A Needs Assessment of Persons With Sickle Cell Disease in a Major Medical Center in North Carolina

Abstract Abstract 1004 Hydroxyurea (HU) is approved for use in adults with Sickle Cell Disease (SCD) and increases the production of fetal hemoglobin (HbF). Increased HbF is associated with decreased clinical severity in adults and children with SCD, such as decreased numbers of vaso-occlusive events, transfusions, and hospitalizations. Higher HbF at initiation of HU is predictive of HbF response, but association between age of hydroxyurea initiation and HbF response has not been investigated. We hypothesize that starting hydroxyurea at an early age may improve hematological and clinical response. In order to determine if younger age at hydroxyurea initiation affects the percentage of HbF achieved with hydroxyurea, we conducted a retrospective cohort study. We identified subjects enrolled in the Duke University Medical Center Comprehensive Sickle Cell program who initiated hydroxyurea when they were less than 17.99 years of age and were prescribed hydroxyurea for at least six months. The following data were abstracted from the medical record between December 1996 and April 2011: age, hemoglobin, percentage HbF, and mean corpuscular volume (MCV) at start of HU and at maximum tolerated dose (MTD) of HU therapy. The correlation coefficient and p-values for various parameters were calculated. Seventy-three patients (41 males and 32 females) were included in the analysis. The mean age at hydroxyurea initiation was 5.5 years (1.2–14.1). The mean hydroxyurea dose at MTD was 28.6 ± 3.2 mg/kg/day. At initiation, the mean hemoglobin was 8.2 ± 1.2 g/dL, the mean MCV was 83±7.4 fl and mean HbF was 10 ± 5.7%. At MTD, the mean hemoglobin was 9.4 ± 1.1 g/dL, the mean MCV was 99 ± 11.1 fl, and the mean HbF was 21.7 ± 9.4%. As expected, at MTD, an elevated MCV was correlated with elevated fetal hemoglobin (r2= 0.19, p= 0.0001) [Table 1]. There was a statistically significant relationship between the age at HU initiation and the HbF at MTD (r2= 0.08, p= 0.015) [Figure 1] as well as the age at HU initiation and the hemoglobin at MTD (r2= 0.19, p= 0.016). The relationship between the age at starting HU and the overall change in HbF (DHbF) was not statistically significant (r2= 0.01, p= 0.41). There was not a statistically significant relationship between age at HU initiation and the MTD of HU (r2= 0.003, p= 0.61). The 6 patients started on HU at age less than 2 years (mean 1.5 ± 0.3 years) maintained a mean elevated HbF of 19.1 ± 5% at last documented follow-up with follow-up ranging from 1.4–13 year of uninterrupted hydroxyurea use. Starting hydroxyurea therapy at a younger age appears to improve HbF response as measured at MTD, although there is variability in the level of fetal hemoglobin attained. There is not an association seen with the DHbF or dose at MTD and age at hydroxyurea initiation. In summary, starting hydroxyurea at a younger age, when HbF is >20%, leads to persistence of HbF production and overall improvement in hematological efficacy. This was not simply the result of achieving MTD at a younger age before physiologic decline of HbF. Disclosures: Off Label Use: Hydroxyurea for complications of sickle cell disease in pediatrics. Shah:Eisai: Research Funding; Adventrx: Consultancy.

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Needs Assessment for Patients with Sickle Cell Disease in South Carolina, 2012

Public Health Reports ◽

10.1177/003335491613100117 ◽

2016 ◽

Vol 131 (1) ◽

pp. 108-116 ◽

Cited By ~ 5

Author(s):

Alyssa M. Schlenz ◽

Andrea D. Boan ◽

Daniel T. Lackland ◽

Robert J. Adams ◽

Julie Kanter

Keyword(s):

South Carolina ◽

Sickle Cell Disease ◽

Needs Assessment ◽

Sickle Cell ◽

Cell Disease

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Emergency Department Encounters, Hospitalizations and ED Reliance Among Medicaid Eligible Patients with Sickle Cell Disease in North Carolina

Blood ◽

10.1182/blood-2019-127196 ◽

2019 ◽

Vol 134 (Supplement_1) ◽

pp. 2113-2113

Author(s):

Paula Tanabe ◽

Nancy Crego ◽

Christian Douglas ◽

Emily Bonnabeau ◽

Marian Earls ◽

...

