Background Hematopoietic stem cell transplantation (HSCT) is a treatment option with curative int... more Background Hematopoietic stem cell transplantation (HSCT) is a treatment option with curative intent for patients with transfusion dependent thalassemia (TDT) but its application is limited by the lack of suitable donors and acceptability due to the related morbidity/mortality. Transplantation of autologous genetically modified hematopoietic cells, gene therapy (GT) is emerging as a promising treatment option for TDT as it eliminates graft versus host disease (GVHD) and need for immunosuppression. Early results of GT suggest that many, but not all patients achieve transfusion independence after the procedure. There is little information about the acceptability of GT in patients with TDT. We sought to examine patient/family knowledge about GT in TDT and to examine factors that influence decision-making about this therapy. Methods Parents of children with TDT and adults with TDT were who provided informed consent underwent semi-structured interviews to understand patient/family knowle...
Background Individuals living with sickle cell disease (SCD) may benefit from a variety of diseas... more Background Individuals living with sickle cell disease (SCD) may benefit from a variety of disease-modifying therapies, including hydroxyurea, voxelotor, crizanlizumab, L-glutamine, and chronic blood transfusions. However, allogeneic hematopoietic stem cell transplantation (HCT) remains the only nonexperimental treatment with curative intent. As HCT outcomes can be influenced by the complex interaction of several risk factors, HCT can be a difficult decision for health care providers to make for their patients with SCD. Objective The aim of this study is to determine the acceptability and usability of a prototype decision support tool for health care providers in decision-making about HCT for SCD, together with patients and their families. Methods On the basis of published transplant registry data, we developed the Sickle Options Decision Support Tool for Children, which provides health care providers with personalized transplant survival and risk estimates for their patients to hel...
Improved outcomes and the availability of clinical trials of hematopoietic cell transplantation (... more Improved outcomes and the availability of clinical trials of hematopoietic cell transplantation (HCT) from alternate donors and genetically modified autologous hematopoietic progenitor cells have expanded the applicability of HCT for sickle cell disease (SCD). To understand the perspective of primary caregivers exploring HCT in the current milieu, we asked the research question “What motivates primary caregivers to decide to consider HCT and to seek, and to attend, an HCT consultation?”
Background: Medication nonadherence is a public health issue that contributes to poor health outc... more Background: Medication nonadherence is a public health issue that contributes to poor health outcomes and health-care costs. Factors influencing long-term medication adherence are known; however, little is known about short-course medication adherence. Objective: This study examined patient perspectives on adherence and factors that influence adherence to short-course pharmacotherapy in diarrhea-predominant irritable bowel syndrome. Method: Twenty-seven participants were interviewed to identify their perceptions of barriers and facilitators to thrice-daily, 14-day rifaximin. Results: Participants were primarily female (89%), aged 18 to 65 years. Sixty-eight percent of interviewees were identified as “low-adherers,” meaning the percentage of days with correct daily dosing of rifaximin was <80%. The final coding framework identified social/economic-related (family support and medication expense), system-related (relationship with provider and medication knowledge), condition-relate...
Background Pain is the hallmark of sickle cell disease (SCD) but burden of pain is underestimated... more Background Pain is the hallmark of sickle cell disease (SCD) but burden of pain is underestimated when measured using health care visits for vaso-occlusive crisis. In the PiSCES study adult patients reported pain on > 50% of diary days but sought care on only 3.5 % of diary days. Accurate assessment of the burden of pain and related morbidity is crucial in clinical care and research studies in SCD. Paper based pain diaries for assessing daily pain are limited by recall bias, errors, inflated retrospective reports and falsely high compliance due to backfilling of entries. Electronic pain diaries facilitate real-time data capture, are convenient, prevent backfilling, maximize compliance and facilitate data management. They have been used in children with arthritis, cancer, abdominal and musculoskeletal pain but no validated instrument is available for use in children with SCD. Objective To develop, establish the face and content validity, and usability of a novel web-based multidim...
