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Insurers’ Denials of Opioid Coverage Spurs CDC to Clarify Guidelines By Amy Norton HealthDay Reporter

TUESDAY, April 9, 2019 (HealthDay News) — People with severe pain from cancer or sickle cell anemia should not be denied coverage for opioid painkillers, a new clarification on federal guidelines states.

In the wake of the national opioid epidemic, various medical societies had encouraged doctors to rein in prescriptions for the powerful painkillers.

In 2016, the U.S. Centers for Disease Control and Prevention published guidelines that said for most patients seen by primary care doctors, opioids should be a last resort.

But there has been an unintended consequence: Some insurers have refused to pay for prescriptions for patients with cancer or sickle cell anemia, or for cancer survivors with complicated chronic pain conditions.

The new clarification was issued in a letter from the CDC to three medical societies who’d brought the insurance problem to the agency’s attention — the American Society of Hematology, the American Society of Clinical Oncology (ASCO) and the National Comprehensive Cancer Network.

The letter, released Tuesday, stresses that the guidelines weren’t intended for patients undergoing cancer treatment.

Beyond that, the letter says, the guidelines weren’t designed to “deny any patients who suffer with chronic pain” the option of opioid medications.

Dr. Deepika Darbari, who is with the hematology society, treats young patients with sickle cell anemia at Children’s National, in Washington, D.C. She said she’s come up against the insurance barrier herself — namely, plans that refused to pay for IV opioids for patients hospitalized with severe pain episodes.

And they’ve cited the CDC guidelines as the reason, Darbari said.

Sickle cell anemia is an inherited disease that causes red blood cells to be crescent-shaped, rather than disc-shaped. The cells also become “sticky” and prone to clotting. Because of poor blood circulation, patients can suffer periodic pain “crises.”

Over time, Darbari explained, sickle cell anemia can cause chronic pain by damaging organs and joints throughout the body.

Patients can first try other pain relievers, like acetaminophen (Tylenol) and ibuprofen (Motrin, Advil), along with nondrug therapies, according to Darbari.

But, she said, some need oral opioids, like Vicodin or OxyContin. And for severe pain episodes, patients may need to be hospitalized and given IV opioids.

The CDC agreed that managing sickle cell pain is complicated. The agency stated in its letter that treatment decisions — and reimbursement — should be based on medical guidelines created specifically for the disease.

Methodist sickle cell clinic in Memphis TN renamed in honor of Mike Conley

The clinic in Methodist Healthcare’s sickle cell research center has a new name, and it has a big Memphis name attached to it.

The Mike and Mary Conley Comprehensive Sickle Cell Clinic has been renamed in honor of the Grizzlies point guard. This announcement came on the heels of Conley donating $500,000 to the hospital for sickle cell research and treatment. Conley’s total donations to Methodist now reach $1 million.

Conley said he has been inspired to raise awareness and funds for sickle cell treatment since he got to the NBA as a 20-year-old.“[The donation] is just a start,” Conley said. “I hope to influence athletes and others to make a difference.”

He said two of his cousins are diagnosed with sickle cell, and it’s hard for him to be able to play basketball for a living, while knowing his cousins and people with sickle cell cannot.

He also said awareness is not where it needs to be for this disease because sickle cell primarily impacts the black community, unlike other diseases.

“It doesn’t get the awareness that it rightfully deserves,” Conley said. “So it’s perfect for me to jump on board like so many others have.”

Conley said it’s very meaningful to have his name on the entryway to the clinic for a disease he is so passionate about.

“That’s unbelievable,” he said. “I never envisioned growing up being able to have the opportunity to leave a legacy, and this is part of it. This is the beginning.”

Scholarships Available for Individuals Living With SCD  Apply by July 1
The International Association of Sickle Cell Nurses and Professional Associates (IASCNAPA) has established a competitive college scholarship program to assist students with sickle cell disease (SCD) who will be attending an institution of higher learning in the United States. IASCNAPA plans to distribute four $1,000 scholarships this year. Applicants must have a form of SCD and be enrolled in, or have been accepted by, a recognized and accredited post-secondary school, including college, university, trade school, or other institution of higher learning. Applications are currently being accepted through July 1. IASCNAPA has two scholarship funds: The Steven Christy Scholarship Fund and the Christine A. Johnson Scholarship Fund.  Steven Christy was a Caucasian male who lived with sickle cell disease and struggled with disease stereotypes throughout his life.  He valued education and struggled to complete college, but persevered and graduated from Fitchburg State University. He spent his career helping others – first as a social worker at Favarh (a center to help people with developmental and intellectual disabilities integrate into the community), and then as a counselor and coordinator of newborn hemoglobinopathy screening programs at the combined UCONN/St. Francis sickle cell clinic. His family founded the scholarship in his honor.   Dr. Christine A Johnson earned her medical degree in 1964 from Tufts University School of Medicine in Boston, MA.  She was a provider and advocate for people with sickle cell disease for most of her adult career. She was the founder and Director of the Pediatric Sickle Cell Program at Wake Forest Baptist Medical Center, and an advocate for people with sickle cell disease for more than 30 years. She excelled in teaching and patient care. She had a tireless work ethic and was admired by all.  Her co-workers founded the scholarship in her honor.  For more information, to contribute to the scholarship fund, or to apply for a scholarship, go to

