PP013 Pain Management And Substance Abuse In Sickle Cell Disease Patients

2017 ◽  
Vol 33 (S1) ◽  
pp. 73-74
Author(s):  
Mariana Santos ◽  
Diego Travi ◽  
Camila Ribeiro ◽  
Thiago Pianca ◽  
Indara Saccilotto ◽  
...  
Keyword(s):  
Substance Abuse ◽  
Substance Use ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Psychotic Disorders ◽  
Negative Consequences ◽  
Cell Disease ◽  
Convenience Sample ◽  
Alcohol And Drugs ◽  
Self Reporting Questionnaire

INTRODUCTION:Drug abuse is a social and public health problem because of its negative consequences of emotional and physical development in individuals. There are few studies evaluating substance abuse by individuals with sickle cell disease (SCD). These patients have severe and recurrent pain crises (1), frequently needing opioids to control it (2). The compromised quality of life can predispose this population to the occurrence of non-psychotic disorders such as depression, making them vulnerable to substance abuse (3).METHODS:We evaluated the consumption of alcohol and drugs in a cohort followed at the Sickle Cell Disease Reference Center (CRAF), at Hospital de Clínicas de Porto Alegre, estimating the percentage of patients in treatment of SCD who abuse alcohol and drugs, mainly opioids. A cross-sectional study was of a convenience sample of 139 patients with SCD treated at CRAF. The pattern of substance use was evaluated using the Brazilian version of Alcohol, Smoking and Substance Involvement Screening Test (ASSIST). The exposure to opioids was measured by their use and prescription in the 24 months before the interview. The Self-Reporting Questionnaire (SRQ-20) was used to estimate the occurrence of non-psychotic disorders in this population. Descriptive analyses were performed using absolute and relative frequencies. The association between the variables was verified using the chi-square test or Fisher's exact test.RESULTS:The prevalence of abusive use was 1.5 percent for alcohol and 3.0 percent for tobacco, with no abusive use of any other substance including opioids was identified. Of note was the pattern for substance use that was not influenced by exposure to substances or the presence of non-psychotic disorders.CONCLUSIONS:Our data shows that use of opioid analgesics for the management of SCD painful crises is safe and does not induce substance abuse. Regular follow-up of these patients is recommended. The results of this study might be useful in other countries.

scholarly journals Barriers to Pediatric Sickle Cell Disease Guideline Recommendations

2019 ◽  
Vol 6 ◽  
pp. 2333794X1984702 ◽  
Author(s):  
Michael D. Cabana ◽  
Julie Kanter ◽  
Anne M. Marsh ◽  
Marsha J. Treadwell ◽  
Michael Rowland ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Support Staff ◽  
Cell Disease ◽  
National Guidelines ◽  
Convenience Sample ◽  
Different Types ◽  
Pediatric Sickle Cell Disease

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; P < .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 ( P < .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).

Characterization of Pica in Adults with Sickle Cell Disease.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3785-3785
Author(s):  
Rupa Redding-Lallinger ◽  
Norman Gray ◽  
Oludamolola Olajide ◽  
Juana Diaz ◽  
Jennifer Brown ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Age Of Onset ◽  
Structured Interview ◽  
Nutritional Deficiency ◽  
Similar Proportion ◽  
Cell Disease ◽  
Convenience Sample ◽  
Iron Deficient ◽  
Familial Environment

