Introduction:In resource-constrained countries, few patients with haemophilia (PWH) have access to clotting factor concentrates (CFC), with increased musculoskeletal (MSK) complications. Physiotherapy actively contributes to preventing MSK complications, minimizing joint damage and reducing pain. Aim: To assess the impact of a 20-week self-and community-based rehabilitation (CBR) programme in Ivorian PWH. Methods: Fifty participants underwent a clinical and functional baseline assessment with identification of joints' functional defects and initiation of an individualized exercise programme comprising exercises to improve strength, joint mobility and proprioception. Hemophilia Joint Health Score (HJHS), 2-minute walking test (2MWT), Timed Up and Go (TUG), goniometry and maximal isometric voluntary contractions using the MicroFET2 were performed at baseline (T1) and at Week 20 (T2).Results: At T2, there was a significant improvement in both the 2MWT and TUG tests.The HJHS total score decreased significantly from 23.6 ± 14.2 at T1 to 20.4 ± 13 at T2. A significant improvement in joint health was found in the left elbow, right knee and right ankle, with elements correlating with joint function responsible for these improvements. A strong programme adherence was observed, with 94% of participants reporting regular exercise performance and a high degree of satisfaction. Conclusion:The programme with its encouraging results is meant to be the first step towards a more ambitious project. Self-based and CBR programmes are inexpensive and efficient treatment options designed to minimize the detrimental effects of joint and muscle bleedings, and to increase the functional independence and quality of life of PWH with limited access to CFC and physiotherapy. K E Y W O R D SCôte d'Ivoire, developing country, exercise, haemophilia, physiotherapy, self-and communitybased rehabilitation programme
Introduction In sub‐Saharan African countries, research on haemophilia is limited. Since 2015, a partnership has been established through the World Federation of Hemophilia (WFH)’s twinning programme between the haemophilia treatment centre (HTC) of the Centre Hospitalier universitaire of Yopougon in Abidjan, Côte d’Ivoire, and the Cliniques universitaires Saint‐Luc of Brussels, Belgium. Aim This study sought to collect accurate, and detailed demographic, clinical, and laboratory data on the whole identified Ivorian haemophilia population. Methods A prospective study was conducted in 2017 in Yopougon’s HTC. Participants were assessed through multidisciplinary workups including interviews, logbook review, pedigree establishment, clinical examination and laboratory testing. Results Data on 81 patients with haemophilia (PWH) (78 severe and moderate) were collected. Postcircumcision bleeding was the most common diagnosis reason (32%). Mouth bleeds and skin wounds accounted for 55.2% of bleeds. Pedigrees revealed 63 deaths in affected relatives among 33 families. Most PWHs (76.5%) were treated on demand, and 21% had never been exposed to clotting factor. Non‐substitutive therapies (tranexamic acid [43%], physiotherapy [11%] and DDAVP [0%]) were underused. Overweight was uncommon. Knees were the most clinically affected joints at the Hemophilia Joint Health Score. Inhibitors were present in 7.8% of previously treated PWHs. Conclusions This study highlights the value of simple, feasible and inexpensive tools to collect data in the Ivorian haemophilia population and provides the basis for developing and implementing locally appropriate strategies to improve screening, diagnosis, preventive care, treatment and education. It demonstrated the WFH twinning programme benefits for haemophilia care in the developing world.
Imatinib mesylate, showed encouraging activity in chronic myelogenous leukemia. However, there are few data regarding his efficacy and response monitoring in Sub-Saharan African patients. Our objective was to assess response to imatinib mesylate (Glivec) in Côte d'Ivoire patients with newly diagnosed Chronic Myeloid Leukemia (CML). From May 2005 to September 2009, we treated 42 patients (40 years; range 16–69) with Philadelphia chromosome (Ph+) positive in chronic phase CML with oral imatinib mesylate at daily doses of 400 mg. Overall survival (OS) and frequency of complete or major cytogenetic remission (CCR/MCR) were evaluated. At a median follow up of 32 (range 7.6–113) months, the CHR rate in our study group was 76%. A major CR was found in 19 patients (45%) with 17% and 29% complete and partial CR respectively. There were no significant differences in the incidence of major cytogenetic response by known prognostics factors. Median time to CHR was 8 months (range 0.4–25), and 16 months (range: 0.1–36) for CR. Projected 5-year OS rate was 72% (95%CI 42–88). We conclude that imatinib therapy sub-Saharan African CML patients is very promising and has favorably changed the prognosis for black African patients with CML.
