Cystic Fibrosis Transmembrane Regulator Modulators: Implications for the Management of Depression and Anxiety in Cystic Fibrosis

Talwalkar, Jaideep S.; Koff, Jonathan L.; Lee, Ho-Chang; Britto, Clemente J.; Mulenos, Arielle M.; Georgiopoulos, Anna M.

doi:10.1016/j.psym.2017.04.001

Cited by 52 publications

(61 citation statements)

References 38 publications

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“…As with the rhinology subset, there was significant improvement in the psychological QOL subset scores. Poor psychological health, which often accompanies a diagnosis of CF, has been shown to have deleterious effects on overall pulmonary function, treatment adherence, hospitalization rates, and healthcare costs . The results of this study suggest that patients have improved psychological health–related QOL following treatment with ivacaftor.…”

Section: Discussionmentioning

confidence: 67%

Ivacaftor improves rhinologic, psychologic, and sleep‐related quality of life in G551D cystic fibrosis patients

McCormick

Cho

Lampkin

et al. 2018

Int Forum Allergy Rhinol

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Background Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator that improves pulmonary function in cystic fibrosis (CF) patients with at least 1 copy of the G551D CFTR mutation. The purpose of this study is to evaluate the impact of ivacaftor on chronic rhinosinusitis (CRS) symptoms in this population. Methods The G551D Observational (GOAL) study was a multicenter prospective cohort study enrolling CF patients ≥6 years with at least 1 G551D mutation. Subjects were provided 20‐item Sino‐Nasal Outcome Test (SNOT‐20) questionnaires prior to ivacaftor therapy and at 1, 3, and 6 months afterward. The impact on rhinologic (R), psychological (P), sleep (S), and ear/facial (E) quality of life (QOL) domains was evaluated separately. Results Of 153 subjects, 129 (84%) completed all questionnaires. Typical baseline symptom burden was low (75% with scores <1) and degree of improvement (ie, reduced scores) was greater with higher baseline scores. SNOT‐20 decreased, reflecting improvement, at all follow‐up intervals (1 month: [mean change ± standard deviation] –0.25 ± 0.53, p < 0.01; 3 months; –0.29 ± 0.58, p < 0.01; 6 months: –0.21 ± 0.58, p = 0.02), but less than the prespecified minimal clinically important difference (0.8). Significant improvement was observed at 1, 3, and 6 months in the R domain (1 month: –0.24, p < 0.01; 3 months: –0.34, p < 0.01; 6 months: –0.25, p < 0.01) and P domain (1 month: –0.25, p < 0.01; 3 months: –0.32, p < 0.01; 6 months: –0.26, p < 0.01), and 1 and 3 months in the S domain (1 months: –0.35, p < 0.01; 3 months: –0.32, p < 0.01; 6 months: –0.18, p = 0.07). There was no improvement in the E domain at any time point. Conclusion Ivacaftor improves QOL in the R, P, and S domains in G551D CF patients, although QOL instruments validated for CRS may not translate well to CF CRS patients because symptom burden was surprisingly low.

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Section: Discussionmentioning

confidence: 67%

Ivacaftor improves rhinologic, psychologic, and sleep‐related quality of life in G551D cystic fibrosis patients

McCormick

Cho

Lampkin

et al. 2018

Int Forum Allergy Rhinol

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“…It does not change the fact that the detection of anxiety and depression in CF patients (by identifying factors triggering negative emotional states) and the implementation of treatment based on the patient's condition are crucial to ensure well-being. Achieving this goal is possible through proper cooperation between CF treatment and research centres 12,13,16,38,39,40 . Negative emotions and sleep disorders disrupt the functioning of the autonomic nervous system, which is associated with a change in response of leukocytes and mediators, and this in turn can adversely affect the course of the disease.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of selected insomnia predictors in adolescents and young adults with cystic fibrosis

Tomaszek

Cepuch²,

Pawlik

2018

BIOMED PAP

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“…An alternative hypothesis is that both lumacaftor and ivacaftor are lipophilic and can cross the blood‐brain barrier to affect CFTR, monoamine transport and 5‐HT2c receptors within the central nervous system. Therefore, close mental health monitoring should be established in CF patients who have baseline anxiety and depression as their antidepressant dose may need to be adjusted as seen in Table 1 25 …”

Section: Commentmentioning

confidence: 99%

A current review of the safety of cystic fibrosis transmembrane conductance regulator modulators

Gavioli¹,

Guardado²,

Haniff³

et al. 2020

J Clin Pharm Ther

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What is known and objective Treatment with cystic fibrosis transmembrane conductance regulator (CFTR) modulators has led to improved clinical outcomes and an increase in lifespans of cystic fibrosis (CF) patients. As CF patients continue to live longer, they are at risk for developing adverse drug reactions associated with polypharmacy and CFTR modulators. Comment The authors aim to describe safety concerns of the current combination CFTR modulators, based upon a literature review, including notable safety concerns and recommendations for drug‐drug interactions. What is new and conclusion Cystic fibrosis transmembrane conductance regulator agents are generally well tolerated with low discontinuation rates when compared to placebo. Elevations in liver enzymes and drug‐drug interactions are the most notable safety concerns. Additionally, lumacaftor/ivacaftor has shown more respiratory‐related adverse events and drug‐drug interactions compared to elexacaftor/tezacaftor/ivacaftor and tezacaftor/ivacaftor. Postmarketing studies are needed to determine long‐term safety concerns.

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Cystic Fibrosis Transmembrane Regulator Modulators: Implications for the Management of Depression and Anxiety in Cystic Fibrosis

Cited by 52 publications

References 38 publications

Ivacaftor improves rhinologic, psychologic, and sleep‐related quality of life in G551D cystic fibrosis patients

Ivacaftor improves rhinologic, psychologic, and sleep‐related quality of life in G551D cystic fibrosis patients

Evaluation of selected insomnia predictors in adolescents and young adults with cystic fibrosis

A current review of the safety of cystic fibrosis transmembrane conductance regulator modulators

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