hand book for dental surgeons - Indian Dental Association ...
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DENTAL MANAGEMENT OF PERSONS<br />
WITH HEMOPHILIA AND OTHER<br />
HAEMOSTATIC DISORDERS<br />
HAND BOOK FOR DENTAL SURGEONS<br />
PUBLISHED BY : INDIAN DENTAL ASSOCIATION, KUNNAMKULAM BRANCH
DENTAL MANAGEMENT OF PERSONS<br />
WITH HEMOPHILIA AND OTHER<br />
HAEMOSTATIC DISORDERS<br />
HAND BOOK FOR DENTAL SURGEONS<br />
PUBLISHED BY:
INDIAN DENTAL ASSOCIATION, KERALA STATE PRESIDENTíS MESSAGE<br />
Bleeding disorders have been of much concern to health professionals including<br />
Dentists <strong>for</strong> quiet some time. Hemophilia patients are special patients from a<br />
<strong>dental</strong> point of view, as the highly vascular oral cavity is definitely a hotspot <strong>for</strong><br />
hemorrhage in this group of patients. It is not infrequently that a dentist become<br />
the first person to diagnose a bleeding disorder while per<strong>for</strong>ming routine <strong>dental</strong><br />
treatments. Thorough knowledge of systematic approach is mandatory <strong>for</strong> any<br />
<strong>dental</strong> health professional raring to <strong>hand</strong>le hemophiliacs the absence of which can lead to<br />
disastrous outcomes.<br />
I am very glad to note that IDA Kunnamkulam Branch is bringing out a <strong>hand</strong><br />
<strong>book</strong> to educate the IDA members about the <strong>Dental</strong> Management of Hemophilia patients. I am<br />
sure this <strong>hand</strong> <strong>book</strong> would be of great help to our IDA Members to learn more about the<br />
necessary precautions to be carried out while treating hemophilia patients in our day to day<br />
practice. I congratulate IDA Kunnamkulam branch especially the Editor, Dr. Joji George <strong>for</strong> the<br />
sincere ef<strong>for</strong>ts in bringing out this <strong>hand</strong><strong>book</strong> and releasing it during the state level observation of<br />
world Hemophilia Day on 17 th April 2011. I hope and wish our fellow members will find this<br />
<strong>book</strong> useful in their practice.<br />
Dr. Santhosh Sreedhar<br />
President ñ IDA Kerala State
IDA KERALA STATE SECRETARYíS MESSAGE<br />
Its indeed heartening to know that IDA Kunnamkulam is publishing a <strong>hand</strong><br />
<strong>book</strong> to <strong>dental</strong> <strong>surgeons</strong> about hemophilia and dentistry .Its a fact that among the<br />
estimated hemophilic patients of around 4,00000, many thousands of affected are<br />
enduring life without proper access to treatment and care. Itís time to express our<br />
courage and perseverance ,accelerate our pace of progress and remove the<br />
remaining barriers <strong>for</strong> adequate care. I am sure this <strong>hand</strong><strong>book</strong> will provide a basis<br />
<strong>for</strong> the development of local protocols <strong>for</strong> the <strong>dental</strong> treatment of patients with inherited bleeding<br />
disorders.<br />
Dr.Shibu Rajagopal<br />
Hon. Secretary<br />
IDA Kerala State
IDA KERALA STATE CDE CHAIRMANíS MESSAGE<br />
The arrival of a hemophiliac patient in the waiting room often sends<br />
shudders down the spine of the dentist. The lack of in depth knowledge<br />
often hinders the management of such patients.Routine <strong>dental</strong> procedures<br />
do not generally involve bleeding; consequently, there is no<br />
contraindication to routine <strong>dental</strong> treatment <strong>for</strong> hemophiliacs. Preventive<br />
dentistry is vital to the younger hemophiliac patient world-wide; older<br />
hemophiliacs may require extensive treatment to restore mouths that have been neglected.<br />
Surgical <strong>dental</strong> procedures may be per<strong>for</strong>med <strong>for</strong> hemophiliacs, but they must be judiciously<br />
coordinated by the <strong>dental</strong> and medical team. The facilities and treatment available will determine<br />
the feasibility of treating such patients in the hospital or on an outpatient basis. It is my earnest<br />
wish to be able to impart useful knowledge so as to arm the dentist with know-how to manage<br />
the hemophilac patient.<br />
I hope this <strong>hand</strong><strong>book</strong> will serve this purpose and wish to thank all those individuals who have<br />
worked to make this a reality.<br />
with regards<br />
Deebu J Mathew<br />
CDE Chairman
Dear friends,<br />
IDA KERALA STATE CDH CHAIRMANíS MESSAGE<br />
Warm greetings to you ! <strong>Indian</strong> <strong>Dental</strong> <strong>Association</strong> Kerala State branch is<br />
stepping ahead once again,by marking itís role in building up a healthy<br />
Nation ! By the<br />
introduction of ASHAKIRAN ( the pain & palliative care project) last year ,<br />
we have<br />
given a new face to our activities. Today ,on this 17th of April ,when we observe the<br />
World Hemophilia Day by conducting a Hemophilia workshop, we are happy to release<br />
a Hand <strong>book</strong> <strong>for</strong> <strong>Dental</strong> Surgeons on management of persons with Hemophilia. This<br />
Hand <strong>book</strong> will be an asset to all <strong>Dental</strong> Surgeons. At the same time, let me thank all<br />
the members of <strong>Indian</strong> <strong>Dental</strong> <strong>Association</strong> <strong>for</strong> giving their whole hearted support in the<br />
new steps of the Council on <strong>Dental</strong> Health,of IDA Kerala State. I congratulate the IDA<br />
Kunnamkulam branch <strong>for</strong> their untiring ef<strong>for</strong>ts to bring out this Hand <strong>book</strong>.<br />
With wram regards & prayers<br />
Dr Abdul Latheef K.H<br />
Chairman<br />
Council on <strong>Dental</strong> Health<br />
IDA Kerala State
IDA KERALA STATE EDITORíS MESSAGE<br />
Hemophilia is a genetic, life threatening bleeding disorder. In persons with<br />
hemophilia (PwH) blood does not clot normally due to deficiency or absence<br />
of clotting proteins called Factors. The disease is a lifelong bleeding disorder,<br />
incurable in nature and a very expensive to manage in terms of medication<br />
and care. Recurrent and prolonged bleeding into joints and muscles can lead<br />
to permanent disability. Bleeding from the sensitive organs can lead even to death. IDA through<br />
its CDH activity can play a major role in helping hemophila patitents.<br />
As apart from providing factor and other medical care such as physiotherapy <strong>for</strong><br />
affected persons, IDA can reach out across the community, educating people<br />
about hemophilia and attempting to de-stigmatize the condition.<br />
Our aim to reach out to community and provide total quality care, education,<br />
make treatment available at af<strong>for</strong>dable cost, psycho-social support, and economic<br />
rehabilitation and thus help them in improving the quality of life without disability<br />
and free of pain.<br />
There are miles to go be<strong>for</strong>e fulfilling the vision of IDA<br />
ìHemophilia without Disability, Free of Pain.î
IDA KUNNAMKULAM BRANCH PRESIDENTíS MESSAGE<br />
<strong>Indian</strong> <strong>dental</strong> association Kunnamkulam branch is extremely happy and<br />
proud to conduct the Kerala State combined CDH ñ CDE Hemophilia<br />
work shop on April 17 th at Kunnamkulam IMA hall, world hemophilia<br />
day. I would like to congratulate and thank the state office bearers,<br />
energetic CDH chairman of State Dr. Abdul Lateef, Co-ordinator Dr.<br />
Sushanth (CC member) and the editor of the <strong>hand</strong> <strong>book</strong> Dr. Jogi George (state executive<br />
member) <strong>for</strong> their great work.<br />
I hope, this <strong>hand</strong> <strong>book</strong> <strong>for</strong> <strong>dental</strong> Surgeons on <strong>Dental</strong> Management of the Hemophilia and other<br />
hemostatic disorders would be a treasure to all of us.<br />
Dr. Geo. Joy. K, President, IDA Kunnamkulam Branch.
EDITORIAL<br />
<strong>Dental</strong> Surgeons face bleeding problems in their day to day clinical<br />
practice. Among them, Hemophilia and other haemostatic disorders<br />
need special attention and management Long back, Hemophilia was<br />
considered to be the Royal disease. But today, the Scenario has<br />
changed. The etiology can be mutation and even drugs. Other<br />
haemostatic disorder like von wille brands disease, thrombocytopenia<br />
etc., are becoming common.<br />
So I hope, this Hand<strong>book</strong> <strong>for</strong> <strong>dental</strong> Surgeons on <strong>Dental</strong> Management of the Hemophilia and<br />
other Haemostatic disorders would be useful to all <strong>dental</strong> <strong>surgeons</strong><br />
I thank all contributors to this venture.<br />
Dr. Joji George.
