Flexibility: The Key to Caring for a Child or Adult with Cystic Fibrosis

If you or a family member has cystic fibrosis, you know that your loved one may be fine one day and in crisis the next day. Home health personal care assistants provide families the flexibility they need to cope with challenges of cystic fibrosis.

According to the American Lung Association, about 30,000 people in the United States live with cystic fibrosis, and doctors diagnose about 1,000 new cases of this hereditary disease each year. The National Heart, Blood, and Lung Institute at the National Institutes of Health report that 10 million Americans that carry the gene for cystic fibrosis but do not have the diseases. While there is not a cure for this disease that affects the secretory glands in the lungs, pancreas, liver, and intestines, advances in treatments for cystic fibrosis that have extended the life expectancy of people afflicted with this disorder.

Thanks to advances in the diagnosis and treatment of cystic fibrosis, people are living longer lives, but they do tend to have symptom flare ups, which means their caregivers need to have the flexibility to ensure their loved one is monitored and following their treatment protocols. Many families rely upon the services of a home health personal care assistant (PCA) to give them the flexibility they need to care for their loved one and continue to work or attend to the needs of other family members. 

How Does Cystic Fibrosis Affect a Loved One?

Cystic fibrosis is transmitted genetically and affects the glands in the body that create mucous. Normally, mucous is a watery fluid, but in people with cystic fibrosis, this substance becomes thin and sticky. This means that instead of flowing through the lungs and other organs, the mucous lodges in the airways and ducts, thus blocking these passageways. The location of the blockage determines the type of symptoms the person with cystic fibrosis experiences.

Some of the organs affected by cystic fibrosis and the symptoms associated with the disease include the following:

  • Lungs: When fluid secreted in the lungs become thick and sticky, it can prevent the free flow of air in and out of the lungs, which leads to shallow breathing and more severe types of respiratory distress. Additionally, the stagnant mucous serves as a breeding ground for bacteria, which leads to severe lung infections that damage their delicate tissue.
  • Pancreas:  The mucous created by the pancreas carries essential chemicals called enzymes to the small intestines, where they break down food so its nutrients can be absorbed. When cystic fibrosis attacks the pancreas, the thick mucous blocks the ducts, which prevents the enzymes from reaching the intestines so that the nutrients cannot be used by the body.   
  • Sweat Glands: When cystic fibrosis affects the sweat glands, the person tends to lose a great deal of salt in their perspiration. This loss is enough to upset the balance of minerals in the blood, which affects heart functioning leading to palpitations and arrhythmias. Additionally, the individual may experience dehydration as well as overwhelming fatigue.

While cystic fibrosis is present at birth, in some infants the symptoms are so mild that the parents do not notice them, especially if they are not aware that they carry the gene for this disease. With advances in genetic counseling and screenings for the disease at birth, doctors are diagnosing cystic fibrosis earlier, which allows for early treatments that can slow the progression of the disease.

During the 1950s, the outlook for children born with cystic fibrosis was dim. In the mid-20th century, the life expectancy was rarely beyond kindergarten. With the development of new screening techniques, breathing treatments, and medications, people diagnosed with cystic fibrosis was 25 years in 1985. By 2007, the overall prognosis improved and the median life expectancy increased to almost 38 years.  

In addition to living longer, the quality of life has improved for people living with cystic fibrosis. Most children are able to go to school, young adults go to college, and many people work and have families in spite of the disease. While doctors and patients can manage the symptoms of cystic fibrosis to a certain extent, the disease tends to flare unexpectedly. This means that the person diagnosed with the disease may be fine one day and bedridden the next day. Caregivers need to have flexible work arrangements or arrange for other people to support them in caring for their loved one. Many families rely upon the services of a home health PCA as part of their support network.

Caring for a Loved One with Cystic Fibrosis

The nature of caring for a person with cystic fibrosis depends upon which organs are affected by the disease. When the lungs are implicated, the patient must do Chest Physical Therapy, which involves pounding on the chest in order to loosen the mucous so it can be expelled. While it is possible to do this by hand, the doctor may recommend that the person uses a machine. If the person’s lungs have been damaged or the airways are severely blocked, the individual may also need oxygen treatment. Additionally, the doctor may also prescribe antibiotics to reduce the risk of infections and pneumonia and bronchodilators to clear airways.

If cystic fibrosis has damaged the person’s sweat glands, he or she may have a port to which an IV can be attached to deliver fluids and minerals. In addition to the challenge of the IV treatments, the individual must be careful when bathing so not to allow water to get into the port. Often families rely upon a PCA to help with bathing and other self-care tasks.

Sound nutrition is also an important part of home care for a person with cystic fibrosis. When adults living independently with cystic fibrosis have a flare up of their symptoms, they often find it difficult to go grocery shopping and prepare meals. During these times, a home health PCA can handle these tasks, as well as housekeeping and providing transportation to doctor appointments.

Fortunately, medical research continues to make discoveries in the treatment of cystic fibrosis. Organizations, such as the Cystic Fibrosis Foundation provide invaluable resources for people living with this disease. To learn more about how the services of a personal care assistant can give your family the flexibility it needs to cope with cystic fibrosis, contact a Minnesota home health care agency.