Title:The Management of Dental Treatment in Individualswith Haemophilia and Inherited Bleeding Disorders
Author[s]:David J Perry
Emily Symington
Creation Date:28th January 2016
Review Date:28th January 2018
- Introduction
Individuals with bleeding disorders do not bleed more profusely, however bleeding may be prolonged or delayed due to clot instability.
There are many inherited bleeding disorders and dental treatment will involve close collaboration between the treating dentist and the local HaemophiliaCentre. Many dental procedures, including routine surveillance, can be carried out in primary care. For more invasive procedures, individuals with a bleeding disordershould be referred to a secondary care setting.
All individualswith inherited bleeding disorders are issued a card that states the nature of their disorder, the main treatment strategies and contact details of their local Haemophilia Centre.
These guidelines will assist dental practitioners in the management of individualswith inherited bleeding disorders.
- Disorders
The following bleeding disorders are described in this document:
2.1.Haemophilia A and B
2.2.Von Willebrand disease
2.3.Factor XI Deficiency
2.1.Haemophilia A and B
2.1.1.There are 2 main types of inherited haemophilia which are both X linked and therefore affect mainly males:
2.1.1.1.Haemophilia A is 5-6X more common than Haemophilia A and is characterised by a deficiency in Factor VIII.
2.1.1.2.Haemophilia B (Christmas disease) is characterised by a deficiency of Factor IX.
2.1.2.Haemophilia A and B have similar clinical manifestations. They can be classified according to the plasma levels of the relevant clotting factor:
Mild / 0.06-0.40 IU/mL / 6-40 %
Moderate / 0.01-0.05 IU/mL / 1-5 %
Severe / <0.01 IU/mL / < 1 %
Individuals with Haemophilia A and B can develop alloantibodies[‘inhibitors’] following exposure to clotting factor concentrates. These Individuals are managed in secondary care centres with expertise in managing haemophilia complicated bya factor inhibitor. Individuals with such antibodies will be aware of this.
2.2.Von Willebrand disease (VWD)
This is the commonest inherited bleeding disorder and is characterised by a deficiency or abnormality of a plasma protein known as von Willebrand factor. These disorders are in general autosomally inherited and therefore affect females and males.
Von Willebrands disease is characterised by mucocutaneous bleeding and often presents following dental extractions.
There are 3 sub types of von Willebrand disease.
Type 1 / A quantitative deficiency of von Willebrand factor. This accounts for 80% of cases of VWD
Type 2 / A qualitative deficiency.
Type 2 VWD is subdivided into Types 2A, 2B, 2M and 2N.
Type 3 / Asevere quantitative deficiency.
This is rare and characterised by almost a complete absence of both Factor VIII and Von Willebrand Factor.
2.3.Factor XI Deficiency
Factor XI deficiency is autosomally inherited and affects both females and male. It differs from Haemophilia A and B in that bleeding phenotype is often unpredictable and may notcorrelate with the plasma concentration of Factor XI.
- Prevention
The prevention of dental problems is an essential part of the management of an individual with an inherited bleeding disorder. This aims to reduce the need for invasive treatments and emergency visits.
All children should have a caries risk assessment, based on the SDCEP prevention and management of caries in children guidance (Scottish Dental Clinical Effectiveness Programme: Children with inherited bleeding disorders should be allocated as high caries risk with enhanced prevention. Emphasis should be placed on the importance of regular dental follow-up (usually every 6 months) to help identify problems at an early stage.
Advice should be given on oral hygiene, including:
- Tooth brushing technique and the use of a fluoride containing toothpaste
- Duration of tooth brushing – ideally 2 minutes twice a day
- Regular replacement of toothbrush
- The use of disclosing tablets to locate inadequately brushed areas
- Dietary advice should be given to each individual. This should include advice regarding reduction in the amount a frequency of foods containing high levels sugar and acid.
