Hospitalizations For Vaso-occlusive Crises Research Articles

The Associations Between Opioid Use Disorder and Healthcare-Related Outcomes in Vaso-occlusive Crisis.

In patients who experience frequent vaso-occlusive crises (VOC), opioid dependence may be due to a need for pain control as opposed to addiction; the implications of opioid use disorder (OUD) in this population are unclear. To compare outcomes in hospitalizations for VOC in those with a history of OUD to those without a history of OUD. A retrospective assessment of hospitalizations for adults in the USA with a primary discharge diagnosis of VOC using the National Inpatient Sample database from 2016 to 2019. We also compared VOC hospitalizations to hospitalizations for all other reasons to assess differences in OUD-associated clinical factors. In total, 273,460 hospitalizations for VOC; 23,120 (8.5%) of these hospital stays involved a secondary diagnosis of OUD. Primary outcomes were length of hospital stay and cost. Mortality was a secondary outcome. Hospital length of stay was increased (mean 6.2 vs 4.9days) in patients with OUD (adjusted rate ratio = 1.24, 95% CI 1.20-1.29, p < 0.001). Mean cost was also higher in those with OUD ($9076) than those without OUD ($8020, p < 0.001). Mortality was decreased in VOC hospitalizations in those with OUD, but the difference was not statistically significant (adjusted OR = 0.64, 95% CI 0.028-1.48, p = 0.30). OUD is associated with increased length of stay and costs in patients with VOC. While there are many possible explanations, providers should consider undertreatment of pain due to addiction concerns as a potential factor; individualized pain plans to mitigate this challenge could be explored.

Early Evaluation of the Use of Crizanlizumab in Sickle Cell Disease: A National Alliance of Sickle Cell Centers Study

Early Evaluation of the Use of Crizanlizumab in Sickle Cell Disease: A National Alliance of Sickle Cell Centers Study

Pulmonary hypertension and right ventricular function in Nigerian children with sickle cell anaemia.

BackgroundPulmonary hypertension (PH), a complication of sickle cell anaemia (SCA), results in considerable morbidity. This study aims to determine the prevalence and associations of echocardiography-suggested PH in children with SCA.MethodsWe performed a cross-sectional comparative study involving 100 systematically sampled SCA subjects 3–14 y of age in their steady state with matched haemoglobin AA phenotype controls. Clinical, laboratory and echocardiography data (including tricuspid regurgitation velocity [TRV], mean pulmonary arterial pressure [mPAP] and tricuspid annular plane systolic excursion [TAPSE]) were obtained from all patients. Statistical analyses were performed using SPSS version 22 (IBM, Armonk, NY, USA). A p-value <0.05 was considered statistically significant.ResultsOf the 100 SCA subjects studied, 22 (22%) had echocardiographic findings suggestive of PH compared with none in the controls. The median TAPSE was significantly lower in the PH group (2.55 cm [interquartile range {IQR} 2.2–2.8]) compared with the no PH group (2.77 cm [IQR 2.4–3.2]) (p=0.03). No significant correlation existed between mPAP and age, nor any laboratory parameters studied. The odds ratio (OR) suggested PH significantly increased with an increase in the frequency of hospitalizations for vaso-occlusive crises within a 12-month period (OR 15.15 [95% CI 1.57 to 146.35], p=0.02) and a lifetime history of blood transfusion (OR 5.44 [95% CI 1.09 to 27.24], p=0.04).ConclusionsEchocardiography-suggested PH is common in children with SCA and is associated with poorer right ventricular function, frequent vaso-occlusive crises and blood transfusions.

