Maintaining High Level Of Care At Outreach Sickle Cell Clinics

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2976-2976 ◽  
Author(s):  
Jennifer Hamm ◽  
Lee Hilliard ◽  
Thomas H. Howard ◽  
Jeffrey D. Lebensburger

Introduction Parents of children with sickle cell disease often seek care at large, university based sickle cell clinics. A major health care barrier for children in Alabama involves the cost and time of travelling to and from university based clinics. To reduce this health care barrier, the University of Alabama at Birmingham (UAB) developed The Children and Youth Sickle Cell Network(®) (CYSN(®)). This network consists of the central sickle cell clinic located at UAB and four outreach sickle cell clinics located in Montgomery (100 miles south of UAB), Opelika (110 miles southeast of UAB), Huntsville (100 miles north of UAB), and Tuscaloosa (60 miles west of UAB). The goal for these clinics is to maintain a similar level of medical care while reducing the health care barrier of transportation. Objective To determine if the outreach clinics provide similar care to university based clinics, we evaluated three surrogate preventive care markers to compare access to care in central vs. outreach clinics: 1) attendance rates, 2) number of patients on hydroxyurea, and 3) percent of MRIs obtained for screening of silent infarct among eligible patients. Methods A retrospective review of all CYSN(r) clinic visits from June 2012 to June 2013 was performed to determine clinic attendance rates. All patients on hydroxyurea were categorized by clinic location. Every patient attending CYSN(r) clinic between ages of 6 and 15 years had their medical record reviewed for completion of a screening MRI/MRA. Results At the central Birmingham clinic, the appointment show rate was 59.8% as compared to the Montgomery, Opelika, Huntsville, and Tuscaloosa show rates which were 57.7%, 73.1%, 59.4%, and 70% respectively. At UAB, institutional guidelines were developed for offering hydroxyurea to patients based on clinical indications and applied to all clinics. The percentage of patients on hydroxyurea therapy in Birmingham is 22.2%, while the percentages in Montgomery, Opelika, Huntsville, and Tuscaloosa are 21.5%, 32%, 21.4% and 24.4%, respectively. Finally, screening MRI/MRA to evaluate for evidence of silent cerebral infarctions is performed in Birmingham but offered to children ages 6-15 years at all sickle cell clinics. In Birmingham, 63.6% of eligible patients completed MRI/MRA screening. This percentage is similar for patients in Montgomery, Opelika, and Tuscaloosa who were screened at 66.7%, 83.3%, and 67.7% respectively. Conclusions Our data suggests that outreach clinics can provide similar levels of medical care for children with sickle cell disease. Sickle cell centers treating patients that must travel long distances should consider developing outreach clinics to help reduce this major health care barrier. Disclosures: No relevant conflicts of interest to declare.

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 564-564
Author(s):  
Mariane De Montalembert ◽  
Frédéric Galacteros ◽  
Jean Antoine Ribeil ◽  
Uwe Kordes ◽  
Jean Benoit Arlet ◽  
...  

