Joint Committee on Health and Children debate -
Thursday, 28 May 2015

Recognition of Deafblindness as Distinct Disability: Discussion

Acting Chairman (Deputy Mary Mitchell O'Connor)

I remind those present to ensure that their mobile phones are switched off as they cause serious problems for broadcasting, editorial and sound staff. Our second session this morning is a landmark session as it is an opportunity for committee members to engage with representatives of the deafblind community in Ireland who are making the case for the recognition of deafblindness as a distinct disability.

I welcome the guests in the Visitors Gallery. They are accompanied by a signer so the proceedings will be accessible to them. The broadcast of this meeting will also be signed. It will be the first public broadcast from the Oireachtas that is accessible to the deafblind community. I welcome all the guests this morning, particularly Mr. Liam O'Halloran, Dr. Derek Lacey, who is sitting behind the witnesses, and Dr. Paul Kenna, an expert on research into Usher syndrome and retinitis pigmentosa. Apologies have been received from Dr. David Keegan, who could not join us this morning because he is involved in surgery.

I welcome Ms Laura English, the service development officer with the Anne Sullivan Centre for Deafblind, Dr. Sandra Cummings from the Irish Society of Hearing Aid Audiologists, Ms Carol Brill, an advocate on behalf of the deafblindness community, and Orla Pearson, who is helping to ensure Ms Brill's technology is working for her. I ask the witnesses to make their case. I will allow a little leeway with time. I ask the members to speak very distinctly so they can be heard.

I advise the witnesses that by virtue of section 17(2)(l) of the Defamation Act 2009, witnesses are protected by absolute privilege in respect of their evidence to the committee. However, if they are directed by the committee to cease giving evidence on a particular matter and they continue to so do, they are entitled thereafter only to qualified privilege in respect of their evidence. They are directed that only evidence connected with the subject matter of these proceedings is to be given and they are asked to respect the parliamentary practice to the effect that, where possible, they should not criticise or make charges against any person, persons or entity by name or in such a way as to make him, her or it identifiable. Members are reminded of the long-standing parliamentary practice and ruling of the Chair to the effect that they should not comment on, criticise or make charges against a person outside the House or an official either by name or in such a way as to make him or her identifiable.

I call on Ms English to make her opening statement.

Ms Laura English

A dhaoine uaisle, I am honoured to be given the opportunity to address the committee today on behalf of people who are deafblind in Ireland. For the purpose of clarity, according to the widely accepted definition across Europe, a person is regarded as deafblind if his or her combination of vision and hearing loss causes difficulty with access to information, communication and mobility. The disability is also referred to as dual sensory impairment or dual sensory loss, and these alternative terms may be used throughout the course of this meeting.

Hearing and vision impairments create barriers to communication that can isolate people and jeopardise independence and well-being. When vision and hearing losses combine, a person's ability to overcome these barriers becomes even further limited because one sense cannot adequately compensate for the loss of the other. Deafblindness is not, therefore, deafness plus blindness; rather, it is a separate and unique disability that affects overall development, socialisation and communication. One plus one equals three.

The Anne Sullivan Centre was established in the late 1980s by a group of pioneering parents whose children were born deaf and blind as a result of congenital rubella syndrome. I am thankful that the State's commitment to ensuring the availability of the MMR vaccine has almost eliminated the incidence of rubella syndrome in Ireland. Currently, however, there are more than 80 different causes of deafblindness, including hereditary and chromosomal conditions, prenatal congenital complications, postnatal non-congenital complications and older age.

Deafblindness is a growing concern. Research has shown that the proportion of the population who experience a combination of vision and hearing loss is expected to rise dramatically as a result of the ageing demographic and increased survival rate for infants with severe disabilities.

In July of last year, I conducted research into deafblindness in Ireland with the assistance of a number of co-operating organisations. As part of the investigation, I requested a cross-tabulation of data from the Central Statistics Office. The data revealed that 1,749 people reported having a combination of blindness or serious vision impairment and deafness or a serious hearing impairment in the 2011 census. An additional 8,616 reported dual sensory impairment with one or more additional disabilities, bringing the total number of those affected to 10,365.

In 2001, the United Kingdom introduced for all local authorities Care and Support for Deafblind Adults and Children Policy Guidance. It recognised deafblindness as a distinct disability and required each local authority to record all people who are deafblind in their catchment area. It also instructed local authorities to appoint a senior level member of staff to take overall responsibility for ensuring that people who are deafblind receive an assessment of need and have access to appropriate services. Three years later, Irish MEPs joined their European counterparts in signing Written Declaration 2004/1, urging all members to recognise deafblindness as a distinct disability and acknowledge that having a distinct disability, deafblind people need specific support provided by people with specialist knowledge. This is a sentiment echoed by the UN Convention on the Rights of Persons with Disabilities 2006, which was adopted in full just last week by the European Parliament. It makes a clear distinction in Article 24 between people who are deaf, people who are blind and people who are deafblind.

Towards 2016 outlines a new and innovative approach to social policy. It focuses on the adoption of the life cycle approach, which places the individual at the centre of policy development and delivery at key stages in his or her life. The new directions policy and the congregated settings Bill also promote a person-centred approach to disability services that emphasises the need to ensure people with disabilities are supported to be active members of their communities. Despite this, fewer than half of the 100 deafblind people who participated in survey research with me reported they were engaged with support services. Those who were receiving services were predominately adults in full-time residential care who had additional disabilities, and children and young people in mainstream or special education.

Working age and older-age people living in the community or with the support of family were undoubtedly under-represented because of the difficulties associated with identification. Those who did participate in the research or who have since contacted our outreach service for support report having limited, if any, access to services.

