High Resource Utilizers From The Multicenter Compact Study Of Complications In Patients With Sickle Cell Disease and Utilization Of Iron Chelation Therapy

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 982-982
Author(s):  
Lanetta B Jordan ◽  
Patricia Adams-Graves ◽  
Julie Kanter-Washko ◽  
Patricia A Oneal ◽  
Medha Sasane ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Research Funding ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Iron Chelation Therapy ◽  
Cell Disease ◽  
High Utilizers ◽  
Ed Visits ◽  
High Utilizer ◽  
High Utilization

Abstract Introduction While treating patients (pts) with sickle cell disease (SCD) can be costly, costs are not evenly distributed across pts; rather, a minority of pts accounts for a majority of costs. Identifying those pts who consume a disproportionately large share of healthcare resources can assist payers and providers in directing appropriate and targeted interventions to deliver better pt care with lower costs. The objective of this study was to understand characteristics of pts who have increased utilization of inpatient (IP) and emergency department (ED) resources in a population of SCD pts ≥16 years old. Method Medical records of 254 SCD pts ≥16 years old were retrospectively reviewed between 8/2011 and 7/2012 at three US tertiary care centers. The high utilization threshold was derived from the literature and defined as pts with ≥ 5 days of IP+ED care (assuming 1 day/ED visit) for SCD-related complications per year (high utilizer group). Pts were also classified into cohorts based on cumulative blood transfusion units and use iron chelation therapy (ICT): <15 units, no ICT (Cohort 1 [C1]), ≥15 units, no ICT (Cohort 2 [C2]), and ≥15 units, with ICT (Cohort 3 [C3]). SCD complication rates were expressed as the number of SCD complications per pt per year (PPPY); rate ratios (RRs) were used for cohort comparisons. A logistic regression was used to identify risk factors associated with high utilization of IP+ED care. Results Of the 254 pts (C1: 69, C2: 91, C3: 94), 30% (n =76) were classified as high utilizers (C1: 14 [18.4%], C2: 37 [48.7%], C3: 25 [32.9%]). Patients in the high utilizer group were younger (median [range] (21 years old [16-65], vs. 23 years old [16-59]) and had shorter follow-up (4.2 years [0.6-23.9], vs. 5.4 years [0.5-33.3]) compared to the rest of the sample. Those in the high utilizer group accounted for 68% of all SCD-related complications and over 88% of all IP+ED days for treatment of these complications. Similar to the rest of the sample, pain (81%) and infection (7%) were the two key complications seen in this high utilizer group. The rate of IP +ED days was significantly higher among the high utilizer group with 16.63 [16.28-16.99] IP+ED days PPPY compared to 0.89 [0.84-0.94] PPPY for other pts. Similarly, the high utilizer group had 4.58 [95% CI: 4.39-4.76] IP+ED visits PPPY, compared to 0.34 [0.31-0.37] visits PPPY for other pts (Table). Among regularly transfused pts (C2+C3) in the high utilizer group, those who received ICT had lower rates of IP+ED visits (C2 vs. C3 rate ratio [RR] [95% CI]: 1.31[1.20-1.44]), IP+ED days (C2 vs. C3 RR: 1.30 [1.24-1.36]), and readmission to IP+ED settings within 30 days (1.70 [1.49-1.93]) compared with those who did not (Table). History of infections (odds ratio: 7.45, p<0.0001) was associated with an increased risk of high utilization of IP+ED care. Conclusion Results from this study show that a relatively small fraction of SCD pts account for the majority of IP+ED visits. Moreover, among regularly transfused pts identified as high utilizers, those who received ICT had lower rates of IP+ED utilization than those who did not. Pts receiving ICT may also receive closer monitoring, which may help with early identification and intervention to delay or prevent the development of complications and improve outcomes. Closer management of pts with SCD, especially those at risk of becoming high utilizers, is critical to lowering IP+ED utilization and reducing the overall costs of care. Disclosures: Jordan: Novartis Pharmaceuticals Corporation: Consultancy. Adams-Graves:Analysis Group, Inc.: Research Funding. Kanter-Washko:Analysis Group, Inc.: Research Funding. Oneal:Novartis Pharmaceuticals Corporation: Honoraria; Analysis Group, Inc.: Research Funding. Sasane:Novartis Pharmaceuticals: Employment. Vekeman:Novartis Pharmaceuticals: Research Funding. Bieri:Novartis Pharmaceuticals Corporation: Research Funding. Marcellari:Novartis Pharmaceuticals Corporation: Employment. Magestro:Novartis Pharmaceuticals: Employment. Adams:Novartis Pharmaceuticals Corporation: Research Funding. Duh:Novartis Pharmaceuticals: Research Funding.