Abstract Background: Pica is known to be common in children with sickle cell disease but there is no information about this disorder in adults with SCD. The cause of pica in SCD is not known; a small minority of cases are due to iron deficiency. Aims: The purposes of this study are to characterize pica in adults with sickle cell disease, to compare it with pica as it occurs in children with the disorder, and to determine the prevalence of pica in the non-SCD siblings of individuals with SCD. The underlying hypothesis is that there is an unidentified nutritional deficiency which stimulates pica in individuals with SCD. The current report presents the data from adult SCD subjects. Subjects and Methods: A structured interview was designed and administered to a convenience sample from Sickle Cell Clinic at UNC. Laboratory and radiologic data were collected from the subjects’ medical records. The data used were from steady state on the date closest to the interview date. No data that were more than 5 years from the interview date were used. Results: There were 86 participants, 73 with SS or Sβ0thal, 13 with SC or Sβ+thal. The mean age was 37 years, with a range of 19 to 63. Fifty-five percent were female. Any pica. Seventy-six percent of the participants had had pica at some time during their lives; a similar proportion was found in both the SS/Sβ0thal as in the SC/Sβ+thal group. Pica was more prevalent in females, with 60% of females reporting ever having had pica vs 40 % of males. The majority of these reported having had pica as a child, with a mean age of onset of 7 years and a mean duration of 6 years. This group was significantly more likely to have a sibling with pica than those who had never had pica(52 vs 5%, p &lt;.0001). Current pica. Sixteen percent (13 individuals) reported having pica currently, 10 females and 3 males; eleven were SS or Sβ0thal, and 2 were SC or Sβ+thal. All reported having begun pica during childhood at a mean age of 6 years. They were significantly more likely to report having been diagnosed with a nutritional deficiency than those without recent pica(39 v 14%, p=.04). Males with current pica had significantly lower bmi than those without pica (20 vs 23kg/m2, p=.0002). Although none with recent pica had ferritin within the iron deficient range, those with recent pica had significantly lower mean ferritin than those without recent pica (374 vs 1256ng/mL. p&lt;.0069). No other hematologic or biochemical difference was seen between those with and without recent pica. There was also no difference in rate of osteopenia in the hip or spine, by xray, between the two groups. Seventy-five percent with current pica had a sibling with pica vs 34% without current pica(p=.007). Eighty-three percent reported having seen other family members eat non-food vs 50% of those without current pica. One third with current pica reported having been influenced by a family member to engage in pica, vs 9% of those without current pica. Conclusions: Pica is common in SCD, but in a majority of cases it is a childhood phenomenon. Persistent pica in adults with SCD is associated with a low-normal bmi in males, significantly different than the unaffected males. Ferritin levels are high overall in this population but are significantly lower in those with recent pica than those without recent pica. The familial environment appears to affect the occurrence of pica; it cannot be determined from these data whether this influence is genetic or environmental.

Low Ascorbate Levels in Transfused Patients Are Associated with Correlates of Vascular Damage in Sickle Cell Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2570-2570
Author(s):  
Susan Claster ◽  
Susan Carson ◽  
Thomas C Hofstra ◽  
Thomas Coates ◽  
John C Wood
Keyword(s):  
Nitric Oxide ◽  
Sickle Cell Disease ◽  
Endothelial Function ◽  
Sickle Cell ◽  
Transferrin Saturation ◽  
Comprehensive Treatment ◽  
Cell Disease ◽  
Free Hemoglobin ◽  
Convenience Sample ◽  
Hs Crp

Abstract Abstract 2570 Poster Board II-547 Introduction: Ascorbate is vital for endothelial homeostasis because it is responsible for regeneration of tetrahydrobiopterin (BH4) from dihydrobiopterin (BH2). BH4 is essential for proper electron transport from oxygen to arginine in the synthesis of nitric oxide (NO) by endothelial nitric oxide synthase (eNOS). When BH4 is deficient, eNOS becomes uncoupled from arginine and synthesizes superoxide, producing a vasoconstrictive rather than a vasodilatory phenotype. Low BH4/BH2 ratios are correlated with decreased flow-mediated dilation, a known marker of nitric oxide-dependent endothelial function. Dietary supplementation with ascorbate has been show to acutely improve NO-mediated vasodilation in smokers and patients with coronary artery disease. Since patients with sickle cell disease (SCD) are known to have low levels of NO and impaired vascular reactivity, we examined the correlates of ascorbate deficiency in our chronically transfused SCD patients. Methods: We recently studied micronutrient levels in convenience sample of our chronically transfused SCD patients. In that study, 56.7% of SCD patients had ascorbate levels below the lower limit of normal (0.2–1.9 mg/dl). We screened the following parameters as predictors of abnormal ascorbate: LDH, cell-free Hb, total bilirubin, liver iron concentration (LIC), pancreas iron, cardiac iron, ferritin, transferrin saturation, insulin, glucose, brain natriuretic peptide (BNP) and high sensitivity C reactive peptide( hs-CRP). A total of 28 patients (21 females, 7 males), ranging in age from 1.4 to 31.4 years old, who had low ascorbate levels at the time of micronutrient measurements were studied. All patients had chelating drugs held for 24 hours prior to being evaluated. Results: None of the above predictors demonstrated significant linear relationships with ascorbate levels. However, low or undetectable ascorbate levels were observed with extremes of a number of parameters including LIC > 30 mg/g, BNP > 40 pg/ml, LDH > 1500 U/Lcell-free hemoglobin > 30 mg/dl,hs-CRP > 7mg/L,and homocysteine > 10 μM/L. LDH, cell-free hemoglobin and hs-CRP exhibited the best specificity for prediction of ascorbate levels and were the only variables to achieve statistical significance by Fischer's Exact test (Table 1). More importantly, there was sufficient independence among these predictors that having and extreme value for one of the above parameters identified 14/15 patients with low ascorbate, with only 1/13 false positives. Discussion: These observations suggest that low ascorbate levels in SCD patients result from a combination of chronic inflammation, hemolysis, and hyperhomocysteinemia. Since ascorbate is vital for proper endothelial function, these observations are consistent with known associations of inflammation and hemolysis with sickle vasculopathy. Although hyperhomocysteinemia is a known vascular stressor in other diseases, its contribution in SCD is less clear because treatment with pyridoxine and folate, which reduce homocysteine levels, do not affect other markers of SCD vascular dysfunction. Taken together, these data suggest that comprehensive treatment of sickle vasculopathy may require multiple interventions, including decreasing hemolysis and inflammation as well as B vitamin supplementation. Ascorbate replacement alone is unlikely to be effective without correction of upstream stressors, similar to its use in other complex vascular disorders. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Psychosocial and Clinical Risk Factors Associated with Substance Use in Observational Cohort of Patients with Sickle Cell Disease

