Haemophilia patients are special patients from a dental point of view as routine dental treatment, including extractions, can be and often is life-threatening.
Primary dental care is where the haemophilia patients themselves, together with their immediate and extended family and friends, all help to prevent dental disease in the first instance.
The two main dental diseases affecting all persons, including those with haemophilia are:
Dental caries; and
Dental gingivitis/periodontitis (gum disease).
Both these diseases are almost totally preventable with the knowledge that is currently available to those who
Dental caries (holes in teeth) occur when the hard tissues of the tooth, namely enamel and dentine, are softened by demineralisation caused by bacteria on foods, especially sugars, which produce an acid that demineralises the hard tooth surface. This results in a collapse of the hard outer enamel covering of the tooth and leads to softening of the dentine, which will eventually increase in size and expose the underlying pulp and nerve tissue, causing dental pain, abscesses and death of the living part of the tooth.
Dental pain as a result of caries only occurs when the condition has developed for months, if not years, once the hole has travelled through the hard tooth structures and is adjacent to or near the pulp or nerve tissue.
The main cause of tooth decay (caries) is unrestricted eating of sugars which are found in various forms including sweets, cake, biscuits and soft drinks.
Excessive drinking of natural fruit juices instead of water can also cause a demineralisation of the enamel of the tooth. The very high acidity levels in natural fruit juices can result in caries-like lesions or demineralisation of the tooth structure. This can also cause some of the teeth to be shortened by being ground down, even by the normal eating patterns of the teeth or by the tongue moving over the back of the demineralised tooth structure. This is not to say that natural fruit juices are no good and wholesome; it is excessive consumption is the problem. This is explained more fully in the section on diet.
Prevention of Dental Caries
The best way to prevent dental caries is to increase the resistance of the tooth to decay by ingesting enough fluoride in the diet.
Fluoride is a naturally occurring element. It is found in rocks, clay and water. When present in water, the fluoride helps strengthen the teeth and make them more resistant to decay.
Natural water fluoridation less than 0.8 ppm is not enough to be effective. In areas of the world where fluoride in the water is absent, fluoridation could be begun as a public health measure, where approximately one part per million of fluoride is added to the public water supply.
The amount of fluoride needed in public water depends on climate, etc. Advice from the local Department of Health or from a dentist should be sought, so that the maximum amount can be supplied. All patients, including those with haemophilia, would benefit by receiving fluoride in the water supply. The resultant caries disease reduction effect is in the order of 60% worldwide.
In areas where this facility is not available or beyond the resources of the community or the engineering capacity to provide it, fluoride can be provided in two other ways:
In tablet form, taken once per day. The dosage depends on the age of the child and the amount of fluoride available in the natural water supply.
In a mouthrinse, administered once a week by local healthcare personnel. Very little specialised training is required for people to implement a healthcare mouthrinse programme. Rinsing programmes are not advised for children less than six years of age, as they are unable to spit the mouthwash out fully.
Current Dosage Schedule for Fluoride Supplements in the UK in Relation to Fluoride Concentration in Drinking-Water
Fluoride dosage (mg/day) by age group
Fluoride in drinking water (mg/litre)
6 mths - 2 yrs
2 - 4 yrs
4 - 16 yrs
0.3 - 0.7mg/litre
Courtesy of WHO Expert Committee on Oral Health Status and Fluoride use - Nov 94 Geneva
Either of the above two ways are more selective, in that the fluoride is delivered directly to those affected by haemophilia. The availability of fluoride in toothpaste is also essential. Some countries are putting fluoride in salt as another method of community distribution.
The overall costs of any of the above services are insignificant compared to the benefit per head of population, particularly of people with haemophilia. In the case of this susceptible group, all means or a combination of the above should be seriously considered in co-operation with the local dental authorities in each jurisdiction to implement a cost-effective, efficient programme. The local Department of Health will advise on details of local application of dosages, etc., in all cases.
