Hydroxyurea Use in Sickle Cell Disease Patients in a Florida Medicaid Population.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 79-79 ◽  
Author(s):  
Jane N. Ritho ◽  
Dionne Y. Mayhew ◽  
Abraham G. Hartzema ◽  
Huazhi Liu ◽  
Richard Lottenberg
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Slow Release ◽  
Medication Possession Ratio ◽  
Pharmacy Claim ◽  
Early Therapy ◽  
Number Of Patients ◽  
Medicaid Population ◽  
Pain Crisis ◽  
Opioid Medications

Abstract Background: Efficacy of hydroxyurea (HU) was demonstrated in the Multicenter Study of Hydroxyurea in Sickle Cell Anemia (MSH) placebo-controlled randomized clinical trial with a reduction in average crisis rate (weighted mean difference −2.80; CI −4.74 to −0.86). HU was FDA-approved in 1998 for treatment of adults with sickle cell anemia experiencing recurrent painful episodes. An observational 9 year follow-up study of the MSH cohort demonstrated improved survival for patients taking HU. Purpose: To examine adoption and utilization of HU in SCD patients in a Medicaid population. Methods: A retrospective cohort study was conducted using Florida Medicaid eligibility, medical and pharmacy data for January 1, 2001 to December 31, 2005. The Medicaid database consists of medical and outpatient pharmacy utilization and reimbursement claims. SCD patients aged between 16 and 64 years with at least one inpatient or two outpatient SCD diagnosis claims (ICD-9-CM 282.6x), and meeting continuous eligibility criteria were included. HU adoption was determined by the presence of at least one HU pharmacy claim using National Drug Codes. Adherence to HU was calculated using the medication possession ratio (MPR) defined as the cumulative daily dose dispensed (excluding the last prescription refill and hospitalizations) divided by the time period between the first and last HU prescription (Rx) dispensed. Descriptive and bi-variate analyses were used to assess the relationship between patient characteristics, treatment and utilization of medical resources. Results: The mean age of the 2,301 SCD patients identified is 25 years ± 10.9(SD). Of those, the majority were female (64%) and younger than 25 years of age (60%). During the study eligibility period, 72% had at least one SCD-related emergency department visit, 88% at least one hospitalization and 53% at least one inpatient claim for SCD with pain crisis. During the study period, 33.4% of the patients had ≥ 3 hospitalizations for SCD with pain crisis in any 12 month period. Approximately one-third of patients had red blood cell transfusions (36%) but only 4.4% had a claim for iron-chelation. Of all SCD patients 26% used outpatient opioid medications with 65.4% receiving slow-release formulations. Nearly 17% of the cohort (n=384) had at least one pharmacy claim for HU. Compared to non-HU users, HU users were more likely to be males (OR 1.79; CI 1.44–2.23), aged ≥ 25 years (OR 1.35; CI 1.08–1.71), with a history of using slow-release opioid medications (OR 5.95; CI 4.65–7.59) or receiving red cell transfusions (OR 4.21; CI 3.35–5.31). Of those SCD patients eligible to receive HU according to the MSH criteria (≥ 3 hospitalizations a year for SCD crisis), only 38% received at least one HU Rx (OR 11.78, CI: 8.26–16.80). For those patients receiving at least two HU Rx, only 30.2% had a MPR of ≥ 0.60 (see Table). Conclusions: The prevalence of HU use in this Medicaid population is low. Our results suggest that only a small subset of SCD patients receive HU prescriptions consistently. Early therapy drop out and low adherence rates are common in patients prescribed HU. Interventions to promote physician adoption and prescribing of HU are needed, as are efforts to increase patient adherence. HU Possession Ratio Number of patients (%) 0 – < 0.2 69 (28.6) 0.2 – < 0.4 46 (19.1) 0.4 – < 0.6 53 (22.0) 0.6 – < 0.8 36 (14.9) > 0.8 37 (15.4) Total 241 (100)

