Pauline Tully (Cavan-Monaghan, Sinn Fein)
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I thank the Ceann Comhairle for selecting this Topical Issue. Epidermolysis bullosa, EB, is an extremely painful and rare skin disease, unknown to many people. People living with EB are missing proteins that bind the layers of their skin together so any minor friction causes it to break and blister. It is a debilitating and excruciating disease, with no known cure. There are four forms of EB that can vary in severity but all carry their own life-limiting symptoms and life-long pain. EB can effect not only the skin but also a person's internal organs. It is an extremely complex condition that requires specialised care in many cases.

On average we have 300 people in Ireland with EB. Recently Members of this House heard from two powerful EB advocates during a briefing in the audiovisual room, Ms Emma Fogarty and Ms Liz Collins. Ms Fogarty spoke about her experience of being born with a severe form of EB. She provided a searingly honest and moving account of the ways in which her life has been affected by EB and of the pain and challenges she faces on a daily basis. Ms Collins gave a powerful and open account of what it is like to care for her daughter who has severe EB. She explained how this disease can have a profound effect on the physical and mental health not only of the person living with the condition but also on his or her family members.

During the same briefing we heard about a new report from University of Limerick, UL, and Debra Ireland, the patient group supporting people affected by EB. This report reviews the impact of EB on the quality of life and examines access to services and supports across five countries, including Ireland. The study shows that people with EB in Ireland have access to care through specialist clinics in Children's Health Ireland, CHI, at Crumlin and St. James's Hospital and through community health teams and while I wish to commend the work of all the dedicated teams, many families continue to face significant challenges in managing this difficult condition. In particular, the report from UL and Debra Ireland found that Ireland is lagging behind other countries by failing to provide a dedicated EB bandage scheme. It also found that challenges remain in the provision of home nursing care for people with EB, including the lack of contingency plans where nursing staff are unavailable and a lack of nurses trained in treating EB to replace those who move on. The report highlights the significant impact of EB on mental health and shows that people caring for a loved one with EB have a lower quality of life than the general population. It also found that the economic burden of EB is approximately €130,000 per year per person in direct and indirect costs.

In its 2024 pre-budget submission Debra Ireland called for an investment of €810,000 in mental health supports, outreach care and home care for people with EB. In relation to home nursing care, Debra Ireland is seeking ring-fenced funding as this would enable the provision of support that reflects the prevalence and impact of the disease, with resources being made available where a person lives. A ring-fenced fund would ensure that people with severe EB can access the essential and complex home nursing care they need, as those needs change over time. In addition, Debra Ireland estimates that €400,000 in annual funding would be required to run an EB-specific bandaging and dressing materials scheme in Ireland, similar to the one that exists in Australia.

Given the significant impact of this disease, will the Minister for Health commit to providing the additional funding required to meet the needs of people living with EB in Ireland?

Mary Butler (Waterford, Fianna Fail)
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I thank Deputy Tully for raising this really important issue. All of us in the House fully appreciate how devastating a diagnosis of a rare disease can be for patients, their families and carers. There is cross-party support for doing everything we possibly can to help patients who live with this awful condition on a daily basis. As Deputy Tully said, EB is a rare, life-long genetic condition which causes fragile, blistering skin. I fully recognise and appreciate the difficulties experienced by patients suffering from this painful condition.

There are approximately 300 patients in Ireland with EB, with around 60 new patients annually. Children with EB are cared for in the multidisciplinary clinics in CHI, Crumlin, while adult patients are treated at a multidisciplinary clinic in St. James's Hospital. Care for EB requires the involvement of many different clinical specialists to extend lifespan and improve quality of life. A considerable amount of work has been undertaken to enhance the availability of treatments for those suffering with EB. For example, in recent weeks the HSE published a rare disease care pathway for the diagnosis and multidisciplinary care of inherited EB.

This Government is fully committed to doing everything possible to assist people living with a rare disease. Significant progress has been achieved under the National Rare Disease Plan 2014 to 2018, including the establishment of the HSE national clinical programme for rare diseases and a national rare disease office. In February, the Minister for Health announced a plan to develop a revised national rare disease plan in line with commitments in the programme for Government. Initial work on the new plan has commenced and a steering group to progress the new plan is expected to be convened in the coming weeks. Furthermore, as part of the development of a new plan, a patient forum will be established to ensure that the plan meets the needs of patients and families affected by rare diseases.

