patient primer
By Lauren Constance Everingham
Care for Neuromuscular Disease
A pulmonologist will perform pulmonary
function tests to determine the best plan of care
for these respiratory difficulties. You will breathe
into a machine called a spirometer that analyzes
your lung function. Your vital capacity, which
is the total amount of air that you can expire
after a complete inspiration, should be measured
while you’re lying down to simulate sleep.
If your vital capacity is too low, you may
need to learn breath stacking. A bag valve mask
or ventilator will force air into your lungs to
help you stack breaths. You will take a breath
and hold it, then take a second breath and try
to hold that. Then you’ll exhale the air into the
spirometer, which determines the maximum
volume of air your lungs can hold. The larger
the volume is, the better you’re able to cough.
A sample of blood drawn from your artery,
called an arterial blood gas test, evaluates the
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Therapy options
Deep breathing and coughing are the most
important respiratory exercises for a person with
neuromuscular disease. Cough therapy can be
performed with the aid of a device that strengthens your coughing mechanism. An intrapulmonary percussionater delivers aerosolized medicine
while it loosens mucus from your airway walls.
You will breathe through the tubing for approximately 20 minutes three to four times a day
until the mucus is cleared.
Another type of machine
imitates a cough by pushing
a certain amount of air into
the lungs and quickly pulling out the same amount of
air from the lungs. This small
electrical machine helps to
expel mucus.
A ventilator can help
when your ability to breathe
has been so compromised
that you can’t get enough oxygen into your
lungs. A noninvasive positive pressure ventilator
that actively assists inspiration often is the first
choice for patients with respiratory insufficiency
due to neuromuscular weakness.
Aerosol therapy may be necessary to combat
infection, and in rare cases, to help muscles
in the airway contract and produce mucus.
Antibiotics, anti-inflammatories and bronchodilators are delivered by a nebulizer or
metered dose inhaler. ■
Editor’s note: Information adapted from the
Acid Maltese Deficiency Association and the
Arizona Respiratory Center Web site.
Lauren Constance Everingham is editorial
a­ ssistant of ADVANCE. She can be reached at
[email protected].
ADVANCE for Managers of Respiratory Care May 2006
www. advance web.com
✃
Testing your lungs
ability of your lungs to move oxygen into the
blood and to remove carbon dioxide from the
blood. Another device that measures oxygen
content of blood is a pulse oximeter that uses a
small clip on your ear or finger. Together, these
results help to track your progress over time.
Your doctor or therapist has given
you this patient education handout to
further explain or remind you about
an issue related to your health. This
handout is a general guide only. If you
have specific questions, discuss them
with your doctor or therapist.
Tom Whalen
W
e need strong muscles to lift,
jump, run and swim, but
most people take for granted
the muscle strength needed
for breathing. Neuromuscular disease weakens
the muscles surrounding the lungs so the body
can’t move air effectively in and out of the
lungs’ airspace.
One of the first signs of poor ventilation due
to neuromuscular weakness is disturbed sleep.
Your body has the lowest natural urge to breathe
during sleep, and when you’re lying down, your
abdominal organs push against your diaphragm,
the body’s large breathing muscle. Examples of
sleep problems are vivid dreams and nightmares,
headaches after waking and daytime fatigue.
Eventually, poor ventilation can cause anxiety, confusion, loss of appetite and weight loss. You’re
voice may get weaker, and as
respiratory muscles continue
to lose strength, you won’t
be able to cough up mucus
effectively. Ineffective cough
becomes especially dangerous if you get a respiratory
infection.
notes