2020 ◽  
Vol 55 (13) ◽  
pp. 2205-2212 ◽  
Author(s):  
J. Deanna Wilson ◽  
Sophie Lanzkron ◽  
Lydia H. Pecker ◽  
Shawn M. Bediako ◽  
Dingfen Han ◽  
...  
Keyword(s):  
Risk Factors ◽  
Substance Use ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Risk Factors ◽  
Cell Disease ◽  
Factors Associated ◽  
Clinical Risk ◽  
Observational Cohort

The Use of Music by Adolescents and Young Adults With Sickle Cell Disease

2020 ◽  
Vol 26 (3) ◽  
pp. 189-196
Author(s):  
Jean C. Solodiuk ◽  
Brian Jantz ◽  
Mark Fuller ◽  
Dana Osterling ◽  
Hannah Foxman ◽  
...  
Keyword(s):  
Young Adults ◽  
Neuropathic Pain ◽  
Sickle Cell Disease ◽  
Music Therapy ◽  
Sickle Cell ◽  
Adolescents And Young Adults ◽  
Cell Disease ◽  
Convenience Sample ◽  
Pain Analgesics ◽  
Chronic Pain Conditions

Pain is common and often severe in people with sickle cell disease (SCD), occurring as acute intermittent pain episodes called vaso-occlusive episodes (VOEs), as well as chronic pain conditions including bone infarctions, avascular necrosis of joints, and neuropathic pain. Analgesics such as opioids, nonsteroidal anti-inflammatory medications (NSAIDS), and anticonvulsants for neuropathic pain, although often necessary to manage these types of pain, are associated with side effects. Nonpharmacologic interventions such as to listening to music and music therapy may reduce pain. The objectives of this study were to determine whether the use of music by adolescents and young adults with SCD was helpful, and if so, the types of music that helped, and how music helped them. A convenience sample of nine English-speaking inpatients ages 13 to 21 years, with SCD and at least two prior hospitalizations for VOE, took part in interviews with music therapists. Participants were asked open-ended questions about both pain and music. Participants identified that music was helpful for pain relief as well as for mood regulation, focusing attention during cognitive tasks such as homework, distraction, relaxation, and feeling understood or connected with others. Despite this, while hospitalized, participants reported that they did not tend to use music to help with pain. These findings support the use of both music medicine and music therapy as interventions for pain and distress in adolescents and young adults with SCD.

scholarly journals Awareness and Use of the Sickle Cell Disease Toolbox by Primary Care Providers in North Carolina

2021 ◽  
Vol 12 ◽  
pp. 215013272110490
Author(s):  
Stephanie O. Ibemere ◽  
Paula Tanabe ◽  
Emily Bonnabeau ◽  
Gary Rains ◽  
Kern Eason ◽  
...  
Keyword(s):  
Primary Care ◽  
North Carolina ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Primary Care Providers ◽  
Care Providers ◽  
Cell Disease ◽  
Convenience Sample ◽  
Health Maintenance ◽  
Blood Disorder

