Having multiple experts on the team allows for cross-specialty coordination and communication. The goal is to provide broader care and support.
Physical Medicine and Rehabilitation: physicians who help improve your muscles’ ability to move
Respiratory Therapist: assesses lung function and helps develop treatment plan
Speech Language Pathologist: evaluates speech and swallowing skills and develops treatment plan
Those with DMD should see a doctor who specializes in respiratory care (a pulmonologist):
These and other important tips on living with DMD can help you improve well-being.
Measuring lung capacity while they’re still able to walk helps boys become familiar with how to do the test. That makes future tests easier to do. The measurements also establish a baseline level of lung muscle strength. Think of it as establishing a lung strength history. Having a history can help guide future treatment decisions. It can also help detect and manage any potential problems early.
After losing the ability to walk, it could take a while before the signs of respiratory loss become obvious. Evaluating respiratory function before problems arise lets doctors monitor progress, detect the first signs of respiratory muscle weakness, and plan the best treatment options to help keep the lungs working as well as possible.
Doctors check respiratory function because it can help signal trouble breathing now or in the future. The results can also indicate respiratory muscle strength and help measure disease progression.
Spirometry is one of the most common ways to learn how well the respiratory muscles are working. A spirometry test can gauge how DMD is progressing and influence treatment decisions. FVC, FEV1 and PEF are all measured by spirometry.
MEP (Maximal Expiratory Pressure) and MIP (Maximal Inspiratory Pressure) are indications of respiratory muscle strength. Together they measure pressure created when you inhale or exhale into a device that creates a certain amount of resistance to inhaling or exhaling. MIP measures how strongly you can breathe in, MEP measures how strongly you can breathe out.
Imagine it this way: say you have a straw in a very, very thick milkshake. You’d use all your might to inhale through your mouth and draw the milkshake up the straw. That’s the kind of pressure MIP measures—it’s the pressure you create by trying to breathe in against a somewhat blocked tube. On the other hand, MEP measures the opposite—the amount of pressure you create trying to exhale against a somewhat blocked tube, like blowing the milkshake out of the straw.
Read about ways you can help maintain respiratory airway clearance.
Sometimes the first signs of respiratory muscles weakening can happen at night. Performing tests let doctors gauge how well the respiratory muscles are working while someone sleeps. The results of these tests can help inform care options.
For this test, a person stays overnight at a special sleep center so specially trained experts can measure how well the breathing muscles and lungs can bring oxygen into the body and remove carbon dioxide.
In addition, when this is measured during deep sleep (REM or dream sleep when the body relaxes completely), it can pick up the early stages of respiratory failure. This would prompt using a breathing machine called a ventilator.
You may hear your care team refer to this test as polysomnography (polly-sum-nog-rah-fee). It’s the gold standard in assessing sleep-related breathing problems. But it’s important to have this test interpreted by a pulmonologist.
This is a test that a person can do in his own bed. How does it work?
A doctor uses oxygen saturation levels to see if there’s enough oxygen in the blood at night. You may hear your care team refer to this test as an oximetry test.
This at-home test is not as extensive as the test in the sleep facility. Your care team can help you decide which test is best for you.
There are ways you may be able to prevent future problems.
When the respiratory muscles weaken, complications can happen. Boys and men may not be able to cough or breathe strongly. This can lead to pneumonia and other serious breathing problems. You should know these complications may be preventable. How? With the close watch of respiratory function and talking to a doctor right away when complications happen.
As the respiratory muscles weaken, it’s harder and harder for the body to clear the airways of secretions and mucus. This buildup can cause an infection to quickly develop into something more serious or prolong the infection. Even what seems like a minor thing, like a cold, can become serious quickly in those with DMD. So, it’s important to closely monitor any medical issues that come up.
Your respiratory system is divided into two sections: the lower and upper respiratory tracts. Each can get different types of infection.
The upper respiratory tract Includes the nose, nasal cavity, and the throat.
The most frequent infection in the upper respiratory tract is the common cold, which is usually caused by a virus. A cold can quickly escalate into sinusitis or become more serious and move into the lower respiratory tract. That’s why you should watch a cold carefully. If it starts getting worse or won’t go away, be sure to talk to your doctor.
The lower respiratory tract includes the windpipe that leads to the lungs as well as air sacs in the lungs that are involved in getting oxygen into the blood.
You can help prevent the flu by getting a flu vaccine every year. Treatment for pneumonia involves antibiotics for bacterial pneumonia and in some cases antiviral medication for viral pneumonia. There’s also a vaccine that helps protect against bacterial pneumonia. Talk to your doctor to see if vaccination is appropriate.
The CDC recommends flu vaccination once a year, for people over 6 months of age, as soon as the vaccine becomes available.
