scholarly journals Clinical presentation of Rett syndrome in relation to quality of life and family functioning

2021 ◽  
Vol 49 (4) ◽  
pp. 030006052110077
Author(s):  
Anna Rozensztrauch ◽  
Agnieszka Sebzda ◽  
Robert Śmigiel
Keyword(s):  
Quality Of Life ◽  
Family Functioning ◽  
Rett Syndrome ◽  
Family Relationships ◽  
Clinical Presentation ◽  
Correlation Study ◽  
Family Impact ◽  
Cross Sectional ◽  
The Family

Objective Rett syndrome (RTT) is a chronic condition that manifest in young children, with concomitant comorbidities such as respiratory problems, scoliosis, epilepsy, and malnutrition, which may affect children’s quality of life (QoL) and family functioning. The objective of this cross-sectional descriptive correlation study was to understand the clinical presentation of RTT in relation to QoL and family functioning. Methods We included 23 parents of children with RTT. In this study, we used the PedsQL™ Family Impact Module, the Pediatric Quality of Life Inventory 4.0 generic core scales (PedsQL™ 4.0), and an author-designed questionnaire to assess QoL and family functioning. Results A significant relationship was observed between PedsQL™ 4.0 score and child’s age in the physical functioning dimension. Children aged 8 to 12 years demonstrated significantly higher scores than those in the other age groups. Malnutrition in the child significantly affected functioning of the family in the family relationships dimension. Children receiving 5 hours of rehabilitation treatment a week had significantly higher QoL in the school functioning dimension. Conclusions QOL in children with RTT, as perceived by their parents, is reduced. RTT has a significant negative correlation with family functioning.

Impact of Sickle Cell Disease on Parents.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3348-3348
Author(s):  
Alison M. Blyth ◽  
Lisa M. Foerster ◽  
Julie A. Panepinto
Keyword(s):  
Quality Of Life ◽  
Sickle Cell Disease ◽  
Family Functioning ◽  
Sickle Cell ◽  
Family Relationships ◽  
Summary Score ◽  
Family Impact ◽  
Cell Disease ◽  
The Impact

Abstract Background: Caregivers of patients with chronic illness experience a heavy burden leading to their own negative health outcomes and decreased quality of life. Parents may experience a similar burden caring for children with sickle cell disease, a chronic illness characterized by intermittent hospitalizations, recurrent painful crises, and reduced life expectancy. There are little data evaluating how caring for a child with sickle cell disease affects family functioning and parental health-related quality of life (HRQL). Purpose: To examine family functioning and HRQL of parents of children with sickle cell disease. Methods: During a routine clinic visit, parents completed the PedsQL Family Impact Module, which measures the HRQL of parents who are caring for a child with chronic disease by assessing parental fatigue, stress and difficulty concentrating, etc. In addition, the PedsQL Family Impact Module measures family functioning by assessing family relationships and daily activities. Scores range from 0, representing poor family functioning or HRQL, to 100, representing better family functioning or HRQL. To highlight the impact of caring for a chronically ill child living at home, we compared mean family functioning and HRQL scores of parents of children with sickle cell disease to parents of children with a complex chronic health condition who lived in a long-term Children’s Convalescent Hospital (Varni et al.) using a one-sample t-test. Results: Forty-three parents (41 female and 2 male) of children with sickle cell disease completed the PedsQL Family Impact Module. The mean age (standard deviation) of the children was 9.9 yrs (4.9). Parents of children with sickle cell disease had a significantly lower HRQL summary score than the comparison population (71.99 versus 83.75; p≤.001). In addition, parents of children with sickle cell disease had a significantly lower family functioning summary score than the comparison population (73.91 versus 84.27; p=.002) Table 1 shows the mean HRQL and family functioning scores for each scale in both groups of parents. Conclusions: Caring for a child with sickle cell disease has a significant impact on the parent’s HRQL and family functioning. Parents of children with sickle cell disease are more fatigued, have more trouble concentrating and remembering things, and struggle with stress within their families. Future research in sickle cell disease needs to examine the impact of the child’s treatment and care on family functioning and parental HRQL to improve the burden on these parents. Additionally, greater parent education, family-centered support, and social support may be needed to lessen this burden. Health Related Quality of Life and Family Functioning of Parents of Children With Sickle Cell Disease Scale Sickle Cell Disease (n=43) Comparison Group (n=12)(Varni et al.) *p<.005 HRQL Physical Functioning 59.07* 82.99 Emotional Functioning 74.63 78.33 Social Functioning 79.07 85.42 Cognitive Functioning 76.17* 88.75 Family Functioning Family Daily Activities 67.46* 85.14 Family Relationships 78.02 83.75

