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Epidermolysis Bullosa
Home Care Programme

Preface

Epidermolysis Bullosa (EB) is the term used for a group of genetically-determined blistering skin disorders. There are a number of different forms of EB ranging from mild to severe. In the Netherlands, around 400 patients have been recorded. EB is not only a skin disease but it also seriously affects other systems, in particular the locomotor apparatus (scarring and deformities can lead to invalidity) and the digestive system (such as deformities of the mouth and tongue, blistering in the mouth and oesophagus and chronic constipation). It is not yet possible to cure EB. The blisters eventually do heal but they usually require interminable treatment. There is a constant threat of wound infection and (deformative) scarring.

The severe forms, which lead to serious invalidity and a sometimes distinctly shortened life expectancy, require intensive care, which is usually provided by parents, carers, partners and/or family. The problems associated with prognosis such as: functional movement, nutritional condition, itch, blistering, dermatitis, scarring and psychosocial functioning are central here. Besides the clinical diagnostics and treatment, little has been developed in the Netherlands in the field of nursing care and treatment of these patients.

Certain groups of patients with this disease could be helped by receiving support from home care services. For example, help with the time-consuming nursing of/care for the skin and advice and support with psychosocial problems (school, work, relief of the social network).

In the Netherlands, experience and knowledge of Epidermolysis Bullosa and related problems has been concentrated in a limited number of centres.

In order to make this knowledge more accessible and adapt it to the practice of careworkers in primary health care settings and careworkers working in institutes where there is no experience, the KITTZ - Home Care Programme Epidermolysis Bullosa was developed.

The development was financially supported by Nationaal Revalidatiefonds and Academisch Ziekenhuis Groningen.

A Home Care Programme is an information file which provides assistance in helping to make sure that a clearly-defined patient group can be safely cared for and/or treated at home. On the basis of the (care)need, it has been established what should be done and also how it should be done. In the home care programme the resulting conditions concerning expertise, materials, organization of care and supply of information are applied to practice-oriented instruments.

The Home Care Programme Epidermolysis Bullosa is not based on the various forms in which the disease can reveal itself but on the patients' problems and questions about their care. The treatment and care has been described and on the basis of this, care can be provided. The nature and intensity may differ for each patient.

The Home Care Programme Epidermolysis Bullosa is mainly based on the experiences of patients, carers and careworkers. These experiences were gathered by interviews and literature study.

For the realization of the home care programme a supervisory committee was formed. The members of this committee have played a role in the assessment of the home care programme.

The members of this committee included:

  • Ms C. Brandt, nurse Ouder- en KindZorg Thuiszorg STAD;

  • Mr J. Doldersum, chairman Stichting E.B.-patiënten;

  • Mr dr. M.F. Jonkman, dermatologist Academisch Ziekenhuis

  • Groningen;

  • Mr R.J.J. Koopman MA, paediatric dermatologist, Medisch Spectrum Twente;

  • Mr J. Lablans MA, manager dermatology Academisch Ziekenhuis Groningen;

  • Mr J.M. Mandema MA, paediatrician Academisch Ziekenhuis Groningen;

  • Ms G. te Nijenhuis, staff nurse Thuiszorg Enschede-Haaksbergen;

  • Ms G. Sluman, dermatology nurse Academisch Ziekenhuis Groningen;

  • Mr dr. G.T. van der Werf, GP in Groningen.

     

    The practicability and applicability of the home care programme was assessed by a KITTZ reference group which includes representatives of the home care organizations:

    • Mr S. Hietkamp, staff nurse Thuiszorg Zuid-West Friesland;

    • Mr M. Kok, staff nurse Thuiszorg Groene Kruis Oost-Groningen;

    • Ms G. te Nijenhuis, specialist nurse Thuiszorg Enschede-Haaksbergen;

    • Ms G. Sportel, specialist nurse Thuiszorg Ommelanden;

    • Ms A. Wiersma, nurse Ouder- en KindZorg Thuiszorg Drenthe.

    Besides the supervisory committee and the reference group the following people have also assessed the practicability and applicability of the home care programme:

    • Mr L. Goffin, nursing home consultant Centrum voor verstandelijk gehandicapten Clinge;

    • Ms M.K. Sanders, paediatrician St. Lucasziekenhuis Amsterdam;

    • Ms J. Veldhuizen, nurse Amsterdam Thuiszorg;

    • Ms G. Bouter, nurse Districtsvereniging Groene Kruis Noord-Limburg;

    • Ms L.J. Molier, social worker, Academisch ziekenhuis Groningen;

    • Ms Y. Klepke, ergotherapist, Academisch ziekenhuis Groningen;

    • Ms J. de Vries, physiotherapist, Rijnlands Zeehospitium Katwijk aan Zee;

    • Ms M. Goverde-Nerfkens, nurse, Academisch Medisch Centrum Amsterdam;

    • Ms M. van Leeuwen, parent.

    In principle the home care programme can be used for the preparation and implementation of patient care throughout the Netherlands. The reading manual explains how to use the home care programme.

    In addition to the home care programme, the Homebook Epidermolysis Bullosa has been developed. The homebook was specifically designed for patients' or carers' use and it provides the information that is necessary for the treatment and care at home. The content has been based on the home care programme. However, since a home care programme is adapted to the practice and care of organization of professional careworkers, this information was modified in order to suit the knowledge and experience of the patient and carer. This means that the information has been phrased and organized differently and that more illustrations have been included, such as photographs of the materials used. In addition, the homebook focuses more on the activities of daily living at home and on the aspects of environmental perception, based on the experiences of other patients and carers. It is advisable to use the homebook simultaneously with the home care programme.

    We sincerely hope that the home care programme comes up to your expectations.

    Suggestions for improvement, supplements, additions, etcetera, for optimization of the material, are always welcome.

    E.S. Bosma,
    Board of directors KITTZ