IMPORTANCE The hallmark of sickle cell disease (SCD) is vaso-occlusive pain that may be acute and... more IMPORTANCE The hallmark of sickle cell disease (SCD) is vaso-occlusive pain that may be acute and episodic or may progress to chronic, persistent pain with unpredictable and disabling exacerbations. Patients with SCD rely on opioids almost exclusively for acute and chronic pain management. OBJECTIVE To understand how the current opioid epidemic and subsequent guidelines from the Centers for Disease Control and Prevention are associated with the management of acute and chronic pain for patients with SCD. DESIGN, SETTING, AND PARTICIPANTS Qualitative study using semistructured interview guides. Interviews 1 hour or longer were conducted over the telephone. Participants were adults (aged Ն18 years) diagnosed with SCD who experienced pain on 3 or more days per week recruited from national SCD conferences, symposiums, and 2 sickle cell clinics. Open coding analysis facilitated thematic analysis of interview transcripts. Data collection took place from May 2017 to June 2018. MAIN OUTCOMES AND MEASURES Participant perspective of any changes to their pain management associated with the 2016 guidelines from the Centers for Disease Control and Prevention. RESULTS The 15 adults interviewed had a median (range) age of 32 (21-52) years; 13 (87%) were female; and all were of African American race/ethnicity. Participants reported that recently their opioid prescriptions had become more restrictive, were more closely monitored, and were increasingly difficult to fill in pharmacies. Participants also described increased stigmatization about opioid use and that their medical care was being affected by the physician's exclusive focus on reducing pain medication use. There was an emerging interest among adult patients in the consideration of the use of alternative therapies, including marijuana, to manage pain. CONCLUSIONS AND RELEVANCE These findings suggest that from the perspective of adults living with SCD, the opioid epidemic may have negatively affected patients' care by increasing barriers to opioids. Patients reported decreased opioid dosing, increased stigmatization regarding opioid use, physician preoccupation with opioid dosage interfering with comprehensive care, and lack of access to alternative therapies.
Despite its efficacy, the uptake of HU in adults with sickle cell disease (SCD) is poor likely du... more Despite its efficacy, the uptake of HU in adults with sickle cell disease (SCD) is poor likely due to a combination of system, provider, and patient-related factors. We investigated attitudes of adult patients towards HU by conducting qualitative interviews with 95 adult SCD patients (age 18 to 67 years old, 71 were female). While 53% of all participants reported that they were currently taking HU, patients ranging in age 18-30 years (Group 1) were more likely to report current HU use as compared to those (Group 2) ranging in age 31-67 years (65% vs. 41% P = 0.01). Most Group 1 participants who reported currently taking HU indicated that the decision to start HU was made by a parent, though some made the decision themselves as a young adult. Group 1 participants expressed trust in the efficacy of HU as well as trust that their physician adequately shared risks and benefits for the medication. The Group 2 participants, who were not currently on HU, were skeptical that all the risks and benefits of HU were known, were concerned that the efficacy of HU was not proven, and that they were not receiving complete information about its potential side effects. Of Group 2 participants who reported currently being on HU, 25% were concerned about the side effects and efficacy of HU and reported continuing HU because of a lack of effective alternatives. These data suggest that there are significant differences by age in adult SCD patients' attitudes towards, utilization and understanding of the risks and benefits of HU.
Sickle cell disease (SCD) is an inherited blood disorder that primarily affects African-American ... more Sickle cell disease (SCD) is an inherited blood disorder that primarily affects African-American and other ethnic minority populations. There are three available disease-modifying therapies for sickle cell disease: hydroxyurea (HU), bone marrow transplantation (BMT), and chronic blood transfusion (CBT). Since these treatments vary in their therapeutic intent, efficacy in preventing progression of the disease, short and long-term adverse effects, costs and patient burden, the decision-making process regarding these therapies is complicated for both the patient and healthcare provider. While previous research has focused on the patient perspective of treatment-related decision making, there is a paucity of research investigating the physician perspective of treatment-related decision making. We conducted a qualitative study with physicians who were experts in the field of SCD. Interviews focused on physician perceptions of patient decisional needs as well as physicians' approach t...
Available modalities for the longitudinal capture and analysis of pain intensity in patients with... more Available modalities for the longitudinal capture and analysis of pain intensity in patients with sickle cell disease (SCD) limit our ability to study intra-and inter-individual variation in pain and the factors influencing the transition from acute to chronic pain in patients with SCD. The objectives of this study were to determine the feasibility of electronic capture of longitudinal outpatient pain intensity data and to test the applicability of novel metrics in the study of intra- and inter-individual variation in pain in patients with SCD. Twenty SCD patients aged 13-21 submitted 2045 diary days of pain intensity data over 229 days using a web-based electronic pain diary or via text message. Participants reported pain (11-point Numerical Rating Score>0) on 1559 diary days (76.2%) suggesting a significant outpatient pain burden. In addition to mean maximum daily pain (MMDP), we calculated the ninetieth percentile of pain intensity reports (p90), the proportion of pain-free da...