Abstract Hydroxycarbamide (HU) is a myelosuppressive drug marketed since 1968 for the treatment of hematological cancer, and authorized since 2007 in Europe as orphan medicinal product for the prevention of recurrent vaso-occlusive crises including acute chest syndrome in adults and children older than 2 years with sickle cell disease (SCD). ESCORT-HU (European Sickle Cell Disease Cohort – Hydroxyurea) is a multicenter prospective non interventional study implemented in Europe to collect more information about the safety profile of HU and morbi-mortality in SCD patients treated with HU. The study responds to EMA (European Medicines Agency) request and has been approved by the Ethical of Necker Enfants Malades Hospital (Paris, France).The ongoing study involves the largest number so far of patients with SCD treated with HU. Primary endpoints of ESCORT HU are to determine frequency of adverse events, and possible consequent changes of HU treatment. Secondary endpoints are to evaluate morbi-mortality of the disease although in the absence of control group. From June 2008 to June 2014, 483 patients (255 females; 228 males) were enrolled from 3 European countries, Greece (24%), Germany (19%), and France (56%). 67% patients were adults, median aged 37.35 yrs (17-83.5) and 33% were children, median aged 11.06 yrs (2.6-16.9). genotypes were HbS/HbS in 71.4% cases, and compound heterozygotous HbS/β-thalassemia in 22.8 % (Table 1). 137 (28.4%) patients experienced 421 events (Table 2). 132 (32.2%) of these events may be attributed to HU. The safety profile is roughly similar in children and adults. As expected the most frequent side effects were firstly blood disorders (n=86 events, 42.4%) such as neutropenia or thrombocytopenia. In all cases, these cytopenias were rapidly resolved with the transitory stop of HU. 71 events related to skin and subcutaneous tissue disorders were observed, mostly cutaneous dryness, skin reactions, alopecia and nails or skin pigmentation; 4 patients had a leg ulcer (34.8%). Most of these events are ongoing or stabilized despit the decrease of HU. No secondary cancer has been reported until now. Even if HU is an old drug with a relatively well-known safety profile, some uncertainties remain in terms of long-term safety as well as tolerance in the youngest people. The main interest of ESCORT HU is to offer the possibility of safety surveillance of hydroxycarbamide in European sickle cell patients. Table 1 Demographic data Adults Children < 17 years old Total Number of patients 322 (67%) 161 (33%) 483 Females/Males 183/139 72/89 255/228 Median age (yrs) (range) 37.35 (17-83.5) 11.06 (2.6-16.9) 28.58 Genotype SS 206 (64%) 139 (86.3%) 345 (71.4%) SC 1 (0.3%) 3 (1.86%) 4 (0.8%) Sβ0 51 (15.8%) 11 (6.8%) 62 (12.8%) Sβ+ 46 (14.2%) 2 (1.2%) 48 (9.9%) Other 18 (5.5%) 6 (3.7%) 24 (4.9%) Treatment with HU before enrollment in ESCORT HU No of pts 232 83 315 (65%) Median duration (range) of HU treatment before ESCORT HU 8.2 yrs (0.5 ans-24 yrs) 3. 1 yrs ( 71 days – 8.9 yrs) 6.85 (71 days-24 years)] HU ESCORT Daily mean dose (mg/kg/d) 16.11 ± 4.79 19.63 ± 4.69 17.32 ± 4.94 Abstract 564. Table 2 The most frequent events of hydroxycarbamide in the two populations of SCD patients ADULTS CHILDREN No ofGerman(%) No of adults No ofEpisodes(%) No of children Total(% /411) Events Related to HU treatment (Siklos®)(%**) Blood and lymphatic system disorders (%) 32 (17.7) 22 54 (31.03) 28 86 (20.9) 56 (65.1) Skin and subcutaneous tissue disorders (%) 42 (23.2) 28 29 (16.7) 19 71 (17.3) 46 (64.8) Nervous system disorders Headache (24), Dizziness/vertigo (14), 32 (17.7) 23 12(6.9) 10 44 (10.7) 11 (25) Gastrointestinal disorders Nausea (14), diarrhea (8), other (14) 20 (11) 17 23 (13.2) 16 43 (10.4) 7 (16.3) Metabolic and nutrition disorders: vit D deficiency (17), weight gain (5) 13 (7) 11 18 (18.3) 18 36 (8.75) 4 (11.1) Fever 11 (6) 10 12(6.9) 7 23 (5.6) 1 (4.3) Cardiac disorders (hypertension, bradycardia, chest pain, cardiomegaly) 4 4 2 2 6 1 (16.6) General disorders : fatigue 5 5 0 0 5 0 Hepatobiliary disorders 2 2 0 0 2 0 Neoplasms benign, malignant and unspecified (incl. cysts and polyps) Harmatoma, benign vulvar sebaceous cyst 2 2 0 0 2 0 Renal & urinary disorders 2 2 0 0 2 0 Reproductive system and breast disorders 3 3 0 0 3 0 Other 13 13 21 14 34 6 (17.1%) 181 80 /181(24.8%) 174 57 / 174(35.4%) 411 132/411 (32.2%) ** compared to the total number of “system organ class” events Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2072-2072 ◽  
Author(s):  
Ewurabena Simpson ◽  
Richard Ward ◽  
Melanie Kirby ◽  
Isaac Odame

