hand book for dental surgeons - Indian Dental Association ...

DENTAL MANAGEMENT OF PERSONS 

WITH HEMOPHILIA AND OTHER 

HAEMOSTATIC DISORDERS 

HAND BOOK FOR DENTAL SURGEONS 

PUBLISHED BY : INDIAN DENTAL ASSOCIATION, KUNNAMKULAM BRANCH

DENTAL MANAGEMENT OF PERSONS 

WITH HEMOPHILIA AND OTHER 


HAND BOOK FOR DENTAL SURGEONS 

PUBLISHED BY:

INDIAN DENTAL ASSOCIATION, KERALA STATE PRESIDENTíS MESSAGE 

Bleeding disorders have been of much concern to health professionals including 

Dentists for quiet some time. Hemophilia patients are special patients from a 

dental point of view, as the highly vascular oral cavity is definitely a hotspot for 

hemorrhage in this group of patients. It is not infrequently that a dentist become 

the first person to diagnose a bleeding disorder while performing routine dental 

treatments. Thorough knowledge of systematic approach is mandatory for any 

dental health professional raring to handle hemophiliacs the absence of which can lead to 

disastrous outcomes. 

I am very glad to note that IDA Kunnamkulam Branch is bringing out a hand 

book to educate the IDA members about the Dental Management of Hemophilia patients. I am 

sure this hand book would be of great help to our IDA Members to learn more about the 

necessary precautions to be carried out while treating hemophilia patients in our day to day 

practice. I congratulate IDA Kunnamkulam branch especially the Editor, Dr. Joji George for the 

sincere efforts in bringing out this handbook and releasing it during the state level observation of 

world Hemophilia Day on 17 th April 2011. I hope and wish our fellow members will find this 

book useful in their practice. 

Dr. Santhosh Sreedhar 

President ñ IDA Kerala State

IDA KERALA STATE SECRETARYíS MESSAGE 

Its indeed heartening to know that IDA Kunnamkulam is publishing a hand 

book to dental surgeons about hemophilia and dentistry .Its a fact that among the 

estimated hemophilic patients of around 4,00000, many thousands of affected are 

enduring life without proper access to treatment and care. Itís time to express our 

courage and perseverance ,accelerate our pace of progress and remove the 

remaining barriers for adequate care. I am sure this handbook will provide a basis 

for the development of local protocols for the dental treatment of patients with inherited bleeding 

disorders. 

Dr.Shibu Rajagopal 

Hon. Secretary 

IDA Kerala State

IDA KERALA STATE CDE CHAIRMANíS MESSAGE 

The arrival of a hemophiliac patient in the waiting room often sends 

shudders down the spine of the dentist. The lack of in depth knowledge 

often hinders the management of such patients.Routine dental procedures 

do not generally involve bleeding; consequently, there is no 

contraindication to routine dental treatment for hemophiliacs. Preventive 

dentistry is vital to the younger hemophiliac patient world-wide; older 

hemophiliacs may require extensive treatment to restore mouths that have been neglected. 

Surgical dental procedures may be performed for hemophiliacs, but they must be judiciously 

coordinated by the dental and medical team. The facilities and treatment available will determine 

the feasibility of treating such patients in the hospital or on an outpatient basis. It is my earnest 

wish to be able to impart useful knowledge so as to arm the dentist with know-how to manage 

the hemophilac patient. 

I hope this handbook will serve this purpose and wish to thank all those individuals who have 

worked to make this a reality. 

with regards 

Deebu J Mathew 

CDE Chairman

Dear friends, 

IDA KERALA STATE CDH CHAIRMANíS MESSAGE 

Warm greetings to you ! Indian Dental Association Kerala State branch is 

stepping ahead once again,by marking itís role in building up a healthy 

Nation ! By the 

introduction of ASHAKIRAN ( the pain & palliative care project) last year , 

we have 

given a new face to our activities. Today ,on this 17th of April ,when we observe the 

World Hemophilia Day by conducting a Hemophilia workshop, we are happy to release 

a Hand book for Dental Surgeons on management of persons with Hemophilia. This 

Hand book will be an asset to all Dental Surgeons. At the same time, let me thank all 

the members of Indian Dental Association for giving their whole hearted support in the 

new steps of the Council on Dental Health,of IDA Kerala State. I congratulate the IDA 

Kunnamkulam branch for their untiring efforts to bring out this Hand book. 

With wram regards & prayers 

Dr Abdul Latheef K.H 

Chairman 

Council on Dental Health 

IDA Kerala State

IDA KERALA STATE EDITORíS MESSAGE 

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. The disease is a lifelong bleeding disorder, 

incurable in nature and a very expensive to manage in terms of medication 

and care. Recurrent and prolonged bleeding into joints and muscles can lead 

to permanent disability. Bleeding from the sensitive organs can lead even to death. IDA through 

its CDH activity can play a major role in helping hemophila patitents. 

As apart from providing factor and other medical care such as physiotherapy for 

affected persons, IDA can reach out across the community, educating people 

about hemophilia and attempting to de-stigmatize the condition. 

Our aim to reach out to community and provide total quality care, education, 

make treatment available at affordable cost, psycho-social support, and economic 

rehabilitation and thus help them in improving the quality of life without disability 

and free of pain. 

There are miles to go before fulfilling the vision of IDA 

ìHemophilia without Disability, Free of Pain.î

IDA KUNNAMKULAM BRANCH PRESIDENTíS MESSAGE 

Indian dental association Kunnamkulam branch is extremely happy and 

proud to conduct the Kerala State combined CDH ñ CDE Hemophilia 

work shop on April 17 th at Kunnamkulam IMA hall, world hemophilia 

day. I would like to congratulate and thank the state office bearers, 

energetic CDH chairman of State Dr. Abdul Lateef, Co-ordinator Dr. 

Sushanth (CC member) and the editor of the hand book Dr. Jogi George (state executive 

member) for their great work. 

I hope, this hand book for dental Surgeons on Dental Management of the Hemophilia and other 

hemostatic disorders would be a treasure to all of us. 

Dr. Geo. Joy. K, President, IDA Kunnamkulam Branch.

EDITORIAL 

Dental Surgeons face bleeding problems in their day to day clinical 

practice. Among them, Hemophilia and other haemostatic disorders 

need special attention and management Long back, Hemophilia was 

considered to be the Royal disease. But today, the Scenario has 

changed. The etiology can be mutation and even drugs. Other 

haemostatic disorder like von wille brands disease, thrombocytopenia 

etc., are becoming common. 

So I hope, this Handbook for dental Surgeons on Dental Management of the Hemophilia and 

other Haemostatic disorders would be useful to all dental surgeons 

I thank all contributors to this venture. 

Dr. Joji George.

1 

Visit: http://image.idakunnamkulam.com/ 

Hand book for dental surgeons on dental management of persons with hemophilia and other haemostatic disorders 

1. World health organization. 

2. World hemophilia federation. 

3. Hemophilia Society of India. 

4. Hemophilia Society Kunnamkulam Chapter. 

5. Indian dental association Kerala state. 

ACKNOWLEDGEMENTS: 

6. Dr.Ashok Varma, M.S., General surgeon, Unity Hospital, Kunnamkulam. 

7. Dr.Varghese Mani, M.D.S., Oral and Maxillofacial surgeon. 

8. Dr.R.Mahesh Kumar, M.D.S., Oral and Maxillofacial surgeon. 

9. Dr.K.Nandakumar, M.D.S., Editor, Kerala Dental Journal. 

10. Dr.S.Balu Mohan , M.D.S., Oral Medicine. 

Visit : http://image.idakunnamkulam.com/ 

Hand book for dental surgeons on dental management of persons with hemophilia and other haemostatic disorders

2 



CONTENTS 

1. HAEMOSTATIC DISORDERS P 3-4 

2. HAEMATOLOGY AND HAEMOSTATIC DISORDERS P 5-6 

3. CLOTTING MECHANISM OF BLOOD P 7 

4. HEMOPHILIA P 8-10 

5. GENERAL MEDICAL TREATEMENT OF HEMOPHILIA P 11-12 

6. DENTAL MANAGEMENT IN PERSONS WITH HEMOPHILIA AND OTHER HAEMOSTATIC 

DISORDERS - AN OVER VIEW 

P 13-23 

7. HEMOPHILIA AND CHILDREN (PEDODONTICS) PG 24 -31 

8. DENTAL TREATMENT MODALITIES FOR HEMOPHILIC PATIENTS ñ SOME TIPS PG 

32-36 

9. GENERAL INSTRUCTIONS TO PERSONS WITH OTHER BLEEDING & CLOTTING DISORDERS PG 37- 

39 

10. COMMUNITY DENTISTRY AND HEMOPHILIA PG 40- 

42 

11. REFERENCE CENTERS AND REFERENCES PG 43-45 

12. GLOSSARY PG 46-50 



3 




Bleeding disorders are conditions that after the ability of blood vessels, platelets and coagulation factors to 

maintain haemostasis inherited bleeding disorders are genetically transmitted. Acquired bleeding disorders 

occur secondary to diseases affecting vascular wall integrity, platelets of coagulation factors, drugs, radiation or 

chemotherapy for cancer. Hemorrhage, intravascular thrombosis and embolism are common clinical 

manifestations of abnormal interactions between components of the vessel, circulating platelets and plasma 

proteins. However, when disease or trauma damage large arteries and veins, excessive bleeding may occur 

despite a normal haemostatic system. 

