Knowledge, attitudes and nursing practices on hemophilia in the university

Introduction

Hemophilia is a bleeding disorder characterizedby deficiency of factor VIII (FVIII) (in the caseof hemophilia A) or factor IX (FIX) (in the caseof hemophilia B) (de Raucourt et al., 2022;Masson, 2017). It can be congenital oracquired. Congenital hemophilia is a sex-linkedrecessive disease (Arbour et al., 2022;Romaric, 2021). It is a rare but seriousdisease. It manifests itself as deep bleeding,notably hemarthrosis and hematoma, but alsoexternal hemorrhage (Natacha et al., 2022;Ramé and Thérond, 2023). According to theWorld Federation of Hemophilia, prevalence isestimated at around one case in 10,000 births. 

Hemophilia affects 1 in 10,000 people.Depending on the type of hemophiliac, it isestimated that 1 in 5000 boys will havehemophilia A and 1 in 30,000 boys will havehemophilia B (Goudemand, 2009). Annualglobal surveys conducted estimate the numberof hemophiliacs worldwide at 400,000 (Thabti,2017). Few cases of hemophilia have beenstudied in Black Africa. The first cases weredescribed in Southern Africa in 1940, followedby Central Africa in 1950 and West Africa in1967 (Diop et al., 2003). In Morocco, the studyinvolved 16 haemophilia patients collected atthe regional haemophilia reference center inthe orient paediatrics department from 2011 to2013. These included 15 type A hemophiliacsincluding 3 cases of severe hemophilia (20%),5 moderate hemophiliacs (33%) and 7 minorhemophiliacs (47%) and one case ofhemophilia B. The mean age of thehemophiliacs was 6 years and 2months +/- 3.7years with a peak between 5 and 10 years(Benajiba et al., 2014). 

In Benin, a study published in 1997 on 29haemophiliacs revealed 27 haemophiliacs A and02 haemophiliacs B. Patients ranged in age from16 months to 35 years, with an average age of19. Anti-haemophilic factor assays revealed76% severe forms, 18.5% moderate forms and7.4% minor forms (Boco et al., 1997). Sincethen, Benin has had no new statistical data onhemophilia, and considering WHO statistics,which estimate that one child in 10,000 suffersfrom hemophilia, 1,200 children in Benin sufferfrom this anomaly in a population estimated at12 million. At present, 125 cases are followed upin the Hematology Clinic of the Centre NationalHospitalier Universitaire Hubert K. MAGA(CNHU-HKM), and belong to the AssociationBéninoise de l'Hémophilie (ABH). The diagnosisof hemophilia is based on the assay of factorVIII by coagulant method associated with thedetection of a coagulation inhibitor on a mixturetest on a TCA and then the titration of thisinhibitor whose threshold of positivity is 0.6Bethesda unit (Le Cam-Duchez, 2015). Theoccurrence of this pathology also requires thesearch for an associated pathology in 50% ofcases acquired haemophilia occurs in a contextfree of any underlying pathology, several typescan be found associated pathologies:autoimmune diseases, cancers, dermatologicaldiseases, infections. Of course, there is also theassociation with postpartum (Le Cam-Duchez,2015). We are convinced, however, that lack ofawareness of this condition among the generalpopulation, and among healthcare staff inparticular, is a cause of early mortality amonghemophiliacs, and moreover explains the rarityof their presence in the various health centers.Nurses are at the heart of patient care, and inorder to contribute to improving the quality oftheir actions in the face of this disease in theClinique Universitaire de Pédiatrie et deGénétique Médicale of the Centre NationalHospitalier Universitaire Hubert K. MAGA(CNHU-HKM), it was opportune to direct ourstudy towards their knowledge, attitudes andpractices in this care. To this end, the aim ofthis study is to evaluate the management ofhemophilia among the nursing staff of theUniversity Clinic of Pediatrics and MedicalGenetics of this center.