Treating Patients With Bleeding Disorders

A bleeding disorder is a condition in which the normal blood clotting process is interrupted due to a deficiency or defect of one or more of the clotting factors necessary to form a blood clot. A deficiency/defect of one or more clotting factors raises the risk for excessive and prolonged bleeding.¹ Symptoms of bleeding disorders include prolonged bleeding, oral bleeding, multiple nosebleeds, heavy menstrual bleeding (menorrhagia), and spontaneous bleeding.² Although excessive gingival bleeding is often reported, studies show that this may not be a true symptom of bleeding disorders.^3–5 The severity of symptoms depends on the amount of clotting factor present in the blood. Due to the risk of increased bleeding, knowledge of bleeding disorders is essential to providing comprehensive care and preventing treatment complications in this patient population.

When injury occurs, the body goes through the clotting cascade that involves many clotting factors working together to form a clot. Upon injury, a hole in the capillary forms, causing bleeding. This capillary tightens up and platelets join together to form a plug, which patches the hole in the capillary. In those without a bleeding disorder, 13 clotting factors in the plasma work together to form a clot over this plug, which strengthens the plug and eventually stops the bleeding. When someone with a bleeding disorder has a deficiency or defect of clotting factor(s), this process is interrupted and bleeding continues.^1,6

MOST COMMON BLEEDING DISORDERS

Von Willebrand Disease (VWD) is the most common inherited bleeding disorder, presenting in roughly 1% of the world’s population.^7,8 In those with VWD, there is a defect or deficiency of VW factor (VWF), a sticky protein that has multiple functions in maintaining hemostasis. VWF itself is not a clotting factor, but it helps platelets stick together and to the capillaries. VWF also carries factor VIII, which is an essential clotting factor.^7,8

There are three types of VWD, all of which can affect men and women. Type 1 VWD is the most common, presenting in approximately 80% of those with VWD.^7,8 In those with type 1, there is a mild to moderate reduction of VWF levels. Type 2 VWD involves qualitative defects in the VWF. There are four subtypes (2A, 2B, 2M, and 2N), each of which has a different qualitative defect in the VWF. Type 3 VWD is characterized by little to no VWF, making it a more severe presentation.^7,8

Those with VWD can manage bleeding using a variety of products, including factor-boosting therapies that increase the VWF and factor (F)VIII levels in the plasma and adjunctive therapies that indirectly control bleeding. The most commonly used factor-boosting product is desmopressin, a synthetic drug that can either be administered intravenously, subcutaneously, or intranasally.⁷ In some cases, plasma-derived concentrates of VWF/FVIII are given intravenously to manage VWD. Adjunctive therapies include using antifibrinolytic agents, fibrin glue, or hormonal therapy to control bleeding in women who experience menorrhagia.⁷

Hemophilia is a relatively rare inherited X-chromosome-linked bleeding disorder that occurs in approximately one in 10,000 births.⁹ Because it is linked on the X chromosome, most individuals with hemophilia are male.10 Hemophilia can exist in women when the hemophilia gene presents on both X chromosomes.¹¹ Women can be carriers of hemophilia when the hemophilia gene is on one X chromosome. However, they may also demonstrate similar bleeding symptoms, which must be considered when providing dental treatment.^9,11,12

Hemophilia A involves a deficiency in clotting factor VIII (FVIII) and makes up roughly 80% to 85% of all hemophilia cases.⁹ The other 15% to 20% have hemophilia B, resulting in a deficiency of factor IX (FIX).⁹ Severity of hemophilia is classified as mild, moderate, or severe, depending on the amount of clotting factor and predicts bleeding risk. Hemophilia can cause joint and muscle bleeds.⁹ Over time, bleeding into the joints can result in hemophilic arthropathy, a joint disease that involves chronic pain, reduced quality of life, and limited joint movement and function.^13–15

Treating and preventing bleeds associated with hemophilia generally involve intravenously administering plasma-derived or recombinant FVIII or FIX concentrate, depending on the clotting factor associated with the disorder.⁹ In some cases, those with hemophilia treat at home with factor-replacement product in order to prevent anticipated bleeding and complications associated with repeated bleeding.^9,16 Those with more severe hemophilia may have a central venous access device (CVAD) placed for easy access in times of infusion.¹⁷ Other products, such as desmopressin and antifibrinolytic drugs, have been used to control bleeding. Adjunctive therapies including first aid, physical therapy, and COX-2 inhibitors can be used to manage pain, inflammation, and duration of bleeding.⁹

Bleeding disorders can involve deficiency in any of the 13 clotting factors, causing similar symptoms and complications as VWD and hemophilia. These disorders are named for the affected factor. Treatment depends on the factor impacted, but generally involves either plasma-derived factor concentrates, desmopressin, antifibrinolytic drugs, or fresh frozen plasma.¹⁸