Primary haemostasis: Stops blood loss from capillaries, small arterioles and venules. It occurs within 

seconds. During this period, platelet plug is formed at the site of injury. Three critical events occur during this 

phase platelet adhesion, granule release and platelet aggregation. 

Secondary haemostasis: Completed in several minutes and is important in bleeding from larger vessels. 

During this period fibrin is formed it is the activation of clotting process in plasma. Which ultimately results in 

the formation of fibrin. Which strengthens primary haemostatic plug. It is a continuous process and there are 

approximately 40 substances, which affect clotting. Some promote clotting (procoagulants) and some inhibit 

clotting (anticoagulants). Normally there is a fine balance between these factors and blood usually does not 

coagulate in the vessel. Whenever there is an injury to the vessel these procoagulants are activated and balance 

tilts infavour of coagulation and there is formation of clot. 



4 



Normal haemostatic mechanism is disturbed when any defect occurs in the mechanism of primary haemostasis 

for secondary haemostasis and it results in bleeding disorders. On this basis, bleeding disorders can be broadly 

classified as due to primary haemostatic disorder or due to secondary haemostatic disorder or due to secondary 

haemostatic disorder. Primary heamostatic (Platelet) disorders are further subdivided in two types non 

thrombocytopenic purpura and thromobocytopenic purpura. 

Non thrombocytopenic purpura is further subdivided into defects of platelet adhesion (Von Willebrandís 

disease, Bernad soulier syndrome), defects of platelet aggregation (Glanzmannís Thrombosthenia), defects of 

platelet release (drug induced granule storage pool defect, uremia, platelet coating by pencillin or paraproteins) 

and defects of platelet coagulant activity (Scottís syndrome). 

Secondary haemostatic (coagulation) disorders are further subdivided into two types inherited 

(hemophilia A, hemophilia B, parahemophilia, etc. ) and acquired (liver disease, renal failure, DIC, anti- 

coagulation drugs, vitamin deficiency) 

The best method of determining whether the defect is in primary haemostasis or secondary haemostasis 

is by various laboratory investigations. Most important screening tests of primary haemostatic system are 

platelet count and bleeding time. 



5 



HAEMATOLOGY AND HAEMOSTATIC DISORDERS 

Normal platelet count is 150000-450000 platelets per microlitre of blood. Symptoms do not arise and 

bleeding time remains normal until it is greater than 100000/micro litre. Mild prolongation bleeding time 

occurs when it is between 50000-100000/micro litre. Easy bruising manifested by skin purpura occurs after 

minor trauma and bleeding after mucous membrane surgery occurs when it is less than 50000/micro litre 

Spontaneous bleeding occurs when its value is less than 20000/micro litre. When qualitative platelet 

abnormality is present then there is normal platelet count and prolonged bleeding time. Bleeding time over 10 

minutes has increased risk of bleeding. Extensive bleeding occurs when bleeding time is greater than 15-20min. 

Due to cyclical variation level of V Wb factor occasionally bleeding time may be normal so repeated testing 

may be necessary to establish diagnosis. 

Plasma coagulation function is readily assessed by partial thromboplastin time (PTT). Prothrombin time 

(PT), thrombin time, clot solubility test and clot lyses test. 

When there is prolonged partial thromboplastin time ( normal is 25-35 seconds) and no clinical bleeding, 

then one could suspect deficiency of factor 12, HMWK, PK. When it is associated, with mild or rare bleeding 

then factor 11 deficiency might be present and when there is frequent severe bleeding then deficiency of factor 

8 & 9 should be suspected. Prolonged prothrombin time (normal is 10-15 sec) indicates deficiency of factor 7, 

vitamin k (early) or warfarin ingestion. When PT & PTT both are prolonged, it indicates deficiency of factor 2, 

5 & 10, vitamin k (late) or warfarin ingestion. 



6 



Among the various disorders of secondary hemostatisis most well known and common is haemophilia 

A. It has an incidence of 1 or 2/100000 of the population and it is caused by a deficiency of antihemophilic 

globulin (AHG or Factor 8) in the plasma. It is inherited as a sex-linked recessive character, appearing only in 

males and transmitted to them by clinically normal female carriers. The defective gene on the X chromosome 

causes a deficiency of Factor 8, Normal plasma contains one unit of a factor 8 per ml. or 100% level. 

Deficiency of factor is expressed as percentage (0.25unit/ml=25%). Patients with less than 1% AHG have 

severe disease. They bleed frequently into soft tissues, muscles and weight bearing joints even without 

discernible trauma. When level is between 1-5%, it indicates moderate disease and there are less severe 

bleeding episodes. Levels above 5% have mild disease in which there is infrequent bleeding usually secondary 

to trauma or surgery levels above 25% cause no troubles unless patient suffers from major trauma. Majority of 

patients have level of factor 8 less than 5%. 

The clotting time is not prolonged until the level of AHG falls below 1%. The only safe test is to 

estimate the AHG level in the patient for diagnosing the presence of this disease. 



7 



CLOTTING MECHANISM OF BLOOD 



8 



Introduction 

HEMOPHILIA 

Hemophilia is a hereditary disorder of coagulation results in deficiency of factor VIII (Hemophilia A) or 

factor IX (haemophilia B). The disease is almost exclusively see in males while females are asymptomatic 

carries. Rarely it can affect females. It can also be caused by mutation. It is characterized by spontaneous or 

trauma related bleeding typically into the large joints or muscles. Untreated or inadequately treated episodes 

lead to joint, muscles or nerve damage. Life threatening haemorrhages can result spontaneously or from trauma 

to the head or internal organs. 

Without proper care, hemophilia can lead to serious damage to limb and joint function within the first 

one to two decades of life. This is due to impaired joint mobility, contractures, muscle atrophy and chronic pan. 

Crippling joint deformities can be effectively prevented by adequate care of each episode of bleeding, including 

replacement of the appropriate coagulation factor lacked by the patient and every deliberated and persistent 

physical therapy. Certain serious complications can further complicate the management of hemophilia. 

Between 10-20% of people with hemophilia A and 23% of those with hemophilia B develop inhibitors against 

the deficient factor. Such patients do not respond to usual factor replacement therapy. Patients exposed to 

factor replacement with products that have not been virus inactivate are prone to acquiring transfusion 

transmitted viral infections such as human immuno-deficiency virus (HIV), hepatitis B and C viruses. The care 

of people with hemophilia therefore often requires a multidisciplinary team to address different aspects of the 

patientís problems. This is the concept of comprehensive care that is recommended by the World Health 

Organization (WHO) and the World Federation of Hemophilia (WFH). 



9 



EPIDEMIOLOGY 

The prevalence of hemophilia is estimated to be about 1 in 10,000 births and that of the severe form of 

the disease to be about 6 per 1,00,000 population. It is therefore likely that there are over 50,000 people with 

severe hemophilia in India. Only about 10% of them have identified. There is obviously a need to establish 

facilities that will help detect many more of the affected people. 

The manifestations of hemophilia depend upon the severity of the disease based on factor levels:- 

Severe Factor VIII or IX level is

10 



Genetics Of Hemophilia-Carrier Detection And Antental Diagnosis 

The gene for factors VIII and IX are both located on the x-chromosome. Hemophilia is 

therefore said to be an x-linked hereditary disorder. This results in males being affected by the disease 

while females are carriers. While affected males with no functioning factor VII or IX gene have very 

low to absent factors in the blood, carrier females have at least one (out of two) functioning X- 

chromosomes with a normal factor VIII or IX gene that provides about 50% factor levels. Carrier 

females who have excessive lyonization of the normal x-chromosome can have very low F VIII levels 

and symptoms of bleeding. 

Identification Of Carriers 

A woman is a definite carrier if: 

Her father has hemophilia 

She has one son with hemophilia and a first degree relative (brother, uncle or other male 

relative) who are affected. 

She has two or more sons with hemophilia. 

A Women Is A Possible Carrier If: 

She has one or more maternal relatives with hemophilia 

She has one son with hemophilia and no other affected relatives. 



