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Swallowing is an important part of our daily lives. Swallowing
transports water and food from our mouth to our stomach to
provide nutrition and hydration. Since eating is often a social
event it also is a part of family and community life. When
swallowing is impaired our physical and social lives are threatened.
Any disease which alters the nerves or muscles which are involved
with swallowing produces swallowing difficulty. This swallowing
difficulty is called dysphagia by the medical community. There
is another medical name that is similar to dysphagia. Dysphasia
means difficulty with language, not with swallowing. The Speech
Pathologist treats both dysphagia and dysphasia.
Many subjects with neuromuscular disease develop dysphagia.
In a European survey (1) significant swallowing problems were
reported in 51% of individuals with polymyositis, in 44% with
myasthenia gravis, in 38% with limb-girdle syndrome, in 33%
of those with myotonic muscular dystrophy, in 32% with spinal
muscular atrophy, in 20% with Duchenne muscular dystrophy,
and in 6% of those with facioscapulohumeral dystrophy. Although
the percentage with amyotrophic lateral sclerosis was not
included in the European survey, the percentage of swallowing
disorders in amyotrophic lateral sclerosis approaches 100%
as the disorder progresses (dysphagia may also be the first
symptom of the disease).
Food is prepared in the oral cavity by chewing, then is transported
to the stomach where the process of digestion is begun. Swallowing
is initiated by a tongue thrust against the palate at the
top of the mouth. This propels the food into the pharynx.
The muscles which move the pharynx coordinate with the muscles
that raise the larynx (the voice box) to propel the food through
the pharynx into a long tube called the esophagus. This takes
one second. The esophagus carries the food into the stomach
within 12 to 16 seconds.
In the middle part of pharynx there is a point which is like
a fork in a road. Food passes into the fork leading to the
stomach. Air for oxygen exchange passes into the fork called
the trachea which leads to the lungs . We do not want food
to pass into the lungs where it leads to pneumonia (called
aspiration pneumonia) or gets stuck in trachea where it produces
complete airway obstruction and death. All families should
know how to do the Heimlich maneuver which is a technique
to move a food particle out from the trachea if it gets stuck
there since all people are at risk for aspiration obstruction
if they talk or laugh while swallowing. Families with members
who have a neuromuscular disease should also know the Heimlich
maneuver since they are at greater risk. The Heimlich maneuver
is taught with any course in cardiopulmonary resuscitation
(CPR).
Problems with swallowing are frequently evaluated and treated
by Speech Pathologists. The initial evaluation of swallowing
problems may be an examination by the Speech Pathologist.
Another method of assisting in determining the extent of the
problem and the methods for treatment is called a dynamic
swallow study (2). In that study a videotape is made of the
swallow of barium. Through sophisticated electronic and computer
technology multiple measurements can be made which lead to
development of treatment techniques that are specific for
that person. At UCDMC the videotape is reviewed by a multi-disciplinary
panel that includes Speech Pathologists, Nurse Clinicians,
and physicians.These health care professionals give suggestions
and advice about treatment. These suggestions are included
in a comprehensive report which goes to the personal physician
of the individual with the swallowing problem.
The kinds of treatment which may be useful for swallowing
problems in neuromuscular disease include:
1) Changes in the type and consistency of the food. Sometimes
pureed foods are better. Sometimes thickened foods are better.
2) Changes in head position such as turning to the right or
left may be beneficial. This is tested during the dynamic
swallow study videotaping.
3) At times it is not safe to swallow (such as when the dynamic
swallow study shows that food is aspirated). When food is
aspirated it falls into the airway where it can produce pneumonia
or death. If aspiration is present it may be necessary to
get food into the stomach by a tube, either a nasogastric
tube through the nose or a gastostomy where the food is given
via a tube which has been placed in the stomach through the
skin.
4) Surgery may be helpful in certain types of problems in
which a muscle that acts as a gate between the pharynx and
the esophagus blocks the passage. This is called a cricopharyngeal
bar. The surgery is called a cricopharyngeal myotomy. This
surgery opens up the passageway to allow more normal passage
of food.