Introduction In sub‐Saharan Africa, access to clotting factor concentrates (CFCs) is often extremely limited and published data on people with haemophilia on prophylaxis are almost not existent. Aims and Methods To assess the feasibility, barriers and outcomes of a low‐dose and low‐frequency prophylaxis with extended half‐life (EHL) recombinant Fc fusion FVIII and FIX in Ivorian children on a two‐year period in the setting of the World Federation of Hemophilia's (WFH) humanitarian aid programme. Results Twenty‐five boys with haemophilia were included. Mean (SD) age at inclusion was 5.6 (2.5) years. The median [range] follow‐up duration was 17 [11–24] months. Regimen of prophylaxis was 20 IU kg−1 1×/week in haemophilia A and every 10 days in haemophilia B. We observed a maximal reduction by 87.6% of the annual spontaneous joint bleeding rate and a slight decrease in the total HJHS scores (p = .047). Adherence problems related to parents' low education level and shortage in CFCs were the main issues to carry out the programme. Inhibitors occurred in 12.5%. Conclusion This study confirms the feasibility and efficacy of low‐dose and low‐frequency prophylaxis in young Ivorian children with haemophilia treated with EHL CFCs donated through the WFH humanitarian aid programme. This work also highlights the crucial role of adherence and the need for appropriate education to achieve prophylaxis. Finally, it reminds the paramount objective of achieving self‐sufficient, sustainable and available haemophilia replacement therapy for all worldwide.
BackgroundLittle data is available on awareness of hemophilia carrier condition or associated bleeding risk and management in Sub-Saharan African countries. This study sought to identify hemophilia carriers in Côte d’Ivoire in order to collect data on demographics, bleeding phenotype, and laboratory results. Another purpose was to provide Ivorian hemophilia carriers with counseling on their risk of bleeding and of having children with hemophilia. A 12-month prospective study was conducted involving Ivorian hemophilia carriers recruited trough pedigree analysis pertaining to 81 hemophilia patients followed-up at the Yopougon Hemophilia Treatment Center in Abidjan. They were assessed using in-depth interviews, pedigree analysis, and laboratory testing.ResultsSixty-one subjects comprising 27 obligate and 34 possible carriers were recruited. None had previously been assessed, with 64% unaware of their carrier status despite a familial history of hemophilia in 69%. The most frequently reported bleeding symptom was menorrhagia (31%). Prolonged bleeding was reported after vaginal delivery in 19.6%, post-surgery in 4.9%, and post-dental extraction in 4.9%. Only one carrier was treated with tranexamic acid, with no other hemostatic therapy recorded. The median (range) clotting FVIII was 0.85 IU/mL (0.24–1.90 IU/mL) and FIX 0.60 IU/mL (0.42–1.76 IU/mL) in hemophilia A and B carriers, respectively. HA carriers had a FVIII < 0.5 IU/mL in 12.5%.ConclusionsThis study highlights the need of implementing care for hemophilia carriers in developing countries, and the high value of pedigree analysis for carrier identification, along with the relevance of diagnosis, treatment, and education of carriers, families, and caregivers.
