Publications by authors named "Marsha J Treadwell"

28 Publications

  • Page 1 of 1

Improving Preventive Care for Children With Sickle Cell Anemia: A Quality Improvement Initiative.

Pediatr Qual Saf 2021 Jan-Feb;6(1):e379. Epub 2020 Dec 28.

Department of Pediatrics, University of California, San Francisco (UCSF), San Francisco, Calif.

Sickle cell disease is a complex chronic disorder associated with increased morbidity and early mortality. The Pediatric Quality Measures Program has developed new sickle cell-specific quality measures focused on hydroxyurea (HU) counseling and annual transcranial Doppler (TCD) screening; however, these measures have not been used in a clinical setting to inform quality improvement (QI) efforts.

Methods: From 2017 to 2018, 9 sickle cell subspecialty clinics from the Pacific Sickle Cell Regional Collaborative conducted a year-long QI collaborative focused on improving the percentage of patients with HU counseling and TCD screening based on the new quality measures. After an initial kick-off meeting, the 9 sites participated in monthly conference calls. We used run charts annotated with plan-do-study-act cycle activities to track each site's monthly progress and the overall mean percentage for the entire collaborative.

Results: There was an overall improvement in the aggregate HU counseling from 85% to 98% ( < 0.01). For TCD screening, referral frequency changed from 85% to 90% ( = 0.76). For both measures, the variation in frequencies decreased over the year.

Conclusion: Over 1 year, we found that a regional QI collaborative increased HU counseling. Although referral for TCD screening increased, there was no overall change in TCD completion. Overall, this QI report's findings can help clinicians adopt and implement these quality measures to improve outcomes in children.
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http://dx.doi.org/10.1097/pq9.0000000000000379DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7781296PMC
December 2020

A National Measurement Framework to Assess and Improve Sickle Cell Care in 4 US Regions.

Public Health Rep 2020 Jul/Aug;135(4):442-451. Epub 2020 Jul 8.

50980 National Institute for Children's Health Quality, Boston, MA, USA.

Objectives: Coordinated measurement strategies are needed to inform collaborative approaches to improve access to and quality of care for persons with sickle cell disease (SCD). The objective of our study was to develop a multilevel measurement strategy to assess improvements in access to and quality of care for persons with SCD in 4 US regions.

Methods: From 2014 through 2017, regional grantees in the Sickle Cell Disease Treatment Demonstration Program collected administrative and patient-level electronic health record (EHR) data to assess quality improvement initiatives. Four grantees-covering 29 US states and territories and an SCD population of 56 720-used a collective impact model to organize their work. The grantees collected administrative data from state Medicaid and Medicaid managed care organizations (MCOs) at multiple points during 2014-2017 to assess improvements at the population level, and local patient-level data were abstracted from site-level EHRs at regular intervals to track improvements over time.

Results: Administrative data were an important source of understanding population-level improvements but were delayed, whereas patient-level data were more sensitive to small-scale quality improvements.

Conclusions: We established a shared measurement approach in partnership with Medicaid and Medicaid MCO stakeholders that can be leveraged to effectively support quality improvement initiatives for persons with SCD in the United States.
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http://dx.doi.org/10.1177/0033354920935068DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7383758PMC
August 2020

Impact of Medicaid expansion on access and healthcare among individuals with sickle cell disease.

Pediatr Blood Cancer 2020 05 8;67(5):e28152. Epub 2020 Mar 8.

Department of Hematology/Oncology, University of California San Francisco Benioff Children's Hospital, Oakland, Illinois.

Purpose: Sickle cell disease (SCD) is associated with high acute healthcare utilization. The purpose of this study was to examine whether Medicaid expansion in California increased Medicaid enrollment, increased hydroxyurea prescriptions filled, and decreased acute healthcare utilization in SCD.

Methods: Individuals with SCD (≤65 years and enrolled in Medicaid for ≥6 total calendar months any year between 2011 and 2016) were identified in a multisource database maintained by the California Sickle Cell Data Collection Program. We describe trends and changes in Medicaid enrollment, hydroxyurea prescriptions filled, and emergency department (ED) visits and hospital admissions before (2011-2013) and after (2014-2016) Medicaid expansion in California.

Results: The cohort included 3635 individuals. Enrollment was highest in 2014 and lowest in 2016 with a 2.8% annual decease postexpansion. Although <20% of the cohort had a hydroxyurea prescription filled, the percentage increased by 5.2% annually after 2014. The ED visit rate was highest in 2014 and decreased slightly in 2016, decreasing by 1.1% annually postexpansion. Hospital admission rates were similar during the pre- and postexpansion periods. Young adults and adults had higher ED and hospital admission rates than children and adolescents.

Conclusions: Medicaid expansion does not appear to have improved enrollment or acute healthcare utilization among individuals with SCD in California. Future studies should explore whether individuals with SCD transitioned to other insurance plans or became uninsured postexpansion, the underlying reasons for low hydroxyurea utilization, and the lack of effect on hospital admissions despite a modest effect on ED visits.
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http://dx.doi.org/10.1002/pbc.28152DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7096276PMC
May 2020

Barriers to Pediatric Sickle Cell Disease Guideline Recommendations.

Glob Pediatr Health 2019 3;6:2333794X19847026. Epub 2019 May 3.

University of California, San Francisco, CA, USA.