Epidemiology and Risk Factors for Pain In Children and Adolescent with Sickle Cell Anemia.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1651-1651 ◽  
Author(s):  
Deepika Darbari ◽  
Onyinye Onyekwere ◽  
Mehdi Nouraie ◽  
Gregory J. Kato ◽  
Caterina Minniti ◽  
...  
Keyword(s):  
Risk Factors ◽  
Confidence Interval ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Odds Ratio ◽  
Severe Pain ◽  
History Of ◽  
Pain Crisis ◽  
Thalassemia Trait

Abstract Abstract 1651 Background: Pain crises are the most common symptom experienced by individuals with sickle cell anemia (SCA). Frequency of pain crises varies significantly and high rate is a risk factor for higher mortality in adults with SCA. The risk factors for pain crises in children and adolescents with SCA are not completely understood. To determine factors associated with frequent severe pain crises, we analyzed the cohort of children and adolescents with SCA who were enrolled in the prospective study “The Pulmonary Hypertension and the Hypoxic Response in SCD (PUSH)”. All children were evaluated in their steady, non-crisis state. Methods: Family-reported history of number of severe pain crises in the preceding 12 months was recorded prospectively in 365 children and adolescents with SCA. Severe pain crises were defined as painful vaso-occlusive episodes requiring evaluation in Emergency Department (ED) or in-patient hospitalization. Lifetime history of red cell transfusions, echocardiography, and laboratory studies were obtained. Clinical and laboratory characteristics of study subjects who had ≥3 severe pain crises in the preceding year were compared to subjects with < 3 severe pain crises. Results: Study subject ranged in age from 3–20 years and 175 (48%) were female. Seventy two children (20 %) had ≥3 severe pain crises in the preceding year (frequent pain crisis group); 293 (80%) children had < 3 severe pain crises (infrequent pain crisis group), including 224 (61%) subjects who had no admissions/ ED visits for pain. Associated factors for frequent pain crisis included older age (odds ratio 1.2; 95% confidence interval 1.12–1.35; P <0.001) and α-thalassemia trait (odds ratio 3.22; 95% confidence interval 1.55–6.69; P =0.002) while higher steady state serum lactate dehydrogenase (LDH) was associated with infrequent pain crisis (odds ratio 0.35; 95% confidence interval 0.13–0.98; P =0.045). In a group of patients without α-thalassemia trait, older age and low LDH were linked to frequent pain crisis. Subjects in the frequent pain crisis group had higher median hemoglobin (9.0 vs. 8.5 gm/dL; p=0.003) and higher ferritin (median 455 vs. 191 ng/mL; p=0.008). Higher ferritin in the frequent pain crisis group was mirrored by the higher percentage who reported >10 lifetime transfusions (42% vs. 22%; p=0.001). Median tricuspid regurgitation jet velocity (TRV) was higher in the frequent pain crisis group (2.41 vs. 2.31; p= 0.001) but the proportion of children with TRV>2.5 was not different (19.4% vs.11.5% in infrequent crises group; p=0.09). Hydroxyurea use was not different between the groups (51% vs. 40%; p=0.08) nor was fetal hemoglobin (10% vs. 12%; p=0.2). Conclusions: The occurrence of severe pain crisis varies among children and adolescents with SCA with a large number of children experiencing no severe painful episodes. Consistent with the Cooperative Study of Sickle Cell Disease report, the risk of severe pain crisis increases with age. Individuals with α-thalassemia trait are likely to experience more frequent pain crises possibly related to higher hemoglobin concentration and viscosity. However, even after controlling for α-thalassemia trait, children and adolescents who reported more frequent severe pain crises showed evidence of less hemolysis, consistent with a hypothetical model associating the hemolytic subphenotype of SCA with less frequent vasoocclusive pain crises. Further studies are indicated to identify the molecular mechanisms of pain in sickle cell anemia. Disclosures: No relevant conflicts of interest to declare.

