Difficulties Experienced by Turkish Parents and Their Coping Strategies: Children With Mucopolysaccharidosis

Zengin, Mürşide; Yayan, Emriye Hilal; Akıncı, Ayşehan

doi:10.1016/j.pedn.2020.03.009

Cited by 15 publications

(42 citation statements)

References 22 publications

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“…There was no disagreement on full-text screening. In total, seven articles were included in the review 14,20,23,24,31-33 . The full study selection process is shown in Figure 1 34 …”

Section: Resultsmentioning

confidence: 99%

“…For the overall quality score, one article met all the appraisal criteria 31 . Additionally, one study met eight criteria, 20 two met seven criteria, 24,33 and three met six criteria 14,23,32 . All studies met the criteria for Q2, Q3, Q4, Q5, and Q8; on the other hand, only 29% of the studies met the criterion for Q1 (Is there congruity between the stated philosophical perspective and the research methodology?…”

Section: Resultsmentioning

confidence: 99%

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Experiences of patients with lysosomal storage disorders who are receiving enzyme-replacement therapy and the experiences of their family members: a qualitative systematic review

Koto

Ueki

Yamakawa

et al. 2021

JBI Evidence Synthesis

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The objective of this review was to investigate the experiences of patients with lysosomal storage disorders who are receiving enzyme-replacement therapy and the experiences of their family members.Introduction: Lysosomal storage disorders are rare diseases caused by mutations in the genes that encode proteins required for lysosomal function. The age of onset of these disorders varies from infancy to adulthood, depending on the specific disease and type. Enzyme-replacement therapy is the standard treatment for some lysosomal storage disorders. However, patients' adherence to this treatment is affected not only by the resultant changes (or lack thereof) in their symptoms, but also by the scheduling of the frequent hospital visits necessary to receive this treatment. No previous qualitative systematic review has examined the experiences of these patients and their families.Inclusion criteria: Qualitative studies on the experiences of patients with lysosomal storage disorders who were receiving enzyme-replacement therapy and/or the experiences of the family members of these patients were included. These experiences could include satisfaction/dissatisfaction with diagnosis, difficulties and expectations regarding continuing treatment, advantages/disadvantages concerning school and work life, the psychological burden on families, and the support provided by families. This review considered studies in all settings because relevant experiences may occur outside medical institutions.Methods: MEDLINE, CINAHL Plus, APA PsycINFO, Scopus, and Igaku Chuo Zasshi were searched for articles published between January 1991 and May 13, 2021. No language restrictions were applied. The study selection, critical appraisal, data extraction, and data synthesis were performed in accordance with the JBI methodology for systematic reviews of qualitative evidence.Results: Seven studies were included in this review, from which 37 findings with narrative illustrations were extracted; of these, 33 were assessed as unequivocal and four as credible. These findings were integrated into 10 categories and three synthesized findings. The first synthesized finding was encouraging awareness of the minor changes in physical symptoms caused by the treatment, which contains categories such as physical change caused by treatment. The second synthesized finding was supporting acceptance of the disease and coping with associated psychological challenges, which contains categories such as concerns regarding the future. The third synthesized finding was customization of treatment plans to minimize restrictions on the lives of patients and their families, which contains categories such as restrictions on patients' lives. According to the ConQual criteria, all three synthesized findings had low confidence levels. Conclusion:Evidence obtained through the synthesized findings produced in this review identified the primary experiences of patients with lysosomal storage disorders who are receiving enzyme-replacement therapy and their family members. These experienc...

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“…There was no disagreement on full-text screening. In total, seven articles were included in the review 14,20,23,24,31-33 . The full study selection process is shown in Figure 1 34 …”

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Experiences of patients with lysosomal storage disorders who are receiving enzyme-replacement therapy and the experiences of their family members: a qualitative systematic review

Koto

Ueki

Yamakawa

et al. 2021

JBI Evidence Synthesis

View full text Add to dashboard Cite

show abstract

“… 7 - 10 Local information on demographic and clinical characteristics is also available. 11 , 12 However, only a single study by Zengin et al 13 has addressed the difficulties of living with MPS in Turkey, based on interviews with parents of 10 children with MPS. Although their study was mainly concerned with the burden of the disease, themes coinciding with our study also emerged, including school attendance and difficulties in social life.…”