Background Sickle cell disease (SCD) is a complex chronic blood disorder characterized by severe disease complications ideally managed by both hematologists and primary care providers (PCP’s). PCP’s report knowledge gaps and discomfort with SCD management. Our team developed and a decision support tool for SCD management (SCD Toolbox) based on the National Heart, Lung, and Blood Institute’s SCD guidelines. We surveyed PCPs in North Carolina (NC) prior to formal dissemination to determine current co-management practices, assess toolbox acceptability, use, format preferences, and understand which algorithms would be most helpful. Method A 23-item baseline needs assessment survey was disseminated to PCPs throughout NC. Results A total of 63 medical providers responded to the survey and of these respondents, 64% reported caring for 1 to 10 patients with SCD. Only 39% of PCPs reported regular communication with an SCD specialist. Providers reported the highest level of awareness of the pediatric and adult health maintenance tools (41% and 39% respectively) and highest use of the pediatric (26%) and adult (28%) health maintenance tools. Respondents also expressed a desire to have access to multiple toolbox formats (37%) (website, mobile app and/or paper). Limitations The use of a convenience sample and low survey response are study limitations which hinder generalizability. Conclusions PCPs rarely co-managed with a specialist, had low awareness and use of SCD toolbox, and requested multiple formats for the toolbox.

Utility of Lung Ultrasound for Evaluating Acute Chest Syndrome in Pediatric Patients with Sickle Cell Disease

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 980-980
Author(s):  
Stephanie G. Cohen ◽  
Zayir M Malik ◽  
Robert Hagbom ◽  
Adina L Alazraki ◽  
April G. Zmitrovich ◽  
...  
Keyword(s):  
At Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Positive Likelihood Ratio ◽  
Observer Agreement ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Convenience Sample ◽  
Lung Consolidation

Abstract Background: Acute chest syndrome (ACS) is a common complication in patients with sickle cell disease (SCD) and is a leading cause of morbidity and mortality. Physical assessment alone is not sensitive for diagnosing ACS and therefore Chest x-ray (CXR) is recommended because of the difficulty of diagnosing ACS on clinical grounds alone. Children with SCD are repeatedly exposed to diagnostic radiation for the evaluation of ACS. Focused chest ultrasound (US) has been used to evaluate for lung consolidation. If lung US can identify patients with ACS this application could potentially limit radiation exposure in patients with SCD at risk for ACS. We evaluated the utility of physician performed US as compared to CXR to identify patients with SCD who have ACS. Methods: This is a prospective observational study that took place from November 2014-July 2015 in 2 urban pediatric emergency departments (EDs). The study population consisted of a convenience sample of patients with SCD from birth to 18 years of age at risk for ACS and who received a CXR for suspected ACS. Medical students and clinicians with training in lung sonography consented patients and performed a focused study to evaluate for lung consolidation. A blinded expert in point-of-care US reviewed for quality assurance and agreement. Sensitivity, specificity, and likelihood ratios were calculated for test performance characteristics of ultrasound using CXR as a reference standard. Inter-observer agreement (κ) between enrolling sonologists and reviewer was also calculated. Results: 85 patients were enrolled for a total of 98 cases. Median age was 7 years (IQR 2-13 years) and 53% of patients were male. The prevalence of ACS by CXR was 14%. Lung US was able to detect consolidation with a sensitivity of 86% (95% CI, 56-97%), specificity of 95% (95% CI, 87%-98%), positive likelihood ratio (LR) 18 (95% CI, 7-48) and negative LR 0.2 (95% CI, 0.04-0.5). The agreement between enrolling novice sonologists' interpretation and blinded reviewer's interpretation was very good with a Cohen κ of 0.86 (95% CI, 0.7-1). Conclusions: Focused lung US was able to identify ACS with high specificity. There was very good agreement between novice and expert sonologist interpretation. Lung US may decrease the need for CXR in patients at risk for ACS. Further studies are needed to see how this test performs within current clinical practice guidelines. Disclosures No relevant conflicts of interest to declare.

scholarly journals Integrated Primary Specialty Care Access for Adults with Sickle Cell Disease in Delaware

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 31-32
Author(s):  
Nina Anderson
Keyword(s):  
Substance Abuse ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Opioid Use Disorder ◽  
Specialty Care ◽  
Cell Disease ◽  
Opioid Use ◽  
Care Clinic ◽  
Primary Specialty ◽  
New Castle County