Opioid Utilization in Adults Hospitalized with Sickle Cell Pain Crises: An Understudied Population

Background: Vaso-occlusive crises (VOC), also known as painful crises, are the hallmark of sickle cell disease (SCD) and adults hospitalized with VOC are among the most severe cases. In the midst of the opioid epidemic, appropriate pain management must be available for these patients when they develop VOC. We describe patient, disease, and treatment characteristics at the time of hospitalization for patients with SCD experiencing VOC.Methods: This retrospective cohort study includes 449 consecutive hospitalizations for VOC among 63 adult patients (≥18 years) with SCD from 2013-2016 at Tufts Medical Center, an academic medical center in Boston, MA. For the current analysis, one hospitalization was randomly selected for each patient (median hospitalizations per patient=4, range=1-45). Demographics, SCD characteristics and complications, pain scores (0-10), and pain medication regimens (pre-admission, inpatient, discharge) were abstracted from electronic medical records (EMR) by trained study staff. History of SCD complications (e.g., acute chest syndrome, avascular necrosis), modeled after the 9-item ASCQ-Me SCD Medical History Checklist (Keller et al. Health and Quality of Life Outcomes, 2017), were obtained from the EMR and subsequently reviewed by two hematologists. Data were described using summary statistics, including means, standard deviations (SD) medians, quartiles (q1, q3), frequencies, and percentages.Results: Mean age was 27.6 years (SD=8.1), 44.4% were male, 77.8% were black, and 19.1% were Hispanic. The majority of patients (92.1%) were publicly insured (27.0% Medicare; 65.1% Medicaid); only 7.9% were privately insured. Patients had a median of 3 medical complications of SCD. Affective disorders were documented for 33.3%; substance use disorders for 6.4%.Median pain scores were 9 (8, 10) at admission and 4 (2, 6) at discharge; the median length of stay was 8 days (5, 11). Prior to admission, 87.3% of patients had a home opioid prescription and 20.6% had a neuropathic pain medication prescription (Table). During the hospitalization, 100% received opioids with 36.5% delivered via patient-controlled analgesia (PCA). Neuropathic pain medication was given to 28.6% of hospitalized patients. At discharge, nearly all (98.4%) were prescribed home opioids and 24.6% were discharged with neuropathic pain medication. With regards to treatment combinations across phases of care, approximately half of patients received both short- and long-acting opioids and less than 20% received long-acting opioids and neuropathic pain medication.Conclusions: All patients with SCD hospitalized with VOC were admitted with significant pain and treated with opioids. Pain decreased during the hospitalization, but did not fully resolve by discharge. Treatment combinations of short- and long-acting opioids and neuropathic pain medication suggest complex acute-on-chronic SCD pain with mixed pain types among this cohort of patients with high-severity SCD. Opioids are an integral component of treatment regimens for patients suffering from debilitating acute-on-chronic pain, such as those with SCD. DisclosuresParsons:Seattle Genetics: Research Funding.

Nucleated Red Blood Cells in Children with Sickle Cell Disease Hospitalized for Acute Vaso-Occlusive Pain

