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1.
Haemophilia ; 30(3): 728-732, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38578181

RESUMO

INTRODUCTION: The PedHAL questionnaire is employed in measuring the activities of haemophilia children. The PedHALshort was developed in 2022 to determine the most relevant item for the assessment. Haemo-QoL questionnaire assesses the quality of life of haemophilia children. AIM: Determine the validity and reliability of the PedHALshort Indonesia language version compared to the Haemo-QoL in the Indonesian population. METHODS: A cross-sectional study was conducted in Jakarta, Indonesia. The subjects were children (4-16 years old) with moderate or severe haemophilia A or B with repeated joint bleeding. The daily activity was assessed using the PedHALshorts and Haemo-QoL. The validity and reliability were tested. The validity was determined with Pearson correlation test, construct validity was assessed using convergent and discriminant validity. The Spearman correlation was used to determine which domains of Haemo-QoL correlated with PedHALshort. The reliability was analyzed using test-retest reliability. Cronbach's α was used to determined internal consistency. RESULTS: Fifty children were included in the study (78% haemophilia A, 70% severe group). All domains were valid (r > .284). The convergent validity test showed PedHALshort had a moderate negative correlation with physical health and sports school domain of Haemo-QoL (r = -.479, p < .001). The discriminant validity test showed the PedHALshort demonstrated a moderate negative correlation with physical health (r = -.585), view (r = -.590), sport school domain (r = -.430) in severe haemophilia subjects. The internal consistency of the PedHALshort questionnaire was high (Cronbach's α of .85 (.74-.92)). CONCLUSION: The PedHALshort is valid and reliable, hence, it can be used to measure the functional physical activity of children with haemophilia.


Assuntos
Hemofilia A , Qualidade de Vida , Humanos , Criança , Indonésia , Inquéritos e Questionários/normas , Adolescente , Masculino , Pré-Escolar , Estudos Transversais , Hemofilia A/psicologia , Reprodutibilidade dos Testes , Feminino
2.
Haemophilia ; 30(3): 809-816, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38616526

RESUMO

BACKGROUND: There is a lack of research on the relationship between pain catastrophizing, kinesiophobia, and physical activity (PA) in people with haemophilia (PWH), and the underlying mechanisms connecting these variables remain unclear. AIM: The study's aim was to clarify the roles of kinesiophobia and self-efficacy in the relationship between pain catastrophizing and PA in PWH. METHODS: This cross-sectional study included adult PWH at the Haemophilia Centre of a Tertiary hospital in Beijing, China. The following questionnaires were used to collect data: the general information, the International Physical Activity Short Questionnaire, the Pain Catastrophizing Scale, the Tampa Scale of Kinesiophobia Scale, and the Exercise Self-Efficacy Scale. RESULTS: The study included a total of 187 PWH, including 154 having haemophilia A and 33 having haemophilia B. The median interquartile range of PA was 594 (198, 1554) MET-min/wk. There were significant differences in PA of patients based on age stage, treatment modality, highest pain score within the last seven days, and presence of haemophilic arthropathy (p < .05). It was showed that pain catastrophizing could directly predict PA (p < .001), accounting for 38.13% of the total effect. Pain catastrophizing also had indirect effects on PA through the mediating factors of kinesiophobia or self-efficacy, and through the chain-mediating effect of kinesiophobia and self-efficacy, accounting for 38.40%, 17.07%, and 6.40%, respectively. CONCLUSION: The study discovered that PWH have limited PA due to pain catastrophizing. This not only directly affects their activity but also indirectly influences it through kinesiophobia and self-efficacy.


Assuntos
Catastrofização , Exercício Físico , Hemofilia A , Autoeficácia , Humanos , Hemofilia A/psicologia , Hemofilia A/complicações , Adulto , Exercício Físico/psicologia , Masculino , Estudos Transversais , Catastrofização/psicologia , Feminino , Pessoa de Meia-Idade , Transtornos Fóbicos/psicologia , Adulto Jovem , Inquéritos e Questionários , Adolescente , Cinesiofobia
3.
Haemophilia ; 30(3): 743-751, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38507035

