Rare diseases: Occupational therapist accompanies children with epidermolysis bullosa – …

German Association for Occupational Therapy (DVE)

Karlovy Vary (ots)

A rare disease is almost always associated with poor public knowledge; there are also significantly fewer experts such as treating physicians or therapists. “This is also the case with epidermolysis bullosa, abbreviated EB. Patients have become known as ‘butterfly children’ because their skin is as sensitive as a butterfly’s wings,” explains Florian Prinz. The occupational therapist worked for several years in the Department of Physical Medicine at the University Hospital Salzburg in close collaboration with EB-Haus Austria, the world’s first specialist clinic for people with epidermolysis bullosa. He emphasizes that networks play a very special role in EB. As an occupational therapist with his own practice, he cares for children with this genetically caused, rare disease. His occupational therapy intervention is about clarifying the social environment, mastering everyday life, supporting motor development and maintaining mobility and thus the functionality of the hands and feet for as long as possible.

It is a gene mutation, ie a change in the genetic material, that causes the rare disease epidermolysis bullosa. The four main forms of EB differ, among other things, in their different degrees of difficulty. The typical appearance of this disease is the formation of blisters on the skin and usually also on the mucous membranes. Internal organs are also affected in certain subtypes of EB. Epidermolysis bullosa simplex is the most common form. Special ointments provide relief and coated bandages protect the affected so they can cope with their daily lives reasonably well. That everyday life is different for children with one of the difficult forms and in many respects requires more attention and affection from the whole family can be explained, among other things, by the high risk of injury and the time required for medical care. It is a cycle of large-scale blistering, resulting wounds, healing process, scarring.

Network – exchange with other parents of children with epidermolysis bullosa

“Despite their limitations, these little heroes tick like other children, they want to play, learn, conquer life, and they are just as happy or serious as anyone else,” Florian Prinz reports on her past encounters and experiences. One of the first tasks that occupational therapists undertake is, for example, to make parents of children with epidermolysis bullosa aware of existing networks, if this has not already happened in the clinic. Affected families help each other best because parents and the affected children themselves are the best experts in everything related to this rare disease. A lively exchange to pass on or receive tips is a significant help, especially in the beginning, just like help from support institutions. When children with EB are still very young, they spend most of their time in the family environment. In this phase, the focus of treatment of occupational therapists like Florian Prinz is primarily on delays in motor development. What Happens: Any change in position or movement that involves straining the knees, palms and soles, among other things, can cause blisters, resulting in widespread sores. To avoid this, many toddlers with EB remain in a passive sitting position for long periods; Standing and walking is delayed. Without proper treatment, for example from occupational therapists, these children are usually restricted later when playing and moving with other children – in addition to the body parts covered with blisters and wounds.

Inclusion – Education of the communities around children with epidermolysis bullosa

As soon as a child with epidermolysis bullosa starts in kindergarten and later in school, occupational therapists are needed to pave the way for inclusion. “First and foremost, it means educational work,” says Florian Prinz. The occupational therapist continues: “Frequently asked questions such as ‘Is this contagious?’ or ‘What tools are needed?’ to answer – the parents, the educators and the educators as well as the other children in the class, if possible. “It is a great help for the social environment to know more about the disease. Teachers should be informed in advance about what to expect when they take a class with a child with epidermolysis bullosa and what support is needed.Will an assistant also be present in the class? Where can the child with EB sit without being bumped into by other children passing by, causing blisters and wounds? How can participation in sports and craft education be made possible? Occupational therapists generally deal with the overall situation and with all aspects to enable participation in everyday life as independently as possible. Thanks to their empathetic nature, they always find practical solutions to questions, occur together and in close collaboration with the educators and the family environment.Above all, they recommend open contact with each other.A child with epidermolysis bullosa is best able to tell about himself, and many a questioner is surprised when he or she hears that hobbies like horseback riding or playing an instrument are quite possible. This is also similar to the current approach to treatment: to teach children with EB as early as possible how they can be mobile through fall prevention, perception training and active motor skills and still organize their daily lives with few injuries.

Hand therapy – occupational therapists’ specialty

Fusions on the hands are a manifestation of a severe subform of epidermolysis bullosa: the skin between the fingers grows together over time, the joints can be gradually bent and stretched less and less, so that the fingers can eventually no longer be moved individually. This process can start very early, from the age of two. There are a number of occupational therapists who specialize in hand therapy. The goal in children with epidermolysis bullosa is to keep the status of the hand stable for as long as possible. Therefore, it is extremely important to start passively moving your hands through as early as possible. Other ways to slow down the adhesions and limited movements of the fingers are special bandaging techniques for the fingers, gloves specially developed for this purpose or splints made by occupational therapists. The process of verse growth can not be stopped, but it is possible to delay an operation in which the adhesions of the hands are loosened as long as possible. For what immediately sounds promising has drawbacks. In addition to the general surgical risk, the subsequent rehabilitation is often challenging because the children are in pain, cannot move their fingers immediately, must have splints on, and touching the operated hands is only possible to a limited extent, as it causes the well-known injuries. . . In addition, the condition of the operated hand or joints deteriorates over time, and the result of the operation usually lasts only two to three years. “This information is part of our communication with the parents of children with EB, or the children are involved in the discussions when they are older and understand what is at stake,” explains occupational therapist Florian Prinz about her delicate task: It is a tight leash . go between the understandable desire of those affected to get healthy hands back and what is realistically possible. If a family decides on the operation, it is the task of the occupational therapist to treat the operated hands professionally and in the best possible way. The goal: to preserve the newfound freedom of movement and function of the fingers as long as possible.

Coping with everyday life – tools and always new tips from occupational therapists

Occupational therapists’ field of work is primarily aimed at coping with everyday life. That’s what Florian Prinz’s intervention units are all about. The occupational therapist: “For example, I make sure that the children with EB that I care for are and are allowed to use the right aids for them”. These include special seating, special ergonomic chairs, a recorder or electronic devices that can be used to take notes despite limited hand function, locking aids that ensure children can open and close locks or keys on their own. .. the list can be continued at will. “We can do nothing about the disease itself, but by providing support we can ensure that children with epidermolysis bullosa can go about their daily lives as independently as possible.” The positive thing is that thanks to the support and impulses that the sick children and their families receive, new ideas are constantly emerging that make life easier so that the beautiful can be expressed and a changed perception can take place.

Information material on the various topics of occupational therapy is available from the occupational therapists on site; Occupational therapists near your place of residence on the association’s website at https://dve.info/service/therapeutensuche

More information about epidermolysis bullosa: https://www.debra-austria.org and https://www.ieb-debra.de

Press contact:

Angelika Reinecke, German Occupational Therapy Association (DVE),

Original content from: Deutscher Verband Ergotherapie eV (DVE), broadcast by news aktuell

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