11 



GENERAL MEDICAL TREATEMENT OF HEMOPHILIA 

Hemophilia is treated by replacing the missing clotting factor in the blood. This is done by injecting a 

product that contains the needed factor into a vein. Bleeding stops when enough clotting factor reaches the 

bleeding site. It is very important that treatment is given as quickly as possible to prevent long-term damage. 

With an adequate quantity of treatment products and proper care, people with hemophilia can live 

perfectly healthy lives. 

Clotting factors are found in the following blood products in order of decreasing concentration: 

* factor concentrates 

* cryoprecipitate 

* plasma 

* whole blood 

Factor concentrates are the treatment of choice for hemophilia. They can be made from human blood 

(called plasma-derived products) or manufactured using genetically engineered cells that carry a human factor 

gene (called recombinant products). Factor concentrates are made in sophisticated manufacturing facilities. All 

commercially prepared factor concentrates are treated to remove or inactivate blood-borne viruses. 

People with mild hemophilia A sometimes use desmopressin (also called DDAVP), a synthetic hormone 

that stimulates the release of factor VIII. 

Cryoprecipitate is derived from blood and contains a moderately high concentration of 

factor VIII (but not IX) clotting factor. It is effective for joint and muscle bleeds, but it is less safe from viral 

contamination than concentrates and is harder to store and administer. Cryoprecipitate can be made at local 

blood collection facilities. 



12 



In fresh plasma, the red cells have been removed, leaving the blood proteins including the clotting 

factors. It is less effective than cryoprecipitate for the treatment of hemophilia A because the factor VIII is less 

concentrated. Large volumes of plasma must be transfused. This can cause circulatory overload. 

The life of the clotting factors is preserved by making a product called fresh frozen plasma (FFP). FFP 

is still the only product available for treatment of hemophilia A and B in some countries. 

There are considerable drawbacks to the use of whole blood in the treatment of hemophilia. First, it must 

be fresh because the activity of the clotting factors in the drawn blood decreases quickly with time. second, the 

red cells it contains must, of course, be compatible with those of the recipient. third, it takes a large volume of 

whole blood to stop a bleed and this volume may overload the circulation and cause the heart to fail. 

Antifibrinolytic medicines (including tranexamic acid and aminocaproic acid) may be used with 

replacement therapy. Theyíre usually given as a pill, and they help keep blood clots from breaking down. 

These medicines most often are used before dental work or to treat bleeding from the mouth or nose or mild 

intestinal bleeding. 

Researchers are trying to find ways to correct the faulty genes that cause hemophilia. Such gene therapy 

hasnít yet developed to the point that itís an accepted treatment. But researchers continue to test gene therapies 

for hemophilia in clinical trials. 



13 



DENTAL MANAGEMENT IN PERSONS WITH HEMOPHILIA 

AND OTHER HAEMOSTATIC DISORDERS - AN OVER VIEW 

Numerous procedures in dental practice may cause bleeding. Uncontrolled bleeding after minor surgical 

procedures like tooth extraction being one of the main complications encountered in clinical practice in normal 

circumstances, dental procedures can be performed with little or no risk to the patient. However, a patient, 

whose ability to control bleeding is altered by drug or disease is in grave danger unless the dentist identifies the 

problem before performing any surgery. Therefore, it is very important that proper medical and family history 

of the patient is collected before commencing any surgical procedure, and routine blood investigations should 

be carried to identify the bleeding disorder to reduce the risks associated with dental procedures. 

The dental treatment of patients with inherited bleeding disorders has been widely discussed in the literature 

with the aim of developing guidelines for common procedures. the majority of guidelines recommend the use of 

clotting factor replacement therapy before invasive oral surgery and the use of the inferior alveolar nerve block 

for restorative dental treatment. the dose of clotting factor used varies and this may be due to problems relating 

to both the availability and cost of factor concentrate in different parts of the world. 



14 



These protocols suggest the use of factor concentrate along with the use of local hemostatic techniques, such as 

suturing, and local measures, such as the use of oxidized cellulose, for example Surgical or fibrin glue in 

conjuction with post-operatively administered anti fibrinolytic agents where appropriate. The use of local 

techniques has resulted in certain minor oral surgery procedures being done with minimal or no coagulation 

factor replacement. 

Sindet-Pedersen suggests that the dose of factor replacement therapy can be significantly reduced if used 

with an oral rise of an antifibrinolytic agent(tranexamic acid) is used following a dental extraction. 

Desmopressin, a synthetic derivative of the hormone vasopressin, he has been shown to increase factor VIII 

level in some patients with mild or moderate forms of hemophilia A or type 1 von Villebrand disease. 

Unfortunately, not all patients respond to this should be checked before performing any surgical procedure. Its 

use is well documented in cases of mild and moderate hemophilia. DDAVP releases bound factor VIII and is 

therefore not used to treat patients with hemophilia B. Fibrin glue is used as a local hemostatic measure in some 

centres for achieving hemostasis and reducing the needs for clotting factor replacement therapy. 



15 



Dental infections 

ORAL MEDICINE 

Many patients with infections of dental origin are managed without the use of antibiotics but instead by dental 

extraction or endodontic treatment. Antibiotics are often used to treat an acute bacterial infection.This should be 

considered for all patients with inherited bleeding disorders since surgical intervention should be avoided if 

possible. The initial treatment usually starts based on the normal oral pathogens, Streptococcus viridans, 

anaerobic gram-negative rods. Antibiotic regimes should cover all of these groups of organisms. 

Penicillin is a first line antibiotic used to treat dental infections.It can be taken orally in the form of pencillin 

V.Metronidazole is extremely effective in treating anaerobes and is often used in combination with pencillin to 

give good coverage of both the aerobic and anaerobic bacteria present in the oral cavity. The does of the drugs 

may vary depending on the availiblity in different countries but the treatment should continue for 5-7days. A 

number of different formulations of penicillin are available with a broader spectrum of activity. These can be 

used either alone or in conjunction with metronidazole. However, it is important to remember that if these drugs 

are ineffective treatment of the infection will become more complicated. Erythromycin and clindamycin have 

been prescribed to patients who are allergic to penicillin. These drugs can be used in conjuction with 

metronidazole. Erythromycin may be effective in people with penicillin allergies, but it may not be suitable for 

more severe infections. Clindamycin produces high alveolar concentrations, and bactericidal activity is reached 

with the usual recommended oral dose of 150mg every 6 hous. In more severe cases it can be given 

intravenously. It has been reported that clindamycin can cause antibiotic-associated colitis and therefore is 

often reserved for the treatment of the more serious infections or when penicillin has failed. 



16 



Periodontal pockets host a veriety of different bacteria, the majority of them being anaerobic. Regular oral 

hygiene prevents these bacteria from causing gingival inflammation. In patients with severe gingival 

inflammation, in particular those who are immune compromised; the use of an antimicrobal agent may be 

indicated Metronidazole is considered the drug of choice due to its action against anaerobic organisms. It may 

be used in conjunction with either penicillin or erythromycin. However, antimicrobial therapy is no substitute 

for oral hygiene treatment. 

Topical treatment 

The most common forms of topical treatment involve the use of an antibacterial mouthwash. These have 

value as an adjunct to hygiene phase treatment. The most common treatments are: 

* Chlorhexidine gluconate: Chlorhexidine is available as a mouthwash, spray, and toothgel. It is most 

commonly used as a twice a day mouthwash with the patient rinsing their mouth with the solution for 

30-60 seconds. The toothgel may be used in addition to the mouthwash. Chlorhexidine has a tendency 

to stain teeth so the length of each course of treatment should be limited. 

* Providine-iodine: providine-iodine is available as a mouthwash and can be used for the treatment of 

acute periodontal problems. In addition,it may be useful to irrigate the periodontal pockets. It must be 

used with caution during pregnancy. 

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. The most common 

dental problems are pain due to caries and bleeding from the periodontal tissues. Pain related to caries can 

usually be trated with either antibiotics or pulpectomy in order to allow time for the planning of the extraction. 

Bleeding from the periodontal tissues can usually be controlled with antibiotics until an appointment with a 

hygienist can be arranged. 



17 



The management of dental trauma is more complex as it usually involves both the gingiva and the teeth. 

Local measures will usually control gingival bleeding and temporary splinting can be used for fratured or loose 

teeth. With dental trauma, it is important to remember that we are part of the comprehensive hemophilia care 

team treating these patients. Treatment planning for an emergency requires input from the whole team to reduce 

the risk of further problems. 

ORAL AND MAXILLOFACIAL SURGICAL MANAGEMENT 

However where extractions, deep scaling, and/or inferior dental nerve (lower jaw) injections deep into the tissue 

are anticipated, then a 50% factor rise would be the legal of choice. Where the procedures intended are much 

more extensive, or become more extensive as the procedure develops, such as a surgical extraction, 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 500mg. one tablet four times a day to be taken for 10days 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 

be taken for 3 to 7 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. 



