Haemophilia | 2021
A survey on practice of circumcision in children with severe haemophilia in Eastern Mediterranean Region
Abstract
Dear editors The benefits of infant male circumcision outweigh the rare and generally minor potential harms of the procedure.1 Recent report from CDCwhich included 547 babies, of whom 82% had haemophilia A, out of which 70% were diagnosed owing to post-circumcision bleeding.2 Although correct surgical haemostasis can typically be achieved by the infusion of factor concentrate at the adequate dose and timing; the recommendation for surgeons is always to have local fibrin glue (FG) by their side.3 Circumcision can be performed by using diathermic knife which proved to reduce postoperative bleeding.4 Some of the patients had significant bleeding despite adequate factor replacement before and after the procedure.5–7 Kearney performed a survey of pediatric haematologists fromHaemophilia Treatment Centers (HTC) across the United States to better understand the attitudes towards the neonatal circumcision in patients with haemophilia8 A 15 questions survey was developed to try to identify common practice of circumcision for patientswith severe haemophilia in the EasternMediterranean Region (EMR) countries. The surveywas initially posted electronically through the web site of Hemophilia East Mediterranean Network (HEMNET) Forum II to the registered participants. Hard copy of the survey was distributed to all attendants of HEMNET Forum II. The survey was voluntary and anonymous, and participation implied verbal consent. A 15 min presentation to introduce the survey in a special session of the Forum addressing circumcision was followed by another 15 min to answer the survey. All responders who answered the survey were to have direct contact with haemophilia patients either Paediatric or adult haematologists. Nurses and patients were excluded while adult haematologists (n = 7) were allowed to respond to the survey. Three attendees from EU replied to the survey; but replies were not included in final analysis (annex 1). Descriptive data are presented as absolute counts and percentages. Statistical tests were performed using SPSS 16.0 for Windows (SPSS, Inc., Chicago, IL, USA). All 64 paediatric and adult haematologists from 16 countries; 32 centres (1-16 participants from each country; median = 2) answered the questions of the survey by choosing only the onemost appropriate answer.Haematologists were from Hemophilia Treatment Centers (HTC) across EMR countries three EU countries (replies were not included in analysis) attending HEMNET II congress in Cairo; November 2019; to better understand the opinions and attitudes of haematologists towards the management of circumcision in severe haemophilia infants and children. Only 25% (16 out of 64) answered the questionnaire online ahead of the forum and re-answered the hard copy; they had exactly the same choices. All 64 physicians from 16 countries (32 centres) who attended HEMENT II in Cairo completed the questionnaire. Demographics of participants were as follow; 62.2% of them had experience > 10 years, 50% were treating > 100 Haemophilia patients/year; with a nearly total of 5000 haemophilia patientswere followed-up regularly by the participants. Of the 5000 haemophilia patients almost 90 % of those above 5 years were circumcised. As for responders opinion about the procedure, 91% of respondents (n = 56/61) would allow this procedure while five (four adult and one paediatrician) did not approve it and considered it against medical advice. Almost all paediatricians allowed the circumcision, while adult haematologists were mostly against it, however they answered the survey not differently from those who allowed the circumcision. The most preferred age of circumcisionwas<2 years in 50.8%, 14.7%preferred to postponewith other surgery, while > 2 years in 34.4%. The most reported concern regarding circumcision in haemophilia patientswas the risk of development of inhibitor (n=34/61; 55.7%) followed by the concern for bleeding (n= 24/61; 39.3%). As regards the type of factor replacement used during circumcisionwaseither plasmaderivedproduct (ClottingFactor Concentrate [CFC] or SolventDetergent [S/D]Cryoprecipitate) chosen by62% (10out of 16), RecombinantCFCwas chosenby6/16 (37.5%) of EgyptianHaematologists respectively; while it was the preferred product in countries other than Egypt; 35/45 (77.7%). This can be explained either by availability or cost or concerns related to the increased risk of development of inhibitors when using Recombinant CFC in early exposure days due to participation of two Egyptian Hemophilia Treatment Centers in SIPPET study.9 Plasma derived products were chosen by almost two-thirds of Egyptians in contrast to almost to a quarter of non-Egyptian participants. Recombinant productwas chosen by nearly third of Egyptian participants in contrast to three quarter by others non Egyptian participants (Figure 1). Regarding the recommended factor dose, all respondents recommended at least one preoperative dose of factor replacement. Variability in postoperative doses was great, as 56.25%of Egyptian group (n= 9/16) did not usemore than one dose of postoperative factor replacement in contrast to 40.0 % (n = 18/45) of non-EgyptianEMRwho reporteduse ofmore thanone to less than four doses postoperatively.When circumcisionwas performed undermultidisciplinary consultation, patients received less postoperative replacement doses (mostly non-use to less than four doses). On the other hand some haematologists with limited experience of surgical CFC prophylaxis used more postoperative doses for fear of bleeding. It should be emphasised that the highest risk of developing inhibitors is observed