Health Care Utilization for Painful Events Is Associated with Early Mortality in a Contemporary Population of Adults with Sickle Cell Anemia

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2115-2115
Author(s):  
Deepika S. Darbari ◽  
Mariana Hildeshem ◽  
Caterina Minniti ◽  
Gregory J. Kato ◽  
James G. Taylor
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Severe Pain ◽  
Early Mortality ◽  
Care Utilization ◽  
Cell Disease ◽  
History Of ◽  
Pain Crisis ◽  
Ed Visits ◽  
Painful Events

Abstract Abstract 2115 Background: Painful vaso-occlusive crises are the most common symptom associated with sickle cell anemia (SCA) and show a large inter-patient variability in the frequency and severity. Historically a high rate of admissions for pain is associated with early mortality in SCA adults. Modern day management of SCA includes screening for life threatening complications such as pulmonary hypertension and disease modifying therapies such as hydroxyurea and chronic blood transfusions. It has also become clear that all acute painful events including some self reported crisis do not lead to ED visit/ hospitalization therefore use of healthcare utilization may underestimate the actual frequency of painful vaso-occlusive crises. The present study determined if ED visit/ hospitalizations for painful vaso-occlusive crises remain a marker of early mortality in the modern era. Methods: To determine the relative contribution of pain and other established risk factors for death in a contemporary adult cohort with sickle cell anemia, we analyzed data of SCA individuals who were evaluated at NIH between February 2001 and June 2007 for screening of the IRB approved protocol to study pulmonary hypertension in sickle cell disease. Self reported history of vaso-occlusive painful events requiring visit to an emergency department (ED) or in-patient hospitalization in 12 months preceding the enrollment was used to stratify study subjects into no-ED visit/ hospitalization or ED visit/hospitalization group. Characteristics between the groups were compared. Doppler echocardiography was used to estimate systolic pulmonary artery pressure through measurement of the tricuspid regurgitation velocity (TRV). Study subjects were followed prospectively and age at death was recorded. Results: One hundred and four (40%) SCA subjects reported no ED visit/ hospitalization while 160 (60%) reported at least one ED visit/ hospitalization in 12 months preceding the enrollment. Although not statistically significant, a higher proportion of study participants in the ED visit/ hospitalization group were on hydroxyurea (38 vs. 69%; P=0.4), and more likely to have received > 10 red cell transfusions (37 vs. 48%; P= 0.09). The no-ED/ hospitalization group and ED/ hospitalization groups were not different in median age (33 vs. 31; P=0.1) or fetal hemoglobin concentration (7.7 vs. 6.9%; P=0.3). Overall forty (15.1%) individuals died during the median follow-up period of 5 years. Cox proportional hazards analysis was performed to determine if severe pain crisis requiring ED visits/hospitalizations were associated with early mortality after adjusting for other known risk factors for mortality (Table 1). Risk for mortality was significantly associated with a history of severe pain crisis requiring ED visit/hospitalization in preceding year (RR 2.81, 95% CI: 1.2–6.4, P= 0.04), elevated TRV ≥ 3 m/s (RR 4.07, 95% CI: 1.3–12.8, P= 0.02) and elevated ferritin (RR 2.31, 95% CI: 1.0–5.0, P= 0.04). Conclusions: Severe painful episodes requiring health care utilization are associated with premature mortality and despite likely underestimation of actual frequency of pain, remain a marker of disease severity. Elevated TRV a marker of elevated pulmonary arterial pressure and early mortality has a cumulative effect with pain on reduced survival. Consistent with Cooperative Study of Sickle Cell Disease (CSSCD) report, higher hemoglobin (and lower LDH) was associated with reports of ED visit/ hospitalization likely related to increased viscosity. We conclude that as a group, SCA patients reporting severe pain requiring ED visits /hospitalization and elevated TRV are at the highest risk for shortened survival. Disclosures: No relevant conflicts of interest to declare.