National guidelines recommend that providers counsel all patients with sickle cell anemia about hydroxyurea (HU) therapy and screen children with sickle cell anemia annually for the risk of stroke with transcranial Doppler (TCD). We surveyed a national convenience sample of sickle cell disease clinicians to assess factors associated with low adherence. Adherence was 46% for TCD screening. Low adherence was associated with a lack of outcome expectancy (eg, a belief that there would be poor patient follow-up to TCD testing; < .05). Adherence was 72% for HU counseling. Practice barriers (eg, lack of support staff or time) and a lack of agreement with HU recommendations were associated with low adherence ( < .05). This study demonstrates that different types of strategies are needed to improve TCD screening (to address follow-up and access to testing) versus HU counseling (to address physician agreement and practice barriers).
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http://dx.doi.org/10.1177/2333794X19847026DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6501475PMC
May 2019

Lessons Learned from Building a Pediatric-to-Adult Sickle Cell Transition Program.

South Med J 2019 03;112(3):190-197

From the Department of Internal Medicine, Division of General Internal Medicine, Department of Pediatrics, Division of Pediatric Hematology/Oncology and Department of Psychology, Virginia Commonwealth University, Richmond, and Department of Hematology/Oncology, University of California, San Francisco Benioff Children's Hospital, San Francisco.

Objective: More effective transitions and transfers of young people with sickle cell disease (SCD) into the adult healthcare setting is a focus of both primary care and specialty care medical organizations. Effective transition and transfer requires six core elements: establishing a policy, tracking progress, administering transition readiness assessments, planning for adult care, transferring to adult care, and integrating into an adult practice. We developed a program using these six core elements. The objective of our report was to assess the development and implementation of this program.

Methods: We used the six core elements to develop and implement a program at Virginia Commonwealth University for children and adolescents with SCD to transition to adult health care.

Results: We assessed individuals' differences by age and grade, their independent living skills, their feelings about moving to adult care; tallied and analyzed several assessment scales; and assessed transfer success and patient retention.

Conclusions: The principles and lessons we learned in developing and implementing this program over 5 years, accompanied by caring, flexible, and dedicated care team members, often can overcome even severe barriers to care transitions.
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http://dx.doi.org/10.14423/SMJ.0000000000000950DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6590675PMC
March 2019

Relation Between Religious Perspectives and Views on Sickle Cell Disease Research and Associated Public Health Interventions in Ghana.

J Genet Couns 2018 Sep 1. Epub 2018 Sep 1.

Department of Hematology/Oncology, UCSF Benioff Children's Hospital Oakland, 747 52nd Street, Oakland, CA, 94609, USA.

Sickle cell disease (SCD) is highly prevalent in Africa with a significant public health burden for under-resourced countries. We employed qualitative research methods to understand the ethical, legal, and social implications of conducting genomic research in SCD under the Human Heredity and Health in Africa (H3Africa) initiative. The present study focused on religious and cultural aspects of SCD with the view to identifying beliefs and attitudes relevant to public health interventions in Ghana. Thematic analyses from individual and group interviews revealed six key areas of importance, namely, reliance on a supreme being; religion as a disruptive influence on health behaviors; role of religious leaders in information sharing and decision-making; social, religious, and customary norms; health and religious/supernatural beliefs; and need for social education and support through church and community. Findings suggest that public health programs in Ghana should not only aim at increasing knowledge and awareness about SCD and its management but also create an understanding of the relevance of genomics and alternative technological advancement to diagnosis and ethical decision-making around available options for health seeking. Future research should engage communities to help address the ethical and social implications of a persuasive religious influence on SCD-related health decisions.
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http://dx.doi.org/10.1007/s10897-018-0296-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6395545PMC
September 2018

Sensitivity of alternative measures of functioning and wellbeing for adults with sickle cell disease: comparison of PROMIS® to ASCQ-Me℠.

Health Qual Life Outcomes 2017 Jun 2;15(1):117. Epub 2017 Jun 2.

Division of Hematology, University of Colorado, 12700 E. 19th Avenue, Rm 9122 RC 2/MS B170, Aurora, CO, 80045, USA.

Background: Sickle Cell Disease (SCD) causes profound suffering and decrements in daily functioning. Demand is growing for valid and reliable measures to systematically document these effects, particularly in adults. The Adult Sickle Cell Quality of Life Measurement System, ASCQ-Me℠, was developed for this purpose. ASCQ-Me℠ is one of four measurement systems housed within the Person-Centered Assessment Resource (PCAR), funded by the National Institutes of Health, to support clinical research. To help users select the best of these measures for adults with SCD, we evaluated and compared two PCAR systems: one designed to be "universally applicable" (the Patient-Reported Outcome Measurement Information System, PROMIS®) and one designed specifically for SCD (ASCQ-Me℠).

Methods: Respondents to PROMIS and ASCQ-Me questions were 490 adults with SCD from seven geographically-disbursed clinics within the US. Data were collected for six ASCQ-Me measures (Emotional Impact, Sleep Impact, Social Impact, Stiffness Impact, Pain Impact, SCD Pain Episode Frequency and Severity) and ten PROMIS measures (Pain Impact, Pain Behavior, Physical Functioning, Anxiety, Depression, Fatigue, Satisfaction with Discretionary Social Activities, Satisfaction with Social Roles, Sleep Disturbance, and Sleep-Related Impairment). Statistical analyses, including analysis of variance and multiple linear regression, were conducted to determine the sensitivity of measures to SCD severity. SCD severity was assessed via a checklist of associated treatments and conditions.

Results: For those with the most severe SCD, PROMIS scores showed worse health compared to the general population for nine of ten health domains: the magnitude of the difference ranged 0.5 to 1.1 standard deviation units. The PROMIS domains most severely affected were Physical Functioning and Pain (Impact and Behavior). Significant differences by tertile of the SCD-MHC were shown for most PROMIS short forms and all ASCQ-Me short and fixed forms. In most models, ASCQ-Me measures explained statistically significant unique variance in SCD-MHC scores complementary to that explained by corresponding PROMIS measures.