18 



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 factor replacement treatment 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 expensive. 

In relation to any oral surgical intervention, it is of paramount importance that there is the least possible 

interference with the attached gingival (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 ìkey holeî surgery, where there is as little interference as possible with the attached gum to minimize 

post-operative bleeding. 

Start Tranexamic Acid the night before the procedure: 250mg for a child and 500mg for an adult for times daily. 

Continue for 5-7 days after the procedure. 

If there is an infection start an antibiotic the day before the procedure. 

Bacampicillin 400mg (Trade name - Penglobe) twice daily for dults and 200mg twice daily for children. 

Or Metrondazole 400mg (Trade name - flagyl) thrice daily for adults and 2000mg thrice daily for children. 

One dose of factor concentrates 10units/kg to be administered before a major procedure and repeated if 

necessary. 



19 



Anaesthesia and pain management. 

Dental pain can usually be controlled with a minor analgesic such as paracetamol (acetaminophen). 

Aspirin should not be used due to its inhibitory affect on platelet aggregation. The use of any non-steroidal anti- 

inflationary drug (NSAID) must be discussed beforehand with the patientís 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. 

A buccal infiltration can be used without any factor replacement. It will anesthetize all the upper teeth 

and lower anterior and premolar teeth. 

The mandibular molar teeth are usually treated using the inferior alveolar nerve block. This should only 

be given after raising clotting factor levels by appropriate replacement therapy, as there is a risk of bleeding into 

the muscles along with potential airway compromise due to a hematoma in the retromolar or pterygoid 

technique should be considered instead of the mandibular block. Articaine has been used as a buccal infiltration 

to anesthetize the lower molar teeth. A lingual infiltration also requires appropriate factor replacement since the 

injection is into an area with a rich plexus of blood vessels and the needle is not adjacent to bone. There is a risk 

of a significant airway obstruction in the event of bleed. 



20 



Surgery 

Surgical treatment, including a simple dental extraction, must be planned to minimize the risk of 

bleeding, excessive bruising, or hematoma formation. The following points will help prevent problems. 

Emergency surgical intervention in dentistry is rarely required as pain can often be con trolled without 

resorting to an unplanned treatment. All treatment plans must be discussed with the hemophilia unit if they 

involve the use of prophylactic cover. 

1. Treatment plan 

The treatment plan should be formulated using the following guidelines. 

* Conduct a thorough clinical and radiographic examination. 

* Identify which treatment may require prophylactic cover. If multiple extractions are required, only one 

or two teeth should be extracted at the first appointment to ensure that hemostsasis can be achieved. It is 

important to remember the patientís social circumstances (Do they live alone? What are their 

preferences for treatment) as well as the clinical condition when making this decision. 

* Observe all patients for a prolonged period after a dental extration. this may be for a few hours for those 

patients with a mild bledding tendency whilst those with more severe conditions or a history of 

prolonged bleeding despite hemostatic cover may require supervision overnight in hospital. 

* Discuss treatment requiring the administration of coagulation factor or desmopressin (DDAVP) with the 

hemophilia unit. They will be responsible for arranging the administration and monitoring of treatment 

products. 

* Discuss the use of local hemostatic agents. This could include the use of oxidized cellulose(Surgicel) or 

fibrin glue. Fibrin glue should not normally be used in patients who have never received human -derived 

blood products or those who are receiving treatment with recombinant factor VIII of IX because of the 

potential risks of human viral transmission. 



21 



* Consider whether to use antibiotics following a dental extraction. This is contraverseal, but there are a 

number of anecdotal reports suggesting that their use may prevent a late bleed, which is thought to be 

due to infection before treatment , it should be treated with antibiotics. 

* Always carry out treatment as atraumatically as possible. 

2. Pre-operative period 

* Ensure that the oral cavity is as healthy as possible before any surgical procedure. This can be achieved 

by arranging treatment with hygienist to remove as much calculus and plaque as possible. The regular 

use of an antibacterial mouthwash, for example chlorhexidine, may also help. 

* Consider using an antifibrinoytic agent. It may be helpful to start the treatment the day before the 

surgery. Tranexamic acid(usual adult dose 1g three times a day) and epsilon aminocaproic 

acid(EACA)(50 mg/kg four times a day), are the most commonly used drugs. They should be continued 

for a total of 7 days. 

3. Peri-operative period 

* Have the patient rinse with chlorexidine mouthwash for 2 minutes before the administration of the local 

anesthetic. 

* Carry out the extraction out as atraumatically as possible., 

* Suture the socket if the gingival margins do not oppose well. Brewer reports a small series where sutures 

were not used routinely and there was no significant increase in post extraction hemorrhage Resorbable 

and non-resorbable sutures may be used at the operators discretion. The only problem with non- 

resobable sutures is the need for a post operative visit and the possibility of bleeding when the 

suture is removed. Use local hemostatic measures if indicated. These include the use of oxidized 

cellulose or fibrin glue(see notes on the use of fibrin glue) Use a soft vaccum formed splint to protect 

the socket if needed. 



22 



4. Post -operative Period 

The patient must be given detailed postoperative instructions: 

No mouth rising for 24 hours 

No smoking for 24 hours 

Soft diet for 24 hours 

No strenuous activities for 24 hours 

Prescribed medication must be taken as instructed 

Analgesia should be prescribed for use if required. 

Salt water mouth washes (1 teaspoon of salt in as glass of warm water) should be used four times a day starting 

the day after the extraction for 7 days 

Antibacterial mouthwash may be used; 

Emergency contact details must be be given to the patient in case of problems. 

Post-extraction hemorrhage 

Careful pre-operative planning and the use of antifibrinolytic agents will prevent many post operative problems. 

However post extraction bleeding will occur on occasion. If post extraction hemorrhage occurs. 

Contract the hemophilia unit and consider using additional factor concentrate. Inspect the site of the bleed. If 

there is any evidence of a tear in the gingiva or other obvious bleeding point this should be treated using local 

measures as previously described. 

Instruct the patient top sit up and bite on a damp gauze swab for at least 10 minutes. 

Use a 10% solution of tranexamic acid or EACA to dampen the swab or as a mouthwash if the bleeding is 

difficult to stop. 



23 



Monitor the patientís blood pressure as it may increase due to worry and pain. If the patient has pain, a 

suitable analgestic should be prescribed whilst if there is no pain a small dose of a benzodiazepine or similar 

will help to reduce the worry and reduce the blood pressure. 

Fibrin glue 

In some hemophilia centres, fibrin glue is used as a local hemostatic measure, along with an oral 

antifibrinolytic agent, to achieve hemostasis and reduce the need for clotting factor replacement therapy. All 

fibrin glue contains human or animal components, which has made a number of physicians and patients being 

hesitant to use this treatment articularly for patients who are receiving recombinant factor concentrates or have 

never received blood products derived from humans. Fibrin glue mimics the final pathway of coagulation 

cascade at the point where fibrinogen is converted into fibrin in the presence of thrombin, factor XIII, 

fibronection, and ionized calcium. the cascade reaction provides the cleavage of fibrinogen through thrombin, 

forming fibrin peptides A and B from each molecule of fibrinogen resulting in the formation of the fibrin 

monomers. thrombin itself also activates factor XIII, which in the presence of calcium, permits the stabilization 

of the clot. fibronectin takes part in the process as well and its inclusion in the adhesive system appears to 

promote cellular migration and then activation of fibroblasts in the area where the fibrin glue was applied. 

Splints 

Soft vacuum-formed splints can be used to provide local protection following a dental extraction or 

prolonged post-extraction bleed. the following techniques are used to construct the splint pre-operatively: 

* Take a dental impression before the extraction and cast a model in the laboratory. 

* Remove the tooth being extracted from the model. 

* Construct a soft vacuum-formed splint to cover the socket completely. 

* Keep the splint in place for at least 48 hours before checking the socket. If there is any sign of bleeding 

it should be replaced and checked every 24 hours. 



24 



HEMOPHILIA AND CHILDREN (PEDODONTICS) 

Several inborn abnormalities of metabolism, which manifest themselves as hemostasis disorders, have 

been given the collective name hemophilia. these disorders may result from lack of any one of the substances 

necessary for normal thromboplastic activity. 

The most common hemophilic condition is the disorder caused by dificiency of factor VIII 

(antihemophilic globulin). This condition is transmitted as an X-linked mendelian recessive and occurs 

exclusively in males. Although children born with factor VIII deficiency are potential ìbleedersî from birth, 

excessive bleeding may not be observed until increased activity begins, at about 6 months of age. Bleeding may 

occur from any site, but is most common in the muscles, kidneys, mouth, and joints. The knee joints of the 

infant who is beginning to walk are especially vulnerable. 

