DISEASE SCANNER

Global Incurable Diseases Tracker

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Genetic Disorder

Cystic Fibrosis

HIGH SEVERITY

An autosomal recessive disorder caused by mutations in the CFTR gene on chromosome 7, affecting chloride ion transport. Results in thick, sticky mucus buildup in lungs, pancreas, and other organs, leading to severe respiratory and digestive problems.

Global Affected

70.0K

Countries

15

Symptoms

Persistent cough with mucus
Frequent lung infections
Wheezing
Poor growth and weight gain
Constipation
Salty-tasting skin

Treatment Options

Genetic counseling
Supportive care
Symptom management
Enzyme replacement (if applicable)
Physical therapy
Regular monitoring
Multidisciplinary care

Risk Factors

1Family history
2Genetic mutations
3Consanguinity
4Advanced paternal age
5Ethnic predisposition

Diagnostic Methods

  • 1Genetic testing
  • 2Clinical evaluation
  • 3Family history assessment
  • 4Specialized laboratory tests
  • 5Imaging studies
  • 6Biopsy (if applicable)

Prognosis

Dramatically improved with CFTR modulators. Median survival now 50+ years with modulators. Without modulators: 40-45 years. Transplant adds 5-10 years for end-stage patients. Quality of life greatly improved.

Prevention

  • Genetic counseling
  • Carrier screening
  • Prenatal diagnosis
  • Preimplantation genetic diagnosis
  • Family planning

Research Status

Trikafta (elexacaftor/tezacaftor/ivacaftor) transforms treatment for ~90% of patients. Gene therapy (4D-710) and mRNA therapies in clinical trials. Life expectancy increased to 50+ years with treatment.

Sources

  • https://www.ncbi.nlm.nih.gov/books/NBK1116
  • https://medlineplus.gov/genetics
  • https://rarediseases.org

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.