We investigated the conditions of in-home care patients with ALS who live in our prefecture, under the research team of the Ministry of Health, Labour and Welfare in Japan, over a period of 7 years. During the first year, we undertook a questionnaire survey for ALS patients, family caregivers, hospitals, home nursing stations, visiting nurses, and public health nurses, about the formation of an in-home care system, interest in a community network for home-based care, problems experienced during in-home care, and requests for starting up a care network. We found that a limited network centered upon a patient already existed, and it was shown that patients hoped to have home care for as long a period as possible. The following year, we investigated the formation and function of small networks for patients by visiting them. It was found that there are many differences between networks, and it was realized that a unified care approach is good for patients and for establishing a larger social network. To this end, we created a guidebook to home care. Two years after publication of the guidebook, we undertook a questionnaire survey covering the same areas as the first survey. The main results were that mutual communication had been facilitated among small networks and the quality of care had become standardized. The analysis of results was shared with patients, families and care staff. We also assessed the methods of communication in ALS patients with moderate to severe motor weakness, and associated in-home care provision, by use of a questionnaire and inspection visits over the last 2 years. We emphasized the need for a variety of communication methods, and highlighted the incomplete utilization of social resources including various official support.

Following on from these surveys, it should be possible to construct an individual support system for at-home patients with ALS. We had aimed to combine individual systems by sharing information and techniques for home care. The recognition of an individual’s role and responsibility in participating in their home care would lead to a better QOL among patients and their families, especially caregivers.

To provide unified home care services, the building of a network is one of the best methods. Although it is not easy to build a large, fully-working network system, a guidebook that serves to unify the provision of home care in small networks, is relatively easy to create. There is a need for a range of support services to be made available, from which carers can select those most appropriate to them. However, some support services are not always available for carers, and accordingly there is a need for carers to have better access to training in physical care (O’Brien et al. 2012). Guidelines for the multidisciplinary care of home-ventilated ALS patients have been proposed (Eng 2006).

Based on the results of analysis of the questionnaire survey mentioned above, we published a guidebook for home care on behalf of ALS patients and caregivers at home. The book was written with the primary objective of maintaining a good QOL (Fig. 11.2). The book consists of seven chapters, namely About ALS, Impairments and rehabilitation, Basic methods of care, Preparation for home care (from hospital), Utilization of social resources for home care, Illustration of a case, and Some information on home care tools. We designed it to be easy to understand through the inclusion of many photographs and illustrations. The guidebook was sent to patients, hospitals, clinics, home care nurse stations, and health care centers in our prefecture. The guidebook was introduced at a meeting of the research team of the Ministry of Health, Labour and Welfare, and an affiliated patients association (JALSA; Japanese Amyotrophic Lateral Sclerosis Association). We recently presented the guidebook (written in Japanese) to the public, via the homepage of our department (URL:http://​www.​med.​yamanashi.​ac.​jp/​clinical/​neurol/​home.​html).

We have been holding a seminars on neurodegenerative diseases for medical staff and public directors twice a year since 2009. Seminars by neurology specialists, and presentations of several subjects regarding intractable diseases, including case presentations, have been held alternately. The titles of past seminars are: Home-based care support for patients with intractable diseases, Medical practice of spinocerebellar degeneration, Medical practice of dystonia, Clinical history and future applications of muscular dystrophy, and Approach of a care network supporting patients with intractable diseases in the Neurology Department of Kyushu University – a look back at the progress made over 16 years.

The aims of this program are: linkage between medical staff and public bureaus, the sharing of new information about concepts and treatment of diseases, and home care support for patients through the creation of patient-centered networks. Although our prefecture (Yamanashi), is relatively small, it should in theory be relatively easy to create and build networks. However, despite this, there are difficulties experienced when building networks due to a shortage of staff and a low population density. As a result, medical staff are required to move over a relatively large area. Through these seminars, it is possible to share information about many aspects of intractable diseases with people occupying various roles in the caregiving process. The seminars are also helpful as exchange meetings for home care networks.

We recently presented our experience on the positive outcomes of NIPPV and palliative care with morphine in ALS patients (the data were also presented at a meeting of the Japanese Medical Network for Seriously Intractable Disease). The presentation was significant because home care staff usually learn about practical issues without any comprehensive data. So the information provides home care staff relief, confidence and knowledge of practical methods.

In Japan, several nationwide clinical and genetic surveys of patients with neurodegenerative diseases, such as those by the Japan Spastic Paraplegia Research Consortium (JASPAC) for hereditary spastic paraplegia (Takiyama et al. 2010; Shimazaki et al. 2012) and the Japan Multiple System Atrophy Research Consortium (JAMSAC) for multiple system atrophy (Ichikawa et al. 2011), have been performed. In 2006, a longitudinal multicenter study of Japanese ALS patients using a telephone survey system, by the Japanese Consortium for Amyotrophic Lateral Sclerosis research (JaCALS), was started (Atsuta et al. 2011). This multicenter study is useful in obtaining large amounts of clinical data, and can contribute to elucidation of disease pathogeneses and the development of treatments.

We will probably be able to provide home medical care to patients, and to obtain physical information on patients using an IT network system in the near future. Such an IT network is operated by staff including hospital staff, co-workers, patients and caregivers. In our university hospital, a system for sharing a patient’s laboratory data, vital signs and other information among doctors belonging to our university hospital, home doctors and visiting nurses, has been in operation for a while. This system is mainly used for diabetes mellitus and ophthalmic diseases. We had a chance to try out this system for an ALS patient at home with a ventilator. In the future, home- or community-based care may well move in this direction and can be developed and improved through the use of an IT system.