African Burkitt lymphomas (BL) are highly aggressive lymphomas mainly affecting children and young adults in Africa. This lymphoma was marked by its high sensitivity to chemotherapy in comparison to Sporadic Burkitt lymphoma. In this study, we evaluated the treatment response and survival of patients with CMA protocol. Eighty-five of the 105 children registered were evaluated for response; there were 46 boys and 39 girls, whose age at diagnosis ranged from 3 to 18 years (median 11 years), admitted to the Hematology National Teaching Hospital of Abidjan in the period 1998-2008 with a diagnosis of BL on histological review and who were given CMA chemotherapy with curative intent are included in this analysis. CMA protocol is a low intermediate regimen of 3 drugs [Cytarabin (ara-C), Methotrexate (MTX), and Cyclophosphamide] with CNS-directed treatment by intrathecal MTX, ara-C and corticosteroid. Fifty-five of 85 patients obtained CR after induction therapy and 10 after 3 supplementary cycle because of partial response. The overall complete remission was 76%. Fifty-three of patients were alive in first CR at a median survival rate period of 2 years (range 82 days to 9 years) and are continuously disease free from Burkitt lymphomas. Twelve patients relapse after CR and died of lymphoma progression. More than 32 patients died, as a result of lymphoma progression. Among the 32 dead, 10 were in Murphy stage IV and all the patients who presented bone marrow involvement died. The projected 5-year overall survival rate was 62%. In conclusion, CMA protocol shows the high sensitivity of African Burkitt lymphoma. This can be considered as a successful result for people living in poor socio-economic conditions with no health insurance.
Introduction Patient education is the cornerstone of the management of chronic diseases like haemophilia. The education of patients with haemophilia (PWH), haemophilia carriers and their families requires educational materials adapted to their socio‐cultural situations for maximum effectiveness. These tools are currently lacking in developing countries like Côte d'Ivoire. Aims We sought to develop educational materials adapted to the Ivoirian context, assess their short‐ and long‐term impacts on knowledge about haemophilia and evaluate the participants’ motivation and their satisfaction with the tools. Methods Following their elaboration, the materials were administered to 71 participants (37 PWH, 29 carriers and 5 fathers), whose level of knowledge was assessed before (T0), just after (T1), and 1 year following the intervention (T2). We evaluated, analysed and compared the scores at T0, T1 and T2 and evaluated motivation at T0 and satisfaction at T1. Results All participants significantly improved their skills at T1 (P < 0.001), maintaining a sustained and significant improvement at T2 in comparison with T0 (P < 0.001). In all participants, we observed a high degree of motivation towards improving their knowledge and a high degree of satisfaction with the materials. Conclusions Appropriate, culturally adapted educational tools focused on haemophilia are now available in Côte d’Ivoire. These materials will likely contribute to improving haemophilia awareness, to implementing screening, prevention and self‐management of the disease and to positively impacting the outcomes of Ivoirian PWH in the long term.
Introduction In Sub‐Saharan Africa, inhibitor prevalence data in people with haemophilia (PWH) are scarce, as are data on genetic or treatment‐related risk factors. Aims and methods We performed a prospective study on PWH from Côte d’Ivoire to collect data into inhibitor prevalence, create a database of haemophilia genotypes, establish correlations between inhibitor presence and genetic variants identified amongst Ivoirian PWHs and evaluate exposure to CFCs. Results The study included 54 unrelated participants (43 severe, four moderate, two mild haemophilia A and five severe haemophilia B). PWH were treated on‐demand with various product types for short periods, non‐intensively, and using low‐dose regimens. We reported similar distributions of intron 22 inversions (39.5%), point pathogenic variants (32.6%) and rearrangements in Ivoirian severe haemophilia A patients versus non‐African ethnic groups. The haplotypes H1 (29.6%), H2 (36.3%) and H3 (34.1%) frequencies in haemophilia A were consistent with results published on African populations. We identified eight new causal variants. An inhibitor was found in 12% of haemophilia A patients previously exposed to replacement therapies. Among PWH with inhibitors, 66.7% had a positive intron 22 inversion and 50% the H1 haplotype. Conclusion This study provides original data on molecular diagnosis of haemophilia, inhibitor prevalence and risk factors for inhibitor development previously associated with inhibitors in Côte d’Ivoire. The low inhibitor prevalence likely reflects the limited exposure to replacement therapy in Côte d’Ivoire. Further larger, multicentric and international studies are needed to gain more insight on inhibitor incidence and risk factors in African PWH.
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