PROPOSAL FOR
THE JOINT COMMITTEE ON HEALTH AND CHILDREN.
Date 8th April 2014
Organisation Background
Sickle Cell and Thalassaemia 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:
1
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
1. 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.
2. 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.
3. Encourage adequate support for research activities leading to improved treatment
and eventual cure.
4. Organize and/or participate in national and regional educational conferences to
promote awareness of SCD and Thalassaemia.
5. 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
1. 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
hospitals for early detection and proper treatment of haemoglobinopathies.
2
2. 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 related haemoglobin 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.
3. 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
4. Training Initiative
Members of SCTI living with Sickle Cell disease currently experience difficulties with regard
to their primary treatment in some 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.
3
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 nurses and 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.
5. 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 a need 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.
4
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, multiracial 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
www.sicklecellireland.ie.
5