DENTAL PROBLEMS 

Although the hemophiliac has no characteristic dental problems, any dental treatment these children 

require must be considered as serious because of the grave risks involved. The necessity for good oral care and 

prevention of dental disease for the hemophiliac cannot be overemphasized, since preventive dentistry for these 

children minimizes the need for the hazards of restorative treatment. 

Dental Treatment 

Even during the most routine dental treatment for the hemophilic child the dentist must exercise extreme 

caution to prevent tissue lacerations. Scaling and polishing, reduction of subgingival tooth structure, and 

adaptation of matrix bands must be managed with great care to maintain the integrity of the periodontal tissues. 

Should minor bleeding problems occur, they can usually be controlled by pressure packs in conjunction with 

hemostatic agents such as thrombin. 



25 



The use of local anesthesia is contraindicated for these children except when pain is extreme and then it should 

be used with caution. The mandibular block should be avoided, because this form of injection may cause 

hemorrhage into the lateral pharyngel spaces, where it is difficult to apply controlling measures. 

A carefully placed rubber dam will serve a twofold purpose in the dental treatment of the hemophilic. 

Besides securing a dry field for the placement of restorative materials, it also will help protect the soft tissues 

from accidental lacerations. 

Tooth extraction for the hemophiliac should be considered only as a last resort, after the possibility of 

maintaining the teeth by conventions pulpectomy or root canal therapy has been rejected. 

Extreme precautionary measure should be practiced for all patients suffering from any type of 

hemorrhagic disorder, as for example with the so-called pseudo hemophiliac, who may be a male or a female 

with a characteristically prolonged bleeding time. 

Removing Teeth 

When extractions or other surgical procedures are unavoidable and excessive bleeding is anticipated, the 

patient should be hospitalized for more adequate handling of any complications that might arise. 

Prior to the patientís admission to the hospital, the dentist should organize a plan for dental treatment. 

This plan, in conjunction with the patientís medical history, should be discussed by the dentist, the physician, 

and the hospital staff to outline a medical work-up that will include a detailed blood analysis for determining the 

type of bleeding problem, the coagulation and bleeding time, and the platelet count. After these steps are 

completed, the hematologist should coordinate the medical management for the child. 



26 



Premedication of the hemophiliac is indicated when lengthy procedures are planned or when the patient is 

apprehensive or unruly. Preoperative plasma transfusion may be recommended if excessive bleeding is 

anticipated. 

As mentioned earlier, local anesthesia is usually contra indicated for a child with hemophilia. A general, 

anesthetic, administered by a trained anesthesiologist, who is cognizant of the bleeding problem, should be a 

relatively safe procedure for the hemophiliac patient. 

Before surgical treatment is attempted, all necessary restorative procedures should be completed with 

strict adherence to the precautionary measures already described. The extractions and other surgical treatment 

must be performed with the least possible trauma. Use of pressure packs for the initial control of hemorrhage 

should be followed by packing the socket with Gelofoam or some other hemostatic agent that can be held in 

nplace by gauze impregnated with petrolatum to prevent the incorporation of the gauze in the blood clot. 

A follow-up transfusion schedule should be planned by the hematologist and the dentist. The duration 

of this therapy depends on the approximation of the healing time for the surgical wounds. the patient should 

receive nothing by mouth for 4 hours after the operation and should receive only liquids for the subsequent 24 

hours. The important of then patientís diet and postoperative home care must be carefully explained to his 

parents. 

NITROUS OXIDE ANALGESIA 

With the handicapped child, inhalation analgesia with nitrous oxide can be a safe and effective method 

of decreasing apprehension or resistance to dental treatment. Except for children who have severe mental 

retardation or emotional disturbance, there are few contraindications to its use. 

A dentist experienced in administering nitrous oxide analgesia can combine this procedure with 

premedication and local anesthesia to overcome many of the problems associated with handicapped children. 

Nitrous oxide analgesia decreases the muscular spasticity and the uncoordinated movements of the cerebral 

palsied child and lessens the physical stress and discomfort, thereby making it possible for him to tolerate 

longer periods of treatment. 



27 



Nitrous oxide analgesia for the handicapped child should be limited to the stage of relative analgesia, by the use 

of relatively low nitrous oxide and high oxygen flows, safely below the excitement level. The major purpose of 

a relative analgesia level is torelance the patient and increase his cooperation. During the most painful periods 

of the dental treatment, this analgeis level can be supplemented by the use of local anesthesia when the patientís 

condition permits. 

Careful consideration and management of the introduction of nitrous oxide analgesia and its initial 

administration to the handicapped child are the governing factors for a successful utilization of the procedure. 

For most handicapped children, premedication is needed to allay the apprehension that frequently accompanies 

their first experience with analgesia. The attending physician should be consulted regarding the choice of durg 

therapy for each patient. 

Particular patience and understanding is imperative at the initial administration of 

nitrous oxide analgesia. The child must have time to adjust to this new experience, the use of the nosepiece 

should be demonstrated and the flow of gases can be played on the childís hands and cheeks before placement 

of the nosepiece. Resistence may be countered with gentle physical restraint and flow of 50 percent nitrous 

oxide directed close to the nostrils. This mixture may have a mild euphoric effect and relax the patient 

sufficiently to permit seating of the nosepiece, after which the concentration of nitrous oxide should be reduced 

to the appropriate level, usually a 10 to 15 percent flow. 

Since verbal communication with the handicapped child is frequently difficult and unreliable, the dentist 

must be able to evaluate the level of analgesia from the physical and behavioural changes in the patient. When 

the appropriate level of analgesia has been reached. The dentist can begin the treatment. Operative procedures 

in these cases vary little from those routinely employed. the rubber dam and a mouth prop are helpful; however, 

it is important to remember that the use of the rubber dam lessens the dilution effect created when the mouth is 

open, thereby necessitating a decrease in the proportion of nitrous oxide. 



28 



GENERAL ANAESTHESIA 

Risks of vomiting, spasm, and apnea invariably exist in association with the use of general anesthetic; 

therefore, it is expedient that milder, though possibly less potent, measures be considered when an anesthetic is 

in order. 

A childís response, especially with the aid of premedication, is usually cooperative when the dentist exercise 

patience and understanding. This is true even with children who may appear incapable of the mental and 

physical control needed for successful dental treatment. Should these patient management procedures fail, or if 

a severely handicapped child needs extensive dental care, the use of general anesthesia offers a possible solution 

for the problem. 

Indications for General Anesthesia 

Children in one of the following categories usually require general anesthesia: 

1. Then uncooperative child who resists treatment after all conventional management procedures 

have been tried. 

2. The child with a hemostasis disorder who needs extensive dental service. 

3. The mentally retarded child so severaly handicapped that dentist-patient communication is impossible. 

4. The child suffereing from central nervous disorders manifested by extreme involuntary movements. 

5. The child with severe congenital heart disease who is considered incapable of tolerating the excitement 

and fatigue of extensive dental service. 

Preoperative Procedures 

Although the utilization of a general anesthetic in the dental office is acceptable, provided the essential 

equipment and a qualified anesthesiologist are available, the safest place for dental treatment under general 

anesthesia is undoubtedly the hospital. 



29 



When a childís dental treatment requires hospitalization and a general anesthetic, the best way to insure 

cooperation from the parents is to orient them as to the programme planned for their child and their 

responsibilities concerning his hospitalization. 

Since considerable paper work is associated with any hospitalization, the dentist should familiarize 

himself with accepted hospital protocol before a definite admission date is arranged for his patient. 

Treatment Procedures 

After the patientís hospital admission, the dentist should discuss plans for the treatment with the 

anesthesiologist, who determines the tolerance limit of each patient for the general anesthetic, with this as a 

guideline, the dentist can then set up a rigid time schedule for treatment. 

After evaluating the patientís medical history and determining the extent of the required treatment, the 

most suitable premedication and anesthetic can then be chosen. Since it was first tried in pediatric anesthesia, 

some 15 years ago, gluothane (halothane) has gained wide acceptance because of its low irritant quality, no 

flammability, and the rapid awakening of a patient who has received it. 

Nasal endotracheal intubation, where the tube is out of his way, simplifies the treatment procedures for 

the dentist. After intubation, the patientís eyes should be covered with damp gauze for protection against dental 

and material debris. Great care must be taken to prevent blood or any type of debris from entering the patientís 

throat. After complete anesthetization a throat pack of moist gauze should be laid over the pharyngeal opening, 

across the tonsillar area, and under the tongue . For its easy, safe removal, the end of the throat pack, with a 

string securely attached to it, should extrude from the oral cavity. 



30 



Efficient use of aspiration equipment facilities dental procedures for the anesthetized child. A mouth prop may 

be used, when needed, to keep the childís mouth open. For any restorative treatment on the anesthetized patient, 

use of rubber dam provides the dentist with a dry field and better visibility, and serves as an adjunct to the throat 

pack in preventing debris from entering the patientís throat. Prior to extractions or other surgical treatment, all 

restorative procedures should be finished; with this done, the mouth should be cleaned, the throat pack replaced 

with fresh gauze, and the surgical procedures initiated. 

