To be affected by the disease EB, places great consequences on the patient and his/her environment. There are for example, blistering, wound infection, scarring, itch, pain and poor teeth. The disease is accompanied by all sorts of restrictions and feelings of sadness, helplessness and (over) fatigue. EB does not only result in physical problems but it also affects the family, social contacts, school, work, etcetera.

The psychosocial effects described are, of course not necessarily present in all patients, parents and family members and the degree in which the problems occur may vary as well.

In addition, a distinction has to be made between the problems that are caused by the disease and problems that may occur in any `healthy' person or relationship.

This module subsequently describes:

From the moment of birth or as soon as the disease has manifested itself, EB will determine the child's development. Depending on the type and degree of gravity the disease may also lead to emotional problems.

A young child that is seriously troubled by pain and itch will cry more, be agitated and sleep worse than its peer group. A child that is not feeling well and agitated will start scratching and rubbing which is bad for the skin. If a child wants something but is unable to do it, it experiences feelings of helplessness. It is hard to express these feelings because stamping, hitting and the like are instantly punished with pain. This means that it is hard for the child to learn to deal with his/her emotions.

Frequent hospital admissions and contact with doctors and nurses that are constantly `touching your skin' may cause stress. Especially for young children who cannot yet be told what exactly is going on, or why admission is needed and what is going to happen.

As a child grows older it becomes more and more aware of the disease and the accompanying restrictions. A child that is a little bit older will wonder:

`Why do I have this disease?'

`What will my future look like?'

`Why is everybody looking at me?'

`Can other people see my scars too and do they think I am ugly?'

`When will I not have to be admitted to hospital all the time any more?'

The child may feel rebellious or deny the disease. He/she may feel insecure and helpless about what is going to happen in future. He/she may be sad because of all the things he/she cannot do and embarrassed about the altered appearance. Feelings of denial, rebelliousness, depression and acceptance will alternate. Repression of these feelings will cause problems in the long-run.

In adolescence one's appearance plays an especially important role and blisters and scars may cause embarrassment or fear of reactions by outsiders. One person avoids contact as much as possible then and the other assumes an attitude of `What do I care?'. They feel that although they do not conform to the ideal image that is imposed on by the mass media that they do indeed have something to offer to others.

When parents have a child with EB it causes often shock and disbelief at first because they are confronted with a chronically ill child. Questions come up such as:

`Why is this happening to us?'

`I feel so helpless when I see my child lying like that.'

`What is ahead of us?'

`I feel so very alone, others do not understand what it means.'

`It is easy to say that you must accept it, but how do you do that?'

Parents may react differently to the far-reaching effects of their child's disease. Some parents become rebellious, insecure or sad. There is a feeling of not having any grip on the disease and people are ashamed of the nasty scars on the skin of the child. When family and friends do not sympathize with the situation and when people are largely housebound, it may result in a feeling of isolation. Especially because it is often difficult to explain to the outside world what exactly is going on.

In the beginning there is usually concern, disbelief or denial. Later, these emotions change to sadness, acceptance and efforts to deal with the disease. Frequently, there are also feelings of continuing helplessness, despair and feeling (over)burdened.

To have a child with EB is, especially when it involves a serious version of the disease, a heavy burden for the parents. In the first few years in particular, a physical and a mental overload often occurs. Young children with EB fre-quently only sleep for a couple of hours in succession and are awake a lot during the night. The care and dressing of the blisters and wounds take a lot of time every day. The care is intensive and often very specific and therefore not easy to delegate to others. Older children are often more capable themselves of indicating what is needed and what they are used to.

The disease is chronic and can take, despite the enormous de-dication to the care, an unpredictable course. Thus, a wound that took months before it was finally healed may be entirely (scratched) open in a couple of minutes.

It is, as a parent described, `difficult to see your child in so much pain. You feel so helpless as a parent because you can do so little to diminish the symptoms of the disease. You, therefore, wish sometimes in your despair to be freed of all the troubles and you think `If only the child was in hospital' or `If only the child was dead'. And subsequently, you feel guilty for having these thoughts about your own child, because you want nothing more than that your child feels comfortable and is able to enjoy life.'

The relationship between the parents may also come under great pressure due to (over)fatigue and/or different responses to the situation. Because of the seriousness of the child's disease, tension may build up in the family because siblings might be feeling neglected.

During the development from infant to becoming a child of school age, all sorts of problems might occur in the up-bringing that are part of the normal development of a child: problems with eating and sleeping, temper and stubbornness, problems with toilet-training, crying, problems in the motor development. Every child learns by falling and standing up and learns his/her possibilities and limitations in this way. This holds for a child with EB as well, only the possibilities are more restricted and the limitations reached sooner. Moreover, the limitations vary daily. It may be difficult for the pa-rents to distinguish properly which kind of behaviour is part of the `everyday' problems of upbringing and which problems relate to the disease.

Every child has for example, a period in which it does not eat well. A child with EB however, often needs extra nutrients. Eating less, may exacerbate the problem because as a parent you are afraid that the child's intake of nutrients is insuf-ficient.