When a child receives a cystic fibrosis diagnosis, often, a parent’s first reaction is to ask if there is a cure. Second, parents usually ask what is required of parents who must care for a child with cystic fibrosis. Read on for some information about cystic fibrosis as well as some tips regarding caring for a loved one with cystic fibrosis.
What Is Cystic Fibrosis?
Cystic fibrosis is a heritable genetic condition that limits lung function by causing frequent, persistent infections, and limiting the person’s ability to breathe. Specifically, a gene mutation causes a protein dysfunction, resulting in an inability to transport chloride to the surface. Since the chloride isn’t present to attract water to the surface of the skin, the water stays in the lungs and becomes thick mucus, obstructing the airways and providing a trap for bacteria, viruses and other intruders.
While more than half of the population that has cystic fibrosis is over 18, nearly 75% of cases are diagnosed in children age 2 or younger. Although the presence of cystic fibrosis is indicated by a sweat test, genetic testing can confirm the presence of cystic fibrosis. In fact, the American College of Obstetricians and Gynecologists recommend carrier screening for cystic fibrosis and other conditions to be performed on women considering becoming pregnant. In this way, carrier testing can detect those who may carry the gene mutation for cystic fibrosis to screen during pregnancy and prepare to offer early intervention for babies born with cystic fibrosis.
How Can You Support a Child with Cystic Fibrosis?
Although individual experiences with cystic fibrosis vary as widely as the children who have the condition, children with cystic fibrosis often experience the following treatments daily:
Airway clearance techniques, such as coughing, chest physical therapy, and vibrations. Younger children often need additional help with these techniques.
Administration of medicines for airway clearing and antibiotics, usually inhaled through a nebulizer
Administration of pancreatic enzyme supplements to improve the absorption of nutrients
Following an individual fitness plan
Certainly, the frequency with which children with cystic fibrosis must take medications, participate in airway clearance techniques that may be physically uncomfortable, and sit relatively still while breathing through a nebulizer makes administering cystic fibrosis treatment difficult. In fact, a study revealed that children ages 6 to 14 participated in treatments only 54% to 75% as often as they should.
So, what helps families cope? Routine could help, researchers say. If a family can build a regular routine in which the treatments function as a normal part of the family’s day, the child feels supported instead of isolated during treatment. Particularly, administer treatment in an area of the home where the child feels included, and can participate in a family discussion or otherwise spend time with loving parents.
A child with cystic fibrosis must also interact frequently with members of a care team, and parents can use the support of the care team to help build routine and incentive at home. Together, parents and older children can work with the care team to develop a plan that works for everyone. Giving older children agency in their own treatment can help to eliminate reluctance or opposition.
Researchers also found value in discussing the effects of each medication and treatment with the patient as well as in providing choices. If a child shows opposition to taking an enzyme supplement, for example, parents have experienced success in describing its function, its importance to the child’s health, and providing an option for taking the supplement with juice or water. Members of a care team can assist in this area as well.