RESUMO

INTRODUCTION: The physical pain and disability affecting many people with haemophilia A (PwHA) are known detractors from psychological wellbeing. While psychosocial support is considered a core tenet of the haemophilia comprehensive care structure, the extent to which mental health challenges are detected and monitored by the individuals treating haematologist remains relatively unexplored. AIM: To describe prevalence of anxiety and depression in a real-world cohort of adult PwHA and evaluate the congruence in reporting of anxiety or depression (A/D) between PwHA and their treating physicians. METHODS: Data for PwHA without inhibitors was drawn from the European 'Cost of Haemophilia: A Socioeconomic Survey II' (CHESS II) study. Haematologist-indicated comorbidities of anxiety and depression were unified into a single A/D indicator. The EQ-5D-5L health status measure was used to characterise self-reported A/D, with individuals stratified into two non-mutually exclusive subgroups based on level of A/D reported (Subgroup A: 'some' or above; Subgroup B: 'moderate' or above). RESULT: Of 381 PwHA with evaluable EQ-5D-5L responses, 54% (n = 206) self-reported at least some A/D (Subgroup A) and 17% (n = 66) reported at least moderate A/D (Subgroup B). Patient-physician congruence in A/D reporting was 53% and 76% for Subgroups A and B, respectively. Descriptive analysis suggested that individuals with physician- and/or self-reported A/D experienced worse clinical outcomes (bleeding events, joint disease, chronic pain). CONCLUSION: While adverse clinical outcomes appear to correlate with A/D, self-reports of moderate-severe symptoms occasionally lacked formal recognition from treating physicians. Cross-disciplinary surveillance of mental health issues could improve both psychological and clinical outcomes among PwHA.


Assuntos
Ansiedade , Depressão , Hemofilia A , Humanos , Hemofilia A/complicações , Hemofilia A/psicologia , Depressão/epidemiologia , Depressão/etiologia , Depressão/psicologia , Ansiedade/psicologia , Ansiedade/epidemiologia , Adulto , Masculino , Europa (Continente) , Pessoa de Meia-Idade , Feminino , Médicos/psicologia , Adulto Jovem , Qualidade de Vida
4.
Haemophilia ; 30(3): 800-808, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38523258

RESUMO

BACKGROUND: Chronic joint pain is a significant and widespread symptom in people with haemophilia (PWH). Despite medical advancements, effective pain management remains challenging. AIM: This study presents an innovative approach that integrates remote physical exercises, pain neuroscience education, and coping strategies to address chronic pain in PWH. METHODS: The remote intervention consisted of sixteen 5-min videos encompassing physical exercises for chronic pain management and pain education strategies. These videos formed an 8-week remote intervention program. Clinical and physical assessments were conducted before and after the intervention. RESULTS: A total of thirty-one PWHs, with a median age of 34 years (ranging from 16 to 59 years), completed the remote intervention. The study revealed significant improvements in pain intensity, disability, and physical performance among PWH with chronic pain. Enhanced functional capacity was evident in the Timed Up and Go and Single Leg Stance tests, accompanied by improved scores on the Functional Independence Score in Haemophilia (FISH). Although lacking a control group, our findings are consistent with other successful exercise and pain education programs. CONCLUSIONS: This innovative intervention holds promise for managing chronic pain in PWH, underscoring patient empowerment, education, and collaboration. Notably, our study stands out by uniquely combining pain education and coping strategies, bolstering evidence for effective pain management.


Assuntos
Adaptação Psicológica , Hemofilia A , Humanos , Hemofilia A/complicações , Hemofilia A/psicologia , Hemofilia A/terapia , Adulto , Masculino , Adolescente , Adulto Jovem , Pessoa de Meia-Idade , Manejo da Dor/métodos , Feminino , Exercício Físico/psicologia , Educação de Pacientes como Assunto/métodos , Dor Crônica/psicologia , Dor Crônica/terapia , Terapia por Exercício/métodos , Capacidades de Enfrentamento
5.
Int J Psychiatry Med ; 59(1): 20-33, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37565475

RESUMO

OBJECTIVE: This study described the prevalence of and correlates of depression and anxiety in adult patients with hemophilia A and B. METHODS: In this cross-sectional study, we investigated patients with hemophilia who were being seen at an adult hemophilia center in Turkey. Participants were screened for depression and anxiety during their annual clinic visit, which included administration of the Beck Depression Inventory and State-Trait Anxiety Scale. RESULTS: Of the 90 patients, 22 (24.4%) met criteria for significant depressive symptoms and 67 (74.4%) met criteria for significant anxiety symptoms. There were no significant associations between depression and any psychosocial or clinical characteristics or adherence to hemophilia prophylaxis, except for educational status. No association was found between state and trait anxiety scores and psychosocial and clinical characteristics other than hemophilia type. After adjusting for confounding factors, multivariable analysis showed that high school education level was associated with depression (OR: 1.87, CI: 1.31-2.36, P = .010) and type B hemophilia was associated with anxiety (OR: 1.32, CI: 1.09-1.75, P = .042). CONCLUSION: Depression and anxiety are major psychiatric comorbidities in patients with hemophilia in Turkey. Routine evaluation for mood and anxiety disorders are important in the routine care of patients with haemophilia.