Upon completion of the planned treatment, any hemorrhage must be controlled and all debris carefully 

evacuated from the oral cavity; after this, the throat pack can be removed and the child taken to the recovery 

room. 

Before hospital dismissal, an appointment should be set up for a postoperative check on the patient 

within the following two weeks. On this visit, the parents must be impressed with the urgency for careful oral 

home care for their child and reducing his intake of cariogenic food as a means of avoiding the ordeal or 

repetitive dental treatment. 

Despite the fact that different blood components are involved en each of bleeding and clotting diseases, 

their clinical and oral manifestations are identical. They will therefore be described together as a single disease. 

Oral Manifestations. Hemorrhage from many sites in the oral cavity is a common finding in 

hemophilia, and gingival hemorrhage may be massive and prolonged. Even the physiologic process of tooth 

eruption and exfoliation may be attended with severe prolonged hemorrhage. 

The oral manifestations of the various forms of hemophilia have been discussed by Spiegal and by Steg 

and his co-workers. In addition, mandibular ìpseudsotumorî of hemophilia has been reported by Stoineman and 

Beierl, a condition in which there is subperiosteal bleeding, with reactive new bone formation causing tumor- 

like expansion of the bone. 



31 



The problem of dental extractions is a difficult one in hemophiliacs. Without proper premedication, even 

a minor surgical procedure may result in death from exsanguination. tooth extraction by means of rubber bands 

has often been used successfully, the rubber band being placed around the cervix of the tooth a nd allowed to 

migrate apically, causing exfoliation of the tooth through pressure necrosis of the periodontal ligament. 

Laboratory Findings. The characteristic defect of hemophilia is a prolonged coagulation time. 

the bleeding time is normal, as is the prothombin time and platelet agregation. In vitro, the deficiency of the 

clot-prompting factor in the plasma of hemophiliacs impairs clotting because it appears to retard development 

of the substance responsible for conversion of prothrombin to thrombin. Separation of the various forms of 

hemophilia and proper diagnosis depends upon demonstration that the plasma of a patient with a known form of 

hemophilia does not correct the plasma clotting defect in the patient under observation. 

Treatment and Prognosis. There is no known cure for hemophilia. the affected persons should be 

protected from traumatic injuries. 

If a surgical procedure such as tooth extraction must be carried out, the operation should be considered a 

major one and performed only in a hospital. 

The greatest numbers of fatalities in hemophiliacs have resulted from surgical procedures, including 

tooth extraction. Preoperative transfusion of whole blood and the administration of antihemophilic factor 

concentrate are recommended. Nevertheless, oral surgery is a dangerous procedure and should be avoided 

whenever possible. Unfotunately, a small percentage of hemophiliacs have circulating anti-coagulant, probably 

an antibody, which specifically inactivates hemophilic factor, negating the effects of transfusion. 

The prognosis is variable, and many affected persons die during childhood. 



32 



DENTAL TREATMENT MODALITIES FOR HEMOPHILIC PATIENTS ñ SOME TIPS 

DENTAL PAIN MANAGEMENT 

Dental Pain can be managed depending on the patientís pain threshold and the type of treatment to be 

undertaken. 

Option of treatment without anaesthesia may also be considered. 

Sedation & hypnosis are methods to control the anxiety; thus eliminating the need of anaesthesia in 

some kind of treatment. 

Intravenous diazepam or Nitrous oxide analgesia are best options. 

NSAIDs are to be avoided. 

ANAESTHESIA 

Intra pulpal anaesthesia is safe and effective, after accessing and extirpating pulp. 

Periodontal ligament and papillary injections with minimal volume and speed are also advised. 

Anaesthesia with vasoconstrictors should be used when possible 

Injections 

Infiltration for maxillary teeth with slow speed may be done 

Local pressure should be applied to the injection site for 3-4 minutes. 

If haematoma develops ice packs should be applied and emergency factor replacement therapy should 

be administered in a hospital. 

Block injections like inferior alveolar posterior Superior alveolar, infra orbital and even long buccal 

place the anaesthetic solutions to highly vascularised, loose connective tissues with no boundaries. 



33 



where formation of hematoma is a great problem. So it is advised to avoid block injections in these 

patients. 

Intra muscular injections should also be avoided because of chances of haematoma formation 

ORAL SURGERY 

Mandibular block injections are absolutely Contra indicated due to persistent haemorrhage into para 

pharyngal tissues. 

Local infiltration causes bleeding at each point 

Safe site of local infiltration is down the periodontal membrane. 

Haemorrhage plate may be given to protect the blood clot from trauma of food and tongue. 

Atraumatic extraction should be done 

Sides of Sockets should be gently squeezed. 

It is better to avoid Suturing because of needle wound as well as blood may be directed to the facial 

planes of neck. 

Antifibrinolytics like EACA (Epsilon amino Caproic acid) and tranexamic acid may be used for 

stabilization of clot. 

Tranexamic acid is more potent and longer acting (IV and oral 500mg tab dissolved in, oral of water) 

Avoid aspirin like drugs 

Paracetamol or dihydrocodeine may be effective. 

NSAIDs are to be avoided. 



34 



Prosthodontic treatment 

Generally excessive muco compressive method may be avoided to avoid unnecessary bleeding. 

Fixed prosthesis is preferred over removable prosthesis. 

Denture trauma caused by the denture must be minimized. 

Denture stomatitis (perleche) must be specially taken care of due to untoward haemorrhage 

Alveoloplasty and pre prosthetic surgeries should be done after hospitalization and factor replacement 

therapy. 

Conservative dentistry 

Conserving the teeth is of prime concern 

Minimize the risk of laceration of soft tissues in the operative field. 

Avoid creating ecchymoses and haematomas using high speed evacuators and saliva ejectors. 

Rubber dam isolation is advised to minimize soft tissue laceration. At the same time, clamp must be 

taken care of; not to traumatize gingival. 

Matrices, wedges and hemostatic gingival retraction cord can be used with caution. 

Restorations should not be the causative agents to accumulate food particles. 

Finishing, polishing & accessibility to cleaning by tooth brush must be property looked upon. 

Periodontal management 

Periodontal health is of critical importance because of hyperaemia causing gingival bleeding and 

periodontitis causing tooth morbidity leading to extraction of teeth. 

Most of the patients are afraid of tooth brush induced bleeding so plaque and calculus would be very 

common. 



35 



Periodontal probing, supragingival scaling with fine scalers and polishing must be done routinely to 

avoid chances of gingivitis and periodontitis which may cause severe bleeding. 

Careful subgingival scaling may be done with very fine scalers 

Deep subgingival scaling and root planning may be done quadrant wise to reduce gingival area 

exposed to potential bleeding. 

Prior to subgingival scaling, supra gingival scaling is mandatory. 

Local application of pressure should be done after scaling. 

Antifibrinolytic oral rinses are usually successful in preventing oozing of blood. 

Mucogingival surgery may need factor replacement therapy after hospitalization. 

Elevation of factor level may be achieved (up to 50%) in circulation after the use of antifibrinolytic 

mouth washes. 

Endodontic therapy 

It is the treatment of choice to a patient with severe hemophilia. 

It avoids the unnecessary bleeding caused by extraction of teeth to hemophiliacs. 

Instrumentation should not go beyond the apex of tooth. 

Filling of root beyond apical seal should be avoided. 

Application of vasoconstrictors like epinephrine at the apical area provides hemostasis. 

Intrapulpal anaesthesia is the best advised. 

Factor replacement therapy may be needed for endodontic surgical procedures just like any other oral 

surgical procedures. 



36 



Pedodontic management 

During the exfoliation of primary teeth, prolonged oozing of blood may be observed. Curettage of the 

area and pressure cold application may be sufficient to control the bleeding; Gauze pressure and 

seepage stops the bleeding within 12 hours (to a maximum). 

Pulpotomies may be performed without excessive pulpal bleeding. 

Stainless steel crowns should be prepared to allow minimal removal of enamel and gingival area. 

Non invasive, prophylactic and Restorative treatments should be given. 

Orthodontic treatment 

Care must be taken to avoid laceration by orthodontic bands brackets and wires. 

Fixed orthodontic treatment is preferred over removable orthodontics. 

Shorter treatment durations and use of extraoral force decrease the potential for bleeding 

complications. 

Proper Oral hygiene instructions and review appointments are highly essential for a good result in this 

case. 

The use of extra oral force and short treatment may be advised. 