Keyword(s):

North Carolina ◽

Sickle Cell Disease ◽

Old Age ◽

Sickle Cell ◽

Research Funding ◽

Claims Data ◽

Age Group ◽

Cell Disease ◽

Old Patients ◽

The Mean

Introduction: Sickle cell disease (SCD) is a complex disease for which pain is the hallmark. Pain from vaso-occlusive episodes is the number one reason for ED visits and hospital admissions. This paper reports Medicaid claims data from NC for individuals with SCD, including: 1) ED encounters and re-encounters within 7, 14 and 30 days; 2) hospitalization and re-hospitalization within 7, 14 and 30 days; and 3) ED reliance (EDR) score. Methods: We examined Medicaid claims data from for patients with a diagnosis of SCD (ICD 9 CM codes: 282.6x, ICD 10 CM codes: D57.0x, D57.1, D57.2x, D57.4x, D57.8x) in North Carolina. Data is reported for a cohort of 2,790 patients with a diagnosis of SCD, age 1 to 65+ and enrolled at least 11 months in NC Medicaid between March 1, 2016 and February 28, 2017. ED re-encounters and re-hospitalizations within 7, 14 and 30 days were identified using the time between the date of service listed on the ED or hospital claim and the next date of service in the subsequent claim. Individual ED Reliance (EDR) score was calculated as the total number of ED encounters divided by the total ambulatory visits (outpatient + ED encounters) per enrollee, (ambulatory visits reported elsewhere). Similar to Kroner et al, an EDR of >0.33 was considered a high score. Inpatient claims were identified using a category of service code indicating hospitalization. Results: The participants in the sample (n=2790) were majority female (57.92%), lived in metropolitan areas (77.63%) and had a mean age of 23.05 years old (SD=16.06). Of the 9,075 total ED encounters, 69.86% of the total sample had an ED encounter during the 12-month study period. There was a mean of 3.25 (SD=7.38) and median of 1 (IQR = 0 - 3) ED encounters per patient for the sample. Those who were 18-30 years old had the highest mean and median ED encounters per patient (4.98, SD= 9.34 and 2, IQR 1 to 5). The 31-45 year old group had the second most, with 4.82 (SD= 11.03) total ED encounters. The percentage of the sample with an ED re-encounter within 7, 14, and 30 days was also highest among the 18-30 year old group (29.17%, 33.98% and 40.89%) followed by those 31-45 years old (23.71%, 28.49%, and 34.80%), respectively. The 31-45 age group had the second most hospitalizations/patient and re-hospitalizations. The mean EDR was highest among 18-30 year old patients (0.35) and 46.48% of this age group had an EDR of 0.33 or greater. In the 31-45 year-old age group, the mean EDR was 0.28 and 35.18% had an EDR of 0.33 or greater. The overall sample had a mean of 1.30 (SD= 2.75) hospitalizations/patient. The 18-30 year old age group also had the highest mean total hospitalizations (2.08, SD= 3.72) and mean re-hospitalizations within 7 (0.16; SD=0.77), 14 (0.41; SD=1.68), and 30 (0.82; SD=2.79) days. The 31-45 age group had the second most hospitalizations/patient and re-hospitalizations (Table 1). Conclusions: Overall, increasing age coincided with increased ED and inpatient utilization, as well as with the period of transition from pediatric to adult SCD care. Furthermore, high EDR was most prevalent in the 18-30 age group. Our study further supports the need for increased focus on acute care utilization in the 18-45 year-old age group and considerations for improved care transition interventions. Disclosures Tanabe: NIH: Research Funding; AHRQ: Research Funding. Shah:Novartis: Consultancy, Research Funding, Speakers Bureau; Alexion: Speakers Bureau; GBT: Research Funding.

