Cystic Fibrosis

Cystic fibrosis

Cystic fibrosis is an uncommon genetic disorder. It primarily affects the respiratory and digestive systems. Symptoms often include chronic cough, lung infections, and shortness of breath. Children with cystic fibrosis may also have trouble gaining weight and growing.Treatment revolves around keeping the airway clear and maintaining adequate nutrition. Health problems can be managed, but there’s no known cure for this progressive illness.

 CF was first identified by a woman

In 1938, Dr. Dorothy Andersen made an astounding discovery when she identified cystic fibrosis after performing autopsies of children who were believed to have died from Celiac disease. She noticed the unique scarring of the pancreas and was able to determine CF was a completely separate disease. From her research, she created a test to diagnose cystic fibrosis.

Causes

Cystic fibrosis (CF) is a disease that is passed down through families. It is caused by a defective gene that makes the body produce abnormally thick and sticky fluid, called mucus. This mucus builds up in the breathing passages of the lungs and in the pancreas.

The buildup of mucus results in life-threatening lung infections and serious digestion problems. The disease may also affect the sweat glands and a man's reproductive system.

Many people carry a CF gene, but do not have symptoms. This is because a person with CF must inherit 2 defective genes, 1 from each parent. 

Most children with CF are diagnosed by age 2, especially as newborn screening is performed. For a small number, the disease is not detected until age 18 or older. These children often have a milder form of the disease.

Salt water is a natural CF treatment

Many people with CF have reported feeling better after visiting or moving to the beach. It’s true that saltwater is a natural treatment for people with CF.10 It thins and rehydrates the sticky mucus. It’s so effective that many people inhale salt water through a nebulizer for the same effect.

What does it mean to live with cystic fibrosis?

People with cystic fibrosis should avoid being very close to others who have it. That’s because each person has different bacteria in their lungs. Bacteria that are not harmful to one person with cystic fibrosis can be quite dangerous to another.

COOL FACT: CF doctors integral in development of heparin lock

A heparin lock is when heparin, a blood thinner, is pushed through an IV line and clamped to keep the line open and free of clots. This practice allows the patient to move freely instead of being bed bound with an IV pole with continuous medications or fluids. Today, this practice is almost universal with IV infusions. Coincidentally, it was a team of CF doctors who pioneered the development of the heparin lock in the 1960s. It allowed their patients to receive antibiotics and participate in airway clearance.

 

Check out some cool facts below: 

https://cystic-fibrosis.com/living/surprising-facts-cf