53 research outputs found
sj-docx-1-phr-10.1177_00333549231170229 – Supplemental material for Increasing Visibility of Sickle Cell Disease in Indiana: Establishing Baseline Prevalence Using Integrated Data From Multiple Sources
No full textSupplemental material, sj-docx-1-phr-10.1177_00333549231170229 for Increasing Visibility of Sickle Cell Disease in Indiana: Establishing Baseline Prevalence Using Integrated Data From Multiple Sources by Amanda I. Okolo, Seethal A. Jacob, Brian E. Dixon, Nimish R. Valvi, Isaac A. Janson and Brandon R. Hardesty in Public Health Reports</p
sj-docx-1-ict-10.1177_15347354231218266 – Supplemental material for Mediators and Moderators of Active Music Engagement to Reduce Traumatic Stress Symptoms and Improve Well-being in Parents of Young Children With Cancer
No full textSupplemental material, sj-docx-1-ict-10.1177_15347354231218266 for Mediators and Moderators of Active Music Engagement to Reduce Traumatic Stress Symptoms and Improve Well-being in Parents of Young Children With Cancer by Sheri L. Robb, Kristin Stegenga, Susan M. Perkins, Timothy E. Stump, Karen M. Moody, Amanda K. Henley, Jessica MacLean, Seethal A. Jacob, David Delgado and Paul R. Haut in Integrative Cancer Therapies</p
Decision Making in Fertility Preservation Prior to Pursuing Curative Treatments for Sickle Cell Disease
No full textAUTHORS: Angela Collins, MPH(1), Josey Noel(1), Olivia Abraham(1), Sydney Hornberger(1), Mahvish Rahim MD, MBA, MSCR(1,2), Seethal Jacob MD, MS, FAAP(1,2), Amanda Saraf DO(1,2).
AFFILIATIONS:
(1) Indiana University School of Medicine, Indianapolis, IN.
(2) Department of Pediatrics, Riley Hospital for Children, Indianapolis, IN.
ABSTRACT:
RELEVANT BACKGROUND: Sickle cell disease (SCD) is one of the most commonly inherited hemoglobinopathies, often well controlled on Hydroxyurea (HU). Curative therapy options exist with stem cell transplant (SCT) and gene therapy. While both the underlying condition and routine therapy such as HU is thought to impact fertility, the chemotherapy used for both SCT and gene therapy can result in permanent sterility. Infertility can have a negative impact on long-term measures of quality of life. As a result, fertility preservation ought to be offered to all patients with SCD planning for curative treatment. Ovarian tissue cryopreservation and mature oocyte or embryo cryopreservation are fertility preservation options available for pre and postpubescent females respectively. Testicular tissue cryopreservation (TTC) is an experimental option for prepubescent males and sperm cryopreservation is utilized for postpubescent males.
CASE DESCRIPTION: We present three cases of patients with SCD who pursued fertility preservation prior to receiving curative therapy with a myeloablative preparative regimen. Patient 1 is a prepubescent 8-year-old male with SCD controlled with HU who opted for TTC as fertility preservation prior to receiving a matched sibling SCT. Patient 2 is a 13-year-old male with SCD controlled with HU who opted for TTC following a failed sperm banking attempt prior to haploidentical SCT. Patient 3 is an 18-year-old female with SCD controlled with HU and Voxelator who opted to have eggs harvested prior to gene therapy.
CLINICAL SIGNIFICANCE: As highlighted by these cases, continued research on safe and effective fertility preservation as well as counseling about both the impact of the underlying disease on fertility and treatment-related fertility risks is imperative to improve long-term quality of life measures.
CONCLUSION: These patients demonstrate a need for further emphasis on fertility risk counseling in this patient population and ensuring that discussions regarding preservation options is standard of practice at every institution
Decision Making in Fertility Preservation Prior to Pursuing Curative Treatments for Sickle Cell Disease
No full textAUTHORS: Angela Collins, MPH(1), Josey Noel(1), Olivia Abraham(1), Sydney Hornberger(1), Mahvish Rahim MD, MBA, MSCR(1,2), Seethal Jacob MD, MS, FAAP(1,2), Amanda Saraf DO(1,2).
AFFILIATIONS:
(1) Indiana University School of Medicine, Indianapolis, IN.
