Sickle cell disease in children: Update for the primary care provider

1998 ◽  
Vol 3 (6) ◽  
pp. 205-208
Author(s):  
Onyinye C. Onyekwere ◽  
Leslie Hoover ◽  
Renuka Gera ◽  
Roshni Kulkarni ◽  
Ajovi B. Scott-Emuakpor
Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4086-4086
Author(s):  
Max A. Brodsky ◽  
Brandi L. McClain ◽  
Jeannie Byrd ◽  
Dionna O. Roberts ◽  
Brittany Danielle Melvin ◽  
...  

Abstract Background: Re-admission to the hospital within 30 days is a measure for quality care and a point of emphasis for reducing health care costs in individuals with chronic disease. Potentially modifiable risk factors for 30 day re-admission in children with sickle cell disease (SCD) includes not being seen in the SCD clinic within 30-days of discharge (OR 7.7, 95% CI 2.4–24.4), 3 or more admissions in the previous 12 months (OR 7.3, 95% CI 2.8–18.9) and co-morbid asthma (OR 2.9, 95% CI 1.2–7.3) (Pediatr Blood Cancer. 2009 Apr;52(4):481-5). Limited data exists regarding potentially modifiable risk factors for 30-day re-admission in adults with SCD. The primary objective of this study was to define modifiable risk factors for 30-day re-admission in adults with SCD, leading to a prospective intervention study to decrease re-admission rates. Procedure: At a tertiary care center, we performed a retrospective analysis of the medical records, from 2010 to 2013, to determine risk factors for 30-day re-admission in adults with SCD. Initial admission was defined as the first admission associated with uncomplicated vaso-occlusive pain episode in each focus year (2011- 2013). To decrease bias associated with multiple admissions from the same individual, the first admission for vaso-occlusive pain in each year was evaluated as the index admission for each record. Cases and controls were defined as adults with SCD initially admitted for pain and subsequently re-admitted to the hospital within 30 days of the initial admission. A multi-variable logistic regression analysis was performed on seven postulated risk factors. All data was collected and double checked by a single reviewer, and at least 10% of the chart was checked by a medical student for further assurance of accuracy. Results : A total of 158 first admissions and 49 re-admissions (31%) were evaluated. The mean age of the cohort was 30.38 (IQR 13.55 years). The median time to re-admission was 10 days (IQR 19 days). Approximately 50% of the cohort was not evaluated in the outpatient setting by the hematology team within 30 days post-discharge. Upon discharge patients either were not given a follow up appointment (35%) or were given an appointment beyond 30 days of discharge (13%). Only two predictors were significantly associated with re-admission within 30 days: not having a primary care provider listed in the electronic medical record (odds ratio 0.35, 95% CI 0.146-0.858; p = 0.022) and the number of hospital admissions due to vaso-occlusive pain in the prior year (odds ratio 1.28, 95% confidence interval 1.15-1.42; p < 0.001), table and figure below. Five covariates were not significantly associated with re-admission within 30 days: age (odds ratio 0.982, 95% CI 0.94-1.02; p = 0.369), sex (odds ratio 0.715, 95% CI 0.28-1.81, p =0.481), hemoglobin phenotype (odds ratio 0.50, 95% CI 0.19-1.287; p = 0.15), median lifetime oxygen saturation (odds ratio 0.892, 95% CI 0.75-1.05; p = .186), and presence of government insurance (odds ratio 1.90, 95% CI 0.67-5.37; p =0.222). Conclusions: Not having a primary care provider listed in the electronic medical record and multiple admissions in the prior year are potentially modifiable risk factors for re-admission within 30 days in adults with SCD. In addition, discharge planning with a hematology visit scheduled within a week of discharge may also impact the 30-day re-admission rate. We recently introduced a strategy focused on improved discharge planning, ensuring a primary care provider for every adult patient with SCD and targeted therapeutic intervention for those with high admissions. Table: Multivariable analysis of risk factors for 30-day re-admission in adults with sickle cell disease over a course of 3 years. A total of 158 admissions were evaluated with 31% being re-admissions within 30 days. Sig. Odds ratio 95% C.I.for EXP(B) Lower Upper Age Upon Admission to the Hospital 0.369 0.982 0.944 1.021 Sex 0.481 0.715 0.281 1.817 Hemoglobin Phenotype 0.152 0.504 0.197 1.287 Median Lifetime Oxygen Saturation Level 0.186 0.892 0.753 1.057 Primary Care Provider 0.022 0.354 0.146 0.858 Government Insurance 0.222 1.907 0.676 5.378 Number of Hospitalizations Due to Vaso-Occlusive Pain in the Prior Year 0.000 1.278 1.148 1.422 Figure. A graph depicting the predicted probablity of a re-admission within 30 days in indivdiuals with and without hospitalization versus the number of hospitalizations in the prior years. Figure. A graph depicting the predicted probablity of a re-admission within 30 days in indivdiuals with and without hospitalization versus the number of hospitalizations in the prior years. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 2058-2058
Author(s):  
Sophie Lanzkron ◽  
Carlton Haywood ◽  
Gladys T Onojobi ◽  
John J. Strouse ◽  
Mary Catherine Beach