Vaso-occlusive pain, the hallmark of sickle cell disease (SCD), is a major contributor to morbidi... more Vaso-occlusive pain, the hallmark of sickle cell disease (SCD), is a major contributor to morbidity, poor health-related quality of life and healthcare utilization associated with this disease. There is wide variation in the burden, frequency and severity of pain experienced by patients with SCD. As compared to healthcare utilization for pain, a daily pain diary captures the breadth of the pain experience and is a superior measure of pain burden and its impact on patients. Electronic pain diaries based on real time data capture methods overcome methodological barriers and limitations of paper pain diaries but their psychometric properties have not been formally established in patients with SCD. To develop and establish the content validity of a web-based multi-dimensional pain diary for adolescents and young adults with SCD and conduct an end-user review to refine the prototype. Following identification of items, a conceptual model was developed. Interviews with adolescents and young adults with SCD were conducted. Subsequently, end-user review with use of the electronic pain diary prototype was conducted. Two iterative cycles of in-depth cognitive interviews in adolescents and young adults with SCD informed the design and guided the addition, removal and modification of items in the multi-dimensional pain diary. Potential end-users provided positive feedback on the design and prototype of the electronic diary. A multi-dimensional web-based electronic pain diary for adolescents and young adults with SCD has been developed and content validity and initial end-user reviews have been completed.
Study Objectives: Experimental evidence links poor sleep with susceptibility to infectious illnes... more Study Objectives: Experimental evidence links poor sleep with susceptibility to infectious illness; however, it remains to be determined if naturally occurring sleep is associated with immune responses known to play a role in protection against infection. The aim of this study was to determine whether sleep duration, sleep efficiency, and sleep quality, assessed in the natural environment, predict magnitude of antibody responses to a novel antigen among community volunteers in midlife. Design: Observational. Measurements and Results: Healthy midlife adults (n = 125; 70 female; age 40-60 yr) received the standard 3-dose hepatitis B vaccination series. Actigraphy and electronic sleep diaries were used to assess sleep duration, sleep efficiency, and subjective sleep quality. Viral-specific antibody titers were obtained prior to the 2nd and 3rd vaccination to assess primary and secondary antibody responses. Clinical protection status (anti-hepatitis B surface antigen immunoglobulin G ≥ 10 mIU/ml) was assessed 6 mo after the final immunization. Regression analyses revealed that shorter actigraphy-based sleep duration was associated with a lower secondary antibody response independent of age, sex, body mass index, and response to the initial immunization. Shorter sleep duration, measured by actigraphy and sleep diary, also predicted a decreased likelihood of being clinically protected from hepatitis B at the conclusion of the vaccination series. Neither sleep efficiency nor subjective sleep quality were significant predictors of antibody response. Conclusions: Short sleep duration in the natural environment may negatively affect in vivo antibody responses to novel antigens, providing a possible explanation for observed associations of poor sleep with increased susceptibility to infectious disease.
Background There is a limited understanding of the patient and family experience of Chronic Trans... more Background There is a limited understanding of the patient and family experience of Chronic Transfusion Therapy (CTT) for prevention of complications of Sickle Cell Disease (SCD). We sought to understand patient and family experience with CTT using qualitative methods. Methods Fifteen parents of children < 18 years old and nine children 12–18 years old with SCD who were receiving CTT for > 1 year were interviewed using a semi-structured interview format, and interviews were analyzed using open coding methods. Results Four themes created a narrative of the patient and family experience of CTT: 1) Burden of CTT, 2) Coping with CTT, 3) Perceived benefits and risks of CTT, and 4) Decision making regarding CTT. Participants reported substantial burden of CTT, including the impact of CTT on daily life and family, distress about venous access, burden of chelation therapy, and anxiety about CTT complications. Participants described how they coped with CTT. Participants reported increa...
