Changes in the management of children with Cystic Fibrosis Caroline - - PowerPoint PPT Presentation

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Jan 07, 2023 213 likes •414 views

Changes in the management of children with Cystic Fibrosis Caroline Murphy & Deirdre ODonovan CF Nurses What Is Cystic Fibrosis? Cystic fibrosis (CF) is an inherited chronic disease that primarily affects the lungs and digestive

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Changes in the management of children with Cystic Fibrosis

Caroline Murphy & Deirdre O’Donovan CF Nurses

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What Is Cystic Fibrosis?

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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

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The fluid lining in the airways to be reduced, resulting in excess sticky secretions that are prone to infection and difficult to expectorate

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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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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

Symptoms of Cystic Fibrosis

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Diagnosis

 Newborn screening  Sweat test  Genotyping  Faecal elastase

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Genetics

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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

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Newborn Screening - Cork data

2 4 6 8 10 12 14 16 18 July 11- 12 July 12 - 13 July 13-14 July 14-15 July 15- 16 July 16-17 diagnosis Calls

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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.

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What are the changes in management

f children with Cystic Fibrosis?

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Nutrition

 Aim to maintain an appropriate weight above the 50TH 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

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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

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Physiotherapy

 Past

child was placed prone for

precussion

 Present

PEP, Acapella &

Autogenic drainage

Increased emphasis on

physical exercise

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Pulmonary management

Excessive mucus secretion

bstruction

Mucus stagnation infection

Inflammation

Lung damage

 PO/IV antibiotics  Nebulised mucolytics  Nebulised antibiotics  +/- steroids  +/- antifungals  Genetic modifiers

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Infection Control l

Microbiology is fundamental to CF management

Most commonly cultured organisms:

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Staph aureus

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Haemophilus influenzae

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Pseudomonas Aeruginosa – chronic conolisation

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Aspergillus

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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

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