Final Results From the Multicenter Compact Study of Complications in Patients with Sickle Cell Disease and Utilization of Iron Chelation Therapy: A Retrospective Medical Records Review.

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 2106-2106
Author(s):  
Lanetta B Jordan ◽  
Patricia Adams-Graves ◽  
Julie Kanter-Washko ◽  
Patricia Ann Oneal ◽  
Francis Vekeman ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Resource Utilization ◽  
Medical Records ◽  
Research Funding ◽  
Index Date ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Complication Rates ◽  
Iron Chelation Therapy ◽  
Cell Disease

Abstract Abstract 2106 Introduction: Over the past few decades, lifespans of sickle cell disease (SCD) patients have increased; hence, they encounter multiple complications. Early detection, appropriate comprehensive care, and treatment may prevent or delay onset of complications. There is a gap in the literature describing the SCD complication rates, blood transfusion patterns, iron chelation therapy (ICT) use, and associated resource utilization in SCD patients ≥16 years old. This study contributes to addressing this gap. Method: Medical records of 254 SCD patients ≥ 16 were retrospectively reviewed between August 2011 and July 2012 at three US tertiary care centers (University of Tennessee: 117; Tulane University: 72; Howard University: 65). Data were collected from patient's first visit after age 16 (index date) until the earliest indication of death, loss to follow-up, or last patient record on file prior to the centers' IRB submission dates. Patients were classified into one of three cohorts based on cumulative units of blood transfused and history of ICT: <15 units of blood and no ICT (minimally transfused, Cohort 1 [C1]), ≥15 units of blood and no ICT (Cohort 2 [C2]), and ≥15 units of blood and receiving ICT (Cohort 3 [C3]). SCD complication rates were expressed as the number of SCD complications recorded from patient charts per patient per year (PPPY) and compared among cohorts using rate ratios (RRs). Results: Cohorts 1, 2, and 3 consisted of 69, 91, and 94 patients, respectively. Mean (range) age at index date was similar across cohorts (27 yrs [16–65]) and all patients were African American. Mean length of observation was shorter among patients in C1 (yrs, C1: 6.6; C2: 8.2; C3: 8.1). Post index date, patients in C1 received an average of 1 unit of blood PPPY (p<0.001 vs. C2 and C3), whereas patients in C2 and C3 received an average of 10 and 15 units PPPY (p=0.112), respectively. Among patients with serum ferritin (SF) assessment within 60 days before ICT (n=57), mean (median) SF level was 4,881 ng/mL (4,040). Across all three cohorts, the most common SCD complication was acute pain crisis (69.8%), followed by infection/sepsis (5.1%), leg ulcers (2.9%), and avascular necrosis (2.3%). The rate (95% CI) of any SCD complications was the highest in C2 at 3.02 PPPY (2.89–3.14), followed by 2.26 PPPY (2.16–2.37) in C3, and 1.66 PPPY (1.54–1.77) in C1 (Table 1). Among transfused patients (C2+C3), those receiving ICT were less likely to experience SCD complications than those who did not (RR [95% CI] C2 vs. C3: 1.33 [1.25–1.42]). Similar trends (RR [95% CI]) were observed in emergency room (ER) visits and hospitalizations associated with SCD complications (C2 vs. C3, ER: 1.94 [1.70–2.21]; hospitalizations: 1.61 [1.45–1.78]), but not in outpatient visits. Conclusion: Results from this study highlight the significant burden of complications and the associated healthcare resource utilization for SCD patients. The results suggest that among regularly transfused patients, those who received ICT were less likely to experience complications than those without ICT. However, transfusions are not necessary for all patients with SCD and patients with more complications may have started transfusion therapy earlier. Patients receiving ICT may also receive closer monitoring, which may help with early identification and intervention to delay or prevent the development of complications and improve outcomes. Disclosures: Jordan: Novartis Pharmaceuticals Corporation: Consultancy, Speakers Bureau. Oneal:Novartis Pharmaceuticals Corporation: Honoraria. Vekeman:Novartis Pharmaceuticals: Research Funding. Bieri:Novartis Pharmaceuticals Corporation: Research Funding. Sasane:Novartis Pharmaceuticals: Employment. Marcellari:Novartis Pharmaceuticals Corporation: Employment. Magestro:Novartis Pharmaceuticals: Employment. Gorn:Novartis Pharmaceuticals Corporation: Research Funding. Duh:Novartis Pharmaceuticals: Research Funding.