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Measuring Transition Readiness in Adolescents and Young Adults with Sickle Cell Disease Using the Transition Readiness Assessment Questionnaire

10.22541/au.162138591.15170944/v1 ◽

2021 ◽

Author(s):

Anjali Oberoi ◽

Alyssa Patterson ◽

Amy Sobota

Keyword(s):

Young Adults ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Medical Center ◽

Self Report ◽

Adolescents And Young Adults ◽

Cell Disease ◽

Readiness Assessment ◽

Transition Readiness ◽

Assessment Questionnaire

Background/Objectives: Adolescents and young adults (AYA) with sickle cell disease (SCD) face challenges related to the disease and its treatment. The Transition Readiness Assessment Questionnaire (TRAQ) is a self-report tool for assessing transition readiness for youth with special health care needs (YSHCN), including SCD. This study uses the TRAQ to understand transition readiness in patients with SCD treated at the Boston Medical Center, evaluates associations between TRAQ scores and transition outcomes (e.g., EDr, EDu), and compares TRAQ scores in this population with other YSHCN. Methods: We reviewed electronic medical records of AYA with SCD who completed the TRAQ in the pediatric hematology clinic between January 1, 2019, and March 1, 2020, and categorized healthcare encounters to calculate EDu and EDr. We used t-tests and ANOVA models to analyze mean TRAQ scores, sex, age, genotype, EDu, and EDr. Results: The sample was 45 AYA patients with SCD between 13 and 22 years old. The mean TRAQ score for the overall patient sample was 3.67. Mean TRAQ scores did not significantly vary by sex or genotype but did significantly increase with age. TRAQ scores were lower in the SCD population than in other YSHCN. TRAQ scores did not correlate to EDu or EDr. Conclusions: AYA patients with SCD have lower transition readiness than other populations of YSHCN. The age of 18 may not be the most reliable attribute of readiness, though older patients do have higher readiness. The relationship between TRAQ scores, EDr, and EDu is not clear and requires further evaluation.

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Impact of the COVID19 Pandemic on Admissions and Emergency Department Visits of Adults with Sickle Cell Disease

Blood ◽

10.1182/blood-2020-142803 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 37-38

Author(s):

Alice J. Cohen

Keyword(s):

Emergency Department ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Medical Center ◽