BLEEDING DISORDERS AND ORAL HEALTH

Relatively few studies have been conducted on the oral health of those with bleeding disorders. Due to the physical symptoms and psychosocial effects of bleeding disorders, oral health, access to dental care, and oral health-related quality of life (OHRQoL) can become compromised. In some cases, individuals with bleeding disorders have been refused treatment by dental offices due to lack of provider knowledge and potential complications.³

One of the most discussed aspects of oral health in those with bleeding disorders is whether gingival bleeding is a true symptom. Gingival bleeding in those with bleeding disorders is caused by the presence of plaque from poor oral hygiene, but exacerbated by the lack of clotting factor.^{3–5,19–21} This may be due to fear of inducing bleeding with normal oral hygiene practices.^20–22 Reluctance to engage in normal oral hygiene practices also increases the risk of periodontal diseases and dental caries. Bleeding may also be found in other places in the oral cavity. Increased bleeding associated with tooth eruption and exfoliation, dental procedures, and oral trauma is common among those with bleeding disorders.^20,22

DENTAL MANAGEMENT OF BLEEDING DISORDERS

Patients with bleeding disorders can often be treated in a private dental practice, but treatment considerations must be taken to ensure safe and effective care. Due to the individuality and complexity of treating this patient population, it is essential to communicate with the patient’s hematology team and the attending dentist to ensure proper measures are taken. The patient may have to infuse with factor replacement before and after dental treatment. However, the amount of factor that patients receive should be minimized. This is due to both cost and the risk of developing antibodies or inhibitors to the factor over time.^23,24 Preventive care is critical to prevent the need for extensive dental work and factor replacement.^24,25

A bleeding disorder can be all-consuming, causing oral healthcare to be a low priority. However, plaque removal is necessary to prevent periodontal diseases and caries.²⁶ Gingival bleeding in those with bleeding disorders is due to the presence of plaque. Personalized oral hygiene recommendations should include proper plaque removal, sealants, fluoride use, regular dental care, and dietary counseling.^{9,20,24,26,27}

To avoid intraoral bleeding, oral trauma should be prevented. Mouth guards should be recommended for sports, but other appliances should be considered for the patient at increased risk for trauma (eg, bruxism).²⁰ All restorations, appliances, and prostheses should be examined to ensure they do not traumatize intraoral soft tissues.

Bleeding during tooth eruption and exfoliation is sometimes exacerbated by a bleeding disorder. If bleeding occurs during infant teething, it is best to discuss with the patient’s hematology team. Antifibrinolytic agents may be recommended if the blood cannot form a clot to stop the bleeding.²⁰ In those with bleeding disorders, teeth should exfoliate on their own. If bleeding occurs with normal tooth exfoliation or post-tooth extraction, pressure with gauze or a tea bag should be applied. When bleeding occurs from tooth eruption in older children and teenagers, pressure and an antifibrinolytic agent can be used. During the time of tooth eruption and exfoliation, straws and hot foods should be avoided, as they can disrupt a clot from forming.²⁰

PAIN CONTROL

The potential bleeding associated with dental pain medications should be discussed with the patient’s hematologist and attending dentist. Generally, a mild analgesic, such as acetaminophen, can be recommended.^{9,20,22,24,27} Due to long-term pain associated with bleeding disorders, patients may take pain medications consistently. This population may experience increased risk for opioid misuse due to long-term use.²⁸

Any type of local anesthesia can be used among patients with bleeding disorders, but local anesthesia in general can lead to complications, especially when administering injections to highly vascular areas such as the inferior alveolar nerve.^{20,22,24,25,27} Inferior alveolar nerve blocks can cause internal bleeding, leading to airway blockage and death; their use should be avoided in patients with bleeding disorders.^22,29–31 Buccal infiltration, intrapapillary injections, and intraligamentary injections should be administered when possible. If nerve blocks or lingual infiltrations are necessary, careful planning may involve asking the patient to pre-treat and post-treat with factor to control any potential bleeding.^20,22,24,25 Patients should be reminded to avoid chewing after local anesthesia to minimize the possibility of soft tissue trauma.