Abstract Abstract 2072 Background: The Hospital for Sick Children (HSC) in Toronto, Canada cares for more than 700 children with sickle cell disease (SCD), which is the largest Canadian population of children with SCD. Since 2009, the SCD Program at HSC has partnered with adult hematologists within the Red Blood Cell Disorders program at Toronto General Hospital (TGH) to develop a coordinated strategy for transitioning the care of young adults with SCD. Hemophilia is a chronic hematological condition which, like SCD, has a spectrum of disease severity that requires multidisciplinary follow up. At HSC, we care for nearly 200 patients with hemophilia A and B and have a long-established partnership with adult hematologists, which has led to an effective transfer of patients with hemophilia into adult care. In Ontario, adult health providers are remunerated according to a fee-for-service billing schedule. In contrast, pediatric subspecialists are mainly salaried under an alternate funding plan. Until 2010, adult hematologists who provided medical care for individuals with hemophilia received a significantly higher pay scale than those who cared for individuals with SCD. This was changed in July 2010 so that adult hematologists receive commensurate remuneration for services rendered for both hemophilia- and SCD-related medical care. Objectives: 1. To compare the patterns for transitioning patients of varying disease severity within the pediatric and adult SCD and hemophilia programs in Toronto, Ontario. 2. To identify barriers and enablers that have influenced the transition of young adults with SCD within a universal health care system. Methods: Data for active, transitioned and inactive patients in the HSC and TGH clinical programs are maintained in a database at HSC. These patient numbers were gathered according to sickle cell genotype. Similar data were available for hemophilia patients who were transitioned from HSC to adult care. Chi-square analyses were used to compare the proportions of patients in the sickle cell and hemophilia programs that were transitioned between 2009 and 2011. Results: Conclusion: The HSC-TGH- partnership has significantly reduced the number of youth with SCD who continue to be followed at HSC or are lost to follow up. However, a significant number of young adults within the HSC SCD program still need to be transitioned to adult care. For the sustainable expansion of this transitional care strategy, health policymakers must collaborate with tertiary and community level health care providers to develop a coordinated and distributed strategy for the effective delivery of comprehensive health care services for young adults with SCD. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4910-4910 ◽  
Author(s):  
Simone CO Gilli ◽  
Fernando V Pericole ◽  
Bruno Deltreggia Benites ◽  
Lilian Castilho ◽  
Marcelo Addas-Carvalho ◽  
...  