Cell Disease ◽

Face To Face ◽

Outpatient Visits ◽

Pain Medications ◽

Time Period ◽

Ed Visits

Background: The most common complication of sickle cell disease (SCD) in adults is vaso-occlusive crisis that is characterized by severe pain. These events can often be managed at home with oral analgesics, but if the pain is not controlled or the patient develops other associated problems, they seek care in an emergency department (ED). In the ED, they receive initial treatment with pain medications and are assessed for other complications such as infection and acute chest syndrome. If an individual's pain is not controlled in a short period of time, the majority of these patients are admitted to the hospital for inpatient management or placed in an observation unit (OBs) for 6-47 hours. The COVID-19 pandemic affected the Greater Newark community starting in mid March with the majority of all inpatient admissions (Ads) being COVID related through the end of May. It has been observed both at our medical center and nationally that during this time period and even afterwards, the number of ED visits and Ads had significantly fallen. The reasons for this finding may include fear of contracting COVID infection at the hospital, regular telemedicine (TM) calls to facilitate outpatient management, and an increase in the number of prescriptions of home pain medications. The purpose of this analysis was to examine patterns of ED visits, Ads, outpatient visits, prescription renewals and nurse (RN) and social worker (MSW) calls in order to determine the impact of COVID-19 infection on the local SCD community. Methodology: A retrospective review was undertaken of billing data and the EMR of all patients with SCD treated at Newark Beth Israel Medical Center (a 450 bed community-based academic tertiary care medical center) between January 2020 and June 2020. Data collected included the number of and reason for ED and OBs, Ads, the number of TM and outpatient visits, and MSW and RN telephone contacts. All patients 18 years of age and older were included. Overall, 100 adults with SCD received care between January and June. Results: Peak hospital COVID Ads, ED and OBs for all patients (SCD and non-SCD) occurred during the weeks between March 25 and May 24, 2020 with a daily inpatient census over 200 between April 7 and 24. SCD Ads at peak COVID (April-May) were significantly lower at 26±2/month compared to 64±11/month pre-COVID (January-February) (p= 0.04). ED and OBs were unchanged. During the peak of COVID, 10/93 (11%) SCD Ads (1 death) were COVID related with 80/96 (86%) for uncomplicated pain crises. MSW and RN called all patients proactively to offer support at onset of COVID pandemic. During this same time period, the number of MSW telephone contacts increased from 138±37/month pre-COVID to 372±21/month during COVID (p=0.02). RN contacts with SCD patients were stable and mostly were for pain prescription renewals. TM was initiated in March 2020 and an increase in these visits correlated with a fall in face to face physician visits: 83.5±11/month pre-COVID to 39.5±8/month peak COVID (p= 0.04), and TM 0/month pre-COVID and 31±4/month peak COVID (0.01). Conclusion: The outbreak of COVID-19 in the community reduced the number of Ads for patients with SCD without an increase in ED and OBs visits. MD face-to-face encounters were reduced but outpatient care continued with the initiation of TM, regular RN contact with maintenance of pain medication prescriptions and a greater numbers of MSW calls for psychosocial support. Further investigation and understanding of the use of Ads for SCD care, and the reduction during COVID, may have implications for current SCD management. Disclosures Cohen: GBT: Speakers Bureau.

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Bacterial Sepsis in Children Who Have Sickle Cell Disease

Pediatrics in Review ◽

10.1542/pir.15.4.136 ◽

1994 ◽

Vol 15 (4) ◽

pp. 136-162

Keyword(s):

Sickle Cell Disease ◽

Mortality Rate ◽

Sickle Cell ◽

Medical Center ◽

Albert Einstein College ◽

Albert Einstein ◽

Febrile Child ◽

Cell Disease ◽

Pneumococcal Sepsis ◽

Montefiore Medical

Montefiore Medical Center/Albert Einstein College of Medicine, Bronx, NY Infection is the principal cause of death in patients who have sickle cell disease. Because the peak incidence of death is between 1 and 3 years, during which period the mortality rate approaches 3%, it is extremely important for the pediatrician to evaluate and treat promptly the febrile child who has sickle cell disease. Under the age of 5 years, as many as 15% of children who have sickle cell disease develop sepsis or meningitis, with a 30% mortality rate. Children in this age group develop pneumococcal sepsis with 400 times the frequency of the general population. Haemophilus influenzae infection is 2 to 4 times as frequent as in other children.

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An Elevated TRV Is Associated with Venous Thromboembolism in Sickle Cell Disease

Blood ◽

10.1182/blood.v120.21.3222.3222 ◽

2012 ◽

Vol 120 (21) ◽

pp. 3222-3222

Author(s):

