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Cystic Fibrosis Mortality: Causes, Life Expectancy, and Progress

Cystic fibrosis (CF) is an autosomal recessive disorder (CFTR mutations) causing thick mucus → multisystem disease.

Leading Causes of Death

  1. Respiratory failure: End-stage bronchiectasis, chronic Pseudomonas/NTM infection, mucus plugging.
  2. Cardiac: Cor pulmonale (RV failure from pulmonary hypertension).
  3. Hepatic: CF-related liver disease (focal biliary cirrhosis) → portal hypertension.
  4. Post-transplant complications: Rejection, infection, malignancy (lung transplant median survival ~6–7 yrs).

    5. Life Expectancy

    • 1950s: <1 year.
    • 2020s: Median predicted survival >50 years (CFF Registry); many live into 40s–50s.
    • Modulator therapies (elexacaftor/tezacaftor/ivacaftor) for F508del + residual function mutations → transformative.

    No Cure Yet

    Gene therapy, mRNA, CRISPR in trials. Multidisciplinary CF center care essential.

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