Adherence to medical treatment for Wilson’s disease in children and adolescents: a cohort study from Turkey
Abstract Background This study aimed to assess medication adherence and demographic, clinical, and psychopathological parameters such as quality of life, depression, and anxiety levels that can affect pediatrics with Wilson’s Disease (WD). Methods A prospective cohort study was conducted at an outpa...
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| Format: | Article |
| Language: | English |
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BMC
2024-03-01
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| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13023-024-03113-0 |
| _version_ | 1797266668844482560 |
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| author | Mehmet Akif Göktaş Nadir Yalcin |
| author_facet | Mehmet Akif Göktaş Nadir Yalcin |
| author_sort | Mehmet Akif Göktaş |
| collection | DOAJ |
| description | Abstract Background This study aimed to assess medication adherence and demographic, clinical, and psychopathological parameters such as quality of life, depression, and anxiety levels that can affect pediatrics with Wilson’s Disease (WD). Methods A prospective cohort study was conducted at an outpatient clinic in Turkey among pediatric patients (2 to 18 years) with WD between November 2022 and April 2023. The Medication Adherence Report Scale (MARS-5) as a subjective and Medication Possession Ratio (MPR) as an objective assessment were scored. Physical, genetic and biochemical parameters, the Pediatric Quality of Life Inventory (PedsQL) for both parents and patients, Childhood Depression Inventory, State Trait Anxiety Inventory were also administered. Results A total of 30 pediatric outpatients who were prescribed D-penicillamine (n = 27) or trientine (n = 3) as chelators and zinc (n = 29) and pyridoxine (n = 19) as supplements were included. Proteinuria (n = 3), skin rash (n = 2), and gastrointestinal upset (n = 2) were observed. When the correlation between MARS-5 and duration of follow-up was examined, a significant negative correlation was found (p = 0.014). According to MPRs, non-adherence rates (missed doses ≥ 20%) were 29.6%, 17.2% and 5.3% for D-penicillamine, zinc and pyridoxine, respectively. PedsQL scores were higher than those of parents, with a positive correlation between them (p < 0.001). Also, there was a significant positive correlation between PedsQL and State Anxiety Inventory (p < 0.001). Comparing the change in urinary copper levels between different levels of treatment knowledge, significant differences were observed between high- and low levels (p = 0.043). Conclusions Overall, nonadherence rates were 23.3% based on MARS-5 and 5.3–29.6% based on MPR. It is essential to consider factors such as the duration of follow-up, biochemical parameters, treatment knowledge, quality of life and anxiety as potential influencers of medication adherence. |
| first_indexed | 2024-04-25T01:04:21Z |
| format | Article |
| id | doaj.art-eab94fe2ce154740816fb560466df4b7 |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-04-25T01:04:21Z |
| publishDate | 2024-03-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-eab94fe2ce154740816fb560466df4b72024-03-10T12:22:00ZengBMCOrphanet Journal of Rare Diseases1750-11722024-03-011911710.1186/s13023-024-03113-0Adherence to medical treatment for Wilson’s disease in children and adolescents: a cohort study from TurkeyMehmet Akif Göktaş0Nadir Yalcin1Division of Pediatric Gastroenterology, Medical Park Göztepe Hospital, Bahçeşehir UniversityDepartment of Clinical Pharmacy, Faculty of Pharmacy, Hacettepe UniversityAbstract Background This study aimed to assess medication adherence and demographic, clinical, and psychopathological parameters such as quality of life, depression, and anxiety levels that can affect pediatrics with Wilson’s Disease (WD). Methods A prospective cohort study was conducted at an outpatient clinic in Turkey among pediatric patients (2 to 18 years) with WD between November 2022 and April 2023. The Medication Adherence Report Scale (MARS-5) as a subjective and Medication Possession Ratio (MPR) as an objective assessment were scored. Physical, genetic and biochemical parameters, the Pediatric Quality of Life Inventory (PedsQL) for both parents and patients, Childhood Depression Inventory, State Trait Anxiety Inventory were also administered. Results A total of 30 pediatric outpatients who were prescribed D-penicillamine (n = 27) or trientine (n = 3) as chelators and zinc (n = 29) and pyridoxine (n = 19) as supplements were included. Proteinuria (n = 3), skin rash (n = 2), and gastrointestinal upset (n = 2) were observed. When the correlation between MARS-5 and duration of follow-up was examined, a significant negative correlation was found (p = 0.014). According to MPRs, non-adherence rates (missed doses ≥ 20%) were 29.6%, 17.2% and 5.3% for D-penicillamine, zinc and pyridoxine, respectively. PedsQL scores were higher than those of parents, with a positive correlation between them (p < 0.001). Also, there was a significant positive correlation between PedsQL and State Anxiety Inventory (p < 0.001). Comparing the change in urinary copper levels between different levels of treatment knowledge, significant differences were observed between high- and low levels (p = 0.043). Conclusions Overall, nonadherence rates were 23.3% based on MARS-5 and 5.3–29.6% based on MPR. It is essential to consider factors such as the duration of follow-up, biochemical parameters, treatment knowledge, quality of life and anxiety as potential influencers of medication adherence.https://doi.org/10.1186/s13023-024-03113-0Wilson diseaseMedication adherenceQuality of lifeDepressionAnxiety |
| spellingShingle | Mehmet Akif Göktaş Nadir Yalcin Adherence to medical treatment for Wilson’s disease in children and adolescents: a cohort study from Turkey Orphanet Journal of Rare Diseases Wilson disease Medication adherence Quality of life Depression Anxiety |
| title | Adherence to medical treatment for Wilson’s disease in children and adolescents: a cohort study from Turkey |
| title_full | Adherence to medical treatment for Wilson’s disease in children and adolescents: a cohort study from Turkey |
| title_fullStr | Adherence to medical treatment for Wilson’s disease in children and adolescents: a cohort study from Turkey |
| title_full_unstemmed | Adherence to medical treatment for Wilson’s disease in children and adolescents: a cohort study from Turkey |
| title_short | Adherence to medical treatment for Wilson’s disease in children and adolescents: a cohort study from Turkey |
| title_sort | adherence to medical treatment for wilson s disease in children and adolescents a cohort study from turkey |
| topic | Wilson disease Medication adherence Quality of life Depression Anxiety |
| url | https://doi.org/10.1186/s13023-024-03113-0 |
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