Most people don’t think about swallowing during meals or as they go about their daily lives, but many people with ALS do.
Weakness and a lack of coordination of the tongue and throat muscles can result in swallowing difficulty (dysphagia), causing choking on foods or liquids, drooling, the sensation that there’s excess saliva in the mouth or phlegm in the back of the throat, and longer mealtimes.
Aspiration, in which foods, liquids or body secretions are routed down the trachea instead of the esophagus, is of particular concern when swallowing problems occur, especially when those difficulties are compounded by a weak cough. Aspiration introduces bacteria to the lungs and may lead to life-threatening infection and pneumonia.
Although aspiration poses serious risks to people with ALS, most tend to focus on excessive saliva as their major complaint, says Laurie Sterling, speech pathologist at the MDA/ALS Clinic at Methodist Neurological Institute in Houston. Drooling can be embarrassing, and it’s scary to cough and choke on your own secretions.
“The concern seems to be about the amount of saliva itself and difficulty ‘breathing through it,’ or coughing on it,” Sterling explains, adding that most aren’t concerned about aspiration or its causes, “perhaps because folks don’t completely grasp the concept of aspiration or realize that you can aspirate your secretions.”
The starting lineup
Several simple and straightforward techniques exist for reducing the risk of aspiration. Sterling suggests:
- tucking your chin down when swallowing to protect your airway;
- thickening liquids to make them easier to control (commercial thickeners, baby cereal, gelatin, cornstarch and instant potato flakes are several options);
- avoiding mixed consistencies (such as chicken noodle soup) that are difficult to manage with tongue weakness;
- taking pills whole with something like pudding rather than water, or crushing them (except for time-release tablets); and
- elevating the head of the bed at least 30 degrees to prevent reflux.
A swallowing evaluation done by a speech pathologist familiar with ALS can identify the specific strategies most helpful for you.
|In a two-step laryngeal diversion procedure, the larynx (above the trachea) is cut in two. The upper part is sewn to the esophagus, routing everything from the mouth down to the stomach and eliminating the risk of aspiration. The bottom part is attached to a tracheostomy tube, through which air is routed to the lungs.
Another factor in aspiration risk is oral health. Aspiration of secretions high in bacteria increases the risk of developing pneumonia, so for someone with ALS, keeping the teeth and tongue clean is as important as ever. (Tip: avoid toothpastes, gels or mouthwashes high in sugar or alcohol, as they may encourage production of saliva.)
"I don't think I can stress enough the importance of good oral hygiene, even if you aren’t eating by mouth any longer," Sterling says. "There are a lot of bacteria in the mouth, even more so if oral hygiene is not maintained.”
Covering the bases
Other more complex means of controlling secretions include steam and vibrating vests, in-exsufflator (cough assist) devices and suction machines, and medication.
Steam and vibrating vests both help break up mucus in the lungs, while the latter can be effective in moving secretions from the lungs to the upper airways. The in-exsufflator device helps clear secretions from the respiratory tract by strengthening the force of an individual’s cough through the use of positive pressure on inhalation and negative pressure on exhalation. Suction machines then can be used to suck out any remaining secretions in the mouth or throat.
Anticholinergic drugs such as atropine sulfate (Sal-Tropine), amitriptyline (Elavil) and glycopyrrolate (Robinul) help decrease saliva production, as do scopolamine patches (Transderm Scop), which usually are prescribed for motion sickness and worn on the skin. These drugs block action of the neurotransmitter acetylcholine, responsible (among other things) for transmitting nerve impulses that increase bodily secretions. They produce typically mild side effects such as sore throat (from decreased mucus production), decreased perspiration and less frequent urination, but their effectiveness varies from person to person.
Also effective at reducing salivation are botulinum (Botox) injections to the parotid gland, the largest of the salivary glands. The botulinum toxin works by suppressing the release of acetylcholine. It’s possible to achieve the same outcome by inducing scarring of the glands through ligation (cutting) or irradiation; however, these methods are permanent and tend to be performed much less frequently.
A “common and appropriate” treatment to prevent aspiration is insertion of a percutaneous endoscopic gastrostomy tube (PEG, or feeding tube), says Stanley H. Appel, director of the MDA/ALS Center at the Methodist Neurological Institute in Houston. He recommends people with ALS consider getting a gastrostomy early in the progression of the disease to help decrease aspiration risks.
The feeding tube ensures nutrition requirements are met while eliminating aspiration risks associated with chewing, swallowing and eating food. However, feeding tubes don’t prevent aspirating saliva, mucus or stomach contents. When all other options have been exhausted, a surgical procedure may be the method of choice for reducing the risk of aspiration to zero.
In a procedure called a laryngeal diversion, a surgeon cuts the trachea in two. The bottom section is bent and brought out the front of the neck for a tracheostomy; the top section is either sewn off (a “one-step” procedure) or sewn side-on-end onto the esophagus (the “two-step” procedure). With the two-step procedure, anything in the mouth will be routed down the esophagus to the stomach.
“The laryngeal diversion, especially if you do the full [two-step] procedure, completely eliminates the risk for aspiration,” says physical medicine and rehabilitation specialist Greg Carter, co-director of the MDA/ALS Center at the University of Washington at Seattle.
The way in which the procedure is done determines whether or not it is reversible, but it, as all surgeries in people with ALS, poses some risk of exacerbating weakness or causing secondary infections. Also, those who have the procedure done will be unable to communicate via voice or sound, as no air will pass over their vocal chords.
Carter says he rarely recommends laryngeal diversion because he rarely recommends tracheostomy. “Improvements in noninvasive ventilation (NIV) over the past decade or so have been so great that I just find that most people don’t need a tracheostomy,” he says, noting however that tracheostomy is appropriate in cases of severe bulbar involvement, where too much upper airway resistance makes NIV ineffective.
Opinions differ on laryngeal diversion. Appel says the procedure is “too aggressive to handle the problem of aspiration,” and has never recommended it, “because the problem can best be handled by the combination of gastrostomy and noninvasive ventilation.”
Carter, however, suggests anyone with ALS who opts for a tracheostomy get the laryngeal diversion procedure done at the same time. Tracheostomy alone, he says, actually increases the risk of aspiration because the tracheostomy tube promotes secretion production and interferes with the normal swallowing mechanism.