Background: Acute vaso-occlusive crises (VOC) represent the most common reason for hospitalization among children with sickle cell disease (SCD). Acute chest syndrome (ACS) and severe hemolysis, both of which may require transfusion, are common complications during hospitalizations for VOC. Despite the high morbidity associated with these complications, there are currently no reliable clinical predictors of ACS and transfusion requirement in this setting. Nucleated red blood cells (NRBC), reported as the number of NRBC/100 white blood cells (WBC) on a complete blood count (CBC), may reflect marrow necrosis or increased erythropoetic drive, yet their relationship to VOC and its complications have not been studied. We aimed to evaluate the relationship between admission NRBC and development of ACS or transfusion requirement in children with SCD hospitalized for VOC. We hypothesized that higher admission NRBC is associated with greater risk of ACS/transfusion in this population. Methods: We performed a single-institution, retrospective review of all hospitalizations from 2011 through 2015 for uncomplicated VOC in children with SCD. Hospitalization encounters were identified by ICD-9 codes for SCD and use of parenteral opioids for at least 24 hours in the electronic medical record. Data extracted from encounters meeting inclusion/exclusion criteria included all CBCs and clinical outcomes. Between-group comparisons were performed by Mann-Whitney U test and Pearson's chi-square test for continuous and categorical variables, respectively, as well as by Spearman's rank order correlation (SPSS V24). The relationship of admission and change in NRBC (∆NRBC) to complications was evaluated by binary logistic regression and adjusted for significant co-variates on bivariate analysis. For ∆NRBC, we used the final CBC obtained prior to a complication or prior to discharge for encounters not resulting in a complication. Results: We reviewed 271 encounters for uncomplicated VOC in 96 patients (mean age 13.1 years, 47% male, and 76% Hb SS or S/β0 thalassemia). Overall, 48/271 (18%) encounters for VOC resulted in ACS/transfusion, or both. Median NRBC on admission was 2 /100 WBC (range 0 to 137 /100 WBC) for all encounters. Admission NRBC was significantly correlated with age (r = 0.15, p = 0.02), % reticulocytes (r = 0.18, p &lt; 0.01) and hemoglobin (r = 0.39, p &lt; 0.001). Median admission NRBC was higher among patients on hydroxyurea (3 vs. 1.8 /100 WBC, p &lt; 0.01) but did not differ by sex or genotype. In general, median admission NRBC did not differ between hospitalizations that did or did not result in ACS/transfusion (2 vs 2.5 /100 WBC, p = 0.91). In our logistic regression model that included NRBC, % reticulocytes, WBC, hemoglobin, platelets and genotype as co-variates, only lower hemoglobin (p = 0.02) and higher WBC (p &lt; 0.001) on admission were independently associated with ACS/transfusion. A second CBC was obtained in 110/271 (41%) encounters reviewed. Median ∆NRBC was 0 /100 WBC (range -37 to 116 /100 WBC) in these encounters. ∆NRBC was significantly correlated with age (r = 0.2, p = 0.04) but not with change in other lab values. Median ∆NRBC was higher among patients with a history of hypertension (0 vs. 13 /100 WBC, p &lt; 0.01) but did not differ by hydroxyurea status, sex or genotype. Importantly, we found that median ∆NRBC was significantly higher in hospitalizations that resulted in ACS/transfusion compared to those that did not (0 vs 2 /100 WBC, p &lt; 0.01). By logistic regression, greater ∆NRBC (p &lt; 0.01) and lesser ∆platelets (p = 0.04) were independently associated with higher risk of ACS/transfusion after adjustment for ∆hemoglobin and genotype. A receiver operating characteristic curve constructed from a logistic regression model that included only ∆NRBC, admission WBC, admission hemoglobin and ∆platelets resulted in an area under the curve of 0.82. Conclusions: We found no relationship between admission NRBC and complications during hospitalization for VOC in children with SCD. Greater ∆NRBC, however, was independently associated with development of ACS/transfusion requirement during hospitalization for VOC, suggesting ∆NRBC may represent a useful biomarker for predicting complications in children with SCD hospitalized for VOC. Clinical decision rules that incorporate the careful monitoring of ∆NRBC in this setting should be prospectively studied. Disclosures No relevant conflicts of interest to declare.

Nutrient Insufficiencies/Deficiencies in Children With Sickle Cell Disease and Its Association With Increased Disease Severity.

Sickle cell disease (SCD) is characteristically described as a disease of hemolytic anemia and vaso-occlusive crises (VOCs). However, patients suffer from a multitude of other problems including impaired development, chronic pain, and increased susceptibility to infection. Nutritional deficiency has been implicated as a contributor to these issues. We reported the nutrition status with respect to vitamin D, zinc, B6, B12, folate, and homocysteine serum levels in Canadian children with SCD (n = 91). We also tested for associations between nutrients and markers of disease severity and growth. Almost half the sample (42%) had multiple nutrient insufficiencies/deficiencies, and a further 27% had a single insufficiency/deficiency. The most common insufficiency/deficiency was zinc in 57% followed by calcidiol (25 dihydroxyvitamin D (25(OH)D)) (52%). Sixteen percent of patients had low vitamin B6 levels, while folate, calcitriol (1,25(OH)D), and homocysteine levels were normal. Increased number of vitamin insufficiencies/deficiencies was associated with increasing disease severity (P = 0.018). Zinc insufficiency/deficiency was significantly associated with an increased number of home pain crises (P = 0.001) and an increased incidence of hospitalizations for VOCs (P = 0.01). Our findings show that patients with SCD commonly have multiple nutrient insufficiencies/deficiencies and support the growing evidence for the link between low zinc and increased VOC. It also indicates that increased nutrient insufficiencies/deficiencies are associated with increased disease severity in SCD. Prospective studies with larger samples are needed to further elucidate the relationship between nutrient deficiencies and SCD, and to determine whether nutrient supplementation can improve the disease course.