1<br />
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Hand <strong>book</strong> <strong>for</strong> <strong>dental</strong> <strong>surgeons</strong> on <strong>dental</strong> management of persons with hemophilia and other haemostatic disorders<br />
1. World health organization.<br />
2. World hemophilia federation.<br />
3. Hemophilia Society of India.<br />
4. Hemophilia Society Kunnamkulam Chapter.<br />
5. <strong>Indian</strong> <strong>dental</strong> association Kerala state.<br />
ACKNOWLEDGEMENTS:<br />
6. Dr.Ashok Varma, M.S., General surgeon, Unity Hospital, Kunnamkulam.<br />
7. Dr.Varghese Mani, M.D.S., Oral and Maxillofacial surgeon.<br />
8. Dr.R.Mahesh Kumar, M.D.S., Oral and Maxillofacial surgeon.<br />
9. Dr.K.Nandakumar, M.D.S., Editor, Kerala <strong>Dental</strong> Journal.<br />
10. Dr.S.Balu Mohan , M.D.S., Oral Medicine.<br />
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Hand <strong>book</strong> <strong>for</strong> <strong>dental</strong> <strong>surgeons</strong> on <strong>dental</strong> management of persons with hemophilia and other haemostatic disorders<br />
CONTENTS<br />
1. HAEMOSTATIC DISORDERS P 3-4<br />
2. HAEMATOLOGY AND HAEMOSTATIC DISORDERS P 5-6<br />
3. CLOTTING MECHANISM OF BLOOD P 7<br />
4. HEMOPHILIA P 8-10<br />
5. GENERAL MEDICAL TREATEMENT OF HEMOPHILIA P 11-12<br />
6. DENTAL MANAGEMENT IN PERSONS WITH HEMOPHILIA AND OTHER HAEMOSTATIC<br />
DISORDERS - AN OVER VIEW<br />
P 13-23<br />
7. HEMOPHILIA AND CHILDREN (PEDODONTICS) PG 24 -31<br />
8. DENTAL TREATMENT MODALITIES FOR HEMOPHILIC PATIENTS ñ SOME TIPS PG<br />
32-36<br />
9. GENERAL INSTRUCTIONS TO PERSONS WITH OTHER BLEEDING & CLOTTING DISORDERS PG 37-<br />
39<br />
10. COMMUNITY DENTISTRY AND HEMOPHILIA PG 40-<br />
42<br />
11. REFERENCE CENTERS AND REFERENCES PG 43-45<br />
12. GLOSSARY PG 46-50<br />
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HAEMOSTATIC DISORDERS<br />
Bleeding disorders are conditions that after the ability of blood vessels, platelets and coagulation factors to<br />
maintain haemostasis inherited bleeding disorders are genetically transmitted. Acquired bleeding disorders<br />
occur secondary to diseases affecting vascular wall integrity, platelets of coagulation factors, drugs, radiation or<br />
chemotherapy <strong>for</strong> cancer. Hemorrhage, intravascular thrombosis and embolism are common clinical<br />
manifestations of abnormal interactions between components of the vessel, circulating platelets and plasma<br />
proteins. However, when disease or trauma damage large arteries and veins, excessive bleeding may occur<br />
despite a normal haemostatic system.<br />
Primary haemostasis: Stops blood loss from capillaries, small arterioles and venules. It occurs within<br />
seconds. During this period, platelet plug is <strong>for</strong>med at the site of injury. Three critical events occur during this<br />
phase platelet adhesion, granule release and platelet aggregation.<br />
Secondary haemostasis: Completed in several minutes and is important in bleeding from larger vessels.<br />
During this period fibrin is <strong>for</strong>med it is the activation of clotting process in plasma. Which ultimately results in<br />
the <strong>for</strong>mation of fibrin. Which strengthens primary haemostatic plug. It is a continuous process and there are<br />
approximately 40 substances, which affect clotting. Some promote clotting (procoagulants) and some inhibit<br />
clotting (anticoagulants). Normally there is a fine balance between these factors and blood usually does not<br />
coagulate in the vessel. Whenever there is an injury to the vessel these procoagulants are activated and balance<br />
tilts infavour of coagulation and there is <strong>for</strong>mation of clot.<br />
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Normal haemostatic mechanism is disturbed when any defect occurs in the mechanism of primary haemostasis<br />
<strong>for</strong> secondary haemostasis and it results in bleeding disorders. On this basis, bleeding disorders can be broadly<br />
classified as due to primary haemostatic disorder or due to secondary haemostatic disorder or due to secondary<br />
haemostatic disorder. Primary heamostatic (Platelet) disorders are further subdivided in two types non<br />
thrombocytopenic purpura and thromobocytopenic purpura.<br />
Non thrombocytopenic purpura is further subdivided into defects of platelet adhesion (Von Willebrandís<br />
disease, Bernad soulier syndrome), defects of platelet aggregation (Glanzmannís Thrombosthenia), defects of<br />
platelet release (drug induced granule storage pool defect, uremia, platelet coating by pencillin or paraproteins)<br />
and defects of platelet coagulant activity (Scottís syndrome).<br />
Secondary haemostatic (coagulation) disorders are further subdivided into two types inherited<br />
(hemophilia A, hemophilia B, parahemophilia, etc. ) and acquired (liver disease, renal failure, DIC, anti-<br />
coagulation drugs, vitamin deficiency)<br />
The best method of determining whether the defect is in primary haemostasis or secondary haemostasis<br />
is by various laboratory investigations. Most important screening tests of primary haemostatic system are<br />
platelet count and bleeding time.<br />
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HAEMATOLOGY AND HAEMOSTATIC DISORDERS<br />
Normal platelet count is 150000-450000 platelets per microlitre of blood. Symptoms do not arise and<br />
bleeding time remains normal until it is greater than 100000/micro litre. Mild prolongation bleeding time<br />
occurs when it is between 50000-100000/micro litre. Easy bruising manifested by skin purpura occurs after<br />
minor trauma and bleeding after mucous membrane surgery occurs when it is less than 50000/micro litre<br />
Spontaneous bleeding occurs when its value is less than 20000/micro litre. When qualitative platelet<br />
abnormality is present then there is normal platelet count and prolonged bleeding time. Bleeding time over 10<br />
minutes has increased risk of bleeding. Extensive bleeding occurs when bleeding time is greater than 15-20min.<br />
Due to cyclical variation level of V Wb factor occasionally bleeding time may be normal so repeated testing<br />
may be necessary to establish diagnosis.<br />
Plasma coagulation function is readily assessed by partial thromboplastin time (PTT). Prothrombin time<br />
(PT), thrombin time, clot solubility test and clot lyses test.<br />
When there is prolonged partial thromboplastin time ( normal is 25-35 seconds) and no clinical bleeding,<br />
then one could suspect deficiency of factor 12, HMWK, PK. When it is associated, with mild or rare bleeding<br />
then factor 11 deficiency might be present and when there is frequent severe bleeding then deficiency of factor<br />
8 & 9 should be suspected. Prolonged prothrombin time (normal is 10-15 sec) indicates deficiency of factor 7,<br />
vitamin k (early) or warfarin ingestion. When PT & PTT both are prolonged, it indicates deficiency of factor 2,<br />
5 & 10, vitamin k (late) or warfarin ingestion.<br />
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Among the various disorders of secondary hemostatisis most well known and common is haemophilia<br />
A. It has an incidence of 1 or 2/100000 of the population and it is caused by a deficiency of antihemophilic<br />
globulin (AHG or Factor 8) in the plasma. It is inherited as a sex-linked recessive character, appearing only in<br />
males and transmitted to them by clinically normal female carriers. The defective gene on the X chromosome<br />
causes a deficiency of Factor 8, Normal plasma contains one unit of a factor 8 per ml. or 100% level.<br />
Deficiency of factor is expressed as percentage (0.25unit/ml=25%). Patients with less than 1% AHG have<br />
severe disease. They bleed frequently into soft tissues, muscles and weight bearing joints even without<br />
discernible trauma. When level is between 1-5%, it indicates moderate disease and there are less severe<br />
bleeding episodes. Levels above 5% have mild disease in which there is infrequent bleeding usually secondary<br />
to trauma or surgery levels above 25% cause no troubles unless patient suffers from major trauma. Majority of<br />
patients have level of factor 8 less than 5%.<br />
The clotting time is not prolonged until the level of AHG falls below 1%. The only safe test is to<br />
estimate the AHG level in the patient <strong>for</strong> diagnosing the presence of this disease.<br />
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CLOTTING MECHANISM OF BLOOD<br />
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Introduction<br />
HEMOPHILIA<br />
Hemophilia is a hereditary disorder of coagulation results in deficiency of factor VIII (Hemophilia A) or<br />
factor IX (haemophilia B). The disease is almost exclusively see in males while females are asymptomatic<br />
carries. Rarely it can affect females. It can also be caused by mutation. It is characterized by spontaneous or<br />
trauma related bleeding typically into the large joints or muscles. Untreated or inadequately treated episodes<br />
lead to joint, muscles or nerve damage. Life threatening haemorrhages can result spontaneously or from trauma<br />
to the head or internal organs.<br />
Without proper care, hemophilia can lead to serious damage to limb and joint function within the first<br />
one to two decades of life. This is due to impaired joint mobility, contractures, muscle atrophy and chronic pan.<br />
Crippling joint de<strong>for</strong>mities can be effectively prevented by adequate care of each episode of bleeding, including<br />
replacement of the appropriate coagulation factor lacked by the patient and every deliberated and persistent<br />
physical therapy. Certain serious complications can further complicate the management of hemophilia.<br />
Between 10-20% of people with hemophilia A and 23% of those with hemophilia B develop inhibitors against<br />
the deficient factor. Such patients do not respond to usual factor replacement therapy. Patients exposed to<br />
factor replacement with products that have not been virus inactivate are prone to acquiring transfusion<br />
transmitted viral infections such as human immuno-deficiency virus (HIV), hepatitis B and C viruses. The care<br />
of people with hemophilia there<strong>for</strong>e often requires a multidisciplinary team to address different aspects of the<br />
patientís problems. This is the concept of comprehensive care that is recommended by the World Health<br />
Organization (WHO) and the World Federation of Hemophilia (WFH).<br />
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EPIDEMIOLOGY<br />
The prevalence of hemophilia is estimated to be about 1 in 10,000 births and that of the severe <strong>for</strong>m of<br />
the disease to be about 6 per 1,00,000 population. It is there<strong>for</strong>e likely that there are over 50,000 people with<br />
severe hemophilia in India. Only about 10% of them have identified. There is obviously a need to establish<br />
facilities that will help detect many more of the affected people.<br />
The manifestations of hemophilia depend upon the severity of the disease based on factor levels:-<br />
Severe Factor VIII or IX level is
10<br />
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Genetics Of Hemophilia-Carrier Detection And Antental Diagnosis<br />
The gene <strong>for</strong> factors VIII and IX are both located on the x-chromosome. Hemophilia is<br />
there<strong>for</strong>e said to be an x-linked hereditary disorder. This results in males being affected by the disease<br />
while females are carriers. While affected males with no functioning factor VII or IX gene have very<br />
low to absent factors in the blood, carrier females have at least one (out of two) functioning X-<br />
chromosomes with a normal factor VIII or IX gene that provides about 50% factor levels. Carrier<br />
females who have excessive lyonization of the normal x-chromosome can have very low F VIII levels<br />
and symptoms of bleeding.<br />
Identification Of Carriers<br />
A woman is a definite carrier if:<br />
Her father has hemophilia<br />
She has one son with hemophilia and a first degree relative (brother, uncle or other male<br />