Abstract Sickle cell disease (SCD) is a chronic inflammatory condition, even in steady state, as indicated by elevated levels of inflammatory cytokines and increased Th17 responses, compared with healthy controls. These inflammatory pathways may be directly regulated by genetic polymorphisms and could be associated to different outcomes of the disease. High levels of a number of different circulating cytokines were found in SCD patients in several studies, and changes in the cytokine balance in SCD patients are an important risk factor for the occurrence of clinical events. Moreover, inter-patient variations in cytokine levels could be attributed to gene polymorphisms. To investigate cytokine polymorphisms and their association with cytokines expression we evaluated the IL4 intron3 VNTR (genotypes 1.1, 1.2, 2.2, 2.3), IL4T590C/T, IL6174G/C and TNFA308A/G polymorphisms and their correlation with TGFB, IL-4, IL-6 and IL-10 expression in steady-state SCD patients. Methods. Fourth-nine patients (24 male and 25 female; 39.8 ± 9.59 years) with SCD and 28 (22 male and 6 female; 35.5 ± 10.2 years) healthy blood donors were evaluated. The polymorphisms were performed by PCR-RFLP analysis described as [individuals (genotype frequencies)] and the expression of TGFB, IL-4, IL-6 and IL-10 by q-PCR expressed as [median (max-min)]. Results. A higher frequency of 1.2, 2.2 and 2.3 genotypes was found in SCD patients compared with normal controls [34(0.69) vs 12 (0.44), P=0.03]; higher expression of IL-4 was found in the ones carrying the 1.1 genotype [0.31 (2.53-0.01) vs 0.05 (0.95-0.0), P=0.047] and although no differences were found in the IL4T590C/T, IL6174G/C and TNFA308A/G polymorphism frequencies, a significantly greater expression of TGFB, IL6 and IL10 was observed in the patients cohort compared to normal individuals [1.55 (9.02-0.0) vs 0.97 (5.46-0.0), P=0.019]; [0.18 (45.45-0.0) vs 0.0 (8.14-0.0), P=0.03] and [0.98 (22.84-0.0) vs 0.0 (9.95-0.0), P<0.001, respectively]. All the genotype frequencies are consistent with Hardy-Weinberg equilibrium. Conclusion: A unique distribution of IL-4 genotypes was observed in our cohort of patients and controls, probably related to the miscellaneous ethnic background of our population. The highest prevalence of the IL4intron3 polymorphism in sickle cell patients suggests a less secretory phenotype associated with increased expression of inflammatory cytokines. IL-4 plays an important role in tissue adhesion and inflammation, including induction of adhesion molecules on vascular endothelial cells and could be responsible for a more “inflammatory” phenotype. Despite the small number of patients enrolled, our study brings insights and new data regarding the deregulation in immune system affecting SCD patients and this information must be investigated in larger cohorts, and may help to better characterize individual variations in immune responses and new markers for disease morbidity. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4955-4955
Author(s):  
Akshat Jain ◽  
Amie Patel ◽  
Udochukwo Oyoyo ◽  
Seth Wiafe