Conclusions: Study results supported the validity of both PROMIS and ASCQ-Me measures for use in adults with SCD. Compared to comparable PROMIS scores, most ASCQ-Me scores were better predictors of SCD disease severity, as measured by a medical history checklist. The clinical implications of these results require further investigation.
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http://dx.doi.org/10.1186/s12955-017-0661-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5455105PMC
June 2017

Long-Term Pulmonary Function and Quality of Life in Children After Acute Respiratory Distress Syndrome: A Feasibility Investigation.

Pediatr Crit Care Med 2017 01;18(1):e48-e55

1Division of Pediatric Critical Care, Department of Pediatrics, UCSF Benioff Children's Hospitals, San Francisco and Oakland, CA.2Department of Anesthesia, Critical Care and Pain Medicine, Massachusetts General Hospital, Boston, MA.3Department of Hematology/Oncology, UCSF Benioff Children's Hospital, Oakland, CA.4Division of Pediatric Pulmonology, Department of Pediatrics, UCSF Benioff Children's Hospital, San Francisco, CA.5Division of Pediatric Critical Care, University of Michigan C.S. Mott Children's Hospital, Ann Arbor, MI.

Objectives: To determine the feasibility of pulmonary function and quality of life evaluations in children after acute respiratory distress syndrome.

Design: A prospective follow-up feasibility study.

Setting: A tertiary PICU.

Patients: Children less than 18 years old with acute respiratory distress syndrome admitted between 2000 and 2005.

Intervention: Pulmonary function testing and patient and parental quality of life surveys approximately 12-month after acute respiratory distress syndrome.

Measurements And Main Results: One hundred eighty patients met acute respiratory distress syndrome criteria; 37 (20%) died, 90 (51%) declined participation, 28 (16%) consented but did not return, and 24 (13%) returned for follow-up visit. Twenty-three patients completed quality of life testing and 17 completed pulmonary functions. Clinical characteristics of those who returned were no different from those who did not except for age (median age, 4.9 vs 1.8 yr). One-third had mild to moderate pulmonary function deficits. Quality of life scores were marginal with general health perception, physical functioning, and behavior being areas of concern. These scores were lower than scores in children with chronic asthma. Parental quality of life assessments report lower scores in single-parent homes but no differences were noted by race or parental employment status.

Conclusions: Valuable information may be discerned from acute respiratory distress syndrome patients who return for follow-up evaluation. In this pilot study, up to one-third of children with acute respiratory distress syndrome exhibit pulmonary function deficits and 12-month postillness quality of life scores are lower than in children with chronic asthma. Parental perceptions of postillness quality of life may be negatively impacted by socioeconomic constraints. Long-term follow of children with acute respiratory distress syndrome is feasible and bears further investigation.
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http://dx.doi.org/10.1097/PCC.0000000000001014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5221949PMC
January 2017

Emergency department utilization by Californians with sickle cell disease, 2005-2014.

Pediatr Blood Cancer 2017 06 21;64(6). Epub 2016 Dec 21.

Division of Blood Disorders, Centers for Disease Control and Prevention, Atlanta, Georgia.

Background: Clinical care for children and adults living with sickle cell disease (SCD) is often provided in the emergency department (ED). Population-based surveillance data can be used to describe the ED utilization patterns of this patient population.

Procedure: A cohort of pediatric and adult California patients with SCD was identified from multiple data sources, and 10 years (2005-2014) of their treat-and-release ED utilization data were analyzed.

Results: Among a cohort of 4,636 patients with SCD, 4,100 (88%) had one or more treat-and-release ED visits. There were 2.1 mean annual visits per person for the cohort (median 0.7; range 0-185). In a single year (2005), 53% had 0 treat-and-release ED visits, 35% had 1-3 visits, 9% had 4-10 visits, and 3% had 11 or more visits; this highest utilization group accounted for 45% of all patients' ED visits. ED utilization in this cohort was highest among young adults and also higher among older adults than pediatric patients.

Conclusion: The majority of identified patients in each of the 10 years did not go to the ED, but nearly all had one or more such visits over the full span of time. This study highlights the power and utility of a multisource longitudinal data collection effort for SCD. Further study of the segment of the population with highest ED utilization may highlight areas where changes in healthcare and health policy could improve and extend the lives of patients with SCD.
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http://dx.doi.org/10.1002/pbc.26390DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5403550PMC
June 2017

Quality of care in sickle cell disease: Cross-sectional study and development of a measure for adults reporting on ambulatory and emergency department care.

Medicine (Baltimore) 2016 Aug;95(35):e4528

American Institutes for Research, Chapel Hill, NC Department of Hematology/Oncology, University of California San Francisco Benioff Children's Hospital Oakland, Oakland, CA Redwood City, CA Division of Hematology, University of Colorado, Aurora, CO Blood Epidemiology and Clinical Therapeutics Branch, Division of Blood Diseases and Resources, National Heart, Lung, and Blood Institute, Bethesda, MD Division of General Internal Medicine, Virginia Commonwealth University, Richmond, VA.

