Circumcision in hemophilia using low quantity of factor concentrates: experience from Dakar, Senegal

Abstract

BackgroundCircumcision in hemophiliacs is a delicate surgery because of bleeding risks that could be avoided by adequate substitution of coagulation factor. This practice is very challenging in countries where anti hemophilic treatment is inaccessible. The study aimed to evaluate a circumcision protocol in hemophilia A using low quantities of factor concentrates.MethodsThis prospective study included 26 hemophiliacs A who underwent circumcision in 2014. Medical treatment protocol using low quantity of factor concentrates was drafted by physicians of the Hemophilia Treatment Center and the surgical protocol by experienced surgeons. Assessment criteria were: number of hospitalization days, number of exposure days to factor concentrates, delay to healing and occurrence of bleeding events.ResultsMean age was 9.6 years (1–30). Hemophiliacs patients were classified as severe (n = 8), moderate (n = 9) and mild form (n = 9). Mean number of exposure days to factor VIII concentrates was 6.9 days (5–12) in children and 10.75 days (7–16) in adults (p = 0.0049); mean number of hospitalization days was 3.68 days (2–10) in children and 13.5 days (13–15) in adults (p = 0.0000); delay to healing was 26.47 days (20–35) in children and 25.25 days (22–30) in adults (p = 0.697); five haemophiliacs (19.2%) presented bleeding events after the circumcision. The mean amount of FIII concentrates used per patient was 1743 IU (810–2340).ConclusionThe study shows treatment protocol using low quantity of factor concentrates is efficient in hemophilia patients who underwent circumcision.

Haemophilia in Côte d’Ivoire (the Ivory Coast) in 2017: Extensive data collection as part of the World Federation of Hemophilia’s twinning programme

“…Detection of haemophilia early in life is critical, as circumcision was major cause of bleeding, transfusion and death in our population. This situation is common in Africa where circumcision is the first surgery in young boys, mainly supported by religious, cultural and social reasons . Thus, promoting adapted educational programmes for PWHs, carriers and health professionals is mandatory to improve awareness of haemophilia and take appropriate preventive and therapeutic measures so as to avoid haemorrhages and their complications.…”

Hemophilia carrier’s awareness, diagnosis, and management in emerging countries: a cross-sectional study in Côte d’Ivoire (Ivory Coast)

“…These data highlight the lack of the carriers’ knowledge of hemophilia inheritance and their bleeding risks, along with the ignorance of healthcare providers concerning their increased bleeding risk and bleeding management. This highly-relevant unawareness about carrier condition has already been reported in studies conducted in India and South Africa [4, 16, 25].…”

“…In developing countries, carrier detection proves even more crucial for early hemophilia diagnosis in children, because they often die early in life due to the lack of diagnostic opportunities, absence of care facilities, and limited access to clotting factor concentrates (CFC) [15]. As circumcision generally represents the first surgical intervention in African boys with hemophilia, it is a major cause of bleeding and death [16]. Unawareness of carrier condition and bleeding risk, as well as healthcare givers’ ignorance concerning appropriate management modalities, are also major issues of concern in these countries [4, 17].…”

Unravelling the knowledge, beliefs, behaviours and concerns of Persons with Haemophilia and their carriers in Senegal

“…Only 16% of carriers expressed that circumcision was the main trigger to diagnosis of their sons, and this can be explained by the young age of their children, which consequently have not yet been circumcised at the time of their diagnosis. Circumcision represents an act of great religious value and social recognition for the boys and is performed in Senegal 10 . The failure of this step induces stigmatization and a social burden to carry, as expressed by the participants.…”