2-deoxy 5-azacytidine and fetal hemoglobin induction in sickle cell anemia

Blood ◽  
2000 ◽  
Vol 96 (7) ◽  
pp. 2379-2384
Author(s):  
Mabel Koshy ◽  
Louise Dorn ◽  
Linda Bressler ◽  
Robert Molokie ◽  
Donald Lavelle ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Complete Blood Count ◽  
Fetal Hemoglobin ◽  
Therapeutic Benefit ◽  
Additional Treatment ◽  
Acute Chest Syndrome ◽  
F Cells ◽  
Pain Crisis ◽  
Globin Synthesis

Augmentation of the fetal hemoglobin (HbF) levels is of therapeutic benefit in patients with sickle cell anemia. Hydroxyurea (HU), by increasing HbF, lowers rates of pain crisis, episodes of acute chest syndrome, and requirements for blood transfusions. For patients with no HbF elevation after HU treatment, augmentation of HbF levels by 5-aza-2′-deoxycytidine (5-aza-CdR, decitabine) could serve as an alternate mode of treatment. Eight adult patients participated in a dose-escalating phase I/II study with 5-aza-CdR at doses ranging from 0.15 to 0.30 mg/kg given 5 days a week for 2 weeks. HbF, F cell, F/F cell, γ-globin synthesis ratio, complete blood count, and chemistry were measured. The average γ-globin synthesis relative to non-α-globin synthesis prior to therapy was 3.19% ± 1.43% and increased to 13.66% ± 4.35% after treatment. HbF increased from 3.55% ± 2.47% to 13.45% ± 3.69%. F cells increased from 21% ± 14.8% to 55% ± 13.5% and HbF/F cell increased from 17% to 24%. In the HU nonresponders HbF levels increased from 2.28% ± 1.61% to 2.6% ± 2.15% on HU, whereas on 5-aza-CdR HbF increased to 12.70% ± 1.81%. Total hemoglobin increased by 1 g/dL in 6 of 8 patients with only minor reversible toxicities, and all patients tolerated the drug. Maximum HbF was attained within 4 weeks of treatment and persisted for 2 weeks before falling below 90% of the maximum. Therefore 5-aza-CdR could be effective in increasing HbF in patients with sickle cell anemia who failed to increase HbF with HU. Demonstration of sustained F levels with additional treatment cycles without toxicity is currently being performed.

scholarly journals Chronic Opioid Use Pattern in Adult Patients with Sickle Cell Disease

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 3400-3400 ◽  
Author(s):  
Jin Han ◽  
Santosh L. Saraf ◽  
Xu Zhang ◽  
Michel Gowhari ◽  
Robert E. Molokie ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Hospital Admissions ◽  
Patient Characteristics ◽  
Clinic Visit ◽  
Opioid Use ◽  
Opioid Medication ◽  
Opioid Dose ◽  
Number Of Patients ◽  
Chronic Opioid Use ◽  
Opioid Medications

Abstract Background: Pain, the hallmark complication of sickle cell disease (SCD), is largely managed with opioid analgesics in the United States (1, 2). There is a common perception that SCD patients tend to use high dose of opioids chronically (3, 4), but comprehensive data regarding the long-term use of opioids in this patient population is lacking. Methods: A cohort of 359 adults (age ≥ 18 years old) with the diagnosis of SCD followed at University of Illinois Hospital in FY 2010-2013 was enrolled in a prospective natural history study. An outpatient clinic visit with comprehensive laboratory closest to the enrollment date was selected as the focal point of this report. A total of 140 patients had consistent outpatient follow-up defined as ³4 visits between 6 months prior to and 6 months after this clinic visit (the study period). Pain medication prescribing records, number of hospital admissions, and other clinical variables during the study period were collected and evaluated with descriptive statistics and Spearman correlation. Results: Among the 140 patients analyzed, 74% took short-acting opioid medications and 31% took long-acting opioid medications (Table 1). The median daily opioid dose was 4.8 mg oral morphine equivalents (OME) with an interquartile range (IQR) of 0 to 14.7 mg. Sixty-six percent of patients used less than 10 mg OME daily whereas 11% used more than 50 mg OME daily (Figure 1). Among the short-acting opioids, acetaminophen-hydrocodone was the most commonly used medication (34% of patients) followed by immediate-release morphine (23%) and acetaminophen-codeine (20%) (Table 2). Extended-release morphine was used by 24% of the patients. Twelve patients (8.6%) were prescribed with nonsteroidal anti-inflammatory drugs (NSAIDs), and 16% used medications treating neuropathic pain. The Spearman correlation test with Bonferroni correction showed that the dose of opioid usage was significantly associated with the number of hospital admissions due to vaso-occlusive crisis (VOC) (p<0.0001) (Table 3). Hydroxyurea use, hemoglobin genotype, history of avascular necrosis (AVN) and 25-OHD levels did not correlate with opioid dose. Summary: The doses and types of opioid medications used by adult SCD patients vary widely. The majority of patients use a relatively low dose (<10 mg OME daily). The dose of chronic opioid use has a strong correlation with the frequency of hospital admissions due to VOC, demonstrating the necessity of finding an alternative pain management approach in treating sickle cell pain. 1. L. R. Solomon, J Natl Med Assoc102, 1025 (Nov, 2010). 2. P. Tanabe, Z. Martinovich, B. Buckley, A. Schmelzer, J. A. Paice, J Emerg Nurs41, 227 (May, 2015). 3. W. T. Zempsky, JAMA302, 2479 (Dec 9, 2009). 4. B. S. Shapiro, L. J. Benjamin, R. Payne, G. Heidrich, J Pain Symptom Manage14, 168 (Sep, 1997). The relative frequency = the number of patients taking certain dose of opioids/total number of patients. Table 1. Patient Characteristics. Table 1. Patient Characteristics. Figure 1. Frequency of Opioid Usage in SCD Patients. Figure 1. Frequency of Opioid Usage in SCD Patients. Table 2. Opioid Medication Usage Pattern by SCD Patients. Table 2. Opioid Medication Usage Pattern by SCD Patients. Disclosures No relevant conflicts of interest to declare.