A second way of reducing the incidence of dental decay is to apply a fissure sealant to the biting surface of back (molar) teeth. This is carried out by drying the biting surfaces of the teeth, applying a special acid for only 15 seconds followed by washing the tooth surface with water for 15-30 seconds to wash the acid away, drying the tooth again and subsequently applying a (biz-gma resin) plastic coat to the microscopically roughened surface of the tooth which will in effect seal the cracks on the biting surface of the tooth. This could further reduce the incidence of decay or caries.
The third method is to cut down eating foods between meals that are high in any of the sugars (fructose, sucrose, maltose, etc.). In effect, eating any food containing sugars between meals causes an increased acidity of the saliva. The pH can drop below 5.5 (normal 7.0), and demineralisation of enamel and dentine can take place at this level. This also applies to natural fruit juices taken instead of plain drinking water.
It would appear that sugars eaten at main meals, or where the incidence is less than three attacks a day which last no more than 45 minutes in all, have an insignificant role, particularly if combated by fluoride applications as mentioned above.
If, however, there is continuous exposure to acidic attacks by continuous eating or drinking with any sugar-containing foods, then the acid attacks could in theory proceed all day. This would result in rampant decay which will progressively cause the teeth to decay within a very short period and then to become unrestorable. This would have a catastrophic effect on any haemophilia patient particularly where replacement factor was unavailable or even restricted. Sugars are found in all processed foods, including sweet cakes, biscuits and soft drinks.
Implementing this dietary restrictive attitude requires co-operation, particularly of the parents, guardians and the haemophilia community. It requires their full co-operation to make sure that the sucrose exposure time is reduced to a minimum to further prevent decay.
This dietary restriction of sugars is not easy on the patients themselves, their families and the community. However, it is a proven fact that if it is rigorously adhered to, then the misery of tooth decay could be eliminated almost completely.
These methods, if used in combination, could effectively reduce the incidence of dental caries in haemophilia patients by a significant amount (70-80%) and save a great deal of pain and worry. This problem can affect people of any age, but it affects mainly children and young adults.
Gum disease is very common in the world and affects all nationalities, creeds and races, irrespective of social/economic group. While it can affect children, it is mainly a disease of adults and the aged.
To develop gum disease, one must have teeth. Gum disease is a disease of the gum and bone which holds the teeth in the mouth.
Development of Gum Disease
Saliva, present in peoples' mouths, is a solution which, when released from the glands in the mouth, allows calcium ions to be released.
In addition, the salivary fluid itself consists of long-chained sticky molecules. These long-chained molecules are deposited on the teeth and are worn off most parts of the teeth under normal eating function, except at the point where the tooth enters the gum. This sticky matrix provides an ideal area for bacteria to attach to the teeth. The calcium ions are also deposited in this matrix film which hardens to form tartar, or calculus.
The sticky matrix or "film" which forms on the teeth is called "plaque". It is a bacterial active matrix and every time a person feeds him- or herself, he or she also feeds the bacteria.
The by-products of such feeding of the bacteria causes the development of endotoxins or poisons which causes an inflammation of the gingival (gum) tissues, bleeding and bad breath.
If this "film" is left undisturbed, then each day allows a new deposit to form on the previous day's deposit. After about four days, if still undisturbed, a more aggressive type of bacteria forms which is much more virulent in causing inflammation of the gums.
To put this into perspective, if it were possible to start with a totally sterile mouth and then allow the normal bacterial flora development to take place in the mouth, there would be a bacteria count of approximately 400 million bacteria attached to the teeth at the end of 24 hours. It is these bacteria which are the active ingredients in the plaque which causes gum disease.
Dental plaque is also called the invisible enemy, in that, under normal circumstances, it cannot be seen by the naked eye. However, it can be made visible using a strong-coloured vegetable dye, which stains plaque a dark purple/red to a light pink colour, depending on the type of dye used. The dye is sometimes manufactured in tablet form which is chewed and swished around in the mouth. The mouth is then rinsed with water, which washes out the dye, but the plaque remains stained. The objective is to make plaque more easily seen by patients, because if they see the plaque, they can more easily understand where it is and have it removed.