Caregivers who work in community living arrangements or intermediate care facilities are responsi... more Caregivers who work in community living arrangements or intermediate care facilities are responsible for the oral hygiene of individuals with intellectual and developmental disabilities. Oral hygiene training programs do not exist in many organizations, despite concerns about the oral care of this population. The purpose of this study was to determine the effectiveness of a caregiver educational program. This study used a quasi-experimental one-group pretest/posttest design with repeated measures to describe the outcomes of an educational program. Program participants demonstrated oral hygiene skills on each other while being scored by a trained observer, after which they completed an oral hygiene compliance survey. After three months, a follow-up included the same posttest, demonstration of oral hygiene skills, and repeat of the compliance survey. Paired-sample t-tests of oral hygiene knowledge showed a statistically significant improvement from pretest to posttest and from pretest...
Acute, intermittent vaso-occlusive pain is the hallmark of sickle cell disease (SCD) and is assoc... more Acute, intermittent vaso-occlusive pain is the hallmark of sickle cell disease (SCD) and is associated with substantial morbidity and impaired quality of life (QOL). The subgroup of adults with SCD who transition from recurrent, acute pain to chronic, persistent pain have even greater QOL impairment and higher rates of healthcare utilization. Self-management is central to SCD management; however, its role in chronic pain management is not established. This qualitative study was conducted to answer the following research questions: (1) What is the chronic pain experience of adults with SCD? (2) What self-management strategies do adults with SCD use for chronic pain? and (3) Do adults with SCD have any needs in the self-management of chronic pain? Eighteen Black adults with SCD completed a demographics questionnaire and an interview. The majority of the participants were 21-30 years of age (mean 33.5, SD 7.6), female (61.1%), employed at least part-time (61.1%), single/never married (72.2%), and had a SCD type of sickle cell anemia (55.5%). Interview analysis revealed three major themes: (1) the chronic pain experience; (2) strategies for managing chronic pain; and (3) challenges and needs in managing chronic pain. Study findings can be used to support chronic pain management among adults with SCD. Further research is needed to devise and implement effective strategies for the prevention and management of chronic SCD pain.
Sickle cell disease (SCD) is one of the most commonly inherited hemoglobin disorders that has a s... more Sickle cell disease (SCD) is one of the most commonly inherited hemoglobin disorders that has a significant impact on quality of life, increased childhood morbidity, and premature mortality. Currently, hematopoietic stem cell transplant (HSCT) is the only treatment with a curative intent. The objective of this study was to determine patients' and caregivers' knowledge of HSCT, the factors influencing the decision to pursue HSCT, their experiences, and the impact of a successful HSCT on their daily living. At Children's Healthcare of Atlanta, we conducted a qualitative study using a semistructured interview guide of patient-caregiver dyads and 2 focus-group sessions of adult long-term survivors of HSCT to elicit key factors in decision making, their experiences with HSCT, and the impact of HSCT. Interviews and focus-group sessions were recorded and transcribed verbatim. Transcripts were coded and analyzed for emerging themes using NVivo 10.0. We enrolled 11 patient-caregiver dyads (n = 6, female patients; n = 10, mothers) in the qualitative interviews and 2 focus groups with 5 (n = 2, females) and 7 (n = 3, females) participants in each group, respectively. Our analysis revealed 3 prominent themes: (1) factors and concerns influencing HSCT decision making; (2) HSCT experiences; and (3) impact of HSCT on daily life. Participants reported that progression of disease-related complications and availability of a matched donor strongly influenced the decision to pursue HSCT. Although patients and caregivers had to deal with the arduous process of HSCT and transplant-related morbidities, participants were satisfied with their decision and expressed no decisional regrets. Decision making for HSCT for patients with SCD is a complex process. Understanding the key influential factors in decision making and the impact HSCT has on these patients and their families will generate crucial insights that can guide the care of future patients and research studies.
BACKGROUND Hydroxyurea, chronic blood transfusion, and bone marrow transplantation are efficaciou... more BACKGROUND Hydroxyurea, chronic blood transfusion, and bone marrow transplantation are efficacious disease-modifying therapies for sickle cell disease (SCD) but are associated with a significant decisional dilemma because of the inherent risk-benefit tradeoffs and the lack of comparative studies. A web-based patient decision aid (PtDA) has the potential to provide patients with high-quality information about their treatment options and associated risks and benefits, help them clarify their values, and allow them to share in the process of informed medical decision making. OBJECTIVE The objective of this study was to develop a literacy-sensitive, web-based, PtDA using the conceptual framework of the Ottawa decision support framework, and to estimate in a randomized clinical trial the effectiveness of the PtDA in improving patient knowledge, and involvement in decision-making. METHODS We conducted population decisional needs assessment in a nationwide sample of patients, caregivers, s...
