Pain reports have become increasingly common and problematic in thalassemia. As patients are living longer, there is a growing need to study pain and explore its impact on patient lives. The Brief Pain Inventory (BPI) was used quarterly to assess pain and pain interference in North American thalassemia patients. The SF-36 and HADS were used to assess quality of life, anxiety, and depression. Of the 252 participants, 56% reported pain at least once over the course of this study, with 32% reporting severe pain (≥7/10); 16% reported pain at all 4 visits. Increased pain severity significantly interfered with daily life (p<0.001; regression analysis), and participants with more sites of pain showed an increase in the amount of daily activities affected by pain (p=0.001). Participants reporting more visits with pain reported a significantly higher impact on affective and physical function (p <0.001). Physical quality of life decreased with increasing numbers of visits with pain (p <0.001). Those who reported one or more sites of pain showed increased symptoms of both depression (p <0.001) and anxiety (p= 0.003). Participants reporting at least two visits with pain had higher symptoms of anxiety (p= 0.002), and those with at least three visits, higher symptoms of depression (p= 0.003). Pain in thalassemia is a common, often chronic condition that interferes with life. The study highlights the significance of pain in thalassemia and its impact should be considered in future research and treatments.

Central retinal artery occlusion (CRAO) is a rare and potentially devastating cause of acute blindness in sickle cell disease (SCD) that is unique compared to classic sickle retinopathy. Few details related to this complication in SCD are known, including its risk factors, pathogenesis, presentation, treatment and outcomes. We present three patients with SCD and retinal artery occlusion. The overall variability in clinical presentation, treatment and prognosis reported in the literature underscores the need for a greater understanding of these factors as they relate to this complication in SCD.

A wide range of local standard care guidelines for blood transfusion therapy exists. The results of this survey indicate that partial manual exchange transfusion is the most commonly used method of chronic blood transfusion therapy in children with SCD and stroke despite the fact that the magnitude of benefit in comparison with simple transfusion has not been established. Factors such as peripheral venous access, compliance with current chelation regimen, and the presence of antibodies are important considerations in the choice of method.

256 Background: Pain is not a symptom generally associated with thalassemia. However, healthcare providers have anecdotally noted increasing patient reports of chronic pain over the last decade creating an impetus for the TCRN to conduct this prospective, observational assessment of pain in patients with thalassemia over the age of 12. Study goals include assessment of pain prevalence, severity and sites and whether these factors are impacted by age, gender or diagnosis. Methods: Pain was assessed quarterly using the Brief Pain Inventory (BPI). Two hundred fifty-one thalassemia patients ranging in age from 12 to 71 (average age of 28.75) receiving care at one of 12 thalassemia centers across the US and Canada participated in the study. Fifty-four percent of participants were female. Diagnoses included: Beta Thalassemia (80%), E Beta thalassemia (11%), Hemoglobin H and H Constant Spring (6%) and other thalassemia conditions (3%). Eighty percent of participants were chronically transfused, 6% intermittently transfused and 14% had never been transfused. This report reviews baseline findings. Results: At study entry, 64% of the 251 participants reported experiencing pain over the last four weeks, of whom 21% reported pain on a daily basis. In comparison, 26% of the American public, 20 years and older, reported pain over a one month period according to National Center for Health Statistics data, 2006. Ordinal regression analysis of participant ratings of worst, least, and average pain over the last seven days demonstrated significant (p<0.001) correlation of increased age with increased pain across all categories irrespective of diagnosis, transfusion status or gender. Similarly, ordinal regression analysis revealed that pain increased with participant age and significantly correlated (p<0.001) with a negative impact of pain on patient's affect and activity as measured by the BPI interference scales. Eighty-two percent of those reporting pain indicated lower back as a site of pain. In logistic regression models, lower back (p=0.046), arm (p=0.047) and hip (p=0.009) pain significantly increased with age. The number of bodily pain sites (p=0.033) also increased with age which was determined using linear regression. Among patients reporting pain in the last seven days, 77% reported having pain for one year or longer and 26% reported pain for 5 or more years. Participants reporting pain in the last 7 days identified the following reasons for their pain: thalassemia (60%), low hemoglobin (55%), bone pain (37%) and muscle spasm (30%). Participants indicated multiple methods of managing pain including: blood transfusion (54%), rest (51%) massage (46%) and heat (39%). Medications were the most frequently cited pain intervention (72 % of participants) with the most common mediations taken being NSAIDs (71%), followed by acetaminophen (48%), short acting narcotics (24%) and long acting narcotics (11%). Twenty-five percent of participants reported they received no pain relief from medications or non-pharmaceutical treatments, and only 4% reported they received complete pain relief with treatment. Half the population reported they gained about 50% relief from pain with treatments. Conclusions: These data show that pain is a significant issue for patients with thalassemia and as patient's age pain increases. Pain assessment should be conducted on a regular basis for all patients with thalassemia since neither transfusion status nor diagnosis are a reliable indicator of pain status. The study also indicates that chronic pain (pain lasting greater than one year) is an issue for thalassemia patients and underscores the need for further study of pain in this population. Analysis of pain follow up data collected at 3 month intervals post baseline is being conducted to assess whether severity levels vary over time. Disclosures: Coates: Novartis: Research Funding, Speakers Bureau. Neufeld:Novartis, Inc: Research Funding; Ferrokin, Inc: Research Funding.