Leg Ulcers in Sickle Cell Disease: Patterns and Practices in the ...

Objectives• Describe the characteristics of the U.S. sicklecell population with leg ulcers• Discuss U.S. providers’ perceptions regardinghydroxyurea use in sickle cell patients withleg ulcers• Review referral practices for sickle cellpatients with leg ulcers3

Introduction• Prevalence varies by geographic region• Associations have been shown withgenotype, hemoglobin levels, hemolysis,priapism, and pulmonary hypertension• Treatment approaches often based onopinion and experience5

IntroductionKoshy M, Entsuah R, et al. Leg ulcer in patients with sickle cell disease. Blood. 1989 Sep;74(4):1403-8.6

IntroductionMinniti CP, Eckman J, et al. Leg Ulcers in sickle cell disease. Am J Hematol. 2010 Oct;85(10):831-37

Methods• Survey AIMS• Patient demographics• Therapies• Referral• Survey e-mailed to 120 members of theSickle Cell Adult Provider Network

SurveyParticipation Rate of 26% (31/120)9

Results - Reported Demographics• Males 2:1 predominance• Hgb SS>SC>SbActive UlcersPast 5 years185 22425Leg Ulcers and Age Distribution201510# ofrespondents5040y10

Results - Leg Ulcer Prevalence2.5-25%75%10%0%

Results - Topical Leg Ulcer TreatmentsTopical Therapies*For select casesUnna’s Boot 100%CompressionStockings100%Skin graft 70%*12

Results - Systemic Leg Ulcer TreatmentsSystemic TherapiesZinc 70%HU 36%RBCTransfusion36%13

Is hydroxyurea beneficial for leg ulcers in patients with SCD?

Hydroxyurea and Leg Ulcers• 25% of respondents thought it is helpful• 33% advocated chronic transfusion therapy• Case reports of ulcer development in otherpatient population with HU use• No RCT specifically evaluating HU for legulcers15

Who should take care of the leg ulcers in sickle cell patients?

Results - Referral Practices1614121086# of responses420ProviderIn-OfficeWoundClinicOutsideWoundSpecialistOther**** Includes Patient Self-Care, Podiatry, Dermatology, Plastic Surgery17

Summary• Leg ulcer demographics changing in the U.S.• The role of hydroxyurea remainscontroversial• Modalities first explored over 30 years agoremain the mainstays of treatment• Research on agents with alternativemechanisms of action is needed

11-H-0121: A Phase I Study of the Use ofTopical Sodium Nitrite for Chronic LegUlcers in Adult Patients with HemolyticDisordersResearch Contact:Marlene Peters-Lawrence, RNPhone: 301-435-2345Email: mpeters@nhlbi.nih.gov

DISCLOSUREI have nothing to Disclose