Background Hematopoietic stem cell transplantation (HSCT) is a treatment option with curative int... more Background Hematopoietic stem cell transplantation (HSCT) is a treatment option with curative intent for patients with transfusion dependent thalassemia (TDT) but its application is limited by the lack of suitable donors and acceptability due to the related morbidity/mortality. Transplantation of autologous genetically modified hematopoietic cells, gene therapy (GT) is emerging as a promising treatment option for TDT as it eliminates graft versus host disease (GVHD) and need for immunosuppression. Early results of GT suggest that many, but not all patients achieve transfusion independence after the procedure. There is little information about the acceptability of GT in patients with TDT. We sought to examine patient/family knowledge about GT in TDT and to examine factors that influence decision-making about this therapy. Methods Parents of children with TDT and adults with TDT were who provided informed consent underwent semi-structured interviews to understand patient/family knowle...
Background Individuals living with sickle cell disease (SCD) may benefit from a variety of diseas... more Background Individuals living with sickle cell disease (SCD) may benefit from a variety of disease-modifying therapies, including hydroxyurea, voxelotor, crizanlizumab, L-glutamine, and chronic blood transfusions. However, allogeneic hematopoietic stem cell transplantation (HCT) remains the only nonexperimental treatment with curative intent. As HCT outcomes can be influenced by the complex interaction of several risk factors, HCT can be a difficult decision for health care providers to make for their patients with SCD. Objective The aim of this study is to determine the acceptability and usability of a prototype decision support tool for health care providers in decision-making about HCT for SCD, together with patients and their families. Methods On the basis of published transplant registry data, we developed the Sickle Options Decision Support Tool for Children, which provides health care providers with personalized transplant survival and risk estimates for their patients to hel...
Improved outcomes and the availability of clinical trials of hematopoietic cell transplantation (... more Improved outcomes and the availability of clinical trials of hematopoietic cell transplantation (HCT) from alternate donors and genetically modified autologous hematopoietic progenitor cells have expanded the applicability of HCT for sickle cell disease (SCD). To understand the perspective of primary caregivers exploring HCT in the current milieu, we asked the research question “What motivates primary caregivers to decide to consider HCT and to seek, and to attend, an HCT consultation?”
Background: Medication nonadherence is a public health issue that contributes to poor health outc... more Background: Medication nonadherence is a public health issue that contributes to poor health outcomes and health-care costs. Factors influencing long-term medication adherence are known; however, little is known about short-course medication adherence. Objective: This study examined patient perspectives on adherence and factors that influence adherence to short-course pharmacotherapy in diarrhea-predominant irritable bowel syndrome. Method: Twenty-seven participants were interviewed to identify their perceptions of barriers and facilitators to thrice-daily, 14-day rifaximin. Results: Participants were primarily female (89%), aged 18 to 65 years. Sixty-eight percent of interviewees were identified as “low-adherers,” meaning the percentage of days with correct daily dosing of rifaximin was <80%. The final coding framework identified social/economic-related (family support and medication expense), system-related (relationship with provider and medication knowledge), condition-relate...
Background Pain is the hallmark of sickle cell disease (SCD) but burden of pain is underestimated... more Background Pain is the hallmark of sickle cell disease (SCD) but burden of pain is underestimated when measured using health care visits for vaso-occlusive crisis. In the PiSCES study adult patients reported pain on > 50% of diary days but sought care on only 3.5 % of diary days. Accurate assessment of the burden of pain and related morbidity is crucial in clinical care and research studies in SCD. Paper based pain diaries for assessing daily pain are limited by recall bias, errors, inflated retrospective reports and falsely high compliance due to backfilling of entries. Electronic pain diaries facilitate real-time data capture, are convenient, prevent backfilling, maximize compliance and facilitate data management. They have been used in children with arthritis, cancer, abdominal and musculoskeletal pain but no validated instrument is available for use in children with SCD. Objective To develop, establish the face and content validity, and usability of a novel web-based multidim...