Recognition of deafblindness as a distinct disability is essential throughout the life cycle. Children with a combination of vision and hearing loss require specialist education provided by people with expert knowledge of the disability. Opportunities for training, education and employment need to be made available to people who are deafblind, based on their unique dual sensory disability as opposed to the severity of a primary disability, as is currently the case.

People who acquire deafblindness later in life need clearly defined pathways to care that allow them to manage and adjust to their loss of vision and hearing and continue to participate actively in society. The majority of the over 10,000-strong deafblind population in Ireland rely entirely on services for the deaf and the blind. In effect, this means that people who are deafblind are divided into parts for support and assistance, without a dedicated professional to co-ordinate the diverse range of services currently available. Difficulties with access to information further limit a deafblind person's ability to access services.

In this regard, I commend and sincerely thank the Clerk Assistant of the Dáil and his colleagues in the broadcasting unit who worked tirelessly in recent weeks to ensure that today's proceedings were accessible to members of the deafblind community. The lengths to which they have been required to go to ensure that people who are deafblind are afforded the same opportunity to access parliamentary proceedings as other citizens of the State who do not have a disability are indicative of the challenges that the community faces. It should not go unmentioned that the Members present are involved in making history today as members of a parliamentary committee whose questions and comments will be accessible to the deaf community through sign language for the first time in the history of the State.

As advocates and members of the deafblind community, we urge the committee to recognise deafblindness as a distinct disability in Ireland. We ask the Government to acknowledge the need for dedicated professionals to be appointed to support those affected, to collect accurate data on the number of people who are deafblind and to co-ordinate the services that are currently available. If a person is profoundly deaf and totally blind, his or her experience of the world extends only as far as fingertips can reach. Such people are effectively alone if no one is touching them. To them, the members are invisible. A dhaoine uaisle, we would like a commitment from members today that, to them, these people are not invisible. Míle buíochas díobh.

Acting Chairman (Deputy Mary Mitchell O'Connor)

I thank Ms English. I invite Dr. Cummings to make her opening statement. Before she does, though, I wish to reiterate our thanks to Mr. Denis McKenna for organising the interpreters for this meeting. It is appreciated.

Dr. Sandra Cummings

I am Dr. Sandra Cummings. I am an audiological scientist at the Beacon Hospital and I represent the Irish Society of Hearing Aid Audiologists. As my colleague has mentioned, deafblindness is a singular impairment that can present concomitantly different degrees of vision and hearing loss. Recognising deafblindness as a singular impairment has a number of advantages for the patients involved as well as the clinicians whom I represent.

First, recognition is vital in terms of developing a care pathway for individuals with a bimodal disability or deafblindness. A care pathway is defined as "a complex intervention for the mutual decision-making and organisation of care processes for a well-defined group of patients during a well-defined period". The defining characteristics of a care pathway include an explicit statement of the goals and key elements of care based on evidence, best practice and patients' expectations and characteristics; the facilitation of communication among the team members and with patients and families; the co-ordination of the care process by co-ordinating the roles and sequencing the activities of the multidisciplinary care team, the patients and their relatives; the documentation, monitoring and evaluation of variances and outcomes; and the identification of the appropriate resources. Only once deafblindness is recognised as a disability can a care pathway be developed and implemented. The aim of a care pathway is, therefore, to enhance the quality of care across the continuum by improving risk-adjusted patient outcomes, providing patient safety, increasing patient satisfaction and optimising the use of resources.

Second, early and correct management of deafblind individuals is the responsibility of many professions and specialties. Otolaryngologists, audiologists and ophthalmologists as well as other specialists from the rehabilitation teams should co-operate closely to achieve the best results. From an audiological perspective, the liaison with the ophthalmology representation is particularly important when considering appropriate rehabilitation of the hearing impairment. It is important that the audiologist be made fully aware of the deteriorating visual acuity, as this has important implications regarding the decision of when to refer for a cochlear implant should the vision be deteriorating quicker than the decline in auditory acuity. Therefore, decisions on when to provide traditional amplification or when to refer for cochlear implantation should depend on the ongoing assessment of the multidisciplinary team.

Third, the recognition of deafblindness will allow for support at a national health care and remuneration level, particularly in terms of aids and appliances submissions and assessment of need submissions. This has particular relevance for hearing aid and cochlear implant assistive technology like FM systems, sound field systems or any assistive technology that enhances the deafblind individual's communicative needs.

Fourth, the appropriate training of professionals who specialise in the rehabilitation and support of the deafblind will be implemented and facilitated by having deafblindness recognised as a singular impairment.

Deafblind people need specific means of communication to have access to education, career opportunities, leisure and social life. Knowledge about the communication abilities and difficulties that a deafblind person has can guide public policies to create measures aimed at improving access to communication and information and, therefore, independence of deafblind people.

Acting Chairman (Deputy Mary Mitchell O'Connor)

I thank Dr. Cummings. I invite Ms Brill to make her opening statement.

Ms Carol Brill

A dhaoine uaisle, I thank members for inviting me to speak today about the urgency of recognising deafblindness as a unique disability.

For a moment, I would like members to experience what it is like to have dual sensory loss. Please imagine that there is a power cut in this room. All the lights go out. The microphones do not work. The Acting Chairman would call a recess to this meeting until power resumed. Members sit here in darkness and silence. They are unable to see people's facial expressions or movements and they struggle to hear what others in the room are saying. Now, please imagine being at a hospital appointment in this dark and silent world. That is the reality for people with dual sensory loss.