scholarly journals MBL2 gene polymorphisms are not related to the occurrence of cerebrovascular disease in sickle cell anemia

2020 ◽  
Vol 9 (7) ◽  
pp. e439974240
Author(s):  
Isabela Cristina Cordeiro Farias ◽  
Taciana Furtado Mendonça-Belmont ◽  
Patrícia Muniz Mendes Freire Moura ◽  
Igor Farias Domingos ◽  
Diego Arruda Falcão ◽  
...  
Keyword(s):  
Cerebrovascular Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Promoter Region ◽  
Gene Polymorphisms ◽  
Control Group ◽  
Mbl2 Gene ◽  
Number Of Patients ◽  
Exon 1 ◽  
The Impact

Objective: This study has as objective to verify whether MBL2 gene polymorphisms are related to the occurrence of cerebrovascular disease (CD) in sickle cell anemia (SCA) patients. Methods: Overall, 259 unrelated SCA patients were enrolled. The patients were divided into three groups: control group, stroke group ad range of risk group. Peripheral blood samples were collected and DNA extraction was performed. All patients were genotyped for exon 1, promoter region -221 and promoter region -550 of MBL2 gene, along with β-globin gene haplotypes. Results: Concerning the genotyping of the MBL2, there was no difference in the frequency of allelic and genotypic variants of the exon 1 and the promoter regions -221 and -550 of the MBL2 gene among the studied groups. Conclusion: Despite the small number of patients, and the lack of association between MBL2 polymorphisms and CD, our study represents an effort to understand the impact of MBL2 polymorphisms in the clinical outcome of patients with SCA.

Academic Community Standards for Chronic Transfusion Therapy In Children with Sickle Cell Anemia and Abnormal Transcranial Doppler Velocities

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 2643-2643
Author(s):  
Banu Aygun ◽  
Lisa Wruck ◽  
William Herbert Schultz ◽  
Isaac Odame ◽  
R. Clark Brown ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Transcranial Doppler ◽  
Hemoglobin Concentration ◽  
Transfusion Practice ◽  
Academic Community ◽  
Eligibility Criteria ◽  
Transfusion Therapy ◽  
Number Of Patients ◽  
Community Standards