scholarly journals Adherence to Iron Chelation Therapy and Associated Healthcare Resource Utilization and Costs in Medicaid Patients with Sickle Cell Disease

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 2177-2177
Author(s):  
Francis Vekeman ◽  
Medha Sasane ◽  
Wendy Y Cheng ◽  
Agnihotram V Ramanakumar ◽  
Jonathan Fortier ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Emergency Room ◽  
Resource Utilization ◽  
Sickle Cell ◽  
Research Funding ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Cost Of Care ◽  
Iron Chelation Therapy ◽  
Cell Disease

Abstract Introduction: While adherence to iron chelation therapy (ICT) is critical for successful iron overload (IO) treatment among patients with sickle cell disease (SCD), published data indicate it is often suboptimal. Deferoxamine (DFO) and Deferasirox (DFX) are ICTs indicated for the treatment of chronic IO in patients with SCD. Lack of patient adherence may impact patient outcomes and increase cost of care. This study evaluated the economic burden of ICT non-adherence in patients with SCD from the state Medicaid program’s perspective. Methods: Patients with SCD were identified from Florida, Iowa, Kansas, Mississippi, Missouri, and New Jersey (1997-2013) state Medicaid programs. Patients were required to have ≥1 ICD-9 diagnosis code for SCD (282.6), ≥1 prescription for DFO or DFX, and ≥6 months of continuous enrollment prior to the 1st DFO/DFX prescription (index date), which was defined as the baseline period. Adherence was estimated using the medication possession ratio (MPR), defined as the sum of the days of medication supply divided by the number of days between 1st and last prescription fill plus the days of supply of the last fill; a threshold of ≥0.80 was used to define optimal adherence. All-cause and SCD-specific resource utilization per-patient-per-month (PPPM) was assessed using cumulative rates, accounting for all visits observed, and compared between adherent and non-adherent patients using cumulative rate ratios (CRR). All-cause and SCD-specific healthcare costs were computed using mean cost PPPM. Regression models adjusting for baseline characteristics were used to assess resource utilization and cost differences between adherent and non-adherent patients. Results: A total of 846 eligible patients with SCD were included with 77 in DFO-only, 686 in DFX-only), and 83 in DFO/DFX switch cohort. Mean (SD) MPR was 0.68 (0.27) for DFO-only patients and 0.75 (0.26) for DFX-only patients (p<0.05). Among all users of ICT, 409 (48.3%) were considered adherent. Adherent patients were slightly younger (19 vs. 21 years, p=0.003) than non-adherent patients. Rates of transfusions were comparable between the two groups (mean [SD] transfusions PPPM, adherent: 0.41 [0.47]; non-adherent: 0.40 [0.54], p=0.456) at baseline. The adjusted rate of all-cause IP visits PPPM was lower in adherent versus non-adherent patients (CRR=0.87 [95% CI: 0.83, 0.91]; p<0.001). The adjusted rates of all-cause outpatient (OP) visits (1.10 [1.08, 1.13], p<0.001) and ER visits (1.06 [1.01, 1.10], p=0.010) PPPM of adherent