Rachel Sommer

Understanding the impact of statural height on health-related quality of life in German adolescents: a population-based analysis

AbstractImproving health-related quality of life (HrQoL) is one aim of growth hormone treatment for short-statured patients. Yet, evidence of the relationship between height and HrQoL is inconclusive, especially for young people. This study investigates the association of height and HrQoL in the general German adolescent population. A total of 6646 adolescents and 6388 parents from the German Health Interview and Examination Survey for Children and Adolescents (KIGGS) were included in the analysis. An analysis of covariance was used to evaluate the effect of height deviation on HrQoL as assessed with the KINDL-R in adolescent self-report and parent-report, taking into account sociodemographic and health-related variables. Height was found to be a weak predictor of HrQoL in both adolescent-report and parent-report. Short and tall adolescents did not substantially differ from the normal statured. Sociodemographic and health-related variables failed to significantly interact with height. In contrast, psychosocial variables contributed to the explained HrQoL variance. Conclusion: Height does not appear to be a strong determinant of HrQoL on the population level. These results suggest that the height gain in short stature treatment may not be the most important objective. Contrary to prevalent stereotypes, short adolescents can experience a high HrQoL.

Evaluation of the American-English Quality of Life in Short Stature Youth (QoLISSY) questionnaire in the United States

BackgroundThe European Quality of Life in Short Stature Youth (QoLISSY) is a novel condition-specific instrument developed to assess health related quality of life (HrQoL) in children/adolescents with short stature from patient and parent perspectives. Study objective was to linguistically validate and psychometrically test the American-English version of the QoLISSY instrument.MethodsUpon conversion of the British-English version to American-English, content validity and acceptance of the questionnaire were examined through focus group discussions with cognitive debriefing in 28 children/adolescents with growth hormone deficiency (GHD) or idiopathic short stature (ISS) and their parents. In the subsequent field test with 51 families and a re-test with 25 families the psychometric performance of the American-English version was examined and compared with the original European dataset.ResultsPilot test results supported the suitability of the American-English version. Good internal consistency with Cronbach’s Alpha ranging from 0.84 to 0.97 and high test-re-test reliabilities were observed in the field test. The QoLISSY was able to detect significant differences according to the degree of short stature with higher HrQoL for taller children. Correlations with a generic HrQoL tool support the QoLISSY’s concurrent validity. The scale’s operating characteristics were comparable to the original European data.ConclusionResults support that the QoLISSY American-English version is a psychometrically sound short stature-specific instrument to assess the patient- and parent- perceived impact of short stature. The QoLISSY instrument is fit for use in clinical studies and health services research in the American-English speaking population.

Leben mit Achondroplasie

OBJECTIVE Presently, little is known aqout the quality of life (QoL) as well as the strengths and difficulties of young people with achondroplasia. This study describes these patient-reported indicators and identifies possible correlates. METHOD At the invitation of a patient organization, a total of 89 short-statured patients aged 8 to 28 years and their parents participated in this study. QoL was assessed cross-sectionally with both generic and disease-specific instruments and the Strengths and Difficulties Questionnaire (SDQ) as a brief behavioral screening. In addition to descriptive analyses, patient data were compared with a reference population. Hierarchical regression analyses reflecting sociodemographic, clinical, and psychological variables were conducted to identify correlates of QoL. RESULTS QoL and the strengths and difficulties of young patients with achondroplasia did not differ substantially from a healthy norm sample. However, the participants reported more behavioral problems and limitations in their physical and social QoL compared to patients with another short stature diagnosis. Strengths and difficulties, height-related beliefs, and social support correlated significantly with QoL. Adding psychological variables to the regression model increased the proportion of variance explained in QoL. CONCLUSIONS Young persons with achondroplasia did not differ in their QoL and strengths and difficulties from healthy controls. Characteristics such as height appear less important for the self-perceived QoL than are strengths and difficulties and protective psychosocia~factors.

