The speed of neurological deterioration in ataxia telangiectasia is like all the other symptoms depending on patients: if for most, walking is acquired at the normal age, the nature and occurrence of disruptive actions, of rigidities or blockages doesn't seem to obey any specific rule.

But once these are in place, it is futile to try to correct them without causing unnecessary suffering.

However, the role of professionals is to maintain skills and independence in the movement as long as possible. To achieve this, they have many materials to meet a psychologically important criterion: independence in mobility.

Feet and ankles

The use of specific equipment on the feet and ankles should meet in three critirias:

• Improve comfort
• Improve stability and posture
• Slow the progression of the deformity

Can be used:

• Soles that can in some cases improve the stability
• The ankle brace, slowing the amplitude of the ankle without restricting it, can also improve the stability
• Orthoses, more stringent, will restrict the amplitude of the ankle, preventing unwanted movements and improving stability. They can also delay the installation of the deformation. There is no requirement to keep them full-time because they are not necessarily comfortable at rest. It depends on the purpose.

Simple ankle boots, or even boots, can bring some benefits of braces and are more aesthetic, which is a criterion not to be neglected for your child.

pay attention however that the soles of shoes are not too adherent because they won't forgive the slightest unbalance. Example: basketball shoes. Paradoxically, a smooth sole leads to be more vigilant and forgives more walking errors.

In all cases, it is your experience and your child who will decide whether such equipment is useful or not. Then you must try!

Walker

When the number of falls increases or the impact of fatigue on his activities due to his moves is too large, the use of a walking aid may restore the independence of the child.

The use of the type of products shown on the left can protect against the back and sides falls. Some walkers, as the one on the right, offer lateral support of the body and seat. The choice depends on the difficulty level of the child. The catalog of products offered is rich enough to find a solution for every case.

Assisted mobility

Always consider a material as an integration tool: acting in the travel difficulties of the child, these tools allow him to remain integrated in the community.

Thus, when the walker is no longer sufficient and the level of fatigue due to his travel becomes too high during long trips, for example, consider switching to the use of a rolling equipment: a stroller, a carrier seat or a wheelchair. Contrary to popular belief, the transition to a wheelchair or an equivalent increases the independence of the child and has no influence on the development of ataxia telangiectasia: it does not aggravate the symptoms.

The transition to a motorized solution must be considered depending on the weight of the person and on the degree but moreover the need for autonomy which increases with age. However, the cost of such equipment is sometimes deterrent, even if some financial aid may be available. One can also look at the used materials, including on the Internet, but then the difficulty is to find the suitable options. It is therefore important to define precisely the requirements before making a choice to avoid paying unnecessary functions.

The criteria for selection of material must of course be viewed with professionals: physical medecine, occupationnal therapist et physiotherapist. Must be considered:

• First, the opinion of the child
• comfort
• easy installation
• deportment in the equipement
• usability: ease of use for himself and his entourage and transportation possibilities
• adapting the device to the environment and activities of the patient: home, school, work. For example, some electrical wheelchair are able to take the stairs.

Again, the choice is vast and the images (links) of this section are only examples. You will find a comprehensive choice on more specialized sites.

 

In conclusion, there is not only one right way to move, but one for each situation: we must let the child decide because his decision is his independence. Children are pragmatic and take what make them autonomous. They generally accept the wheelchair much better than those around them. That is why we must not delay the implementation of these tools, pending the future and the development of exoskeletons.

 

The future

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With improved battery life and power of electric motors, the first models of exoskeletons emerge and are gradually marketed, in Japan first, the world leader in robotics.

An exoskeleton is a kind of combination, robotics or not, close to the body, which provides physical assistance for movement.

The first models are intended for the army, the industry and assistance of medical personnel in hospitals and medical centers, to carry patients more easily and facilitate their transfers. But the final target and clearly exposed by the Japanese is the population of senior citizens whose abilities decline with age.

