What is cystic fibrosis?
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Cystic fibrosis (CF) is an inherited condition that causes mucus and secretions to become thick and sticky. These secretions can then block the lungs, gastrointestinal tract, sinuses, and other parts of the body.
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Children with CF are prone to getting recurrent pneumonias and to having digestive problems because of the mucus blockage of the lungs and digestive system.
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As with most conditions, some children are more severely affected than others.
How common is it?
There are currently about 30,000 children and young adults with CF in the United States.
What are some characteristics of children with cystic fibrosis?
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These children may not appear ill at all, or they may be thin or small.
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They may cough frequently and produce thick mucus. This cough is not contagious like the cough associated with a cold or flu; it is just the body’s way of trying to clear mucus.
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Children with CF may have fingertips that are rounded or have a blue tinge.
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Children with CF may require medications and lung treatments, but these medications will not affect attention or ability to learn.
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Some children with CF may have bulky stools or diarrhea and may pass gas more frequently.
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Their skin may taste salty.
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Children with CF need to take pancreatic enzymes before eating and they may need extra vitamins.
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Many places have specialized CF centers; children should get care from a specialist in this disease as well as from their primary care provider.
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Ask the child’s parents/guardians who is most involved in their child’s care.
Who is the treatment team?
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Children with CF are often cared for in specialized CF centers in addition to their medical home. These centers will often include pediatric pulmonologists, respiratory therapists, nutritionists, and social workers with specific expertise in CF. Other specialists may include gastroenterologists.
What adaptations may be needed?
As always, the Care Plan should be updated after hospitalizations, emergency visits, child absences for illness, and changes in medications.
Medications
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Children with CF are often treated with oral and inhaled antibiotics.
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Nebulized treatments with medications like albuterol may be given to open the airways.
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Other breathing treatments to loosen the mucus are also sometimes given.
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Pancreatic enzymes
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Store at room temperature in a safe location.
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Consider allowing older children to carry their own day’s supply of enzymes, so they do not have to spend most of their mealtime waiting to get medications instead of eating.
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As always, check the expiration date and develop a system to get new supplies as needed.
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Special vaccines may be necessary, and as for all children, but especially important for those with CF, an annual flu shot is recommended.
Dietary considerations
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Children with CF should have increased fluids.
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Most of us try to avoid salt and fat, but children with CF may need a little extra salt or fat or to have increased calories.
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Children with CF need to take enzymes before eating. For young children, the enzymes should be opened and sprinkled on a small amount of food.
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Older children will probably swallow capsules directly.
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Ask parents/guardians what foods are best to mix with the enzymes (applesauce is commonly used).
Physical environment
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Special equipment—some children use a special vest for chest physical therapy.
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Caregivers/teachers may want to schedule a visit with parents/guardians to review the specifics of the child’s condition. The child may want to make a short visit before coming for a full day, especially after hospitalizations.
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Open bathroom access is important.
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Children with CF should clean their hands frequently and staff should be extra careful about cleaning toys and other children’s hands.
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Chest physical therapy helps to clear the mucus out of the airway; children with CF may need chest physical therapy while at child care.
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Children with CF should avoid sharing beverages or foods with other children and other activities that increase the chance of sharing infections because a common cold can turn into pneumonia more easily.
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Absences may occur more often because of hospitalizations and the need to closely monitor minor respiratory illnesses. Help children with CF keep updated with their schoolwork and projects.
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Encourage exercise.
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Children with CF can do almost anything, but may need rest if they tire more easily.
What should be considered an emergency?
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Call emergency medical services/911 for
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Respiratory distress
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Sudden chest pain
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Significant blood in sputum
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Call parents/guardians for
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Fever
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Change in sputum color or blood streaking
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Increased fatigue
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What types of training or policies are advised?
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Recognizing respiratory emergencies
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Background about CF
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Medication administration
What are some resources?
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Cystic Fibrosis Foundation,www.cff.org, 800/344-4823.
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American Lung Association,www.lungusa.org.
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Local CF centers may be able to hold training sessions or provide materials.
Source: Managing Chronic Health Needs in Child Care and Schools: A Quick Reference Guide.
Products are mentioned for informational purposes only. Inclusion in this publication does not imply endorsement by the American Academy of Pediatrics.
Listing of resources does not imply an endorsement by the American Academy of Pediatrics (AAP). The AAP is not responsible for the content of external resources. Information was current at the time of publication.
The information contained in this publication should not be used as a substitute for the medical care and advice of your pediatrician. There may be variations in treatment that your pediatrician may recommend based on individual facts and circumstances.