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If At First You Don't Succeed, Trikafta Again.

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Severe skin reactions to cystic fibrosis transmembrane conductance regulator (CFTR) modulators like Trikafta can occur. This case study details managing a drug eruption in a child with cystic fibrosis through desensitization, enabling continued treatment.

Keywords:
CFTR modulatorcystic fibrosisdesensitizat urticariaelexacaftorivacaftortezacaftor

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Area of Science:

  • Pharmacology
  • Pulmonology
  • Dermatology

Background:

  • Cystic fibrosis transmembrane conductance regulator (CFTR) modulators are crucial therapies for cystic fibrosis.
  • Adverse drug reactions, including severe cutaneous reactions, have been reported with CFTR modulators.
  • Managing these reactions is vital for treatment adherence and patient outcomes.

Purpose of the Study:

  • To report a case of drug eruption in a pediatric patient receiving elexacaftor/tezcaftor/ivacaftor (Trikafta).
  • To describe the successful desensitization protocol implemented for the patient.
  • To review existing literature on adverse reactions to CFTR modulators.

Main Methods:

  • Case presentation of a 7-year-old male with cystic fibrosis experiencing a drug eruption.
  • Implementation of a desensitization protocol for elexacaftor/tezcaftor/ivacaftor.
  • Literature review of similar adverse events and management strategies.

Main Results:

  • The patient developed a drug eruption in response to elexacaftor/tezcaftor/ivacaftor.
  • Desensitization allowed for the successful continuation of CFTR modulator therapy.
  • Literature review identified similar cases and management approaches.

Conclusions:

  • Adverse reactions to CFTR modulators, such as Trikafta, require careful management.
  • Desensitization is a viable strategy to enable continued use of essential CFTR modulator therapies.
  • Effective management of drug reactions is critical for individuals with cystic fibrosis.