relative) who are affected.<br />
She has two or more sons with hemophilia.<br />
A Women Is A Possible Carrier If:<br />
She has one or more maternal relatives with hemophilia<br />
She has one son with hemophilia and no other affected relatives.<br />
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GENERAL MEDICAL TREATEMENT OF HEMOPHILIA<br />
Hemophilia is treated by replacing the missing clotting factor in the blood. This is done by injecting a<br />
product that contains the needed factor into a vein. Bleeding stops when enough clotting factor reaches the<br />
bleeding site. It is very important that treatment is given as quickly as possible to prevent long-term damage.<br />
With an adequate quantity of treatment products and proper care, people with hemophilia can live<br />
perfectly healthy lives.<br />
Clotting factors are found in the following blood products in order of decreasing concentration:<br />
* factor concentrates<br />
* cryoprecipitate<br />
* plasma<br />
* whole blood<br />
Factor concentrates are the treatment of choice <strong>for</strong> hemophilia. They can be made from human blood<br />
(called plasma-derived products) or manufactured using genetically engineered cells that carry a human factor<br />
gene (called recombinant products). Factor concentrates are made in sophisticated manufacturing facilities. All<br />
commercially prepared factor concentrates are treated to remove or inactivate blood-borne viruses.<br />
People with mild hemophilia A sometimes use desmopressin (also called DDAVP), a synthetic hormone<br />
that stimulates the release of factor VIII.<br />
Cryoprecipitate is derived from blood and contains a moderately high concentration of<br />
factor VIII (but not IX) clotting factor. It is effective <strong>for</strong> joint and muscle bleeds, but it is less safe from viral<br />
contamination than concentrates and is harder to store and administer. Cryoprecipitate can be made at local<br />
blood collection facilities.<br />
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In fresh plasma, the red cells have been removed, leaving the blood proteins including the clotting<br />
factors. It is less effective than cryoprecipitate <strong>for</strong> the treatment of hemophilia A because the factor VIII is less<br />
concentrated. Large volumes of plasma must be transfused. This can cause circulatory overload.<br />
The life of the clotting factors is preserved by making a product called fresh frozen plasma (FFP). FFP<br />
is still the only product available <strong>for</strong> treatment of hemophilia A and B in some countries.<br />
There are considerable drawbacks to the use of whole blood in the treatment of hemophilia. First, it must<br />
be fresh because the activity of the clotting factors in the drawn blood decreases quickly with time. second, the<br />
red cells it contains must, of course, be compatible with those of the recipient. third, it takes a large volume of<br />
whole blood to stop a bleed and this volume may overload the circulation and cause the heart to fail.<br />
Antifibrinolytic medicines (including tranexamic acid and aminocaproic acid) may be used with<br />
replacement therapy. Theyíre usually given as a pill, and they help keep blood clots from breaking down.<br />
These medicines most often are used be<strong>for</strong>e <strong>dental</strong> work or to treat bleeding from the mouth or nose or mild<br />
intestinal bleeding.<br />
Researchers are trying to find ways to correct the faulty genes that cause hemophilia. Such gene therapy<br />
hasnít yet developed to the point that itís an accepted treatment. But researchers continue to test gene therapies<br />
<strong>for</strong> hemophilia in clinical trials.<br />
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DENTAL MANAGEMENT IN PERSONS WITH HEMOPHILIA<br />
AND OTHER HAEMOSTATIC DISORDERS - AN OVER VIEW<br />
Numerous procedures in <strong>dental</strong> practice may cause bleeding. Uncontrolled bleeding after minor surgical<br />
procedures like tooth extraction being one of the main complications encountered in clinical practice in normal<br />
circumstances, <strong>dental</strong> procedures can be per<strong>for</strong>med with little or no risk to the patient. However, a patient,<br />
whose ability to control bleeding is altered by drug or disease is in grave danger unless the dentist identifies the<br />
problem be<strong>for</strong>e per<strong>for</strong>ming any surgery. There<strong>for</strong>e, it is very important that proper medical and family history<br />
of the patient is collected be<strong>for</strong>e commencing any surgical procedure, and routine blood investigations should<br />
be carried to identify the bleeding disorder to reduce the risks associated with <strong>dental</strong> procedures.<br />
The <strong>dental</strong> treatment of patients with inherited bleeding disorders has been widely discussed in the literature<br />
with the aim of developing guidelines <strong>for</strong> common procedures. the majority of guidelines recommend the use of<br />
clotting factor replacement therapy be<strong>for</strong>e invasive oral surgery and the use of the inferior alveolar nerve block<br />
<strong>for</strong> restorative <strong>dental</strong> treatment. the dose of clotting factor used varies and this may be due to problems relating<br />
to both the availability and cost of factor concentrate in different parts of the world.<br />
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These protocols suggest the use of factor concentrate along with the use of local hemostatic techniques, such as<br />
suturing, and local measures, such as the use of oxidized cellulose, <strong>for</strong> example Surgical or fibrin glue in<br />
conjuction with post-operatively administered anti fibrinolytic agents where appropriate. The use of local<br />
techniques has resulted in certain minor oral surgery procedures being done with minimal or no coagulation<br />
factor replacement.<br />
Sindet-Pedersen suggests that the dose of factor replacement therapy can be significantly reduced if used<br />
with an oral rise of an antifibrinolytic agent(tranexamic acid) is used following a <strong>dental</strong> extraction.<br />
Desmopressin, a synthetic derivative of the hormone vasopressin, he has been shown to increase factor VIII<br />
level in some patients with mild or moderate <strong>for</strong>ms of hemophilia A or type 1 von Villebrand disease.<br />
Un<strong>for</strong>tunately, not all patients respond to this should be checked be<strong>for</strong>e per<strong>for</strong>ming any surgical procedure. Its<br />
use is well documented in cases of mild and moderate hemophilia. DDAVP releases bound factor VIII and is<br />
there<strong>for</strong>e not used to treat patients with hemophilia B. Fibrin glue is used as a local hemostatic measure in some<br />
centres <strong>for</strong> achieving hemostasis and reducing the needs <strong>for</strong> clotting factor replacement therapy.<br />
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<strong>Dental</strong> infections<br />
ORAL MEDICINE<br />
Many patients with infections of <strong>dental</strong> origin are managed without the use of antibiotics but instead by <strong>dental</strong><br />
extraction or endodontic treatment. Antibiotics are often used to treat an acute bacterial infection.This should be<br />
considered <strong>for</strong> all patients with inherited bleeding disorders since surgical intervention should be avoided if<br />
possible. The initial treatment usually starts based on the normal oral pathogens, Streptococcus viridans,<br />
anaerobic gram-negative rods. Antibiotic regimes should cover all of these groups of organisms.<br />
Penicillin is a first line antibiotic used to treat <strong>dental</strong> infections.It can be taken orally in the <strong>for</strong>m of pencillin<br />
V.Metronidazole is extremely effective in treating anaerobes and is often used in combination with pencillin to<br />
give good coverage of both the aerobic and anaerobic bacteria present in the oral cavity. The does of the drugs<br />
may vary depending on the availiblity in different countries but the treatment should continue <strong>for</strong> 5-7days. A<br />
number of different <strong>for</strong>mulations of penicillin are available with a broader spectrum of activity. These can be<br />
used either alone or in conjunction with metronidazole. However, it is important to remember that if these drugs<br />
are ineffective treatment of the infection will become more complicated. Erythromycin and clindamycin have<br />
been prescribed to patients who are allergic to penicillin. These drugs can be used in conjuction with<br />
metronidazole. Erythromycin may be effective in people with penicillin allergies, but it may not be suitable <strong>for</strong><br />
more severe infections. Clindamycin produces high alveolar concentrations, and bactericidal activity is reached<br />
with the usual recommended oral dose of 150mg every 6 hous. In more severe cases it can be given<br />
intravenously. It has been reported that clindamycin can cause antibiotic-associated colitis and there<strong>for</strong>e is<br />
often reserved <strong>for</strong> the treatment of the more serious infections or when penicillin has failed.<br />
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Periodontal pockets host a veriety of different bacteria, the majority of them being anaerobic. Regular oral<br />
hygiene prevents these bacteria from causing gingival inflammation. In patients with severe gingival<br />
inflammation, in particular those who are immune compromised; the use of an antimicrobal agent may be<br />
indicated Metronidazole is considered the drug of choice due to its action against anaerobic organisms. It may<br />
be used in conjunction with either penicillin or erythromycin. However, antimicrobial therapy is no substitute<br />
<strong>for</strong> oral hygiene treatment.<br />
Topical treatment<br />
The most common <strong>for</strong>ms of topical treatment involve the use of an antibacterial mouthwash. These have<br />
value as an adjunct to hygiene phase treatment. The most common treatments are:<br />
* Chlorhexidine gluconate: Chlorhexidine is available as a mouthwash, spray, and toothgel. It is most<br />
commonly used as a twice a day mouthwash with the patient rinsing their mouth with the solution <strong>for</strong><br />
30-60 seconds. The toothgel may be used in addition to the mouthwash. Chlorhexidine has a tendency<br />
to stain teeth so the length of each course of treatment should be limited.<br />
* Providine-iodine: providine-iodine is available as a mouthwash and can be used <strong>for</strong> the treatment of<br />
acute periodontal problems. In addition,it may be useful to irrigate the periodontal pockets. It must be<br />
used with caution during pregnancy.<br />
<strong>Dental</strong> emergencies<br />
<strong>Dental</strong> emergencies can occur at any time; however, it is important to remember that no treatment<br />
should be carried out without prior planning as this could result in additional problems. The most common<br />
<strong>dental</strong> problems are pain due to caries and bleeding from the periodontal tissues. Pain related to caries can<br />
usually be trated with either antibiotics or pulpectomy in order to allow time <strong>for</strong> the planning of the extraction.<br />
Bleeding from the periodontal tissues can usually be controlled with antibiotics until an appointment with a<br />
hygienist can be arranged.<br />
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The management of <strong>dental</strong> trauma is more complex as it usually involves both the gingiva and the teeth.<br />
Local measures will usually control gingival bleeding and temporary splinting can be used <strong>for</strong> fratured or loose<br />
teeth. With <strong>dental</strong> trauma, it is important to remember that we are part of the comprehensive hemophilia care<br />
team treating these patients. Treatment planning <strong>for</strong> an emergency requires input from the whole team to reduce<br />
the risk of further problems.<br />
ORAL AND MAXILLOFACIAL SURGICAL MANAGEMENT<br />