When I look at my colleague, Ms English, beside me, I can only see her eye. I cannot even see my hand. Without the support of my stenographer, I rely on lip-reading and my hearing aids. My sight and hearing are deteriorating because of Usher syndrome, a rare genetic disease that, after old age, is the most common cause of combined deafness and blindness. There is no treatment or cure. I have little hope of seeing my daughter's first communion next year. I have been told that hearing aids will be no use to me within the next ten years. This is what I am facing as an unemployed single parent to my seven year old daughter, Sara.

Since the age of four years, I have worn hearing aids. Recently, I received extremely high-tech hearing aids that allowed me to hear this world properly for the first time in all of my life. These hearing aids allow me to feel safer as I go out in Dublin when I need to be aware of the dangers, such as the quiet Luas and electric cars that I may not see coming near me. Sadly, they are unavailable to me on the medical card scheme because the hearing aids available through it fall far short of my audiological needs.

I struggled to find an audiologist who fully understood my needs as a person with dual sensory loss. Now in my 40s, I have luckily found an audiologist in the commercial sector. I know that not all people who are deafblind have this experience or can afford it.

At the age of 11 years, my parents were told by an eye specialist that I was going blind and that it would be best to send me to the boarding school for blind girls. Thankfully, they did not listen and were determined that I continue in mainstream schooling.

Throughout my teenage years I struggled with my sight and hearing, unaware of my diagnosis. When I was 21, I found out my diagnosis, that I would go blind due to retinitis pigmentosa. Unfortunately, despite all of the consultations and investigations by various medical professionals throughout my younger years, no link was ever made between my hearing loss and sight loss and no one ever mentioned Usher syndrome.

I persisted with my own research and during a visit to America I met a researcher who was able to tell me that most likely I had Usher syndrome type 2. Only this month, at the age of 45, I have been genetically diagnosed with Usher syndrome type 2a. This genetic diagnosis was only possible through the relationships I have forged with many of the international researchers working in the field. I could not avail of the services of the National Centre for Medical Genetics because it did not have the resources to undertake genetic testing of my disease.

All through my life, I have attended the eye specialist about my sight and an audiologist about my hearing. I do not have a professional monitoring the progression of my Usher syndrome. I have no one to help to predict and plan for the ongoing deterioration and the adjustments I need to make to continue as an active, contributing citizen and, above all, a mother. The Disability Act 2005 entitles children born after 2002 to have their health and education needs assessed and receive a statement on the services available to them. I remain excluded by this Act. There are no statutory guidelines in place for people like me. The life cycle approach advocated for in the policy Towards 2016 has been absent from my experience. As a working age adult who would love nothing more than to work, I am invisible.

My mantra in life is, "Do not focus on the problem, find the solution." I have researched audio equipment in order that I can continue to communicate with people via text and e-mail when my sight goes completely. I have explored the options of cane training, guide dog assistance, braille and sign language. All of the planning needed to meet my communication and mobility needs has come from my own initiative. It has not been easy using my own initiative. When faced with the prospect of losing my vision and hearing, sometimes it is easier to stay curled up in a duvet and avoid facing the world. I am lucky; I have my daughter to keep me strong, but not all deafblind people are as lucky as I am. The truth is that, despite all of my research, I am no clearer about whether I will be able to access basic health and social care services. I honestly do not know how I will make my way in this world for the rest of my life. In a place that proclaims to cherish all citizens equally, I feel invisible. There is no dedicated entry on the national physical and sensory disability database where my needs should be recorded. There is not a single case worker putting my needs and those of other dual sensory impaired people at the top of their priorities.

I see two possible trajectories for my life. My future is that the degeneration of my condition will make it difficult for me to be the great parent I want to be to my daughter. Already, finding employment is extremely difficult and I worry about becoming a prisoner in my own home for the rest of my life. I do not want my daughter to witness this. The other possible future is that, with the necessary supports, I will continue to parent effectively, continue to learn, grow and develop as a person and contribute my learning, knowledge and experience to others and society. I have a passionate belief in my own responsibility to do all that I can to make that second, brighter future possible, but I also recognise that our elected legislators play a vital role in shaping the world in which we live, move, hear and see. They have it in their gift to improve the health and social structures over which we often stumble. Those of us with deafblindness are not invisible. I ask committee members in earnest to positively consider our request to have deafblindness recognised as a unique disability. Please open the doors that will allow us to live and fully participate in this beautiful world alongside them. I am most grateful for their time and attention.

Acting Chairman (Deputy Mary Mitchell O'Connor)

I thank Ms Brill, Ms English and Dr. Cummings. Deputy Caoimhghín Ó Caoláin will be followed by Deputies Seamus Healy, Eamonn Maloney and Sandra McLellan and Senator Jillian van Turnhout.

Deputy Caoimhghín Ó Caoláin

I join the Acting Chairman in welcoming each of the delegates whom I thank sincerely for their contribution. I also thank those who have journeyed with them in support and are sitting in the Visitors Gallery where I hope they are comfortable.

There is no question that the contributions of Ms Brill, Ms English and Dr. Cummings have challenged us all as members and touched us all as human beings. I commend all of the contributions but Ms Brill's will be an abiding memory for me after today. They have touched not only our hearts but will also have touched all those watching. This is an historic opportunity and should be part of the broadcasting of the work of Parliament every day. Perhaps the ability to reach out to people challenged by blindness, deafness and, in this instance, deafblindness, is something we have not achieved. A clear distinction needs to be made in order that we will recognise that there are three very different circumstances to be addressed.