scholarly journals Predictors of Quality of Life among Parents of Children with Chronic Diseases: A Cross-Sectional Study

Healthcare ◽  
2020 ◽  
Vol 8 (4) ◽  
pp. 456
Author(s):  
Filiberto Toledano-Toledano ◽  
José Moral de la Rubia ◽  
Laura A. Nabors ◽  
Miriam Teresa Domínguez-Guedea ◽  
Guillermo Salinas Escudero ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Social Support ◽  
Family Functioning ◽  
Chronic Diseases ◽  
Family Income ◽  
Cross Sectional Study ◽  
Sectional Study ◽  
Cross Sectional ◽  
The Family

Quality of life (QOL) is a key aspect of the health care process for children with chronic diseases and their families. Although clinical evidence regarding the impact of chronic disease on children exists, few studies have evaluated the effects of the interaction between sociodemographic and psychosocial factors on the family caregiver’s QOL, indicating a significant gap in the research literature. The present study aimed to identify the predictors of the QOL of parents of children with chronic diseases. Three parental sociodemographic predictors (age, schooling, and family income) and four psychosocial predictors (family functioning, social support, depression, and resilience) were examined. In this cross-sectional study, 416 parents of children with chronic diseases who were hospitalized at a National Institute of Health in Mexico City were interviewed. The participants completed a sociodemographic variables questionnaire (Q-SV) designed for research on family caregivers of children with chronic disease. The predicted variable was assessed through the World Health Organization Quality of Life Questionnaire. The four psychosocial predictors were assessed through the Family Functioning Scale, Social Support Networks Scale, Beck Depression Inventory, and Measurement Scale of Resilience. The regression model explained 42% of the variance in parents’ QOL. The predictors with positive weights included age, schooling, monthly family income, family functioning, social support networks, and parental resilience. The predictors with negative weights included depression. These findings suggest that strong social relationships, a positive family environment, family cohesion, personal resilience, low levels of depression, and a family income twice the minimum wage are variables associated with better parental QOL.

scholarly journals Quality of Life Outcomes According to Differential Nusinersen Exposure in Pediatric Spinal Muscular Atrophy

Children ◽  
2021 ◽  
Vol 8 (7) ◽  
pp. 604
Author(s):  
Meaghann S. Weaver ◽  
Alice Yuroff ◽  
Sarah Sund ◽  
Scott Hetzel ◽  
Matthew A. Halanski
Keyword(s):  
Quality Of Life ◽  
Family Relationships ◽  
Muscular Atrophy ◽  
Transitional Period ◽  
Clinical Population ◽  
Family Impact ◽  
Cross Sectional ◽  
Negative Findings ◽  
The Impact

The purpose of this study was to explore early changes in patient and family caregiver report of quality of life and family impact during the transitional period of nusinersen use. Communication; family relationships; physical, emotional, social, and cognitive functioning; and daily activities were measured using Pediatric Quality of Life modules (Family Impact Modules and both Patient and Proxy Neuromuscular-Specific Reports) pre- and post-nusinersen exposure. A total of 35 patients with SMA (15 Type 1, 14 Type 2, and 6 Type 3) were grouped according to nusinersen exposure. When analyzed as a whole cross-sectional clinical population, no significant differences were found between the initial and final surveys. Nusinersen therapy was associated with improved communication and emotional functioning in subsets of the population, particularly for patients on maintenance therapy for longer duration. Several unexpected potentially negative findings including increases in family resources and trends towards increases in worry warrant further consideration. Further research is warranted to explore the impact of novel pharmaceuticals on quality of life for children with SMA longitudinally to optimize clinical and psychosocial outcomes.

