What is cystic fibrosis?
Cystic fibrosis affects the cells that produce your body’s secretions (body fluids other than blood) such mucus, sweat, saliva and digestive juices (stomach acid). Normally, these secretions are thin and slippery, but in children with cystic fibrosis, a defective gene causes the secretions to become thick and sticky. The thick mucus can clog the lungs and cause breathing problems. Mucus also can create a block in the pancreas (organ in the body) and other parts of the body causing stomach problems and difficulty digesting food.
Cystic fibrosis, a life-threatening disease, can cause severe lung damage and malnutrition (lack of necessary minerals and vitamins from foods). It is not contagious. Each child with cystic fibrosis is affected differently. Some children with cystic fibrosis are in good or even excellent health. Others are so severely limited by the disease that they may need to be hospitalized or cannot attend school regularly.
Exercise is very good for these children, helping to loosen the mucus that clogs the lungs and increasing the ability to breathe deeply. Some children may tire more easily than other children. In hot weather or when exercising, your child should be encouraged to eat salty snacks and drink extra fluids — about 6-12 ounces of fluid every 20-30 minutes. Avoid caffeinated drinks such as colas because they can increase fluid loss (www.cff.org).
Early identification is important in helping your child to maintain good health. The “sweat test” is the one most often used to determine if a child has cystic fibrosis. This simple and painless procedure measures the salt in a child’s sweat. A high salt level indicates cystic fibrosis.
Cystic Fibrosis Foundation, 2005
What are some common signs of cystic fibrosis?
Children with cystic fibrosis can have any of these symptoms:
- Frequent pneumonia.
- and/or greasy, bulky stools.
- Poor weight gain.
- Cough lasting more than a month.
- Shortness of breath.
- Constant upset stomach.
- Very salty-tasting skin, often noticed by parents when they kiss their child.
Keep in mind that symptoms are very different from child to child. There are more than 1,000 different types of the gene that causes cystic fibrosis.
Cystic Fibrosis Foundation, 2005
What can I do to help my child?
You have made an important first step by reading more information on cystic fibrosis. Another step you can take is to talk with other parents who have children with cystic fibrosis. They have gone through what you are going through now and may be able to help. You also can get help from parent support and advocacy groups. To find groups near you please contact Help Me Grow. Research tells us that the more involved you are in your child’s health, the better the results for your child’s future.
Below are some checklists of things to help your child. Remember that you are not alone.
THINGS I CAN DO TO HELP MY CHILD WITH CYSTIC FIBROSIS
Get your child tested for cystic fibrosis: A number of specialists (usually including a pediatric pulmonologist) can help your child and your family with cystic fibrosis. (See the section on professionals for a complete list.) Check with your pediatrician and insurance carrier for a referral to the specialist(s) who can help your child.
IN THE STATE OF FLORIDA:
Contact Children’s Medical Services (CMS):
To help organize medical care for my child:
Children’s Medical Services
Main Phone: (850) 245-4200
Work with my child’s service providers:
A service provider is anyone who works with you and your child, including doctors, nurses, teachers, social workers and therapists. Remember that you know more about your child than anyone else and are the main influence in your child’s life. Here are some tips for speaking with service providers:
- Feel free to ask questions and make comments. Be specific about what you know about your child and what you want and need for your child. Be honest about what you expect, any worries you may have, or about anything you don’t understand.
- If you think your child needs something in particular (such as a nutrition evaluation), keep asking until you get it or until you understand why it is not needed.
- If your child’s cystic fibrosis is severe, you may need to use your school system’s hospital or homebound service to provide an education for your child.
Work with your child’s school:
Your child may need a Section 504 educational plan to make sure your child’s educational and health care needs are met. Section 504 is part of the Rehabilitation Act of 1973. This federal law allows qualified children with chronic illness to have special services or supports in their schools. For more information on Section 504, see this website: http://www.hhs.gov/ocr/504.html
Talk with my child’s teacher often:
Keeping your child’s teacher updated on your child’s medical condition is vital. Let them know what warning signs to look for if your child should have a medical problem while at school. Make sure your school changes your child’s physical education program if necessary.
Make sure your child’s teacher allows your child access to the bathroom and fluids when necessary. Ask the teacher to let your child leave the classroom when necessary for a drink of water (to prevent a coughing fit). Also ask the teacher to try to ignore any coughing that your child can’t prevent. If adults who come in contact with your child accept the coughing as normal, the class likely will follow the lead.