Documented deficiencies in adult sickle cell disease (SCD) care include poor access to knowledgeable providers and inadequate treatment in emergency departments (EDs).The aim of this study was to create patient-reported outcome measures of the quality of ambulatory and ED care for adults with SCD.We developed and pilot tested SCD quality of care questions consistent with Consumer Assessments of Healthcare Providers and Systems surveys. We applied psychometric methods to develop scores and evaluate reliability and validity.The participants of this study were adults with SCD (n = 556)-63% aged 18 to 34 years; 64% female; 64% SCD-SS-at 7 US sites.The measure used was Adult Sickle Cell Quality of Life Measurement information system Quality of Care survey.Most participants (90%) reported at least 1 severe pain episode (pain intensity 7.8 ± 2.3, 0-10 scale) in the past year. Most (81%) chose to manage pain at home rather than the ED, citing negative ED experiences (83%). Using factor analysis, we identified Access, Provider Interaction, and ED Care composites with reliable scores (Cronbach α 0.70-0.83) and construct validity (r = 0.32-0.83 correlations with global care ratings). Compared to general adult Consumer Assessments of Healthcare Providers and Systems scores, adults with SCD had worse care, adjusted for age, education, and general health.Results were consistent with other research reflecting deficiencies in ED care for adults with SCD. The Adult Sickle Cell Quality of Life Measurement Quality of Care measure is a useful self-report measure for documenting and tracking disparities in quality of SCD care.
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http://dx.doi.org/10.1097/MD.0000000000004528DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5008546PMC
August 2016

Community engagement to inform the development of a sickle cell counselor training and certification program in Ghana.

J Community Genet 2016 Jul 18;7(3):195-202. Epub 2016 Apr 18.

Sickle Cell Foundation of Ghana, Kumasi, Ghana.

Sickle cell disease (SCD) and sickle cell trait (SCT) are highly prevalent in Africa. Despite public health implications, there is limited understanding of community issues for implementing newborn screening and appropriate family counseling. We conducted a 3-day workshop in Kumasi, Ghana, with community leaders as lay program development advisors to assist the development and implementation of a Sickle Cell Counselor Training and Certification Program. We employed qualitative methods to understand cultural, religious, and psychosocial dimensions of SCD and SCT, including the advisors' attitudes and beliefs in relation to developing a culturally sensitive approach to family education and counseling that is maximally suited to diverse communities in Ghana. We collated advisors' discussions and observations in order to understand community issues and potential challenges and guide strategies for advocacy in SCD family education and counseling. Results from the workshop revealed that community leaders representing diverse communities in Ghana were engaged constructively in discussions about developing a culturally sensitive counselor training program. Key findings included the importance of improved knowledge about SCD among the public and youth in particular, the value of stakeholders such as elders and religious and traditional leaders, and government expectations of reduced SCD births. We submitted a report to the Ministry of Health in Ghana with recommendations for the next steps in developing a national sickle cell counselor training program. We named the program "Genetic Education and Counseling for Sickle Cell Conditions in Ghana" (GENECIS-Ghana). The first GENECIS-Ghana Training and Certification Program Workshop was conducted from June 8 to 12, 2015.
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http://dx.doi.org/10.1007/s12687-016-0267-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4960026PMC
July 2016

Teaching About Genetics and Sickle Cell Disease In Fifth Grade.

J Natl Med Assoc 2015 Feb 2;107(1):4-10. Epub 2015 Dec 2.

UCSF Benioff Children's Hospital Oakland.

Acknowledgments: We are grateful to Laura McVittie Gray for her work on the development of the student activities described in this article. This work was made possible by a Science Education Partnership Award (SEPA), Grant Number R25RR020449, from the National Center for Research Resources (NCRR), a component of the National Institutes of Health (NIH). Additional support for this SEPA-funded project was provided by Grant Number UL1RR024131-01 from NCRR. Its contents are solely the responsibility of the authors and do not necessarily represent the official views of NCRR or NIH.

Abstract: A 5-lesson, 5th-grade instructional unit, "Genetics and Sickle Cell Disease," was developed and tested as part of a 40-lesson curriculum entitled SEEK (Science Exploration, Excitement, and Knowledge): A Curriculum in Health and Biomedical Science for Diverse 4th and 5th Grade Students. The genetics lessons include hands-on activities (e.g., DNA extraction from cheek cells), a simulated plant genetics experiment, and a classroom visit by a person with sickle cell disease, as well as by a health care practitioner who works with sickle cell patients or a scientist specializing in genetics. The unit was tested with 82 5th-grade students at public elementary schools in Oakland, CA; 96% were racial and ethnic minorities. The comparison group consisted of 84 5th-grade Oakland students racially/ ethnically, academically, and socio-economically matched to those in the experimental group. Both groups completed a 20-question, multiple-choice pre/posttest covering science concepts, scientific process, lifestyle choices, and careers. The experimental group showed significant improvement on 13 of 20 questions (P<.05, t-tests) and on the test as a whole, whereas the comparison group did not show significant improvement either on any of the questions or on the test as a whole. The experimental group improved on 10 concept questions, 2 scientific process questions, and 1 lifestyle question. Teachers rated the educational value of the unit as 9.5 on a scale from 1 (low) to 10 (high). These results show that genetics and sickle cell disease can be taught successfully in 5th grade, although they are not typically covered at this level.
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http://dx.doi.org/10.1016/S0027-9684(15)30003-1DOI Listing
February 2015

Using formative research to develop a counselor training program for newborn screening in Ghana.

J Genet Couns 2015 Apr 7;24(2):267-77. Epub 2014 Sep 7.