- Smoking should be strongly discouraged
- Management Strategies
- When planning dental treatment there are several factors that should be taken into account:
- The type and severity of the bleeding disorder
- The location and extent of the dental procedure
- The experience and confidence of the treating dentist
- Access to a local Haemophilia Centre
- Options for therapeutic management of procedures include:
- Local haemostatic measures and suturing
- Anti-fibrinolytic agents (e.g. Tranexamic acid) to enhance clot stability
- Desmopressin (DDAVP) to release endogenous factor stores
- Clotting factor replacement
- Local Haemostatic Measures and Suturing
These measures provide a useful adjunctive treatment to factor level increment for dental extractions and invasive dental procedures. Both resorbable and non resorbable sutures have been used.
Local haemostatic measures include oxidised cellulose (Surgicel), resorbable gelatine sponge (Gelofoam), cyanoacrylate tissue adhesives and surgical splints.
- Anti-Fibrinolytic Agents
Tranexamic acid is useful, either as a single agent or in combination with factor augmentation to help minimise bleeding risk in individuals with inherited bleeding disorders. It inhibits clot lysis by competitively inhibiting the activation of plasminogen to plasmin. This can be administered intravenously, orally and as a mouthwash for local effect.
The dose of oral Tranexamic acid is 15-25mg/kg or 1g, for the majority of adults, every 6-8 hours. This should be given 2 hours pre-procedure and for up to 5-7 days post procedure. Alternatively, this can be given as a mouthwash which has the benefit of local anti-fibrinolytic activity whist minimising the systemic effects. Tranexamic acid mouthwashes (10ml of a 5% solution) should be given just prior to the procedure and then continued 6 hourly for 7-10 days. In practice Tranexamic acid mouthwashes may be difficult to source in primary care and therefore Tranexamic acid tablets may be prescribed.
Tranexamic Acid will be prescribed and administered by the Haemophilia Centre
Tranexamic acid is contraindicated in individualswith haematuria.
- Desmopressin (DDAVP)
Desmopressin is a synthetic antidiuretic hormone which stimulates the release of Factor VIII and von Willebrand factor fromthe endothelial stores. This causes a rise in factor VIII and von Willebrand factor to an acceptable level for surgery in those individualswith mild Haemophilia A and von Willebrands disease. These individualsshould have a trial of DDAVP at their local Haemophilia Centre prior to any dental surgery to ensure that they achieve acceptable factor levels.
DDAVP does not cause a rise in Factor IX levels and is not, therefore, a suitable treatment for individuals with Haemophilia B.
DDAVP can be administered subcutaneously, intravenously and intranasal. It should be given at the local haemophilia centre 1 hour prior to the dental procedure. The effects become diminished on repeated administration due to exhaustion of the endothelial stores. Side effects include mild tachycardia, hypotension, facial flushing, headache and abdominal cramps. Fluid intake should be restricted to 1.5L for the 24 hours after administration.
Desmopressin will be prescribed and administered by the Haemophilia Centre
Desmopressin should be avoided in:
Individuals with ischaemic heart disease
Individuals >65 years of age
Children under the age of 2 years.
- Coagulation Factor Replacement
Factor replacement is the treatment of choice for individualswith moderate or severe Haemophilia A or B or von Willebrands Disease who are undergoing invasive dental procedures. Many individualswith severe haemophilia are on regular prophylaxis and any dental procedures should be timed to coincide with this where possible. This minimises the risk of therapy and reduces treatment costs.
The factor concentrate should be given as close to the procedure as possible. This may be given at the local Haemophilia Centre or by the individual themselves or, in a child, the parents of the child. The aim should be to increase factor levels to 0.50IU/ml [50 %] prior to the procedure. Individualswith severe haemophilia may need monitoring for 24 hours for signs of delayed bleeding and for further administration of clotting factor concentrate.
Individualswith Haemophilia A [and very rarely Haemophilia B] complicated by inhibitors and who require invasive dental surgery should be managed in the hospital setting.
Clotting factor concentrates may be prescribed and administered by the Haemophilia Centre but in many individuals on home treatment programmes they will treat themselves [of in the case of the children, the parent] in advance of any dental treatment.
- Dental Treatments
There should be a clear treatment plan for each individualprior to dental intervention.Liaison between the treating dentist and the haemophilia centre is important. This ensures that a management plan is tailored to a particular individual depending on their underlying bleeding disorder and the dental intervention that is required.
It is important to ensure that there is not damage to the oral mucosa during any procedure. This can be achieved by careful use of saliva injectors, careful removal of impressions and care in the placement of radiographic films.