PROPHYLAXIS AND PERIODONTAL TREATMENT

Patients with bleeding disorders should be asked if they have a CVAD or port. If a port is present, antibiotic premedication may be necessary to prevent infection from subgingival procedures.¹⁷ The bleeding and trauma associated with subgingival scaling need to be considered. Factor replacement for subgingival scaling and root planing may be necessary. In cases of severe inflammation, providing supragingival scaling first and then subgingival scaling after inflammation decreases is recommended.^22,27 The use of a chlorhexidine mouthrinse is advised to reduce inflammation prior to scaling. Tranexamic acid mouthrinse may be used to control bleeding after subgingival scaling.²² Due to the high risk of bleeding in those with bleeding disorders, periodontal surgery should be avoided.³² When periodontal surgery is necessary, factor replacement therapy is almost always required. Careful planning with the patient’s hematology team and informing him or her of potential complications is necessary.^24,25

Restorations, endodontic treatment, orthodontic treatment, and prosthodontic procedures generally do not involve much bleeding. However, the armamentarium used during these procedures may result in accidental excessive bleeding. The following recommendations should be considered:^22,24

• Use rubber dams when applicable
• Avoid impingement of soft tissues (eg, rubber dam clamps, matrices, wedges, orthodontic appliances)
• Carefully use saliva ejectors and high-speed suction
• Ensure there are no rough edges on any restoration, appliance, or prostheses that would cause intraoral trauma
• Engage in four-handed dentistry
• Carefully place X-ray films and sensors
• Carefully remove impressions

Oral surgery procedures, including extractions, often place the patient with a bleeding disorder at high risk for severe bleeding. Careful consultation with the hematology team is necessary. Factor infusion and/or desmopressin use are almost always recommended. Patients with severe bleeding disorders may need to be treated in a hospital setting. In addition to systemic medications and products, local agents can be used to control bleeding including, but not limited to pressure, fibrin glue, sutures, surgical stents, tranexamic acid, and epsilon aminocaproic acid.²²

BLEEDING CONTROL

Products and agents may be used to prevent and control bleeding among patients with bleeding disorders. Prior to treatment, a systemic medication or product is often given to prevent procedure-related bleeding. During the appointment, various techniques and agents can be used to prevent and control bleeding such as epsilon aminocaproic acid. A paste can be made with a crushed aminocaproic acid tablet and water that is applied topically to the bleeding site.³³ Table 1 depicts appropriate techniques for management of excessive bleeding before, during, and after dental treatment. Consultation with the patient’s hematology team is highly recommended.^{9,20,22,24,27,33}

RECOGNIZING A BLEEDING DISORDER IN THE DENTAL CHAIR

Intraoral bleeding is a common symptom of bleeding disorders. Many patients may seek dental care in the event of an oral bleed. Oral health professionals may be the first to recognize the presence of an underlying bleeding disorder, especially with the oral trauma that commonly occurs in young children when learning to crawl and walk.³⁴ Prolonged bleeding following dental treatment, oral trauma, tooth eruption, and tooth exfoliation can be precursors to a potential underlying bleeding disorder. When extensive oral bleeding is noted, the patient should be referred to a hematologist or primary care physician for proper testing.

Patients with bleeding disorders are at increased risk for oral bleeding, oral disease, access to dental care issues, lower OHRQoL, and dental treatment complications. Prevention of oral disease should be a priority. With careful planning, including consultation with the patient’s hematologist, clinicians can ensure safe appropriate treatment for patients with bleeding disorders.

