Respi CF

What is cystic fibrosis?

A hereditary disease that affects the lungs and digestive system.

What causes the symptoms of cystic fibrosis?

Thick and sticky mucus that can clog the lungs and obstruct the pancreas.

How is cystic fibrosis inherited?

A person must inherit a defective copy of the CF gene from each parent.

When is cystic fibrosis typically diagnosed?

Usually diagnosed in infancy or early childhood, but may be diagnosed later in life.

What is the main pathophysiological cause of cystic fibrosis?

Mutations or dysfunction in the CFTR protein that transports chloride ions across epithelial cell membranes.

What are the hallmarks of cystic fibrosis pathology?

Bronchial mucus plugging, inflammation, and bronchiectasis.

What commonly occurs when airways are colonized in cystic fibrosis?

Colonization with pathogenic bacteria, usually occurring early in life.

What are common bacteria found in the airways of cystic fibrosis patients?

Staphylococcus aureus and H. influenzae.

What are some pulmonary manifestations of cystic fibrosis?

Productive cough, wheezing, hyperinflation of lung fields, and obstructive disease on pulmonary function tests.

What nonpulmonary manifestations are associated with cystic fibrosis?

Gastrointestinal problems, CP-related diabetes, infertility, and clubbing of digits.

What is required for a diagnosis of cystic fibrosis?

Clinical picture consistent with CF phenotype and laboratory evidence of CFTR dysfunction.

What are some diagnostic findings in cystic fibrosis?

Chronic cough, sputum production, persistent infections, and x-ray evidence of bronchiectasis.

What nutritional issues are common in cystic fibrosis patients?

Pancreatic insufficiency and vitamin deficiencies.

What is a common management strategy for airway infections in cystic fibrosis?

Control of infections through aggressive therapy including airway clearance and antibiotics.

What treatments are typically used for acute exacerbations of cystic fibrosis?

Airway clearance techniques and antibiotics based on sputum cultures.

What does chronic therapy for cystic fibrosis include?

Airway clearance measures, mucolytics, nebulized antibiotics, oral antibiotics, and nutritional support.

What is the role of dornase alpha in managing cystic fibrosis?

It's a nebulized medication that degrades DNA that accumulates in CF mucus.

What supplements do cystic fibrosis patients often require?

Oral pancreatic enzyme supplements and fat-soluble vitamins A, D, E, and K.

What nursing interventions are essential for cystic fibrosis patients?

Managing pulmonary symptoms and preventing complications through techniques like chest physiotherapy.

How should patients be educated regarding respiratory infections?

They should be taught about risk factors and early signs of respiratory infection.

What psychosocial aspects need to be addressed in cystic fibrosis care?

Palliative care and end-of-life concerns should be discussed and documented.

What is an important consideration for families of cystic fibrosis patients?

Support is needed as they face a shortened lifespan and uncertain future.

What does airway clearance in cystic fibrosis involve?

Strategies that promote removal of pulmonary secretions.

What kind of nutritional support is emphasized for cystic fibrosis patients?

Adequate fluid and dietary intake to promote secretion removal and nutritional status.

What condition occurs in cystic fibrosis related to the urogenital system?

Congenital bilateral absence of the vas deferens and obstructive azoospermia.

How do respiratory symptoms manifest in cystic fibrosis when diagnosed later in life?

Respiratory symptoms are frequently the major manifestation.

What is the percentage of cystic fibrosis patients with pancreatic insufficiency?

Approximately 90% of patients have pancreatic insufficiency.