Hematology/Oncology Department, UCSF Benioff Children's Hospital Oakland, 747 52nd Street, Oakland, CA, 94609, USA,

Sickle cell disease (SCD), sickle cell trait (SCT) and related conditions are highly prevalent in sub-Saharan Africa. Despite the public health implications, there is limited understanding of the unique needs regarding establishing and implementing extensive screening for newborns and appropriate family counseling. We sought to gain understanding of community attitudes and beliefs about SCD/SCT from counselors and potential counselors in Ghana; obtain their input about goals for counseling following newborn screening; and obtain guidance about developing effective counselor education. Five focus groups with 32 health care providers and health educators from 9 of 10 regions in Ghana were conducted by trained facilitators according to a structured protocol. Qualitative data were coded and categorized to reflect common themes. Saturation was achieved in themes related to genetics/inheritance; common complications of SCD; potential for stigmatization; marital strain; and emotional stress. Misconceptions about SCT as a form of SCD were prevalent as were cultural and spiritual beliefs about the causes of SCD/SCT. Potential positive aspects included affected children's academic achievement as compensation for physical limitations, and family cohesion. This data informed recommendations for content and structure of a counselor training program that was provided to the Ministry of Health in Ghana.
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http://dx.doi.org/10.1007/s10897-014-9759-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4501216PMC
April 2015

Patient reports of health outcome for adults living with sickle cell disease: development and testing of the ASCQ-Me item banks.

Health Qual Life Outcomes 2014 Aug 22;12:125. Epub 2014 Aug 22.

Department of Health Policy and Research, Quality and Performance Measurement Program, American Institutes for Research, 100 Europa Drive, Suite 315, Chapel Hill 27517-2357, NC, USA.

Background: Providers and patients have called for improved understanding of the health care requirements of adults with sickle cell disease (SCD) and have identified the need for a systematic, reliable and valid method to document the patient-reported outcomes (PRO) of adult SCD care. To address this need, the Adult Sickle Cell Quality of Life Measurement System (ASCQ-Me) was designed to complement the Patient Reported Outcome Measurement Information System (PROMIS®). Here we describe methods and results of the psychometric evaluation of ASCQ-Me item banks (IBs).

Methods: At seven geographically-disbursed clinics within the US, 556 patients responded to questions generated to assess cognitive, emotional, physical and social impacts of SCD. We evaluated the construct validity of the hypothesized domains using exploratory factor analysis (EFA), parallel analysis (PA), and bi-factor analysis (Item Response Theory Graded Response Model, IRT-GRM). We used IRT-GRM and the Wald method to identify bias in responses across gender and age. We used IRT and Cronbach's alpha coefficient to evaluate the reliability of the IBs and then tested the ability of summary scores based on IRT calibrations to discriminate among tertiles of respondents defined by SCD severity.

Results: Of the original 140 questions tested, we eliminated 48 that either did not form clean factors or provided biased measurement across subgroups defined by age and gender. Via EFA and PA, we identified three subfactors within physical impact: sleep, pain and stiffness impacts. Analysis of the resulting six item sets (sleep, pain, stiffness, cognitive, emotional and social impacts of SCD) supported their essential unidimensionality. With the exception of the cognitive impact IB, these item sets also were highly reliable across a broad range of values and highly significantly related to SCD disease severity.

Conclusion: ASCQ-Me pain, sleep, stiffness, emotional and social SCD impact IBs demonstrated exceptional measurement properties using modern and classical psychometric methods of evaluation. Further development of the cognitive impact IB is required to improve its sensitivity to differences in SCD disease severity. Future research will evaluate the sensitivity of the ASCQ-Me IBs to change in SCD disease severity over time due to health interventions.
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http://dx.doi.org/10.1186/s12955-014-0125-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4243820PMC
August 2014

A Quality Improvement Initiative to Improve Emergency Department Care for Pediatric Patients with Sickle Cell Disease.

J Clin Outcomes Manag 2014 Feb;21(2):62-70

Children's Hospital & Research Center Oakland, Oakland, CA.

Objective: To determine whether a quality improvement (QI) initiative would result in more timely assessment and treatment of acute sickle cell-related pain for pediatric patients with sickle cell disease (SCD) treated in the emergency department (ED).

Methods: We created and implemented a protocol for SCD pain management in the ED with the goals of improving (1) mean time from triage to first analgesic dose; (2) percentage of patients that received their first analgesic dose within 30 minutes of triage, and (3) percentage of patients who had pain assessment performed within 30 minutes of triage and who were re-assessed within 30 minutes after the first analgesic dose.

Results: Significant improvements were achieved between baseline (55 patient visits) and post order set implementation (165 visits) in time from triage to administration of first analgesic (decreased from 89.9 ± 50.5 to 35.2 ± 22.8 minutes, < 0.001); percentage of patient visits receiving pain medications within 30 minutes of triage (from 7% to 53%, < 0.001); percentage of patient visits assessed within 30 minutes of triage (from 64% to 99.4%, < 0.001); and percentage of patient visits re-assessed within 30 minutes of initial analgesic (from 54% to 86%, < 0.001).

Conclusions: Implementation of a QI initiative in the ED led to expeditious care for pediatric patients with SCD presenting with pain. A QI framework provided us with unique challenges but also invaluable lessons as we address our objective of decreasing the quality gap in SCD medical care.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4580258PMC
February 2014

A biopsychosocial-spiritual model of chronic pain in adults with sickle cell disease.

Pain Manag Nurs 2013 Dec 14;14(4):287-301. Epub 2011 Dec 14.

Department of Physiological Nursing University of California, San Francisco.

