Inside the lungs are lots of tiny tubes, the bronchi, down which air passes to reach specialized air sacs, the alveoli; here, oxygen enters the bloodstream and carbon dioxide leaves the blood to be exhaled. CF children have normal lungs at birth, but the mucus produced in them is abnormally thick, so it blocks some of the similar airways and leads to infection and, later, to lung damage.
Treatment The aim of treatment is to keep the lungs as normal as possible, and there are two components to this:
- Clearing the sticky mucus from the airways with physiotherapy, breathing exercises, physical exercises, physical exercise, and medication.
- Prevention and prompt treatment of chest infections, usually with antibiotics.
WHAT PARENTS CAN DO: Even with pancreatin, a child with CF may not absorb all the nourishment she needs in order to grow normally. The child will therefore need more calories, so high-energy snacks between meals, such as milkshakes, are helpful. Monitor the child’s growth to ensure she is gaining height and weight as she should.
The only way to learn how to clear the thick mucus from the child’s chest is from a physiotherapist and with lots of practice, so parents should not be afraid to ask for help. Physiotherapy should be started at the time of the diagnoses, and it’s important to get into a routine early on. It will need to be done twice a day when the child is well and more often when she has a chest infection.
Parents should work very closely with a doctor on the prevention and treatment of chest infections. Should an infection occur, the child will need extra physiotherapy and antibiotics.
WHEN TO SEE THE DOCTOR: Your CF child is very vulnerable to chest infections, so it’s important to seek medical help promptly, either from the child’s doctor or hospital clinic, if there is a suspicion that something is wrong. The following symptoms may indicate that a doctor’s visit is needed.
- Decreased or poor appetite
- Weight loss
- Stomach aches
- Frequent or loose stools
- Increased or frequent cough
- Increased sputum
- Change in the color of sputum
- Unwillingness to exercise
- Cold symptoms
Physiotherapy: To clear the thick mucus from the child’s chest, use physiotherapy twice a day, and more often during chest infections.
Immunization and CF
Babies with CF are particularly at risk from the common childhood infectious diseases, especially those that may affect the lungs.
A child with CF must stick rigidly to the normal immunization schedule, and injections should be postponed only in very exceptional circumstances and after consultation with the child’s doctor. Having a cold or a cough is not sufficient reason to delay immunization.
Blocked airways: Thick mucus blocks the airways, starving them of oxygen and ultimately causing parts of the lung to collapse.