The Joint Committee on Health and Children

PROPOSAL FOR

THE JOINT COMMITTEE ON HEALTH AND CHILDREN.

Date 8th April 2014

Organisation Background

Sickle Cell andThalassaemia Ireland CHY 20908.

SCTI is a charitable Organization set up and run by Sickle Cell and Thalassemia families, patients and volunteers to raise awareness and support for Sickle Cell and Thalassemia in Ireland, through educational workshops, seminars, media, printed information, promoting genetic screening, research and support for each other.

SCTI is bound and governed by its Memorandum and Articles of Association as its governing document, with registered address at No. 19 Belvedere Place, Dublin 1.

Since its official registration, the Organisation currently serves more than 50 actively registered members including adults with SCD, parents of children with SCD, and those with the Sickle Cell trait, and the rest of the target groups and immigrant communities who may use the services of the Organisation i.e. information needs.

SCTI works in partnership with other health agencies, medical professionals and other primary stake holders in attaining a positive outcome.

We work with major national organisations such as the Genetic and Rare Disorders Organisation (GRDO), The Irish Platform for Patients' Organisations, Science and Industry (IPPOSI) and the Disability Federation of Ireland(DFI), The Wheel, Cairde, as well as with international bodies such as Global Sickle Cell Disease Network (GSCDN), The UK Thalassemia Society, Sickle Cell Society UK, Thalassaemia International Federation (TIF) and National Human Genome Research Institute (NHGRI) to advance the interest of people with hemoglobinopathies.

Vision:

To implement proper awareness programmes that will promote better understanding of Sickle Cell and Thalassemia. We aim to improve the quality of treatment, patient support and to facilitate research.

Mission:

We aim to fight the growth of SCD and Thalassemia in Ireland by creating a proper support network between the patients, the community and the health professionals.

Our Objectives

To educate the target groups from different nationalities especially from Africa, India and Asia and the general public about the prevalence of Sickle Cell and Thalassemia in Ireland through information sessions, activities, materials, group discussions.

To encourage target groups to avail of genetic screening in order to decrease the prevalence of SCD and Thalassemia in Ireland. Sickle Cell and Thalassemia screening before considering marriage or starting a family. This will be of utmost importance in preventing the growth of Sickle Cell and Thalassemia cases in Ireland, and will enable both partners to make informed decisions.
Encourage adequate support for research activities leading to improved treatment and eventual cure.

Organize and/or participate in national and regional educational conferences to promote awareness of SCD and Thalassaemia.

Develop and promote the implementation of service programs that will be in the best interest of people living with SCD and Thalassemia, the families and carers

Who do we serve?

Individuals with Sickle Cell Disease and Thalassemia

Family members (immediate and extended) and friends of individuals living with Sickle Cell Disease and Thalassemia.

The health care providers – both medical and allied health professionals.

The community representatives- day care workers, teachers, employers and others

Our outreach and education activities are provided to emerging populations such as ethnically, multi-racial and linguistically diverse populations. These programs will be responsible for assessing gaps in educational materials. The community-based Sickle Cell and Thalassaemia disease projects aim to partner with the proposed state new-born screening program, hospitals and health care professionals to provide support to infants screened positive for Sickle Cell and Thalassaemia and their families. This project aims to work cooperatively with the Health Service Executive to implement a model program of Sickle Cell Disease carrier follow-up to include notification, extended family testing, counselling and education of affected individuals and families.

SCTI PROPOSALS TO THE HEALTH COMMITTEE

New-born Screening programme for all target groups, youths and adults.

Approval of the Sickle Cell Disease and New-born Screening program in all maternity hospitalsfor early detection and proper treatment of haemoglobinopathies.

Haemoglobinopathy Registry

SCTI seeks the authorization and access of a Haemoglobinopathy Registry and Surveillance System in conjunction with the Maternity hospitals, children’s hospitals that treat these patients, and the adult centres caring for the adult sufferers.