Chronic pain in adults with sickle cell disease (SCD) is a complex multidimensional experience that includes biologic, psychologic, sociologic, and spiritual factors. To date, three models of pain associated with SCD (i.e., biomedical model, biopsychosocial model for SCD pain, and Health Beliefs Model) have been published. The biopsychosocial multidimensional approach to chronic pain developed by Turk and Gatchel is a widely used model of chronic pain. However, this model has not been applied to chronic pain associated with SCD. In addition, a spiritual/religious dimension is not included in this model. Because spirituality/religion is central to persons affected by SCD, that dimension needs to be added to any model of chronic pain in adults with SCD. In fact, data from one study suggest that spirituality/religiosity is associated with decreased pain intensity in adults with chronic pain from SCD. A biopsychosocial-spiritual model is proposed for adults with chronic pain from SCD, because it embraces the whole person. This model includes the biologic, psychologic, sociologic, and spiritual factors relevant to adults with SCD based on past and current research. The purpose of this paper is to describe an adaptation of Turk and Gatchel's model of chronic pain for adults with SCD and to summarize research findings that support each component of the revised model (i.e., biologic, psychologic, sociologic, spiritual). The paper concludes with a discussion of implications for the use of this model in research.
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http://dx.doi.org/10.1016/j.pmn.2011.06.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3857562PMC
December 2013

Adult sickle cell quality-of-life measurement information system (ASCQ-Me): conceptual model based on review of the literature and formative research.

Clin J Pain 2014 Oct;30(10):902-14

*Children's Hospital & Research Center Oakland, Oakland, CA †Division of Hematology, University of Colorado, Aurora, CO ‡American Institutes for Research, Washington, DC.

Objectives: Research-derived evidence about the impact of sickle cell disease (SCD) on the lives of affected adults is lacking. We conducted formative research to provide the basis for a comprehensive description of how SCD affects the lives of adults, with the goal of developing a SCD-specific quality-of-life measurement system.

Methods: We conducted a comprehensive literature review of patient-reported outcomes, followed by a series of focus groups and structured individual interviews with adults with SCD (n=122) and their health care providers (n=15).

Results: We reviewed 473 abstracts and included 86 articles in the final review. The literature revealed broad categories of the impact of SCD and its treatment on the lives of adults-pain; emotional distress; social-role functioning; overall quality-of-life; and quality of care. We classified 1213 incidents from the focus groups and interviews into a taxonomy (16 domains) that met the criterion for saturation and was demonstrated to be reliable for the classification of incidents. The final conceptual model was built upon the taxonomy.

Discussion: Our conceptual model was similar to previous models with the effects of pain predominating, interwoven with emotional distress, quality of care, and stigmatization. We found a broad range of emotions reflected, including positive effects of SCD. Items for the quality-of-life measure were derived from the taxonomy and the conceptual model may be of use in generating hypotheses for clinical research and improving understanding for clinicians of the lived experience of adults with SCD.
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http://dx.doi.org/10.1097/AJP.0000000000000054DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4993284PMC
October 2014

Screening U.S. college athletes for their sickle cell disease carrier status.

Am J Prev Med 2011 Dec;41(6 Suppl 4):S406-12

Sickle Cell Disease Association of America, Baltimore, Maryland, USA.

There are many issues surrounding the screening of collegiate athletes for their sickle cell disease carrier status (or sickle cell trait), a genetic condition. This paper summarizes the establishment of expert advice given to the Secretary's Advisory Committee on Heritable Disorders in Newborns and Children (SACHDNC) on the issue. The SACHDNC has developed a report to advise the Secretary of the USDHHS about the 2010 rule of the National Collegiate Athletic Association (NCAA) requiring testing for sickle cell trait in all incoming Division I student athletes. The SACHDNC does not support the NCAA's rule to screen collegiate athletes for sickle cell trait.
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http://dx.doi.org/10.1016/j.amepre.2011.09.014DOI Listing
December 2011

Autonomic nervous system reactivity: children with and without sickle cell disease.

Nurs Res 2011 May-Jun;60(3):197-207

Department of Hematology/Oncology, Children's Hospital & Research Center Oakland, California 94609, USA.

Background: Previous studies of healthy children have indicated a link between autonomic nervous system (ANS) reactivity and health outcomes, but there is limited research on whether ANS reactivity is similar for children with chronic conditions.

Objective: The aim of this study was to determine if ANS reactivity differs for children with sickle cell disease (SCD) compared with a community sample of children without SCD.

Method: In two cross-sectional, descriptive studies, 32 public school children without chronic health problems were compared with 33 children with SCD. The children were 5-8 years old and they completed standardized protocols measuring ANS responses (respiratory sinus arrhythmia and preejection period) during rest and challenge conditions in social, cognitive, sensory, and emotion domains. Reactivity was calculated as the difference between challenge response minus rest for each domain and overall.

Results: There were differences in the distributions of the samples in parent education and child age, so these variables were adjusted for in subsequent analyses. The community sample showed parasympathetic withdrawal (low respiratory sinus arrhythmia scores) and greater parasympathetic reactivity (low respiratory sinus arrhythmia difference scores and percentage of negative scores) compared with the children with SCD in the social (p < .05) and sensory (p < .05) domains. The children with SCD showed greater sympathetic reactivity (low preejection period difference scores) compared with the community children in the cognitive domain (p < .05), and a greater percentage of children with SCD versus the community children showed negative preejection period difference scores (sympathetic reactivity) in the social domain (p < .05). The community sample, but not the children with SCD, showed changes in respiratory sinus arrhythmia across domains (p < .05).

Discussion: Children with SCD may display a different pattern of ANS responses to laboratory challenges compared with children without SCD from the same community.
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http://dx.doi.org/10.1097/NNR.0b013e3182186a21DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3130063PMC
July 2011

Chart Card: feasibility of a tool for improving emergency department care in sickle cell disease.

