A Model on the Rehabilitation of Deafblind: A case study

Transcription

1 A Model on the Rehabilitation of Deafblind: A case study Hanna Taranger and Jude Nicholas Regional Resource Center for Deafblind Vestlandet Resource Center Norway Introduction As consultants for adventitiously deafblind we experience the need to have a systemic rehabilitation process for persons with deafblindness. We will present some major issues in a rehabilitation process from a Norwegian Rehabilitation Model applied to a deafblind person with mitochondria disorder. Description of the Model The Norwegian psychologist, Ivar Lie (1996), explains that in principle rehabilitation evolves through three phases. There are no distinct lines between the phases, and they overlap each other. The first phase : This phase consists of a medical evaluation, treatment and follow up that will form the basis for an optimal process of rehabilitation. Adequate medical treatment is fundamental for optimising rehabilitation. Neither rehabilitation of function or rehabilitation of participation can be planned or carried out without taking note of medical considerations. If there are impairments even after a medical treatment, it will raise the question of how to optimise functions in an effort to restore them. The second phase: This phase deals with functioning and the aim is to regain a normal ability of function as far as at all possible. The efforts are workable and appropriate only if they give due consideration to the person involved. Reduced function to what extent can it be restored? Our group persons with acquired deafblindness are in need of different things like: learning how to use technical aids, finding alternative communication systems, learning mobility skills, activity of daily living (ADL), personal support, psychological help, how to cope with their own situation and how to get information. The third phase: 1

2 This phase is about participation. Until now the process has focused on optimizing the possibilities of functioning in terms of the individual. In this third phase of participation it is a question of looking at the person in his own environment. It is most important that persons with a handicap are able to participate in their own family, social environment, as well as in society at large. That is why we need to focus on the person's participation in his environment to normalise as far as possible. Main Issuess in the Model: 1. Assessments of goals Coping with crisis Knowledge of the individual functioning and participation Knowledge of different kinds of intervention Strategies of coping 2. Assessments of problems and resources 3. Problem solutions and type of interventions Joint activities 4. Evaluation 5. Follow up Case Description The person is a 50 year old man. He is single and lives alone. He suffers from a mitochondria disease of a rare and disabling nature. This has by now left him almost deaf and blind. It has proven difficult to assess his cognitive abilities and his brain functions. He lacks sign language and is thus left with practically no means of communication. His medical condition has varied considerably over the past year. He has suffered many epileptic seizures, his body has become very rigid, and he has difficulties in holding it upright. The person today requires constant care and indeed has a caregiver with him at all times. He is mentally unstable. On a number of occasions he has expressed thoughts of not continuing to live because of his progressive disease, and for periods he has refused to take his medicine and been reluctant to eat. 2

3 He is often aggressive and directs this aggression at his family and anyone who happens to be with him at the time. He is seldom satisfied with their efforts and shows this by outbursts. He will often keep repeating the same phrases, days on end. Family and caretakers find it taxing, but they realize that the behavior is related to the illness and his whole life situation. He is an electrician by trade and worked offshore for 13 years. In 1994 he had to leave the North Sea since his failing sight and hearing made it hazardous for him to continue working there. From 1994 to 1999 he worked as an electrician on land, but by December 1999 both his sight and his hearing had been reduced so much that the company he worked for could no longer find suitable work for him. This was a great loss to him. His work had been of great importance in his life, and his identity largely depended on it. So it also took him a long time to come to terms with this decision. He kept talking about his need to recover sufficiently to return to work. He received his first eyeglasses when he was 4, and he was diagnosed as hearing impaired when he was 20, receiving his first hearing aid as at 26. In the fall of 1999 the user expressed worry about vision to his ophthalmologist. He noticed that his sight was reduced, especially his night vision. His physician felt that there was reason to believe that a syndrome was causing his loss of sight and hearing and that his findings might be indicative of Usher s Syndrome Type II. In May 2000 he suffered a fit that was thought to be a stroke. This resulted in substantial loss of mobility in his left hand. He remained hospitalized beyond the treatment period, since it was felt that he would not be able to cope on his own. In September 2000 he underwent a neurological examination at one of the largest hospitals in Norway. In connection with this both his vision and hearing were thoroughly tested. The subsequent findings practically ruled out Usher s Syndrome. A muscle biopsy was performed and the sample sent to England for analysis. In October 2000 he received the result of these tests, the diagnosis was made. He was found to suffer from a mitochondria disease. 3