I have a couple of questions and perhaps an observation or two to make. I thank Ms English for her opening remarks and commend the work of the Anne Sullivan Foundation for Deaf Blind People which deserves great credit for all it has done. It is important to acknowledge that Ms English engaged with us in preparation for the meeting, on which I commend her. It will be of concern to everyone that a dramatic rise in deafblindness is expected in the years ahead, which Ms English attributed to the ageing demographic, meaning that I should be worried, with the increased survival rate for infants with severe disabilities. Are there projections in this regard? Will Ms English confirm that deafblindness can be consequential on a number of circumstances? In some instances such as in the case of Usher syndrome, it is, as Ms Brill pointed out, congenital.

There are other triggers for the advent of deafblindness in one's lifetime. If the delegates could tell us a little more about it, it would be welcome.

The delegates have put down a clear marker on recognising deafblindness as a distinct disability. I noted their engagement with the Minister prior to the commencement of the hearing and hope we, as the elected voices of the people, will be able, as we do on so many things, to unite in a common demand that this happen. The delegates have shared with us that in 2006 the UN Convention on the Rights of Persons with Disabilities echoed this sentiment in terms of this recognition and that only last week the European Parliament accorded full recognition. I must commend our colleagues both here and in all of the member states on this achievement and hope that very shortly we will be able to herald the fact that deafblindness has been formally officially recognised as a distinct disability among our population. That would be the direct consequence of the collective work of the delegates and their presentations.

On clearly defined pathways to care, I know from dealing with people with disabilities that one of the great frustrations is the period of time from a particular stage in the development of a disability to the realisation of the support the person needs. This is a hugely difficult time during which people's abilities are impaired. The word "ability" needs to be emphasised, not just "disability". Ms Brill has highlighted the fact that she has nobody working on guiding and giving her a sense of her particular needs as each year passes. Dedicated support services for people with deafblindness should be able to deal with this issue. It is very important that there be no time lapse between identification of a need and the introduction of services and supports. We need to be able to predict in advance and such services should be immediately accessible when the need presents. We need services to coincide with the identification of need. I ask the delegates to comment on this issue and, given her professional capacity, perhaps Dr. Cummings might like to offer her views on it.

I thank the Acting Chairman for her patience with me. In her contribution Ms English spoke about collecting accurate data for the numbers of people who were deafblind and co-ordinating the services available. She needs to up her game because it is the services that are not available that we really need to provide. This is the only little difference I have with the thrust of her opening remarks. The services available are simply not adequate enough. They do not meet the current or projected needs of Ms Brill's and those like her. This is not a criticism; it is only to say that in any other address I would up the game again and move to another level of demand.

Dr. Cummings has stated it is only with recognition of deafblindness that a care pathway can be developed. This goes back to the point I made to Ms English on the identification of need and about there being no delay in accessing services when the need presents. I invite Dr. Cummings to comment on this issue.

The detail of aids and appliances submissions and assessment of need submissions was not shared in any of the three submissions. As laypeople who, blessed as we are, are not challenged by deafblindness at this stage in our lives - we do not know what the future holds for any of us - will the delegates elaborate a little on what aids and appliances are available? Are there specific aids and appliances available rather than a combination of deaf and blind aids and appliances? Will they elaborate a little and give us a clearer understanding?

I again thank the Acting Chairman. There are no questions I can put to Ms Brill after her contribution; its clarity stands on its own merits. It is the most compelling case I have heard put to us in all my years as a member of the committee and these Houses. Recognising deafblindness is the only result of today's engagement that can be even contemplated. With the Acting Chairman's indulgence and given that we work well as a cohesive health committee, I propose that we write to the Minister about our shared support for extending this recognition which we urge as a cross-party, cross-Government, cross-Opposition committee. We make the case with one voice. I have confidence that the Minister would be responsive, as he would only be expected to be. What is most necessary are the supports for the journey through life when they are needed; it should not have to take years to receive them after they are identified. It must be contemporaneous.

Will Ms Brill tell us a little about the aids and appliances she herself has? Understandably, she spoke with great love and pride about her daughter. I have four daughters. Although I do not need its services, I also have a dog. Has Ms Brill ever considered the services a guide dog might offer? It may be unique or particular to people who are challenged by blindness. Would a guide dog play a role in her particular circumstances as somebody challenged by deafblindness? If she has not, why not? Is it something that could be examined? What other areas does she believe it would be useful for the system to address immediately?

Acting Chairman (Deputy Mary Mitchell O'Connor)

To help the committee understand Usher syndrome and about deafblindness will Ms Brill speak about why she mentioned the Luas? I would also like to speak about the cost of dogs and the money she receives in social welfare payments and difficulties in this regard. It needs to be said at this stage in order that members will understand more when they put their questions.

Deputy Seamus Healy

I welcome the delegates and those who have accompanied them. I also welcome those in the Visitors Gallery. As the Acting Chairman said, this is a landmark session and I am delighted that the deafblind community will be able to understand what is taking place.

All the contributions from all three witnesses are very important to ensure the committee members know exactly what deaf-blindness is and the witnesses have made a compelling case for recognising deaf-blindness as a unique disability. Ms Brill made a most powerful and touching presentation on behalf of the witnesses. I commend Ms English on the work she has done and the research she published in 2014, deaf-blindness in Ireland. I commend the Anne Sullivan Centre on the work it is and has been doing over the years in the area. The main focus of today's meeting is the question of recognition of deaf-blindness as a unique and distinct disability. As we heard in the presentations, it has already been recognised by the UK, EU and UN. I concur with Deputy Ó Caoláin that I am sure the committee will unanimously propose it to the Minister and Department. The committee works particularly well and is generally unanimous in its recommendations.