Assuntos
Hemofilia A , Adulto , Humanos , Hemofilia A/epidemiologia , Hemofilia A/complicações , Hemofilia A/psicologia , Depressão/epidemiologia , Depressão/psicologia , Estudos Transversais , Ansiedade/epidemiologia , Ansiedade/psicologia , Transtornos de Ansiedade/epidemiologia , Transtornos de Ansiedade/complicações
6.
Haemophilia ; 29(6): 1509-1518, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37694815

RESUMO

INTRODUCTION: Joint bleeds in haemophilia cause destruction of articular structures, impaired function and pain. Up to 70% of people with haemophilia (PWH) report chronic pain. Little is known about the pain experiences in PWH in the UK. AIM: To identify prevalence and perceptions of pain among PWH living in the UK. METHODS: A cross-sectional, non-interventional survey study conducted among PWH (all severities). The survey incorporated elements from validated tools (EQ-5D; EQ-VAS) and was distributed via participating treatment centres. RESULTS: Five hundred and ninety-nine PWH responded, 91% aged > 18. 81% used factor prophylactically or on demand. More pain was reported by those treated on demand versus prophylaxis particularly in those who reported daily pain. 65% reported 'problem joints' based on individual impact rather than medically defined 'target joints', 2/3 reported multiple joint issues. The ankle was most commonly affected. 59% reported frequent pain, with 56% aware of pain constantly or most of the time and were more likely to report less favourable EQ-5D or EQ-VAS scores (p < .001). Pain frequency/awareness was consistent across all severities. Most discussed pain with care teams, 31% only when asked; 25% did not discuss it. Pain discussions resulted in physiotherapy referral (63%) analgesia prescription (48%), and a minority specialist pain referral (9%). Most felt well supported with regard to their pain, but 70% reported learning to live with it. CONCLUSION: Pain affects PWH of all ages and severities even in a well-resourced country significantly impacting quality of life. Clinicians must be more aware of chronic pain in PWH. Biopsychosocial approaches to pain assessment and management are recommended.


Assuntos
Dor Crônica , Hemofilia A , Humanos , Hemofilia A/complicações , Hemofilia A/epidemiologia , Hemofilia A/psicologia , Dor Crônica/epidemiologia , Qualidade de Vida , Prevalência , Estudos Transversais , Reino Unido/epidemiologia
7.
Haemophilia ; 29(5): 1226-1233, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37428628

RESUMO

BACKGROUND: Haemophilia is an inherited bleeding disorder. Mothers of children with haemophilia experience stress, anxiety and different types of burdens that affect their life negatively. OBJECTIVES: This study aimed to explore the lived experience of mothers of children with haemophilia. METHODS: A descriptive phenomenological design was utilized. The participants were selected purposively from the Jordanian Association for Thalassemia and Hemophilia. Data saturation was achieved by interviewing 20 mothers. RESULTS: Five themes emerged: (1) the challenges related to the diagnosis, the availability and administration of the clotting factors, and the occurrence of the bleeding emergency; (2) physical, social, psychological and financial burden; (3) fear of child death and disability; (4) stigmatization; and (5) lack of educational and medical support. CONCLUSION: Mothers of children with haemophilia suffer from physical, psychological and social consequences. Healthcare providers should carry out educational sessions regarding the importance of support for the family and throughout the life of the child.