Articles in the medical literature

1.  Eur Heart J. 2019 Apr 21. pii: ehz217. doi: 10.1093/eurheartj/ehz217. [Epub ahead of print] Cardiovascular manifestations of sickle cell disease. Hammoudi N1, Lionnet F2, Redheuil A3, Montalescot G1. Abstract Sickle cell disease (SCD) is the most frequent genetic haemoglobinopathy worldwide. Early childhood mortality has dramatically decreased in high-income countries, and most patients now survive beyond the 5th decade. However, in the aging SCD population, the morbidity related to chronic organ damage, especially kidney and heart, has become a major concern. While pulmonary hypertension has attracted most attention, it appears that this condition is frequently linked to left heart failure (HF). Accordingly, SCD-associated cardiomyopathy is emerging as a major cause of reduced quality of life and early mortality in these patients. The diagnosis of this particular phenotype of high-output HF is challenging. Exercise intolerance and dyspnoea in SCD patients are linked to multiple causes including chronic anaemia. Moreover, echocardiographic features are unusual and can be misinterpreted. The classical diagnosis algorithm for HF is generally not suitable in SCD patients, and HF is poorly recognized and mostly diagnosed at a late congestive stage in routine practice. Such patients need to be identified at an earlier stage of myocardial dysfunction via improved phenotyping. This constitutes the first step towards further investigations in SCD needed to improve the prognosis and the quality of life. This article provides an updated review of the recent advances in the pathophysiology and diagnosis, and in addition, perspectives of new therapeutic approaches in SCD-related cardiac manifestations. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2019. For permissions, please email:

PMID: 31005979 

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2.  Haematologica. 2019 Apr 11. pii: haematol.2018.211391. doi: 10.3324/haematol.2018.211391. [Epub ahead of print] Mental stress causes vasoconstriction in sickle cell disease and normal controls. Shah P1, Khaleel M1, Thuptimdang W2, Sunwoo J2, Veluswamy S1, Chalacheva P2, Kato RM1, Detterich J1, Wood JC1, Zeltzer L3, Sposto R2, Khoo MCK2, Coates TD4. Abstract Vaso-occlusive crisis is a hallmark of sickle cell disease where deoxygenated sickled red blood cells occlude the microvasculature. Any stimulus like mental stress that decreases microvascular blood flow will increase likelihood of red cell entrapment resulting in local vaso-occlusion and progression to vaso-occlusive crisis. Neurally mediated vasoconstriction might be the physiologic link between crisis triggers and vaso-occlusion. In this study, we determined the effect of mental stress on microvascular blood flow and autonomic nervous system reactivity. Sickle cell patients and controls performed N-back, Stroop and pain anticipation mental stress tasks. Blood flow was measured using photo-plethysmography. Autonomic reactivity was derived from electrocardiography. Perceived stress was measured by State-Trait anxiety questionnaire. Stress tasks induced significant decrease in microvascular blood flow, parasympathetic withdrawal and sympathetic activation in all subjects. Pain anticipation caused the highest degree of vasoconstriction among all tasks. The magnitude of vasoconstriction, sympathetic activation and perceived stress was greater in Stroop than N-back indicating the relationship between magnitude of experimental stress and degree of regional vasoconstriction. Baseline anxiety had a significant effect on the vasoconstriction response in sickle cell subjects but not controls. In conclusion, mental stress causes vasoconstriction and autonomic nervous system reactivity in all subjects. Although the pattern of responses were not significantly different between two groups, the consequences of vasoconstriction can be quite significant in sickle cell disease because of the resultant entrapment of sickle cells in the microvasculature. This suggests that mental stress precipitates vaso-occlusive crisis in sickle cell disease by causing neural mediated vasoconstriction. Copyright © 2019, Ferrata Storti Foundation. Free Article 