Certain neuromuscular diseases are more susceptible to dysphagia
(the medical name for a swallowing disorder). Individuals
with oculopharyngeal muscular dystrophy frequently have a
cricopharyngeal bar and require a cricospharyngeal myotomy.
This is also sometimes true for individuals with limb-girdle
syndrome. Some limb-girdle individuals have mild or no swallowing
problems; some have severe problems. Individuals with polymyositis
and dermatomyositis have swallowing problems that are severe
and occur early in the disease process. Sometimes the first
and only clinical symptom in polymyositis or dermatomyositis
is dysphagia.. Individuals with polymyositis and dermatomyositis
may respond to oral corticosteroid medications. Individuals
with myasthenia gravis also have swallowing problems early
that produce dysphagia. Swallowing therapies have not
been developed for this neuromuscular junction disorder, however,
because the treatment for myasthenia muscle weakness is surgical
(thymus excision) or pharmacologic (neostigmine or other anticholinesterase
medications). These treatments usually produce a distinct
improvement in muscle strength, including the muscles for
chewing and swallowing, which leads to improved swallowing
and does not usually require remedial dysphagia treatment.
Most individuals with neuromuscular disease have a slowly
progressive problem where the swallowing disorder comes only
after several years. The swallowing problems in myotonic muscular
dystrophy are usually mild to moderate and come later in the
disease process. A third of the individuals with myotonic
muscular dystrophy or spinal muscular atrophy have swallowing
problems; however, the problem in spinal muscular atrophy
is usually more severe. Individuals with facioscapulohumeral
syndrome have a low incidence of dysphagia (less than 10 %),
but for the ones with swallowing problems evaluation and treatment
are important.
Amyotrophic lateral sclerosis produces marked swallowing and
nutritional problems since the motor neurons in the brain
stem that control swallowing deteriorate. It is important
for individuals who have amyotrophic lateral sclerosis and
their families to have access to a Speech Pathologist with
experience with the disorder to assist in maintaining hydration
and nutrition.
The Department of Otolaryngology and Department of Physical
Medicine and Rehabilitation at UCDMC have completed dynamic
swallow videotape studies on over seventy individuals with
neuromuscular disease. Those studies are currently being measured
with sophisticated computer technology. The work is being
supported by a Research and Training Grant from the National
Institute for Disability Rehabilitation Research. The goals
of that study are a) to understand the neuromuscular dynamics
of swallowing in individuals with neuromuscular disease b)
to develop effective remedial treatment techniques for dysphagia
in individuals with neuromuscular disease c) to develop a
handbook for treatment of swallowing disorders in neuromuscular
disease d) to provide individual evaluations and treatment
programs through consultation from physicians.
Many of the swallowing problems in neuromuscular disease are
mild and can be dealt with by the individual through care
and caution while swallowing and by care with the type and
consistency of food. However, some require formal work-up
and evaluation. The reasons to obtain a formal work-up are:
If you have any of these problems you should discuss it with
the physician who provides your care to see if there is need
for a dysphagia evaluation.
And remember, all families should be trained in the Heimlich
maneuver in order to provide resuscitation if a family member
chokes on something that blocks the airway. This is especially
important in families where there is an individual with neuromuscular
disease since there is an increased risk of aspiration of
food and death from that aspiration. If you need training
in the Heimlich maneuver contact your local chapter of the
American Red Cross or the American Heart Association.
References:
1) Willig T, Paulus J, Lacau Saint Guilly J, Beon C, Navarro
J. Swallowing problems in neuromuscular disease. Archives
of Physical Medicine and Rehabilitation 1994; 75: 1175-81.
2) Johnson E, McKenzie S. Kinematic pharyngeal transit times
in myopathy. Dysphagia 1993; 8: 35-40.
From the RRTC Newsletter, February 1997.