The number of people affected by deaf-blindness in my constituency, Tipperary South, took me aback. The census showed that 44 suffer from deafblindness and a further 246 have an additional disability. This is reflected in the national figures of 1,749 deafblind and approximately 10,300 with an additional disability. Do we know how many people will be affected in the future? It is important from the point of view of the services that need to be put in place. As Dr. Cummings said, the recognition of deaf-blindness as a unique and distinct disability is vitally important if we are to implement pathways to care and the various services needed by people with this disability. Ms Brill has clearly outlined the whole area of quality of life, or the lack thereof, of sufferers. If services are to be put in place, we need to recognise the disability and put in place pathways of care and recommendations for services that would need to be available. What sorts of services are available for people with this disability? Do the services reach all or most of the people affected? What services need to be put in place for the future including outreach and outpatient services and residential services?

Again, I thank the witnesses for their important and powerful contributions on the subject and I assure them that the committee and I will support their presentations and their call for recognition of deaf-blindness as a distinct disability.

Ms Carol Brill

I thank the committee for embracing our statement about why deaf-blindness needs to be recognised. I live on a social welfare payment called the blind pension. This is evidence that I am treated as a blind person, not a deafblind person or a person with dual sensory loss. It is well known that it is difficult to live on a limited income. Although I would dearly love to work, given the deterioration of my dual sensory faculties, it is very difficult to gain employment. I could not even work on a supermarket checkout. I would dearly love to be able to do so. Given the restrictions of living on a low income, I am unable to commit to applying for a guide dog. While the Irish Guide Dogs for the Blind centre in Cork commits to providing one with a trained dog, one is responsible for veterinary fees, a special diet for the dog, grooming and compulsory pet insurance. It comes to approximately €25, a very large proportion of the blind pension.

I have worn hearing aids from the age of four. At first, the HSE provided my hearing aids. Unfortunately, as my hearing is deteriorating, my audiological needs are increasing, and the hearing aids offered through the medical card scheme are not sufficient to my needs. Thankfully, technology has progressed and in the commercial sector I am able to avail of high-tech hearing aids. The latest hearing aids I am wearing come at a very significant cost of approximately €6,500. I have been gifted these hearing aids because they are the best for my audiological needs and visual safety because I can use an app on my iPhone to adapt to my environment. If the environment is noisy, I can change the setting. My privacy is also protected. Visually impaired people use voice technology on their phones that reads messages and e-mails aloud. The iPhone app allows e-mails and messages to be read, via Bluetooth, straight into my hearing aid, which protects my privacy to some extent and ensures I am able to hear what I am engaging with. Unfortunately, my hearing loss is getting worse.

I ask Mr. Derek Lacey, who is here as my audiologist to expand a little bit further about the future prognosis of my hearing loss because it is essential to the progression of my care under the health and social services.

Acting Chairman (Deputy Mary Mitchell O'Connor)

I will have to get advice here. I ask Mr. Lacey to move up to the microphone.

Mr. Derek Lacey

Ms Brill suffers from Usher syndrome a sense and oral hearing loss, which we can say is age related. As time goes on her understanding of speech gets worse; it is clarity issues for her. As members will have noticed Ms Brill has participated in this meeting quite well. She has been able to read. She has been able to hear and able to converse with all present. This is because of the sense of the device she is wearing. It is able to take the speech and reduce it by compression to give the high frequencies more information so she is able to distinguish what is actually being said.

Ms Carol Brill

I ask Mr. Lacey to speak about the cochlear implants.

Mr. Derek Lacey

Moving forward with Carol, we have had to look at a decision in the past couple of months because her hearing loss is getting worse. We cannot give an estimate of how long she can maintain the level of loss she has. However, we are starting to prepare her for the cochlear implant. It is getting her to understand what is required and what she will have to achieve to get there.

Acting Chairman (Deputy Mary Mitchell O'Connor)

I thank Mr. Lacey.

Some other questions came up about statistics that are not known, including the numbers of deaf-blind.

Ms Laura English

I thank Deputies Ó Caoláin and Healy for their support on the issue both at the meeting and in advance of today in taking time to speak to us beforehand in order to get a good grasp of the issue.

I mentioned that a dramatic rise was expected in the number of deaf-blind people in Ireland and elsewhere. There is no Irish context-specific information on that. However, a UK study in 2010 used different data collection methods, including household budgets and some of the population census material to assess the number of people. It estimated that at present, 210 people per 100,000 are affected by deaf-blindness in the UK. With the ageing population the UK expects that number to rise to 343 per 100,000, which is quite significant.

On the other end of the spectrum, due to medical advances a greater number of children are surviving prematurity and from different complications, postnatal and prenatal, and are living but they are living with additional and severe disabilities. A number of the children who were involved in that research were from places that look after children with brain injuries and different multiple disabilities. Part of that multiple disability would be deaf-blindness.

On pathways to care, I mention again the lifecycle process we mentioned at the beginning. Pathways to care need to be accessible at all ages throughout the lifecycle. Dr. Cummings can elaborate on that based on her professional experience. Access to information is a huge issue for people who are deaf-blind. HSE leaflets in doctors' surgeries are not accessible to members of the deaf community who use sign language. Their first language is Irish sign language and to understand and really grasp all the services available to them in a second language is difficult. For people who are blind, material is often not available in Braille. The deaf-blind community in general have difficulty with access to information, which needs to be addressed to ensure they are aware of the different services available to them and the different pathways to care they can use and the routes they can access.