If it is not unusual that the first applications are targeting significant business opportunities, the improvement of these exoskeletons and a wider distribution, inevitably resulting in price reductions, will certainly make them available for handicap people, with hope to give them real autonomy of movement, and why not return to standing and walking. The first products will also soon be available, such as these models: ReWalk company Argo Medical Technologies , Berkeley Bionics or HAL from Cyberdyne . Rewalk de la société Argo Medical Technologies , Berkeley Bionics ou encore HAL de Cyberdyne.

order helvetica, cure sans-serif;">In the act of feeding for children with ataxia telangiectasia can arise three types of problems:

• Autonomy
• Swallowing
• Quality and especially quantity of food
  

Promote autonomy at table

It is the role of the occupational therapist to provide specific tips and tools to retain the autonomy for self feeding. For example

• Give priority to objects with handles for a better grip. Thicken the handle of spoons, forks and knives.
• Give an angle to the handle of spoons to reduce the rotation of the wrist when approaching the mouth
• Encourage the catch with full hands which improves stability
• Even if it is contrary to good manners, allow elbows on the table that allow greater stability of the hands
• Increase the stability of the dinnerware by sticking underneath materials that cling
• Decrease the distance between mouth and plate by raising it
• Work on the presentation and texture of foods so that they are easy to take and to chew

If you are not handyman, you can use the tools offered by some manufacturers.

Eating with swallowing problems

The SLP may, by appropriate exercises, maintain the ability of swallowing. But above all, the respect for simple instructions allows the swallowing to take place under optimum conditions:

• Eat more often, four to six times per day, smaller quantities to reduce the length of the meals and thus fatigue
• Stand straight and head up, not backward
• Take small bites
• Alternate food and liquids to clean the mouth and the throat
• Choose or change the texture of foods. Focus on mashed vegetables, pudding, well cooked pasta, cooked fruits and vegetables, ground meat mixed with sauce
• To drink: focus on rich and creamy liquids like: milkshakes, fruit nectar, creamy soup, whole milk instead of skim milk.

You should avoid foods:

• that make crumbs, or moisten them or even prepare them in a sauce
• difficult to chew like pieces of meat, raw vegetables and fruits,...
• Sticky: mashed vegetables too thick, white bread,...

For drinks, avoid:

• Fluids without nutritional value and too liquid, like water! These are the most difficult for people with AT.
• Pay attention to the use of straw: avoid too much liquid arriving too quickly in the mouth. One sucking up each time is enough. You can also pinch the straw.

Emergency plan

To face the problem of false routes including the fact that they are getting more and more silent over time, it is necessary to draw up an emergency plan with:

• You
• Your child
• A physical therapist or a SLP
• The emergency services

The general principles are:

• Stay calm
• Force or encourage your child to cough and breath between blocks
• Learn and perform the Heimlich maneuver
• Call the emergency services
• Have a code with the child. For example, a blow on the table indicates that there is no problem, one hand on the throat to the contrary that there is one.
• Adapt the plan according to the evolution of the child

The gastrostomy feeding tube

When eating becomes really problematic and that the ingested amount of food is not sufficient for daily needs and normal growth, it may be decided to use a gastrostomy tube.

Implanted by surgery, it helps to supplement food directly into the stomach while allowing to continue to eat and drink normally.

According to the feedback from the Ataxia Telangiectasia clinic of the Johns Hopkins Hospital in Baltimore, the best results are obtained when the implantation is done when the nutrition and swallowing disorders appear.

find helvetica, doctor sans-serif;">Whereas for other acts of daily life, find the only objective is ultimately to succeed, wash adds the challenge of security because the bathrooms are dangerous places: risk of slipping, drowning, burns with the hot water, electrocution ...

In response, fittings standards for the "bathroom" and "toilet" exist and are indicative of changes that can be made to facilitate access of a person using a wheelchair or with a disability. They are treated under "Interior ".

We shall speak here only of little tricks that allow an autonomous washing:

• Put on hand and available all the necessary amenities, without specific barriers and properly stored, for example in a box with compartments which will be stabilized to prevent it from falling. Avoid the bottom of the closet and bulk storage.
• Use a washcloth that will hang easaly on the hand
• Use liquid soap (even better if it is wall mounted), or a solid soap with an integrated rope to avoid the difficulty of recovering it if it falls in the bath or the shower.
• Prefer electrical appliances, for shaving or brushing teeth, which will greatly reduce the number of necessary arm movements. Rechargeable models are preferable because they avoid the action of connecting a socket that requires precision and becomes dangerous when the person presents neuromotor difficulties.