Get more information:
Visit more websites. Read a book. Watch a video on cystic fibrosis. Include your child and family. Talk with another parent. For local support groups please call 2-1-1 and ask for Help Me Grow
I will have my child wear a Medic Alert bracelet:
Thus, in case of an emergency, vital information on your child’s condition is available.
Include my child in all parts of daily life:
Encourage your child to take part in family fun, recreation activities and play that exercises lungs. Laughing, running, chattering are all fun and good.
Encourage my child to cough:
Children with cystic fibrosis must cough and bring up large amounts of mucus. Your child may be embarrassed, however, to cough in front of friends. Assure family and friends that cystic fibrosis is not contagious, that your child can’t give it to another child.
Encourage my child to take medicine on time, every time:
Some children want to take medicine privately, just before eating. Others may not take their medication or “forget” to take their medication rather than take them in front of their friends. Ask your child what feels most comfortable. Then ask the teacher and school to allow that. Have your child keep a daily calendar to monitor the use of medications and fluid intake. This will help foster independence and keep the child on track.
Encourage my child to exercise every day:
Children with cystic fibrosis benefit from exercise because it helps loosen the mucus that clogs the lungs. Exercise lungs daily to handle cystic fibrosis. Your child may tire faster than other children. Ask your child’s teachers to include your child in every possible game and activity.
Help your child to eat a high-calorie, high-salt diet:
Do this in consultation with a registered nutritionist. Most cystic fibrosis centers have nutritionists skilled at helping choose the best foods and drinks. This diet usually includes high-calorie drinks (e.g., Carnation Instant Breakfast®, Scandishakes®, Pediasure®, Boost®, Boost Plus®, Ensure®, Ensure Plus®), vitamin supplements and an increased use of butter, cheeses and peanut butter. Increased salt intake is particularly important in hot weather and when your child is exercising.
Encourage my child to drink a lot of fluids:
Water or sports drinks such as Gatorade should be available to your child during physical activities as well as in the classroom throughout the day. Drinking a lot of fluid is vital to keeping your child healthy.
Be careful about exposing my child to germs and harmful bacteria:
Teach your child about good hand-washing by demonstrating this yourself. Frequent hand-washing with soap or using antibacterial gel helps prevent infection. If there is dirt on a child’s hand, use soap and water. If you can’t see the dirt, use antibacterial hand gel.
Teach your child to wash or using gel on hands after coughing or sneezing and after blowing his or her nose. Your child also should wash or use gel on his or her hands before eating and after going to the bathroom. Before-and after-breathing treatments, before-and after- airway clearance and before taking medicine also are vital times to wash or use gel on hands, both yours and your child’s.
Make sure my child has a trusted adult with whom to talk:
Sometimes children talk more easily with adults who are not their parents. Usually it is because they do not want to worry their parents. Children need an adult with whom to talk freely. A therapist, a psychologist or a teacher often can have a conversation with children that they may not share with a parent.
Do many children have cystic fibrosis?
Cystic fibrosis affects about 30,000 people in the U.S. Most children with cystic fibrosis are diagnosed before the age of 4. Cystic fibrosis is more common among people of European ancestry than among African Americans or Asian Americans. Cystic fibrosis is inherited. Thus, some parents carry a gene that makes it more likely that their children will get the disease. That also means that parents with cystic fibrosis are likely to pass it on to their children.
CF Foundation, 2005
Is there a cure for cystic fibrosis?
There is no cure for cystic fibrosis yet. Research is being done on gene therapy and other ways to cure cystic fibrosis. But there are effective ways to control the disease. In the 1970s, children diagnosed with cystic fibrosis were not expected to live beyond their teens. Today, better treatments allow people with cystic fibrosis to live well into adulthood — and also live fuller, more comfortable lives. These new treatments focus on four things:
- Reducing secretions from the lungs.
- Replacing enzymes missing from the pancreas.
- Reducing or replacing salt and energy loss.
- Treating frequent lung infections.
Fletcher-Janzen & Reynolds, 2003; AAP, Caring for Baby and Young Child, 1999
What does cystic fibrosis mean for the health of my child?
Most children with cystic fibrosis take several different medicines every day. Some are taken by mouth, and some inhaled through a spray. Each medicine is designed to help with one of these goals: (1) to thin mucus in the lungs, (2) to treat lung infections, or (3) to replace pancreatic enzymes (missing chemicals in the pancreas).
