Purpose: The purpose of this study was to explore adjustment pattern of illness process of people with hemophilia in Korea.
Method: 23 people with hemophilia had participated for this study. The data were collected through in-depth interviews and analyzed using Strauss & Corbin's grounded theory method.
Result: "would be free from" was emerged as a core category and it reflects that all participants wanted to be free from the constraints of the disease. The adjustment process was categorized into two stage, the 'unstable stage' and the 'stable stage'. In the process of "would be free from" four different patterns were identified: hopelessness type; appreciation type; challenge type; and transcendence type. These types were identified based on the degree of pursuing normal life and managing the disease, and social support. The most frequently occurring type was hopelessness type but the participants of this type suffered the most. The transcendence type was the most ideal type, but it occurred the least.
Conclusion: The results of this study indicate that people with hemophilia in Korea still suffer from the disease and they need supports. The results would be useful for health care professionals in establishing education and counseling program for the people with hemophilia.
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http://dx.doi.org/10.4040/jkan.2004.34.1.5 | DOI Listing |
Haemophilia
December 2024
Advanced Center for Oncology, Hematology and Rare Disorders (ACOHRD), K.J. Somaiya Super Speciality Hospital & Research Center, Somaiya Ayurvihar, Sion East, Mumbai, Maharashtra, India.
Introduction: Mortality and morbidity in persons with haemophilia (PWH) have decreased due to improved diagnosis and treatment along with comprehensive population outreach efforts, but the impact is not uniform in different countries.
Aim: The study aims to assess all-cause and intracranial haemorrhage (ICH)-specific mortality of PWH in India.
Methods: This is a retrospective, observational, multi-centric cohort study of 1020 haemophilia patients from three centres in India.
BMJ Open
December 2024
Department of Paediatrics, Nara Medical University, Kashihara, Japan.
Introduction: Emicizumab prophylaxis is approved for people of all ages with haemophilia A (HA) including infants and children. Although previous studies have demonstrated the efficacy and tolerability of emicizumab in infants with HA, real-world data on emicizumab use in infants are limited. The Haemophilia A in Infancy and NewbOrns: multi-instituional prospective observational study to assess the efficacy anD safety of Emicizumab (HINODE) study aims to evaluate the coagulation potential and safety of emicizumab prophylaxis in infants with congenital HA from birth to <12 months of age.
View Article and Find Full Text PDFAnn Hematol
December 2024
Shandong Blood Center, Shandong Hemophilia Treatment Center, Jinan, China.
Hemophilia A (HA) is an X-chromosome-linked recessive genetic disorder. Female carriers may have bleeding symptoms, but rarely have moderate or severe disease. We identified a female patient with moderate HA by pedigree tracking and genetic testing in a HA family involving consanguineous marriage.
View Article and Find Full Text PDFJ Thromb Haemost
December 2024
Department of Orthopaedic Surgery, The Second Hospital of Anhui Medical University, Hefei, Anhui, China. Electronic address:
Background: Hemophilic arthropathy (HA) is a joint disease characterized by local iron overload, stemming from erythrocyte rupture and closely linked to synovial lesions. However, the precise molecular characteristics of clinical HA synovial samples remain to be defined.
Objectives: To gain insight into HA synovial tissue lesions, we utilized a metalloprotein strategy to compare the metal and protein spectra of HA with those of osteoarthritis (OA) and rheumatoid arthritis (RA).
Clin Exp Med
December 2024
Discovery Research, Scientific Innovation Office, Grifols, Palou 3, 08150, Parets del Vallès, Barcelona, Spain.
Hemophilia A (HA) patients under emicizumab prophylaxis may require the concomitant use of procoagulant factors for breakthrough bleedings or immune tolerance induction (ITI). The aim of this study is to evaluate the ex vivo procoagulant effect of plasma-derived FVIII concentrates containing von Willebrand factor (pdFVIII/VWF) in samples from patients with severe HA without inhibitors on emicizumab prophylaxis. Samples from healthy controls (HC) and HA patients were drawn in sodium citrate plus corn trypsin inhibitor tubes and spiked with increasing concentrations of pdFVIII/VWF concentrates (10-400 IU/dL) (Fanhdi/Alphanate, Grifols), activated prothrombin complex concentrate (aPCC, 0.
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