INTRODUCTION A major gap in US care for Sickle Cell Disease (SCD) is a dearth of healthcare providers with expertise in adult SCD management. In 2014 TOVA Community Health launched a community-based primary specialty care clinic in Delaware (DE). The Delaware Department of Health and Social Services found that 96 individual adults in DE with Sickle Cell Disease (SCD) utilized hospital services 99.5% of the time for sickle cell crisis (Anderson et al., 2014). The majority of those adults lived in New Castle County, DE. The dearth of sickle cell hematologist/oncologists for adults in this county may drive them to seek recurrent care through the hospital system. Recognizing this as a nationwide public health crisis, the Delaware Department of Health and Social Services (DHSS) partnered with TOVA Community Health in 2014 to establish an adult primary specialty care clinic medical home in New Castle County, DE. This report describes the progress/impact of an integrated primary specialty clinic for adults with SCD METHODS Prospective/ Retrospective tracking of best practices for SCD are featured in the NIH 2014 guidelines. The staff included a hematologist with expertise in SCD, a primary care provider, an advanced practice nurse, a Social Worker (MSW), a nurse care coordinator, and a community health worker, were funded by Sickle Cell Disease Association of America (SCDAA) and Health Resources and Services Administration (HRSA). From 2014 to 2018, integrated primary specialty care services were provided for 33 discrete patients. RESULTS This first cohort of patients was notable for health complications that may be attributed to the lack of coordinated primary specialty sickle cell care access. Therapeutic counseling, support groups and preventive health maintenance services were measured by: outpatient visits to the clinic, access to disease modifying therapies (hydroxyurea therapy or chronic transfusion therapy), immunizations (Pneumococcal), Depression Screens and Personalized Sickle Care Plans (Table). 21 % percent of the patients had no sickle cell provider and/or hematologist/oncologist other than ED or inpatient care for the 12 months preceding their first visit to our clinic. 48% of the patients received a pneumococcal vaccine (Pneumovax 23 val - n=16/33 and Prevnar 13 - n=7/33) and three (n=3/33) received both Pneumococcal vaccines. 30% percent of the patients (n=10/33 patients) seen were offered access to HU but were not on the drug and 42% (n=14) were prescribed HU. All patients did not have a Personalized Sickle Cell Care Plan prior to receiving services at the Primary Specialty Care clinic. 12% (n=4/33) patients had a diagnosis of Substance Abuse Disorder, 15% (n=5/33) had a diagnosis of Opioid Use Disorder and 3% (n=1/33) had both Substance Abuse and Opioid Use Disorder. 70% (n= 7/10) were male and 30% (n-3/10) were female with a diagnosis of Substance Abuse and/or Opioid Use Disorder. 45% (n=15/33) had a Pain Management Agreement established within the time period at the primary specialty clinic. CONCLUSION The creation of an integrated practice primary specialty care clinic for adults with SCD in New Castle County, Wilmington, DE demonstrates the successful leverage of regional networks that engages an academic institution for telehealth consultation, public health, and community-based organizations to develop an adult SCD primary specialty care model. This safety net clinic provides team based primary specialty care to adults whose only option previously was the hospital and/or emergency room. Unique challenges in managing adult SCD acute and chronic pain were addressed. Overall, this is one model for access to integrated primary specialty care and behavioral health services for persons with SCD across their lifespan. Figure 1. Disclosures No relevant conflicts of interest to declare.

Tricuspid Annular Plane Systolic Excursion as a Potential Marker of Hospital Utilization in Patients with Sickle Cell Disease

2021 ◽  
Vol 6 (2) ◽  
Author(s):  
Seyller HR ◽  
◽  
Harkins AP ◽  
Rodriguez A ◽  
Mahrat S ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pulmonary Artery Hypertension ◽  
Hospital Utilization ◽  
Cell Disease ◽  
Test Analysis ◽  
Convenience Sample ◽  
Potential Marker ◽  
Ed Visits ◽  
The Mean

Objective: Pulmonary Artery Hypertension (PAH) is an independent risk factor for increased morbidity in Sickle Cell Disease (SCD) patients. Tricuspid Annular Plane Systolic Excursion (TAPSE) can be used as a surrogate measure for PAH. The objective of this study was to determine whether lower TAPSE values are associated with increased Emergency Department (ED) and hospital utilization. Methods: TAPSE measurements were retrospectively obtained from bedside echocardiograms from a convenience sample of 28 SCD patients presenting to the ED with SCD pain. TAPSE was considered abnormal if <24.9mm, one standard deviation below the mean TAPSE for SCD patients. Medical records were reviewed to determine ED visits and hospital overnight stays over a two-year period. A t-test analysis and Pearson’s correlation were used for each variable. Results: The initial sample included 28 SCD patient encounters. TAPSE measurements were abnormal in 5 patients and normal in 23 patients. The mean number of ED visits/year for the abnormal and normal TAPSE group were 23.00 and 16.87, respectively (p=0.57) with moderate negative linearity (p=0.03). The mean number of hospitalized days for abnormal and normal TAPSE groups was 108.8 and 59.6, respectively (p=0.10) with moderate negative linearity (p=0.07). Conclusion: Lower TAPSE values (<24.9mm) in SCD patients were associated with higher ED and hospital utilization. If findings are replicated in larger studies, TAPSE may serve as a marker of morbidity in SCD patients presenting to the ED.

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