Exchange Transfusion In Adult Patients With Sickle Cell Disease With Refractory Vaso-Occlusive Crises

Sickle cell disease occurs in 1/500 African-American births. Pain is one of the most common complications of sickle cell disease and is associated with depression, anxiety, decreased quality of life and poor sleep patterns. Vaso-occlusive crises resulting in three or more hospitalizations per year occur in 48% of patients with sickle cell disease (Annals of Emergency Medicine - May 2009 (Vol. 53, Issue 5, Pages 587-593). Hydroxyurea has been shown to ameliorate the frequency of painful vaso-occlusive crises in sickle cell anemia (Charache et al, Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia NEJM. 1995), unfortunately many eligible patients are not treated due to psychosocial reasons and fear of teratogenicity or malignancy or have painful crisis refractory to hydroxyurea. A panel of experts has suggested that RBC exchange transfusion in these patients may decrease ED visits and subsequent hospitalizations based on anecdotal evidence [Best practices for transfusion for pateitns with sickle cell disease. T Wun, K Hagel. Hematology Review 2009, July 1; 1(22); e22]. We present the case of a 31 year old African-American male with Hgb S/beta thalassemia + with complications of sickle cell disease including Parvovirus B19 induced aplastic anemia, iron overload secondary to multiple simple transfusions, multiple vaso-occlusive crises as well as a left lower extremity ulcer precipitated by treatment with hydroxyurea. He was treated with folic acid as well as deferasirox for iron chelation. He began PRBC exchange transfusions every 6 weeks in March of 2011. In the one year prior to exchange transfusion initiation he had 13 ED visits and 4 hospitalizations for vaso-occlusive crises. After initiation of exchange transfusions he had 11 ED visits and 2 hospitalizations for vaso-occlusive crises the following year. An elevated WBC is associated with poor outcomes in sickle cell disease (Miller et al Predictors of adverse outcomes in children with sickle cell disease. N Engl J Med 2000). His average WBC level was 13 x 109/L, which decreased to 11 x 109/L after initiation of exchange transfusion. The patient underwent extended typing for E,C, and Kell RBC antigens to minimize development of antibodies. Though monthly exchange transfusions are costly and carry the attendant risk of antibody formation, this may be offset by reducing the financial and psychological burden of frequent hospitalizations for management of refractory vaso-occlusive crises. Exchange transfusion may also ameliorate the cost associated with long-term iron chelation. Further investigation is warranted to determine whether a program of monthly exchange transfusions can curtail ED visits and hospitalizations in adult patients with sickle cell disease and multiple, refractory vaso-occlusive crises. Disclosures: No relevant conflicts of interest to declare.

Safety and Efficacy of Adjuvant Low-Dose Ketamine Therapy for Patients with Sickle Cell Disease

