In mid-March, Mark Wenzel, who has ALS, and his wife, Martha, were visiting friends when a mucus plug blocked Mark’s airway.
|Mucus plugs became a problem for Mark Wenzel when his cough reflex diminished and he lost the strength to produce a forceful cough.
Something that most would simply cough away left Wenzel, 55, unable to breathe, and — due to a “perfect storm” of circumstances — unable even to signal for help. The result was a wave of panic as the minutes ticked by.
Because mucus plugs represent a very real danger, it’s imperative that individuals with ALS and their caregivers learn how to prevent and cope with them.
Wenzel, who lives in Phoenix, received an ALS diagnosis in September 2002, and his cough reflex began to wane about a year later. In 2004 he opted for a tracheostomy.
Wenzel uses a pulse oximeter (a small device that clips onto a finger or toe) to monitor his blood oxygen saturation, and a Ballard closed suction system for suctioning secretions from his airway and maintaining ventilation.
But that day in March, Wenzel had left his oximeter at home. He was sitting with Martha and their friends on a patio, with a waterfall and pool nearby that masked the sounds made when the mucus began to obstruct his airway.
“It was a sunny afternoon, so I had sunglasses on, which hid the look of panic in my eyes from my friends sitting beside me,” he says via e-mail, which he operates by an eye-control system. “Fortunately for me, my wife glanced at me from where she was sitting and saw my gray color, and quickly moved to suction my airway. It was a close thing.”
Generally, Mark is on the pulse oximeter day and night, says Martha. But the device’s internal battery doesn’t hold a charge very long, so the Wenzels don’t take it with them when they leave the house, leaving Mark “more vulnerable when we are out.”
Additionally, Wenzel says, he was around “people who were unfamiliar with how quickly a serious problem could develop.”
Excess mucus and inadequate coughing
Mucus plugging occurs when bronchial secretions accumulate to the point that they obstruct airflow. If the cough is weak and the plug cannot be cleared, the situation can be deadly.
Normally, the moist lining of the lungs produces small amounts of clear mucus, which trap dirt and bacteria from the air. This mucus is slowly swept out of the lungs by tiny hairs called cilia, and is cleared from the body by the forceful expulsion of air from the lungs — a cough.
In ALS, however, several factors reduce cough effectiveness:
- Weak muscles in the abdomen make it hard to cough forcefully.
- Weak muscles in the throat impair closing the glottis, or top of the throat, in order to build up pressure inside the chest for a cough.
- Weak diaphragm muscles, or a chest wall that’s stiff from underuse, make it difficult to take a good deep breath to initiate a forceful cough.
- The result of a weak cough can be the buildup of dangerous quantities of mucus, creating a life-threatening medical situation.
Trachs add to risk
Tracheostomy tubes increase the likelihood of mucus plug formation, says John Bach, a physical medicine and rehabilitation specialist at University Hospital in Newark, N.J., and co-director of the MDA clinic there.
“Mucus plugs affect anyone with very weak throat muscles,” he says, “but tracheostomy tubes cause mucus plugs even when throat muscles are not weak.”
This is because the tracheostomy tube often stimulates increased secretion production. A trach also bypasses the natural defense systems that filter and humidify the upper airway.
In addition, lack of airflow over the larynx can lead to reduced sensation in that area and decreased reflexes to cough or clear the throat.
In the clear
The only treatment for a mucus plug is to somehow get it out.
One mucus-removal method is to produce a cough, either with a manual assist from a caregiver or through the use of a cough machine.
An abdominal thrust is a way to manually assist a cough. The person with ALS takes a deep breath and holds it. The caregiver places a hand on the person’s abdomen and thrusts in at the same time as the person coughs. If the person can take in enough air at the outset, this maneuver supplies the extra force needed to clear the airway. (Note, ask your MDA clinic for a demonstration of this maneuver.)
A cough machine, such as the In-Exsufflator distributed by Respironics, first delivers an inflow of air, and then rapidly reverses the pressure to produce a cough. Adapters can be purchased that allow these machines to be used with tracheostomies.
Another way to remove mucus is to suck it out. Suction machines use a wand or catheter to capture secretions from the mouth or throat.
“It can be difficult sometimes to get the plug high enough to reach with the suction catheter,” Mark notes. “When this happens, we’ve found that tilting the chair back is helpful or, sometimes, going to bed to get horizontal is helpful.”
Pulse ox to the rescue
Bach says he always prescribes pulse oximeters for people with weak coughs and ventilation problems. The device can be programmed to issue an alarm when oxygen saturation drops below 95 percent. This allows a growing mucus plug or other respiratory problem to be handled before it becomes life-threatening.
“Normal is 95 percent or greater oxygen saturation,” Bach says. “If it falls below 95 percent, it means either hypoventilation or airway mucus plugging,” and failure to take care of the latter can lead to pneumonia, lung collapse, and respiratory failure.
|A pulse oximeter painlessly measures a person’s blood oxygen saturation. It can alert a user to airway mucus plugging or other respiratory problems.
In the beginning, Martha Wenzel says, she and Mark used the oximeter primarily at night. If Mark’s oxygen levels drop below a pre-set level or his pulse rate becomes too fast, an audible alarm alerts Martha to the problem.
“The audible alarm is what makes it so helpful,” Martha says, adding that Mark has had many close calls, some due to mucus plugs, and “the pulse ox has saved him many times.”
Other prevention strategies
When a trach tube is in place, using a humidifier can lessen the number and severity of mucus plugs by partially substituting for the humidifying function of the bypassed upper airway.
Wenzel uses a humidifier attached directly to his ventilator to warm and moisten the air coming through the trach tube.
Thinning the mucus offers another route for helping to prevent a plug. The first step is to keep well hydrated. From there, some physicians recommend over-the-counter expectorants such as guaifenesin (i.e., Mucinex), which thin out secretions and make them easier to cough up. Papaya, pineapple or lemon juice in water also can thin mucus. Surfactants, or wetting agents such as saline, loosen up mucus and help the lungs inflate more easily. Ice cream lovers beware: Some people find that dairy products seem to thicken their mucus.
Although there’s nothing that can definitively prevent a mucus plug, knowing what to expect and being prepared for the eventuality can turn a potentially life-threatening situation into one you can handle.