(2) Department of Pediatrics, Riley Hospital for Children, Indianapolis, IN.
ABSTRACT:
RELEVANT BACKGROUND: Sickle cell disease (SCD) is one of the most commonly inherited hemoglobinopathies, often well controlled on Hydroxyurea (HU). Curative therapy options exist with stem cell transplant (SCT) and gene therapy. While both the underlying condition and routine therapy such as HU is thought to impact fertility, the chemotherapy used for both SCT and gene therapy can result in permanent sterility. Infertility can have a negative impact on long-term measures of quality of life. As a result, fertility preservation ought to be offered to all patients with SCD planning for curative treatment. Ovarian tissue cryopreservation and mature oocyte or embryo cryopreservation are fertility preservation options available for pre and postpubescent females respectively. Testicular tissue cryopreservation (TTC) is an experimental option for prepubescent males and sperm cryopreservation is utilized for postpubescent males.
CASE DESCRIPTION: We present three cases of patients with SCD who pursued fertility preservation prior to receiving curative therapy with a myeloablative preparative regimen. Patient 1 is a prepubescent 8-year-old male with SCD controlled with HU who opted for TTC as fertility preservation prior to receiving a matched sibling SCT. Patient 2 is a 13-year-old male with SCD controlled with HU who opted for TTC following a failed sperm banking attempt prior to haploidentical SCT. Patient 3 is an 18-year-old female with SCD controlled with HU and Voxelator who opted to have eggs harvested prior to gene therapy.
CLINICAL SIGNIFICANCE: As highlighted by these cases, continued research on safe and effective fertility preservation as well as counseling about both the impact of the underlying disease on fertility and treatment-related fertility risks is imperative to improve long-term quality of life measures.
CONCLUSION: These patients demonstrate a need for further emphasis on fertility risk counseling in this patient population and ensuring that discussions regarding preservation options is standard of practice at every institution
No improvement in clinical trial enrollment for adolescents and young adults with cancer at a children's hospital
No full text
A feasibility study of telemedicine for paediatric sickle cell patients living in a rural medically underserved area
No full textIntroduction: Sickle cell disease (SCD) is the most common inherited haematological disease, with potentially devastating complications. Improvements in therapies have increased the life span of patients with SCD, but this is contingent on receiving timely evidence-based medical care, including regular evaluations with haematologists, disease-specific education and psychosocial care. Our objective was to evaluate the feasibility of utilizing telemedicine for the provision of subspecialty paediatric SCD care in a rural medically underserved area.
Methods: This was a cross-sectional, observational, feasibility study. All patients 0-21 years old with SCD seen at Riley Hospital for Children Comprehensive Pediatric Sickle Cell Clinic who lived within 30 miles of the spoke telemedicine facility were eligible for recruitment. The Telehealth Satisfaction Scale (TeSS) was adapted for the SCD population and administered at each visit.
Results: Ten SCD patients, ranging in age from 10 months to 18 years old, initiated telemedicine visits during this timeframe. Some 60% were lost to follow-up or did not attend >50% of scheduled visits prior to beginning telemedicine visits. Following initiation of telemedicine, all Hb SS patients were started and/or maintained on hydroxyurea. Nine out of 10 patients who participated during this timeframe had a 100% follow-up rate. All who participated rated the comfort and ease of using the telehealth system as good or excellent and would do a telemedicine visit again.
Discussion: This study provides critical information to determine the feasibility and acceptability of a telemedicine intervention to aid in SCD care. To our knowledge, this is the first study to examine the effectiveness of telemedicine to deliver comprehensive paediatric SCD care. Future research with a larger sample size is needed to confirm findings of our study, including expansion of telemedicine sites to include more urban areas
National Alliance of Sickle Cell Centers consensus recommendations on sickle cell disease health maintenance: A consensus statement
No full textIMPORTANCE: The most common inherited blood disorder in the world, sickle cell disease (SCD) is a known global health concern. While survival of children into adulthood has improved significantly over the last few decades in high-resource countries, substantial gaps in care standards and limited practice harmonization persist.
OBJECTIVE: To provide up-to-date recommendations for preventive care and treatment of all people living with SCD and its related comorbidities.