Abstract Abstract 2058 Background: Healthcare professionals caring for patients with sickle cell disease (SCD) anxiously await the release in late 2012 of evidence-based guidelines for primary care physicians. These guidelines are anticipated to include a number of recommendations for health care strategies designed to improve outcomes for SCD patients. As these guidelines become widely disseminated, the evaluation of patient adherence to recommended preventative care will be an essential component of efforts to monitor the quality of health and health care for the SCD population. Unfortunately, there is a dearth of baseline information regarding current levels of SCD patient adherence to recommended therapies. The objective of our study was to describe current levels of self-reported adherence to recommended therapies among a large sample of adults with SCD. Methods: We used data collected as part of a large cohort study of sickle cell patient experiences with care to describe sickle cell patient's self-reported adherence to a number of currently recommended preventative care behaviors. We also examined the association of different levels of adherence with self-reported levels of acute emergency department (ED) and inpatient hospital utilization over the prior 12 months. Results: 292 individuals completed baseline study questionnaires and had completed chart abstractions. The average age of participants was 34.5 (95% CI 33.1–36.), 97% were black or African American and 54% were female. 70% had either SS or SB0thal (sickle cell anemia-SCA), 21% were SC and 9% had Sb+thal. Of the 252 respondents who provided info on income, 50% reported an annual income less than $30k/yr. Those with SCA were significantly younger than those with other genotypes (33 yrs v 37 yrs p=0.02). 91% of patients reported seeing a sickle provider every year, while 73% reported seeing a primary care provider once a year. 45% of patients reported seeing a dentist in the prior year, 58% of all patients reported seeing an eye doctor in the last year. However among the 24% of patients with documented retinopathy, only 65% had seen an eye doctor in the prior year. 82% of patients had received both an influenza vaccine in the prior year and a pneumococcal vaccine within 5 years. Those with low income were less likely to report seeing a SCD provider in the prior 12 months than those with higher incomes (87% v 94% p=0.049). A significant majority of patients (88%) reported high levels (i.e. a self-report of often/very often) of adherence to taking medications as prescribed. 79% reported high-levels of adherence to keeping their clinic appointments. 87% reported high-levels of adherence in following their doctor's directions. In bivariate analyses examining those preventative care behaviors with a significant impact on outcomes, we found that those patients reporting high-levels of adherence to their medical appointments reported fewer ED visits (p=0.015) and fewer inpatient hospitalizations (p=0.005) over the prior 12 months than those with lower levels of adherence. High self-reported levels of compliance with doctor's instructions was associated with fewer ED visits, but not fewer inpatient visits, over the same 12-month period. There was no bivariate correlation between seeing a sickle cell provider or primary care provider annually with outcomes, though this may be due to the overall high levels of adherence to these behaviors that we observed in our sample. In ordinal logistic regression analyses controlling for age, education, and poverty levels, high self-reported levels of compliance with doctor's instructions exhibited an independent association with lower levels of ED visits (OR = 0.44, 95%CI [0.21, 0.90]). Conclusions: We report relatively high levels of self-reported adherence to some, but not all, recommended preventative care behaviors among a sample of adults with SCD. The relatively low levels of adherence to dental and eye care recommendations should be noted as areas of focus for future interventions. Relationships between providers and SCD patients are notoriously rife with conflict. The association of high levels of compliance with doctor's instructions with lower levels of acute care utilization observed here suggests a need to identify the specific factors and mechanisms within provider-SCD patient relationships that successfully lead to improved clinical outcomes. Disclosures: Lanzkron: Hemaquest: Membership on an entity's Board of Directors or advisory committees; NHLBI: Research Funding. Haywood:NHLBI: Research Funding. Strouse:NHLBI: Research Funding. Beach:NHLBI, NIMH: Research Funding; Merck: Speakers Bureau.