However where extractions, deep scaling, and/or inferior <strong>dental</strong> nerve (lower jaw) injections deep into the tissue<br />
are anticipated, then a 50% factor rise would be the legal of choice. Where the procedures intended are much<br />
more extensive, or become more extensive as the procedure develops, such as a surgical extraction, 100% rise<br />
may be necessary. This might necessitate an increase in the rise from 50% to 100% immediately post-<br />
operatively, if the treatment being undertaken becomes more extensive than was originally planned.<br />
Surgical extractions should not be undertaken lightly. They should only be undertaken when the circumstances<br />
absolutely demand it.<br />
In all cases of patients undergoing <strong>dental</strong> treatment involving blood clotting, the patient should be prescribed<br />
tranexamic acid. The adult dose is 500mg. one tablet four times a day to be taken <strong>for</strong> 10days post operatively to<br />
prevent the breakdown of any clot that <strong>for</strong>ms. <strong>for</strong> children, the above dose must be modified <strong>for</strong> their size and<br />
age. If no extractions or periodontal treatment is undertaken , only restorations, then the tranexamic acid should<br />
be taken <strong>for</strong> 3 to 7 days.<br />
While the use of sutures following extractions can be warranted there is normally no necessity <strong>for</strong> these to be in<br />
place longer than about 24 hours.<br />
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Four extractions in different areas of the mouth are less traumatic than four extractions next to each other. In the<br />
latter instance, there would be an extensive wound; because of the scalloped edge of the gum around the teeth it<br />
can be at least 50% larger than the distance corresponding to the extracted teeth width. If the sutures are<br />
removed within 24 hours, there is usually no need <strong>for</strong> a follow-up factor replacement treatment rise. Sometimes,<br />
it may be necessary to retain sutures <strong>for</strong> longer, but a dentist would decide when it is appropriate, usually it<br />
would only occur in an exceptional case if the <strong>dental</strong> surgery is very expensive.<br />
In relation to any oral surgical intervention, it is of paramount importance that there is the least possible<br />
interference with the attached gingival (gum) around the teeth and periosteum. Simply lifting the attached gum<br />
tissues from the underlying tissue or periosteum, even in healthy patients will cause post-operative bleeding. the<br />
goal is ìkey holeî surgery, where there is as little interference as possible with the attached gum to minimize<br />
post-operative bleeding.<br />
Start Tranexamic Acid the night be<strong>for</strong>e the procedure: 250mg <strong>for</strong> a child and 500mg <strong>for</strong> an adult <strong>for</strong> times daily.<br />
Continue <strong>for</strong> 5-7 days after the procedure.<br />
If there is an infection start an antibiotic the day be<strong>for</strong>e the procedure.<br />
Bacampicillin 400mg (Trade name - Penglobe) twice daily <strong>for</strong> dults and 200mg twice daily <strong>for</strong> children.<br />
Or Metrondazole 400mg (Trade name - flagyl) thrice daily <strong>for</strong> adults and 2000mg thrice daily <strong>for</strong> children.<br />
One dose of factor concentrates 10units/kg to be administered be<strong>for</strong>e a major procedure and repeated if<br />
necessary.<br />
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Anaesthesia and pain management.<br />
<strong>Dental</strong> pain can usually be controlled with a minor analgesic such as paracetamol (acetaminophen).<br />
Aspirin should not be used due to its inhibitory affect on platelet aggregation. The use of any non-steroidal anti-<br />
inflationary drug (NSAID) must be discussed be<strong>for</strong>e<strong>hand</strong> with the patientís hematologist because of their effect<br />
on platelet aggregation.<br />
There are no restrictions regarding the type of local anesthetic agent used although those with<br />
vasoconstrictors may provide additional local hemostasis. It is important to advise patients and parents of<br />
children about the risks of local oral trauma be<strong>for</strong>e the anesthetic wears off.<br />
A buccal infiltration can be used without any factor replacement. It will anesthetize all the upper teeth<br />
and lower anterior and premolar teeth.<br />
The mandibular molar teeth are usually treated using the inferior alveolar nerve block. This should only<br />
be given after raising clotting factor levels by appropriate replacement therapy, as there is a risk of bleeding into<br />
the muscles along with potential airway compromise due to a hematoma in the retromolar or pterygoid<br />
technique should be considered instead of the mandibular block. Articaine has been used as a buccal infiltration<br />
to anesthetize the lower molar teeth. A lingual infiltration also requires appropriate factor replacement since the<br />
injection is into an area with a rich plexus of blood vessels and the needle is not adjacent to bone. There is a risk<br />
of a significant airway obstruction in the event of bleed.<br />
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Surgery<br />
Surgical treatment, including a simple <strong>dental</strong> extraction, must be planned to minimize the risk of<br />
bleeding, excessive bruising, or hematoma <strong>for</strong>mation. The following points will help prevent problems.<br />
Emergency surgical intervention in dentistry is rarely required as pain can often be con trolled without<br />
resorting to an unplanned treatment. All treatment plans must be discussed with the hemophilia unit if they<br />
involve the use of prophylactic cover.<br />
1. Treatment plan<br />
The treatment plan should be <strong>for</strong>mulated using the following guidelines.<br />
* Conduct a thorough clinical and radiographic examination.<br />
* Identify which treatment may require prophylactic cover. If multiple extractions are required, only one<br />
or two teeth should be extracted at the first appointment to ensure that hemostsasis can be achieved. It is<br />
important to remember the patientís social circumstances (Do they live alone? What are their<br />
preferences <strong>for</strong> treatment) as well as the clinical condition when making this decision.<br />
* Observe all patients <strong>for</strong> a prolonged period after a <strong>dental</strong> extration. this may be <strong>for</strong> a few hours <strong>for</strong> those<br />
patients with a mild bledding tendency whilst those with more severe conditions or a history of<br />
prolonged bleeding despite hemostatic cover may require supervision overnight in hospital.<br />
* Discuss treatment requiring the administration of coagulation factor or desmopressin (DDAVP) with the<br />
hemophilia unit. They will be responsible <strong>for</strong> arranging the administration and monitoring of treatment<br />
products.<br />
* Discuss the use of local hemostatic agents. This could include the use of oxidized cellulose(Surgicel) or<br />
fibrin glue. Fibrin glue should not normally be used in patients who have never received human -derived<br />
blood products or those who are receiving treatment with recombinant factor VIII of IX because of the<br />
potential risks of human viral transmission.<br />
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* Consider whether to use antibiotics following a <strong>dental</strong> extraction. This is contraverseal, but there are a<br />
number of anecdotal reports suggesting that their use may prevent a late bleed, which is thought to be<br />
due to infection be<strong>for</strong>e treatment , it should be treated with antibiotics.<br />
* Always carry out treatment as atraumatically as possible.<br />
2. Pre-operative period<br />
* Ensure that the oral cavity is as healthy as possible be<strong>for</strong>e any surgical procedure. This can be achieved<br />
by arranging treatment with hygienist to remove as much calculus and plaque as possible. The regular<br />
use of an antibacterial mouthwash, <strong>for</strong> example chlorhexidine, may also help.<br />
* Consider using an antifibrinoytic agent. It may be helpful to start the treatment the day be<strong>for</strong>e the<br />
surgery. Tranexamic acid(usual adult dose 1g three times a day) and epsilon aminocaproic<br />
acid(EACA)(50 mg/kg four times a day), are the most commonly used drugs. They should be continued<br />
<strong>for</strong> a total of 7 days.<br />
3. Peri-operative period<br />
* Have the patient rinse with chlorexidine mouthwash <strong>for</strong> 2 minutes be<strong>for</strong>e the administration of the local<br />
anesthetic.<br />
* Carry out the extraction out as atraumatically as possible.,<br />
* Suture the socket if the gingival margins do not oppose well. Brewer reports a small series where sutures<br />
were not used routinely and there was no significant increase in post extraction hemorrhage Resorbable<br />
and non-resorbable sutures may be used at the operators discretion. The only problem with non-<br />
resobable sutures is the need <strong>for</strong> a post operative visit and the possibility of bleeding when the<br />
suture is removed. Use local hemostatic measures if indicated. These include the use of oxidized<br />
cellulose or fibrin glue(see notes on the use of fibrin glue) Use a soft vaccum <strong>for</strong>med splint to protect<br />
the socket if needed.<br />
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4. Post -operative Period<br />
The patient must be given detailed postoperative instructions:<br />
No mouth rising <strong>for</strong> 24 hours<br />
No smoking <strong>for</strong> 24 hours<br />
Soft diet <strong>for</strong> 24 hours<br />
No strenuous activities <strong>for</strong> 24 hours<br />
Prescribed medication must be taken as instructed<br />
Analgesia should be prescribed <strong>for</strong> use if required.<br />
Salt water mouth washes (1 teaspoon of salt in as glass of warm water) should be used four times a day starting<br />
the day after the extraction <strong>for</strong> 7 days<br />
Antibacterial mouthwash may be used;<br />
Emergency contact details must be be given to the patient in case of problems.<br />
Post-extraction hemorrhage<br />
Careful pre-operative planning and the use of antifibrinolytic agents will prevent many post operative problems.<br />
However post extraction bleeding will occur on occasion. If post extraction hemorrhage occurs.<br />
Contract the hemophilia unit and consider using additional factor concentrate. Inspect the site of the bleed. If<br />
there is any evidence of a tear in the gingiva or other obvious bleeding point this should be treated using local<br />
measures as previously described.<br />
Instruct the patient top sit up and bite on a damp gauze swab <strong>for</strong> at least 10 minutes.<br />
Use a 10% solution of tranexamic acid or EACA to dampen the swab or as a mouthwash if the bleeding is<br />
difficult to stop.<br />
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Monitor the patientís blood pressure as it may increase due to worry and pain. If the patient has pain, a<br />
suitable analgestic should be prescribed whilst if there is no pain a small dose of a benzodiazepine or similar<br />
will help to reduce the worry and reduce the blood pressure.<br />
Fibrin glue<br />
In some hemophilia centres, fibrin glue is used as a local hemostatic measure, along with an oral<br />
antifibrinolytic agent, to achieve hemostasis and reduce the need <strong>for</strong> clotting factor replacement therapy. All<br />
fibrin glue contains human or animal components, which has made a number of physicians and patients being<br />
hesitant to use this treatment articularly <strong>for</strong> patients who are receiving recombinant factor concentrates or have<br />
never received blood products derived from humans. Fibrin glue mimics the final pathway of coagulation<br />
cascade at the point where fibrinogen is converted into fibrin in the presence of thrombin, factor XIII,<br />