Assuntos
Hemofilia A , Mães , Feminino , Criança , Humanos , Mães/psicologia , Hemofilia A/psicologia , Jordânia , Medo , Ansiedade
8.
Haemophilia ; 29(4): 1056-1062, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-37291684

RESUMO

INTRODUCTION: Von Willebrand disease (VWD) is the most widespread congenital bleeding disorder. Caregivers are highly involved in its treatment, and from the time of the child's bleeding diagnosis, they face new demands such as recognition of bleeds and treatment options. AIM: The aim of this study was to assess Health related quality of life (HRQoL) in caregivers of children with moderate and severe VWD in Sweden, and to describe the impact of psychosocial aspects on the burden. METHODS: A multicentre, cross-sectional study. The Short Form 36 Health Survey (SF-36) was used to assess HRQoL. Caregiver burden was measured using The HEMOphilia associated Caregiver Burden scale (HEMOCAB). Children´s clinical data were collected from the Swedish national registry for bleeding disorders. RESULTS: Seventy caregivers of children with moderate or severe VWD were included. Caregivers of children with moderate VWD scored significantly lower in the mental health domains on SF-36, compared to matched normative data. Psychosocial aspects that significantly impacted the caregiver burden negatively measured by HEMOCAB total score were: if the caregiver reported that VWD affected their life in general (p = .001), if the child was absent from preschool/school ≥2 day/12 months due to VWD (p = .002) or that VWD had a financial impact on the family (p = .001). CONCLUSION: This study contributes to knowledge about caregivers' HRQoL and highlights the situation of caregivers of children with moderate VWD. Furthermore, the caregiver burden was negatively affected by psychosocial aspects. Clinical follow-ups should include assessment of psychosocial aspects to identify caregivers that are at risk of high burden.


Assuntos
Hemofilia A , Doenças de von Willebrand , Humanos , Criança , Pré-Escolar , Doenças de von Willebrand/diagnóstico , Qualidade de Vida , Cuidadores/psicologia , Estudos Transversais , Hemorragia , Hemofilia A/psicologia
9.
Blood Coagul Fibrinolysis ; 34(S1): S1-S4, 2023 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-37254721

RESUMO

People with haemophilia tend to experience pain from an early age because of venipuncture and hemarthrosis. If pain is not properly managed, it can become chronic and bedevil patients throughout their lives. Therapies are currently available that have been shown to effectively treat the different types of pain and their causes. Patients with haemophilia tend to experience either nociceptive or mixed pain. Identification of the cause of pain by means of imaging techniques, and understanding the characteristics, location and intensity of the pain, are essential for a more targeted therapeutic approach. Current data reveal that the most effective measures are administration of haematological and analgesic medication, intraarticular injections, and physical exercise. However, multimodal strategies such as lifestyle changes, physical modalities, psychological support and orthopaedic surgery can also prove of use. This article will look at the most effective analgesic measures used as a part of conservative clinical treatment. Collaborative studies are needed to better understand the nature of pain in the context of haemophilia.


Assuntos
Dor Crônica , Hemofilia A , Humanos , Hemofilia A/complicações , Hemofilia A/tratamento farmacológico , Hemofilia A/psicologia , Dor Crônica/etiologia , Dor Crônica/complicações , Analgésicos/uso terapêutico , Hemartrose/etiologia
10.
Blood Coagul Fibrinolysis ; 34(3): 138-143, 2023 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-36719804

RESUMO

Congenital bleeding disorders (CBDs), including inherited platelet function disorders and rare and common bleeding disorders, are a heterogeneous group of bleeding disorders with a wide range of clinical presentations, including psychological complications. Due to the chronic nature of CBDs, psychological complications are relatively common in these patients, which can affect treatment adherence, quality of life, and even the frequency of bleeding episodes. Chronic pain causes many psychological problems in CBDs, disrupting their social interactions, affecting all aspects of their lives, including their emotional functioning and behavior, and eventually leading to social exclusion. About one-third of patients with severe hemophilia A suffer from anxiety, 64% from depression, and 60% from other minor psychological complications such as withdrawal/depression, anxiety/depression, attention problems, and emotional problems. Anxiety, depression, and uncontrolled pain interfere with treatment adherence. For this reason, psychological interventions are needed in people with CBDs. Psychological interventions increase quality of life and treatment adherence. Therefore, early recognition of psychological complications in CBD patients may increase treatment adherence, leading to a reduction in bleeding episodes and thus an improvement in quality of life.