Development and pilot-testing of a condition-specific instrument to assess the quality-of-life in children and adolescents born with esophageal atresia

The survival rate of children with esophageal atresia has today reached 95%. However, children are at risk of chronic morbidity related to esophageal and respiratory dysfunction, and associated anomalies. This study describes the pilot testing of a condition-specific health-related quality-of-life instrument for children with esophageal atresia in Sweden and Germany, using a patient-derived development approach consistent with international guidelines. Following a literature review, standardized focus groups were conducted with 30 Swedish families of children with esophageal atresia aged 2-17 years. The results were used for item generation of two age-specific pilot questionnaire versions. These were then translated from Swedish into German with considerations of linguistic and semantical perspectives. The 30-item pilot questionnaire for children aged 2-7 years was completed by 34 families (parent report), and the 50-item pilot questionnaire for children aged 8-17 years was completed by 52 families (51 child report, 52 parent report), with an overall response rate of 96% in the total sample. Based on predefined psychometric criteria, poorly performing items were removed, resulting in an 18-item version with three domains (Eating, Physical health and treatment, Social isolation and stress,) for children aged 2-7 years and a 26-item version with four domains (Eating, Social relationships, Body perception, and Health and well-being) for children aged 8-17 years. Both versions demonstrated good internal consistency reliability and acceptable convergent and known-groups validity for the total scores. The study identified specific health-related quality-of-life domains for pediatric patients with esophageal atresia, highlighting issues that are important for follow-up care. After field testing in a larger patient sample, this instrument can be used to enhance the evaluation of pediatric surgical care.

Associations between Psychological Problems and Quality of Life in Pediatric Short Stature from Patients’ and Parents’ Perspectives

Short stature has been associated with psychosocial impairments, but whether treatments and achieved height impact on health-related quality of life (HrQoL) and psychological functioning of children/adolescents is still controversial. This study aimed to examine the effects of height deviation and treatment status on psychosocial adaptation outcomes and to identify clinical and psychosocial determinants of internalizing/externalizing problems in a large cohort of short statured children/adolescents from seven European countries. Participants were 345 children aged 8–18 years with a clinical diagnosis of short stature and 421 parents of 4–18 year-old patients. Children and parents reported on psychological problems (Strengths and Difficulties Questionnaire), generic (KIDSCREEN) and condition-specific HrQoL (QoLISSY). According to analyses of covariance, children/adolescents with current short stature presented more parent-reported internalizing problems and lower self- and parent-reported condition-specific HrQoL, compared to patients with an achieved height above -2SD. Treated children self-reported better HrQoL than the untreated group. Hierarchical regression analysis showed that, rather than height–related clinical variables, children’s sex, younger age and poorer HrQoL were the best predictors of psychological problems, explaining 39% of the variance in patient- and 42% in parent-reported internalizing problems, and 22% of the variance in patient- and 24% in parent-reported externalizing problems. Treatment status also moderated the negative links between patient-reported HrQoL and internalizing problems, explaining 2% of additional variance. These results suggest that children with current short stature are at greater risk for internalizing problems. Routine assessment of HrQoL in pediatric healthcare may help identify children for referral to specialized psychological assessment and intervention.

An ICF-CY-based approach to assessing self- and observer-reported functioning in young persons with achondroplasia – development of the pilot version of the Achondroplasia Personal Life Experience Scale (APLES)

Abstract Purpose: Achondroplasia is the most common disproportionate short stature which impacts patients’ well-being. Little is known about the burden of disease in terms of functioning of patients and few disease-specific patient-reported outcome (PRO) measures exist. To understand the consequences of achondroplasia, the objective of the study was to develop an achondroplasia-specific PRO tool. Method: Focus group discussions including 34 German patients (age 8–18 years) and 21 parents were conducted and qualitatively analyzed. To identify relevant concepts, statements were coded according to the International Classification of Functioning, Disability and Health: Children & Youth version (ICF-CY). Upon condensation, relevant statements were reformulated as items. Results: 1950 statements related to 125 ICF-CY categories were identified. After condensation and prioritization, 59 items were retained. These were generated based on the ICF-CY domains environmental factors (27 items), activities and participation (18 items), body functions (9 items), and body structures (5 items). Conclusions: A new instrument, the Achondroplasia Personal Life Experience Scale (APLES) to assess burden of disease and functioning from the patients perspective of children and adolescents with achondroplasia has been developed in its pilot version based on the classification of the ICF-CY. Psychometric performance of the APLES is currently being examined. Implications for Rehabilitation Using the ICF-CY within the development of a disease-specific instrument to assess burden of disease and functioning provides guidance for clinicians and researchers in the selection of appropriate instruments complying with ICF categories. Qualitative research based on focus group interviews is an effective method to elicit experiences, beliefs, points of view, understandings, perceptions, impressions, needs and concerns as well as attitudes from the perspective of the individuals on a specific topic such as functioning. Direct assessment of the patient perspective is necessary to thoroughly understand a patient’s experience of disease and treatment, the impact on their functioning and relevant health-care needs. Including the perspective of the patient and its family is an important component in the provision of health-care services. Patient-reported outcomes are not only important for the development of interventions and the use in clinical trials but can also be used in medical decision-making processes.

