Health library / CYSTIC FIBROSIS

CYSTIC FIBROSIS

  • Cystic fibrosis is a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time.

Symptoms

  • Meconium Ileus
  • Chest Pain
  • Cough (With Thick Mucus)
  • Recurrent Wheezing
  • Recurrent Pneumonia
  • Shortness Of Breath
  • Difficulty In Gaining Weight
  • Diarrhoea
  • Constipation
  • Greasy, Foul-Smelling Stools
  • Loss Of Appetite
  • A Stuffy Nose

Causes

  • Cystic fibrosis occurs due to mutation or change in the “cystic fibrosis transmembrane conductance regulator” gene, or CFTR gene.

Risk Factors

  • An Inherited Disorder
  • Diagnostic Tests
  • Genetic test for detecting defective CFTR gene Sweat Chloride Test
  • Immunoreactive Trypsinogen (IRT) Test
  • CT Scan
  • Sputum microbiology
  • Pulmonary function testing
  • Differential Diagnosis
  • Acute Sinusitis
  • Bronchiolitis
  • Nutritional Considerations in Failure to Thrive
  • Pediatric Aspergillosis
  • Pediatric Asthma
  • Pediatric Bronchiectasis
  • Pediatric Celiac Disease
  • Primary Ciliary Dyskinesia
  • Short Stature
  • Treatments
  • No cure for cystic fibrosis.
  • Treatments are available that relieve symptoms and reduce the risk of complications include
  • Chest Physical Therapy
  • Nasal polyp removal
  • Pneumothorax
  • Oxygen therapy
  • Endoscopy and lavage
  • Rectal prolapse
  • Lung transplant
  • Gastrostomy tube placement for supplemental feeding
  • Medications
  • Nonsteroidal anti-inflammatory drugs
  • CFTR Potentiators
  • Bronchodilators
  • Oral pancreatic enzymes
  • Antibiotics
  • Mucus-thinning medications
  • Lifestyle Management
  • Drink plenty of fluids
  • Exercise regularly
  • Avoid smoke, pollen, and mold
  • Get influenza and pneumonia vaccinations regularly
  • Music therapy
  • Relaxation exercises
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