fibronection, and ionized calcium. the cascade reaction provides the cleavage of fibrinogen through thrombin,<br />
<strong>for</strong>ming fibrin peptides A and B from each molecule of fibrinogen resulting in the <strong>for</strong>mation of the fibrin<br />
monomers. thrombin itself also activates factor XIII, which in the presence of calcium, permits the stabilization<br />
of the clot. fibronectin takes part in the process as well and its inclusion in the adhesive system appears to<br />
promote cellular migration and then activation of fibroblasts in the area where the fibrin glue was applied.<br />
Splints<br />
Soft vacuum-<strong>for</strong>med splints can be used to provide local protection following a <strong>dental</strong> extraction or<br />
prolonged post-extraction bleed. the following techniques are used to construct the splint pre-operatively:<br />
* Take a <strong>dental</strong> impression be<strong>for</strong>e the extraction and cast a model in the laboratory.<br />
* Remove the tooth being extracted from the model.<br />
* Construct a soft vacuum-<strong>for</strong>med splint to cover the socket completely.<br />
* Keep the splint in place <strong>for</strong> at least 48 hours be<strong>for</strong>e checking the socket. If there is any sign of bleeding<br />
it should be replaced and checked every 24 hours.<br />
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HEMOPHILIA AND CHILDREN (PEDODONTICS)<br />
Several inborn abnormalities of metabolism, which manifest themselves as hemostasis disorders, have<br />
been given the collective name hemophilia. these disorders may result from lack of any one of the substances<br />
necessary <strong>for</strong> normal thromboplastic activity.<br />
The most common hemophilic condition is the disorder caused by dificiency of factor VIII<br />
(antihemophilic globulin). This condition is transmitted as an X-linked mendelian recessive and occurs<br />
exclusively in males. Although children born with factor VIII deficiency are potential ìbleedersî from birth,<br />
excessive bleeding may not be observed until increased activity begins, at about 6 months of age. Bleeding may<br />
occur from any site, but is most common in the muscles, kidneys, mouth, and joints. The knee joints of the<br />
infant who is beginning to walk are especially vulnerable.<br />
DENTAL PROBLEMS<br />
Although the hemophiliac has no characteristic <strong>dental</strong> problems, any <strong>dental</strong> treatment these children<br />
require must be considered as serious because of the grave risks involved. The necessity <strong>for</strong> good oral care and<br />
prevention of <strong>dental</strong> disease <strong>for</strong> the hemophiliac cannot be overemphasized, since preventive dentistry <strong>for</strong> these<br />
children minimizes the need <strong>for</strong> the hazards of restorative treatment.<br />
<strong>Dental</strong> Treatment<br />
Even during the most routine <strong>dental</strong> treatment <strong>for</strong> the hemophilic child the dentist must exercise extreme<br />
caution to prevent tissue lacerations. Scaling and polishing, reduction of subgingival tooth structure, and<br />
adaptation of matrix bands must be managed with great care to maintain the integrity of the periodontal tissues.<br />
Should minor bleeding problems occur, they can usually be controlled by pressure packs in conjunction with<br />
hemostatic agents such as thrombin.<br />
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The use of local anesthesia is contraindicated <strong>for</strong> these children except when pain is extreme and then it should<br />
be used with caution. The mandibular block should be avoided, because this <strong>for</strong>m of injection may cause<br />
hemorrhage into the lateral pharyngel spaces, where it is difficult to apply controlling measures.<br />
A carefully placed rubber dam will serve a twofold purpose in the <strong>dental</strong> treatment of the hemophilic.<br />
Besides securing a dry field <strong>for</strong> the placement of restorative materials, it also will help protect the soft tissues<br />
from acci<strong>dental</strong> lacerations.<br />
Tooth extraction <strong>for</strong> the hemophiliac should be considered only as a last resort, after the possibility of<br />
maintaining the teeth by conventions pulpectomy or root canal therapy has been rejected.<br />
Extreme precautionary measure should be practiced <strong>for</strong> all patients suffering from any type of<br />
hemorrhagic disorder, as <strong>for</strong> example with the so-called pseudo hemophiliac, who may be a male or a female<br />
with a characteristically prolonged bleeding time.<br />
Removing Teeth<br />
When extractions or other surgical procedures are unavoidable and excessive bleeding is anticipated, the<br />
patient should be hospitalized <strong>for</strong> more adequate <strong>hand</strong>ling of any complications that might arise.<br />
Prior to the patientís admission to the hospital, the dentist should organize a plan <strong>for</strong> <strong>dental</strong> treatment.<br />
This plan, in conjunction with the patientís medical history, should be discussed by the dentist, the physician,<br />
and the hospital staff to outline a medical work-up that will include a detailed blood analysis <strong>for</strong> determining the<br />
type of bleeding problem, the coagulation and bleeding time, and the platelet count. After these steps are<br />
completed, the hematologist should coordinate the medical management <strong>for</strong> the child.<br />
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Premedication of the hemophiliac is indicated when lengthy procedures are planned or when the patient is<br />
apprehensive or unruly. Preoperative plasma transfusion may be recommended if excessive bleeding is<br />
anticipated.<br />
As mentioned earlier, local anesthesia is usually contra indicated <strong>for</strong> a child with hemophilia. A general,<br />
anesthetic, administered by a trained anesthesiologist, who is cognizant of the bleeding problem, should be a<br />
relatively safe procedure <strong>for</strong> the hemophiliac patient.<br />
Be<strong>for</strong>e surgical treatment is attempted, all necessary restorative procedures should be completed with<br />
strict adherence to the precautionary measures already described. The extractions and other surgical treatment<br />
must be per<strong>for</strong>med with the least possible trauma. Use of pressure packs <strong>for</strong> the initial control of hemorrhage<br />
should be followed by packing the socket with Gelofoam or some other hemostatic agent that can be held in<br />
nplace by gauze impregnated with petrolatum to prevent the incorporation of the gauze in the blood clot.<br />
A follow-up transfusion schedule should be planned by the hematologist and the dentist. The duration<br />
of this therapy depends on the approximation of the healing time <strong>for</strong> the surgical wounds. the patient should<br />
receive nothing by mouth <strong>for</strong> 4 hours after the operation and should receive only liquids <strong>for</strong> the subsequent 24<br />
hours. The important of then patientís diet and postoperative home care must be carefully explained to his<br />
parents.<br />
NITROUS OXIDE ANALGESIA<br />
With the <strong>hand</strong>icapped child, inhalation analgesia with nitrous oxide can be a safe and effective method<br />
of decreasing apprehension or resistance to <strong>dental</strong> treatment. Except <strong>for</strong> children who have severe mental<br />
retardation or emotional disturbance, there are few contraindications to its use.<br />
A dentist experienced in administering nitrous oxide analgesia can combine this procedure with<br />
premedication and local anesthesia to overcome many of the problems associated with <strong>hand</strong>icapped children.<br />
Nitrous oxide analgesia decreases the muscular spasticity and the uncoordinated movements of the cerebral<br />
palsied child and lessens the physical stress and discom<strong>for</strong>t, thereby making it possible <strong>for</strong> him to tolerate<br />
longer periods of treatment.<br />
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Nitrous oxide analgesia <strong>for</strong> the <strong>hand</strong>icapped child should be limited to the stage of relative analgesia, by the use<br />
of relatively low nitrous oxide and high oxygen flows, safely below the excitement level. The major purpose of<br />
a relative analgesia level is torelance the patient and increase his cooperation. During the most painful periods<br />
of the <strong>dental</strong> treatment, this analgeis level can be supplemented by the use of local anesthesia when the patientís<br />
condition permits.<br />
Careful consideration and management of the introduction of nitrous oxide analgesia and its initial<br />
administration to the <strong>hand</strong>icapped child are the governing factors <strong>for</strong> a successful utilization of the procedure.<br />
For most <strong>hand</strong>icapped children, premedication is needed to allay the apprehension that frequently accompanies<br />
their first experience with analgesia. The attending physician should be consulted regarding the choice of durg<br />
therapy <strong>for</strong> each patient.<br />
Particular patience and understanding is imperative at the initial administration of<br />
nitrous oxide analgesia. The child must have time to adjust to this new experience, the use of the nosepiece<br />
should be demonstrated and the flow of gases can be played on the childís <strong>hand</strong>s and cheeks be<strong>for</strong>e placement<br />
of the nosepiece. Resistence may be countered with gentle physical restraint and flow of 50 percent nitrous<br />
oxide directed close to the nostrils. This mixture may have a mild euphoric effect and relax the patient<br />
sufficiently to permit seating of the nosepiece, after which the concentration of nitrous oxide should be reduced<br />
to the appropriate level, usually a 10 to 15 percent flow.<br />
Since verbal communication with the <strong>hand</strong>icapped child is frequently difficult and unreliable, the dentist<br />
must be able to evaluate the level of analgesia from the physical and behavioural changes in the patient. When<br />
the appropriate level of analgesia has been reached. The dentist can begin the treatment. Operative procedures<br />
in these cases vary little from those routinely employed. the rubber dam and a mouth prop are helpful; however,<br />
it is important to remember that the use of the rubber dam lessens the dilution effect created when the mouth is<br />
open, thereby necessitating a decrease in the proportion of nitrous oxide.<br />
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GENERAL ANAESTHESIA<br />
Risks of vomiting, spasm, and apnea invariably exist in association with the use of general anesthetic;<br />
there<strong>for</strong>e, it is expedient that milder, though possibly less potent, measures be considered when an anesthetic is<br />
in order.<br />
A childís response, especially with the aid of premedication, is usually cooperative when the dentist exercise<br />
patience and understanding. This is true even with children who may appear incapable of the mental and<br />
physical control needed <strong>for</strong> successful <strong>dental</strong> treatment. Should these patient management procedures fail, or if<br />
a severely <strong>hand</strong>icapped child needs extensive <strong>dental</strong> care, the use of general anesthesia offers a possible solution<br />
<strong>for</strong> the problem.<br />
Indications <strong>for</strong> General Anesthesia<br />
Children in one of the following categories usually require general anesthesia:<br />
1. Then uncooperative child who resists treatment after all conventional management procedures<br />
have been tried.<br />
2. The child with a hemostasis disorder who needs extensive <strong>dental</strong> service.<br />
3. The mentally retarded child so severaly <strong>hand</strong>icapped that dentist-patient communication is impossible.<br />
4. The child suffereing from central nervous disorders manifested by extreme involuntary movements.<br />
