Stimulation of anatomically proper: sitting, lying down and standing all day long until the end of the child's growth, is of a special importance for the Rett syndrome. Due to the nature of the syndrome, the body position must be incessantly corrected, if we want the child to grow up normally. There are special aids for this which can be ordered through the Internet, and the choice is large. I would make a brief review of those which I consider to be the most useful for the Rett girls.
Why is positioning important?
___ 1 ___ This syndrome enormously affects muscles. It causes their asymmetric convulsion, which makes progress in years, thus producing curving of the spine, arms and legs joints, and the deformation of fingers and hand. All this CAN be avoided by proper positioning from the very beginning, while a child is still small, and in some cases, light deformations can be corrected to certain extent with younger children. I must point out that there are pediatricians, therapists and general practitioners who DO NOT believe those body deformations could be prevented, and advise parents not to undertake anything and to let it go. Why do they think that way? Because these experts mostly have no experience either with the therapy of the Rett syndrome or with the modern aids that can be found on the market today. These aids are relatively new, they are versatile and more simple to use, they are soft and light, and have been made sophisticated much faster than most doctors manage to follow. Many do not know what material the latest models are made of, asuming they are still made of gypsum and metallic bars, and that they are heavy, bulky and immobile, so they avoid them. Besides, most of the therapists have met in their career ONE or maximum two children with this syndrom, and they base their knowledge about its course on the obsolete litarature which was taking the Rett syndrome as a degenerative one. Today's researches have proved this is NOT true. That means that deformation prevention is definitely possible, and all we need is a little bit more will and persistence, and of course: INFORMATION.
I just want to remind you that until several years ago, medical "experts" did not always identify the Rett syndrome. From the year 1999, it is possible to make a diagnosis on the basis of the DNA analysis. That is why nothing was done with these children and consequently the results were mostly bad. Many doctors today refer to those results, but they forget the results are the consequence of "not working" with Rett children. That is why I advise parents to get information on positioning aids by themselves as best as they can, and then, bringing along all the information and catalogues, go to see their doctors and consult them about the aid selection. I believe that many doctors will then agree with the parents' suggestion.
___ 2 ___ Positioning is also important because curving of bones, espacially of spine and feet, may cause aggravated walking ability, and even its loss, with children who can already walk, and which can further bring them into a more passive state, and result in new deformations. This is an endless spiral. Therefore, it is better to prevent it in time.
___ 3 ___ There is an opinion of some therapists and pediatricians who are deeper in this issue, that the use of the aids causes the laziness of muscles, and because of the support the muscles get, they become less active and invigorate slowlier. This remark is correct with most disorders, however, it is NOT correct in the case of the Rett syndrome, because the children who have it, are naturally passive and will never find themselves in the situation to use and strengthen their muscles. They tend to remain in the same position for hours, but the contraction of a large group of muscles still goes on, and without their will. The Rett syndrome child does not even try to use her legs or arms, neither will she correct her sitting and lying, as her muscles are inactive themselves. This means that her muscles are passive (hypotonia) with or without the aid, and it is only the aid that can prevent excessive convultion and deformation of joints. Therefore, at any state of rest, positioning of the body is more useful than turning to spontaneous twisting and curving.
___ 4 ___ Positioning is also important because it secures proper posture of the body which enables: PROPER breathing and digestion. Both are rather disordered with the Rett children, but prevention of spine deformation and permanent body curving could be considerably alleviated. It is known that scoliosis causes curving not only of the spinal column, but of the ribs and thorax, thus diminishing the lungs function, making constant pressure both on the stomach and belly.
___ 5 ___ At the end, it is important to say that it is not necessary to pay special attention to positioning of the children who are constantly active and included in the programme of intensive daily therapy, since those girls do not sit long in the same position as it happens with passive children. Aids should be used exclusively in case when the body position remains unchanged for a long time, or when a child who cannot walk, sits in a chair for hours. The sitting posture, if not symetrical and proper, with both feet on the same level surface, very badly affects the spine, curving of hips and legs hanging loose, and on the deformation of feet. That is why lying down or semi-sitting posture is uncomparably better.
Today's positioning aids are divided into two groups: static and mobile. The static ones are those which fix the body position at rest, sleep or when transported, and a child in it can be passive but not necessarily. The mobile ones fix the body in proper symetric postion, but at the same time enable movement and playing.
For both groups, we can find aids for: lying down, sitting and standing posture.
Individual body parts can be also properly fixed, with the capacity of movement, or without it. Those are splints for arms, elbow, hand, fingers, or knee, foot, leg, according to personal choice and kind of problem. Sometimes it is necessary to use them with the active children who are intensively exercising.