Assuntos
Dor Crônica , Hemofilia A , Humanos , Qualidade de Vida/psicologia , Depressão/complicações , Ansiedade/complicações , Hemofilia A/complicações , Hemofilia A/psicologia , Dor Crônica/complicações
11.
Haemophilia ; 29(2): 513-520, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36719261

RESUMO

INTRODUCTION: Mothers of children with haemophilia (CWH) experience guilt related to this genetic condition. Several factors contributing to maternal guilt have been identified, but the scope and extent of guilt have not previously been quantified. AIM: This study provides insight into the experience of mothers of CWH and how they perceive and manage guilt. It then identifies the most common and helpful coping mechanisms. METHODS: Between May and October 2021, we distributed an anonymous electronic survey to mothers of CWH. The Parent Experience of Child Illness measured maternal guilt, the PROMIS Parent Proxy for Life Satisfaction measured perception of their child's life satisfaction and additional questions explored specific guilt factors and coping strategies. RESULTS: Eighty-seven mothers responded to the survey. Forty percent of mothers experienced increased guilt. The most common reasons for guilt included putting their child through pain during infusions and passing on the affected X chromosome. Perceived life satisfaction, increased age and genetic counselling were associated with less guilt. The most common coping strategies involved utilizing social support, self-education and connecting with other mothers in the community. CONCLUSION: Some mothers experienced increased feelings of guilt, illustrating the need for providers to tactfully provide anticipatory guidance and counselling. Tangible manifestations of haemophilia were more likely to trigger feelings of guilt than familial factors. Community immersion was beneficial, as other mothers in the community served as a source of social and educational support. Most mothers did not report guilt, illustrating the adaptability and resilience of the haemophilia community.


Assuntos
Hemofilia A , Feminino , Criança , Humanos , Hemofilia A/psicologia , Mães/psicologia , Adaptação Psicológica , Culpa , Pais/psicologia
13.
Artigo em Português | LILACS | ID: biblio-1401356

RESUMO

A qualidade de vida é um aspecto importante a ser considerado no plano terapêutico de qualquer paciente, principalmente aqueles que sofrem de doenças crônicas, como a hemofilia. Diante disso, esse estudo teve como objetivo avaliar a qualidade de vida em pacientes portadores de hemofilia atendidos pelo Hemocentro de Alagoas e descrever o perfil demográfico e socioeconômico. Foram 50 pacientes envolvidos na pesquisa, maiores de dezoito anos e portadores de hemofilia acolhidos pela associação e pelo hemocentro de Alagoas. A coleta de dados foi realizada por entrevistas utilizando-se de dois questionários: um sobre características demográficas e socioeconômicas e outro sobre avaliação da qualidade de vida relacionada à saúde (Haem-A-QoL). Desse total, 100% eram do sexo masculino, em que a maioria tinha idade entre 20 e 40 anos (80%), solteiro (62%), residentes no interior de Alagoas (58%), aposentado (56%), beneficiários da aposentadoria por complicação da hemofilia (60%), com renda pessoal no último mês de até um salário mínimo (78%) e sem plano de saúde privado (86%). A média do escore total do Haem-A-QoL foi 40,08 (variação de 0­96,46) com pior desempenho nos domínios "esporte e lazer" (média igual a 55,52) e "saúde física" (média igual a 50,16) e melhor nos campos de "relacionamentos e sexualidade" (média igual a 17,48). De modo geral, os hemofílicos do centro estudado apresentaram boa QVRS. A versão brasileira do Haem-A-QoL, demostrou ser um instrumento confiável, com boa consistência interna, revelando alguns problemas enfrentados pelos hemofílicos e que impactam na QVRS (AU)


Quality of life is a critical aspect in the therapeutic plan of any patient, especially those who suffer from chronic diseases, such as hemophilia. Thus, this study aimed to assess the quality of life in patients with hemophilia treated by the Blood Center of Alagoas and describe the demographic and socioeconomic profile. 50 individuals were enrolled in the study, being over 18 years old and with hemophilia treated by the Association and the Blood Center of Alagoas. Data collection was carried out through interviews using two questionnaires: one on demographic and socioeconomic characteristics, and the other on health-related quality of life (Haem-A-QoL). Of this total, 100% were male, most aged between 20 and 40 years (80%), single (62%), living in the interior of Alagoas (58%), retired (56%), beneficiaries of retirement due to hemophilia complications (60%), with personal income in the last month of up to one minimum wage (78%) and without a private health plan (86%). The mean of the total Haem-A-QoL score was 40.08 (ranging from 0-96.46), with the worst performance in the domains "sports and leisure" (mean of 55.52) and "physical health" (mean of 50.16), and the best performance in the domain "partnership and sexuality" (mean of 17.48). In general, the hemophiliacs in the center studied reported good health-related quality of life. The Brazilian version of Haem-A-QoL proved to be a reliable instrument, with good internal consistency, revealing some problems faced by hemophiliacs that impact on HRQoL