Rachel M Adams ◽

Lianne S Kopel ◽

Demedrick Anton Bland ◽

Elizabeth S Klings

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Conflicts Of Interest ◽

Medical Center ◽

Heart Catheterization ◽

Cell Disease ◽

Complete Evaluation ◽

Ct Angiogram ◽

Significant Difference ◽

History Of

Abstract Abstract 3222 Objective: An elevated tricuspid regurgitant jet velocity (TRV) by echocardiography, used to screen for pulmonary hypertension (PH) and mortality in sickle cell disease (SCD), likely has a multi-factorial etiology. SCD is a hyper-coagulable state, yet, the contribution of venous thromboembolic disease (VTE) to an elevated TRV is unknown. Based on the known association between VTE and PH, we hypothesized that SCD patients with an elevated TRV will have an increased prevalence of VTE. Methods: We reviewed data collected prospectively as part of the PH in SCD study conducted at Boston University/Boston Medical Center from 2004–2010. Subjects were included if they underwent both echocardiography and testing for VTE. An elevated TRV was defined as > 2.5 m/sec or the presence of PH on right heart catheterization. A history of VTE was defined by a positive CT/angiogram, ventilation/perfusion scan or Duplex ultrasound of an extremity (not catheter-related). Data were analyzed using a Chi-Square test and an odds ratio for VTE was calculated. Results: We reviewed the records of 162 patients enrolled in the PH of SCD study; 97 underwent both echocardiography and an evaluation for VTE.5/53 patients (9.4%) with normal echocardiography had a history of VTE, compared with 13/44 (29.5%) in the elevated TRV group. SCD patients with an elevated TRV were four times more likely to have a history of VTE compared to those with a normal echocardiogram (OR: 4.03, 95% CI 1.31–12.41, p=0.011). There was no significant difference in age, gender, history of asthma, hemoglobin genotype, hematologic profiles or renal function between patient groups. Conclusion: An elevated TRV and PH are associated with a history of VTE in SCD patients. This suggests a role for thrombosis in disease modulation and underscores the need for a complete evaluation for VTE in SCD patients with an elevated TRV. Disclosures: No relevant conflicts of interest to declare.

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Attitudes of Primary Care Physicians Toward Sickle Cell Disease Care, Guidelines, and Comanaging Hydroxyurea With a Specialist

Journal of Primary Care & Community Health ◽

10.1177/2150131916662969 ◽

2016 ◽

Vol 8 (1) ◽

pp. 37-40 ◽

Cited By ~ 6

Author(s):

Joseph Lunyera ◽

Charles Jonassaint ◽

Jude Jonassaint ◽

Nirmish Shah

Keyword(s):

Primary Care ◽

North Carolina ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Primary Care Physicians ◽

Mobile Apps ◽

Multidisciplinary Care ◽

Cell Disease ◽

Care Guidelines ◽

Complex Chronic Disease

Background: Sickle cell disease (SCD) is a complex chronic disease requiring multidisciplinary care that involves primary care physicians (PCPs) working with a hematologist or SCD specialists. However, PCPs often lack access to SCD specialists and are unaware of SCD guidelines or efficacious treatment. Methods: We partnered with Community Care of North Carolina (CCNC) to identify assigned PCPs for SCD patients with Medicaid across North Carolina. CCNC network administrators distributed a web-based questionnaire for completion. The questionnaire involved 12 self-reported items on a yes-no or a 1 to 5 Likert-type scale that assessed PCP attitudes toward SCD care, awareness of recent guidelines, and comanaging hydroxyurea. Results: Of the 53 PCPs who completed the electronic survey, 73% felt they were comfortable with the number of SCD patients in their practice. Most PCPs reported having infrequent communications with an SCD specialist (67%) and most were also not aware of the 2014 SCD guidelines (66%). Many reported that they would frequently use the new SCD guidelines if provided to them (76%). Furthermore, 51% of PCPs expressed comfort with using mobile apps to access SCD guidelines and provided email contact to receive further information. The majority also reported being comfortable comanaging hydroxyurea with an SCD specialist (65%). Conclusion: Few PCPs in North Carolina were aware of the new SCD guidelines or had regular communication with an SCD specialist. The majority of PCPs, however, demonstrated a favorable attitude toward receiving the SCD guidelines and comanaging hydroxyurea with a specialist. In response to this gap in care, we have developed a mobile-based SCD toolbox specifically for PCPs to provide guidelines, algorithms, and a method to communicate with local SCD specialists. With the interest in receiving these guidelines, we are confident the toolbox will provide an easy to use platform to assist PCPs to utilize the SCD guidelines.

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