1. Presented by Jacinta Mc Aree-Murphy Health Promotion Co-ordinator Cavan/Monaghan Hospitals North Eastern Health Board Éire Holistic Approach to Managing Patient Care with Genetically Acquired Haemochromatosis

2.  What is haemochromatosis?  Who does it affect?  Why early identification is important? Outline of Presentation

3.  To build healthy alliances with our clients so we can provide a better quality service.  To empower local sufferers to affiliate to the National Haemochromatosis Society and self manage.  To work in collaboration with St James Hospital on a research project on the level of incidence in the area. Rationale

4. What is Haemochtomotosis?  It is a condition of iron overload due to abnormal regulation of iron absorption in the duodenum (gut).  It is due to inheritance of a mutated gene (HFE gene) which stops the body from correctly regulating iron intake.  Patients with HH continue to absorb iron from the diet despite excess stores.  Excess iron is toxic and may cause irreversible damage to body tissues and organs in which it is stored.

5. Who does it effect?  It is the most common genetic disorder in Caucasian (white) populations, concentrated in Celts and Nordic ancestry  Prevalence: 0.3 – 0.5% (1 in 200) in NW Europe 0.7% UK 1.0 – 2.1% (1 in 80) in Ireland  Two mutations in the HFE gene found in HH patients are C282Y (90%) and H63D (5%).  Carrier status (one copy of the mutation) is more common (1 in 10).

6. Why early identification is important? Early diagnosis represents a major chronic disease prevention strategy. (Reyes et al, 2003) If detected early it is easily treated. Prevention is better than cure.

7. Aim  To create opportunities to heighten educational awareness of this disease and prevention opportunities for family members in the community  To empower individuals to take responsibility for their own disease management and prevention strategies ably assisted by the day ward team.

8. Objectives 1. To deliver a quality patient centred nurse led service based on individual need. 2. To empower the ward multi-disciplinary team to educate themselves about the condition. 3. To develop a training package enabling nursing personnel to cannulate and provide a total care package within one year. 4. To build a healthy alliance with the laboratory.

9. Objectives Cont’d 5. To promote and develop relationships with the National Haemochromatosis Society and medical experts working in the field, to advance the knowledge and treatment of haemochromatosis. 6. To increase the public awareness of haemochromatosis, how it manifests, the destruction it can cause if untreated and how easily it can be managed.

10. Methodology  Staff in-service training to develop competencies in cannulation.  Comprehensive literature review.  Initial contact with Irish Haemochromatosis Society.  Elicit the commitment of and the co- operation of, hospital management, medical, nursing, allied health professionals and voluntary agencies.  Agree time-frame.

11. Methodology Cont’d  An awareness evening was organised to meet the needs of the target population.  Extensive advertisement via the print and radio media, in Dr’s surgery’s and in parish bulletins.  Information leaflets were designed in line with evidence based practice.   Treatment card was designed to enable individuals become familiar with their care management.

12. Outcomes Patient Stream line service 1 st National affiliated branch Haemochromatosis Society Invitation to participate in research St James Hospital Eire Equity in care delivery Patient empowerment Increase in patients knowledge of disease Nursing Practice Extended role for nurses Nurse led service Competencies in cannulation Protocols agreed & implemented. Service Computer links with laboratory Fast track results Quality of service improved. Direct access to lab via shute system

13. Evaluation  On-going evaluation of service.  On-going review of staffing requirement levels.  Compile final report and dissemninate to fellow hospital colleagues locally, nationally and globally.  Contribute to future research in the area.

14.  Day ward staff, consultants and patients. Acknowledgements  Nursing and Admin Management  Laboratory Personnel  Dr.Norris Consultant Hepathologist St. James Hospital  Annemarie Flanagan CNS Haemochromatosis Mater Hospital Dublin  Liz Ellis CNS Haemochromatosis St. James Hospital Dublin  National Irish Haemochromatosis Association - Margaret Mullett, Jim Fenton

15. Conclusion Go raibh maith agaibh go léir  All of the objectives were met within the 1 year time frame.  Process evaluation via focus groups indicate this is a worthy initiative.