patients were higher in adherent patients than those in non-adherent patients. A similar trend was observed in SCD-specific resource utilization except for rates of ER visits, which were similar between cohorts. From cost perspective, total all-cause and SCD-specific costs were lower in adherent versus non-adherent patients primarily due to lower IP costs (Table 1). SCD-specific ER and OP costs were similar in both cohorts. All-cause pharmacy costs were higher in adherent versus non-adherent patients. Conclusion: Published studies have reported low adherence to ICT, and a similar trend was found in this study. Adherent patients were observed to have less frequent hospitalizations and lower overall and SCD-specific IP costs compared to non-adherent patients. It should be noted that the rate of OP visits was higher in the adherent patients compared to non-adherent patients suggesting that adherent patients may be more closely monitored potentially resulting in better overall patient management and fewer hospitalizations. Additional analyses are needed to explore differences between adherent and non-adherent patients. Table 1 Costs PPPM Adherent patients (N=409) [A] Non-adherent patients (N=437) [B] Adjusted cost difference[A] – [B] P -value All-cause, mean [SD] $4,766 [$4,388] $5,304 [$4,725] -$724 0.072 Inpatient $1,911 [$3,647] $2,996 [$4,439] -$947 0.016 Emergency room $27 [$87] $40 [$88] -$203 0.104 Outpatient $580 [$697] $485 [$617] $49 0.500 Pharmacy $2,248 [$1,949] $1,783 [$1,449] $432 0.004 Pharmacy without ICT $215 [$482] $274 [$544] -$50 0.192 SCD-specific, mean [SD] $2,237 [$3,679] $3,116 [$4,301] -$952 0.0160 Inpatient $1,776 [$3,546] $2,782 [$4,268] -$855 0.0160 Emergency room $18 [$63] $28 [$69] -$199 0.1200 Outpatient $443 [$658] $306 [$548] $105 0.1120 Disclosures Vekeman: Novartis Pharmaceuticals: Research Funding. Sasane:Novartis Pharmaceuticals: Employment. Cheng:Novartis Pharmaceuticals: Research Funding. Ramanakumar:Novartis Pharmaceuticals: Research Funding. Fortier:Novartis Pharmaceuticals: Research Funding. Duh:Novartis Pharmaceuticals: Research Funding. Paley:Novartis Pharma: Employment. Adams-Graves:Novartis Pharmaceuticals: Consultancy, Honoraria, Speakers Bureau.

scholarly journals The effect of iron chelation therapy on overall survival in sickle cell disease and β-thalassemia: A systematic review

2018 ◽  
Vol 93 (7) ◽  
pp. 943-952 ◽  
Author(s):  
Samir K. Ballas ◽  
Amer M. Zeidan ◽  
Vu H. Duong ◽  
Michelle DeVeaux ◽  
Matthew M. Heeney
Keyword(s):  
Systematic Review ◽  
Overall Survival ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Iron Chelation Therapy ◽  
Cell Disease

Effectiveness, Time Utilization and Clinical Outcome of Partial Manual Red Cell Exchange in Patients with Sickle Cell Disease

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2326-2326
Author(s):  
Kevin H.M. Kuo ◽  
David Barth ◽  
Richard Ward
Keyword(s):  
Sickle Cell Disease ◽  
Clinical Outcome ◽  
Iron Overload ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Venous Access ◽  
Iron Chelation Therapy ◽  
Red Cell ◽  
Cell Disease ◽  
Hemoglobin S