STANDING
The aid for proper trunk positioning in standing posture must be: stable, safe and the standing posture should be completely symetrical, with your back straight and properly set pelvis belt. A child must not be inclined aside, or stand on one leg for a longer time. It is also important that the position of the aid is not completely vertical, because it loads the spinal column, and should be slightly inclined forward (towards the table), or backward (normal standing). The aid device itself may be static or mobile with casters. I think the child should not be kept in it for a long time, because those are children who can neither stand, nor walk alone, so this is useful for them only to strengthen the muscles of legs and spine. But it is better for them to be more in the lying posture, prostrate on the floor, and not supine, and ground exercises should be done with them.
http://www.r82.com
http://www.nenko.nl
http://www.adaptivemall.com
There are positioning aids at standing posture which also enable child's moving. They are usually used with the children who can partially stand alone, but have not mastered walking, and that is also a useful exercise for them:
SITTING
Positioning at sitting posture is of a special importance, since scoliosis is one of the most frequent symptoms of Rett. It is important for this type of aid to enable proper sitting, meaning: symetrical body position, straight hips and sholders, sitting on both legs equally and fixing feet at the same level at the right angle. For this, we usually use various correction belts, and they are quite sufficient with the children who still have no deformations. It is necessary to use both belts for hips and upper part of the trunk at the same time, because Rett children have all muscles affected by the syndrome. This type of aid is preventive, light and easy to use:
http://www.adaptivemall.com
With those children who already have curving begun on one side, there is a special correction belt,
and it should be used under the supervision of a good specialist:
http://www.r82.com/english/product/accessories/vestbelts/1.asp
This group of aids also include special chairs for getting into a car, which can be easily rotated towards the exit.
and the bath/shower chairs:
http://www.adaptivemall.com
http://www.acessinc.com
LYING DOWN
Most of the Rett girls spend their whole day lying down at home. People do physical exercises with them very little. Those are mostly totally immobile children with frequent epileptic fits, hyper-ventilation and low muscle tone, who have got heavy deformations. I think that especially with them, we can do a lot through intensive exercises, but if their surroundings (parents/lounge) cannot offer it to them, it very important to provide for them at least proper lying position: supine, on the side, or prostrate in activity posture. Here are several good examples of positioning, showing individual elements which can be bought and combined according to personal needs:
http://www.adaptivemall.com
I only want to add that proper positioning is neither difficult nor unpleasant to children. It is important to begin with it as early as possible, and the child will excellently get used to the aids, and in addition to that, they will be growing normally and will breathe better. According to my experience, children who are left to spontaneous convultions of muscles of legs, arms and trunk, really suffer, and curving of bones is not painless at all. The curved spine later has serious consequences for the health, and I would say that positioning is more human than giving freedom to Rett to show its real form.
SPLINTS
Some time ago, these splints were heavy, bulky and made of metal, gypsum and hard plastic. Today, they use soft materials, they are easy to bend and have minimum metallic reinforcements or sillicon parts, and they look nice. They are made to measure for particular parts of the trunk, which are the most jeopardized by convultion of muscles. Arms splints are most frequently used with the Rett syndrome (we can fix with them elbow, hand joint, hand or only fingers). They are rarely used for the legs, for walking and standing correction; or for fixing feet at long sitting/lying down and for the stability of hips while walking and sitting. Corsets and brace for trunk are seldom used. I am going to mention herein several examples of all said types of splints. Some of them we have used too.
Splints for the ARMS
Dejana mostly holds both of her thumb fingers in her mouth, and we have used this product to prevent deformation and to protect the child's skin from damage. It is light and soft, easy to wash and enables full mobility of the thumb. It fixes the thumb only to some extent and improves the tweezers grasp.
http://www.mckiesplints.com
From time to time, we have used the splint for hand and joint with free fingers. It is also made of the light material which does not irritate the skin, it is easily mounted and removed, and maintained without any problem. However, it totally fixes the hand joint because both at the front and on internal side of the hand, it has one long hidden metallic insert, two centimetres wide. The choice of shapes and colour are great, and they all help us break the hand stereotype for some time, and to let hand and joint muscles calm down and relax. It is noticed that some children have an increasing concentration and interest for the surroundings:
http://www.benik.com
However, when the joints deformation begin, different aids are necessary. They are made individually according to the mould, and must follow the deformity. Such aids are used in cooperation with the orthopedist, but the parents can always suggest it to be made according to some existing models from the catalogue.
So far, with the Rett syndrome people have used mostly splints which straighten hands and do not let the stereotype bending. They were made of rigid and heavy materials, but that has not been done any more, and out of all those conventional aids this is the best and the lightest one today, and at the same time easy to use:
The splint is useful for Rett girls who firmly hold their hands joined and fingers entangled, and keep them in proper position. If a child can stand it and if she is passive sitting and lying down all day long, it would be useful to hold it on the arms for several hours. There are many models of this splint and it would be easy to choose the one which suits best the current problem of the girl, whether it is twisting of fingers, convultions, separation or something else:
Splints for the LEGS
The Rett syndrome can also hit feet, joints and legs, particularly of the children who do not walk, and who are left in a pram the whole day, which is a very bad habit. In order to hinder feet curving and its permanent defomarion, and inside twisting, we can use special splints to this purpose. They could be mobile or immobile. Otherwise, the Rett children can bear legs fixing easier than arms fixing, so if a child does not walk, you can keep the foot splint the whole day. When a child is walking, it should be removed and let the leg find its way:
There are special aids which hinder twisting of legs and knees inwards for the children who can stand or walk:
This company has a vast range of products on its website and it is possible to shoose many more useful aids, which I did not mention herein. You can look for yourself what is the most suitable for your child.
The useful site with a large number of products for special needs and addresses:
http://www.makoa.org/cmpyinfo.html
http://www.beyondplay.com/CATALOG/POS1.HTM