5. The child with severe congenital heart disease who is considered incapable of tolerating the excitement<br />
and fatigue of extensive <strong>dental</strong> service.<br />
Preoperative Procedures<br />
Although the utilization of a general anesthetic in the <strong>dental</strong> office is acceptable, provided the essential<br />
equipment and a qualified anesthesiologist are available, the safest place <strong>for</strong> <strong>dental</strong> treatment under general<br />
anesthesia is undoubtedly the hospital.<br />
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When a childís <strong>dental</strong> treatment requires hospitalization and a general anesthetic, the best way to insure<br />
cooperation from the parents is to orient them as to the programme planned <strong>for</strong> their child and their<br />
responsibilities concerning his hospitalization.<br />
Since considerable paper work is associated with any hospitalization, the dentist should familiarize<br />
himself with accepted hospital protocol be<strong>for</strong>e a definite admission date is arranged <strong>for</strong> his patient.<br />
Treatment Procedures<br />
After the patientís hospital admission, the dentist should discuss plans <strong>for</strong> the treatment with the<br />
anesthesiologist, who determines the tolerance limit of each patient <strong>for</strong> the general anesthetic, with this as a<br />
guideline, the dentist can then set up a rigid time schedule <strong>for</strong> treatment.<br />
After evaluating the patientís medical history and determining the extent of the required treatment, the<br />
most suitable premedication and anesthetic can then be chosen. Since it was first tried in pediatric anesthesia,<br />
some 15 years ago, gluothane (halothane) has gained wide acceptance because of its low irritant quality, no<br />
flammability, and the rapid awakening of a patient who has received it.<br />
Nasal endotracheal intubation, where the tube is out of his way, simplifies the treatment procedures <strong>for</strong><br />
the dentist. After intubation, the patientís eyes should be covered with damp gauze <strong>for</strong> protection against <strong>dental</strong><br />
and material debris. Great care must be taken to prevent blood or any type of debris from entering the patientís<br />
throat. After complete anesthetization a throat pack of moist gauze should be laid over the pharyngeal opening,<br />
across the tonsillar area, and under the tongue . For its easy, safe removal, the end of the throat pack, with a<br />
string securely attached to it, should extrude from the oral cavity.<br />
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Efficient use of aspiration equipment facilities <strong>dental</strong> procedures <strong>for</strong> the anesthetized child. A mouth prop may<br />
be used, when needed, to keep the childís mouth open. For any restorative treatment on the anesthetized patient,<br />
use of rubber dam provides the dentist with a dry field and better visibility, and serves as an adjunct to the throat<br />
pack in preventing debris from entering the patientís throat. Prior to extractions or other surgical treatment, all<br />
restorative procedures should be finished; with this done, the mouth should be cleaned, the throat pack replaced<br />
with fresh gauze, and the surgical procedures initiated.<br />
Upon completion of the planned treatment, any hemorrhage must be controlled and all debris carefully<br />
evacuated from the oral cavity; after this, the throat pack can be removed and the child taken to the recovery<br />
room.<br />
Be<strong>for</strong>e hospital dismissal, an appointment should be set up <strong>for</strong> a postoperative check on the patient<br />
within the following two weeks. On this visit, the parents must be impressed with the urgency <strong>for</strong> careful oral<br />
home care <strong>for</strong> their child and reducing his intake of cariogenic food as a means of avoiding the ordeal or<br />
repetitive <strong>dental</strong> treatment.<br />
Despite the fact that different blood components are involved en each of bleeding and clotting diseases,<br />
their clinical and oral manifestations are identical. They will there<strong>for</strong>e be described together as a single disease.<br />
Oral Manifestations. Hemorrhage from many sites in the oral cavity is a common finding in<br />
hemophilia, and gingival hemorrhage may be massive and prolonged. Even the physiologic process of tooth<br />
eruption and exfoliation may be attended with severe prolonged hemorrhage.<br />
The oral manifestations of the various <strong>for</strong>ms of hemophilia have been discussed by Spiegal and by Steg<br />
and his co-workers. In addition, mandibular ìpseudsotumorî of hemophilia has been reported by Stoineman and<br />
Beierl, a condition in which there is subperiosteal bleeding, with reactive new bone <strong>for</strong>mation causing tumor-<br />
like expansion of the bone.<br />
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The problem of <strong>dental</strong> extractions is a difficult one in hemophiliacs. Without proper premedication, even<br />
a minor surgical procedure may result in death from exsanguination. tooth extraction by means of rubber bands<br />
has often been used successfully, the rubber band being placed around the cervix of the tooth a nd allowed to<br />
migrate apically, causing exfoliation of the tooth through pressure necrosis of the periodontal ligament.<br />
Laboratory Findings. The characteristic defect of hemophilia is a prolonged coagulation time.<br />
the bleeding time is normal, as is the prothombin time and platelet agregation. In vitro, the deficiency of the<br />
clot-prompting factor in the plasma of hemophiliacs impairs clotting because it appears to retard development<br />
of the substance responsible <strong>for</strong> conversion of prothrombin to thrombin. Separation of the various <strong>for</strong>ms of<br />
hemophilia and proper diagnosis depends upon demonstration that the plasma of a patient with a known <strong>for</strong>m of<br />
hemophilia does not correct the plasma clotting defect in the patient under observation.<br />
Treatment and Prognosis. There is no known cure <strong>for</strong> hemophilia. the affected persons should be<br />
protected from traumatic injuries.<br />
If a surgical procedure such as tooth extraction must be carried out, the operation should be considered a<br />
major one and per<strong>for</strong>med only in a hospital.<br />
The greatest numbers of fatalities in hemophiliacs have resulted from surgical procedures, including<br />
tooth extraction. Preoperative transfusion of whole blood and the administration of antihemophilic factor<br />
concentrate are recommended. Nevertheless, oral surgery is a dangerous procedure and should be avoided<br />
whenever possible. Unfotunately, a small percentage of hemophiliacs have circulating anti-coagulant, probably<br />
an antibody, which specifically inactivates hemophilic factor, negating the effects of transfusion.<br />
The prognosis is variable, and many affected persons die during childhood.<br />
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DENTAL TREATMENT MODALITIES FOR HEMOPHILIC PATIENTS ñ SOME TIPS<br />
DENTAL PAIN MANAGEMENT<br />
<strong>Dental</strong> Pain can be managed depending on the patientís pain threshold and the type of treatment to be<br />
undertaken.<br />
Option of treatment without anaesthesia may also be considered.<br />
Sedation & hypnosis are methods to control the anxiety; thus eliminating the need of anaesthesia in<br />
some kind of treatment.<br />
Intravenous diazepam or Nitrous oxide analgesia are best options.<br />
NSAIDs are to be avoided.<br />
ANAESTHESIA<br />
Intra pulpal anaesthesia is safe and effective, after accessing and extirpating pulp.<br />
Periodontal ligament and papillary injections with minimal volume and speed are also advised.<br />
Anaesthesia with vasoconstrictors should be used when possible<br />
Injections<br />
Infiltration <strong>for</strong> maxillary teeth with slow speed may be done<br />
Local pressure should be applied to the injection site <strong>for</strong> 3-4 minutes.<br />
If haematoma develops ice packs should be applied and emergency factor replacement therapy should<br />
be administered in a hospital.<br />
Block injections like inferior alveolar posterior Superior alveolar, infra orbital and even long buccal<br />
place the anaesthetic solutions to highly vascularised, loose connective tissues with no boundaries.<br />
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where <strong>for</strong>mation of hematoma is a great problem. So it is advised to avoid block injections in these<br />
patients.<br />
Intra muscular injections should also be avoided because of chances of haematoma <strong>for</strong>mation<br />
ORAL SURGERY<br />
Mandibular block injections are absolutely Contra indicated due to persistent haemorrhage into para<br />
pharyngal tissues.<br />
Local infiltration causes bleeding at each point<br />
Safe site of local infiltration is down the periodontal membrane.<br />
Haemorrhage plate may be given to protect the blood clot from trauma of food and tongue.<br />
Atraumatic extraction should be done<br />
Sides of Sockets should be gently squeezed.<br />
It is better to avoid Suturing because of needle wound as well as blood may be directed to the facial<br />
planes of neck.<br />
Antifibrinolytics like EACA (Epsilon amino Caproic acid) and tranexamic acid may be used <strong>for</strong><br />
stabilization of clot.<br />
Tranexamic acid is more potent and longer acting (IV and oral 500mg tab dissolved in, oral of water)<br />
Avoid aspirin like drugs<br />
Paracetamol or dihydrocodeine may be effective.<br />
NSAIDs are to be avoided.<br />
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Prosthodontic treatment<br />
Generally excessive muco compressive method may be avoided to avoid unnecessary bleeding.<br />
Fixed prosthesis is preferred over removable prosthesis.<br />
Denture trauma caused by the denture must be minimized.<br />
Denture stomatitis (perleche) must be specially taken care of due to untoward haemorrhage<br />
Alveoloplasty and pre prosthetic surgeries should be done after hospitalization and factor replacement<br />
therapy.<br />
Conservative dentistry<br />
Conserving the teeth is of prime concern<br />
Minimize the risk of laceration of soft tissues in the operative field.<br />
Avoid creating ecchymoses and haematomas using high speed evacuators and saliva ejectors.<br />
Rubber dam isolation is advised to minimize soft tissue laceration. At the same time, clamp must be<br />
taken care of; not to traumatize gingival.<br />
Matrices, wedges and hemostatic gingival retraction cord can be used with caution.<br />
Restorations should not be the causative agents to accumulate food particles.<br />
Finishing, polishing & accessibility to cleaning by tooth brush must be property looked upon.<br />
Periodontal management<br />
Periodontal health is of critical importance because of hyperaemia causing gingival bleeding and<br />
periodontitis causing tooth morbidity leading to extraction of teeth.<br />
Most of the patients are afraid of tooth brush induced bleeding so plaque and calculus would be very<br />
common.<br />
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Periodontal probing, supragingival scaling with fine scalers and polishing must be done routinely to<br />
avoid chances of gingivitis and periodontitis which may cause severe bleeding.<br />
Careful subgingival scaling may be done with very fine scalers<br />
Deep subgingival scaling and root planning may be done quadrant wise to reduce gingival area<br />
exposed to potential bleeding.<br />
Prior to subgingival scaling, supra gingival scaling is mandatory.<br />
Local application of pressure should be done after scaling.<br />
Antifibrinolytic oral rinses are usually successful in preventing oozing of blood.<br />
Mucogingival surgery may need factor replacement therapy after hospitalization.<br />
Elevation of factor level may be achieved (up to 50%) in circulation after the use of antifibrinolytic<br />
mouth washes.<br />
Endodontic therapy<br />