4 Neither he, his family, nor any of his caregivers had ever heard of this disease before. This also meant that we knew nothing of its implications. We thus asked permission to contact the neurologist who had examined him in order to learn what this diagnosis might involve. We asked for specifics about the diagnosis, especially in terms of symptoms, development of further symptoms and prognosis. We also asked for information about the impact of the illness on the senses (vision/hearing), musculoskeletal and central nervous systems (neurological features). This information was considered vital to our further efforts to rehabilitate our user. Brief on mitochondrial disorders The mitochondria are called the powerhouse of the cell because they extract energy from nutrients and oxygen and in turn provide energy in a more usable form to energize essentially all cellular functions. Virtually all cells in humans depend on mitochondrial oxidative phosphorylation to generate energy, acounting for the remarkable diversity of clinical disorders associated with mitochodrial DNA mutations. However, certain tissues are particularly susceptible to mitochondrial dysfunction, resulting in recognizable clinical syndromes. Mitochondrial DNA mutations have been linked to seizures, strokes optic atrophy, neuropathy, myopathy, cardiomyopathy, sensorineural hearing loss and diabetes mellitus. One group of mitochondrial disorders due to specific mutations of mitochondrial DNA includes MELAS (Mitochondrial Encephalopathy with Lactic Acidosis and Stroke like episodes) MERRF (Myoclonic Epilepsi with Ragged Red Fibres) NARP (Neurogenic muscle weakness, Ataxia and Retinitis Pigmentosa) LHON (Lebers`s Hereditary Optic Neuroretinopathy) All these diseases are transmitted through maternal line. Mitochondrial disorders give rise to visual and auditive problems, due to various reasons such as ophthalmoplegia, optic atrophy, dysfunction of the cochlea and impaired cerebral glucose uptake in the occipital and temporal lobes. 4

5 Application of the Rehabilitation Model Ivar Lie's Model emphasizes that it is the user's own choices that shall determine the rehabilitationprocess. To be able to make his own choices he must get enough information and insight concerning both possibilities and the limits related to his situation. It also means that he by testing and to practice becomes aware of the functional possibilities of his situation. By building up competence in such a way he will be able to make his own choices. To be able to do this it requires that it is possible to communicate with the user either by speak, signlanguage, writing or other forms of communication. Due to the great communication problem we have had with this user this has been very difficult, not to say impossible. We still mean it is possible to use Ivar Lie's Model in the work with this user, and we will make a connection between the work we have done within his Model. Due to the three phases in the rehabilitationprocess we have been working both in phase 2 and phase 3. The great difficulties with communication has caused that our main focus in our work with the user has been in phase 3 which deals with participation and how the person function in his environment. The objectives have been for the user to be able to, in a certain degree, be part of and to participate in the family and in the society. Increased competence about the dual loss of vision and hearing to his family and network will be of importance for the user's daily life. When we look at Ivar Lies's Model we discover that some point's are more usable for our work as consultants than others. We will describe how we have worked in relation to phase two and three with the person and his network. Phase 2. Information Tecnical aids ADL (Activities in Daily Life) Environmental adjustments Training Care management Phase 3 Information about the dual loss of vision and hearing to his social and professional network Information about Mitochondria disorder, possibilities and limits 5

6 Education in alternative communication to his social and professional network Information supervision and counseling to his social and professional network Psychological support Coordination of efforts and follow up. Conclusion: Is it possible to say that we have been successful in rehabilitation of this person? It is difficult to "measure" objectively the positive changes which have occurred due to the nature of the disorder. What we can conclude is that without this holistic type of intervention the situation would have been different and difficult for the network. This has been to a large degree confirmed by the network and his family. Thus an adequate, effective program of deafblind rehabilitation must, of necessity, be organized and operated along holistic lines. Bibliograhy: Lie, I. (1996); Rehabilitering og habilitering. AD Notam Gyldendal Simon, D.K & Johns, D.R (1999). Mitochondrial disorders: Clinical and genetics features. Annu Rev Med, 27. 6