IMPORTANCE The hallmark of sickle cell disease (SCD) is vaso-occlusive pain that may be acute and... more IMPORTANCE The hallmark of sickle cell disease (SCD) is vaso-occlusive pain that may be acute and episodic or may progress to chronic, persistent pain with unpredictable and disabling exacerbations. Patients with SCD rely on opioids almost exclusively for acute and chronic pain management. OBJECTIVE To understand how the current opioid epidemic and subsequent guidelines from the Centers for Disease Control and Prevention are associated with the management of acute and chronic pain for patients with SCD. DESIGN, SETTING, AND PARTICIPANTS Qualitative study using semistructured interview guides. Interviews 1 hour or longer were conducted over the telephone. Participants were adults (aged Ն18 years) diagnosed with SCD who experienced pain on 3 or more days per week recruited from national SCD conferences, symposiums, and 2 sickle cell clinics. Open coding analysis facilitated thematic analysis of interview transcripts. Data collection took place from May 2017 to June 2018. MAIN OUTCOMES AND MEASURES Participant perspective of any changes to their pain management associated with the 2016 guidelines from the Centers for Disease Control and Prevention. RESULTS The 15 adults interviewed had a median (range) age of 32 (21-52) years; 13 (87%) were female; and all were of African American race/ethnicity. Participants reported that recently their opioid prescriptions had become more restrictive, were more closely monitored, and were increasingly difficult to fill in pharmacies. Participants also described increased stigmatization about opioid use and that their medical care was being affected by the physician's exclusive focus on reducing pain medication use. There was an emerging interest among adult patients in the consideration of the use of alternative therapies, including marijuana, to manage pain. CONCLUSIONS AND RELEVANCE These findings suggest that from the perspective of adults living with SCD, the opioid epidemic may have negatively affected patients' care by increasing barriers to opioids. Patients reported decreased opioid dosing, increased stigmatization regarding opioid use, physician preoccupation with opioid dosage interfering with comprehensive care, and lack of access to alternative therapies.
Despite its efficacy, the uptake of HU in adults with sickle cell disease (SCD) is poor likely du... more Despite its efficacy, the uptake of HU in adults with sickle cell disease (SCD) is poor likely due to a combination of system, provider, and patient-related factors. We investigated attitudes of adult patients towards HU by conducting qualitative interviews with 95 adult SCD patients (age 18 to 67 years old, 71 were female). While 53% of all participants reported that they were currently taking HU, patients ranging in age 18-30 years (Group 1) were more likely to report current HU use as compared to those (Group 2) ranging in age 31-67 years (65% vs. 41% P = 0.01). Most Group 1 participants who reported currently taking HU indicated that the decision to start HU was made by a parent, though some made the decision themselves as a young adult. Group 1 participants expressed trust in the efficacy of HU as well as trust that their physician adequately shared risks and benefits for the medication. The Group 2 participants, who were not currently on HU, were skeptical that all the risks and benefits of HU were known, were concerned that the efficacy of HU was not proven, and that they were not receiving complete information about its potential side effects. Of Group 2 participants who reported currently being on HU, 25% were concerned about the side effects and efficacy of HU and reported continuing HU because of a lack of effective alternatives. These data suggest that there are significant differences by age in adult SCD patients' attitudes towards, utilization and understanding of the risks and benefits of HU.
Sickle cell disease (SCD) is an inherited blood disorder that primarily affects African-American ... more Sickle cell disease (SCD) is an inherited blood disorder that primarily affects African-American and other ethnic minority populations. There are three available disease-modifying therapies for sickle cell disease: hydroxyurea (HU), bone marrow transplantation (BMT), and chronic blood transfusion (CBT). Since these treatments vary in their therapeutic intent, efficacy in preventing progression of the disease, short and long-term adverse effects, costs and patient burden, the decision-making process regarding these therapies is complicated for both the patient and healthcare provider. While previous research has focused on the patient perspective of treatment-related decision making, there is a paucity of research investigating the physician perspective of treatment-related decision making. We conducted a qualitative study with physicians who were experts in the field of SCD. Interviews focused on physician perceptions of patient decisional needs as well as physicians' approach t...
Available modalities for the longitudinal capture and analysis of pain intensity in patients with... more Available modalities for the longitudinal capture and analysis of pain intensity in patients with sickle cell disease (SCD) limit our ability to study intra-and inter-individual variation in pain and the factors influencing the transition from acute to chronic pain in patients with SCD. The objectives of this study were to determine the feasibility of electronic capture of longitudinal outpatient pain intensity data and to test the applicability of novel metrics in the study of intra- and inter-individual variation in pain in patients with SCD. Twenty SCD patients aged 13-21 submitted 2045 diary days of pain intensity data over 229 days using a web-based electronic pain diary or via text message. Participants reported pain (11-point Numerical Rating Score>0) on 1559 diary days (76.2%) suggesting a significant outpatient pain burden. In addition to mean maximum daily pain (MMDP), we calculated the ninetieth percentile of pain intensity reports (p90), the proportion of pain-free da...
