The support of EB-patients and their direct environment is complex. Time and again, for every individual and every si-tuation an assessment must be made to determine what form of support is most appropriate for the problems of that moment. The answer to the question what help is most adequate, also depends on the stage of the disease and the acceptance pro-cess. If the patient and his/her direct environment (parents / family) are still in the initial stage of the disease, then there is often denial and there are problems accepting the disease. At this stage, information on care, home-alteration or professional help will not (yet) be effective. At such a moment, it is better to listen to the concerns and experiences of the people involved and to sympathize with this.

The psychosocial effects may be supported in different ways, such as offering emotional, practical and informative support. In the final section advice is given that might be of help in the upbringing of the child.

It is a challenge for the patient with EB and his/her en-vironment to learn to deal with complex problems. These pro-blems differ for every age and stage of the disease. When parents of children with EB and carers pay attention to the practical aspects of care they should also keep an eye on the emotional aspects. Just like others in difficult situations, patients with EB need people who will understand them.

When giving emotional support the following points should be taken into consideration:

• patients / parents are able to express their feelings;

• listen, be understanding and show respect;

• support acceptance of the disease;

• check whether people have someone to talk to, give information on discussion groups and/or contact with fellow-sufferers, for example, the EB-patient association;

• accessibility and availability.

In general the patient finds it easier to deal with the si-tuation and the parents can carry on caring for the child longer when they are given support and the care is distributed among several people. In this way, people do not feel they are all on their own.

This is especially true for parents who see the care of the patient as a heavy burden, for example, in the case of many care duties. A lack of support forms then an even greater burden.

As the support, which is available for the main carer from the social network, is often decisive for coping and carrying on with the care, it is important to find out to what extent the family assists one another practically and emotionally and how much this contributes to the increase of the supporting

Practical support refers to the offer of forms of (pro-fessional) help, such as, for example, by the home care organization, child-minding service, day care or voluntary help. The aim of this practical support is to (temporarily)

take over the care for the patient. In addition, practical support includes giving practical tips on nursing and caring for the patient.

The EB-patient is usually fully-informed on how to deal with his or her health problem. Throughout the years different methods on how to care for the skin have undoubtedly been tried and the patient will have found his/her own way of implementing one of these methods. Still, patients or their direct environment may need some practical advice. Questions may concern both care of the affected skin and how to deal with problems that more or less stem from the disease. Practical support can be provided to the patient or his/her direct environment on the following subjects:

• skills involving the care of EB;

• dealing with family-strife;

• maintaining a good relationship with the patient / family / friends;

• self-care or helping to realize this;

• problem-solving skills:

- sorting problems;

- determination of burden perceived;

- first deal with the problem which causes the most stress and the one which can be solved in the short term;

- make a timetable.

Besides the emotional support for parents who care for a child with EB discussed earlier, general practical advice can be given which relates to daily contact and care tasks. Depending on the child's age and the seriousness of the disease this may be summed up as follows:

• involve the child in the daily care;

• let the child set out all the materials that are necessary for washing and dressing;

• let the child take care of its own skin as early as possible;

• let the child go to spend the night occasionally elsewhere;

• let the child do the shopping on its own;

• let the child ask for information (by phone or in writing);

• let the child make its own appointments;

• teach the child to cook;

• teach the child to operate domestic appliances;

• teach and stimulate the child to engage in activities outside (to go out, to go and stay the night, travel);

• teach the child to handle money (savings).

 

Informative support

Education or information may be supplied on a lot of subjects, for example, the disease itself, the treatment, the nursing care and the general care. Below, a number of likely subjects are summed up on which education and information may be supplied.

Parents must feel confident about the care of and the contact with the child. This is one of the main factors for a stable development of the child.

It is difficult for the parents to deal with the assumed vulnerability of their child. Instructions for several caring tasks such as feeding and bathing the child are very im-portant. Feeding the child which has EB may sometimes be difficult. The parents have to bear several things in mind for medical reasons. Yet, it is important to stimulate the contact between the parents and the child and that the parents learn to appreciate feeding their child as something pleasant and positive.

Pain and discomfort may be the reason why the child becomes afraid, angry sad and disappointed. While bathing, putting clothes on and taking them off, taking medicine and playing they are continuously confronted with it. The physical limitations and the constant `patient role' may provoke anger and defiance. This regularly creates tension between parents and child.

Especially in the first years of life it is important for a child to experience that parents are there in difficult moments and try to help him/her to, for example, deal with feelings of helplessness. Some parents give their child, when it is angry, a soft pillow to hit on or allow the child to throw with soft objects, for example, with cuddly toys. Tell the parents that they must allow the child to scream extra loudly or teach the child that singing is also possibly a way for expressing emotions; cheerful songs for when the child feels happy and cheeky songs for when the child feels defiant.

It is important for the child to become independent as soon as possible. The parents must encourage the child and learn to build up confidence. It is the natural instinct of parents to protect their child and restrict activities that might bear a risk. However, by focusing on a future for the child that is as independent as possible, it is better to let the child join in everyday life as much as possible. That entails, building up a circle of friends, going out, developing hobbies, learning to be assertive and playing. Playing with other children is important for children with EB as well despite the playing about and romping with other children which may result in blisters. The child should not be protected constantly; the overprotected child that is never allowed to do anything by him/herself assumes that he/she has no value as a child. Independent behaviour can already start to develop from a couple of weeks after the birth by, for example, leaving the child in the care of friends or a babysitter so the child sees or hears different faces and voices.

If there are more children in the family, the child with EB should be allowed to join in outings and possibly participate in activities which should be balanced. The same applies for hobbies. Encourage those hobbies in which the child runs little risk of damaging him/herself. The options are, for example, collecting things, photography, drawing, bowling or playing a musical instrument. No single statement can be given as to the choice of a hobby. It depends on how much the child dares and what it likes to do. Some children will go to certain extremes to be able to do the things they are fond of doing despite the accompanying risks. To them, pain does not seem to play a dominant role.

The following advice on playing is listed below:

• invite other children over to play with the child;

• provide enough of the same kind of toys, so that the children will not fight about wanting to have the same toy;

• use a baby walker with foam plastic around the wooden legs if the child is not able to stand firmly on its feet yet. In this way,

the child is able to move freely. This is also a good exercise for further development of the muscles.

Make expectations for the future subject of discussion

Parents should not avoid discussions on future expectations and (in some cases) on the life expectancy either.

In preparation for the future, the child must be helped by the parents to develop emotional stability. In addition, the child must learn to look after him/herself. Performing domestic tasks, assuming responsibility, learning social skills and learning how to recognize one's physical needs can only take place through contact with others in just as many varied situations.