In terms of data collection, I have quoted figures from the national census from 2011. As Ms Brill has said, we would like to see a specific box to tick on the census that would indicate deaf-blind as opposed to ticking deafness and blindness. It should be clear that dual sensory loss is a distinct disability. We would also like to see that reflected in the national disability database, physical and sensory. There are issues on that. I have asked them to give us figures on how many people on the national physical sensory disability database at the moment experience a combination of vision and hearing loss. Even though it has been established since 2002, the numbers were below 500, which is quite stark considering that there are more than 10,000 people on the national census. The Health Research Board that runs it would advocate for people over the age of 65 to be included in that data collection which is not the case currently.

The register is done by voluntary participation. Perhaps if that were rolled out more through community voluntary organisations and the voluntary participation was stepped up a gear it might have the resources to collect more accurate information on the number of people who are deafblind in Ireland.

We were asked about what technology might be available. I attended the international Acquired deaf-blindness Network Conference held in Belfast last year. I saw there a number of deaf-blind people from across the world using different forms of communication which are not as readily accessible in Ireland. A number of people use tactile communication and sign language. If they had already acquired sign language earlier in life and then lost their sight they would use sign language into the hand. There are a number of different methods. Some would include each finger being a vowel, a, e, i, o and u, and the consonants would be in the palm. A speech could be spelled out to them in their hand. At the moment plenty of sign language classes are available in the country but not many people can help a deaf person to convert that conventional sign language into a tactile hands-on communications system. We would also like to work on that.

Last year one of our events was attended by the only deaf-blind priest in the world. He communicates through a Braille system that works through his iPhone. He will enter a message via Braille, send it off in an e-mail and will receive it back on his Braille machine. There is also a Lorm glove, which works on the concept of the manual alphabet on one's palm, but somebody can send one a text message and wearing the glove it will vibrate in certain parts of one's palm. Those kinds of technological devices are available and information on them can be found through Google. However, there is no information for deaf-blind people to find those kinds of devices in an Irish context.

I ask Dr. Cummings to elaborate on the pathways to care.

Dr. Sandra Cummings

Care pathways are the route map patients will have from when they are diagnosed. What is the route they will take? Who are the people involved in assessing them? How often will the assessments take place? What other professional bodies will be involved? Who will provide educational support? It requires getting these people in touch with the patient and following them step by step so they are never left in the lurch.

We should know at any point in time what the visual acuity is like and what the heating ability is like. Where do we need to up the game? Do they need better hearing aids? Going into the classroom, do they need classroom support? There has to be a designated care pathway that this patient will take. That is their routine life. What are the stops along the way? Who will help them along the way? That has to be formalised because at the moment people are just veering and not coming back to the central pathway of the aim of how we need to help these people in every aspect of their lives.

For example, with the assessment of need, someone with a mild hearing loss cannot really get teaching support. However, there should be extra teacher support in the classroom for someone with a mild hearing loss and a visual impairment. That is one key aspect where this should be recognised as a single disability.

We do this all the time. A person with mild hearing loss will not receive extra support. Someone whose hearing is fine may be okay but if he or she has another disability, it will make things much more difficult in the classroom setting. The most important issue to recognise is that we can work out the care pathway this individual needs to ensure he or she does not get lost along the way.

Deputy Eamonn Maloney

I apologise for being slightly late. I was attending to business in the Dáil.

It is extraordinary that we are discussing the recognition of deaf-blindness as a distinct disability given that lobbying has been taking place on the issue for many years. Earlier this week, while checking something about previous Ministers for Health, I was fascinated to note the lack of progress on this issue. People may disagree but in my view politicians should hang their heads in shame because some of the issues raised by the witnesses were being raised as far back as 1967. Ms English used the word "invisible" to describe deaf-blind people. That description sums up the position regarding efforts to have deaf-blindness recognised as a distinct disability for a long period.

Despite what one hears, Ireland is a country of substantial wealth. The issue is one of wealth distribution. If that were not the case, the witnesses would not need to come before the joint committee seeking basic changes. It is extraordinary that, having been discriminated against by nature, deaf-blind people continue to be discriminated against by society.

I agree with the sentiments expressed by Deputies Healy and Ó Caoláin. It would be a positive outcome of today's work if we were to move in the direction of finally securing State recognition for deaf-blindness. The least members can do, collectively and irrespective of our political differences, is move in this direction.

Senator Colm Burke

I thank the witnesses for their presentations and apologise for my late arrival. I had a prior commitment to raise an issue in the Seanad. However, I have read the presentations.

The issue of deaf-blind and deaf people who require full-time care arose three or four years ago when we discussed deaf-blind and deaf people who are in care facilities that lack communication and co-ordination. They must be in facilities that offer support. Have consultations or discussions taken place with the Health Service Executive on the need to co-ordinate services?

Ms English stated the deaf-blind population numbers approximately 10,000. A certain proportion of deaf-blind people living at home with family members will require full-time care at some point in future. We need to plan for our long-term elderly care needs. The number of people aged 65 years and over will increase from 585,000 to 990,000 in the next 15 years. I am concerned at the absence of planning for this scenario, including in respect of the cohort of deaf-blind people. Has there been any consultation or discussion with the HSE on co-ordinating services, especially for deaf-blind people as they get older? If a deaf-blind person is accommodated with 50 people who are not deaf-blind, he or she will not have any support.

I thank the witnesses for their comprehensive presentations.

Acting Chairman (Deputy Mary Mitchell O'Connor)

Deputy Buttimer, the Chairman, and Deputy Conway were unable to attend today as they both have hospital appointments.

Representatives of Fighting Blindness are in attendance. A strategy has been adopted to try to pull together the various organisations involved in this area. Dr. Paul Kenna and Mr. David Keegan are involved.

