PB0448 - Menorrhagia in adolescent girls with underlying bleeding disorders; effect of a low cost intervention

Background: Data on real-world-experience of menorrhagia in girls with underlying bleeding disorder is sparse. The bleeding is severe and quality-of-life (QOL) poor, especially with poor access to factor concentrates.

Aims: We describe our experience from a tertiary-care centre on the effect of managing with a low-cost scheduled intervention of iron, contraceptive pill and tranexamic acid.

Methods: Girls with primary bleeding disorders managed for menorrhagia between 2020-2023 were included. Patients were assessed for menorrhagia using PBAC score, bleeding using BAT score and QOL using PedsQL4.0-SF15 score. Treatment included progesterone only pills regularly for 21 days (week 1-3) followed by 7 days of tranexamic acid (week 4) and daily oral iron at 2-3 mg/kg/day. Clotting factors were used sparingly.

Results: 14 children qualified as per inclusion criteria. This included 9 von Willebrand disease, 3 chronic immune thrombocytopenia, 1 congenital afibrinogenemia and 1 Factor VII deficiency. The median PBAC at presentation was 300 (190-500) suggesting severe menorrhagia. The median age of menarche was 12 years. Other sites of bleeding were oral, dental, joints and post-operative. 7/13 had anemia and 5 received red cell transfusions for the same. 7 had positive family history. Median follow-up for menorrhagia was 4 years (1.5-6 years). After initiation of treatment, PBAC reduced to a median value of 100 (60-150). The median follow up was 2.2 years. QOL assessment for physical, emotional, social and school functioning revealed >50 score for all patients with VWD. 1 patient each of chronic ITP, afibrinogenemia and factor VII deficiency reported low QOL. Low hemoglobin, poor compliance to treatment were related to low QOL and high PBAC score.

Conclusion(s): Menorrhagia in children with bleeding disorders are a cause of morbidity and poor QOL. Optimal management with low-cost strategies, can help reduce need for transfusions and improve QOL without the need for factor replacement.