AbstractAbstract ▪3226▪This icon denotes a clinically relevant abstract Background/Objectives:Opioids are the mainstay of therapy for painful vasoocclusive crises (VOCs) in sickle cell disease (SCD). For some patients, opioid-induced hyperalgesia caused by activation of N-methyl-D-aspartate (NMDA) receptors has been considered to contribute to poor analgesia. Ketamine, an NMDA receptor antagonist, could be a useful adjunct therapy; however safety concerns remain with its use. We examined records of SCD patients at our institution who received ketamine as an adjuvant to opioids at the discretion of anesthesia pain services over the past 4 years. We sought to explore the safety of ketamine and determine its effect on daily opioid requirement in SCD patients hospitalized for VOCs. Methods:The Institutional Review Board at Children's National Medical Center approved this study. A retrospective case-crossover study was conducted through review of the electronic medical record. For each patient we selected 2-paired hospitalizations occurring within 2 years of each other. One hospitalization where the patient received low-dose ketamine infusion in addition to opioid patient controlled analgesia (PCA), and a second hospitalization where the same patient received opioid PCA alone. We compared clinical characteristics of hospitalizations where patients did or did not receive ketamine. Exploratory bivariate analysis (paired t-test and McNemar's test) was used to compare variables between the pairs. Results:Thirty-three patients were identified to have at least 2 hospitalizations for VOC within 20 months of each other where they received adjuvant ketamine infusion during one, while not during the other. Average age was 15.6 ± 3.4 years, 67 % were females, and 36 % were on hydroxyurea therapy. SCD genotypes included homozygous SS 70%, SC 24 %, S-beta-zero thalassemia 3%, and S-beta-plus thalassemia 3%. Mean number of admissions in the 6 months prior to the ketamine hospitalization was 2 (range 0–5) and 64 % had ≥ 2 prior admissions within 6 months. Ketamine dose was 0.1 mg/kg/h for all patients except one who briefly received 0.15 mg/kg/hr. During the ketamine and opioid PCA hospitalization patients reported overall higher pain scores (6.53 vs. 5.94 out of 10; p = 0.0356), required higher doses of opioid (0.0395 mg/kg/hr vs. 0.0323 mg/kg/hr; p = 0.0038), and had a longer length of stay (LOS) (5.6 vs. 4.4 days; p =0.0148) as compared to the PCA alone hospitalization. Patients were more likely to have a diagnosis of acute chest syndrome (ACS) at some point during the ketamine and opioid PCA hospitalization (42% vs. 15 %; p = 0.0126) as compared to the opioid PCA alone hospitalization. Patients were more likely to be treated with additional adjuvant pain control agents (diazepam, lorazepam, gabapentin, pregabalin, amitripyline, or duloxetine) during the ketamine and opioid PCA admission as compared with opioid PCA alone admissions (45% vs. 9% p = 0.0013). Rates of red blood cell transfusion and ICU transfer were not different between the hospitalizations. In 3 patients ketamine was discontinued due to vivid dreams delusions, or dizziness. Nausea, pruritis, sedation, and use of complementary therapies were similar between hospitalizations. Conclusions:We did not observe an opioid sparing effect of ketamine infusion as hypothesized in this group of frequently hospitalized patients. Low-dose ketamine is a safe adjuvant medication for SCD patients hospitalized for VOCs. Higher opioid use during ketamine and opioid PCA admissions is likely due to patients experiencing more severe VOCs as indicated by higher pain scores involving multiple sites, higher rates of ACS, and longer LOS. These severity measures may have contributed to the decision of the pain medicine service to add low-dose ketamine infusion to standard opioid PCA in this retrospective sample. Finally, VOCs in this group of frequently admitted individuals may represent chronic pain which is known to be minimally responsive to most pharmacologic therapies. Patients receiving ketamine appear to be using additional adjuvant pain agents and may be underutilizing hydroxyurea. Prospective randomized studies of adjuvant ketamine therapy in patients with SCD are warranted to determine true efficacy. Disclosures:Off Label Use: Ketamine is a non-barbiturate phencyclidine derivative that is approved for use as a surgical anesthetic. It is not approved for use in pain management, however is commonly used in low-doses as an adjunct to traditional pain control therapies.

Vitamin D and Zinc Deficiency Associated with Increased Pain Episodes in Children with Sickle Cell Disease

