CDHO Advisory Hemophilia, Von Willebrand Disease Or Other Bleeding Disorder, 2019-11-04
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CDHO Advisory | Hemophilia, VWD and O ther B leeding D isorder COLLEGE OF DENTAL HYGIENISTS OF ONTARIO ADVISORY ADVISORY TITLE Use of the dental hygiene interventions of scaling of teeth and root planing including curetting surrounding tissue, orthodontic and restorative practices, and other invasive interventions for persons1 with hemophilia, von Willebrand disease or other bleeding disorder. ADVISORY STATUS Cite as College of Dental Hygienists of Ontario, CDHO Advisory Hemophilia, von Willebrand Disease or Other Bleeding Disorder, 2019-11-04 INTERVENTIONS AND PRACTICES CONSIDERED Scaling of teeth and root planing including curetting surrounding tissue, orthodontic and restorative practices, and other invasive interventions (“the Procedures”). SCOPE DISEASE/CONDITION(S)/PROCEDURE(S) Hemophilia, von Willebrand disease or other bleeding disorder INTENDED USERS Advanced practice nurses Nurses Dental assistants Patients/clients Dental hygienists Pharmacists Dentists Physicians Denturists Public health departments Dieticians Regulatory bodies Health professional students ADVISORY OBJECTIVE(S) To guide dental hygienists at the point of care relative to the use of the Procedures for persons who have hemophilia, von Willebrand disease or other bleeding disorder, chiefly as follows. 1. Understanding the medical condition. 1 Persons includes young persons and children Page | 1 CDHO Advisory | Hemophilia, VWD and O ther B leeding D isorder 2. Sourcing medications information. 3. Taking the medical and medications history. 4. Identifying and contacting the most appropriate healthcare provider(s) for medical advice. 5. Understanding and taking appropriate precautions prior to and during the Procedures proposed. 6. Deciding when and when not to proceed with the Procedures proposed. 7. Dealing with adverse events arising during the Procedures. 8. Keeping records. 9. Advising the patient/client. TARGET POPULATION Child (2 to 12 years) Adolescent (13 to 18 years) Adult (19 to 44 years) Middle Age (45 to 64 years) Aged (65 to 79 years) Aged 80 and over Male Female Parents, guardians, and family caregivers of children, young persons and adults with hemophilia, von Willebrand disease or other bleeding disorder. MAJOR OUTCOMES CONSIDERED For persons who have hemophilia, von Willebrand disease or other bleeding disorder: to maximize health benefits and minimize adverse effects by promoting the performance of the Procedures at the right time with the appropriate precautions, and by discouraging the performance of the Procedures at the wrong time or in the absence of appropriate precautions. RECOMMENDATIONS UNDERSTANDING THE MEDICAL CONDITION Terminology used in this Advisory Resources consulted . Bleeding Disorders: World Federation of Hemophilia . Hemophilia: MedlinePlus . PAIN – The Fifth Vital Sign: Canadian Hemophilia Society . What is hemophilia: Canadian Hemophilia Society . What is Hemophilia?: National Heart, Lung, and Blood Institute Bleeding disorder, a general term for a wide range of conditions that 1. lead to impairment of blood clotting, which results in a. prolonged bleeding b. continuous bleeding Page | 2 CDHO Advisory | Hemophilia, VWD and O ther B leeding D isorder 2. are classified as a. coagulation factor deficiencies i. congenital 1. hemophilia a. hemophilia A b. hemophilia B 2. von Willebrand disease 3. other factor deficiencies (rare) ii. acquired 1. liver disease associated with a. drugs of abuse, such as alcohol or heroin (CDHO Advisory) b. viral hepatitis associated with illicit injection drug use (CDHO Advisory) c. vitamin K deficiency (CDHO Advisory) 2. medications, including those that aggravate bleeding b. systemic conditions that interfere with coagulation, such as i. renal failure and uremia (CDHO Advisory) ii. liver failure (CDHO Advisory) iii. bone marrow failure c. thrombocytopenia d. defects in clotting processes that involve fibrin. Other terminology is as follows. 1. Angiodysplasia, a condition of the colon in which enlarged and fragile blood vessels cause bleeding into and therefore from the gastrointestinal tract. 2. Clotting factors, proteins in the blood that a. act in sequence to stop bleeding by forming a clot b. result in bleeding disorders when deficient in i. factors VIII and factor IX, the most commonly deficient ii. factors I, II, V, VII, X, XI, or XIII, which are only rarely deficient. 3. Disseminated intravascular coagulation, in which certain proteins in the blood become abnormally active causing small blood clots in the liver, brain or kidney. 