CIRCUMCISION: MEDICAL AND CULTURAL CARE FOR A PERSON WITH HEMOPHILIA

Nairobi, Kenya

June 25, 2013

• 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

• 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

• 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

• 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

RISKS/COMPLICATIONS OF CIRCUMCISION

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

• 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

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

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

• 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

• 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

• 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

• 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

• 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

• 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

• 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

• 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

• 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

ANNE-LOUISE CRUICKSHANK Haemophilia Nurse CoordinatorWestern Cape South Africa

Acknowledgements:Anne Gilham, Mirriam Mokwena, Henry Steenkamp, and the Eastern Cape Department of Health

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