I put this page together with the aid of a little booklet distributed by DEBRA UK called “A guideline to physiotherapy for parents of Children with Dystrophic Epidermolysis Bullosa”.
As the booklet states, this page is most useful for parents who have a child with Recessive Dystrophic Epidermolysis Bullosa.
The importance of movement
Most people move their joints fully every day and don’t hold them stiffly unless they are hurt-in EB there is often blistering (with scarring) of the skin near and around the joints which can stop them from moving fully. This, in turn, makes the joints stiff. This is why movement of the joints is vital to help prevent movement from becoming permanently lost.
We are all guilty of trying to prevent our loved ones from being hurt. It is particularly difficult for parents of EB children not to wrap them up in cotton wool and stop them from being adventurous. But mobility is absolutely necessary for these children. They need to keep their joints mobile to prepare them for all of life’s activities.
Babies should be put on their stomach to play (PLAY ONLY NOT SLEEP!!!). This is a good position to learn movement, and it helps prevent tightness developing at the hips and knees
Because of scar tissue at the joints, muscles and other soft tissues shorten around the joint which in time can permanently lose some of its movement. If this continues, they can worsen and may make certain activities difficult or even impossible. If you notice your child being unable to fully move any of his joints, it is a good idea to encourage him to move to its limit and then repeat asking him to go as far as possible each time. When done early, he can get back to normal, avoiding further stiffness.
It is important for your child to walk a short distance each day (if old enough) usually regardless of blisters on the feet. When they are very sore, encourage the use of tricycles or other activity toy to move independently.
This is a lovely form of exercise. By encouraging your child to participate early he will be allowed to compete on equal terms with his peers. Make sure to go armed with plenty of moisturizers for afterwards.
When to exercise
As soon as little stiffness is noticed it is a good idea to begin. Exercises are best done several times a day if there is stiffness, if not, allowing the child to be generally active is good.
Which exercises are most important
- All children with EB should be encouraged to lie on their stomach daily.
- Mouth exercises are of benefit to people with RDEB as most have some tightness. They should be done each day with the care of teeth.
- Hand exercises should start immediately.
Hips often stiffen, particularly if a child sits for much of his time. Prone lying each day helps stretch them out. Lie on front. Lift right leg straight behind. Repeat with left leg.
OR This same exercise can be done standing holding on to something for support-such as a chair.
Knees too can lose flexibility and if bent for long periods can result in an inability to straighten fully. Prone lying will help to prevent this. Lying on back- Tighten muscles hard and pull feet “up” at same time count to 5 slowly.
then Straighten one knee fully and tighten. Lift that leg into the air, then lower. Repeat with other leg. (NEVER do both legs together as this can lead to back problems)
OR Straighten one leg when sitting on a chair and hold for the count of 5. Repeat with other leg. Make sure the chair has a back and that your child does not cheat by leaning back (holding on to the front of the chair with his hands may stop this)
then Bend right knee up taking the heel toward the bottom. Repeat with left knee. This exercise can be done lying on one side if for some reason your child cannot lie on his back.
Most children with RDEB develop tightness of the mouth stopping them from opening it fully. The tongue can become immobile too. This makes dental care more difficult. It is important to combine these exercises with daily teeth cleaning (which is so essential). Stick the tongue out as far as possible and move it up and down and side to side. then Open the mouth as wide as possible (saying “eee”).
If your child is sensible he can gently pull the corners sideways with a finger at each side to give a gentle stretch. then Open the mouth as wide as possible to form an
if your child’s mouth cracks, use a little moisturizer or a lip balm before starting exercises.
If the neck stiffens, these exercises help stop this from happening. Turn head to right fully and then to left fully keeping shoulders facing forwards.
then With face looking straight ahead, take the left ear towards the left shoulder-Repeat to right.
then Look up to the ceiling and then down to the floor.
Because clothing frequently causes blisters and dressings are difficult to keep in place, children often avoid reaching with their arms above their heads as it pulls or clothes rub. This can lead to permanent tightness. These exercises are to be encouraged when reduced movement is first noticed. Lift arms out to side up to shoulder level and then to ears and lower.
then Lift arms forwards and straight above head with elbows straight.
Elbows too can become a little difficult to fully straighten or palms turn up. Bend elbows and touch shoulders, then straighten with PALMS UP. Check that the wrists can move fully up and down.
RDEB sufferers experience particular problems with joints of the fingers and hands. Regularly check that your child’s fingers will fully straighten. This can be done by putting them flat on a table. If they have become a little bent or are being always held bent start daily exercises. The webs between the fingers can begin to creep up towards the tips in this condition, exercises cannot stop the webbing, but dressings and hand wrappings seems to help. With the fingers a little apart, straighten the fingers fully. Check that they are completely straight on a flat surface.
Blisters on the feet can discourage your child from walking. Bandages should not be too tight over the tips of the toes so that they can grow properly. Ensure that your child’s ankles are not stiff as a result of habitually walking on its heels or toes to avoid blistered areas. If tightness is noted, this exercise should help. Pull feet up and then push down.