Abstract Abstract 2326 Introduction: Red cell exchange transfusion (RBCX) is used to treat and prevent selected complications from Sickle Cell Disease (SCD) where there is a need to reduce hemoglobin S level, blood viscosity, improve oxygen carrying capacity, and to avoid rapid iron overload from simple transfusions. Partial manual red cell exchange is sometimes employed in the chronic maintenance of low hemoglobin S levels. Data on the efficacy and clinical outcome of SCD patients on partial manual RBCX are limited. Methods: All partial manual RBCX from the University Health Network, a SCD comprehensive care center between April 1st, 2010 and April 30th, 2011 were retrospectively reviewed. Patients were exchanged at a frequency of 4 to 6 weeks where each session consists of two 500cc phlebotomy with an infusion of 500cc normal saline in between the phlebotomies, and transfusion of 2 units of packed red cells (pRBC). The procedure was repeated until pre-RBCX hemoglobin S (HbS) level <50% was reached (for patients without overt stroke for >4 years). Phlebotomy was reduced or omitted during episodes of symptomatic anemia at the discretion of the treating hematologist. Patients with poor venous access had indwelling line with chronic, therapeutic anticoagulation against line-related thrombosis. Results: Nineteen patients (16 HbSS, 2 HbSC, 1 HbSD) totalling 176 exchange sessions were reviewed. Indications for RBCX include primary and secondary stroke prevention (n = 14), recurrent painful vaso-occlusive crises intolerant or refractory to hydroxyurea (n = 3), pulmonary hypertension confirmed on right heart catheterization with hypoxia (n = 1), and prevention of intrahepatic cholestasis in a liver allograft (n = 1). Mean frequency of RBCX was 4.8 weeks (95% CI 3.9, 5.6 weeks). There were 2 transfusion-related (fever, pruritis) and 1 phlebotomy-related (pre-syncope) adverse events. There were 23 partial/cancelled phlebotomy sessions, mostly due to symptomatic anemia. Mean post-RBCX hematocrit was 0.296 (95% CI 0.280, 0.312) and pre-RBCX HbS level was 0.439 (95% CI 0.387, 0.490). Pre-RBCX HbS level of <50% was achieved in 74% of exchanges. Reasons for not achieving the target HbS level include: exchange interval >4.0 weeks, not on any transfusion regime prior to initiating partial manual RBCX, reduced or no phlebotomy in previous session, and non-adherence to treatment. Patients who were adherent to treatment had no recurrent events related to their initial indication for RBCX (one patient has possible Moyamoya formation but no clinically overt stroke), while 3 of the 6 patients who were not adherent had events during the study period (2 had painful vaso-occlusive crisis requiring hospital admission and 1 had new Moyamoya-like changes on cerebral angiogram). It took a median time of 90 minutes to phlebotomize 1,000cc whole blood and 176 minutes to transfuse two units of pRBC. There was no significant difference between the time required to phlebotomize or transfuse via peripheral vein versus an indwelling line (55 vs. 53 minutes/500cc; P = 0.7572 and 88 minutes vs. 88 minutes/unit; P = 0.9859). Eleven patients were also on iron chelation therapy for iron overload from previous simple transfusion, and patients who were adherent to RBCX (n = 7) had either a stable or reduction in ferritin level. Discussion: Patients who are adherent on partial manual RBCX can maintain a pre-RBCX HbS <50% with good clinical outcomes and low rates of adverse events, reduced blood consumption compared to automated RBCX, and obviate the need for ongoing iron chelation in those without pre-existing iron overload. In patients with iron overload, RBCX combined with iron chelation therapy can maintain iron balance. In patients with good peripheral venous access, indwelling lines do not confer an advantage to the speed of phlebotomy or transfusion. Patient with pre-RBCX HbS level >50% may benefit from a single session of automated RBCX to “reset” their HbS level before commencing chronic partial manual RBCX. Further prospective studies will aim to determine the rate of new or progressive silent infarcts and vasculopathy and reduction of iron balance via partial manual RBCX. Disclosures: Kuo: Novartis Canada: Research Funding.