Self Management Menentukan Kualitas Hidup Pasien Diabetes Mellitus

2019 ◽  
Vol 4 (2) ◽  
pp. 402
Author(s):  
Iskim Luthfa ◽  
Nurul Fadhilah
Keyword(s):  
Quality Of Life ◽  
Diabetes Mellitus ◽  
Correlation Study ◽  
Self Management ◽  
Rank Test ◽  
P Value ◽  
Cross Sectional ◽  
Spearman Rank ◽  
Consecutive Sampling

<p><em>People with diabetes mellitus are at risk of developing complications, so that it affects the quality of life. These complications can be minimized through self-care management. This study aims to determine the relationship between self management with the quality of life for people with diabetes mellitus. This research is a kind of quantitative research with correlation study. This research used cross sectional design. The sampling technique uses non probability with estimation consecutive sampling. The number of respondents in this research are 118 respondents. Instrument for measuring self management used diabetes self management questionnaire (DSMQ), and instruments to measure quality of life used quality of life WHOQOL-BREEF. The data obtained were processed statistically by using spearman rank test formula and p value of 0,000 There is a significant relationship of self management with the quality of life of people with diabetes mellitus.</em></p><p> </p><p><em>Penderita </em><em>Diabetes mellitus </em><em>beresiko mengalami komplikasi yang dapat mempengaruhi kualitas hidupnya. Komplikasi tersebut dapat diminimalkan melalui manajemen perawatan diri (self management). Penelitian ini bert</em><em>ujuan </em><em>untuk</em><em> menganalisis hubungan self management dengan kualitas hidup pasien diabetes melitus. </em><em>Jenis p</em><em>enelitian ini </em><em>adalah</em><em> deskriptif korelasi</em><em> dengan desain cross sectional</em><em>. Teknik pengambilan sampel menggunakan non probability </em><em>sampling </em><em>dengan pendeka</em><em>t</em><em>an consecutive sampling</em><em>.</em><em> </em><em>J</em><em>umlah </em><em>sampel sebanyak</em><em> </em><em>118 responden.</em><em> </em><em>Instrumen </em><em>penelitian </em><em>untuk mengukur self management </em><em>menggunakan</em><em> </em><em>diabetes self management questionnaire</em><em> (DSMQ), </em><em>dan instrumen untuk mengukur kualitas hidup menggunakan </em><em>quality of life </em><em>WHOQOL-BREEF.</em><em> Analisis data menggunakan spearman rank dan didapatkan hasil nilai </em><em>p value 0,000</em><em> dan r 0,394.Terdapat </em><em>hubungan </em><em>antara </em><em>self management</em><em> dengan kualitas hidup pasien diabetes mellitus</em><em> dengan arah korelasi positif.</em></p>

Clinical phenotype, fibrinogen supplementation and health-related quality of life in patients with afibrinogenemia

Blood ◽  
2021 ◽  
Author(s):  
Alessandro Casini ◽  
Sylvia von Mackensen ◽  
Cristina Santoro ◽  
Claudia Djambas Khayat ◽  
Meriem Belhani ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Family Relationships ◽  
Thrombotic Event ◽  
Young Patients ◽  
Severe Bleeding ◽  
Health Related Quality ◽  
Cross Sectional ◽  
Related Quality ◽  
Health Related

Due to its low prevalence, epidemiologic data on afibrinogenemia are limited and none are available on health-related quality of life (HRQoL). We conducted a cross-sectional international study to characterize the clinical features, the fibrinogen supplementation modalities and their impact on HRQoL in patients with afibrinogenemia. A total of 204 patients (119 adults and 85 children) from 25 countries were included. The bleeding phenotype was severe: 68 (33.3%) patients having at least one bleed per month and 48 (23%) a history of cerebral bleeding. About 35% (n=72) of patients were treated with fibrinogen concentrates or cryoprecipitates as prophylaxis, 18.1% (n=37) received more than one injection per week and 16.6% (n=34) were on home treatment. A thrombotic event was reported in venous and/or arterial territories by 37 (18.1%) patients. Thrombosis occurred even in young patients and recurrence was frequent (7.4%). The total HRQoL was lower in children than in adults. Discomfort linked to treatment and limitations to sports and leisure were the main concerns. Women and children were particularly affected in family relationships. In multivariate analyses, younger age, residence in Asia or Africa and a previous thrombotic event were statistically correlated with a worse HRQoL. In conclusion, our study underlines the severe bleeding and thrombotic phenotype and their impact on HRQoL in afibrinogenemia. The optimal strategy for fibrinogen supplementation needs to be determined.