Assuntos
Humanos , Masculino , Feminino , Adulto , Qualidade de Vida , Doença Crônica , Serviço de Hemoterapia , Hemofilia A/psicologia , Hemofilia A/terapia
14.
BMJ Case Rep ; 15(8)2022 Aug 29.
Artigo em Inglês | MEDLINE | ID: mdl-36038157

RESUMO

We detail the case of a man in his 80s who was a Jehovah's Witness, presenting to hospital for the second time in 1 week with atraumatic, severe bruising affecting his right thigh and flank. He was subsequently diagnosed with idiopathic acquired haemophilia A (AHA) and was urgently treated with recombinant factor concentrate and immunosuppressive therapy. Management of his bleeding disorder and resultant severe anaemia was adapted in line with his religious beliefs. AHA is a rare bleeding disorder which should be considered in patients with an isolated prolonged activated partial thromboplastin time and a history of recent or acute bleeding. Prompt diagnosis and management are essential as delays may result in increased mortality. Given that this patient declined blood transfusion, management of his bleeding disorder presented a unique challenge.


Assuntos
Hemofilia A , Testemunhas de Jeová , Idoso de 80 Anos ou mais , Transfusão de Sangue , Hemofilia A/complicações , Hemofilia A/diagnóstico , Hemofilia A/psicologia , Hemofilia A/terapia , Hemorragia/complicações , Hemorragia/terapia , Humanos , Testemunhas de Jeová/psicologia , Masculino
15.
Rev. cuba. hematol. inmunol. hemoter ; 38(2): e1589, abr.-jun. 2022. tab
Artigo em Espanhol | LILACS, CUMED | ID: biblio-1408458

RESUMO

Introducción: La hemofilia se caracteriza por la ocurrencia de hemorragias frecuentes y afecciones del aparato locomotor graves en los pacientes que causan daños físicos, sociales y psicológicos y afecta directamente su calidad de vida relacionada con la salud. Objetivo: Evaluar la calidad de vida de personas con hemofilia en el noreste de Brasil. Métodos: Estudio epidemiológico transversal cuantitativo realizado con 13 personas con hemofilia de un Banco de Sangre en el noreste de Brasil. La recolección de datos se realizó mediante la aplicación de un cuestionario sociodemográfico y clínico y de Haem-A-Qol, utilizado para evaluar la calidad de vida de las personas con hemofilia. Los valores brutos y medios se calcularon en general para todos los dominios y luego para cada dominio para que pudieran mostrarse. Resultados: Se obtuvo un promedio total de 30,94 en relación a los diez dominios evaluados. Los dominios con los promedios más altos fueron "salud física" con 49,23 y "afrontamiento" con 35,89 y con el promedio más bajo fue "planificación familiar". Conclusiones: Los participantes de la investigación tienen una calidad de vida insatisfactoria en relación con la salud física y el afrontamiento y, satisfactoria en relación con las relaciones y la sexualidad(AU)


Introduction: Hemophilia is characterized by the occurrence of frequent bleeding and serious musculoskeletal conditions in patients that cause physical, social and psychological damage and directly affect their health-related quality of life. Objective: To evaluate the quality of life of people with hemophilia in the northeast of Brazil. Methods: Quantitative cross-sectional epidemiological study conducted with 13 people with hemophilia from a Blood Bank in northeastern Brazil. Data collection was performed by applying a sociodemographic and clinical questionnaire and Haem-A-Qol, used to assess the quality of life of people with hemophilia. Raw and mean values were calculated overall for all domains and then for each domain so that they could be displayed. Results: A total average of 30,94 was obtained in relation to the ten domains evaluated. The domains with the highest averages were "physical health" with 49,23 and "coping" with 35,89 and with the lowest average was "family planning". Conclusions: The research participants have an unsatisfactory quality of life in relation to physical health and coping, and satisfactory in relation to relationships and sexuality(AU)