On funding, following the announcement of the budget allocation for the Department, the Minister for Health, Deputy Stephen Donnelly, will shortly be issuing a letter of determination to the HSE outlining my priorities and funding levels for 2024. This will be the basis on which the HSE will draw up the national service plan. It is not until the conclusion of the process that a detailed breakdown of new development will be available.

I would also like, as Deputy Tully has done, to acknowledge the brilliant work done by Debra Ireland on behalf of patients and families impacted by EB. I take on board what Deputy Tully said about home nursing care - not so much the lack of training but where if one nurses moves on to another job, the continuation may not be there. As it is a rare disease, with 300 patients in Ireland suffering from it, I can understand the point Deputy Tully made about not everyone having up-to-date information on how to deal with. However, I will certainly bring her concerns back to the Minister. I am familiar with both EB and with briefings that Debra Ireland has given previously. It is great that we have people like Emma and Liz coming forward and talking out about how difficult it is with EB.

Pauline Tully (Cavan-Monaghan, Sinn Fein)
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I thank the Minister of State. Debra Ireland is an excellent charity. It provides brilliant support to people with EB and their families. As the Minister of State has responsibility for mental health, I want to focus for on mental health issues, a subject I mentioned in my opening remarks.

In a survey conducted by Debra Ireland in 2019, people living with or caring for someone with EB spoke about experiencing feelings of isolation, despair, loneliness, an inability to cope and helplessness when a child is in pain. The pain is excruciating. When Emma was in that day in the audiovisual room, when Debra Ireland was giving a briefing, she talked about how every day she goes through excruciating pain. It can take three to four hours for her bandages to be changed. When the bandages are removed, blisters must be burst, and then ointment and new bandages put on. Obviously, how long it takes to change the bandages depends on the severity of the disease, but it is pain every day. There is bound to be an impact on mental health. A number of international studies have also examined the psychological impact of EB. One such study found prevalence rates of 63.6% for depression, 45.5% for anxiety and a lower quality of life generally for all of those affected by EB.

Debra Ireland provides a grant for counselling and therapy to fill the gaps in the context of what is not provided by the mental health services. It said that the demand for the grant increased by 73% between 2021 and 2022. It is looking for an investment of €80,000 per annum towards a clinical psychologist based at St. James's Hospital. That would have such an impact because there would be a person dedicated to people with EB. I wonder if that is something that could be considered, when it is decided where budget allocations are going?

There was also an EB outreach nurse appointed to Children's Health Ireland at Crumlin but none has been appointed to St. James's Hospital for people who move into adulthood. That would make such a difference because an EB outreach nurse can deliver early intervention, for example, in the case of problem wounds, and therefore reduce the need for hospital visits to access treatment for wounds and possible infections. Again, even an investment in an EB outreach nurse would make such a difference to the people living with the disease.

I have already talked about the bandage scheme. Some of the people say that sometimes when they go to collect their bandages, they are the wrong size or they are not available, and they have to travel then to one of the hospitals to get them. These are small things that could make such a difference to the lives of people with EB and their families.

Mary Butler (Waterford, Fianna Fail)
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The Deputy has summed it up there. When one has a child living in pain every day, it is very difficult for parents. As stated, the care needs of people affected by EB are currently being treated on the basis of a two-centre approach delivered through Children's Health Ireland and St. James's Hospital. I take on board what the Deputy said in that the outreach nurse is available for Children's Health Ireland, but not for the adults. On the request for funding for a clinical psychologist, which is really important, the HSE service plan is currently being configured. We support various NGOs with over €100 million from the mental health budget, and I will certainly take a look at this. Young people and adults who are living with this difficult, lifelong genetic condition, which causes fragile and blistering skins, have it really difficult on a daily basis. They are dealing with it every day of the week, and it can be very difficult. I will bring the Deputy's concerns back to the Minister, and I will also look at the issues she has raised myself.