Vaso-occlusive pain, the hallmark of sickle cell disease (SCD), is a major contributor to morbidi... more Vaso-occlusive pain, the hallmark of sickle cell disease (SCD), is a major contributor to morbidity, poor health-related quality of life and healthcare utilization associated with this disease. There is wide variation in the burden, frequency and severity of pain experienced by patients with SCD. As compared to healthcare utilization for pain, a daily pain diary captures the breadth of the pain experience and is a superior measure of pain burden and its impact on patients. Electronic pain diaries based on real time data capture methods overcome methodological barriers and limitations of paper pain diaries but their psychometric properties have not been formally established in patients with SCD. To develop and establish the content validity of a web-based multi-dimensional pain diary for adolescents and young adults with SCD and conduct an end-user review to refine the prototype. Following identification of items, a conceptual model was developed. Interviews with adolescents and young adults with SCD were conducted. Subsequently, end-user review with use of the electronic pain diary prototype was conducted. Two iterative cycles of in-depth cognitive interviews in adolescents and young adults with SCD informed the design and guided the addition, removal and modification of items in the multi-dimensional pain diary. Potential end-users provided positive feedback on the design and prototype of the electronic diary. A multi-dimensional web-based electronic pain diary for adolescents and young adults with SCD has been developed and content validity and initial end-user reviews have been completed.
Study Objectives: Experimental evidence links poor sleep with susceptibility to infectious illnes... more Study Objectives: Experimental evidence links poor sleep with susceptibility to infectious illness; however, it remains to be determined if naturally occurring sleep is associated with immune responses known to play a role in protection against infection. The aim of this study was to determine whether sleep duration, sleep efficiency, and sleep quality, assessed in the natural environment, predict magnitude of antibody responses to a novel antigen among community volunteers in midlife. Design: Observational. Measurements and Results: Healthy midlife adults (n = 125; 70 female; age 40-60 yr) received the standard 3-dose hepatitis B vaccination series. Actigraphy and electronic sleep diaries were used to assess sleep duration, sleep efficiency, and subjective sleep quality. Viral-specific antibody titers were obtained prior to the 2nd and 3rd vaccination to assess primary and secondary antibody responses. Clinical protection status (anti-hepatitis B surface antigen immunoglobulin G ≥ 10 mIU/ml) was assessed 6 mo after the final immunization. Regression analyses revealed that shorter actigraphy-based sleep duration was associated with a lower secondary antibody response independent of age, sex, body mass index, and response to the initial immunization. Shorter sleep duration, measured by actigraphy and sleep diary, also predicted a decreased likelihood of being clinically protected from hepatitis B at the conclusion of the vaccination series. Neither sleep efficiency nor subjective sleep quality were significant predictors of antibody response. Conclusions: Short sleep duration in the natural environment may negatively affect in vivo antibody responses to novel antigens, providing a possible explanation for observed associations of poor sleep with increased susceptibility to infectious disease.
Background There is a limited understanding of the patient and family experience of Chronic Trans... more Background There is a limited understanding of the patient and family experience of Chronic Transfusion Therapy (CTT) for prevention of complications of Sickle Cell Disease (SCD). We sought to understand patient and family experience with CTT using qualitative methods. Methods Fifteen parents of children < 18 years old and nine children 12–18 years old with SCD who were receiving CTT for > 1 year were interviewed using a semi-structured interview format, and interviews were analyzed using open coding methods. Results Four themes created a narrative of the patient and family experience of CTT: 1) Burden of CTT, 2) Coping with CTT, 3) Perceived benefits and risks of CTT, and 4) Decision making regarding CTT. Participants reported substantial burden of CTT, including the impact of CTT on daily life and family, distress about venous access, burden of chelation therapy, and anxiety about CTT complications. Participants described how they coped with CTT. Participants reported increa...
Caregivers who work in community living arrangements or intermediate care facilities are responsi... more Caregivers who work in community living arrangements or intermediate care facilities are responsible for the oral hygiene of individuals with intellectual and developmental disabilities. Oral hygiene training programs do not exist in many organizations, despite concerns about the oral care of this population. The purpose of this study was to determine the effectiveness of a caregiver educational program. This study used a quasi-experimental one-group pretest/posttest design with repeated measures to describe the outcomes of an educational program. Program participants demonstrated oral hygiene skills on each other while being scored by a trained observer, after which they completed an oral hygiene compliance survey. After three months, a follow-up included the same posttest, demonstration of oral hygiene skills, and repeat of the compliance survey. Paired-sample t-tests of oral hygiene knowledge showed a statistically significant improvement from pretest to posttest and from pretest...