I have a particular interest in this issue as I have a family member with Usher syndrome, a genetic condition that may affect more than one member of a family. I ask Dr. Kenna to provide a brief outline of the syndrome in terms of its genetic role and research being done in this area. I met Dr. Kenna 25 years ago and I am aware that research in this area has made significant progress. From my experience of having a family member with Usher syndrome, I can say it is hell on earth. Families do not know who to turn to for help when a family member loses his or her sight. They will call this, that or the other group because there is no co-ordinated service available.

The lack of regulation for the provision of hearing aids is a serious problem. People are paying sums of between €4,000 and €6,000 for hearing aids which are sometimes substandard and do not help the person with the hearing difficulty. This issue needs to be addressed because some people are spending all their money on these products. This industry must be regulated. I am not referring to audiologists but to companies which are setting up shop in various towns and cities and are not subject to regulation. These companies can put whatever price tag they wish on a hearing aid.

Isolation was alluded to but depression is a major problem among deaf-blind people. Perhaps Dr. Kenna will address the issue of suicide ideation because it needs to be discussed.

Ms Brill referred to public services, including the Luas transport mode. The colour of Luas trams the fact that they are extremely quiet mean it is difficult for deaf-blind people to see or hear them. Public bodies must address these types of issues.

The witnesses made the point that high technology hearing aids are not available through the HSE. We need to consider that issue seriously because it is a huge investment and I understand the hearing insert in the ear needs to be changed every six to 12 months, which also costs money. Support services are limited in this area. The Ann Sullivan Centre is doing its best but doctors should be trained in dual multiple sensory impairment. I have experience of accompanying a deaf-blind person on visits to doctors and consultants who ignored the patient and directed their questions at me. It is important that the dignity of the deaf-blind person is respected. I would like to see more audiologists and experts in Usher's syndrome being trained in how to deal with blindness, as well more ophthalmologists being trained to deal with hearing problems.

Ms Laura English

I will bring together several strands arising from the questions on pathways to care. I spoke about co-ordination of services because there is a shortage of deaf-blind specific services, and that stretches the capacity of other organisations. Clearly we want deaf-blind specific services but we also recognise that a number of deaf and blind services are doing fantastic work. For example, Fighting Blindness offers a counselling service and there may be opportunities to pool resources by adding a sign language interpreter to its counselling services. The way forward will be through co-operation with other organisations that are already providing services to people who are deaf-blind while also working to develop deaf-blind specific services.

In regard to schools, we have a person working in our outreach service and a number of our staff are trained to masters level in deaf-blind communication. Currently they are finding it difficult to get places on multi-disciplinary teams. A multidisciplinary team might comprise a physiotherapist, an occupational therapist and a speech and language therapist but none of the team will have specific deaf-blind specific expertise. We want to reach the point where deaf-blindness is recognised as a distinct disability requiring a professional with deaf-blind specific knowledge. We have also started to roll out training and support in this regard. I have been in contact with UCD and gave a speech to nurses who will be graduating next year on dealing with a deaf-blind person in a hospital setting. This training needs to reach a wider audience, including ophthalmologists and audiologists.

In regard to the deaf-blind specific services provided in Ireland, the Ann Sullivan Centre provides residential care to people who are deaf-blind and have additional disabilities. The majority of these are intellectual disabilities, which we are not confident were always present rather being due to sensory deprivation from an early age. Full-time residential care is not necessary for everybody. As Ms Carol Brill demonstrates, a number of deaf-blind people do not have additional disabilities but they need to be supported in living as independently as possible in their communities. Senator Colm Burke asked about elderly care and access to residential care. A cohort of the deaf-blind population will need the residential support we currently provide but there is a lack of support for people who want to remain in their homes and close to their families and friends. If Ms Brill had somebody who could support her in simple tasks, such as shopping or cooking, it would make a massive difference to her life as her condition progresses. We need to enable people to live in their homes.

Ms Carol Brill

It is sometimes assumed that those with deaf-blindness automatically become cared for but I am a vibrant and passionate person, and the fact that my eyes and ears do not work very well does not prevent me from contributing to society. I want to be able to do that. Recognition of deaf-blindness will help me to make that dream come true. It is not my dream to end up in residential care; it is my dream to be a vibrant citizen who lives a full life despite hearing and sight loss.

Acting Chairman (Deputy Mary Mitchell O'Connor)

I ask Ms Brill to speak about hearing aids. While she may not have experienced particular problems because she has a good consultant, what is the experience of the deaf community and the deaf-blind community of hearing aids?

Ms Carol Brill

I am involved with a network of people who are suffering dual sensory loss, mainly due to Usher's syndrome. The most common complaint pertains to the quality of hearing aids available through the medical card scheme. It is not simply a matter of being unable to hear properly but also the impact on families. It can cause friction between family members when they are asked to turn around to facilitate lip reading. If the hearing aids were better quality, that friction would not arise. Poor quality hearing aids impact on every aspect of family life. We want to live in harmony. I have tried various hearing aids throughout my life. In my first 40 years of life, I never had an audiologist explain to me what my hearing loss entailed and why I needed to wear a hearing aid. I never understood the extent of my hearing loss until I attended a commercial audiologist who was kind and patient in explaining every aspect of my hearing loss. That should have happened before I reached the age of 40, which was five years ago. I should not have gone through life with that experience.

Social infrastructure, such as the Luas, can be detrimental. There is no care or thought for people with deaf-blindness. For some reason, Dundrum Town Centre, which is my local shopping centre, has erected silver and grey posts on grey concrete. The streets are covered with grey concrete and silver street poles. The Luas is supposed to reflect the colour of our grey sky. This is why I feel I am becoming invisible. If deaf-blindness was recognised, it would have an enormous impact on architects and other professionals in terms of improving the quality of life for people with dual sensory loss.