Assuntos
Humanos , Masculino , Feminino , Qualidade de Vida/psicologia , Bancos de Sangue , Adaptação Psicológica , Hemofilia A/psicologia , Hemorragia , Brasil , Estudos Epidemiológicos , Estudos Transversais , Serviços de Planejamento Familiar/métodos
16.
Medicine (Baltimore) ; 100(50): e27863, 2021 12 17.
Artigo em Inglês | MEDLINE | ID: mdl-34918635

RESUMO

INTRODUCTION: Literature provides unclear evidence of the effectiveness of many physiotherapy interventions on pain intensity, quality of life, and functional ability in hemophilic patients, and suggests that rehabilitation programs should be focused on functional goals and the disability of patients. AIM: The aim of the present study is to present the outcome of a case series of patients with hemophilia in which a rehabilitation program has been carried out on the basis of a specific individual patient's functional impairment. METHODS: Fifty-one patients were enrolled: 32 patients (Rehab Group) received a rehabilitative treatment, 19 patients for different reasons (living far from the hospital, family problems) did not receive rehabilitation (Control Group). The rehabilitation program was planned with respect to the emergent problems: musculoskeletal pain, joint range of motion limitation, muscle flexibility, walking difficulties, the appearance of hematomas/hemartro, and postural problems. All the patients were assessed at baseline (T0), after 1 month (T1), and after 3 months (T2) through visual analogic scale for musculoskeletal pain, the Hemophilia Joint Health Score for joint range of motion, the Functional Independence Score in Hemophilia for disability, and postural assessment by visual inspection. RESULTS: A significant reduction of pain and improvement of Hemophilia Joint Health Score and Functional Independence Score in Hemophilia score was found in the Rehab Group along with the follow-up. Pain in the Control Group was lower with respect to the other group at baseline and significantly decreased after 1 month. CONCLUSION: A rehabilitation program focused on individual impairment of patients with hemophylia determined satisfying results in terms of pain control, functional, and disability improvement in 3 months follow-up.


Assuntos
Terapia por Exercício/métodos , Hemofilia A/complicações , Dor Musculoesquelética/reabilitação , Adulto , Feminino , Hemofilia A/psicologia , Hemofilia A/reabilitação , Humanos , Masculino , Pessoa de Meia-Idade , Dor Musculoesquelética/etiologia , Dor Musculoesquelética/psicologia , Medição da Dor , Qualidade de Vida , Amplitude de Movimento Articular , Resultado do Tratamento
18.
Am J Case Rep ; 22: e929598, 2021 Apr 22.
Artigo em Inglês | MEDLINE | ID: mdl-33883542

RESUMO

BACKGROUND People with hemophilia A have shown osteomuscular complications that have a significant impact on their quality of life (QoL) and on health care costs. Patients with hemophilia A with inhibitors living in developing countries such as Chile face a high disease and treatment burden. Emicizumab, a humanized bispecific monoclonal antibody, is associated with improvements in QoL and reduction in the financial impact of the disease related to treatment. This case report describes the impact of emicizumab on a patient with severe hemophilia A with inhibitors in terms of breakthrough bleeding control, improvements in QoL, and reduced financial impact after a year of treatment, in a country where this medication is not routinely available. CASE REPORT A 10-year-old child with severe hemophilia A with inhibitors had several restrictions in his daily life due to multiple incidences of breakthrough bleeding. He was on episodic treatment with bypassing agents, and in the year prior to treatment with emicizumab he had 18 bleeding episodes. After 1 year on prophylaxis treatment with emicizumab, the patient had only 1 bleeding episode (94.4% of reduction), improved pain control (5-point reduction on the visual analogue scale), a decrease in the Hemophilia Joint Health Score from 39 to 19, the QoL perception increased by 86% on the standardized Haemo-QoL-kids, and a 70% reduction in treatment costs versus the costs of episodic treatment with bypassing agents. CONCLUSIONS After 1 year of treatment with emicizumab, this patient had substantial improvements in the evaluated parameters. Further investigations with emicizumab are needed to assess its possible effects on public health policies.