BMJ ◽  
2010 ◽  
Vol 341 (oct05 2) ◽  
pp. c5132-c5132 ◽  
Author(s):  
E. Dormandy ◽  
M. Gulliford ◽  
S. Bryan ◽  
T. E. Roberts ◽  
M. Calnan ◽  
...  

2015 ◽  
Vol 108 (9) ◽  
pp. 531-536 ◽  
Author(s):  
Lauren N. Whiteman ◽  
Carlton Haywood ◽  
Sophie Lanzkron ◽  
John J. Strouse ◽  
Leonard Feldman ◽  
...  

2014 ◽  
Vol 3 (1) ◽  
pp. 76-79 ◽  
Author(s):  
M S Basati

A case of difficult diagnosis of a patient with sickle cell disease and tooth pain fuelled a literature review by a primary care general dental practitioner. A literature search was conducted to review the relationship between sickle cell disease and pulpal necrosis. The results indicated statistically significant associations of sickle cell disease and tooth pain without any carious or traumatic pathology. It is important for the primary care dentist to be aware of the relationship between sickle cell disease and pulpal necrosis to prevent misdiagnosis and consequently mistreatment. Background A female patient, 49, of African-Carribean descent with sickle cell disease presented to the dental clinic with chronic pain. The tooth involved was an upper right first premolar free of caries and trauma. On a periapical radiograph the surrounding alveolar bone had large areas of ‘black banding’. The patient reported that large ‘black bands’ had also been seen on radiographs of her hip and shoulder and eventually the orthopaedic physician had come to the diagnosis of bone necrosis secondary to the patient's sickle cell disease. With this information, a provisional diagnosis of pulpal necrosis secondary to avascular necrosis was made, once all other possible diagnoses had been excluded. The premolar was extirpated and at a review appointment there was complete resolution of symptoms. The author performed a literature review because as a primary care dentist he had never come across this diagnosis before and noted that other primary care practitioners might be interested to learn about it.


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5923-5923
Author(s):  
Lisa Marie Shook ◽  
Christina Bennett Farrell ◽  
Karen A. Kalinyak ◽  
Stephen C Nelson ◽  
Brandon M. Hardesty ◽  
...  

