Sepsis and Cystic Fibrosis

Cystic fibrosis is one of the most common progressive, genetic diseases in the United States. People with cystic fibrosis (CF) develop frequent and chronic infections, which can lead to sepsis.

Sepsis, which was often called blood poisoning, is the body’s life-threatening response to infection. Like strokes or heart attacks, sepsis is a medical emergency that requires rapid diagnosis and treatment.

Cystic fibrosis is a progressive disease caused by a defective CF gene. If two adults who each carry this gene have a child together, the child has a:

  • 50% chance of being a carrier of the CF gene
  • 25% chance of having CF
  • 25% chance of not being a carrier or having the disease

Adults may have no idea they are CF carriers if they are unaware of CF in their family.

There are many aspects to CF, but the hallmark of the disease is thick mucus, sweat, and digestive juices that the body produces. Mucus normally helps protect and lubricate body tissues. But with CF, the mucus and liquids coat and eventually damage organs, like the lungs. The mucus clogs the airway, making it hard to breathe. It also causes scarring in the lungs and increases the risk of infection by blocking microbes (germs) in the airway.

Cystic fibrosis also affects the digestive system. As mucus builds up on the intestine walls, the body can’t absorb nutrients from the food and the pancreas can’t produce enough insulin. Without treatment, this leads to malnutrition.

Suggested Citation:
Sepsis Alliance. Sepsis and Cystic Fibrosis. 2023. https://www.sepsis.org/sepsisand/cystic-fibrosis/ 

Updated December 12, 2023.

 

More About Cystic Fibrosis

Symptoms

Children with CF are usually diagnosed before they are two years old, although there are people with milder cases who only learn about it when they are teens.

Very early on, a newborn with CF may have meconium ileus, an intestinal blockage.

A child with CF may also show signs like:

  • Oily, thick, greasy bowel movements
  • Constipation or diarrhea
  • Frequent lung infections (pneumonia)
  • Difficulty breathing, wheezing
  • Difficulty exercising or exerting
  • Stuffy nose, sinuses
  • Coughing, producing thick mucus
  • Very salty sweat
  • Failure to grow or gain wait
Treatment

There is no cure for CF so treatment focuses on managing and easing symptoms, as well as preventing and treating infections that inevitably occur.

Infection Prevention

Aside from managing CF symptoms, people with CF must follow strict infection prevention techniques. These include:

  • Frequent and thorough hand washing
  • Frequent and thorough disinfecting of common surfaces
  • Recommended vaccines and boosters
  • Healthy diet (malnutrition increases infection risk)
  • Physical distancing when around other people
  • Medical or surgical masks when in common areas with others

People with CF are encouraged to always keep at least six feet away from others with CF, unless they live together. This is because people with CF often contract infections that are not common among the general population and they could pass these along easily to others with the disease.

People with CF are vulnerable to infections, so it is vital to watch for any signs and symptoms. Early identification and treatment of infections can reduce the risk of sepsis. It is possible for an infection to trigger sepsis without warning, however, because infections are not always obvious. Knowing the initial and most common sepsis symptoms allows for early recognition and treatment.

Related Resources

Leanna Babet

My sweet 27-year-old daughter passed away on October 9th after 1 week in intensive care. She had a seizure on night 1 in the emergency department and was placed in a coma soon after. Her organs all started to fail, complicated by the fact she also had cystic fibrosis. (Sepsis and Cystic Fibrosis) We kissed her good-bye. Still waiting for a diagnosis of what infection caused the sepsis, now hoping the coroner will find an answer for us. I had no idea about sepsis and never imagined she’d be taken so quickly from us. We’re shattered. Read Full Story

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Cystic Fibrosis

Cystic fibrosis is one of the most common progressive, genetic diseases in the United States. People with cystic fibrosis (CF) develop frequent and chronic infections, which can lead to sepsis.