Abstract Abstract 2643 Children with sickle cell anemia (SCA) and abnormal transcranial Doppler (TCD) velocities receive chronic transfusion therapy in an effort to prevent a primary stroke. Typically the goal is maintaining sickle hemoglobin (HbS) <30%, however this goal may be difficult to achieve in actual clinical practice. The NHLBI-sponsored TCD With Transfusions Changing to Hydroxyurea (TWiTCH) trial will compare standard therapy (transfusions) with alternative therapy (hydroxyurea) for the prevention of primary stroke. The transfusions will be given according to current transfusion practices at the participating academic sites. To determine the current academic community standards for primary stroke prophylaxis in children with SCA, 32 potential clinical sites collected data for children with abnormal TCD velocities who are receiving chronic transfusion therapy to prevent a primary stroke. The eligibility criteria included all children with SCA, age 4.0–15.9 years who were on chronic transfusion therapy to prevent a primary stroke due to previous abnormal TCD examination. After IRB-approval, subject and transfusion-related data were collected for all TWiTCH-eligible patients over the 12-month period from 9/1/2008 to 8/31/2009. Variables included year of birth, gender, year chronic transfusions began, TCD velocities that led to transfusion therapy, weight, %HbS transfusion goal, type and volume, pre-transfusion hemoglobin concentration and %HbS, and interval between transfusions. Data were analyzed both “per transfusion” and “per patient.” A total of 3970 transfusions were administered to 340 pediatric patients (mean approximate age at time of survey 10.2 ± 4.1 years, M: F = 0.9:1) over the 12-month period, with a mean of 11.6 ± 2.8 transfusions per patient. The average of the highest TCD velocity that led to transfusion therapy was 221 ± 27 cm/sec. Maximum velocities for right and left hemispheres were 201.2 ± 35.3 and 207.7 ± 31.8 cm/sec, respectively, supporting equal susceptibility. The mean approximate age at the start of transfusion therapy was 6.2 ± 2.6 years with average transfusion duration of 4.0 years. Most children (79%) received primarily simple transfusions, while 19% had primarily exchange transfusions (11% manual and 8% automated), and 2% multiple transfusion types. The transfusion goal was HbS <30% at almost all sites (84%). An equal number of patients developed erythrocyte alloantibodies (13.6%) and autoantibodies (13.7%). Transfusions were considered late if administered later than 7 days beyond the scheduled date. On average, late transfusions were given 1.3 ± 5.5 days after the 7 day allowable window. The average pre-transfusion hemoglobin concentration was 9.0 ± 0.9 gm/dL. The average pre-transfusion %HbS was 34.1 ± 11.2%, with a median value of 32.9%. The 75th percentile for HbS values was 40.9%, while the 90th percentile was 49.5%. There were profound differences among institutional pre-transfusion %HbS values, ranging from 23 ± 14% HbS at one institution where 103 transfusions were given to 9 patients during the 12-month period to 48 ± 15% at another institution where 95 transfusions were administered to 9 patients during the same time frame. These data indicate that current transfusion practice to prevent primary stroke varies among academic pediatric institutions, and 30%HbS is not an easily attainable goal for the TWiTCH study that includes chronic transfusion therapy within the Standard Arm. As a result of this analysis, the TWiTCH study will recommend chronic transfusions with the goal of maintaining HbS <30%, but only record a protocol violation when the HbS value exceeds 45%. Disclosures: Off Label Use: It will include the use of hydroxyurea in children with sickle cell anemia.

Erythropoietin Levels in Patients with Sickle Cell Disease Not in Vaso-Occlusive Crisis

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3242-3242
Author(s):  
Dianne Pulte ◽  
Srikanth Nagalla ◽  
Jaime Caro
Keyword(s):  
Sickle Cell Disease ◽  
Kidney Function ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Hemoglobin Level ◽  
Laboratory Evaluation ◽  
Cell Disease ◽  
Chronic Anemia ◽  
Number Of Patients ◽  
Erythropoietin Level