Was ist Schuppenflechte? - Wahrnehmung und Bewertung der Psoriasis in der deutschen Bevölkerung: Wahrnehmung der Psoriasis in der deutschen Bevölkerung

Die Versorgung der Psoriasis vulgaris nimmt in der Dermatologie einen wichtigen Stellenwert ein. Im Zuge der WHO‐Resolution 2014 und des WHO Global Reports 2016 hat die öffentliche Aufmerksamkeit für die Psoriasis zugenommen. Unklar ist, wie Psoriasis in der Allgemeinbevölkerung wahrgenommen wird. Ziel ist die Erfassung der Wahrnehmung und Bewertung von Psoriasis in der Bevölkerung.

The Wound-QoL questionnaire on quality of life in chronic wounds is highly reliable

The Wound‐QoL questionnaire has been tested in a previous study, indicating that the instrument is a reliable and valid measurement for the assessment of HRQoL in chronic wounds. The aim of this study was to examine the test–retest reliability of the Wound‐QoL. Patients were asked to complete the Wound‐QoL twice within 3–7 days. Next to descriptive statistics, internal consistency was determined. Test–retest reliability was calculated with the intraclass correlation coefficient. The data showed ICCs between 0.79 and 0.86, which can be considered evidence of excellent reliability. Another indicator of very good reliability, high internal consistency of both global score, and subscale scores were found. The availability of the Wound‐QoL provides a step forward in allowing measurement of the HRQoL in chronic wounds. The instrument is fit for use to measure outcomes in clinical trials and as well to assess treatment outcomes in routine practice.

Linking a short-stature specific health-related quality of life measure (QoLISSY) to the International Classification of Functioning – Children and Youth (ICF-CY)

Abstract Purpose: The study aimed to examine and compare the content of the quality of life in short-stature youth questionnaire (QoLISSY) with the International Classification of Functioning, Disability and Health – Children and Youth (ICF-CY). Methods: Items of the QoLISSY questionnaire for children/adolescents and their parents were coded within the framework of the ICF-CY. Linkage was performed according to predetermined standards by two health professionals experienced in the conceptual fundaments of the ICF-CY. Results: Within the linking process 58 meaningful concepts were identified from the 55 items out of the QoLISSY parent questionnaire and 55 meaningful concepts from the 50 items of the QoLISSY (children) questionnaire. In total, 57 concepts (parent-questionnaire) and 54 concepts (children-questionnaire) were linked to the different categories of the ICF-CY. Twenty-seven categories (parents-version) and 20 categories (child-version) referred to the component body functions, 34 categories (parent-version) and 30 categories (child-version) represented the activity and participation component and in each version 30 categories represented the environmental factors component. Conclusions: The present study indicates that the ICF-CY provides a useful frame of reference to compare and examine the content of HrQoL instruments for short-statured children and adolescents and their parents. The linkage of the QoLISSY instrument within the framework of the ICF-CY translates its content into a universal language. Implications for Rehabilitation The linkage of Quality of Life – Questionnaires to the ICF-CY provides guidance for clinicians and researchers in the selection of appropriate instruments complying with ICF categories. Linking the QoLISSY instrument to the ICF may raise awareness in clinicians and researchers about domains relevant for young people with short-stature from a well-being and functioning perspective. In the linkage exercise, the ICF serves as a reference matrix which can be used to compare and better understand QoL domains of different instruments. With the linkage, effects of interventions as assessed via QoLISSY can be tied to ICF categories.

Differences between patient- and proxy-reported HRQoL using the Wound-QoL: Patient- versus proxy-reported HRQoL

The purpose of this study was to examine the patient‐ and proxy‐report agreement on the Wound‐quality of life (QoL) questionnaire to assess health‐related QoL in patients with chronic wounds. Patients and their proxies (in this case, relatives) were recruited via different nursing services in Germany and asked to complete the Wound‐QoL. Intraclass correlation coefficients were calculated for each Wound‐QoL scale and the total score, and weighted Cohens Kappa coefficients for individual items. Proxies tended to report lower health‐related QoL in the Wound‐QoL than their relatives with chronic wounds. Agreement between the two perspectives was moderate on single‐item level and excellent on scale level. Proxy‐reports can be helpful, if patients are unable to provide information, although caution remains when interpreting the results. However, the patients own perspective remains preferable.