Abstract Introduction: Starting in March 2020, the coronavirus 19 disease (COVID-19) pandemic affected the United States (US) health care system. Many individuals were afraid to seek medical care due to fear of obtaining COVID-19; therefore, there was a high frequency of adults forgoing medical care. Chronic conditions including diabetes, COPD, mental health, and HTN outcomes worsened due to decrease in routine care during the pandemic. Sickle cell disease (SCD) is a chronic blood disorder that causes numerous complications including but not limited to vaso-occlusive crisis, strokes, and chronic kidney disease. Individuals who have routine follow up with a hematologist have been shown to have decreased hospitalizations, ER visits, length of stay, and opiate usage. The Inland Empire region of Southern California is one of the densest hot spots for patient's living with sickle cell disease in terms of disease burden and health care utilization. Therefore, we evaluated how the COVID-19 pandemic affected health care utilizations with individuals with SCD in this area. Methods: A retrospective analysis of the health care utilization in the inpatient setting was performed at three time points in line with the C.D.C. COVID pandemic declaration (Pre COVID: September 2019 to February 2020, Intra COVID 1: March 2020 to Sept 2020, Intra COVID 2: October 2020 to February 2021). Primary endpoints included emergency room visits, inpatient admissions, and length of stay, blood product utilization, and opioid utilization. Secondary endpoints and analyses included pediatric vs. adult health care utilization disparity and outcomes. Descriptive analysis was done by calculating frequency and percentages for categorical variables and mean and standard deviation for continuous variables. Results: Shown in Result TABLE attached .Patients seeking inpatient care for scheduled procedure's/ transfusion were excluded from this analysis. Patients with SCD diagnoses of acute SCD crises (pan crisis /chest syndrome / COVID infection / Pneumonia) were included in assessment. Absolute number of patients per ED visit, patient per Inpatient admission frequency has been shown in more detail in the bar chart in Figure and will be presented in the oral presentation along with detailed data analysis box plots and charts. Discussion: Fewer patients' sought timely care for acute SCD event leading to fewer but prolonged hospitalizations, a trend seen in our adult and pediatric patient's across the board during intra COVID 1 and 2 time points, likely due to the fear of contracting COVID infection at an acute health care setting in a declared pandemic . Similarly, we found there were less unique sickle cell patients using the ER for both pediatric and adult patients during the pandemic. There was a decrease in the amount of hospital admission related to sickle cell disease, which was similar to results published in the United Kingdom. More sickle cell vaso-occlusive crisis were being managed at home, likely due to avoidance of COVID exposures at health care facilities, likely thus leading to certain individuals having more severe crisis as shown by increased LOS in our adult population. Additionally, only a few individuals with likely more severe crisis needed more RBC transfusions. The general trend for different patients requiring RBCs and IV opiates decreased during the pandemic. Due to more people foregoing medical care especially in the minority populations who had restrictions to access to healthcare, there was an overall decrease in inpatient healthcare utilization in sickle cell population during the pandemic. Additionally care disparities in outcome of pediatric and adult SCD patients were highlighted by our study. Pediatric patients commonly cared at a center of excellence by the dedicated team of sickle cell stakeholders, seemed to do well overall as compared to adult SCD patients who notoriously have has inequitable access to comprehensive Sickle Cell care globally. Infrastructure around better outpatient support, care optimization with sickle cell disease modifying agents and timely access to inpatient care even in a pandemic, that is ongoing could be strategies to reduce stress on the health care systems and better utilization of scarce resources in current times of surge from an International health care crisis such as the SAR CoV2 pandemic. Figure 1 Figure 1. Disclosures Jain: GBT: Consultancy; CSL Behring: Consultancy, Speakers Bureau; Takeda: Consultancy, Speakers Bureau; Octapharma: Consultancy; Blue Bird Bio: Consultancy.


2019 ◽  
Vol 111 (1) ◽  
pp. 54-61 ◽  
Author(s):  
Monica Ter-Minassian ◽  
Sophie Lanzkron ◽  
Alphonse Derus ◽  
Elizabeth Brown ◽  
Michael A. Horberg

PEDIATRICS ◽  
1991 ◽  
Vol 88 (6) ◽  
pp. 1226-1232
Author(s):  
Barbara S. Shapiro ◽  
David E. Cohen ◽  
Kenneth W. Covelman ◽  
Carol J. Howe ◽  
Sam M. Scott

This article is a report of our experience with an interdisciplinary pain service in a large tertiary care pediatric hospital. During the first 2 years of operation, we received 869 consultations and referrals from more than 19 hospital divisions. Postoperative pain was the most frequent reason for consultation (56% of patients). Patients with pain related to cancer and sickle cell disease comprised 25% of the consultations. The remaining patients had a wide variety of primary diagnoses and causes of pain. We calculated the time spent by pain service physicians in direct patient care. The majority (63%) of physician time was spent with a small number of patients (17%). Most of these patients had pain that was unrelated to surgery, cancer, or sickle cell disease, and many posed dilemmas in diagnosis and treatment. Physician time was correlated directly to the use of psychologic and physical therapies for the pain, involving multiple team members. This experience supports the demand for an interdisciplinary pain service in a tertiary care children's hospital. A significant amount of physician time is necessary to provide patient care and to maintain a team approach, however, and pediatricians and other health care professionals who aim to implement such services should be cognizant of the time required.


2020 ◽  
Author(s):  
Jacquelyn Baskin ◽  
Anne Nord ◽  
Dawn Canada ◽  
Kelly Russell ◽  
Payal Shah ◽  
...  