J Natl Med Assoc 2010 Nov;102(11):1017-23

Children's Hospital and Research Center Oakland, 747 52nd St, Oakland, CA 94609, USA.

Background: Patients with sickle cell disease (SCD) are concerned with emergency department care, including time to treatment and staff attitudes and knowledge. Providers are concerned about rapid access to patient information and SCD treatment protocols. A software application that stores and retrieves encrypted personal medical information on a plastic credit card-sized Chart Card was designed.

Objective: To determine the applicability and feasibility of the Chart Card on patient satisfaction with emergency department care and provider accessibility to patient information and care protocols.

Methods: One-half of 44 adults (aged -18 years) and 50 children with SCD were randomized to either the Chart Card or usual care. Patient satisfaction was surveyed pre and post implementation of the Chart Card program, and emergency department staff was surveyed about familiarity with SCD treatment protocols.

Conclusion: Patient satisfaction with emergency department care and efficacy in health care increased post Chart Card implementation. Providers valued immediate access to patient information and SCD treatment guidelines. The technology has potential for application in the treatment of other illnesses in other settings.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4950679PMC
http://dx.doi.org/10.1016/s0027-9684(15)30728-8DOI Listing
November 2010

A review of the literature on the multiple dimensions of chronic pain in adults with sickle cell disease.

J Pain Symptom Manage 2010 Sep 24;40(3):416-35. Epub 2010 Jul 24.

Department of Physiological Nursing, University of California at San Francisco, San Francisco, California 94143-0610, USA.

Sickle cell disease (SCD) is a major health care and societal problem that affects millions of people worldwide. In Nigeria, 45,000 to 90,000 babies are born each year with SCD. In the United States, SCD is the most common genetic disorder, affecting more than 80,000 people, the majority of whom are African American. Sickle cell pain is the hallmark feature of SCD. Most of the research on pain from SCD has focused on children with acute pain associated with sickle cell crisis. Consequently, very little is known about the occurrence and characteristics of chronic pain, especially in adults with SCD. Individuals with SCD who experience chronic pain are often underserved, and their pain is undertreated. This undertreatment may result in millions of dollars per year spent on emergency room visits, hospitalizations, and lost work productivity. The primary purpose of this literature review was to summarize the findings from studies that evaluated the characteristics of chronic pain in adults with SCD. Each of the studies included in this review was evaluated to determine if it provided data on the following multidimensional characteristics of chronic pain: occurrence, number of pain episodes, duration, pattern, quality, location, intensity, aggravating factors, relieving factors, and impact of pain on function. A secondary purpose was to identify gaps in knowledge and directions for future research on the multiple dimensions of chronic pain in adults with SCD.
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http://dx.doi.org/10.1016/j.jpainsymman.2009.12.027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2989986PMC
September 2010

Stress reactivity as a moderator of family stress, physical and mental health, and functional impairment for children with sickle cell disease.

J Dev Behav Pediatr 2010 Jul-Aug;31(6):491-7

Hematology/Oncology Department, Children's Hospital & Research Center, Oakland, CA 94609, USA.

Objective: To evaluate whether autonomic nervous system (ANS) reactivity modifies the relation between family stress, and physical and mental health, and functional impairment for children with sickle cell disease.

Methods: Thirty-eight 5-to 8-year old children with sickle cell disease completed a 20-minute ANS reactivity protocol measuring respiratory sinus arrhythmia and pre-ejection period during comparison and challenge tasks in social, cognitive, sensory, and emotion domains. Domain-specific reactivity was calculated as the difference between challenge and comparison tasks; overall reactivity was calculated across domains as the mean of the difference scores. ANS profile scores combined the overall respiratory sinus arrhythmia and pre-ejection period reactivity scores. Caregivers completed measures of family stress, child physical and mental health symptoms, and functional impairment.

Results: Family stress was associated with child functional impairment whereas overall and cognitive ANS reactivity was associated with co-morbid internalizing and externalizing mental health symptoms. Interaction models showed that children with the classic ANS profile (parasympathetic inhibition and sympathetic activation) in the cognitive and emotion domains were most vulnerable to the effects of stress, with more functional impairment and injuries when family stress was high, controlling for age, sex, and parent education.

Conclusion: The costs to patients and families in diminished quality of life and to the health care system could be reduced by further exploration of strategies to identify children with sickle cell disease who are most vulnerable under conditions of high family stress and heightened psychobiologic reactivity.
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http://dx.doi.org/10.1097/DBP.0b013e3181e2830fDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4593493PMC
January 2011

Detection and assessment of stroke in patients with sickle cell disease: neuropsychological functioning and magnetic resonance imaging.

Pediatr Hematol Oncol 2008 Jun;25(5):409-21

Department of Anesthesiology Critical Care Medicine, Keck School of Medicine, University of Southern California, and Childrens Hospital Los Angeles, Los Angeles, California 90027, USA.