Morbidity Pattern and Its Determinants in Urban Field Practice Area of VIMS, Bellary - A Cross-Sectional Study

2021 ◽  
Vol 8 (30) ◽  
pp. 2763-2767
Author(s):  
Pratibharani Reddy ◽  
Ramesh K ◽  
Anju Mariam Jacob ◽  
Gangadhara Goud T
Keyword(s):  
Quality Of Life ◽  
Disease Burden ◽  
Sampling Technique ◽  
Cross Sectional Study ◽  
Age Group ◽  
Sectional Study ◽  
Cross Sectional ◽  
Training Centre ◽  
The Family

BACKGROUND India is doubly burdened with communicable and non-communicable diseases (NCD). Knowledge regarding morbidity profile is important for timely intervention so as to improve the quality of life. For effective health strategies, it is important to know the disease burden of a community. As for the effective preventive strategies, it’s important to know the information regarding disease burden and changing trends of diseases in the locality. Hence this study was done to find the morbidity pattern of urban population in Bellary district, Karnataka. METHODS A cross sectional study was carried out in Millerpet, urban health training centre (UHTC), Bellary, Karnataka. The respective UHTC covers 69195 populations, which has eight wards. Simple random sampling technique was adopted to select the ward. The study was carried out in the selected ward and the study duration was for a period of 3 months. Based on the estimated sample size, 416 houses were selected using random number method. Statistical package for social sciences (SPSS) software version 26 was used for analysing data. Descriptive statistics were used to describe socio demographic and morbidity conditions. RESULTS The most common morbidity among 416 houses were found to be diabetes (22.8 %) followed by hypertension (20 %) and musculoskeletal problems (9 %). Majority of the houses were of nuclear type and the most common age group was 31 - 60 (91.8 %) years followed by 13 - 30 years (80.8 %). 167 (40.1 %) houses had at least one morbidity and 451 (41.4) subjects had at least one morbidity. Socio-demographic variables like age group, family size, monthly income, occupation of head of the family and type of the family were found to be statistically significant. CONCLUSIONS The study revealed that non communicable are the most common diseases present and there is a need to further evaluate the factors responsible so that preventive measures can be taken at the earliest so as to improve the quality of life. KEYWORDS Morbidity Pattern, Urban, Bellary

scholarly journals The impact of dental caries and its treatment under general anaesthetic on children and their families

2020 ◽  
Author(s):  
R. Knapp ◽  
Z. Marshman ◽  
F. Gilchrist ◽  
H. Rodd
Keyword(s):  
Quality Of Life ◽  
Dental Caries ◽  
Health Utility ◽  
General Anaesthetic ◽  
Family Impact ◽  
Score Difference ◽  
The Family ◽  
Related Quality ◽  
The Impact

Abstract Objective To assess the impact of dental caries and treatment under general anaesthetic (GA) on the everyday lives of children and their families, using child-reported measures of quality of life (QoL) and oral health-related quality of life (OHRQoL). Method Participants, aged 5–16 years old having treatment for dental caries under GA, were recruited from new patient clinics at Charles Clifford Dental Hospital, Sheffield. OHRQoL was measured before and 3-months after treatment using the Caries Impacts and Experiences Questionnaire for Children (CARIES-QC). Overall QoL was measured using the Child Health Utility 9D (CHU9D). Parents/caregivers completed the Family Impact Scale (FIS). Results Eighty five parent–child dyads completed the study. There was statistically significant improvement in OHRQoL (mean interval score difference in CARIES-QC = 4.43, p < 0.001) and QoL (mean score difference in CHU9D = 2.48, p < 0.001) following treatment, with moderate to large effect sizes. There was statistically significant improvement in FIS scores (mean score difference = 5.48, p = 0.03). Conclusions Treatment under GA was associated with improvement in QoL and OHRQoL as reported by children, and reduced impacts on the family. This work highlights the importance of GA services in reducing the caries-related impacts experienced by children. Further work is needed investigate the impact of clinical, environmental and individual factors.