Transfusion and Iron Chelation Therapy in Thalassemia and Sickle Cell Disease

2010 ◽  
pp. 689-744 ◽  
Author(s):  
Janet L. Kwiatkowski ◽  
John B. Porter ◽  
Martin H. Steinberg ◽  
Bernard G. Forget ◽  
Douglas R. Higgs ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Iron Chelation Therapy ◽  
Cell Disease

Serum Ferritin Elevation and Use of Iron Chelation In Chronically Versus Sporadically Transfused Sickle Cell Patients

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 2671-2671
Author(s):  
Ismael Shaukat ◽  
Faraz Khan ◽  
Andrew Eisenberger ◽  
Marcus Stevenson ◽  
Alice J. Cohen
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Renal Dysfunction ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Iron Chelation Therapy ◽  
Cell Disease ◽  
The Mean

Abstract Abstract 2671 Background: Red cell transfusions play an integral role in the treatment and prevention of serious complications related to sickle cell disease. It has been shown that in other hemoglobinopathies, such as β-Thalassemia, patients (pts) suffer from iron overload which can result in end organ damage. There is concern that heavily transfused sickle cell pts may also develop iron overload with consequent morbidity and mortality. While pediatric pts routinely receive blood transfusions and iron chelation therapy, adult pts often discontinue chronic transfusion programs and are transfused sporadically. These pts may not receive routine iron chelation therapy. Methods: A retrospective review of our sickle cell database from 1988–2010 which also included those pts who were not routinely followed at the comprehensive sickle cell clinic. Adult pts (>18 yrs of age) with serum ferritin (SF) levels >1000 ng/ml (criteria for iron overload in our institution) were identified and use of iron chelation was reviewed in this population. Clinical characteristics evaluated were age, type of sickle cell disease, frequency of transfusions (chronic vs. sporadic), total units transfused, use and type of chelation, as well as reasons for non-use of chelation therapy. Results: 65/170(38%) pts were identified with SF >1000. The mean age is 33 years (range 19–70). 38/65 (59%) have the SS phenotype, 25/65 (38%) have the Sβ phenotype and 2/65 (3%) have the SC phenotype. The mean SF is 3697 ng/ml (range 1012–14312). Of those pts considered to have iron overload, 28/65 (43%) were treated with iron chelation: 27/65 (42%) received deferasirox and 1/65 (2%) received deferoxamine. Of the untreated pts, 24/37 (65%) had no identifiable reason for lack of chelation therapy, 10/37 (27%) had renal dysfunction, 1/37(3%) had hepatic impairment. 16/65 (25%) were transfused chronically, while 49/65 (75 %) were transfused sporadically. Chronically transfused pts received a mean of 81 units throughout their lifetime, while sporadically transfused pts received 30 units (p=0.01). The mean SF for chronically transfused pts was 5891, while the mean SF for pts transfused sporadically was 2981 (p=0.01). Of pts transfused chronically, 11/16 (69%) were on chelation therapy. Of the pts receiving sporadic transfusions, only 16/49 (33%) were on iron chelation (p= 0.01). In all pts chronically transfused, the reason for non-use of chelation therapy was renal dysfunction. In sporadically transfused pts, 33/49 (51%) had no identifiable reason for lack of chelation therapy. Conclusion: SF levels are significantly lower in pts who are sporadically transfused, though levels are high. Adult pts receiving sporadic transfusions are not routinely receiving iron chelation therapy despite elevated SF. The need for chelation therapy in both sporadically and chronically transfused pts remains to be determined. Disclosures: No relevant conflicts of interest to declare.

Characteristics of Sickle Cell Patients with Frequent ED Visits and Hospitalizations: Demographic, Clinical, Laboratory and Psycho-Social Aspects

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 2501-2501 ◽  
Author(s):  
Kyle Michael Kidwell ◽  
Camila Albo ◽  
Michael Pope ◽  
Latanya Bowman ◽  
Hongyan Xu ◽  
...  
Keyword(s):  
Health Care ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Laboratory ◽  
Anxiety And Depression ◽  
Cell Disease ◽  
High Utilizers ◽  
The Us ◽  
Ed Visits ◽  
High Utilizer