37 



GENERAL INSTRUCTIONS TO PERSONS WITH OTHER BLEEDING & CLOTTING DISORDERS 

Despite the fact that different blood components are involved en each of bleeding and clotting diseases, 

their clinical and oral manifestations are identical. They will therefore be described together as a single disease. 

* Brushing twice daily with a fluoride toothpaste. 

- 1,000-ppm fluoride toothpaste for children under 7 years of age. 

- 1,400-ppm fluoride toothpaste for people over 7 years of age. 

The use of fluoride toothpaste depends on the fluoride concentration in the water 

supply as well as the use of additional fluoride supplements. It should not be used if fluoride supplements are 

taken or if the water supply has a fluoride content of 1ppm or more. 

* The tooth brush should have medium texture bristles because hard bristles can cause abrasion of the 

teeth and soft bristles are inadequate to remove plaque. 

* Interdental cleaning aids, such as floss, tape, and interdental brushes, should be used to prevent the 

formation of dental caries and periodontal disease. 

* Fluoride supplements may be used, but are not recommended if the water supply has a fluoride content 

of 1ppm or more. 

Supplements include: 

- Fluoride drops 

- Fluoride tablets 

- Topical application of fluoride using trays. 

- Fluoride mouthrinses which can be used on either a daily or a weekly basis. 



38 



* The consumption of foods and drinks with a high sugar or acid content should be limited to 

mealtimes. Three exposures per day is the recommended maximum. The aim is to ensure that the intake of 

food and drink does not cause the pH of the oral cavity to fall below the critical level of pH 5.5. 

* Artificial sweeteners can be used as an alternative to sugars in food and drinks. 

Examples are aspartame, sorbitol, acesulfamate, etc. 

* Regular dental visits, usually every 6 months, will help identify problems early, 

reinforce prevention, and emphasize the importance of reducing, the intake of food and drink 

containing high levels of sugar or acid. 

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, 

particularly in the sublingual region; 

Protection of soft tissues during restorative treatment by using a rubber dam or applying yellow soft paraffin 

(vaseline) 

Periodontal treatment 

Healthy periodontal tissue is essential to prevent bleeding and tooth loss. If oral hygiene is poor, treatment must 

start as soon as possible after the patient has had a dental examination and treatment plan formulated to prevent 

additional damage to the periodontal tissues. In cases of severe periodontal disease, it may be necessary to carry 

out supragingival scaling initially along with oral hygiene education. Subgingival scaling can start as soon as 

the inflammation has decreased. 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. 

* 



39 



Blood loss of all kinds can be controlled locally with direct pressure or periodontal dressings with or without 

topical antifibrinolytic agents. 

Periodontal surgery in patients with bleeding disorders must always be regarded as a high risk procedure with a 

significant risk of blood loss. It should only be considered were conservative treatment has failed and oral 

hygiene is good. Periodontal surgery can be a greater challenge to hemostasis than a simple extraction. The 

procedure must be carefully planned and the risks fully explained to the patient. 

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. 

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. 

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 edges. This can be controlled by local means or the 

application of topical agents. 



40 



Endodontics 

Endodontic treatment is generally low risk for patients with bleeding disorders. If a pulpectomy is indicated, the 

possibility of the tooth requiring conventional endodontic treatment must also be considered. It is important that 

the procedure be carried out carefully with the working length of the root canal calculated to ensure that the 

instruments do not pass through the apex of the root canal. The presence of bleeding in the canal is indicative of 

pulp tissue remaining in the canal. Sodium hypochlorite should be used for irrigation in all cases, followed by 

the use of clacium hydroxide paste to control the bleeding. Formaldehyde-derived substances may also be used 

in cases where there is persistent bleeding or even before the pulpectomy. 

COMMUNITY DENTISTRY AND HEMOPHILIA 

All patients with hemophilia 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 procedure, so that if an opportunity 

developers at some time in the future, they could be called up at short notice and have their dental needs 

attended to. This identification of persons with hemophilia would not only allow their dental treatment needs to 

be unidentified, but also allows this group of patients and their families to be specially targeted with the 

preventive dental care message to reduce the progression of existing disease and diminish future dental 

treatment needs. 

Patients with hemophilia are different from ordinary patients in that the latter group can have multiple 

visits to achieve a definitive treatment even on one tooth. this is luxury that it is not usually available to persons 

with hemophilia: therefore, a much more restrictive view should 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 persons 

with hemophilia are much more restricted. There are multiple causes for this including costs, availability of 

service, and availability of factor replacement post-operative care. 

In relation to infiltration anesthesia used mainly in the upper jaw, a 30% factor rise is a normal requirement. 



41 



Psychosocial Aspects Of Hemophilia Care 

A person with hemophilia and his family need psychological social support in coping with an 

illness that is chronic, often painful and sometimes life threatening. It is financially a burden and places 

restriction on several aspects of normal living. Psychological Support is an essential ingredient of any 

successful medical management 

Most effective when given by the primary physician. 

Simple to implement when essential techniques are applied. 

General Principles 

Provide privacy. 

Seat patient comfortably at eye level. 

Make eye contact 

Use open ñended questions, eg. ìHow are you feelingî rather that :how is the pain today?î 

This allows the person to express any problem being experienced rather than restricting him 

to only pain. 

Use simple languages; avoid medical jargon. 

Use body languages to advantage-leading forward, nodding, maintaining eye contact, 

avoiding making full notes while subject is speaking-are all powerful signals which convey 

empathy and interest. 

Use silences effectively. It is useful to pause statement that are likely to cause distress, eg. 

disclosure of diagnosis. Continue to display a listening posture. Important concerns and fear 

will be elicited. 



42 



SUPPORT FROM A SOCIAL WORKER 

The role of a social worker in helping in the management of chronic illness is being 

increasingly recognized. The social worker coordinates efforts of different services at a hemophilia 

center to individualize it to the patientís specific needs. Family visits help in providing psychological 

support, aid in assessment of problems faced in day-to-day living and community of care. In developing 

countries the concept of a co-ordinated team based care remains in the future. However, local groups have 

developed networks for small areas that provide similar care. 

PREVENTION OF HEMOPHILIA 

For disease like hemophilia, where cure is not yet possible, prevention is crucial. 

This is now possible with>99% accuracy using DNA analysis 

It is best done by obtaining DNA from the foetus around the end of the 10 th week of pregnancy 

by chorionic villous sampling or the 16-18 weeks by amniocentesis. 

If the family has been evaluated before, then results should be available within a week otherwise 

it may take 1-2 weeks 

Prenatal diagnosis should be only be attempted after appriopriate counseling of the family 

and ascertaining that the couple desires termination of pregnancy,If the foetus is affected. 



43 



Reference Centres 

At present comprehensive care facilities for persons with hemophilia (including genetic diagnosis) are available 

at- 

Christian medical college & Hospital 

Vellore 663-004 

Tel: 0416-2223603/2225506/22 (Ext 

2352/2353) Fax: 0416- 

2232035/223205 

HEMOPHILIA SOCIETY OF INDIA 

Hemophilia Society - Angamaly 

Mr. Paul Nettikkadan 

Vappalassery P.O. Via Angamaly-683572 

(Kerala) 

Tel: 0484-2452546 Extn: 413 (Hosp) 

Tel: 0484-2452410 

Email: angmaly@hemophilia.in 

K.E.M. Hospital 

Mumbai 400 00 

Tel:022-24131763/24136051 

(Ext:2358/2142) 

Fax: 022-24138521 

Hemophilia Society - Calicut 

Dr. C.P. Aboobacker 

Near AWH Medicals, Annie Hall Road Calicut- 

673002 (Kerala) 

Tel: 0495-2300284 

Mob: +91-94476 38294 (Hameed) 

Email: calicut@hemophilia.in 



44 



Hemophilia Society - kunnamkulam 

Mr. E. Raghunandan 

Smrithy, Akkikavu Karikkad, Kunnamkulam- 

680519, (Kerala) 

Tel: 04885-226639, 9847622366 

(Raghunandan) 

Mob: +91-9447236361 

Email: kunnamkulam@hemophilia.in 

Hemophilia Society - Cochin 

Shyamala Ramaswami 

XX/142 D, Thripthi Apartments 

Flat No-A ñ 2nd Floor, Near Agasthya Medical 

Centre, Old Bus Stand 

Thripunithura, Cochin-682301, (Kerala) 

Tel: 0484- 2774083 

Email: cochin@hemophilia.in 

Hemophilia Society - Trivandrum 

Mr. Vishnu M. S. 