It is the treatment of choice to a patient with severe hemophilia.<br />
It avoids the unnecessary bleeding caused by extraction of teeth to hemophiliacs.<br />
Instrumentation should not go beyond the apex of tooth.<br />
Filling of root beyond apical seal should be avoided.<br />
Application of vasoconstrictors like epinephrine at the apical area provides hemostasis.<br />
Intrapulpal anaesthesia is the best advised.<br />
Factor replacement therapy may be needed <strong>for</strong> endodontic surgical procedures just like any other oral<br />
surgical procedures.<br />
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Pedodontic management<br />
During the exfoliation of primary teeth, prolonged oozing of blood may be observed. Curettage of the<br />
area and pressure cold application may be sufficient to control the bleeding; Gauze pressure and<br />
seepage stops the bleeding within 12 hours (to a maximum).<br />
Pulpotomies may be per<strong>for</strong>med without excessive pulpal bleeding.<br />
Stainless steel crowns should be prepared to allow minimal removal of enamel and gingival area.<br />
Non invasive, prophylactic and Restorative treatments should be given.<br />
Orthodontic treatment<br />
Care must be taken to avoid laceration by orthodontic bands brackets and wires.<br />
Fixed orthodontic treatment is preferred over removable orthodontics.<br />
Shorter treatment durations and use of extraoral <strong>for</strong>ce decrease the potential <strong>for</strong> bleeding<br />
complications.<br />
Proper Oral hygiene instructions and review appointments are highly essential <strong>for</strong> a good result in this<br />
case.<br />
The use of extra oral <strong>for</strong>ce and short treatment may be advised.<br />
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GENERAL INSTRUCTIONS TO PERSONS WITH OTHER BLEEDING & CLOTTING DISORDERS<br />
Despite the fact that different blood components are involved en each of bleeding and clotting diseases,<br />
their clinical and oral manifestations are identical. They will there<strong>for</strong>e be described together as a single disease.<br />
* Brushing twice daily with a fluoride toothpaste.<br />
- 1,000-ppm fluoride toothpaste <strong>for</strong> children under 7 years of age.<br />
- 1,400-ppm fluoride toothpaste <strong>for</strong> people over 7 years of age.<br />
The use of fluoride toothpaste depends on the fluoride concentration in the water<br />
supply as well as the use of additional fluoride supplements. It should not be used if fluoride supplements are<br />
taken or if the water supply has a fluoride content of 1ppm or more.<br />
* The tooth brush should have medium texture bristles because hard bristles can cause abrasion of the<br />
teeth and soft bristles are inadequate to remove plaque.<br />
* Inter<strong>dental</strong> cleaning aids, such as floss, tape, and inter<strong>dental</strong> brushes, should be used to prevent the<br />
<strong>for</strong>mation of <strong>dental</strong> caries and periodontal disease.<br />
* Fluoride supplements may be used, but are not recommended if the water supply has a fluoride content<br />
of 1ppm or more.<br />
Supplements include:<br />
- Fluoride drops<br />
- Fluoride tablets<br />
- Topical application of fluoride using trays.<br />
- Fluoride mouthrinses which can be used on either a daily or a weekly basis.<br />
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* The consumption of foods and drinks with a high sugar or acid content should be limited to<br />
mealtimes. Three exposures per day is the recommended maximum. The aim is to ensure that the intake of<br />
food and drink does not cause the pH of the oral cavity to fall below the critical level of pH 5.5.<br />
* Artificial sweeteners can be used as an alternative to sugars in food and drinks.<br />
Examples are aspartame, sorbitol, acesulfamate, etc.<br />
* Regular <strong>dental</strong> visits, usually every 6 months, will help identify problems early,<br />
rein<strong>for</strong>ce prevention, and emphasize the importance of reducing, the intake of food and drink<br />
containing high levels of sugar or acid.<br />
It is essential to prevent acci<strong>dental</strong> damage to the oral mucosa when carrying out any procedure in the<br />
mouth. Injury can be avoided by: Careful use of saliva ejectors;<br />
Careful removal of impressions;<br />
Care in the placement of X-ray films,<br />
particularly in the sublingual region;<br />
Protection of soft tissues during restorative treatment by using a rubber dam or applying yellow soft paraffin<br />
(vaseline)<br />
Periodontal treatment<br />
Healthy periodontal tissue is essential to prevent bleeding and tooth loss. If oral hygiene is poor, treatment must<br />
start as soon as possible after the patient has had a <strong>dental</strong> examination and treatment plan <strong>for</strong>mulated to prevent<br />
additional damage to the periodontal tissues. In cases of severe periodontal disease, it may be necessary to carry<br />
out supragingival scaling initially along with oral hygiene education. Subgingival scaling can start as soon as<br />
the inflammation has decreased. The treatment may need to be carried out over several visits to prevent<br />
excessive blood loss. In addition, chlorhexidine gluconate mouthwash can be used to control periodontal<br />
problems. Antibiotics may be required to help reduce the initial inflammation.<br />
*<br />
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Blood loss of all kinds can be controlled locally with direct pressure or periodontal dressings with or without<br />
topical antifibrinolytic agents.<br />
Periodontal surgery in patients with bleeding disorders must always be regarded as a high risk procedure with a<br />
significant risk of blood loss. It should only be considered were conservative treatment has failed and oral<br />
hygiene is good. Periodontal surgery can be a greater challenge to hemostasis than a simple extraction. The<br />
procedure must be carefully planned and the risks fully explained to the patient.<br />
Removable prosthodontics.<br />
Patients with bleeding disorders can be given dentures as long as they are com<strong>for</strong>table. If a partial denture is<br />
provided it is important that the periodontal health of the remaining teeth is maintained.<br />
Orthodontic treatment<br />
Fixed and removable orthodontic appliances may be used along with regular preventive advice and hygiene<br />
therapy. Special care should be taken when treating patients with a severe bleeding disorder to ensure that the<br />
gingiva is not damaged when fitting the appliance.<br />
Restorative procedures<br />
Restorative treatment can be undertaken routinely providing care is taken to protect the mucosa. There is a risk<br />
of bleeding with the use of matrix bands or wooden edges. This can be controlled by local means or the<br />
application of topical agents.<br />
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Endodontics<br />
Endodontic treatment is generally low risk <strong>for</strong> patients with bleeding disorders. If a pulpectomy is indicated, the<br />
possibility of the tooth requiring conventional endodontic treatment must also be considered. It is important that<br />
the procedure be carried out carefully with the working length of the root canal calculated to ensure that the<br />
instruments do not pass through the apex of the root canal. The presence of bleeding in the canal is indicative of<br />
pulp tissue remaining in the canal. Sodium hypochlorite should be used <strong>for</strong> irrigation in all cases, followed by<br />
the use of clacium hydroxide paste to control the bleeding. Formaldehyde-derived substances may also be used<br />
in cases where there is persistent bleeding or even be<strong>for</strong>e the pulpectomy.<br />
COMMUNITY DENTISTRY AND HEMOPHILIA<br />
All patients with hemophilia should be assessed <strong>for</strong> treatment needs, not only in the procedure that<br />
should be undertaken, but also in the time it would take to carry out such procedure, so that if an opportunity<br />
developers at some time in the future, they could be called up at short notice and have their <strong>dental</strong> needs<br />
attended to. This identification of persons with hemophilia would not only allow their <strong>dental</strong> treatment needs to<br />
be unidentified, but also allows this group of patients and their families to be specially targeted with the<br />
preventive <strong>dental</strong> care message to reduce the progression of existing disease and diminish future <strong>dental</strong><br />
treatment needs.<br />
Patients with hemophilia are different from ordinary patients in that the latter group can have multiple<br />
visits to achieve a definitive treatment even on one tooth. this is luxury that it is not usually available to persons<br />
with hemophilia: there<strong>for</strong>e, a much more restrictive view should be taken assessment of the necessity of<br />
treatment must be taken, as there may be only one opportunity <strong>for</strong> <strong>dental</strong> intervention to take place. So by<br />
definition, the options available to the dentists responsible <strong>for</strong> making the decisions <strong>for</strong> treatment of persons<br />
with hemophilia are much more restricted. There are multiple causes <strong>for</strong> this including costs, availability of<br />
service, and availability of factor replacement post-operative care.<br />
In relation to infiltration anesthesia used mainly in the upper jaw, a 30% factor rise is a normal requirement.<br />
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Psychosocial Aspects Of Hemophilia Care<br />
A person with hemophilia and his family need psychological social support in coping with an<br />
illness that is chronic, often painful and sometimes life threatening. It is financially a burden and places<br />
restriction on several aspects of normal living. Psychological Support is an essential ingredient of any<br />
successful medical management<br />
Most effective when given by the primary physician.<br />
Simple to implement when essential techniques are applied.<br />
General Principles<br />
Provide privacy.<br />
Seat patient com<strong>for</strong>tably at eye level.<br />
Make eye contact<br />
Use open ñended questions, eg. ìHow are you feelingî rather that :how is the pain today?î<br />
This allows the person to express any problem being experienced rather than restricting him<br />
to only pain.<br />
Use simple languages; avoid medical jargon.<br />
Use body languages to advantage-leading <strong>for</strong>ward, nodding, maintaining eye contact,<br />
avoiding making full notes while subject is speaking-are all powerful signals which convey<br />
empathy and interest.<br />
Use silences effectively. It is useful to pause statement that are likely to cause distress, eg.<br />
disclosure of diagnosis. Continue to display a listening posture. Important concerns and fear<br />
will be elicited.<br />
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SUPPORT FROM A SOCIAL WORKER<br />
The role of a social worker in helping in the management of chronic illness is being<br />
increasingly recognized. The social worker coordinates ef<strong>for</strong>ts of different services at a hemophilia<br />
center to individualize it to the patientís specific needs. Family visits help in providing psychological<br />
support, aid in assessment of problems faced in day-to-day living and community of care. In developing<br />
countries the concept of a co-ordinated team based care remains in the future. However, local groups have<br />
developed networks <strong>for</strong> small areas that provide similar care.<br />
PREVENTION OF HEMOPHILIA<br />
For disease like hemophilia, where cure is not yet possible, prevention is crucial.<br />
This is now possible with>99% accuracy using DNA analysis<br />
It is best done by obtaining DNA from the foetus around the end of the 10 th week of pregnancy<br />
by chorionic villous sampling or the 16-18 weeks by amniocentesis.<br />
If the family has been evaluated be<strong>for</strong>e, then results should be available within a week otherwise<br />
it may take 1-2 weeks<br />
