Management of patients with elevated Self-BAT scores or other bleeding symptoms

Case description

F.A. is a 22-year-old woman who sees her family physician after completing the online Let’s Talk Period Self-BAT. She did the assessment because she has had heavy menstrual periods and iron deficiency since menarche. She has a score of 6 (normal female range 0 to 5). Her family physician is not familiar with the Self-BAT and wants to know how the bleeding score will affect clinical management.

Sources of information

The poster tools and reference guide resources were created by synthesizing information from primary research sources and clinical review articles and by synthesizing formal data and qualitative feedback from 10 individuals (4 research staff, 2 nurses involved in the care of bleeding disorder patients, 1 hematologist, 1 family physician, and 2 medical students, all from Queen’s University in Kingston, Ont) who were interviewed to identify gaps in clinical practice in the fall of 2019.

Main message

Recommendations for management of a patient with an elevated Self-BAT score or other bleeding symptoms presenting to a primary care provider include reviewing the results of the Self-BAT to clarify relevant symptoms, performing initial diagnostic laboratory tests, and doing basic symptom management. Determining the threshold for referral to a hematologist is up to the primary care provider’s clinical judgment, but referral should be considered if the bleeding score is abnormal or if initial management options are ineffective.

Self-BAT review with patient. Since the Self-BAT was developed and validated as a patient-administered version of the health care professional–administered ISTH-BAT, either tool can be used in the clinical setting to review a patient’s bleeding symptoms.^12,16,19 Both tools consider a score of 4 or more in males and 6 or more in females as abnormal, with higher scores indicating a greater likelihood of the presence of an inherited bleeding disorder.¹³ It is important to note that a normal bleeding score does not always rule out the presence of a bleeding disorder, especially in men and children who may not have had the opportunity to bleed. Clinical judgment plays an important role in decision making and thresholds for referral.

Diagnostic tests. Recommended initial laboratory tests include complete blood count, ferritin level, and activated partial thromboplastin time (aPTT) and prothrombin time.²⁰ Activated partial thromboplastin time and prothrombin time tests can rule out factor deficiencies as the cause of bleeding; however, normal test results or an isolated, prolonged aPTT do not rule out VWD in a patient presenting with bleeding symptoms. Von Willebrand disease tests (ie, von Willebrand factor [VWF] antigen tests, platelet-dependent VWF function tests such as those measuring the binding of VWF to the gain-of-function mutant glycoprotein Ib fragment and those measuring factor VIII coagulant activity) can be ordered by initial primary care provider workup or by a hematologist, and they can confirm VWD. Depending on the laboratory, assays ordered in the community can have up to a 30% false-positive rate because of preanalytical variables (ie, the effect of delayed transport or testing and samples not being kept at the proper temperature).^12,21 It is recommended to interpret a positive result with caution or to have results confirmed by a hematology clinic.^20,22

Clinical pearls. During the process of reviewing Self-BAT results with patients, certain symptoms will need to be explored and managed. Helpful clinical pearls for each of the Self-BAT symptom categories are shown in Figure 2 and can assist in the referral process, as not all symptoms warrant evaluation by a hematologist or other specialist.

Bleeding disorder presentation may differ in pediatric versus adult populations. While a single symptom in an adult rarely justifies referral to a hematologist, single bleeding symptoms in pediatric patients—such as the presence of recurrent bruising, procedural bleeds, epistaxis, or heavy menstrual bleeding—may suggest an underlying bleeding disorder.

Management. There are many treatment options for a family physician to trial before or in tandem with a hematologist or obstetrician-gynecologist referral. Hematologic treatment options may include oral or intravenous iron supplementation, hemostatic medications such as tranexamic acid or desmopressin, or factor replacement therapy.²⁰ Gynecologic management for heavy menstrual bleeding may include hormonal contraceptives, intrauterine device insertion, endometrial ablation, or sometimes hysterectomy.^23-25

Case resolution

F.A.’s family physician reviews the results of her completed Self-BAT. Besides having menorrhagia and iron deficiency since menarche, F.A. also experiences epistaxis and easy bruising, and she had prolonged bleeding following dental extraction. After performing a more in-depth history, the family physician is unable to determine a known cause for the multiple bleeding symptoms. For example, the epistaxis from both nostrils has no seasonal variation and the patient is not on any medications that could potentially increase bleeding risk. The presence of multiple unexplained bleeding symptoms leads the physician to be suspicious of an inherited bleeding disorder. Basic blood work is ordered that confirms iron-deficiency anemia; prothrombin time is normal and aPTT is prolonged. Abnormal aPTT and the presence of bleeding symptoms prompt the physician to order a workup for VWD. The results come back positive and a diagnosis of VWD is made. To treat the bleeding symptoms, she prescribes and inserts a levonorgestrel intrauterine device with the goals of reducing bleeding and pain during menstruation and of lessening the need for iron supplementation. Additionally, she prescribes an oxymetazoline nasal spray for the management of epistaxis and tranexamic acid tablets for the management of bleeding episodes. A referral to a hematologist for long-term VWD management and education is made.