The objective of any oral hygiene cleaning procedure is to lower if not eliminate the bacterial count to a level where the bacteria can be dealt with by the patient's natural disease resistance.
Cleaning can be achieved by oral hygiene aids, such as toothbrushes, inter-dental sticks or chewing sticks. The objective, again, is to remove the plaque which is stained. When the amount of plaque on the tooth surfaces is lower than 20%, there is usually little gum disease.
There are other chemical ways used to lower the bacteria count, such as 0.2% of chlorhexidine, which is the most economical and efficient chemical that can be used to reduce the bacteria count. This chemical has been used for years in a mouthwash and is very effective. However, the teeth must be cleaned initially, otherwise a brown stain may become visible. This is a surface stain and can be removed; it is not disease.
All toothpastes are simply lubricants with some abrasive properties and a nice taste which may help to make it more pleasant and, therefore, increase the frequency of use. Usually it contains no medicinal properties. However, if it is used frequently, mainly in the morning and before going to bed at night, it is very effective as an aid to brushing/cleaning.
Some toothpastes do contain fluoride, which is absorbed by the outer layers of the enamel. This produces a much stronger enamel surface, which is more resistant to decay. See the section on fluoride for details.
Some toothpastes may also contain another agent, namely an active enzyme, which acts on the plaque itself and reduces its formation.
Susceptibility to Gum Disease
It would appear that in any given population, even with the most stringent oral hygiene procedures, about 10% of the population would be susceptible to the by-products of bacteria (namely endotoxins or poisons), i.e., the inflammatory response is in excess of what might be seen in the normal population. Despite extensive research, there is, as yet, no way that these susceptive individuals can be predetermined. The progression of gum disease in these individuals is faster than normal, resulting in loss of gum from around the teeth and, more importantly, loss of the underlying bony support holding the teeth in position. Stringent oral hygiene will reduce the rate at which this gingival support is lost, but may not eliminate it entirely, even in susceptible patients.
Who Can Support Its Message?
The first and most important group to bring this message to haemophilia patients is the patients themselves, closely supported by their family, enlarged family and friends.
This message must also be supported by the professionals involved with the patient, whether a doctor, dentist, nurse, nurse's aide or other healthcare professional associated with the patient and his family.
In various countries and regions throughout the world, some dental professionals/dentists are trained to enable direct assistance to the patients and their families.
These healthcare workers (H.C.W.) can be trained to teach the preventive oral hygiene practices to individuals to care for their own teeth. They can and should train the parents and/or guardians of such patients also. One of the important issues is to assist patients in maintaining their own oral hygiene, and so prevent caries and gum disease.
Where Do We Start?
It should be the prerogative of the national haemophilia organisation in each country to identify, with the assistance of the national medical and dental teams' support, all persons with haemophilia. These persons should then be specially targeted with a dental preventive programme to reduce, if not eliminate, the incidence of preventable dental disease that causes these patients such a problem. In this way, the major problem in providing dental treatment for patients suffering from haemophilia could be diminished to a level which could be managed in each given region in a more economical and efficient way and create less problems for patients.
All patients with haemophilia should be assessed for treatment needs, not only in the procedure that should be undertaken, but also in the time it would take to carry out such a procedure, so that if an opportunity develops at some time in the future, they could be called up at short notice and have their dental needs attended to.
This identification of haemophilia patients would not only allow their dental treatment needs to be identified, but also allow this group of patients and their families to be specially targeted with the preventive dental care message to reduce the progression of existing diseaseand diminish future dental treatment needs.
This may necessitate, depending on circumstances, yearly reviews, so that the list of patients with haemophilia is constantly updated and modified. In doing so, a realistic, holistic treatment plan is constantly available to manageable groups of patients who can be treated at a pre-determined time when any replacement factors become available.
This requires co-operation and co-ordination between the medical and dental team, which is best achieved when they work in an integrated fashion and have a good understanding of the problems that each team has.
