Cystic Fibrosis Awareness Month- May 2023

May is Cystic Fibrosis Awareness Month and below are some helpful facts put together by our president, Jane Greenblatt, RN, BSN and some from the Cystic Fibrosis Foundation.

What is cystic fibrosis and is it catchy? 

Cystic Fibrosis is an inherited disorder that causes severe damage to the lungs, digestive system, and other body organs. No, it is not catchy.

What are its mechanisms, and how does it work?

Cystic Fibrosis affects the cells that produce mucous, sweat, and digestive juices. These secretions are usually thin and slippery. But with CF, a defective gene causes secretions to become sticky and thick. Instead of acting like a lubricant which happens normally, the secretions plug up tubes, ducts, and passageways, especially in the lungs and pancreas.

Will it go away on its own?

Cystic Fibrosis is a progressive disease and requires daily care – however, folks with CF often are able to attend school and work. Today they have a better quality of life than in previous decades. Improvements in screening and treatment mean people with CF now may live into their mid to late 30s-40s and some are living into their 50s, with some even living longer if they receive a lung transplant.

How is this diagnosed in children and young adults?

In the United States there is newborn screening. Cystic fibrosis can be diagnosed in the first month of life, even before symptoms occur. 

What are the symptoms of cystic fibrosis?

Cystic fibrosis signs and symptom vary depending on the severity of the disease.  In the same person, symptoms may worsen or improve as time passes. Some people do not experience symptoms until they are teenagers or adulthood. People who are not diagnosed until adulthood typically have a milder form of the disease and are more likely to have recurring bouts of an inflamed pancreas or pancreatitis, infertility, and recurring pneumonia. People with CF have a higher-than-normal level of salt in their sweat, and parents can often taste the salt when they kiss their children. Most other sings and symptoms affect the respiratory and digestive systems.

What are the respiratory symptoms seen in cystic fibrosis?

The thick and sticky mucous plugs the airways of an individual with CF clogging the tubes which bring air in and out of the lungs. This can cause signs and symptoms such as:

  • A persistent cough that produces thick mucous
  • Wheezing
  • Exercise intolerance
  • Repeated lung infections
  • Inflamed nasal passages or stuffy nose
  • Recurrent sinusitis

What are the signs and symptoms of digestive disorders with cystic fibrosis?

  • Foul-smelling, greasy stools
  • Poor weight gain and growth
  • Intestinal blockage, particularly in newborns (meconium ileus)
  • Chronic or severe constipation, which may include frequent staining while trying to pass stool, eventually causing part of the rectum to protrude outside of the anus (rectal Prolapse)

What are other complications associated with cystic fibrosis?

  • Diabetes due to injury to the pancreas
  • Liver disease due to bile duct blockages to the small intestines
  • Intestinal Blockages intussusception, a condition in which a segment of the intestine slides inside an adjacent section of the intestines like a collapsible telescope.
  • Distal intestinal obstruction syndrome (DIOS), partial to full obstruction where the small bowel meets the large bowel.
  • Infertility in men because the vas deferens is blocked. 100% of all CF men are infertile. Some treatments can occur so that these men can become biological fathers
  • Reduced fertility in women. While women can become pregnant with CF, it often times exasperates symptoms
  • Thinning of the bones (osteoporosis) may also experience joint pain and arthritis
  • Electrolyte imbalances and dehydration because of the extra high level of salt in their bodies
  • Mental health problems dealing with a chronic disease

Is there a cure for cystic fibrosis?

There are no cures for cystic fibrosis, but there are a range of treatments that can help control the symptoms, prevent, or reduce complications and make living with this condition a little easier. Cystic fibrosis clients are treated by a team of healthcare providers and sometimes treatment needs to occur in a hospital setting. The following are examples of things that can be done, and medications that are provided to treat and prevent lung problems:

  • Antibiotics to prevent and treat chest problems
  • A combination of 3 medications (Kaftrio) to treat the root causes of in clients over 12 years of age
  • Medication to thin secretions thus making it easier to cough up
  • Bronchodilators help to open airway passages making it easier to breath
  • Steroid Medications to decrease swelling and to treat nasal polyps
  • Exercise any kind of physical activity can help clear the lungs and improve physical strength
  • The use of airway clearance techniques, breathing techniques to clear the lungs, compression vest to loosen secretions,
  • Managing dietary needs and good nutrition to help with food digestion as the pancreas does not work well for CF patients making it harder to digest food

I hope this has been informative and helpful providing some understanding of this very hard disease.

~Jane Greenblatt, RN, BSN, President of Home, Hope and Healing

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