AbstractAbstract ▪3237▪This icon denotes a clinically relevant abstract Background:Sickle cell disease (SCD) is characteristically described as a disease of hemolytic anemia and vaso-occlusive crises (VOC). However, patients suffer from a multitude of other problems including impaired growth and development, chronic pain, increased susceptibility to infection, poor bone health, and impaired cell-mediated immunity. Nutritional deficiency has been implicated as a contributor to these issues. The objective of this study was to provide a comprehensive overview of the nutrition status of pediatric SCD patients, and test for previously studied associations between nutrients and markers of disease severity and growth. Methods:Retrospective cross-sectional pilot study at a Canadian pediatric tertiary care centre from March to June 2012. Patients with all SCD genotypes who were between 2–18 years were eligible. As part of a routine follow-up clinic visit, patients were tested for serum levels of vitamin D (calcidiol and calcitriol), zinc, B6, B12, folate, and homocysteine. Note that all patients were prescribed folic acid supplementation. Information regarding laboratory values, growth, and markers of disease severity was obtained via medical record abstraction. Disease severity was categorized as mild (healthy patients with no hospital visits in past 12 months), moderate (emergency department (ED) visits related to SCD in past 12 months, home narcotic usage, and/or hydroxyurea usage) or severe (hospitalizations related to SCD in past 12 months, transfusion requirement, and/or end-organ damage (e.g. retinopathy)). Total nutrient deficiencies were categorized into none, single, or multiple. Results:Preliminary analysis of 43 patients was conducted (11.2 + 4.1 yrs, 40% female). Half the sample (50%) had multiple nutrient deficiencies, and 31% had a single deficiency. Deficiencies were seen most frequently in calcidiol (25(OH)D) (49% with insufficiency) and zinc (64% with deficiency), although there was no association between them (r=0.1, p=0.4). See Table 1 for an overview of nutrition status of all patients. Of all the markers of growth and disease severity tested, VOC was significantly associated with both vitamin D (calcidiol) deficiency and zinc deficiency. More specifically, calcidiol deficiency was moderately and significantly associated with total number of pain episodes (hospital, ED, and home) over the past 12 months (r=−0.4, p=0.02). Zinc deficiency was moderately and significantly associated with number of pain episodes managed at home over the past 12 months (r=−0.34, p=0.04).Other variables showed no significant findings. There were no gender differences in number of deficiencies (p=0.2). In terms of growth, patients had normal mean height-for-age (45.6 + 29.1 percentile) and weight-for-age (47.8 + 25.7 percentile). There was no association between disease severity and height-for-age (r=−0.2, p=0.2), weight-for-age (r=−0.1, p=0.6), or number of deficiencies (r=0.2, p=0.4). Calcidiol deficiency was not associated with height-for-age (r=0.6, p=0.7). Zinc deficiency was not associated with height-for-age (r=0.05, p=0.8), or with number of hospitalizations for VOC (r=−0.26, p=0.1) or infection (r=−0.1, p=0.6) in the past 12 months. Conclusions:This is the first study to provide a more complete overview of nutrition status in children with SCD. The majority of patients had multiple nutrient deficiencies, primarily in vitamin D (calcidiol) and zinc. Calcidiol deficiency was associated with increased VOC. Finally, this study contributes to growing evidence for the link between zinc's anti-sickling properties and reduced VOC, although we only found a significant association for home pain crises; this may be due to the low overall rate of hospitalizations in our sample. Prospective studies with larger samples are needed to further elucidate the relationship between nutrient deficiencies and SCD, and to determine whether nutrient supplementation can improve the disease course.Table 1Nutrition Status of Children with SCDRangeNutrientVitamin D (25(OH)D)Vitamin D (1,25(OH)D)ZincB6B12FolateHomo-cysteineVery low27% (11)0%46% (18)3% (1)0%0%–Low22% (9)0%18% (7)14% (5)5% (2)0%–Normal49% (20)97% (28)36% (14)81% (29)93% (40)100% (43)98% (40)Above normal2% (1)3% (1)0%3% (1)2% (1)–2% (1) Disclosures:No relevant conflicts of interest to declare.

Increased prevalence of osteonecrosis of the femoral head in children and adolescents with sickle‐cell disease

