in the DMD community there’s a lot of hope and anticipation about a number of new therapies on the horizon. Many pharmaceutical companies are running clinical trials with the goal of bringing new treatments to DMD.

You can talk to your doctor about the DMD clinical studies that might impact you the most. Or you can see the latest news and updates on DMD clinical studies by visiting clinicaltrials.gov.