1. Sickle Cell Vascular Disease Section, Cardiovascular Pulmonary Branch, National Heart, Lung and Blood Institute, National Institutes of Health Catherine A. Seamon, Darlene T. Allen, James G. Taylor VI, Jonathan Wilson, Gregory J. Kato

2. Disclosures No conflicts of interest to disclose. Funded by the NIH Division of Intramural Research.

3. Objectives Determine prevalence of tobacco, alcohol, and illicit drug use in sickle cell anemia. Discuss impact of “drug seeker” or “addict” labels on the management of acute pain crises.

4. Leading barrier to effective pain management is negative provider perceptions Avoidance of emergency rooms Delayed administration of analgesics Mistrust Ineffective medical care Background Haywood, C., et al. A systematic review of barriers and interventions to improve appropriate use of therapies for sickle cell disease. J Natl Med Assoc. 2009; 101(10): 1022-1033

5. “When you see us, across an examination table, hospital bed or ER gurney it is but a microcosm of our quotidian trials. You may be skilled in healing but we are expert in surviving… We have and still do hope, no pray for the cure that will deliver us from this incessant suffering, the tortuous pain that pits us against implacable providers of relief who dole out miniscule amounts of analgesic. We want to have a say in what is written about us. We want to call ourselves by our names and define our situational crises as we see fit. We want to critique our critics and allies before misleading comments are committed to paper in medical annals, forever shaping the version of who we are and what we do…” Quote taken with permission of author from the Sickle Cell Disease Care and Research: Past, Present, and Future, Panel presentation at the James B. Herrick Symposium (Nov 16, 2010)

6. SCD Evaluation Study: – Pulmonary Hypertension, etc. – To identify genetic modifiers of sickle cell disease complications and severity Age ≥ 18 Compensation Subject Interviews National Institutes of Health

7. National Survey on Drug Use and Health, 2009 Survey of civilian, non-institutionalized population Evenly divided among states and age categories: 12-17, 18-25, and 26 years or older Compensation Computer assisted surveys Substance Abuse and Mental Health Services Administration. Results from the 2009 national survey on drug use and health: Volume I. summary of national findings. Rockville, MD: Office of Applied Studies; 2010. NSDUH Series H-38A, HHS Publication No. SMA 10-4586Findings.

8. Population Demographics NHLBI (%) National (%) National (A.A.) (%) Race African American Other 97 3 12.8 87.2 100 0 Age 12-17 yrs 18-25 yrs 26-34 yrs >35 yrs 0.2 26.5 28.9 44.4 31.7 34.1 10.1 24.1 34.5 35.9 9.5 20.1 Sex Male Female 46 54 44 47 46 54

9. Self-Reported Results N NIH (%) Nat (%) Z Score Nat ( A.A. ) (%) Z Score Tobacco651321.3-8.0827.7-7.20 Alcohol1262542.8-12.8351.9-8.49 Drugs2148.6-3.778.7-4.49 *Participants were classified as current users, if they had used a substance within the year prior to evaluation

10. Limitations Age-matched population comparisons (NSDUH) not performed Ascertainment Methods Differed – Interviews versus computer assisted survey

11. Drug abuse stigma could lead to ineffective pain treatment. A change in provider perception has the potential to positively impact trust in providers. – Future area for study Implications

12. Acknowledgements Thank you! Patients Sickle Cell Vascular Disease Section Clinic Nurses Clinical Research Nurses

13. Cassie Seamon, RN Clinical Research Nurse cseamon@nhlbi.nih.gov 301-451-6361 Contacts Darlene T. Allen, MT (ASCP) Study Coordinator darlenea@nhlbi.nih.gov 301-435-1495 James G. Taylor, VI Assistant Clinical Investigator Genomic Medicine Section jamesta@nhlbi.nih.gov 301-435-7895