Psychometric properties of the Turkish version of the pediatric quality of life: The family impact module in parents of children with type 1 diabetes

2019 ◽  
Vol 49 (1) ◽  
pp. 87-99
Author(s):  
Kübra Pınar Gürkan ◽  
Zuhal Bahar ◽  
Cantürk Çapık ◽  
Nihal Gördes Aydoğdu ◽  
Ayşe Beşer
Keyword(s):  
Quality Of Life ◽  
Type 1 Diabetes ◽  
Psychometric Properties ◽  
Family Impact ◽  
Turkish Version ◽  
The Family

scholarly journals SAT-427 Quality of Life Following Treatment for Graves’ Disease: A Comparison of Radioactive Iodine Ablation and Surgery

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Christopher W Rowe ◽  
Nicholas D Blefari ◽  
Natalie Rutherford ◽  
Cino Bendinelli ◽  
Chris O’Neill
Keyword(s):  
Quality Of Life ◽  
Family Relationships ◽  
Radioactive Iodine ◽  
Physical Symptom ◽  
Treatment Modalities ◽  
Graves Disease ◽  
Tertiary Institution ◽  
Cross Sectional ◽  
The Impact

Abstract Introduction: Quality of Life (QoL) is reduced in patients with Graves’ disease, however the impact of treatment modality is unclear, with conflicting evidence from recent studies (1–3). We hypothesized that surgery would have a greater impact than radioiodine on QoL in Graves’ disease, especially with regard to the physical-symptom subdomain. Methods: Patients treated with thyroid surgery for benign disease (n=425) or radioactive iodine for hyperthyroidism (n=1637) at a single tertiary institution between 2000 and 2017 were invited to complete a validated thyroid-specific QoL instrument (City of Hope), reporting scores 0–10, where higher scores are associated with greater QoL. Responses were received from 307 patients, of whom 114 (37%) had Graves’ disease, treated with total thyroidectomy (n=23) or doses of 15mCi (550MBq) radioiodine (n=91, including 19 patients receiving 2 or more doses). The results of patients with Graves’ disease are reported here. Medians [interquartile ranges] are compared with the Mann-Whitney test (alpha 0.05). Results: 85% of respondents were female, with no difference in gender between groups (p=0.11). Thyroidectomy patients were more likely to be younger (36 [31–49] vs 50 [39–59] years, p=0.004); and have a shorter duration between treatment and survey (5.9 [2.4–9.6] vs 7.6 [4.9–11.6] years, p=0.04). No overall QoL deficit was seen in patients treated with surgery compared to radioiodine (6.8 [5.2–7.3] vs 7.0 [5.8–8.1], p=0.08). However, patients treated with surgery reported reduce QoL in psychological (6.6 [4.7–7.5] vs 7.0 [5.9–8.2] p=0.05) and social (7.9 [6.4 – 8.9] vs 8.9 [7.4–9.8] p=0.01) subdomains compared to radioiodine-only treated patients. Within the social subdomain, the QoL detriment was driven by lower scores relating to questions regarding impact on personal and family relationships, support, and isolation (p&lt;0.001), and not by impact on activities of daily living or employment concerns. Interestingly, there was no between group difference in the physical symptom subdomain (p=0.16). QoL differences between treatment modalities were preserved when patients receiving multiple doses of radioiodine were excluded. Discussion In this non-randomised cross-sectional study from a tertiary-hospital population in Australia, a QoL deficit was demonstrable in subdomain scores for patients with Graves’ disease treated with surgery compared to radioiodine, although the overall result was not significantly different. Better understanding of patient experiences is required to guide treatment strategies and appropriately counsel patients. References (1) Torring et al. Thyroid. 2019;29(3):322–31. (2) Abraham-Nordling et al. Thyroid. 2005;15(11):1279–86. (3) Ljunggren et al. Thyroid. 1998;8(8):653–9.

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