Haemophilia and Dental Care

Hemophilia is a genetic, life threatening bleeding disorder. In persons with hemophilia (PwH) blood does not clot normally due to deficiency or absence of clotting proteins called Factors. PwH tend to bleed internally and externally even with a minor injury.

Classification

1. Hemophilia A:Hemophilia A is a deficiency of factor VIII
2. Hemophilia B:Hemophilia B (Christmas disease) is a deficiency of factor IX.

Types of Hemophilia A and B
Types of Hemophilia A and B	Degree of Deficiency/Plasma activity	Risk of a Bleed After Trauma or Surgery
Mild	5% to 30% |6 and 40 IU/dL	Delayed onset of a bleed with trauma or surgery or dental extractions
Moderate	1% to 5%| 2 and 5  IU/dL	Excessive bleeding with surgery
Severe	<1 IU/dL	Excessive bleeding with trauma or surgery

Dental treatment 

It is essential to prevent accidental damage to the oral mucosa when carrying out any procedure in the mouth. Injury can be avoided by: 

• Careful use of saliva ejectors; 
• Careful removal of impressions; 
• Care in the placement of X-ray films, 
• Protection of soft tissues during restorative treatment by using a rubber dam or applying yellow soft paraffin.

Periodontal treatment

Periodontal health is of critical importance for the hemophiliac for two principle reasons:-

1. Hyperemic gingival contribute to spontaneous and induced gingival bleeding.
2. Periodontitis is a leading cause of tooth morbidity, necessitating extraction.

Periodontal surgery requires LA and Factor VIII replacement to a level of between 50 and 75%. As expected postoperative factor level maintenance is case-dependent. In all but severe hemophiliacs, scaling can be carried out with topical anesthesia. . The treatment may need to be carried out over several visits to prevent excessive blood loss. In addition, chlorhexidine gluconate mouthwash can be used to control periodontal problems. Antibiotics may be required to help reduce the initial inflammation. Blood loss of all kinds can be controlled locally with direct pressure or periodontal dressings with or without topical antifibrinolytic agents. 

Restorative procedures 

Restorative treatment can be undertaken routinely providing care is taken to protect the mucosa. There is a risk of bleeding with the use of matrix bands or wooden wedges. This can be controlled by local means or the application of topical agents.

Endodontics

In all but severe hemophiliacs endodontic treatment (taking into consideration the question of LA discussed previously) can be usually carried out under antifibrinolytic cover (usually tranexamic acid). Avoiding instrumentation through the periapex is of prime importance in endodontic therapy. The use of electronic endometric instruments will reduce the number of intraoperative radiographs. Rubber dam usage is mandatory as expected. Intracanal injection of LA solution containing adrenaline or topical application (using paper points) of adrenaline 1:1000 may be useful to minimize bleeding.

Orthodontic treatment 

Fixed and removable orthodontic appliances may be used along with regular preventive advice and hygiene therapy. Special care should be taken when treating patients with a severe bleeding disorder to ensure that the gingiva is not damaged when fitting the appliance.

Removable prosthodontics 

Patients with bleeding disorders can be given dentures as long as they are comfortable. If a partial denture is provided it is important that the periodontal health of the remaining teeth is maintained.

Anesthesia and pain management

Dental pain can usually be controlled with a minor analgesic such as paracetamol .Aspirin should not be used due to its inhibitory affect on platelet aggregation. The use of any non-steroidal antiinflammatory drug (NSAID) must be discussed beforehand with the patient's hematologist because of their effect on platelet aggregation. 

There are no restrictions regarding the type of local anesthetic agent used although those with vasoconstrictors may provide additional local hemostasis. It is important to advise patients and parents of children about the risks of local oral trauma before the anesthetic wears off. 

Infiltration injections pose no risk to patients with hemophilia. However, they should be administered slowly. Inferior Dental Blocks (ID Blocks) may potentially cause a muscle bleed which might compromise the airway. These should be avoided where possible. Alternative techniques such as buccal infiltrations with articaine or intra-ligamentory routes should be considered for anaesthesia of molar teeth. If an ID Block is unavoidable, patients with less than 30% factor levels will require factor replacement prior to its administration.

Surgery 

Extractions and surgery, gingival surgery/deep root planing and implant placement all pose a bleeding risk, even in patients with mild hemophilia so it is essential that these procedures are carried out with the necessary pre-operative special measures such as factor replacement, DDAVP or Cyclokapron tablets. If such procedures are required, please refer your patient with a dental treatment plan and suitable radiograph to your hemophilia treatment centre, and the on-site dental team will organise and/or carry out the treatment if required.

Dental emergencies 

Dental emergencies can occur at any time; however, it is important to remember that no treatment should be carried out without prior planning as this could result in additional problems.