Cystic Fibrosis: Nutrition and Antimicrobial Therapy

Questions and Answers

What is the primary aim of managing pancreatic exocrine insufficiency in children with cystic fibrosis?

To ensure adequate digestion and absorption of nutrients (correct)

To prevent respiratory infections

To increase hydration levels

To monitor growth parameters

What is the recommended starting dose of lipase for adult patients with pancreatic exocrine insufficiency?

30,000 units with each main meal

40,000 units with each main meal (correct)

20,000 units with each main meal

60,000 units with each main meal

Which of the following nutritional requirements should be considered for children with cystic fibrosis?

50-70% of the daily energy requirements

Elimination of salt from the diet

110-200% of the daily energy requirements (correct)

Normal hydration without supplementation

Which treatment is indicated for aiding digestion in patients with pancreatic exocrine insufficiency?

Oral pancreatic enzyme supplements Signup and view all the answers

What vitamin supplementation is critical for children with cystic fibrosis to support their nutritional status?

Fat-soluble vitamins A, D, E, and K Signup and view all the answers

What is the first-line therapy recommended for managing Cystic Fibrosis-Related Diabetes (CFRD)?

Insulin Signup and view all the answers

Which of the following dietary changes is recommended for individuals with Cystic Fibrosis-Related Diabetes?

High-calorie diet Signup and view all the answers

What is one of the aims of managing bone health in individuals with cystic fibrosis?

Decrease the onset of osteopenia and osteoporosis Signup and view all the answers

What is a common cause of kidney disease in individuals with cystic fibrosis?

Aminoglycosides antibiotics Signup and view all the answers

Which supplement is often included in the management plan for bone health in cystic fibrosis?

Vitamin D Signup and view all the answers

What gastrointestinal issue occurs frequently in individuals with cystic fibrosis?

Cholelithiasis Signup and view all the answers

What management strategy is emphasized to maintain renal function in cystic fibrosis patients?

Using inhaled antibiotics when appropriate Signup and view all the answers

What is a significant impact of repeated doses of aminoglycosides in cystic fibrosis patients?

Hearing loss Signup and view all the answers

What is the primary aim of antibiotic therapy in cystic fibrosis management?

To eradicate infection before biofilm formation Signup and view all the answers

Which organism often becomes dominant in the lung infections of cystic fibrosis patients during teenage years?

Pseudomonas aeruginosa Signup and view all the answers

Which of the following treatments aims to improve CFTR function in cystic fibrosis patients?

Ivacaftor Signup and view all the answers

What essential dietary modifications are recommended for cystic fibrosis patients?

High calorie, high salt, high fat Signup and view all the answers

What is the purpose of administering pancreatic enzyme replacement therapy to cystic fibrosis patients?

To aid digestion and absorption of nutrients Signup and view all the answers

What non-respiratory symptom may cystic fibrosis patients experience during exacerbations?

Loss of appetite Signup and view all the answers

Which treatment method can be used to eliminate secretions from the bronchi in cystic fibrosis management?

Hypertonic saline Signup and view all the answers

Which nutrient is particularly important to supplement in cystic fibrosis patients due to malabsorption issues?

Vitamin D Signup and view all the answers

What is the purpose of the Mannitol Tolerance Test prior to the use of inhaled dry powder mannitol?

To assess for bronchospasm risk Signup and view all the answers

Which aspect is NOT a focus of the management principles for cystic fibrosis?

Continuous lung transplant evaluation Signup and view all the answers

What are the typical signs of respiratory exacerbations in cystic fibrosis patients?

Cough, increased sputum, tachypnoea Signup and view all the answers

Why is it crucial to obtain respiratory cultures four times a year in cystic fibrosis patients?

To guide antibiotic therapy and assess infection strains Signup and view all the answers

What is a common misconception about cystic fibrosis prognosis?