Acute, intermittent vaso-occlusive pain is the hallmark of sickle cell disease (SCD) and is assoc... more Acute, intermittent vaso-occlusive pain is the hallmark of sickle cell disease (SCD) and is associated with substantial morbidity and impaired quality of life (QOL). The subgroup of adults with SCD who transition from recurrent, acute pain to chronic, persistent pain have even greater QOL impairment and higher rates of healthcare utilization. Self-management is central to SCD management; however, its role in chronic pain management is not established. This qualitative study was conducted to answer the following research questions: (1) What is the chronic pain experience of adults with SCD? (2) What self-management strategies do adults with SCD use for chronic pain? and (3) Do adults with SCD have any needs in the self-management of chronic pain? Eighteen Black adults with SCD completed a demographics questionnaire and an interview. The majority of the participants were 21-30 years of age (mean 33.5, SD 7.6), female (61.1%), employed at least part-time (61.1%), single/never married (72.2%), and had a SCD type of sickle cell anemia (55.5%). Interview analysis revealed three major themes: (1) the chronic pain experience; (2) strategies for managing chronic pain; and (3) challenges and needs in managing chronic pain. Study findings can be used to support chronic pain management among adults with SCD. Further research is needed to devise and implement effective strategies for the prevention and management of chronic SCD pain.
Sickle cell disease (SCD) is one of the most commonly inherited hemoglobin disorders that has a s... more Sickle cell disease (SCD) is one of the most commonly inherited hemoglobin disorders that has a significant impact on quality of life, increased childhood morbidity, and premature mortality. Currently, hematopoietic stem cell transplant (HSCT) is the only treatment with a curative intent. The objective of this study was to determine patients' and caregivers' knowledge of HSCT, the factors influencing the decision to pursue HSCT, their experiences, and the impact of a successful HSCT on their daily living. At Children's Healthcare of Atlanta, we conducted a qualitative study using a semistructured interview guide of patient-caregiver dyads and 2 focus-group sessions of adult long-term survivors of HSCT to elicit key factors in decision making, their experiences with HSCT, and the impact of HSCT. Interviews and focus-group sessions were recorded and transcribed verbatim. Transcripts were coded and analyzed for emerging themes using NVivo 10.0. We enrolled 11 patient-caregiver dyads (n = 6, female patients; n = 10, mothers) in the qualitative interviews and 2 focus groups with 5 (n = 2, females) and 7 (n = 3, females) participants in each group, respectively. Our analysis revealed 3 prominent themes: (1) factors and concerns influencing HSCT decision making; (2) HSCT experiences; and (3) impact of HSCT on daily life. Participants reported that progression of disease-related complications and availability of a matched donor strongly influenced the decision to pursue HSCT. Although patients and caregivers had to deal with the arduous process of HSCT and transplant-related morbidities, participants were satisfied with their decision and expressed no decisional regrets. Decision making for HSCT for patients with SCD is a complex process. Understanding the key influential factors in decision making and the impact HSCT has on these patients and their families will generate crucial insights that can guide the care of future patients and research studies.
BACKGROUND Hydroxyurea, chronic blood transfusion, and bone marrow transplantation are efficaciou... more BACKGROUND Hydroxyurea, chronic blood transfusion, and bone marrow transplantation are efficacious disease-modifying therapies for sickle cell disease (SCD) but are associated with a significant decisional dilemma because of the inherent risk-benefit tradeoffs and the lack of comparative studies. A web-based patient decision aid (PtDA) has the potential to provide patients with high-quality information about their treatment options and associated risks and benefits, help them clarify their values, and allow them to share in the process of informed medical decision making. OBJECTIVE The objective of this study was to develop a literacy-sensitive, web-based, PtDA using the conceptual framework of the Ottawa decision support framework, and to estimate in a randomized clinical trial the effectiveness of the PtDA in improving patient knowledge, and involvement in decision-making. METHODS We conducted population decisional needs assessment in a nationwide sample of patients, caregivers, s...
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Papers by Diana Ross