Cystic Fibrosis (CF)

February 12, 2020
Janelle Thomas MSN, RN
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Most people take breathing for granted. It’s something that’s done automatically, and it’s practically not thought about — until someone has difficulties bringing air into their lungs. Such is the case with cystic fibrosis. Getting a diagnosis can be terrifying, but with proper management and lifestyle changes, you can have as normal a life as possible. Knowing the risk factors and symptoms is the first step in understanding the disease and what you can do to make breathing as easy as possible.

What is Cystic Fibrosis?

Cystic fibrosis (CF) is a hereditary condition — passed on from parent to child through a recessive gene — that affects the respiratory and digestive systems, as well as a person’s sweat glands. When a person has CF, their body produces thick mucus that’s much stickier than ordinary mucus. This makes breathing difficult, and no matter how much the person coughs, the mucus won’t come out. This mucus also makes it more difficult for the intestines to absorb nutrients. The condition also causes the person to produce an excessive amount of salt in their sweat.

Everyone who has cystic fibrosis is born with the condition. It is because of this reason that every single baby born in the United States is screened for CF. However, this screening process is relatively recent, so it’s possible to receive a diagnosis later in life.

The silver lining about babies being diagnosed as soon as they’re born is that early diagnosis has several benefits — including helping parents better understand the illness, reducing the number of hospitalizations, and ensuring a significantly longer lifespan.

Cystic Fibrosis Symptoms

Symptoms of CF vary from one person to another. However, some of the most common signs of cystic fibrosis include:

  • Shortness of breath
  • Wheezing
  • Recurrent chest colds
  • Persistent coughing
  • Persistent allergies
  • Frequent sinus or respiratory tract infections
  • Fatigue
  • Breathlessness
  • Inflamed nasal passages or a stuffy nose
  • Foul-smelling, greasy stools
  • Constipation
  • Poor growth or slow to gain weight despite healthy eating

Cystic Fibrosis Management

Managing cystic fibrosis requires understanding that since the illness also affects the patient’s digestive system, you need to be proactive about ensuring that nutrients are absorbed and that they maintain a healthy weight. Pay attention to the following:

Getting enough nutrition. A person with CF typically needs as much as twice as many calories as a person of the same age without cystic fibrosis. This is due to two major factors — since the condition affects digestion, the intestines do not absorb as many nutrients; and since it affects the respiratory system, the body spends more energy attempting to breathe normally. Therefore, make it a point to feed your loved one with cystic fibrosis a high-calorie, high-fat diet.

Maintain a healthy weight. The Cystic Fibrosis Foundation recommends a body mass index (BMI) of at least 22 for women, and at least 23 for men. To find out your body mass index, enter height and weight into a BMI calculator. To make sure you maintain a healthy weight, discuss a diet and nutrition plan with your doctor. You’ll likely need to find ways to add calories to your meals. Do keep in mind that it’s recommended to increase fat intake from healthy sources, such as avocado, nuts, almond or peanut butter, and extra virgin olive oil.

Add nutritional supplements. There are many options to increase the number of vitamins and caloric intake, such as protein powders and shakes. However, use them to complement actual meals — never as meal replacements. Look for products with vitamins A, D, E, and K, specifically, since they will aid in growth and nutrition. Other vitamins that are easily absorbed by the body include vitamin C, folic acid, and pantothenic acid.

Stay hydrated. Staying hydrated will allow the cells in the patient’s body to work optimally. You can get water from either H2O or juices, as well as from fruits, vegetables, and soups. Do pay close attention to symptoms of dehydration so that you can resolve the issue as soon as it occurs. These signs include headaches, nausea, and dark yellow or brownish urine.

Exercise. Talk with your doctor to figure out a physical exercise program. This is important to promote lung health, as well as strengthen the heart. Another benefit is that physical activity can help clear mucus buildup from the lungs, which makes breathing easier.

Risk Factors for Cystic Fibrosis

Since the condition is hereditary, a child will be more likely to develop cystic fibrosis if one or both parents carry the mutated gene. There are five classes of mutation, with classes I, II, and III causing more severe symptoms. Having other genes interact with the mutation can also affect the severity of the condition.

To find out whether you or your significant other have the cystic fibrosis mutation, you can do genetic counseling. This consists of undergoing a wide range of tests and reviewing your family’s medical history.

Cystic Fibrosis Complications

When a person has cystic fibrosis, their lung function declines with age. Therefore, it’s crucial to pay close attention to diet and exercise — doing so can add years to the life of a person living with CF. In addition, a person with cystic fibrosis could experience malnutrition, diabetes, chronic respiratory failure, and bowel problems — such as intestinal blockage and rectal prolapse.

Contact Care Options for Kids for Home Health Care

While a child with cystic fibrosis could lead a fulfilling life, it can have an extensive impact on your life and that of your loved ones. Let us help you. Our caregivers are experienced in assisting pediatric patients with CF and can help you to either undergo this transition or take care of your family member full-time.

If you are considering pediatric home health care services, contact the caring staff at Care Options for Kids. Call today (888) 592-5855.