Prenatal diagnosis should be only be attempted after appriopriate counseling of the family<br />
and ascertaining that the couple desires termination of pregnancy,If the foetus is affected.<br />
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Reference Centres<br />
At present comprehensive care facilities <strong>for</strong> persons with hemophilia (including genetic diagnosis) are available<br />
at-<br />
Christian medical college & Hospital<br />
Vellore 663-004<br />
Tel: 0416-2223603/2225506/22 (Ext<br />
2352/2353) Fax: 0416-<br />
2232035/223205<br />
HEMOPHILIA SOCIETY OF INDIA<br />
Hemophilia Society - Angamaly<br />
Mr. Paul Nettikkadan<br />
Vappalassery P.O. Via Angamaly-683572<br />
(Kerala)<br />
Tel: 0484-2452546 Extn: 413 (Hosp)<br />
Tel: 0484-2452410<br />
Email: angmaly@hemophilia.in<br />
K.E.M. Hospital<br />
Mumbai 400 00<br />
Tel:022-24131763/24136051<br />
(Ext:2358/2142)<br />
Fax: 022-24138521<br />
Hemophilia Society - Calicut<br />
Dr. C.P. Aboobacker<br />
Near AWH Medicals, Annie Hall Road Calicut-<br />
673002 (Kerala)<br />
Tel: 0495-2300284<br />
Mob: +91-94476 38294 (Hameed)<br />
Email: calicut@hemophilia.in<br />
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Hemophilia Society - kunnamkulam<br />
Mr. E. Raghunandan<br />
Smrithy, Akkikavu Karikkad, Kunnamkulam-<br />
680519, (Kerala)<br />
Tel: 04885-226639, 9847622366<br />
(Raghunandan)<br />
Mob: +91-9447236361<br />
Email: kunnamkulam@hemophilia.in<br />
Hemophilia Society - Cochin<br />
Shyamala Ramaswami<br />
XX/142 D, Thripthi Apartments<br />
Flat No-A ñ 2nd Floor, Near Agasthya Medical<br />
Centre, Old Bus Stand<br />
Thripunithura, Cochin-682301, (Kerala)<br />
Tel: 0484- 2774083<br />
Email: cochin@hemophilia.in<br />
Hemophilia Society - Trivandrum<br />
Mr. Vishnu M. S.<br />
T.C 29/1124(74/1977 New) House No-4,<br />
Devinagar, Palkulangara,<br />
Petah P.O, Trivandrum- 695024 (Kerala)<br />
Tel: 0471- 5520911<br />
Mob: +91-9895095518<br />
Email: trivandrum@hemophilia.in<br />
Hemophilia Society - Vellore<br />
President: Dr Ikram<br />
Secretary: Mr Kannan<br />
KGN Hospital No.94, Arcot Road, Vellore ñ<br />
632004, (Tamil Nadu)<br />
Tel: +91-98430 25146, 0416- 2225146<br />
Email: vellore@hemophilia.in<br />
Hemophilia Society - Mumbai<br />
Mr. Balshiram Gadhave<br />
Flat No. 205, 2nd Floor, Sai Apartment, 64,<br />
B, Swarajya Bhavan,<br />
Bhagwan Rao Parelkar Marg, Parel Village,<br />
MUMBAI - 400 012 (Maharashtra)<br />
Tel: 022-24158315, 24184071<br />
Email: mumbai@hemophilia.in<br />
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References<br />
1. White GC 2nd, Rosendaal F, Aledort LM, Lusher JM, Rothschild C, Ingerslev J. Factor VIII and Factor<br />
IX Subcommittee. Definitions in hemophilia, Recommendation of the scientific subcommittee on factor<br />
VIII and factor IX of the scientific and standardization committee of the International Society on<br />
Thrombosis and Haemostasis. Thomb Haemost, 2001;85(3):560.<br />
2. Nilsson IM. Hemophilia. Stockholm: Pharmacia Plasma Products: 1994.<br />
3. Fried MW: Management of hepatitis C in the hemophilia patient. Am J Med<br />
1999;107(6B):85S-89S.<br />
4. Kumar A, Kulkami R, Murray DL, et al. Serologic markers of viral hepatitis A, B, C and D in patients<br />
with hemophilia. J Med Virol 1993; 41:205-9.<br />
5. Eyster ME, Diamondstone LS, Lien JM, et al. Natural history of hepatitis C virus<br />
infection in multitransfused hemophiliacs: effect of coinfection with human immune deficiency virus.<br />
The Multicenter Hemophilia Cohort Study. J Acquir Immune Defic Syndr Hum Retrovirol 1993; 6:602-<br />
10.<br />
6. Hyper links : www.wfh.org/, http://www.hemophilia.in, coolhealthtips.com, i<strong>dental</strong>hub.com<br />
7. Text Book of oral Pathology ñ Shafer, Text Book of Oral Medicine ñ Burkitt, Text Book Of Oral<br />
Surgery- Killey and Kay, Text <strong>book</strong> of Pedodontics ñ Finn, Hemophilia and Dentistry ñ Cunning Ham.<br />
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GLOSSARY<br />
Anemia - A condition in which the blood is either deficient in red cells, in hemoglobin, or in total volume.<br />
Antifibrinolytic - Drugs that can help stop the normal breakdown of blood clots and help speed recovery from a<br />
bleed.<br />
Antihistamine - A drug that can reduce or prevent allergic reactions.<br />
Anti-inflammatory - A drug that can reduce or prevent pain, redness, and swelling caused by inflammation (the<br />
body's reaction to irritation, injury, or infection).<br />
Artery - A large tube or blood vessel that carries blood from the heart through the body. The body has several<br />
arteries. AIDS (Acquired Immune Deficiency Syndrome) - A disease that attacks and destroys the bodyís<br />
immune system, leaving the patient abnormally vulnerable to infections and many other diseases.<br />
Bleeding Disorders - A group of distinct conditions in which a personís body cannot properly develop a clot,<br />
causing an increased tendency <strong>for</strong> bleeding.<br />
Calf - The area of the leg between the knee and the ankle.<br />
Capillary - Any of the very small tubes or blood vessels that <strong>for</strong>m a network to carry blood through the body.<br />
The body has many capillaries<br />
Carrier - person who carries a gene that causes a disorder, usually showing no symptoms.<br />
Cartilage - The smooth surface covering the ends of the bones in a joint.<br />
Cell - The smallest independent living thing on earth. The human body is made of trillions of tiny cells.<br />
Chromosome - A very fine, threadlike strand of proteins and DNA in the centre of human, animal, and plant<br />
cells. Two<br />
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chromosomes (called X and Y) decide a person's sex. Females are born with two X-chromosomes (XX = girl).<br />
Males<br />
are born with one X and one Y chromosome (XY = boy).<br />
Clot - A thick lump of blood <strong>for</strong>med by clotting factors that work together to help stop bleeding.<br />
Cryoprecipitate - A treatment product made from blood plasma. It contains proteins, such as factor VIII (eight)<br />
and von Willebrand factor, but not factor IX (nine). It is infused into a vein over a period of time. First<br />
discovered<br />
by Dr. Judith Graham Poole in 1965.<br />
Asymptomatic carrier - A carrier who shows no outward signs of a condition.<br />
Symptomatic carrier - A carrier who has low factor levels and manifests bleeding symptoms.<br />
Coagulation disorders - a large group of conditions in which a person experiences excessive bleeding or<br />
clotting.<br />
Clotting factors - These are important proteins needed to <strong>for</strong>m blood clots.<br />
Desmopressin Acetate (also DDAVP or StimateÆ) - A synthetic hormone used to treat some people with mild<br />
hemophilia or von Willebrand disease. The product increases the factor VIII levels or von Willebrand factor<br />
levels in blood.<br />
Diluent - The liquid that is mixed with factor concentrate powder. The final product is injected to treat bleeds.<br />
Factor Assay - A specialized lab test used to determine the level of circulating factor VIII or IX in a personís<br />
body. The test results are reported as a percentage of normal levels.<br />
Factor deficiencies - These are rare disorders identified by the particular deficient or missing clotting factor in a<br />
personís body. These conditions include: Factor I, II, V, VII, VIII, IX, X, XI, XII, and XIII.<br />
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Factor concentrate - A type of treatment that replaces the missing factor VIII (eight) or IX (nine) by injection<br />
into a vein. Factor can be made from human blood plasma and then dried to a powder. This is dissolved in<br />
diluents be<strong>for</strong>e injection. See recombinant <strong>for</strong> description of another kind of factor concentrate.<br />
Gene - A section of DNA, the chemical code of the body that controls production of a protein. Genes carry<br />
messages about the way the cells of the body work. For example, they determine a person's hair and eye colour.<br />
Hemophilia is passed on through a person's genes.<br />
Gene Therapy - A method of replacing, manipulating or supplementing a dysfunctional gene with a functioning<br />
one. This evolving technique is currently being researched in several inherited diseases, including hemophilia.<br />
There is hope that gene therapy will lead to better treatments, and eventually cures.<br />
Hemoglobin - The protein that carries oxygen and carbon dioxide in red blood cells.<br />
Hematologist - A physician who specializes in disorders of the blood.<br />
Hemostasis - The process by which the body stops bleeding. It is the stoppage of blood flow through a blood<br />
vessel or an organ of the body.<br />
Hepatitis - A group of viruses that can lead to infection and inflammation of the liver.<br />
Hereditary disease - A condition that is genetically passed down to oneís offspring.<br />
HIV (Human Immunodeficiency Virus) - The virus that causes AIDS.<br />
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Hemophilic arthritis - Inflammation of a joint, usually with pain and swelling, due to repeated bleeds. Cartilage<br />
in the joint breaks down and some bone wears away. Sometimes the joint cannot move.<br />
Home Therapy - Injection or infusion with a treatment product away from the hospital, usually at home.<br />
Infusion - Injection of a treatment product into a vein.<br />
Inheritance - The biological process of transmitting certain characteristics or conditions from parents to<br />
offspring.<br />
Inhibitor - An antibody that develops in direct response to infused clotting factor concentrates. As a result of<br />
this rare complication, standard treatment is temporarily made ineffective.<br />
Joint - The place where two bones meet.<br />
Joint capsule - The area or sleeve that holds the bones together in a joint (where two bones meet).<br />
Nerve - The sensitive, cordlike fibers that pass messages through the body, including pain.<br />
NSAIDS or non-steroidal anti-inflammatory drug - drug, such as ibuprofen, that reduces pain and fever but<br />
does not contain steroids.<br />
Orthopedic - A term having to do with the bones, the skeleton or associated structures.<br />
Plasma - Part of blood that contains fibrin and clotting factors.<br />
Platelets - The blood cells that make a plug to patch holes in arteries, veins, and capillaries.<br />
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Prophylaxis - A treatment regimen aimed at preventing bleeding episodes among people with hemophilia.<br />
Primary prophylaxis - A method of regularly scheduled treatments to prevent bleeding episodes be<strong>for</strong>e they<br />
occur. Today, the majority of people with severe hemophilia begin this regimen at a young age to prevent long-<br />
term damage to joints.<br />
Recombinant - A type of factor concentrate that is manufactured in a laboratory instead of being separated from<br />
human blood. Recombinant proteins are copies of certain kinds of proteins found in human blood plasma.<br />
Spontaneous bleeding - Bleeding that happens <strong>for</strong> no clear reason (not after an injury or surgery).<br />
Synovium - The lining of the joint capsule. It is made of special cells that make a slippery, oily fluid that helps<br />
the joint move easily.<br />
Spontaneous mutation - The development of a hereditary disease <strong>for</strong> which there is no family history.<br />
Target joint - A term <strong>for</strong> a particular joint that has experienced repeated bleeds or at least four bleeds into one<br />
joint within a six-month period.<br />
Thrombophilia - A categorical term <strong>for</strong> several distinct conditions where there is an increased tendency <strong>for</strong><br />
excessive clotting.<br />
Von Willebrand Disease - A bleeding disorder in which von Willebrand factor, a blood protein, is either<br />
missing or does not function properly. Von Willebrand disease is the most common bleeding disorder since<br />
both men and women can inherit it equally.<br />
Venepuncture - This can be done to take blood or to give an intravenous injection.<br />
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