anemia. Blood 2010;115:5300–5311. 10. Thornburg CD, Calatroni A, Telen M, Kemper AR. Adherence to hydroxyurea therapy in children with sickle cell anemia. J Pediatr 2010;156:415–419. 11. Sichle Cell Disease Guidelines-Hydroxyurea Chapter. http://rover.nhlbi.nih. gov/guidelines/scd/about.htm. Accessed November 21, 2011. 12. de Montalembert M, Brousse V, Elie C, et al. Long-term hydroxyurea treatment in children with sickle cell disease: Tolerance and clinical outcomes. Haematologica 2006;91:125–128. 13. Zimmerman SA, Schultz WH, Davis JS, et al. Sustained long-term hematologic efficacy of hydroxyurea at maximum tolerated dose in children with sickle cell disease. Blood 2004;103:2039–2045. 14. Burgess SW SP, Morawska A, Devadason SG. Assessing adherence and factors associated with adherence in young children with asthma. Respirology 2008;13:559–563. 15. Jentzsch NS, Camargos PA, Colosimo EA, Bousquet J. Monitoring adherence to beclomethasone in asthmatic children and adolescents through four different methods. Allergy 2009;64:1458–1462. 16. Thornburg CD, Calatroni A, Telen M, Kemper AR. Adherence to hydroxyurea therapy in children with sickle cell anemia. J Pediatr 2010;156:415– 419. 17. Osterberg L, Blaschke T. Adherence to medication. N Engl J Med 2005;353: 487–497. 18. Franklin VL, Waller A, Pagliari C, Greene SA. A randomized controlled trial of Sweet Talk, a text-messaging system to support young people with diabetes. Diabet Med 2006;23:1332–1338. 19. Lee SJ, Joffe S, Kim HT, et al. Physicians’ attitudes about quality-of-life issues in hematopoietic stem cell transplantation. Blood 2004;104:2194– 2200. 20. Wong EC, Perez-Albuerne E, Moscow JA, Luban NL. Transfusion management strategies: A survey of practicing pediatric hematology/oncology specialists. Pediatr Blood Cancer 2005;44:119–127. 21. Streiff MB, Smith B, Spivak JL. The diagnosis and management of polycythemia vera in the era since the Polycythemia Vera Study Group: A survey of American Society of Hematology members’ practice patterns. Blood 2002;99: 1144–1149.

High resource hospitalizations among children with vaso‐occlusive crises in sickle cell disease

Vaso-occlusive crises (VOCs) contribute to frequent hospitalizations among children with sickle cell disease (SCD). The objective of this study was to identify factors associated with high resource utilization during hospitalizations for VOC. We analyzed pediatric discharges 0-18 years of age with a primary diagnosis of SCD with crisis from the 2006 Kids' Inpatient Database, a nationally representative sample of pediatric hospital discharges. High resource hospitalizations were defined as those in the highest decile for total charges. We conducted sample-weighted regression analyses to determine associations between independent variables (patient demographics, hospital characteristics, illness severity) and high resource use. There were 9,893 (0.371%) discharges for children with VOCs. Median total hospitalization charges were $10,691. In multivariate analysis, children 15-18 years of age (odds ratio [OR] 3.39, 95% confidence interval [CI] 2.54-4.53), 10-14 years of age (OR 2.72, 95% CI 2.07-3.59), and 5-9 years of age (OR 1.74, 95% CI 1.30-2.34) had higher odds of high resource hospitalizations compared to children 0-4 years of age. Care in a children's hospital had three times the odds of high resource use compared to care in a general hospital. Discharges with secondary diagnoses including pneumonia (OR 2.46, 95% CI 1.96-3.09) and constipation (OR 1.78, 95% CI 1.31-2.40) were also associated with high resource use. Older age and secondary diagnoses were associated with high resource use during VOC hospitalizations. These findings suggest the need to improve adherence to comprehensive care among older children to prevent VOCs and standardize protocols to manage VOC complications.

Clinical and Laboratory Effects of Hydroxyurea in Children and Adolescents with Sickle Cell Anemia: A Portuguese Hospital Study

Our aim was to assess the efficacy and safety of hydroxyurea (HU) in children with severe forms of sickle cell anemia followed in a Portuguese hospital. We carried out an open-label, uncontrolled prospective study, which included children with severe forms of sickle cell anemia. Hydroxyurea was started at 15 mg/kg/day and increased to a maximum dose of 25 mg/kg/day. Patients were monitored to assess compliance, clinical and hematological response and toxicity. Nine children and adolescents, five girls and four boys, with a median age of 13 years (range 8 to 16) were enrolled in the study during a period of 24 months. All patients completed at least 15 months of therapy. Hb F was significantly increased, from a mean of 7.0 ± 3.9% to 13.7 ± 5.3% (p = 0.028). Clinically, all patients responded significantly with a reduction of 80% in the number of vaso-occlusive crises (VOC), 69% in hospital admissions, 76% in hospitalization days and 67% in transfusion requirements, without significant toxicity. We concluded that, in our population, HU proved to be effective in increasing Hb F levels, and in decreasing hospitalizations for VOC and transfusion requirements with no major side effects. Long-term clinical follow-up is important to certify benefit maintenance.