Section: Discussionmentioning

confidence: 99%

“…Although their study was mainly concerned with the burden of the disease, themes coinciding with our study also emerged, including school attendance and difficulties in social life. 13 A German study on 10 MPS-VI patients with a Turkish background also addressed the difficulties and coping strategies but mainly in relation to the disease rather than the treatment. 14 To the best of our knowledge, ours is the first study from Turkey to investigate the difficulties related specifically to ERT in MPS.…”

Section: Discussionmentioning

confidence: 99%

“…It has been reported that cultural factors, including religious background, which were not explored in our questionnaire, can influence how Turkish families deal with MPS. 13 , 14 In fact, faith is a common coping mechanism for caregivers of chronic patients, 23 and its influence may apply to the burden of the treatment as well as that of the disease. This study suggests that in contrast to missing ERT doses, more disruption to family life is associated with receiving ERT infusions at our center.…”

Section: Discussionmentioning

confidence: 99%

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Difficulties Associated with Enzyme Replacement Therapy for Mucopolysaccharidoses

Yıldız¹,

Sivri²

2021

Turk Arch Pediatrics

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Background: Mucopolysaccharidoses are extremely rare, progressive, often severe multisystem disorders, some of which are managed by weekly intravenous enzyme replacement therapy. This study aimed to determine the difficulties faced by the patients with mucopolysaccharidosis and their families due to enzyme replacement therapy. Methods: A questionnaire about demographics, enzyme replacement therapy-related characteristics, and specific enzyme replacement therapy-related difficulties was conducted over the telephone with mucopolysaccharidosis patients (or their parents) followed at a referral center in Turkey, who have been on enzyme replacement therapy for ≥12 months. The responses were analyzed with chi-square, Mann–Whitney U , Kruskal–Wallis tests, Spearman's rank correlation, and binary logistic regression. Results: A total of 54 patients (median age: 13 years) participated, who had been receiving enzyme replacemnt therapy for a median of 5.02 years, 83.3% of whom had mucopolysaccharidosis-IVA or -VI. About 72.2% went to school or work, 64.1% of whom missed a full day every week due to enzyme replacement therapy. About 63% missed at least 1 dose in the past 6 months, mostly due to not being able to obtain doses or having intercurrent infections. Significantly more enzyme replacement therapy doses were missed or unobtained in Central (non-Ankara) and Eastern Anatolia, but enzyme replacement therapy-related disruption to family life was more severe in families living in Ankara. Conclusions: We provide the first Turkish data about mucopolysaccharidosis patients’ subjective enzyme replacement therapy experience, which is influenced by actionable inequalities and hurdles, partially related to geographical factors. Access to the drugs can be facilitated, and the clash of enzyme replacement therapy infusions with school and work should be avoided. Multi-center studies using more objective data sources are needed.

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A systematic review and integrative sequential explanatory narrative synthesis: The psychosocial impact of parenting a child with a lysosomal storage disorder

Hassall

Smith

Rust

et al. 2022

J of Inher Metab Disea

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Lysosomal storage disorders are rare multiorgan, degenerative conditions requiring invasive treatment. Rare disorders pose unique challenges; therefore, exploring their impact is crucial for understanding family needs. This novel review aimed to understand the psychosocial outcomes for parents of children with lysosomal storage disorders. Five electronic databases were systematically searched. Thirty‐eight (23 qualitative, 10 qualitative and 5 mixed methods) studies were included, analysed using a sequential explanatory narrative synthesis and appraised for their methodological quality. Quantitative data revealed the multifaceted impact on parents' psychological and social wellbeing. Qualitative data informed the challenges that these parents faced which were expressed within three main themes: (a) Uncertainty and the unknown, (b) All‐encompassing impact and (c) Finding a way forward. The synthesis demonstrated that factors associated with the condition (symptoms, behaviour and severity) had a substantial negative impact on parental outcomes, upheld by concurrent loss (deterioration and poor prognosis) and uncertainty. This substantive integrated review revealed considerable unmet parental psychosocial needs.

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Difficulties Experienced by Turkish Parents and Their Coping Strategies: Children With Mucopolysaccharidosis

Cited by 15 publications

References 22 publications

Experiences of patients with lysosomal storage disorders who are receiving enzyme-replacement therapy and the experiences of their family members: a qualitative systematic review

Experiences of patients with lysosomal storage disorders who are receiving enzyme-replacement therapy and the experiences of their family members: a qualitative systematic review

Difficulties Associated with Enzyme Replacement Therapy for Mucopolysaccharidoses

A systematic review and integrative sequential explanatory narrative synthesis: The psychosocial impact of parenting a child with a lysosomal storage disorder

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