Abstract Sickle Treatment and Outcomes Research in the Midwest (STORM) is a regional sickle cell network, funded by the Health Resources and Services Administration Treatment Demonstration Project (HRSA U1EMC27863), established to improve outcomes for individuals with sickle cell disease (SCD) living in Indiana, Illinois, Michigan, Minnesota, Ohio and Wisconsin. The STORM network is led by pediatric and adult hematologists who coordinate network activities in each state, along with a Regional Coordinating Center that organizes efforts throughout the Midwest. The goal of the STORM network is to increase the number of pediatric and adult primary care providers (PCP) who are knowledgeable about the management and treatment of SCD, and who are willing to prescribe and manage hydroxyurea therapy as a means to improve medical care for the approximately 15,000 individuals living with SCD in the Midwest. One PCP engagement strategy that has been implemented to increase provider knowledge in the region is replication of the Project ECHOTM (Extension for Community Healthcare Outcomes) telementoring model. Project ECHO was developed by the University of New Mexico to utilize low-cost, high-impact video technology to link expert inter-disciplinary specialist teams with primary care providers to improve management of chronic diseases. This guided practice telementoring model delivers complex specialty medical care to underserved areas, reduces health disparities, and increases workforce capacity. Project ECHO's methodology is based on 1) using telehealth technology to build healthcare resources where they are scarce; 2) sharing best practices to reduce variation in clinical care; 3) utilizing practice-based learning to develop specialty expertise among providers; and 4) monitoring and evaluating provider outcomes. Project ECHO has demonstrated improved healthcare outcomes in Hepatitis C and several other chronic diseases, and is now being piloted by STORM to test its feasibility and applicability for SCD by using a regional approach with CME accreditation. STORM network site physician leads in each state are recruiting multi-disciplinary primary care teams to participate as "spokes" in monthly SCD TeleECHO clinics. The "hub" led by the STORM Regional Coordinating Center, located at Cincinnati Children's Hospital Medical Center, coordinates implementation and evaluation of the telementoring clinics, delivered through monthly teaching sessions. STORM TeleECHO participants log onto an internet-based virtual meeting site, using a webcam to interact during the session. STORM TeleECHO clinics include brief didactic presentations from nationally-recognized SCD content experts with topics and curriculum based on the National Heart Lung and Blood Institute Evidence-Based Management of Sickle Cell Disease guidelines released in 2014. TeleECHO teaching clinics also include 1-2 de-identified, HIPAA protected case discussions (pediatric and adult) presented by providers who would like medical and psychosocial feedback on management of challenging clinical scenarios. Providers participating in the STORM TeleECHO complete an initial survey assessing knowledge and comfort levels, practice behaviors (including hydroxyurea prescribing practices) and clinic demographics. Satisfaction surveys are sent to participants after each session as part of the CME-credit evaluation. Follow-up surveys at 6 months and 1 year will assess satisfaction, knowledge, comfort level and changes in practice. STORM's TeleECHO was launched in March 2016. Preliminary data indicate an interest in STORM TeleECHO teaching sessions by both pediatric and adult providers across the Midwest region. Future efforts will expand the network to more PCPs in the region, while improving the applicability and utility of STORM TeleECHO in SCD through provider assessment. Disclosures Ware: Global Blood Therapeutics: Consultancy; Biomedomics: Research Funding; Bristol Myers Squibb: Research Funding; Addmedica: Research Funding; Nova Laboratories: Consultancy; Bayer Pharmaceuticals: Consultancy.


2017 ◽  
Vol 4 (3) ◽  
pp. 47
Author(s):  
Daiana Márcia Melo Cruz ◽  
Seyna Ueno Rabelo Mendes ◽  
Bruno Sousa Pires

No presente relato os autores descrevem um caso de Doença Falciforme em uma criança de 04 anos de idade destacando a importância do seu acompanhamento pela atenção básica. Essa doença decorre de uma mutação no gene que produz a hemoglobina A, originando outra, denominada hemoglobina S, de herança recessiva. O curso clínico é o de uma doença falciforme de intensidade menos grave. As crises hemolíticas são mais amenas. O baço está aumentado na criança, poedendo persistir na idade adulta. Além disso, há perda da função esplênica de forma gradual. O diagnóstico é realizado através da Eletroforese de Hemoglobina. A doença não tem cura, necessitando do acompanhamento regular do paciente pelo serviço de saúde para controle da doença e prevenção de complicações.   Palavras-chave: doença falciforme, hemoglobina, atenção básica. ABSTRACT There is no report and description of a Sickle Cell Disease case in a 4 years old child. highlighting the importance of their follow-up for primary care. This disease arises from a non-gene mutation that produces a hemoglobin A, originating another, called hemoglobin S, of recessive inheritance. The clinical course is a sickle cell disease of less severe severity. As hemolytic crises are milder. The spleen is increased in the child, and may persist in adulthood. In addition, there is gradual loss of splenic function. The diagnosis was made through Hemoglobin Electrophoresis. The disease has no cure, requiring regular monitoring by the health service to control the disease and prevent complications. Keywords: Sickle Cell Disease, hemoglobin, primary care.


2012 ◽  
Vol 3 (11) ◽  
pp. 1-5 ◽  
Author(s):  
G Al Juburi ◽  
O Okoye ◽  
A Majeed ◽  
Y Knight ◽  
SA Green ◽  
...  

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