It must be remembered that, in the case of dental disease for patients with haemophilia, prevention is better, cheaper and safer than the treatment of the result of dental disease itself.
Development of a Dental Team with Regard to Treatment of Patients with Haemophilia
For the best results, the dental team which provides services to patients with haemophilia should be part of an overall integrated medical team. As such, the dental team should be housed in, or extremely close to the haemophilia centre. In this way, they should be able to achieve a holistic approach to any individual haemophilia patient.
Dental personnel with an interest in haemophilia should be identified, if not already contacted, by the local medical directors. Sources for interested personnel are the local dental school or hospital which is usually situated in a large city, and through the local branch offices of the national dental association.
If resources and/or distance is a problem, in some areas, members of the dental profession, either nationally or internationally, would be available to assist or train appropriate healthcare workers, who could carry out the duties as described in the preventive programme above.
Specialized dental healthcare workers, whose aim would be to develop the appropriate approach to prevention at a local and personal level, could then return to their local centre or area and bring the message "home" to both the parents and the patients.
Since the first tooth erupts in a normal baby at approximately 6 months, it is never too early to start the preventive approach. In that context, normal tooth development and eruption and/or normal shedding of baby teeth do not usually cause a major problem for patients with haemophilia.
The Ideal Solution
The ideal solution would be to have a dental team in attendance which is directly associated with the various medical centres. This would necessitate assigning them an area where the dentist could examine and assess each patient, as to their appropriate dental needs. Once the treatment has been decided, it then must be implemented.
The necessary medical back-up would then be readily available for the patient, where factor replacement therapy (F.R.T.) is available. Where full F.R.T. is available, it should be supplied to the patients prior to the commencement of dental treatment. However, in some instances, due to costs and/or availability of F.R.T., dental teams should be contacted after a full dental assessment has been undertaken, including assessment of the prevention and compliance of existing preventive programmes mentioned above. In these circumstances, only dental treatment which has a definitive diagnosis should be undertaken for patients with haemophilia and a definitive treatment plan implemented.
Helpful Guidelines in Developing a Dental Treatment Strategy
Patients with haemophilia are different from ordinary patients in that the latter group can have multiple visits to achieve a definitive dental treatment, even on one tooth. This is a luxury that is not usually available to patients with haemophilia; therefore, a much more restrictive view must be taken. Assessment of the necessity of treatment must be taken, as there may be only one opportunity for dental intervention to take place.
So by definition, the options available to the dentists responsible for making the decisions for treatment of patients with haemophilia are much more restricted. There are multiple causes for this, including costs, availability of service, and availability of factor replacement (F.R.) post-operative care.
When F.R.T. is available in conjunction with the medical team, the actual treatment has been decided on (assuming that the restrictions mentioned above can be undertaken) and the factor level is replaced, patients will behave similarly to those without haemophilia.
In relation to infiltration anaesthesia used mainly in the upper jaw, a 30% factor rise is a normal requirement.
However, where extractions, deep scalings, and/or inferior dental nerve (lower jaw) injections deep into the tissue are anticipated, then a 50% factor rise would be the level of choice.
Where the procedures intended are much more extensive, or become more extensive as the procedure develops, such as a surgical extraction, then a 100% rise may be necessary. This might necessitate an increase in the rise from 50% to 100% immediately post-operatively, if the treatment being undertaken becomes more extensive than was originally planned.
Surgical extractions should not be undertaken lightly. They should only be undertaken when the circumstances absolutely demand it.
In all cases of patients undergoing dental treatment involving blood clotting, the patient should be prescribed tranexamic acid. The adult dose is 500 mg, one tablet four times a day, to be taken for 10 days post-operatively to prevent the breakdown of any clot that forms. For children, the above dose must be modified for their size and age. If no extractions or periodontal treatment is undertaken, only restorations, then the tranexamic acid should only be taken for three to seven days.
While the use of sutures following extractions can be warranted, there is normally no necessity for these to be in place longer than about 24 hours.