9.1.Scaling and Periodontal Treatment
Routine periodontal probing, supragingival scaling and polish are unlikely to cause excessive bleeding and do not require additional intervention.
If gingival health is poor, an immediate treatment plan may be needed which may involve several visits to prevent excessive bleeding. This may require additional intervention and the Haemophilia Centre should be contacted to make a joint management plan prior to treatment.
9.2.Prosthodontic Treatment
Full or partial dentures and removable prosthesis can be used without any further intervention.
9.3.Orthodontic Treatment
Fixed and removable orthodontic appliances can be used in individuals with bleeding disorders. The importance of oral hygiene should be stresses along with enhanced preventive advice. The appliance should be designed to ensure that there are no sharp edges or wires that could damage the buccal mucosa. Bands on molars and premolars should be avoided. Temporary Anchorage Devices (mini-implants and mini - plates) should be avoided and alternative, less invasive anchorage reinforcement methods should be used instead.
9.4.Restorative Procedures
Restorative procedures, including crowns and bridges, can safely be carried out in general dental practice as the bleeding risk is minimal. However, coagulation factor replacement will be required if an inferior dental block or lingual infiltration is required (see guidance on anaesthesia).
9.5.Endodontic (root canal) Treatment
The risks of bleeding are minimal and the majority of cases can be performed in general dental practice. Bleeding and pain may cause problems if vital pulp tissue is present at the apical foramen. The use of 4% sodium hypochlorite for irrigation and calcium hydroxide paste appears to minimise this problem.
- Implants
Treatment should be individually planned with the haemophilia centre as evidence based protocols have not been established.
- Dental Extractions and Oral Surgical Procedures
Dental extractions should be planned with liaison between the dentist carrying out the procedure and the local haemophilia centre. An individualised treatment plan should be made taking into account an individual’s underlying bleeding disorder, geographical distance between the dentist and haemophilia centre and the nature of the surgery required.
If factor replacement is required this should be with an aim to bring factor levels up to 0.50 IU/mL [50 %] just prior to the procedure.
In individualswith an inhibitor to factor VIII or IX any surgical intervention should be planned carefully. The Haemophilia Centre will arrange treatment with bypassing agent but such cases are rare and treatment should be performed in a secondary care setting.
- Anaesthesia and Pain Management
In adults, local infiltration with a modern fine gauge, single use needle, using a slow injection technique, can be used without the need for factor replacement. In children advice should be sought from the haemophilia centre.
Procedures that do NOT require Factor replacement (Adults only.For children seek advice from the haemophilia centre) / Procedures that require factor replacement (Adults and children)
Buccal infiltration / Inferior dental block
Intra-papillary injection / Lingual infiltration
Intra-ligamentary injections
Analgesia for dental pain may be necessary if there is infection or post-procedure.Paracetamol and codeine based drugs are safe analgesics for individuals with Haemophilia and similar bleeding disorders.Aspirin and aspirin containing medications must be avoided in individualswith inherited bleeding disorders.
Non-steroidal anti-inflammatory medications should generally be avoided but a COX 2 inhibitor may be beneficial and its use should be discussed with the haemophilia centre.
If there is evidence of infection and antibiotics are required these can safely be prescribed in individuals with inherited bleeding disorders.
- Transfusion Transmitted Infections
In the past individualswith haemophilia may have contracted hepatitis or HIV from plasma derived products from infected donors. There is no evidence to suggest that HIV has any influence in the outcomes for dental procedures.
There are no special precautions required for individualswith bleeding disorders and the same level of infection control should be implemented for all individuals.
Any individual treated with UK-sourced plasma products between 1990 and 2001 are categorised as ‘at risk’ of Creutzfeldt-Jakob disease (CJD) including variant CJD [vCJD], for public health purposes. These individualsshould be treated as any member of the general population. It is important that the individuals‘at risk’ status is conveyed to the individualsdentist by the haemophilia centre. Satisfactory decontamination procedures are required.
References
2013 / British Dental Journal, 215:497-504, 2013 / Guidance on the dental management of patients with haemophilia and congenital bleeding disorders / British Dental Journal / May 2016Page | 1