EVIDENCE REVIEW: As part of the National Alliance of Sickle Cell Centers (NASCC), 41 pediatric and adult sickle cell disease experts developed consensus standards and recommendations based on available evidence and expert consensus garnered during an in-depth review of the literature. For these recommendations, there were 3 separate consensus panels (0-2 years, 2-18 years, and \u3e18 years]) held in 2022. The initial expert panels included representation from 41 NASCC-recognized SCD centers (during the first 2 votes) and the third and final vote in 2023 included representatives from 81 NASCC-recognized centers. The primary focus was on: (1) preventative care including screening recommendations, (2) management of acute complications, and (3) treatment options.
FINDINGS: In this modified Delphi consensus statement, the experts developed consensus standards and recommendations for the clinical care of people living with SCD throughout the lifespan, as well as identified gaps in current evidence that need additional research. Standards and recommendations included routine screening and education, management of acute complications, as well as disease-modifying treatments such as hydroxyurea.
CONCLUSIONS AND RELEVANCE: People with SCD require coordinated and consistent care starting soon after birth in an SCD center and throughout their lives; SCD is a chronic condition with a highly heterogenous phenotype necessitating lifelong care through an SCD center in collaboration with clinicians in multiple areas of medicine including primary care. Future work should prioritize the management of timing of initiation and precision use of disease-modifying therapies, infection management and prevention, and discussion on transformative therapies
Maintenance of an Immunogenic Response to Pneumococcal Vaccination in Children With Sickle Cell Disease
No full textPatients with sickle cell disease (SCD) are at increased risk for invasive pneumococcal disease because of splenic dysfunction. To mitigate this risk, patients are protected with prophylactic penicillin until completion of pneumococcal vaccination series. The objective of this study was to assess the maintenance of a protective immune response to pneumococcal vaccination in children with SCD. A retrospective review was conducted between June 2019 and June 2020 of all patients with SCD patients for whom it had been 5±1 year since completion of PPSV23 vaccination series. A total of 41 patients were analyzed. The majority of children (68%) were able to maintain an adequate immune response. There was no identifiable disease characteristic associated with maintenance of an appropriate immunogenic response. This study finds that patients with SCD are able to maintain an adequate immune response at the 5±1 year time point from completion of PPSV23 vaccination series. Similarly, patients were not found to have an increased rate of invasive pneumococcal disease even if not meeting criteria for adequate pneumococcal serum titer levels. Maintenance of pneumococcal titers suggests that there may not be a need for revaccination at the 5-year time point in this patient population
Neighborhood Disadvantage and Healthcare Utilization in Pediatric Sickle Cell Patients
No full textBackground: Sickle cell disease (SCD) is a complex heritable blood disorder associated with higher acute healthcare utilization further complicated by psychosocial and socioeconomic factors. Other studies have indicated a relationship between neighborhood disadvantage and increased healthcare utilization. We hypothesize that those with greater neighborhood disadvantage will have greater acute healthcare utilization.
Methods: Data was collected retrospectively from the electronic medical record between 9/30/2021 and 7/11/2023 for patients followed at Riley Hospital for Children’s Pediatric SCD Clinic who completed a psychosocial needs assessment (PAT). Number of emergency department (ED) visits, hospitalizations, and missed clinic visits were collected from the time the PAT was completed up to 1 year after (or the end of the data collection period). Data regarding area deprivation index (ADI) and childhood opportunity index (COI) were collected utilizing patient home address. Descriptive statistics were performed on all data above, as well as t-tests and chi square tests for univariate analyses.
Results: 142 patients completed a PAT during the study period. 88% had a primary care provider documented, and 73% had public insurance. The median state and national ADI (6, 76.5) and COI (2, 2) demonstrated greater neighborhood disadvantage and less childhood opportunity. There was no statistically significant difference between ADI or COI and number of ED encounters or hospitalizations. However, patients who missed 1 or more SCD visits had a higher median state ADI than those who did not miss a visit (7.5 vs 5, p=0.002). Median state COI was also lower in this group than those who did not miss a visit (1 vs 2, p=0.001).
Conclusion: Those with higher ADI and lower COI could benefit from more directed support to improve access to preventative care visits. Further analysis accounting for comorbidities (seizures, asthma, depression) could disambiguate a relationship between ADI or COI and acute care utilization
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