Four extractions in different areas of the mouth are less traumatic than four extractions next to each other. In the latter instance, there would be an extensive wound; because of the scalloped edge of the gum around the teeth it can be at least 50% larger than the distance corresponding to the extracted teeth width. If the sutures are removed within 24 hours, there is usually no need for a follow-up F.R.T. rise. Sometimes, it may be necessary to retain sutures for longer, but a dentist would decide when it is appropriate; usually it would only occur in an exceptional case if the dental surgery is very extensive.
In relation to any oral surgical intervention, such as extractions, it is of paramount importance that there is the least possible interference with the attached gingiva (gum) around the teeth and periosteum Simply lifting the attached gum tissues from the underlying tissue or periosteum, even in healthy patients will cause post-operative bleeding. The goal is "key hole" surgery, where there is as little interference as possible with the attached gingiva (gum), to minimise post-operative bleeding.
Restorations for Treatment of Caries
Restorations or fillings should only be undertaken where there is a minimum disease process in the tooth involved. This will mean that the resultant restoration should have a sound long-term prognosis. It is my belief that restorations of doubtful prognosis should not be undertaken in patients with haemophilia, especially where there is restriction in the amount of F.R.T available or post-operative care.
It must be remembered that the placement of fillings in teeth is more time-consuming than extractions. It is much better that patients, especially those with haemophilia, retain their teeth. The extra time involved could restrict the number of patients who could be treated within a fixed period of time, especially if the amount of F.R.T available was restricted.
There is sound scientific basis that periodontal treatment can be managed by the patient in a preventive programme. This means that pocket depths or loss of attachment of the gingiva (gum) up to 4 mm has a very sound prognosis and should be capable of being maintained by a patient on a realistic homecare programme, which they must comply with.
However, for patients with more extensive periodontal disease or, more especially, with infra-bony defects between the roots of some of the molar teeth, then the prognosis is much more guarded and as such makes treatment probably unsuitable for patients with haemophilia, especially if the service has to be limited in any way. In addition, the time element in undertaking such a procedure and the necessity of repeating procedures makes it a far from realistic treatment goal.
Dentures or Orthodontic Treatment
There is normally no problem in providing a routine prosthesis or denture for a patient with haemophilia or undertaking simple orthodontic procedures with either fixed or removable appliances. However, these prostheses in themselves, as with all prostheses, generate an accumulation of plaque around them, which increases the necessity for homecare preventive programmes, some of which have to be specially tailored for the individual needs of the patient.
This preventive programme can be best undertaken by healthcare workers, specially trained in the field to look after such patients, as mentioned earlier.
Where Factor Replacement Therapy Is Not Readily Available
In centres where F.R.T. is not available, alternative treatments of plasma replacement, whole blood, and DDAVP have severe limitations in their own right. In circumstances such as these, treatment other than for the relief of pain, such as extractions, may be the only realistic treatment option available to patients with haemophilia.
Conclusion It gives me great pleasure to bring my experience in treating patients with haemophilia to those who have the disorder and to those in the healthcare field who are having difficulties treating dental disease in this very special group of patients or who wish to set up such a programme.
I know that dental and medical services are not readily available to the vast majority of hemophilia patients.
I hope, however, to raise the level of awareness that dental disease, in the majority of cases, is almost totally preventable and should not be a major affliction, even to those who are restricted in the level of service available to them.
In areas with limited service, the emphasis should be on getting the co-operation of the existing medical, dental and health authorities and national haemophilia organisations to pool their resources together to get this message across to the people that matter - the patients and their families.
1 Treatment of Hemophilia Monograph Series, Number 3. World Federation of Hemophilia: 1996, revised 2000.
2 School of Dentistry, Trinity College, Dublin, at the Dublin Dental Hospital. Lincoln Place, Dublin 2, Ireland; Tel.: +353 +1 +6127321; Fax: +353 +1 +6127298; E-mail: firstname.lastname@example.org. I would be delighted to assist in the development of dental programmes, especially for haemophilia patients.