Assuntos
Anticorpos Biespecíficos/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Inibidores dos Fatores de Coagulação Sanguínea , Hemofilia A/tratamento farmacológico , Metrorragia , Qualidade de Vida/psicologia , Criança , Chile , Fator VIII , Feminino , Hemofilia A/psicologia , Humanos , Masculino
19.
Rev. gaúch. enferm ; 42: e20200097, 2021. tab, graf
Artigo em Inglês | LILACS, BDENF - Enfermagem | ID: biblio-1251775

RESUMO

ABSTRACT Objective To understand the experience of men in relation to the process of living with hemophilia. Method Qualitative study using the theoretical and methodological referential Symbolic Interactionism and the constructivist strand of Grounded Theory. Participated 12 men with hemophilia followed at a Blood Center in the south of Rio Grande do Sul, Brazil. Intensive interviews, genograms and field notes were used for data collection, with concomitant analysis, following the initial, focused, and theoretical coding stages. Results The theoretical model was built, which has as central category Adapting to (co)exist with hemophilia and the categories Knowing yourself and the illness: knowing how to deal, Adjusting life: taking care of yourself and Getting used to the illness: being almost normal. Conclusion It was revealed the experience of men who adapt to (co)exist with hemophilia and their actions and behaviors, aiming to preserve them away from bleeding, pain, and the risk of death.


RESUMEN Objetivo Comprender la experiencia de los hombres en relación con el proceso de vivir con hemofilia. Método Estudio cualitativo usando los referenciales teórico y metodológico Interaccionismo Simbólico y la línea constructivista de la Teoría Fundamentada. Participaran 12 hombres con hemofilia acompañados en un Banco de Sangre en el sur del Rio Grande do Sul, Brasil. Se utilizó para recolecta de datos entrevista intensiva, genograma y nota de campo, con análisis concomitante, siguiendo las etapas de codificación inicial, focalizada y teórica. Resultados Se construyó el modelo teórico, que tiene como categoría central Adaptando para (con)vivir con hemofilia y las categorías Conociendo a sí mismo y la enfermedad: sabiendo lidiar, Ayustando la vida: cuidando de sí y Acostumbrándose con la enfermedad: siendo casi normal. Conclusión: Se reveló la experiencia de hombres que se adaptan a (con)vivir con hemofilia y sus acciones y comportamientos, con el objetivo de preservarlos lejos del sangrado, el dolor y el riesgo de muerte.


RESUMO Objetivo Compreender a experiência de homens em relação ao processo de viver com a hemofilia. Método Estudo qualitativo usando os referenciais teórico e metodológico Interacionismo Simbólico e a vertente construtivista da Teoria Fundamentada nos Dados. Participaram 12 homens com hemofilia acompanhados em um Hemocentro situado no sul do Rio Grande do Sul, Brasil. Utilizou-se para a coleta de dados entrevista intensiva, genograma e nota de campo, com análise concomitante, seguindo as etapas de codificação inicial, focalizada e teórica. Resultados Construiu-se o modelo teórico, que tem como categoria central Adaptando para (con)viver com a hemofilia e as categorias Conhecendo a si e a doença: sabendo lidar, Ajustando a vida: cuidando de si e Acostumando-se com a doença: sendo quase normal. Conclusão Revelou-se a experiência de homens que realizam adaptações para (con)viver com a hemofilia e suas ações e comportamentos, visando preservá-los longe de sangramentos, da dor e do risco de morte.


Assuntos
Humanos , Masculino , Adulto , Pessoa de Meia-Idade , Autocuidado , Adaptação a Desastres , Hemofilia A/psicologia , Brasil , Teoria Fundamentada , Enfermeiras e Enfermeiros
20.
Blood Coagul Fibrinolysis ; 31(1S): S12-S14, 2020 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-33351496

RESUMO

The carrier testing, genetic counseling and prenatal diagnosis have become an integrated part of the comprehensive care for hemophilia, but quality of life and management of carriers frequently remain suboptimal. It is paramount to evaluate the physical and psycho-social impact of them as problematic physical, psychological and social situations are not uncommon. There are to instruct them about their hemorrhagic possible symptoms and condition for offering solutions that meet their needs and help them enjoy a satisfactory quality of life in all life stages.


Assuntos
Hemofilia A , Hemofilia B , Qualidade de Vida , Gerenciamento Clínico , Feminino , Triagem de Portadores Genéticos , Aconselhamento Genético , Hemofilia A/epidemiologia , Hemofilia A/genética , Hemofilia A/psicologia , Hemofilia A/terapia , Hemofilia B/epidemiologia , Hemofilia B/genética , Hemofilia B/psicologia , Hemofilia B/terapia , Humanos , Gravidez , Diagnóstico Pré-Natal
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