4. Ecchymosis a. the purple or black-and-blue area resulting from a bruise b. the escape of blood from blood vessels into the surrounding tissue to form a purple or black-and-blue spot on the skin. 5. Fibrin, a protein in the blood plasma that a. is essential for the coagulation of blood clotting b. is formed by many as 20 different clotting factors which act together with other chemicals. 6. Hemarthrosis, bleeding into a joint. 7. Hematoma, localized swelling filled with blood. 8. Hemophilia, a group of bleeding disorders in which clotting is abnormally slow. 9. Menorrhagia, excessive uterine bleeding occurring at the expected intervals of the menstrual periods. 10. Mutation, permanent changes in genes. Page | 3 CDHO Advisory | Hemophilia, VWD and O ther B leeding D isorder 11. Palliative care, services of care for persons towards the end of life with terminal illnesses, when the focus of the care a. is relieving symptoms b. attending to physical and spiritual needs. 12. Petechiae, round pinpoint spots on the skin a. caused by bleeding under the skin b. appear red, brown or purple c. may look like a rash d. are flat and do not lose their colour when pressed e. may indicate serious conditions. 13. pH, hydrogen ion concentration, expressed as a set of values ranging from 1 to 14 where 1 is very acidic; 7, neutral; and 14, very alkaline. 14. Platelets, cells in the blood that help the blood clot. 15. Supportive care, services of care to help persons meet the physical, emotional and spiritual challenges arising from serious illness or its treatment. 16. Thrombocytopenia, any disorder in which platelets are abnormally low, and which may be associated with abnormal bleeding. 17. von Willebrand disease (VWD), comprises a family of hereditary bleeding disorders caused by abnormality of the von Willebrand factor (VWF). 18. von Willebrand factor (VWF), a protein which a. is required for normal platelet function b. is normally the carrier for factor VIII 19. Wilms’ tumour, cancer of the kidney, one of the most common types of childhood cancer, affecting 1 in every 10,000 children. Overview of hemophilia, von Willebrand disease or other bleeding disorder Resources consulted . Bleeding Disorders of Importance in Dental Care and Related Patient Management: Journal of the Canadian Dental Association . Bleeding Disorders Resources: World Federation of Hemophilia . College of Dental Hygienists of Ontario o CDHO Fact Sheet Hemophilia A o CDHO Fact Sheet Hemophilia B o CDHO Fact Sheet von Willebrand Disease . Disseminated intravascular coagulation: MedLine Plus . Hemophilia: MedlinePlus . PAIN – The Fifth Vital Sign: Canadian Hemophilia Society . Platelet disorders: Medline Plus . Treatments and drugs: Mayo Clinic . Von Willebrand Disease: eMedicine . What is a Bleeding Disorder?: National Hemophilia Foundation . What is hemophilia: Canadian Hemophilia Society . What is Hemophilia?: National Heart, Lung, and Blood Institute Occurrence, types and causes of bleeding disorders generally Bleeding disorders 1. occur when certain clotting factors are low or missing, causing bleeding problems which range from mild to severe Page | 4 CDHO Advisory | Hemophilia, VWD and O ther B leeding D isorder 2. may be present at birth, arising from rare inherited disorders 3. occur as various types, including a. hemophilia A b. hemophilia B c. von Willebrand disease 4. have various causes, including a. immune system-related effects, such as i. allergies to medications ii. reactions to infection iii. antibodies’ destruction of blood clotting factors b. cancer, especially of the blood and blood forming organs, such as leukemia c. thrombocytopenia d. bone marrow disease e. liver disease f. disseminated intravascular coagulation g. medications that i. aggravate bleeding ii. involve long-term use of antibiotics h. vitamin K deficiency. Occurrence of hemophilia Hemophilia 1. occurs in persons of all races and ethnic origins 2. is a life-long condition 3. is hereditary, passed from mother to child, and a. therefore occurs in children b. occurs in its most severe forms i. in males, almost always ii. in which the blood clots abnormally because it is lacking a blood-clotting factor essential for stopping or preventing bleeding c. seriously affects females only if the father is a hemophiliac and the mother is a carrier, a rare occurrence 4. reduces quality of life for women who are carriers and who also have symptoms of mild hemophilia 5. occurs as a. hemophilia A, which i. affects fewer than 1 in 10,000 people, about 2500 Canadians ii. is also called 1. factor VIII deficiency hemophilia 2. classical hemophilia, the most common of the factor deficiencies b. hemophilia B, which i. affects about 1 in 50,000 people, about 600 Canadians ii. is also called 1. factor IX deficiency hemophilia 2. Christmas disease2 6. is classified according to its severity a. mild i. clotting factor VIII or clotting factor IX level is 5 percent of normal or greater 2 Named for the Canadian, Steven Christmas who, in 1952,