Acting Chairman (Deputy Mary Mitchell O'Connor)

Perhaps Dr. Paul Kenna can help me to conclude this meeting with a message of hope for people diagnosed with Usher's syndrome in the future.

Dr. Paul Kenna

I work in the research department of the Royal Victoria Eye and Ear Hospital, which has a long-standing interest in the genetics of inherited retina degeneration. Usher's syndrome is a significant subset of inherited retina degeneration, whereby retina degeneration is combined with hearing loss. I started my genetic work in collaboration with the ocular genetics unit in Trinity College Dublin more than 25 years ago. In 1989, we were the first to identify a gene responsible for any form of retinopathy and since then progress has been much more rapid than I would have predicted.

With advances, particularly in our ability to sequence and identify the building blocks of genes and to identify changes which may be disease-associated, over 205 genes have now been identified in a whole variety of inherited retinal degenerations. The identification of genes responsible for Usher syndrome has been even greater than that. It is estimated that we know 60% to 70% of the genes that may cause inherited retinal degeneration, but in the case of Usher syndrome it is estimated that there are 12 different genes and we know ten of them at the moment. Essentially, therefore, the genetic mystery of Usher syndrome has largely been solved. We can now make genetic diagnosis relatively easily with the newer technologies for identifying disease-associated gene changes.

The research which I and others have been involved in for the past 25 years and longer is now transitioning to becoming a diagnostic test. I would urge that consideration be given to the importance of doing that gene testing, particularly on very young children who have been identified with congenital hearing loss because a significant portion of those will actually turn out to have Usher syndrome. At the moment the visual part of Usher syndrome only comes to light generally when they are in their teenage years or even a little older whereas if the genetic testing could be done as soon as the significant congenital hearing loss is identified, the true diagnosis comes to light and the clinical care pathway which Dr. Cummings commented on, can be started from the outset. Therefore, adaptations to education and so on can be set in motion when they will be of value and before the patient reaches a stage where they have a significant visual problem which, unfortunately, is the way most patients with Usher syndrome come to light at the moment. Their hearing loss is known from a very early age but it is only subsequently that the vision deficit becomes apparent.

Another result from the advance in genetics and the identification of genes responsible for Usher syndrome is that there are realistic prospects in the foreseeable future of that knowledge being used to develop gene-based treatments. There is already one human clinical trial which has just started - we do not have any results, it is in a very early stage - basically replacing the defective gene in patients with a form of Usher syndrome due to the Myosin 7A gene, and replacing the defective copies of that gene with normal copies of the gene with the hope of either stabilising visual function or there may even be a prospect of visual improvement.

There are two clinical trials going on of similar gene replacement in non-syndromic forms of retinitis pigmentosa. In both of those the data would indicate that not only does the gene treatment stabilise the condition, but it actually leads to vision improvement. The same may be the case with Usher syndrome. There will be greater treatment options based on knowledge of what genes are causing particular forms of Usher syndrome. The treatments will very likely be gene-specific, so patients with Usher syndrome will have to know what genes are causing their particular condition.

Acting Chairman (Deputy Mary Mitchell O'Connor)

I thank Dr. Kenna. Are there any other questions? Or do the expert witnesses have any other comments they would like to make before we conclude?

Mr. Derek Lacey

A point was raised about regulation for audiologists in Ireland. The Irish Society of Hearing Aid Audiologists, ISHAA, has been actively working in Europe with the EU standard. We have adapted the EU standard here in the society. Our members adhere to that standard to the best of their abilities. As was rightly said, there is no regulation or legislation. Anybody can walk in here and sell Ms Brill A, B and C, claiming to be an audiologist or a scientist audiologist. There is no regulation for that. We are actively working with the Department in trying to get some sort of regulation and, hopefully, that will happen in the near future. The only Department that has been really significant in this is the Department of Social Protection through the PRSI scheme. Through that scheme we are able to monitor and ensure that good practice is adhered to at all times. That is the only bite that we have.

Acting Chairman (Deputy Mary Mitchell O'Connor)

Thank you Mr. Lacey. I am going to conclude but I want to make clear that we will write that letter seeking recognition of deaf-blindness. We will also send a transcript of today's meeting to the Minister for Health, Deputy Varadkar, and to the Minister of State, Deputy Kathleen Lynch.

Together with the group, we will try to work through their issues concerning social welfare, legislation and the proper provision of hearing aids. Have I left anything out?

Deputy Caoimhghín Ó Caoláin

The census.

Acting Chairman (Deputy Mary Mitchell O'Connor)

Yes. We need to look at the numbers on the census and know what kind of numbers we are talking about.

Deputy Caoimhghín Ó Caoláin

Medical card holders should have access to upgraded devices.

Acting Chairman (Deputy Mary Mitchell O'Connor)

They should have access to top-of-the-range devices through the medical card. That would be something significant that could come out of this meeting. We will work on it and will report back to the witnesses.

I thank Ms Carol Brill so much for coming in and giving us the true life experiences of someone with deafblindness. I also wish to thank Ms Laura English for her work with the Ann Sullivan Foundation. In addition, I thank Dr. Cummings, Mr. Lacey, the audiologist, and Dr. Paul Kenna for all their research work. I also thank Ms Orla Pearson for attending as an interpreter, and for our signer, and the audience in the Public Gallery. Go raibh míle maith agaibh go léir.

The joint committee adjourned at 12.47 p.m. until 5.15 p.m. on Tuesday, 9 June 2015.