Abstract Vaso-occlusive episodes (VOE), are considered a hallmark of sickle cell disease (SCD), and account for >90% of health care encounters for this patient population. Although an orphan disease (total number of patients in the US ~110,000), SCD has a huge medical-economic impact, with annual hospitalization costs of nearly $ 0.5 billion by 2004 figures, mostly for VOEs. The Cooperative Study of Sickle Cell Disease (CSSCD) was a large natural history study, carried in 23 Centers in the US, enrolling >3000 patients between 1977-93. An analysis of the frequency of VOEs in CSSCD showed that the majority of patients (80%) experienced 0-3 major pain crises/year; this was similar across all genotypes of the disease (SS, SC, Sβ0 thalassemia, Sβ+ thalassemia). Only a small minority (~5%) experienced ≥6 VOEs/year. Similar findings have been reported in subsequent, smaller studies, with 5-10% of SCD patients with frequent ED visits and hospitalizations. Hydroxyurea (HU), the only disease modifying agent approved for adults with SCD, results in a ~50% reduction in the frequency of VOEs and hospitalizations. Despite this, HU is underutilized in SCD. Recent NIH evidence based guidelines includes strong recommendations for HU therapy in the majority of SCD patients. Although 230 of the 650 adult SCD patients at our Center are prescribed HU, in a recent study we found that ~40% of these patients were non-adherent, based upon their own admission, and/or the lack of anticipated change in several laboratory parameters (Hb F, MCV, Hb, WBC, reticulocyte and platelet count). A minority of SCD patients followed at our Center have frequent ED visits and hospitalizations. We conducted a study analyzing demographic, clinical, laboratory and psycho-social characteristics of 25 patients with ≥6 ED visits and hospitalizations per year (high utilizers), and compared these with 9 patients (controls) who experienced ≤2 ED visits/hospitalizations (low utilizers). Data on gender, age, genotype, number of ED visits and hospitalizations, CBC, chemistry panel, Hb F, HU usage, opioid use (morphine equivalents), education level, employment, annual income and marital status were collected. All subjects were also administered a depression, anxiety and Health Locus of Control Questionnaire, and underwent quantitative sensory testing (QST) with three different modalities: pressure pain sensitivity with a hand held, computerized algometer (AlgoMed, Medoc, Israel), heat and cold sensitivity (Q-Sense, Medoc, Israel) and Von Frey monofilament testing for neuropathic pain. Of the 25 patients (13M, 12F), 19 were SS, 4 SC, 1 SD-Los Angeles and 1 Sdβ-thalassemia. The median age was 28 (range 21-49). In the low utilizer group (6M, 3F) all were SS with a median age of 39 (range 23-46). The average number of hospitalizations in the high utilizer group was 15.6/year (range 6-33), while in the low utilizer group it was 0.44/year. Annual opioid usage as mg morphine equivalents was significantly higher in the high utilizers (12125.7 mg vs 2423.1 mg, p=0.0048). 19/25 (76%) of high utilizers, and 7/9 (78%) of low utilizers were on HU. Laboratory data on both groups are shown in Table 1. High utilizers had significantly higher WBC, ANC, total bilirubin and lower MCV. Hb F was also lower in the high utilizers (11.8% vs 17.5%) although this did not reach significance. QST results showed significantly lower pressure pain threshold at ulna (224.4 KPa vs 338.9 KPa, p=0.04), and higher baseline sensitivity with von Frey monofilament (0.92 vs 0.33, p=0.056) in the high utilizer group. High utilizers also had a higher anxiety score (9.0 vs 4.6, p=0.039) and depression score (10.0 vs 6.0, p=0.051). While the low utilizers had higher education levels with more associate and bachelor degrees (p=0.009), there was no difference in household income. These data show that multiple factors contribute to high health care utilization in SCD; these include biologic (younger age, disease severity as indicated by higher WBC and ANC, bilirubin, opioid induced hyperalgesia, and poor adherence to HU) as well as psycho-social factors (higher anxiety and depression scores, lower educational status). A multi-disciplinary and multi-faceted approach, including a sound patient education and transition program, a concerted effort to increase adherence to HU therapy, and psycho-social as well as pharmacologic approaches to anxiety and depression will be required to address this complex problem. Disclosures Kutlar: Novartis Pharmaceuticals: Research Funding.

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