5 Things Every Cystic Fibrosis Caregiver Should Know

A chronic genetic disease for which there is no cure, cystic fibrosis affects 35,000 people in the United States, according to the Center for Disease Control. It is one of the most common and severe genetic diseases in the U.S, According to BioNews, Inc., and is caused by a genetic disorder where the lungs and digestive systems become clogged with mucus. Healthy people’s mucus is watery and slippery, keeping the internal organs healthy. But in individuals who have cystic fibrosis, the mucus is thick and sticky and leads to many health problems. 

Cystic fibrosis impacts the gastrointestinal, pancreatic, respiratory, and reproductive systems. The mucus can trap germs and make infections more likely, block airways, and lead to lung damage. The disease decreases the body’s ability to absorb nutrients from food by preventing proteins needed for digestion from reaching the intestines.

It is a lifelong disease that changes as individuals age. Caregivers to family, friends, and clients with cystic fibrosis often play many different roles. Sometimes, a caregiver needs to help administer breathing treatments and medication. Some days, a caregiver needs to be a cheerleader, and help keep mental health positive. Cystic fibrosis is a grueling disease and can be extremely difficult emotionally for both the individual affected and their caregiver. It’s vital to know as much about the disease as possible in order to best care for loved ones. 

Here are five things caregivers need to know about cystic fibrosis: 

Learn the symptoms of cystic fibrosis

Improvements in the diagnosis of cystic fibrosis have made early treatment possible in children. Newborns are screened for the disease now, and children are in many cases diagnosed by age 2. Cystic fibrosis runs in families, but not all families are aware of their genetic history, and the diagnosis can be startling. It’s important for children diagnosed with cystic fibrosis to receive treatment as soon as possible, as symptoms are milder, and quality of life is higher. 

In milder cases of cystic fibrosis, signs may not be seen until adulthood, according to the Center for Disease Control. The symptoms and signs of cystic fibrosis include frequent lung or sinus infections, wheezing or shortness of breath, salty-tasting skin, a cough that doesn’t go away (often seen with thick mucus or blood), poor growth, or weight gain in childhood, and male infertility. 

Older individuals with cystic fibrosis experience symptoms including breathlessness, repeated lung infections, inflamed nasal passages or a stuffy nose, poor growth and weight gain, intestinal blockage, and severe constipation. 

Good nutrition is vital 

People with cystic fibrosis often suffer from pancreatic insufficiency, which affects the body’s ability to absorb nutrients and digest food. In addition, because they have to work harder to breathe, oftentimes calorie deficiency causing malnutrition occurs. Some people with cystic fibrosis may need as much as two times as many calories as people without the disease, with a significant portion of those calories coming from fat. Peanut butter and avocado are two nutrient-rich and high-calorie foods that are touchstones to an overall cystic fibrosis diet. Additionally, it might be necessary for individuals with cystic fibrosis to take pancreatic enzyme supplements and vitamins.

Everyday Health, a website with healthy recipes, has a section of recipes specifically for individuals with pancreatic insufficiency. Recipe suggestions include oatmeal with nuts, fish with white rice, sliced apple with peanut butter, and suggestions for skipping dessert and going for fresh fruit instead.

Take precautions to prevent infection

Individuals with cystic fibrosis need to be extremely careful to not catch viruses and develop infections as much as possible, given that they are already plagued by chronic lung infections. As all of us learned during the coronavirus pandemic, standing six feet away from someone who is ill is a thoughtful way to try to prevent the spread of disease, and it is the way individuals with cystic fibrosis must live their lives always. 

It’s important for caregivers to take protocols to be sure they’re not spreading germs to individuals with cystic fibrosis. Wearing a facemask, washing hands often, and keeping the home very clean, are important ways to keep infections from spreading. Being outside and keeping the body healthy through regular exercise help keep the immune system in good shape to help individuals with cystic fibrosis fight infections as well. 

Know when to call the doctor 

Cystic fibrosis symptoms can worsen over time. Respiratory symptoms don’t always limit younger people, but when individuals age, the shortening of breath often becomes more acute. Disease exacerbations (or flare-ups) sometimes become more frequent, with a longer-lasting recovery. 

Providing care to an individual with cystic fibrosis requires knowing the signs of increased health risk and the potential need for emergency care. Caregivers should call a doctor if an individual with cystic fibrosis has not had a bowel movement in more than one or two days, has severe stomachaches, has bloody bowel movements, is vomiting, has a fever, experiences more shortness of breath than usual, loss of appetite, loss of weight new or worse coughing and wheezing, and other new symptoms, according to the Cystic Fibrosis Foundation.

Understand treatment options & the possibility of a lung transplant 

According to the Center for Disease Control, there are treatments for improving breathing and digestion, thinning mucus, and preventing infections. There have been some advances in medicine that target the underlying cause of cystic fibrosis. However, medical treatment only treats some of the mutations of the disease. Other treatments include therapy to clear mucus out of the lungs, treatments to improve breathing and digestion and prevention of infections. 

In some cases, a lung transplant is necessary. Individuals with severe cystic fibrosis can have greater longevity and better health with a lung transplant. However, complications can arise with organ transplants, including organ rejection. And, recovery is a long process, requiring great care of the new lungs. Advances in medical treatment hopefully will make the likelihood of lung transplants less likely in the future.