Abstract Abstract 3242 Background: Previous studies of erythropoietin production in sickle cell disease have shown that patients with sickle cell disease generally have an erythropoietin level in the lower end of the expected range. However, this finding was based on a relatively small number of patients including some who were in crisis when the erythropoietin level was drawn. A crisis might affect erythropoietin level by increasing inflammation beyond background and if the patient is transfused, the erythropoietin may not be well correlated with the apparent hemoglobin or hematocrit. In this study, we examine erythropoietin levels in patients with sickle cell disease seen in the outpatient clinic and not in crisis when the level was drawn. Methods: Patients receiving outpatient care at the Thomas Jefferson University Cardeza Sickle Center underwent laboratory evaluation as part of routine standard of care for their disease. Hemoglobin level, creatinine, erythropoietin level, lactate dehydrogenase, and reticulocyte count were extracted anonymously from patient charts for evaluation of erythropoietin in sickle patients in the outpatient setting without crisis and correlation of level with kidney function, hemoglobin level, and ongoing hemolysis. Results: Forty-eight measurements of erythropoietin from 39 unique patients were extracted. Two were not included as chart review suggested that the patient was in crisis when the values were obtained. Of the remaining 36 patients, three patients had SC disease, four sickle-thalassemia, the rest sickle cell anemia. Three patients received exogenous erythropoietin for hypoplastic anemia or chronic renal failure complicating sickle cell disease. Of the remaining 30 patients, creatinine ranged from 0.2–1.8. Erythropoietin was higher than the normal range for most patients, as would be expected in a chronic anemia. Erythropoietin level was roughly correlated with hemoglobin, but some patients had erythropoietin levels much lower than expected for their hemoglobin (figure). Erythropoietin was not well correlated with creatinine or with hemolysis. Hemoglobin was higher for patients with SC disease than for sickle cell anemia or sickle-thalassemia, but erythropoietin levels were not significantly different between disease types. Conclusions: Patients with sickle cell disease who are not in crisis have erythropoietin levels that are elevated, but lower than expected for a healthy patient with chronic anemia, with a greater lack in patients with sickle cell anemia as compared to SC disease. Erythropoietin did not correlate well with kidney function as measured by creatinine or calculated glomerular filtration rate, suggesting that current measures of kidney damage may not be entirely adequate to detect early kidney disease in sickle cell disease. Disclosures: No relevant conflicts of interest to declare.

Prognostic Significance of Early Vaso-Occlusive Complications in Children Who Have Sickle Cell Anemia.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3176-3176
Author(s):  
Charles T. Quinn ◽  
Elizabeth P. Shull ◽  
Naveed Ahmad ◽  
Zora R. Rogers ◽  
George R. Buchanan
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Strong Predictor ◽  
Prognostic Significance ◽  
Adverse Outcomes ◽  
Acute Chest Syndrome ◽  
Disease Modifying Therapy ◽  
Pain Crisis ◽  
Disease Modifying