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 13-13
Author(s):  
Oladipo Cole ◽  
Asia Filatov ◽  
Javed Khanni ◽  
Patricio Espinosa

Moyamoya disease, well described in literature, is a chronic cerebrovascular occlusive disorder. It is characterized by progressive stenosis/occlusion of the terminal portions of the internal carotid arteries (ICA) and the proximal portions of the middle cerebral arteries (MCA). Less frequently described is Moyamoya syndrome, the name given to radiographic findings consistent with Moyamoya disease, but with an identifiable cause. The diseases associated with Moyamoya Syndrome include Sickle Cell Disease (SCD), Thalassemias, and Down's Syndrome to name a few. Common complications of Moyamoya include both ischemic and hemorrhagic strokes. Upon literature review, Moyamoya syndrome caused by SCD is not well described. When it is, the discussion is centered around the pediatric patient population and surgical management. Our case report describes a 22-year-old African American female with SCD who initially presented with Acute Chest Syndrome. Her hospital course was complicated by development of overt debilitating neurologic deficits. Subsequently, she was found to have Moyamoya Syndrome on neuroimaging. She was successfully treated with medical management without any surgical intervention. This case highlights the necessity of thorough examination, differential diagnosis, imaging findings, and consideration of predisposing syndromes in the work-up for Moyamoya syndrome; especially individuals with Sickle Cell Disease. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 10-11
Author(s):  
Satish Maharaj ◽  
Simone Chang ◽  
Karan Seegobin ◽  
Marwan Shaikh ◽  
Kamila I. Cisak

Background: Acute chest syndrome (ACS) frequently complicates sickle cell disease (SCD) and is a leading cause of hospitalization and mortality. Many factors have been implicated in ACS, including infections, thrombosis, fat and pulmonary emboli. However, a clear etiology is not defined in 50% of the cases and ACS is considered a clinical endpoint for different pathogenic processes (Vichinsky et al 2000). The non-specific nature of ACS makes diagnostic tests challenging, and there are no serum tests clinical used to aid diagnosis. Procalcitonin (PCT) is a prohormone of calcitonin and serum PCT rises within hours of an inflammatory stimulus. PCT has clinical utility as a marker of severe systemic inflammation, infection, and sepsis (Becker et al. 2008). Few studies have evaluated PCT as a biomarker for ACS in patients presenting with vaso-occlusive crises (VOC). Two studies have reported no difference in PCT (Biemond et al. 2018 and Stankovic et al 2011), while one study reported higher PCT between ACS and VOC (Patel et al 2014). Methods: We retrospectively reviewed 106 patients with SCD who presented to the emergency department with fever and painful crises during 2015-2019. The patients were divided into two categories based on discharge diagnoses - patients with VOC only (n=88) and patients with ACS (n=18). Inclusion criteria for both groups were patients with SCD, 17 years and older and PCT measurement on presentation. Exclusion criteria were defined as patients who had received empiric antibiotics prior to PCT testing. Data collected on presentation included genotype, age, gender, complete blood count, PCT, creatinine, total bilirubin and hydroxyurea use. Length of stay was recorded. Data was analyzed between the two groups using descriptive statistics and accounting for unequal variances, withp-value set at 0.05 for significance. Results: Demographics and clinical characteristics are summarized in Table 1 (Figure). The sample included primarily adult males (77%), with about two-thirds on hydroxyurea. Genotype HbSS (73.6%) was most prevalent followed by HbSC (22.6%) and HbSβ (3.8%). The ACS group had a higher percentage of HbSS, lower use of hydroxyurea and higher mean bilirubin. Mean PCT for the ACS group was 0.52 ng/mL (range, 0.05-2.04), compared to 0.31 ng/mL (range, 0.02-6.82) in the VOC group; withp=0.084. ROC analysis showed a PCT&gt;0.5ng/mL had 39% sensitivity and 85% specificity for ACS in this sample. Conclusion: In this sample, PCT on presentation was higher in those with ACS compared to VOC, but this difference did not achieve statistical significance. Further study in a larger population would be useful to evaluate this finding. Disclosures No relevant conflicts of interest to declare.


Sign in / Sign up

Export Citation Format

Share Document