Breakthrough - Osteogenesis Imperfecta Foundation

Q&
A
Respiratory
Concerns
Answers to Members’ Questions
Respiratory Concerns
for Children & Adults
This article is based on information from the fact sheet, OI Issues:
Maintaining Health During the Adult Years, and book, Osteogenesis
Imperfecta: A Guide for Nurses, both published by the OI Foundation.
What is the repiratory system?
The respiratory system’s job is to bring oxygen into the
body and remove carbon dioxide, the waste product of
breathing, from the body. The respiratory system is made up
of two parts: the upper respiratory tract includes the nose,
ears, sinuses, mouth and the structures found in the throat,
and the lower respiratory tract includes the trachea, bronchial
tubes and the lungs. The ribs and diaphragm assist the
lungs as they expand and contract with each breath. In the
lungs, impurities from the air are filtered out and the oxygen
is transferred to the blood system. Oxygen is the fuel that
all cells and all organs in the human body need to function.
Getting enough oxygen is important for good health.
What are the common causes of upper respiratory
problems?
• Viral infections such as colds and flu.
• Bacterial infections such as bronchitis and pneumonia.
• Allergies.
Who should be concerned about respiratory
function?
Respiratory health is a concern for children and adults
with OI who:
• Are short in stature.
• Have scoliosis, kyphosis or both (kyphoscoliosis).
• Have chest or rib cage deformities such as pectus
carinatum or pectus excavatum.
• Lead a sedentary life style.
As a rule, the more severe the OI, the more susceptible
the person will be to lung or breathing problems.
Why does OI affect respiration?
Deformities of the spine and ribs can limit the space
available for the lungs to expand. This decreases the
amount of air in the lungs, which means that less oxygen is
available to the body and that the lungs cannot get rid of all
of the carbon dioxide. Pulmonary (lung) complications can
occur due to rib fractures, muscle weakness of the chest
wall, heart valve disorders, chronic bronchitis and asthma.
What problems will this cause?
Breathing problems can lead to shortness of breath,
tiredness, insomnia, migraine headaches, sleep apnea, and
can make the person more susceptible to infections such as
pneumonia. Restrictive pulmonary disorder, a reduction of
lung capacity, is common in people with severe OI or any
one who has decreased chest volume, chronic bronchitis or
asthma. Respiratory complications leading to pneumonia or
heart failure represent a significant cause of death for
people with Type II or Type III OI.
What can be done to prevent a serious
respiratory problem?
There are four things that every person with OI can do to
protect themselves from serious respiratory problems.
1. Treat all respiratory infections, even colds and
coughs, immediately.
2. Seek prompt treatment for any difficulty breathing.
3. Have your respiratory function tested. Adults
with Type III or Type IV OI, or spine curvature,
should see a pulmonologist every 1-2 years.
4. Exercise to promote deep breathing. Talk with
your doctor about how to do this safely.
What else can be done?
• Children and adults should have any spine curvature
monitored. In some cases surgery may be necessary.
• Discuss with your doctor how you can exercise safely
to develop muscle strength and lung capacity.
• Talk with your doctor about whether you are a candidate
for the flu shot or pneumonia vaccine.
• Do not smoke, and avoid exposure to second-hand
smoke.
• Use of supplemental oxygen and use of BiPAP, a
positive pressure breathing device, can help manage
pulmonary function.
• Asthma inhalers during the day are sometimes beneficial.
People with chronic asthma may need additional
medications.
Breakthrough Winter 2004 Page 6