Learn more about what you can do to fight off illness with flu shots and vaccines.
A cough involves bringing air in and forcing it out of the lungs. As respiratory muscles weaken someone with DMD may not be able to cough or breathe well. In addition, infections and complications can last longer. A strong cough involves three phases:
Deep breath in
Forceful breath out, at first, with the vocal cords closed for a second to build up pressure
The vocal cords open and there’s a large flow of air outwards
Since the respiratory muscles allow a person to breathe in or out, as the muscles weaken so does the strength and effectiveness of the cough.
When a cough isn’t working as well as it should to clear the airways, it needs some assistance. There’s a range of ways to get this assistance. The goal in all these options is to maximize a person's ability to cough and clear his airways effectively.
There is a way of measuring the effectiveness of a cough using something called Peak Cough Flow (PCF), sometimes referred to as Cough Peak Flow (CPF). PCF measures how fast you can breathe air out when you cough. The PCF score is an indication of how well mucus and other secretions are being removed from the airways. Scores will vary so patients should talk to their doctor to see what a healthy score is for them.
This technique involves another person helping a patient cough. Someone trained in this technique presses on the patient’s abdomen while the patient coughs to make the cough more forceful.
The idea here is to stack one small breath on top of another small breath until the lungs are filled. You might also hear a doctor refer to breath stacking as lung volume recruitment (LVR).
Breath stacking involves a mouthpiece or facemask and a manual resuscitator bag (also known as an Ambu® Bag). The manual resuscitator is an inflatable air bag that looks a little like a balloon. Squeezing the air bag sends air to the lungs.
He squeezes the bag several times in a row, holding his breath between each squeeze until his lungs are full. Then he can cough more forcefully.
These devices mechanically simulate a natural cough by inflating then helping to pull air out of the lungs. They create a greater speed in a cough than the manual assisted cough method.
When a person breathes in, the machine delivers air to the lungs to help inflate them (called positive pressure). When it’s time to breathe out, the machine helps pull air out of the lungs (called negative pressure). This changing of pressure from positive to negative helps create a more effective cough.
Even when feeling well, patients should continue to practice their cough assistance techniques. Why? Because it will help the technique become second nature. So when it has to be performed in a more urgent situation, it can be done easily.
Using these techniques regularly can also benefit the lungs. The more often you fully inflate the lungs, the more elastic the tissues stay. And there’s evidence regular use can help prevent atelectasis.
Doctors can help answer that question. Boys might start with manual techniques and when those become less effective, move to mechanical devices. A doctor can help decide which technique is best, when to initiate it, and how often to use it.
Typically, boys with DMD first need help breathing at night. As DMD progresses, they may also need help breathing during the day. Specially designed machines can support breathing and improve a boy’s quality of life.
There are two types of ventilation: noninvasive and invasive. Choosing which type to use is a very personal decision; what’s right for one boy may not be right for another. By talking to the care team, caregivers and the boy can decide what type of ventilation is best for him.
Noninvasive devices deliver breaths into a patient via:
There’s no surgery, nothing breaks the skin, or is permanently attached to the body.
Potential benefits of noninvasive ventilation support:
Bi-level ventilation provides 2 levels of pressure. One level to push air into the lungs, then a second, lower level to allow breathing out. You may also see these named BiPAP™ machines (for bi-level positive airway pressure ventilators).
How they work:
Unlike BiPAP machines, CPAP (continuous positive airway pressure) machines deliver the same level of pressure to breathe in and out. Why use one versus the other? Typically those with DMD use BiPAP. Here’s why: weakened respiratory muscles need extra support. The dual pressure levels of BiPAP give greater assistance to the muscles that control breathing in and little or no resistance to breathing out. CPAP machines can’t do that because they provide only one level of pressure. CPAP machines are typically for sleep apnea caused by an obstruction in the airway.
As the respiratory muscles weaken, those with DMD may need even more ventilation support.
Volume cycled ventilators deliver a specific volume of air to the lungs instead of providing pressure like a BiPAP ventilator machine does. That matters because volume cycled ventilators may provide more breathing support than bi-level ventilators. That’s why some doctors recommend volume cycled ventilators as DMD progresses and the respiratory muscles continue to lose strength.
Invasive ventilation involves inserting a tube in the throat to serve as an airway. Usually it’s an option considered once other noninvasive options have been explored.
Advantages of invasive ventilation:
Disdvantages of invasive ventilation:
Performing a Tracheostomy
A tracheostomy is invasive ventilation because it involves a surgical procedure. An incision is made in a person's neck and through the trachea (windpipe) and a tube is inserted. Air now goes in and out of the tube and he no longer breathes through his nose and mouth.