1. CIRCUMCISION: MEDICAL AND CULTURAL CARE FOR A PERSON WITH HEMOPHILIA Nairobi, Kenya June 25, 2013

2. Discuss the traditional and cultural aspects of circumcision Explain the reasons for and risks of circumcision in boys with hemophilia Provide guidelines and protocols for hospital and cultural circumcision Discuss how nurses can assist families with this traditional or hospital procedure OBJECTIVES

3. Circumcision is one of the oldest and most commonly performed operations in the world It is also a traditional procedure One in 7 males worldwide is circumcised Timing of circumcision is different depending on race and culture CIRCUMCISION AND PROTOCOL

4. Social and cultural integration of boys with hemophilia is essential With the availability of factor products, circumcision can be carried out on these patients The addition of tranexamic acid and fibrin glue enhance hemostatic management CULTURAL INTEGRATION

5. Some studies have shown that in circumcised males there are: −Lower rates of sexually transmitted disease and HIV −Lower rates of penile cancer −Lower rates of cervical cancer in partners Conflicting studies re: urinary tract infection Evidence of benefit supported by WHO However, many national health organizations suggest the decision should be made by the family and child, if he is old enough to declare MEDICAL REASONS FOR CIRCUMCISION

6. Pain Excessive bleeding Inadequate removal of the foreskin Meatal stenosis and ulcer Fistulas Loss of penile sensitivity Skin necrosis Amputation Septicemia Dehydration RISKS/COMPLICATIONS OF CIRCUMCISION

7. CIRCUMCISION IN BOYS WITH HEMOPHILIA Without proper management, boys with hemophilia are at risk of death from bleeding at circumcision camps. To ensure the safety of the patient, circumcision should be carried out as a surgical procedure −Performed by qualified medical professionals −Under hygienic conditions and factor cover The duration of factor substitution varies around the world, from 2-8 days In 1998, Martinowitz et al showed that using fibrin glue cut the use of factor products back to 2-4 days

8. Hospital circumcision Factor replacement for 3 days with the first day b.d Aim to keep level above 50–60% Fibrin glue at time of circumcision Tranexamic acid commenced 12 hrs pre-op and given orally every 8 hrs for 7 days Despite this 22% had some bleeding THE IZMIR PROTOCOL: TURKEY

9. Communication and liaison with all players: PWH and families Healthcare personnel Government coordinators Traditional surgeons and healers Traditional leaders and chief in the community THE HEMOPHILIA NURSE

10. Principles of care Procedure should be in tertiary institution knowledgeable re PWH Planned with the HCCC and relevant urology dept Coordinated by the hemophilia nurse coordinator and/or hematologist Factor replacement therapy to be ordered before procedure. Treat as for major surgical procedure Can be done as outpatient with LA if on established home therapy protocol Follow-up is essential HOSPITAL CIRCUMCISION

11. Must be seen by hematologist within last 6 months Need recent blood results, weight, etc. Do inhibitor screen Must not have had any recent infections or infectious wounds If dental procedure also required, can try to coordinate at the same time to save on factor budgets Can be done as outpatient or inpatient if PWH not on home therapy Order factor replacement therapy for whole procedure and write up pain medication e.g. panadene HOSPITAL CIRCUMCISION: PRE-OP WORK-UP

12. If under general anesthetic: Nil by mouth and routine pre-operative management Give factor as for major bleed 30 minutes prior to surgery Post-op treatment with factor BD after surgery for 2 days, then daily for 5 days Remove dressing and re-assess in clinic after 7 days Some PWH may need factor for a few more days post op. (depends on healing) HOSPITAL CIRCUMCISION: DAY OF AND POST-OP

13. Protocol for Circumcision in Eastern Cape Act No 6 of 2001 (EC) Needs to be utilized for circumcisions done at circumcision school These rules and regulations are in effect to protect the initiate CULTURAL CIRCUMCISION SCHOOL

14. The traditional surgeon needs to be qualified and have permission from the regulator authority to perform the procedure Must use appropriate tools to perform the operation Needs to have permission to have a circumcision school and also written permission from a recognized doctor to perform the procedure CULTURAL CIRCUMCISION: STATE REGULATIONS

15. Initiate needs to go via the correct channel Initiate must be seen by one of the hemophilia nurse circumcision coordinators Consent must be signed after consultation with traditional surgeon and initiate’s family members Treatment will be as for hospital circumcision Factor VIII or FIX must be obtained prior to the procedure and decision made of who will be responsible for the treatment of the initiate (dates of procedure, doses of factor, and practical application) TRADITIONAL CIRCUMCISION

16. Below age 21, parent or guardian must sign the consent Initiate must be 18 years old or 16 if requested by the parents The traditional nurse can visit the circumcision school to ensure good practice e.g. hydration, choice of instrument, infusion of factor, etc The medical officer can remove the initiate at any time if the initiate is not in good health CIRCUMCISION SCHOOL

18. Be sensitive to community’s beliefs, customs, norms, and other cultural issues in practice Be respectful and mindful of the customs Be understanding, supportive, and provide education to ensure informed and safe decision HEMOPHILIA NURSE’S ROLE

19. Circumcision in boys with hemophilia is possible, provided appropriate care and management We have to understand and be respectful of all the cultural beliefs and practices and the importance of circumcision to ensuring acceptance in certain communities PWH must be educated about the protocols available (hospital or cultural) SUMMARY

20. I Sasmaz et al. Circumcision and complications in patients with haemophilia in southern part of Turkey: Cukurova experience. Haemophilia 2012 18; 426 – 430 A single centre experience in circumcision of haemophilia patients : Izmir protocol. Haemophilia 2010; 16, 888-891 Rodriguez et al. To circumcise or not to circumcise? Circumcision in patients with bleeding disorders Haemophilia 2010;16, 272-276 OB Shittu, WA Shokunbi. Circumcision in haemophiliacs: the Nigerian experience Haemophilia 2001; 7, 532-536 Kavakli, Aledort et al. Circumcision and haemophilia: a perspective. Haemophilia 1998; 4, 1-3 REFERENCES

21. ANNE-LOUISE CRUICKSHANK Haemophilia Nurse Coordinator Western Cape South Africa Acknowledgements: Anne Gilham, Mirriam Mokwena, Henry Steenkamp, and the Eastern Cape Department of Health MERGER AVEC SLIDE 1