Epidermolysis Bullosa Home Care Programme Treatment fusions/contractures

Fusions are the result of atrophic scaring of wounds. Fusions are particularly present in the fingers and toes (`webbing' and acquired syndactyly) and the mouth (microstomia).

Contractures may be the result of scarring (due to the blis-tering) and a bad posture (because of the fear of falling or pain). This decreases the movements of the joint, the skin around the joint becomes less supple and muscular and collagen tissue is shortened. This may lead to permanent loss of the joint's mobility.

Fusions and contractures are invalidating and cause restric-tions in various areas.

This module describes how to prevent the fusions and contractures and how they, when they are present, may be treated.

Prevention and treatment of fusions and deformities

Fingers

The fusion of fingers can be prevented and should not be con-sidered as an inevitable consequence. The function of the hand can be maintained to its optimum by exercising, dressing and through the use of splints. The aim of this is to keep the fingers `separate' from one other, especially in the case of blistering. In the chapter Expertise: Exercises, a number of interventions is described that are aimed at preventing digi-tal fusion.

Mouth

To prevent the mouth opening from becoming smaller, exercises should be carried out so the corners of the mouth are `stretched'. This can be done by opening the mouth as wide as possible. The corners of the mouth may possibly be slightly pulled aside with the fingers. This stretching should be carried out very carefully to prevent rupturing of the corners of the mouth. A lubricative cream should regularly be put on the lips and especially the corners of the mouth. This keeps the skin in good condition and prevents rupturing of the corners of the mouth.

The mobility of the tongue may be increased by sticking the tongue out as far as possible and by moving the tip of the tongue towards the nose. The exercises mentioned are best implemented at a particular moment of the day, for example, before brushing the teeth. Some children with EB try to spare the tongue and do not pronounce certain consonants. For example, they do not use a `r' or `l'.

Sometimes professional help is required to stimulate the speech.

Prevention and treatment of contractures

Contractures may be prevented by moving the joint. In the chapter Expertise: Exercises, a number of exercises is described that are aimed at preventing contractures.

Stimulation of sensorymotor development

Feeling

Small children discover and learn a lot through their senses. One of these senses is touch. The superficial sense of touch in the skin indicates, for example, whether something is hard or soft, or whether something is cold or warm or maybe pain-ful.

The deeper sense of touch in the joints and muscles gives information on the position of the arms and legs in relation to the body.

In a child with EB the sense of touch may develop differently: because the skin is often covered with dressings and causes the child to perceive little through the skin and because of which the joints might be less mobile. The child may perceive the sense of touch as something unpleasant and even painful. It will therefore be afraid to touch unknown objects or mate-rials in particular, let alone play with them. Even wearing clothes or simply laying down might be painful.

Because of the disruption of the sense of touch the child will probably experiment more slowly and more carefully with feeling and touching. This will result in a slower development of the sense of touch.

Movement

Because of the slower development of the sense of touch and the disruption of deeper touch the child will be very cautious when moving. After all, every movement may be painful. The normal developmental path of learning to walk (lying, turning, sitting, crawling and walking) will probably be complicated and evolve differently. A healthy child learns to walk by fal-ling and standing up. The falls the child with EB suffer from will have serious consequences. Besides the pain and the in-juries sustained he or she will have to be (even more) careful in future with experimental movements such as crawling. Because the child moves less, the joints will become less supple. This will complicate the learning process of how to move (also locomotion), just as it will affect the development of the sense of balance and sense of speed.

Stimulation of feeling and movement

There are several ways in which the child's feeling and move-ment may be stimulated safely. Important in this is that the child may indicate what it does and does not dare. For the parents it is important that the child is allowed to `stand on its own feet' and therefore, allowed to fall occasionally. Overprotection against pain, bumping and falling may also lead to a disrupted de-velopment of touch and movement. A child needs to learn its own boundaries.

Possibilities for touching:

• drawing on a mirror with shaving cream;

• playing with toys made of light soft material such as foam or polystyrene foam blocks or balloons;

• if it is not possible to give the child a good strong hug, the child may be gently caressed, touched or massaged on the intact skin. This method of touch is also important for the contact between parent and child; touch is a form of communication. When the only thing a child feels is bandages, it gets a one-sided (impression of) pressure on the skin. Massaging the skin with oil is not only good for the skin but also for the perception of the skin and the skin contact.

Possibilities for moving:

• Walking in a baby walker when the child cannot yet stand steadily. Thus, it can move freely which is a good exer-cise for the muscular development. Be prepared that some-times blisters might occur on the upper legs.

• Let the child pick up small objects from the ground. When bending, the child exercises the knees and the feet/ ankles. When picking the object up, it exercises the hands and wrists.

• In order to develop a sense of balance, speed and direc-tion, playing volleyball with a balloon, hockey in a wheelchair and swimming, in particular, may be good exercises.

For certain play activities, adaptations are necessary, for example:

- swinging in a padded swing tub or hammock;

- sliding off a slide on a blanket;

- cycling on a tricycle;

- learning to walk with a stroller.

• In the swimming pool the child will be able to move more freely in the water. It is advisable to find a time at which it is not so busy at the pool. If wounds are open and or infected it is advisable to consult the doctor in attendance.

In consultation with the physiotherapist, there might be other possibilities:

• In a bath with balls a child can move freely without bumping or rubbing. A bath of balls consists of a basin filled with small, soft balls. Such a bath induces children to move.

• Exercising on a large ball. A physiotherapist can teach the parents to practice with a so-called Bobath-ball. The child may, for example, lie with its belly on the ball. When the ball rolls forward, the child has to stretch out its hands to touch the ground. The child may also sit on the ball and pull up its legs to exercise the knees and hips.

Let the child play in a safe environment in which it will not be run over by other children. This is possible by, for example, putting the child in a large inner tube of a car on the ground or in a specific screened off corner. This way the child does not have to keep an eye on its surroundings and is able to focus completely on its game.