REFERENCES

1. World Federation of Hemophilia. Inherited Bleeding Disorders. Available at: https:/​/​elearning.wfh.org/​elearning-centres/​inherited-bleeding-disorders/​. Accessed July 1, 2020.
2. National Hemophilia Foundation. What Is a Bleeding Disorder? Available at: hemophilia.org/​Bleeding-Disorders/​What-is-a-Bleeding-Disorder. Accessed July 1, 2020.
3. Vanduine S, Ridley K, Bashutski J, et al. Gingival bleeding and oral hygiene in women with von Willebrand Disease (VWD): a pilot study. J Haemophilia Practice. 2017;4:49–57.
4. Weickert L, Miesbach W, Alesci SR, et al. Is gingival bleeding a symptom of patients with type 1 von Willebrand disease? A case-control study. J Clin Periodontol. 2014;41:766–771.
5. Epping L, Miesbach W, Nickles K, Eickholz P. Is gingival bleeding a symptom of type 2 and 3 von Willebrand disease? PLoS ONE. 2018;13:e0191291.
6. American Society of Hematology. Bleeding Disorders. Available at: hematology.org/​Patients/​Bleeding.aspx. Accessed July 1, 2020.
7. Lillicrap D, James P. Von Willebrand disease: an introduction for the primary care physician. Available at: www1.wfh.org/​publication/​files/​pdf-1204.pdf. Accessed July 1, 2020.
8. Nichols WL, Hultin MB, James AH, et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia. 2008;14:171-232.
9. Srivastava A, Brewer AK, Mauser-Bunschoten EP, et al. Guidelines for the management of hemophilia. Haemophilia. 2013;19:e1–47.
10. Israels S, Schwetz N, Boyar R, McNicol A. Bleeding disorders: characterization, dental considerations and management. J Can Dent Assoc. 2006;72:827–827l.
11. United States Centers for Disease Control and Prevention. Women and hemophilia. Available at: cdc.gov/​ncbddd/​hemophilia/​features/​women-and-hemophilia.html. Accessed July 1, 2020.
12. Hemophilia of Georgia. What Are Symptomatic Carriers? Available at: hog.org/​handbook/​article/​2/​12/​what-are-symptomatic-carriers. Accessed July 1, 2020.
13. Nacca CR, Harris AP, Tuttle JR. Hemophilic arthropathy. Orthopedics. 2017;40:e940–e946.
14.  Melchiorre D, Manetti M, Matucci-Cerinic M. Pathophysiology of hemophilic arthropathy. J Clin Med. 2017;6:63–70.
15. Fischer K, de Kleijn P, Negrier C, et al. The association of haemophilic arthropathy with health-related quality of life: a post hoc analysis. Haemophilia. 2016;22:833–840.
16. Acharya SS. Advances in hemophilia and the role of current and emerging prophylaxis. Am J Manag Care. 2016;22(5 Suppl):s116–125.
17. Valentino LA, Kapoor M. Central venous access devices in patients with hemophilia. Expert Rev Med Devices. 2005;2:699–711.
18. World Federation of Hemophilia. Rare Clotting Factor Deficiencies. Available at: https:/​/​elearning.wfh.org/​elearning-centres/​rare-clotting-factor-deficiencies/​. Accessed  July 1, 2020.
19. McCarthy P. Gum bleeding. Available at: haemophilia.org.au/​publications/​national-haemophilia/​2015/​no-189-march-2015/​gum-bleeding. Accessed July 1, 2020.
20. Scully C, Diz Dios P, Giangrande P. Oral Care for people with hemophilia or a hereditary bleeding tendency. Available at: www1.wfh.org/​publications/​files/​pdf-1164.pdf. Accessed July 1, 2020.
21. Benito Urdaneta M, Benito Urdaneta M, Ferrara Mendez V, Bernardoni S, Vizcaino MA. Evaluating periodontal conditions in patients with von Willebrand’s disease in Hospital Universitario de Maracaibo (University Hospital, Maracaibo)-Venezuela. Med Oral Patol Oral Cir Bucal. 2008;13:E303–306.
22. Gupta A. Bleeding disorders of importance in dental care and related patient management. J Can Dent Assoc. 2007;73:77–83.
23. Franchini M, Castaman G, Coppola A, et al. Acquired inhibitors of clotting factors: AICE recommendations for diagnosis and management. Blood Transfus. 2015;13:498–513.
24. Brewer A, Correa M. Guidelines for dental treatment of patients with inherited bleeding disorders. Available at: www1.wfh.org/​publications/​files/​pdf-1190.pdf. Accessed July 1, 2020.
25. Australian Haemophilia Centre Directors’ Organisation. A Consensus Statement on the Dental Treatment of Patients with Inherited Bleeding Disorders. Available at: ahcdo.org.au/​documents/​item/​14. Accessed July 1, 2020.
26. Gibbons RJ, van Houte J. Dental caries. Annu Rev Med. 1975;26:121-136.
27. Shastry SP, Kaul R, Baroudi K, Umar D. Hemophilia A: dental considerations and management. J Int Soc Prev Community Dent. 2014;4(Suppl 3):S147–S152.
28. Mora E. Opioids, addiction, and bleeding disorders. Available at: hemophiliafed.org/​news-stories/​2016/​01/​opioids-addiction-bleeding-disorders/​. Accessed July 1, 2020.
29. Archer WH, Zubrow HJ. Fatal hemorrhage following regional anesthesia for operative dentistry in a hemophiliac. Oral Surg Oral Med Oral Pathol. 1954;7:464–470.
30. Leatherdale RAL. Respiratory obstruction in haemophilic patients. Br Med J. 1960;1:1316–1320.
31. Bogdan CJ, Strauss M, Ratnoff OD. Airway obstruction in hemophilia (factor VIII deficiency): a 28-year institutional review. Laryngoscope. 1994;104:789–794.
32. Sciullo PA, Nacht ES, Tesone AR. Postsurgical complications in an undiagnosed hemophiliac: a case report. ASDC J Dent Child. 1972;39:194–196.
33. Madan N, Rathnam A, BajaJ N. Treatment of an intraoral bleeding in hemophilic patient with a thermoplastic palatal stent—novel approach. Int J Crit Illn InJ Sci. 2011;1:79–83.
34. Sonis AL, Musselman RJ. Oral bleeding in classic hemophilia. Oral Surgery, Oral Medicine, Oral Pathology. 1982;53:363—366.

From Dimensions of Dental Hygiene. July/August 2020;18(7):36-39.