Can respiratory problems be prevented? Yes, but only to a certain extent and for a limited amount of time, if the neuromuscular disease attacks the respiratory muscles.

Flu shots are important for people with neuromuscular disorders, especially if they have weakened respiratory muscles.

A little common sense, being prepared for emergencies with backup plans and equipment, and not taking unnecessary chances will take you a long way. For example, get a flu shot (influenza vaccine), and avoid extended exposure to people with colds. Be familiar with the symptoms of chronic underventilation so you can speak with your doctor if they arise.

The keys to limiting respiratory problems are:

STAY WELL

Avoid colds and other respiratory infections as much as is practical.

GET IMMUNIZED

Keep routine childhood immunizations (such as those against polio, measles and mumps) up to date, with advice from your doctor.

Get a yearly flu shot if you’re a child or adult with a neuromuscular disease, or a family member. Ask about pneumonia vaccinations.


COUGH

Some of the muscles you use in breathing are needed for coughing. Your doctor can tell you if your ability to cough isn’t sufficient to clear secretions from your lungs and trachea.

The CoughAssist delivers air to the lungs and then quickly reverses air flow to pull out secretions.

The cough reflex is an explosive expulsion of a volume of air from the lungs. The high pressure and speed of this air propel irritants, such as mucus, up and out of the lungs. Neuromuscular disorders can weaken the ability to cough, but coughing is needed to remove mucus, especially during an acute respiratory infection.

A simple way to increase the effectiveness of your cough is a maneuver known as a manual cough assist. This maneuver involves a caregiver’s pressing on the upper abdomen as you cough. A respiratory therapist or other health care professional can show you how to do this.

A cough assistance method that’s effective even when cough muscles are severely weakened is a small electrical machine that first delivers a large volume of air and then quickly reverses air flow to pull out secretions. A brand name for such a device is the CoughAssist.

A caregiver can deliver manual cough assistance by pressing on the upper abdomen, just beneath the ribs, as the patient coughs.

TREAT INFECTIONS

Monitor and treat respiratory infections promptly.

Once an infection starts, poor air exchange and weak coughing muscles make it difficult to clear mucus from your lungs. The mucus can become thick and can plug the airways, which can cause areas of the lung to collapse. (The medical term for lung collapse is atelectasis.)

Your doctor may suggest a chest X-ray for accurate determination of the presence of pneumonia or atelectasis.

Antibiotics will likely be prescribed if the infection is bacterial.

Cough assistance, either manual or via the CoughAssist, may be needed.

Some situations respond well to high-frequency chest wall oscillation, a form of vibration therapy delivered through a vest that shakes up mucus and moves it up where it can be coughed out.

WATCH FOR SCOLIOSIS

Scoliosis — a sideways curvature of the spine — is a common complication in neuromuscular disorders. Scoliosis prevents full expansion of the chest and can interfere with breathing.

Scoliosis in these diseases occurs because of weakening of the muscles that normally support the spine. At MDA clinic visits, especially in growing children, the spine will be physically examined for curvature, and the degree of the curve may be measured by X-ray. A curvature may progress surprisingly quickly in a child.

Keep a close eye on a progressing spinal curvature, which may crowd the lungs, and consider spine-straightening surgery on your doctor’s recommendation.

IDENTIFY UNDERVENTILATION

The first consequence of weakened respiratory muscles usually is inadequate breathing during sleep. This underventilation occurs at night because the natural urge to breathe is lower during sleep, and because the abdomen pushes up against the diaphragm when you lie down.

The most common symptoms indicating insufficient breathing (underventilation or hypoventilation) are fatigue, poor sleep, nightmares or night terrors, and headaches, especially right after waking.

For many people with neuromuscular diseases, the beginnings of ventilatory failure come on slowly and may be mistaken for other problems. Shortness of breath — the best-known symptom of too little oxygen — may not occur if skeletal muscle weakness prevents you from exercising.

It’s important to watch for the other signs of possible respiratory failure. These may include anxiety, confusion, loss of appetite and weight loss, weakening of the voice and weak coughing that doesn’t move mucus up toward the mouth.

PROPERLY TREAT SLEEP APNEA

Some studies have shown a high incidence of sleep disorders in people with neuromuscular diseases. But the cause of these problems may be different than for those without muscle diseases, and the treatment is different as well.

Among the general population, common causes of respiratory problems during sleep include pauses in breathing (apnea) either caused by brain abnormalities (called central apnea), or by collapse of the upper airway, blocking breathing (called obstructive apnea).

But for those with neuromuscular diseases, breathing problems during sleep may be caused or complicated by the fact that the muscles that aid respiration — the diaphragm and intercostals — have been weakened by muscle disease.

Weak respiratory muscles can lead to nocturnal hypoventilation (ineffective breathing during sleep) or nocturnal apnea (periodic cessation of breathing during sleep), when gravity, body position and neurological factors naturally make breathing efforts less effective.

A simple method to assess nighttime breathing difficulties is to measure exhaled carbon dioxide in combination with pulse oximetry, which painlessly measures blood oxygen levels through the placement of a small clip on a finger or toe. Typically these tests may be done at home while the patient sleeps.

A more sophisticated assessment tool is a sleep study or polysomnogram (PSG), which pinpoints the causes of disrupted sleep through a combination of measurements, including encephalographic (brain) activity, eye movement, muscle activity, heart rhythm, respiratory effort and others. Polysomnograms are performed in a sleep laboratory and last between seven and 12 hours.

For the general population, continuous positive airway pressure, or CPAP, is the ventilation therapy commonly prescribed for obstructive apneas. CPAP blows in a continuous flow of air at a set pressure, keeping the airway from collapsing and obstructing breathing.

But CPAP often isn’t appropriate for people whose problem is caused by weak respiratory muscles, because the muscles have to work harder to exhale against the constant inward flow of air.

In those cases, bilevel positive airway pressure ventilation, typically called BiPAP, is more commonly used. (BiPAP is a registered trademark of Respironics.) Air is administered at a higher pressure level on inhalation and a lower (or zero) pressure on exhalation.

In muscle diseases, the BiPAP “span,” or difference between the inhalation and exhalation airflow pressures, is typically high to provide greater assistance to the inspiratory muscles and little or no resistance during exhalation.

Because sleep-breathing problems can be an effect of neuromuscular diseases, it’s important to seek help at the first symptoms of underventilation. Be sure to consult an experienced, certified sleep specialist who knows which therapeutic solutions are appropriate for people with neuromuscular diseases.