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 Caring for Pediatric Patients with Cystic Fibrosis

Caring for Pediatric Patients with Cystic Fibrosis with a Digestive Manifestation (ICD E84. 19)

Author: Rashida Humphrey-Wall, BS, RN, MSN, DNP Lead REM Clinical Care Coordinator

Disease Overview: Cystic Fibrosis (CF) is a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time and may also cause severe digestive issues along with other organ damage (usually diagnosed by two years of age). There is no cure for CF, but the life expectancy has increased greatly by almost 20 years. There are currently more adults with CF than children and newborns with CF are now expected to live to be at least 40 years of age, if not greater.

Symptoms & Causes: People with Cystic Fibrosis (90%) have sticky mucus that blocks ducts in the pancreas and prevents enzymes from reaching the small intestine to digest food. Undigested food in the intestines can cause pain, slow weight gain/growth, cramping, gas, and either loose, greasy, floating stools or constipation and blockages. Cystic fibrosis is an inherited disease caused by mutations in a gene called the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
• Chronic coughing
• Recurring chest colds
• Wheezing or shortness of breath
• Frequent sinus infections
• Very salty-tasting skin

What’s Working: One approach is called pancreatic enzyme replacement therapy. There are also medications to reduce stomach acid, helping the pancreatic enzymes to work better. Supplements of vitamins A, D, E, and K to replace the fat-soluble vitamins that CF patients have trouble absorbing. There are surgical measures, but ideally proactive and preventative practices are best if effective.

Condition Management Challenges: Compliance is very important with CF no matter the treatment route or age of the patient. Staying active is very important and hygiene which in turn lessens some levels of germ exposures. With children especially, you must get creative with medication/supplement administration and breathing treatment administration/oxygen use if needed. Overall, this patients with CF can do everything those without CF can do when well controlled. The barriers presented by Covid-19 are no different than those for other medically fragile or compromised patients. In a way being “quarantined” and seen virtually has been beneficial to this population’s respiratory health safety especially during respiratory season.

Care Management Opportunities: Care management is beneficial for patients with CF especially because of the multi-organ system involvement that can possibly lead to organ transplants, bowel obstructions, motility issues, bacterial overgrowth in organ systems (i.e., the intestine due to antibiotic overuse), GERD, frequent absences from school/work etc. These patients see many specialists and require daily management of symptoms and intermittent exacerbations/frequent infections. Care management resources are beneficial across the spectrum from medical/supply support to physical, emotional/mental health support.

Resources: The REM resources can be utilized by this population of patients; The Arc of PG is a helpful resource, and The CF Foundation has a DC Metro Area Chapter and a Maryland Chapter that are both very active and offer supports from research to activities and nutrition plans; there are even supports to help with day to day living and useful tips for parents raising children with CF. Children’s National Medical Center treats pediatric CF patients and Johns Hopkins has an extensive CF Clinic/Team Equipment Professionals
• The Arc of PG is a helpful resource
• The CF Foundation has a DC Metro Area Chapter and a Maryland Chapter that are both very active and offers support from research to activities and nutrition plans; there are even supports to help with day to day living and useful tips for parents raising children with CF.
• Children’s National Medical Center treats pediatric CF patients
• Johns Hopkins has an extensive CF Clinic/Team Equipment Professionals.

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