It has a cure Signup and view all the answers

Study Notes

Overview of Cystic Fibrosis

Inherited autosomal recessive condition leading to bronchiectasis and chronic lung disease.
Caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, affecting CFTR production and function.
Prevalence in Australia is approximately 1 in 2500.

Nutrition in Cystic Fibrosis

Good nutrition correlates with improved growth and respiratory outcomes.
Individuals may experience pancreatic exocrine insufficiency, necessitating dietary adjustments.
Recommended caloric intake: 110-200% of the general population's daily energy requirements.
Key nutrients include fat-soluble vitamins (A, D, E, K) and increased salt intake.

Management of Pancreatic Exocrine Insufficiency

Aim for proper digestion and nutrient absorption via oral pancreatic enzyme supplements.
Starting adult dose: 40,000 units of lipase with main meals, 20,000 units with snacks.
Dosing adjustments based on symptoms, with maintenance at the lowest effective dose.

Gastrointestinal Health Issues

Common issues include abdominal bloating, constipation, and diarrhea.
Potential mild elevations in liver enzymes, particularly alkaline phosphatase, and risks of cholelithiasis.

Occurrence rates rise with age; approximately 10% in adolescents and 40% in adults.
Management involves insulin therapy and a high-calorie diet, contrasting typical diabetes diet restrictions.

Bone Health Considerations

Heightened risk for osteoporosis and osteopenia.
Management includes nutrition, exercise, vitamin D, calcium supplementation, and bisphosphonates.
Additional strategies: delay chronic lung disease progression, vitamin K supplementation.

Kidney Health Risks

Increased incidence of kidney disease with age; standard creatinine clearance not sensitive in CF.
Causes include antibiotic toxicity and calcineurin drugs post-transplant.
Strategies to preserve renal function involve inhaled antibiotics and therapeutic drug monitoring (TDM).

Hearing Loss

Repeated use of aminoglycoside antibiotics can lead to hearing impairment.

Prognosis and Lifespan

Cystic fibrosis is a lifelong condition with no cure, though average predicted survival has increased to 44 years.

Management Principles

Effective airway clearance, anti-inflammatory therapy, aggressive antibiotic management, and infection control are crucial.
Non-respiratory issues must also be managed to maintain overall health.

Typical Treatment Regimen

Intensive daily physiotherapy for lung clearance, enzyme replacement to aid digestion, and cessation of infections.
Incorporation of high-calorie, high-salt diets along with regular exercise.

Airway Clearance Techniques

Focus on removing airway secretions to reduce blockage and infection via a combination of physical and medicinal approaches.
Mucolytics such as Dornase alfa enhance sputum clearance.

Aerosolised Mucolytics

Dornase alfa reduces sputum viscosity; administered via nebulization (2.5 g daily).
Nebulised hypertonic saline has shown to improve lung function (6% or 3% solutions).

Anti-inflammatory Treatments

Azithromycin is commonly prescribed to improve lung function and reduce exacerbations, specifically effective in Pseudomonas infections.
Caution against unnecessary corticosteroid use.

CFTR Modulator Therapy

Specific medications (Ivacaftor, Lumacaftor/Ivacaftor combinations, etc.) target CFTR function based on mutation types.

Exacerbations Management

Early treatment of respiratory infections is crucial, with symptoms including cough, sputum increase, and loss of appetite.
Non-respiratory signs may include fatigue and weight loss.

Infection Management

Aim to eradicate infections before chronic colonization occurs to prevent lung damage.
Common pathogens include Staphylococcus aureus and Pseudomonas aeruginosa.

General Principles of Antibiotic Therapy

Focus on early eradication of infections or suppression of established ones.
Treatment choices are customized based on local guidelines, culture results, and patient management plans.

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Description

This quiz explores empirical antimicrobial therapy for children with cystic fibrosis, focusing on the balance between eradication and exacerbation of symptoms. It also delves into the importance of good nutrition in promoting growth and improving respiratory outcomes, particularly in the context of pancreatic exocrine insufficiency.