Abstract Sickle cell anemia (SS) is a phenotypically variable disease whose course is difficult to predict. The Cooperative Study of Sickle Cell Disease (CSSCD) found that dactylitis in the 1st year of life predicted adverse outcomes in later childhood. We aimed to determine whether early vaso-occlusive complications, including dactylitis, were prognostic in the Dallas Newborn Cohort. We studied all cohort members with SS or sickle-β0-thalassemia who were &lt;1 yr of age at their first clinic visit, ≥5 yrs of age at last follow-up, and who had complete records. We defined 3 potential “early” (occurring in the first 3 yrs of life) predictors: any hospitalization for (1) pain crisis (non-dactylitis), (2) dactylitis, or (3) acute chest syndrome (ACS). We studied the associations of these predictors with the following “late” (occurring on or after the 3rd birthday) outcomes: death of any cause; overt stroke; use of hydroxyurea (HU), chronic transfusion (CT), or stem cell transplantation (SCT); and mean number of hospitalizations for late pain crisis and ACS. Late pain and ACS episodes were enumerated for each patient between the 3rd birthday and the last clinical encounter or the start date of a disease-modifying therapy (HU, CT, or SCT), whichever was first. Mean number of pain and ACS events was analyzed for the late follow-up period in total and in 2-yr intervals. Outcomes up to age 20 were included. Two-sided Fisher exact and t-tests were used appropriately. There were 264 subjects (256 SS; 54.9% male). Mean age at first visit was 4.1±2.3 mos (±S.D.) and mean follow-up was 12.1±4.3 yrs. The following early hospitalizations occurred: 53 subjects (20.1%) had pain crisis; 16 (6.1%) had dactylitis, and 85 (32.9%) had ACS. There were 5 deaths and 30 overt strokes. Sixty-six subjects were treated with HU (37), CT (40), and/or SCT (1). We found that subjects who had early pain, dactylitis, or ACS (compared with those who did not) were not more likely to die (1.7 vs. 2.1%; P&gt;0.99) or have a stroke (12.2 vs. 10.3%; P=0.69). However, the use of a disease-modifying therapy was more common among subjects who had early pain (37.7 vs. 19.9%; P=0.01) and ACS (37.6 vs. 16.2%; P&lt;0.001), but not dactylitis (18.8 vs. 23.6%; P&gt;0.99). This prediction held only for HU use when the treatments (HU, CT, or SCT) were analyzed separately. Subjects who experienced early pain or ACS had on average a 2.2-fold (P=0.02) or 2.1-fold (P=0.01), respectively, higher number of late pain crises between ages 3 and 11, but not beyond (all P&gt;0.05). Dactylitis did not predict a higher number of late painful events at any age (all P&gt;0.05). Likewise, neither early pain nor dactylitis was associated with a higher number of hospitalizations for late ACS (all P&gt;0.05). However, subjects who had early ACS had a 1.7 to 3.6-fold higher mean number of ACS events throughout all late age groups (all P&lt;0.05). In summary, early hospitalization for pain, dactylitis, or ACS did not predict death or stroke. Early pain and ACS were associated with use of HU in later childhood, but not CT or SCT. Early pain and ACS predicted an increased number of hospitalizations for pain until age 11, but not beyond. Early ACS was a strong predictor of recurrent ACS throughout childhood. Notably, we found that hospitalization for dactylitis had no particular prognostic significance, unlike the CSSCD. In conclusion, the prognostic significance of early vaso-occlusive complications is limited.

scholarly journals Dental care provided to sickle cell anemia patients stratified by age: A population-based study in Northeastern Brazil

2016 ◽  
Vol 10 (03) ◽  
pp. 356-360 ◽  
Author(s):  
Cyrene Piazera Silva Costa ◽  
Bárbara Tamires Cruz Aires ◽  
Erika Bárbara Abreu Fonseca Thomaz ◽  
Soraia de Fátima Carvalho Souza
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Dental Care ◽  
Dental Treatment ◽  
Age Groups ◽  
Secondary Data ◽  
Northeastern Brazil ◽  
Endodontically Treated Teeth ◽  
Number Of Patients ◽  
Dental Records

ABSTRACT Objective: To assess differences in the dental care provided to sickle cell anemia (SCA) patients depending on age. This retrospective study used secondary data from the dental records of the Center of Hematology and Hemotherapy in Maranhão (HEMOMAR). Materials and Methods: Data were obtained from 574 dental records of patients with SCA treated or under treatment in the Dental Department of HEMOMAR from 2000 to 2011. Data on the gender, age, duration of dental treatment, number of patients submitted to periodontal treatment (PT), number of filled teeth (FT), teeth extracted (EX), endodontically treated teeth (ET), and reason for the dental procedures were collected. The Kruskal–Wallis test together with Dunn's post hoc test, Chi-square test, and Spearman's correlation was used for statistical analysis. An alpha error of 5% was considered acceptable. Results: Significant differences were found for FT, EX (P < 0.05), ET and PT (P < 0.001) between the age groups. There were fewer FT in children compared to other age groups (P < 0.001). The most common reasons for restorations and endodontic treatment were dental caries (100%) and irreversible pulpitis (55.6%), respectively. The main reasons for teeth extractions were residual roots (21.3%), chronic apical periodontitis (19.7%), and crown destruction (19.3%). There were positive correlations between age and EX (r = 0.93; P = 0.025) and ET (r = 0.92; P = 0.028). Conclusions: FT, ET, EX, and PT procedures become more common in older patients. Tooth decay is the main reason for dental treatment in SCA patients.

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