Treatment

Apart from external blistering internal blisters occur in the larynx and pharynx. Blistering in the mouth does occur when for example, hard or crunchy food (for example, a rusk) has been chewed. The (internal) blistering may cause a number of problems such as tender mucous membrane, smaller mouth opening, decreased saliva production and loss of tongue mobility because of the fusion of the tongue to the floor of the mouth.

The EB-patient has often poor teeth due to the poor quality of the dentine and/or enamel. Therefore, the teeth are prone to caries. Moreover, the teeth often have an abnormal structure with absent dental elements. The possible effects of the mouth problems and poor teeth can be divided into the following categories:

• problems with nutritional intake;

• problems with chewing and swallowing of food;

• speech problems;

• negative self-image because of altered appearance and speech problems.

This module discusses mouth and dental care. The problems with nutritional intake and the problems with chewing and swallowing of food are described in the module: Obtaining and maintaining a good nutritional status.

Support to improve speech is described in the module: Prevention and treatment of fusions and contractures.

The emphasis in teeth care is in the prevention of caries. Dental caries is caused by acid formed by bacterial breakdown in plaque of sugar in the diet. This acid affects the enamel and dentine. In order to prevent caries the intake of sugar should be restricted. However, these patients have a higher requirement for a high calorie diet. It is better to restrict high sucrose foods to meal-times and use as little sugary snacks as possible. Use snacks such as sausage or cheese instead of these.

The teeth are often insufficiently cleaned or they are not cleaned at all because of the sensitive oral mucous membrane and because the contact with the toothbrush is so painful. Furthermore, the mouth cannot be opened wide enough to brush the teeth properly; especially the dental elements further to the back of the mouth cannot or hardly be reached.

Exercises can prevent the mouth opening from becoming too small (see: Prevention and treatment of fusions and contractures).

The teeth should be cleaned with a small, soft brush (for example, a baby toothbrush). The head of this toothbrush is small enough to reach the dental elements further back as well and soft enough not to irritate or damage the oral mucous membrane. However, this kind of toothbrush wears out quickly.

In stead of (or in addition to) the toothbrushing, the mouth may be rinsed with chlorhexidine 0,1% or HextrilŽ. This kills off the bacteria that are present in the mouth. It does not remove plaque (which is exactly the ideal culture medium for bacteria) and subjectively does not give a sense of clean teeth either.

The mouth may also be rinsed with a mouth spray. When a mouth spray is used hard jets of water should be avoided because they may irritate or damage the oral mucous membrane.

Preventative dental care is required to keep the teeth in an optimal condition. The use of specific devices for reaching the small corners and cavities of the teeth and removing the plaque effectively may be helpful. The condition of the teeth needs to be checked regularly too, and problems must be identified at an early stage.

If despite all preventative measures and care the condition still deteriorates, dental treatment will be required.

Dental treatment is usually performed under a general anaesthetic because:

• the work area cannot be reached or hardly be reached due to:

- the contracted mouth opening;

- the sensitivity of the oral mucous membrane.

• the risk of complications that require acute action such as:

- blistering in the mouth or throat. This may obstruct the airway;

- other `alarming situations' such as the breaking off of the root rests or rests of filling material. These rests may be breathed in, swallowed or (unnoticeably) disappear in the sinuses.

Which elements need to be extracted is not only determined by the condition of the teeth, but also by the question of whether the elements can still be reached in a couple of years for daily maintenance or dental treatment, because usually an increase in the contraction of the mouth can be expected.

Since the mouth of a human being opens hingewise (and not vertically as in a snake) every vertical loss of mouth opening means loss of workspace on the place of the molars. The further the mouth can open the more vertically the teeth can be extracted which causes a minimum of damage to the bone on the side; because of this the elements with the strongest roots are in the most difficult place to reach.

The time of treatment might depend on the general medical condition of the patient. For example, dental treatment (or a thorough inspection of the teeth) may be performed when the patient has been admitted for an operation and is therefore administered an anaesthetic anyway. Particularly patients with fusions in the hands will have to be admitted regularly for corrective surgery. Depending on the moment of surgery, this means that molars might be extracted that would have been satisfactory for a number of years.