T.C 29/1124(74/1977 New) House No-4, 

Devinagar, Palkulangara, 

Petah P.O, Trivandrum- 695024 (Kerala) 

Tel: 0471- 5520911 

Mob: +91-9895095518 

Email: trivandrum@hemophilia.in 

Hemophilia Society - Vellore 

President: Dr Ikram 

Secretary: Mr Kannan 

KGN Hospital No.94, Arcot Road, Vellore ñ 

632004, (Tamil Nadu) 

Tel: +91-98430 25146, 0416- 2225146 

Email: vellore@hemophilia.in 

Hemophilia Society - Mumbai 

Mr. Balshiram Gadhave 

Flat No. 205, 2nd Floor, Sai Apartment, 64, 

B, Swarajya Bhavan, 

Bhagwan Rao Parelkar Marg, Parel Village, 

MUMBAI - 400 012 (Maharashtra) 

Tel: 022-24158315, 24184071 

Email: mumbai@hemophilia.in 



45 



References 

1. White GC 2nd, Rosendaal F, Aledort LM, Lusher JM, Rothschild C, Ingerslev J. Factor VIII and Factor 

IX Subcommittee. Definitions in hemophilia, Recommendation of the scientific subcommittee on factor 

VIII and factor IX of the scientific and standardization committee of the International Society on 

Thrombosis and Haemostasis. Thomb Haemost, 2001;85(3):560. 

2. Nilsson IM. Hemophilia. Stockholm: Pharmacia Plasma Products: 1994. 

3. Fried MW: Management of hepatitis C in the hemophilia patient. Am J Med 

1999;107(6B):85S-89S. 

4. Kumar A, Kulkami R, Murray DL, et al. Serologic markers of viral hepatitis A, B, C and D in patients 

with hemophilia. J Med Virol 1993; 41:205-9. 

5. Eyster ME, Diamondstone LS, Lien JM, et al. Natural history of hepatitis C virus 

infection in multitransfused hemophiliacs: effect of coinfection with human immune deficiency virus. 

The Multicenter Hemophilia Cohort Study. J Acquir Immune Defic Syndr Hum Retrovirol 1993; 6:602- 

10. 

6. Hyper links : www.wfh.org/, http://www.hemophilia.in, coolhealthtips.com, identalhub.com 

7. Text Book of oral Pathology ñ Shafer, Text Book of Oral Medicine ñ Burkitt, Text Book Of Oral 

Surgery- Killey and Kay, Text book of Pedodontics ñ Finn, Hemophilia and Dentistry ñ Cunning Ham. 



46 



GLOSSARY 

Anemia - A condition in which the blood is either deficient in red cells, in hemoglobin, or in total volume. 

Antifibrinolytic - Drugs that can help stop the normal breakdown of blood clots and help speed recovery from a 

bleed. 

Antihistamine - A drug that can reduce or prevent allergic reactions. 

Anti-inflammatory - A drug that can reduce or prevent pain, redness, and swelling caused by inflammation (the 

body's reaction to irritation, injury, or infection). 

Artery - A large tube or blood vessel that carries blood from the heart through the body. The body has several 

arteries. AIDS (Acquired Immune Deficiency Syndrome) - A disease that attacks and destroys the bodyís 

immune system, leaving the patient abnormally vulnerable to infections and many other diseases. 

Bleeding Disorders - A group of distinct conditions in which a personís body cannot properly develop a clot, 

causing an increased tendency for bleeding. 

Calf - The area of the leg between the knee and the ankle. 

Capillary - Any of the very small tubes or blood vessels that form a network to carry blood through the body. 

The body has many capillaries 

Carrier - person who carries a gene that causes a disorder, usually showing no symptoms. 

Cartilage - The smooth surface covering the ends of the bones in a joint. 

Cell - The smallest independent living thing on earth. The human body is made of trillions of tiny cells. 

Chromosome - A very fine, threadlike strand of proteins and DNA in the centre of human, animal, and plant 

cells. Two 



47 



chromosomes (called X and Y) decide a person's sex. Females are born with two X-chromosomes (XX = girl). 

Males 

are born with one X and one Y chromosome (XY = boy). 

Clot - A thick lump of blood formed by clotting factors that work together to help stop bleeding. 

Cryoprecipitate - A treatment product made from blood plasma. It contains proteins, such as factor VIII (eight) 

and von Willebrand factor, but not factor IX (nine). It is infused into a vein over a period of time. First 

discovered 

by Dr. Judith Graham Poole in 1965. 

Asymptomatic carrier - A carrier who shows no outward signs of a condition. 

Symptomatic carrier - A carrier who has low factor levels and manifests bleeding symptoms. 

Coagulation disorders - a large group of conditions in which a person experiences excessive bleeding or 

clotting. 

Clotting factors - These are important proteins needed to form blood clots. 

Desmopressin Acetate (also DDAVP or StimateÆ) - A synthetic hormone used to treat some people with mild 

hemophilia or von Willebrand disease. The product increases the factor VIII levels or von Willebrand factor 

levels in blood. 

Diluent - The liquid that is mixed with factor concentrate powder. The final product is injected to treat bleeds. 

Factor Assay - A specialized lab test used to determine the level of circulating factor VIII or IX in a personís 

body. The test results are reported as a percentage of normal levels. 

Factor deficiencies - These are rare disorders identified by the particular deficient or missing clotting factor in a 

personís body. These conditions include: Factor I, II, V, VII, VIII, IX, X, XI, XII, and XIII. 



48 



Factor concentrate - A type of treatment that replaces the missing factor VIII (eight) or IX (nine) by injection 

into a vein. Factor can be made from human blood plasma and then dried to a powder. This is dissolved in 

diluents before injection. See recombinant for description of another kind of factor concentrate. 

Gene - A section of DNA, the chemical code of the body that controls production of a protein. Genes carry 

messages about the way the cells of the body work. For example, they determine a person's hair and eye colour. 

Hemophilia is passed on through a person's genes. 

Gene Therapy - A method of replacing, manipulating or supplementing a dysfunctional gene with a functioning 

one. This evolving technique is currently being researched in several inherited diseases, including hemophilia. 

There is hope that gene therapy will lead to better treatments, and eventually cures. 

Hemoglobin - The protein that carries oxygen and carbon dioxide in red blood cells. 

Hematologist - A physician who specializes in disorders of the blood. 

Hemostasis - The process by which the body stops bleeding. It is the stoppage of blood flow through a blood 

vessel or an organ of the body. 

Hepatitis - A group of viruses that can lead to infection and inflammation of the liver. 

Hereditary disease - A condition that is genetically passed down to oneís offspring. 

HIV (Human Immunodeficiency Virus) - The virus that causes AIDS. 



49 



Hemophilic arthritis - Inflammation of a joint, usually with pain and swelling, due to repeated bleeds. Cartilage 

in the joint breaks down and some bone wears away. Sometimes the joint cannot move. 

Home Therapy - Injection or infusion with a treatment product away from the hospital, usually at home. 

Infusion - Injection of a treatment product into a vein. 

Inheritance - The biological process of transmitting certain characteristics or conditions from parents to 

offspring. 

Inhibitor - An antibody that develops in direct response to infused clotting factor concentrates. As a result of 

this rare complication, standard treatment is temporarily made ineffective. 

Joint - The place where two bones meet. 

Joint capsule - The area or sleeve that holds the bones together in a joint (where two bones meet). 

Nerve - The sensitive, cordlike fibers that pass messages through the body, including pain. 

NSAIDS or non-steroidal anti-inflammatory drug - drug, such as ibuprofen, that reduces pain and fever but 

does not contain steroids. 

Orthopedic - A term having to do with the bones, the skeleton or associated structures. 

Plasma - Part of blood that contains fibrin and clotting factors. 

Platelets - The blood cells that make a plug to patch holes in arteries, veins, and capillaries. 



50 



Prophylaxis - A treatment regimen aimed at preventing bleeding episodes among people with hemophilia. 

Primary prophylaxis - A method of regularly scheduled treatments to prevent bleeding episodes before they 

occur. Today, the majority of people with severe hemophilia begin this regimen at a young age to prevent long- 

term damage to joints. 

Recombinant - A type of factor concentrate that is manufactured in a laboratory instead of being separated from 

human blood. Recombinant proteins are copies of certain kinds of proteins found in human blood plasma. 

Spontaneous bleeding - Bleeding that happens for no clear reason (not after an injury or surgery). 

Synovium - The lining of the joint capsule. It is made of special cells that make a slippery, oily fluid that helps 

the joint move easily. 

Spontaneous mutation - The development of a hereditary disease for which there is no family history. 

Target joint - A term for a particular joint that has experienced repeated bleeds or at least four bleeds into one 

joint within a six-month period. 

Thrombophilia - A categorical term for several distinct conditions where there is an increased tendency for 

excessive clotting. 

Von Willebrand Disease - A bleeding disorder in which von Willebrand factor, a blood protein, is either 

missing or does not function properly. Von Willebrand disease is the most common bleeding disorder since 

both men and women can inherit it equally. 

Venepuncture - This can be done to take blood or to give an intravenous injection. 

******************************

hand book for dental surgeons - Indian Dental Association ...

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