The goal of this initiative is to establish a Haemoglobinopathies Data System (HDS) that will be used to describe the epidemiology and clinical characteristics of SCD, thalassemia, and other relatedhaemoglobin disorders in Ireland.

Many of the immigrants come from areas of the world where SCD is prevalent and many of them may be carriers of the Trait and have never been screened for it. As more unscreened immigrants produce offspring amongst themselves and with members of other ethnic groups, the prevalence of SCD Trait and SCD is certain to rise within Ireland.

Therefore, aggressive outreach, education and voluntary testing efforts are needed to prevent the increase of SCD in the population.

This data can be used for research, information dissemination, policy decisions, and health care planning at the local, regional, and national level.

Access to Medical Cards

SCD also produces economic pain. It requires extensive access to and utilization of medical services, equipment and prescription medications. In many cases, employment is difficult to find and/or maintain. This creates high-stress cash flow management issues that bring their difficulties.

Patients with Sickle Cell disease or Thalassaemia must receive adequate and proper access to treatment immediately during crises. Adults without medical cards may find the high costs of medication for treatment of their condition a hindrance to avail of medical services in fear of medical bills which may lead to silent constant pain and possibly death.

Recommendation:

Approval of medical cards for people with Sickle Cell disease and Thalassaemia

Training Initiative

Members of SCTI living with Sickle Cell disease currently experience difficulties with regard to their primary treatment insome major hospitals in Ireland. There is still a significant amount of medical inexperience regarding the treatment and patient management protocols for SCD. Many doctors and nurses have never treated a SCD patient.

This is understandable because Sickle Cell and Thalassaemia is relatively new in Ireland and the number of adults who have SCD is low but unknown.

However it is not good that the patient who ventures into an emergency room while in the middle of a Crisis goes through such vigorous pain while the medical professionals try to find the cause of this pain.

Recommendation:

The need to improve medical provider knowledge must be addressed with vigour but doing so will require significantly more resources than are currently available.
Involved discussions with relevant medical Colleges to increase training on haemoglobinopathies for doctors and nursesand allied health professionals.
As SCD becomes more common in the Republic of Ireland, every hospital should be able to provide basic inpatient and outpatient care for local patients and all hospitals which have an emergency department and/or acute paediatric unit should be able to provide emergency care of acute sickle problems, most commonly severe pain.
However, children with this chronic condition should also have the benefit of specialised knowledge, full support services (including education, housing and welfare benefits) so that the quality of life is maximized for each patient and their family.

Social Services and Support Centres

As more people living with SCD join the Organisation (SCTI), there is two major similarities in common, isolation and lack of support and information about how to avail of support services for the sufferers.

Recommendation:

There is aneed for Sickle Cell and Thalassaemia community support centres that provide an information resource, support and advice to families, training for health and other professionals as well as genetic counselling and specialist nursing in the nearest future.
SCD and Thalassaemia should be considered as chronic illnesses with acute exacerbations that have far-reaching effects on education, family life, social integration and the emotional wellbeing of the child and family. It is important that all families feel supported and have access to specialist advice and treatment.

Note: During our on-going qualitative research study, SCTI has learned that when families are supported they are able to cope with their child’s condition.

OUR ROLE

Community Support and Care

• Educating and supporting patients and carers in self-management of their condition.

• Supporting the patients and carers in home management of milder sickle cell crises and supervision after discharge from hospital

• Education of and support to patients and carers in adherence with home medication

• Liaising with and facilitating access to community health services, social services, educational services, welfare and housing benefits for people living with SCD and Thalassaemia.

Note:

SCTI will maintain close collaboration with the hospital-based paediatrician/haematologist who is responsible for Sickle Cell and Thalassaemia care.

Encourage health promotion and healthy lifestyle advice and support.

Provide outreach and education activities to emerging populations such as ethnically, multi-racial or linguistically diverse populations.

Such programs will help in assessing gaps in educational materials of SCD and Thalassaemia in Ireland with joint support from the specialist haematologists and Health Service Executive.

THANK YOU.

Proposed by

Lora Ruth Wogu

Founder

Sickle Cell and Thalassaemia Ireland