Changes in the management of children with Cystic Fibrosis. Caroline Murphy & Deirdre O Donovan CF Nurses

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1 Changes in the management of children with Cystic Fibrosis Caroline Murphy & Deirdre O Donovan CF Nurses

2 What Is Cystic Fibrosis? Cystic fibrosis (CF) is an inherited chronic disease that primarily affects the lungs and digestive system of about 1200 children and adults in Ireland (70,000 worldwide). In Cork, the Paediatric population is 100, the Adult population is 170. A defective gene and its protein product cause the body to produce unusually thick, sticky secretions which causes The fluid lining in the airways to be reduced, resulting in excess sticky secretions that are prone to infection and difficult to expectorate In the pancreas, the sticky secretions block the flow of digestive juices into the gut leading to impaired digestion and poor absorption of food

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4 Symptoms of Cystic Fibrosis very salty-tasting skin productive cough frequent chest infections wheezing or shortness of breath poor growth/weight gain in spite of a good appetite frequent greasy, bulky stools or difficulty in passing stools

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6 Diagnosis Newborn screening Sweat test Genotyping Faecal elastase

7 Genetics

8 Newborn Screening Aim of NBS is early diagnosis before presentation with respiratory symptoms and malabsorption. Introduced in July 2011 Heel Prick test 6 designated CF centres First phone call contact by CF Nurse to parents

9 Newborn Screening - Cork data July July July July July July diagnosis Calls

10 Statistics About 50 new cases of Cystic Fibrosis in Ireland are diagnosed each year. Because of new born screening, most babies with CF should now be diagnosed quickly Around 55% of the CF patient population in Ireland is aged 18 or older. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease in Ireland can now expect to live into their 30s, 40s and beyond.

11 What are the changes in management of children with Cystic Fibrosis?

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14 Nutrition Aim to maintain an appropriate weight above the 50 TH centile Appropriate weight tends to correlates with good pulmonary function 95% of CF patients are pancreatic insufficient Creon is the pancreatic enzyme given to aid absorption of fats. Stools should be monitored by parents, if stools are oily this means creon needs to be adjusted by the dietician Fat soluble vitamins A,D,E,K are supplemented. Levels are checked annually Calcium supplements maybe required to prevent osteoporosis- bone density scan every 5yrs. High fat diet maybe required depending on BMI. Nutritional supplements e.g Calogen, Fortisip, Shakes Enteral feeds (PEG) if low BMI and FTT

15 Nutrition Appetite stimulants H2 antagonists and proton pump inhibitors CF patients are at risk of Distal Intestinal Obstruction Syndrome (DIOS) and constipation. CF Patients may develop Cystic Fibrosis Related Diabetes (CFRD) Increased salt intake in hot weather and avoid dehydration

16 Physiotherapy Past - child was placed prone for precussion Present - PEP, Acapella & Autogenic drainage - Increased emphasis on physical exercise

17 Pulmonary management Lung damage Excessive mucus secretion obstruction PO/IV antibiotics Nebulised mucolytics Nebulised antibiotics +/- steroids +/- antifungals Genetic modifiers Inflammation Mucus stagnation infection

18 Infection Control Microbiology is fundamental to CF management Most commonly cultured organisms: Staph aureus Haemophilus influenzae Pseudomonas Aeruginosa chronic conolisation Aspergillus Non tubercular mycobacteria Antimicrobials are decided by what is cultured on throat swab or sputum if a child is symptomatic Preventing bacterial acquisition & minimising cross infection Single en-suite room No sharing of toilets as inpatient or at clinic Avoid interaction with other CF patients Bring own medical devices Hospital Segregation policies High standard of hand hygiene must be practiced by healthcare professionals l

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