Adherence to Therapy in Chronic Granulomatous Disease and Disease Outcomes: A Possibility for Development of New Therapeutic Delivery Devices

RationaleInfections in chronic granulomatous disease (CGD) are prevented through the administration of antibiotic and antifungal agents in addition to subcutaneous interferon-γ (IFN- γ) therapy; however, medication adherence remains a barrier.MethodsMeasurements of adherence including frequency of hospitalizations, number of hospital days, number of days of medication adherence by self report, and medication side effects were reviewed for 5 patients with CGD.ResultsMedian age is 13 years of age (range 5–17 years). Three patients have autosomal recessive CGD; 2 patients have X-linked CGD. Patient A had 8 admissions while non-adherent to IFN-γ (53 hospital days over 40 months) and 1 admission while adherent. Patient B had 7 admissions while non-adherent (47 hospital days over 27 months) and zero admissions while adherent. Patient C had 12 admissions while non-adherent (57 hospital days over 36 months) and 4 admissions while adherent. Patient D had no admissions prior to or during periods of adherence. Patient E had 2 admissions while non-adherent (38 hospital days over 41 months) and no admissions during 28 months of adherence. Patients reported injection site pain and inconvenience as frequent reasons for non-adherence. One patient reported having fevers after every injection and a febrile seizure.ConclusionsPatients with CGD frequently report non-adherence to IFN-γ for extended periods which result in frequent and prolonged hospitalizations for infections. In the 5 years of this review, patients were more frequently non-adherent than adherent to therapy. Barriers to therapy and possible technological solutions should be pursued for this medication. RationaleInfections in chronic granulomatous disease (CGD) are prevented through the administration of antibiotic and antifungal agents in addition to subcutaneous interferon-γ (IFN- γ) therapy; however, medication adherence remains a barrier. Infections in chronic granulomatous disease (CGD) are prevented through the administration of antibiotic and antifungal agents in addition to subcutaneous interferon-γ (IFN- γ) therapy; however, medication adherence remains a barrier. MethodsMeasurements of adherence including frequency of hospitalizations, number of hospital days, number of days of medication adherence by self report, and medication side effects were reviewed for 5 patients with CGD. Measurements of adherence including frequency of hospitalizations, number of hospital days, number of days of medication adherence by self report, and medication side effects were reviewed for 5 patients with CGD. ResultsMedian age is 13 years of age (range 5–17 years). Three patients have autosomal recessive CGD; 2 patients have X-linked CGD. Patient A had 8 admissions while non-adherent to IFN-γ (53 hospital days over 40 months) and 1 admission while adherent. Patient B had 7 admissions while non-adherent (47 hospital days over 27 months) and zero admissions while adherent. Patient C had 12 admissions while non-adherent (57 hospital days over 36 months) and 4 admissions while adherent. Patient D had no admissions prior to or during periods of adherence. Patient E had 2 admissions while non-adherent (38 hospital days over 41 months) and no admissions during 28 months of adherence. Patients reported injection site pain and inconvenience as frequent reasons for non-adherence. One patient reported having fevers after every injection and a febrile seizure. Median age is 13 years of age (range 5–17 years). Three patients have autosomal recessive CGD; 2 patients have X-linked CGD. Patient A had 8 admissions while non-adherent to IFN-γ (53 hospital days over 40 months) and 1 admission while adherent. Patient B had 7 admissions while non-adherent (47 hospital days over 27 months) and zero admissions while adherent. Patient C had 12 admissions while non-adherent (57 hospital days over 36 months) and 4 admissions while adherent. Patient D had no admissions prior to or during periods of adherence. Patient E had 2 admissions while non-adherent (38 hospital days over 41 months) and no admissions during 28 months of adherence. Patients reported injection site pain and inconvenience as frequent reasons for non-adherence. One patient reported having fevers after every injection and a febrile seizure. ConclusionsPatients with CGD frequently report non-adherence to IFN-γ for extended periods which result in frequent and prolonged hospitalizations for infections. In the 5 years of this review, patients were more frequently non-adherent than adherent to therapy. Barriers to therapy and possible technological solutions should be pursued for this medication. Patients with CGD frequently report non-adherence to IFN-γ for extended periods which result in frequent and prolonged hospitalizations for infections. In the 5 years of this review, patients were more frequently non-adherent than adherent to therapy. Barriers to therapy and possible technological solutions should be pursued for this medication.