Sickle cell disease (SCD) is associated with increased risk of stroke and cognitive impairment. This study describes a retrospective review of 65 patients who underwent routine neuropsychological testing and MRI during treatment at a comprehensive sickle cell center. It was hypothesized that (1) children with no evidence of CVA would perform lower than expected on cognitive tasks compared to population-based normative data, (2) children with strokes and children with silent infarcts would perform lower on cognitive tasks and motor skills as compared to patients with no evidence of CVA, and (3) children with evidence of silent infarcts would perform better than children with known overt strokes. This final hypothesis has not been studied previously, as children with known overt stroke and silent infarct were grouped together. Sixty-five children with SCD who were sent for routine neuropsychological testing and brain MRI were identified via retrospective chart review. Patients had been administered neuropsychological tests to assess cognitive, executive and motor function. Brain MRI was obtained from each patient and was analyzed for evidence of cerebrovascular accident (CVA). Based on MRI analysis, 27% of patients with SCD had experienced a stroke and 13% a silent infarct. The majority (59%) of patients diagnosed with stroke or infarct sustained cortical damage to the frontal lobe. Patients with SCD and no evidence of CVA functioned normally on tests of cognitive ability and achievement, but patients with CVA displayed impairments in cognitive function and comparatively lower scores on verbal and performance scales. Neuropsychological testing can identify impairments in patients with SCD with no known cerebrovascular accident. Investigations of neurocognitive functioning will help characterize patterns of deficits and can inform the ability to implement comprehensive care strategies for patients with SCD and cognitive impairment.
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http://dx.doi.org/10.1080/08880010802107497DOI Listing
June 2008

Using qualitative and quantitative strategies to evaluate knowledge and perceptions about sickle cell disease and sickle cell trait.

J Natl Med Assoc 2006 May;98(5):704-10

Children's Hospital and Research Center at Oakland, 747 52nd St., Oakland, CA, USA.

Objectives: To evaluate knowledge, perceptions and the effectiveness of different sources of information about sickle cell trait (SCT) and sickle cell disease (SCD); to determine individual knowledge of SCT status.

Methods: 28 individuals participated in three focus groups (healthcare providers, people affected by SCD or SCT, and community members). Surveyors interviewed 282 respondents within their neighborhoods.

Results: Common themes across the focus groups included the limited general awareness of SCD and SCT, the emphasis on the benign nature of SCT rather than on future implications, and the need for public health education campaigns about SCD and SCT involving media strategies. The majority of community survey respondents (n = 243, 86.2%) had correct general knowledge about the genetic basis and severity of SCD, but only 16% (n = 45) knew their own trait status. When respondents had received information about SCD from friends and acquaintances, they were three times more likely to know their SCT status, compared with respondents who had not received information from a personal source (p < 0.01).

Conclusions: Despite a screening history in the 1970s fraught with controversy, sickle cell disease management and detection can be a model for the empowerment of communities in making informed decisions about theirs and their families' futures, given the burgeoning of genetic information.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2569269PMC
May 2006

Barriers to adherence of deferoxamine usage in sickle cell disease.

Pediatr Blood Cancer 2005 May;44(5):500-7

Children's Hospital and Research Center at Oakland, Oakland, California 94609, USA.

Background: We hypothesized that child cognitive disability would be a significant risk factor for non-adherence with home deferoxamine (DFO) administration and that a factor that would contribute to improved adherence would be sharing of responsibilities for chelation between parents and patients. We explored the influences on adherence of behavioral and psychological adjustment; family stress; perceived convenience of and satisfaction with the DFO regimen; and parent and patient knowledge about DFO.

Procedure: Fifteen pediatric patients with sickle cell disease (SCD) who had evidence of excessive iron stores, and their parents, were interviewed about adherence and responsibility for chelation therapy. A neuropsychological assessment battery was administered to the patients. Family stress, the child's emotional and behavioral status, knowledge about chelation and iron overload were explored. Adherence was rated objectively using pharmacy refill patterns and observable signs of chelation.

Results: Sharing of responsibilities for chelation between parents and children was related to better adherence while neuropsychological status bore a complex relation to adherence. Of the exploratory variables, low family stress were related to better adherence while satisfaction with the home care regimen and convenience ratings were not useful in predicting adherence. No one element of adherence, even objective measures, was capable of classifying adherence, while a multifactorial scheme categorizing adherent, partially adherent and non-adherent groups demonstrated good face validity.

Conclusions: Supporting developmentally appropriate sharing of responsibilities for self-care is critical, taking patient neurocognitive status into consideration. Clinicians should evaluate adherence using a multifactorial model that highlights the most salient targets for intervention.
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http://dx.doi.org/10.1002/pbc.20290DOI Listing
May 2005

Using quality improvement strategies to enhance pediatric pain assessment.

Int J Qual Health Care 2002 Feb;14(1):39-47

Sickle Cell Center, Children's Hospital Oakland, CA 94609, USA.

Objective: To evaluate the impact of a quality improvement approach to implementing developmentally appropriate pain assessment guidelines for pediatric inpatients. Patient and staff satisfaction with pain assessment and management, and staff documentation were evaluated one year following the implementation of the revised pain assessment guidelines.

Design: Quasi-experimental design.

Setting: The pediatric hematology/oncology unit of a regional children's hospital. Study participants. A convenience sample of 36 children and 68 staff at time 1 (TI) and 49 children and 82 staff at time 2 (T2).

Interventions: Staff were educated on the use of pediatric pain assessment tools and a standardized pain assessment protocol was put into practice. Children or their primary caregivers were interviewed, using a questionnaire modified from the American Pain Society quality assurance guidelines, regarding their experiences with pain assessment and management on the unit at T1, just prior to the staff education, and one year later at T2. Multidisciplinary unit staff completed a parallel questionnaire at T1 and T2.

Main Outcome Measures: Patient and staff satisfaction with pain assessment and management, and chart audit of compliance with pain assessment documentation.

Results: Patients, family members, and staff reported increased pain assessment, improved staff responsiveness to patients' pain and greater satisfaction with assessment tools between TI and T2. Increased compliance with the assessment guidelines was confirmed by chart audit.

Conclusions: Improved pain assessment and management practices with resultant increase in patient and staff satisfaction can be achieved and sustained over time using quality improvement strategies.
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http://dx.doi.org/10.1093/intqhc/14.1.39DOI Listing
February 2002