Clearing mucus from the lungs is a vital part of daily life for children with cystic fibrosis. The clearing can be done several ways. Most often, the mucus clearing involves chest physical therapy, sometimes called chest PT. Chest physical therapy, a form of airway clearance, involves strong clapping on the back and chest. This clapping is done to loosen the thick mucus from the lungs, allowing the child to cough it up.
Another way is for a child to do special breathing exercises twice a day for about 30 minutes each. An introduction to “airway clearance techniques” can be found at www.cff.org. Respiratory failure (lungs don’t work and breathing is impossible) is the most dangerous risk of cystic fibrosis. Clearing the lungs each day is vital to avoid this problem.
When cystic fibrosis affects the pancreas, your child also must eat a special diet to grow and develop. These children take daily vitamins and eat a high-calorie diet. They also take “pancreatic enzyme supplements” with meals to help their bodies take in the proper level of nutrients. Enzymes help the body digest food. Even with supplements and enzymes, children with cystic fibrosis may still have more than usual pain, gas and/or diarrhea
Cystic Fibrosis Foundation, 2005
What does cystic fibrosis mean for my child’s learning?
Cystic fibrosis does not usually cause problems with learning. But difficulties breathing or having a poor appetite and weight loss can make a difference in how your child feels about learning and being in social situations.
If there are problems with learning, typically accommodations in the regular classroom and in regular physical education classes will be enough. Sometimes students with cystic fibrosis receive special-education services. Services from physical therapy and school nurses can be vital for the student to receive appropriate schooling.
Who are some professionals my child may need to see?
Your child may need to see many different health care specialists. For example:
- Pediatrician: A doctor who specializes in treating children. The word “pediatric” in front of a professional’s title means he or she works with children.
- Geneticist: A doctor specializing in the study of DNA and heredity, and who work directly with parents and families at risk of having diseases and conditions that pass from parents to children through genes.
- Pediatric pulmonologist: A doctor who specializes in treating children with cystic fibrosis and other lung-related conditions.
- Pediatric nutritionist: A registered dietitian who designs special diets for children with cystic fibrosis and other special health needs.
- Pediatric otolaryngologist: A doctor who specializes in treating conditions of the ears, nose and throat in children, and sometimes called, an ear, nose and throat specialist.
- Certified pediatric nurse practitioner: A nurse with advanced training in treating pediatric patients and who may specialize in caring for children with such specific conditions as cystic fibrosis.
- Respiratory therapist: A medical professional who evaluates, treats and cares for patients with breathing or other cardiopulmonary problem (related to the heart and lungs) using such physical techniques as chest physical therapy, sometimes called chest PT. He or she works under the direction of your pediatric pulmonologist.
- Social worker and counselor: A professional who provides counseling and emotional support for the child and family, and may help coordinate services, too.
- Care coordinator: An individual responsible for organizing the details across agency lines and serving as your contact to help you and your family get services and assistance.
What are some websites where I can read more about cystic fibrosis?
Cystic Fibrosis Foundation http://www.cff.org Offers good, basic information about cystic fibrosis.
CysticFibrosis.com http://cysticfibrosis.com/ is a great resource for cystic fibrosis information
Cystic Fibrosis Research, Inc. http://www.cfri.org Independent, non-profit volunteer organization dedicated to saving lives of children and adults with cystic fibrosis.
Bandaids and Blackboard http://www.lehman.cuny.edu/faculty/jfleitas/bandaides/sitemap.htmll A great site for kids, teens and parents of kids with medical challenges.
NIH MedlinePlus: Cystic Fibrosis www.nlm.nih.gov/medlineplus/cysticfibrosis.html A comprehensive source of links providing news, overviews, diagnosing, symptoms, treatments, clinical trials and related issues pertaining to cystic fibrosis. This is a service of the U.S. National Library of Medicine and the National Institutes of Health.
What are some books I may want to read with my child?
Living With Cystic Fibrosis (Living Well Chronic Conditions) by Susan H. Gray. Introduces readers to cystic fibrosis, its causes, how it affects the body, and what it’s like to live with the condition. (Ages 4-8)
Taking Cystic Fibrosis to School by Cynthia S. Henry, Tom Dineen (illustrator). This book educates the reader about cystic fibrosis and helps children deal with classmates or friends with cystic fibrosis. (Ages 4 to 8)