Cystic Fibrosis (CF) is a genetic disorder that primarily affects the lungs and digestive system, caused by mutations in the CFTR gene. This gene normally produces a protein that regulates the movement of salt and water in and out of cells, but in CF, the faulty protein leads to thick, sticky mucus buildup in organs.
In the lungs, this mucus clogs airways, making breathing difficult and increasing the risk of chronic infections like pneumonia. In the digestive system, it can block ducts in the pancreas, hindering enzyme production needed for nutrient absorption, which may cause malnutrition or growth issues. Symptoms often include persistent coughing, frequent lung infections, salty-tasting skin, poor weight gain, and digestive problems. CF is typically diagnosed in childhood, often through newborn screening or sweat tests, which detect high salt levels in sweat, a hallmark of the condition..
Symptoms of CF
People with CF can have a variety of symptoms, including:
- Very salty-tasting skin
- Persistent coughing, at times with phlegm
- Frequent lung infections including pneumonia or bronchitis
- Wheezing or shortness of breath
- Poor growth or weight gain in spite of a good appetite
- Frequent greasy, bulky stools or difficulty with bowel movements
- Nasal polyps
- Chronic sinus infections
- Clubbing or enlargement of the fingertips and toes
- Rectal prolapse
- Male infertility
Books on Cystic Fibrosis for Parents
| Cystic Fibrosis: A Guide for Patient and Family by David M. Orenstein et al. Published 2017, a comprehensive guide with practical advice on managing CF for families. Salt in My Soul: An Unfinished Life by Mallory Smith |
Little Matches: A Memoir of Finding Light in the Dark by Maryanne O’Hara Published 2021, a mother’s story of raising a daughter with CF, blending hope and loss. Coughing It All Up: Chronicles of a Remarkable Life by Jonathan S. Bull |
| Cystic Fibrosis Foundation Provides extensive resources including a “Newly Diagnosed” guide, school support materials, and access to accredited care centers nationwide for parents. National Heart, Lung, and Blood Institute (NHLBI) KidsHealth by Nemours Children’s Minnesota – Cystic Fibrosis Program |
National Jewish Health Offers a parent resource hub with info on treatments, research, and managing CF at home, from a leading U.S. respiratory care center. Seattle Children’s Cystic Fibrosis Program HealthyChildren.org (American Academy of Pediatrics) March of Dimes |
Our children with Cystic Fibrosis
* Listed children often have multiple diagnoses and attention should be given to the specific information in each child’s profile.
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