PMID: 30975906 

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3.  World J Hepatol. 2019 Mar 27;11(3):287-293. doi: 10.4254/wjh.v11.i3.287. Extreme hyperbilirubinemia: An indicator of morbidity and mortality in sickle cell disease. Haydek JP1, Taborda C2, Shah R2, Reshamwala PA2, McLemore ML3, Rassi FE3, Chawla S4. Abstract BACKGROUND:  Sickle cell disease (SCD) is a disorder that results in increased hospitalizations and higher mortality. Advances in management have resulted in increases in life expectancy and led to increasing awareness of sickle cell hepatopathy (SCH). However, its impact in patients on the natural history and outcomes of SCD is not known. Our study aims to describe the prevalence of extreme hyperbilirubinemia (EH), one form of SCH, its effect on morbidity and mortality, and correlations between sickle cell genotype and SCH type. We hypothesize that EH is associated with higher morbidity and mortality. AIM:  To investigate the effects of EH on morbidity and mortality among patients with SCD. METHODS:  This retrospective cohort study was performed using a database of patients with SCD treated at Grady Memorial Hospital between May 2004 and January 2017. Patients with EH (defined as total bilirubin above 13.0 mg/dL) were identified. A control group was identified from the same database with patients with total serum bilirubin ≤ 5.0 mg/dL. Electronic medical records were used to extract demographic information, laboratory values, radiology results, current medications, need for transfusions and mortality data. Two samples T-test, chi-squared test and Fisher’s exact test were then used to compare the parameters between the two groups. RESULTS:  Out of the database, fifty-seven charts were found of patients with bilirubin > 13 mg/dL. Prevalence of severe SCH as defined by EH was 4.8% (57/1172). There were no demographic differences between patients with and without EH. Significant genotypic differences existed between the two groups, with hemoglobin SS SCD being much higher in the EH group (P < 0.001). Patients with severe EH had a significant elevations in alanine aminotransferase (157.0 ± 266.2 IU/L vs 19.8 ± 21.3 IU/L, P < 0.001), aspartate aminotransferase (256.5 ± 485.9 U/L vs 28.2 ± 14.7 U/L, P < 0.001) and alkaline phosphatase (218.0 ± 176.2 IU/L vs 85.9 ± 68.4 IU/L, P < 0.001). Patients with EH had significantly higher degree of end organ failure measured with quick Sequential Organ Failure Assessment scores (0.42 ± 0.68 vs 0.01 ± 0.12, P < 0.001), increased need for blood products (63% vs 5%, P < 0.001), and exchange transfusions (10.5% vs 1.3%, P = 0.022). CONCLUSION:  Among patients with SCD, elevated levels of total bilirubin are rare, but indicative of elevated morbidity, mortality, and need for blood transfusions. Large differences in sickle cell genotype also exist, but the significance of this is unknown. PMCID: PMC6447425 Free PMC Article 

PMID: 30967906 

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Conflict of interest statement Conflict-of-interest statement: This study was retrospective in nature and was not associated with any clinical intervention and thus was exempt from informed consent per our institutional policies. Conflict-of-interest statement: We have no financial relationships to disclose.
4.  Neurosci Lett. 2019 Apr 6;705:1-6. doi: 10.1016/j.neulet.2019.04.011. [Epub ahead of print] Psychosocial and affective comorbidities in sickle cell disease. Pecker LH1, Darbari DS2. Abstract Psychosocial and affective comorbidities are common in sickle cell disease (SCD) and can strongly influence disease outcomes, especially those related to pain such as frequency and intensity of pain, use of emergency- and hospital-based care and opioid use. Depression, anxiety, sleep disorders, and substance use challenges are among the common comorbidities that inform the patient experience of SCD. Underlying neurocognitive changes may also contribute to the expression of affective disorders in people with SCD. The neurobiological basis of these comorbidities in SCD is being investigated. In this mini-review, we discuss psychosocial and affective disorders that can coexist in children and adults with SCD and highlight how these common psychological pathologies may interact with complications associated with SCD. Patients with SCD should be screened for these comorbidities using standardized screening tools and managed appropriately to improve outcomes. Copyright © 2019 Elsevier B.V. All rights reserved.

PMID: 30965108 

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5.  Blood. 2019 Apr 5. pii: blood.2019000424. doi: 10.1182/blood.2019000424. [Epub ahead of print] Red blood cells modulate structure and dynamics of venous clot formation in sickle cell disease. Faes C1, Ilich A2, Sotiaux A2, Sparkenbaugh EM3, Henderson MW4, Buczek L2, Beckman JD2, Ellsworth P2, Noubouossie DF2, Bhoopat L5, Piegore M4, Renoux C6, Bergmeier W7, Park Y2, Ataga KI2, Cooley BC8, Wolberg AS2, Key NS3, Pawlinski R9. Abstract Sickle cell disease (SCD) is associated with chronic activation of coagulation and an increased risk of venous thromboembolism (VTE). Erythrocyte sickling, the primary pathologic event in SCD, results in dramatic morphological changes in RBCs due to polymerization of the abnormal hemoglobin. We used a mouse model of SCD and blood samples from sickle patients to determine if these changes affect the structure, properties and dynamics of sickle clot formation. Sickling of RBCs and a significant increase in fibrin deposition were observed in venous thrombi formed in sickle mice. During ex vivo clot contraction, the number of RBCs extruded from sickle whole blood clots was significantly reduced compared to the number released from sickle cell trait and non-sickle clots in both mice and humans. Entrapment of sickled RBCs was largely factor XIIIa-independent and entirely mediated by the platelet-free cellular fraction of sickle blood. Inhibition of phosphatidylserine, but not administration of anti-sickling compounds, increased the number of RBCs released from sickle clots. Interestingly, whole blood, but not plasma clots from SCD patients were more resistant to fibrinolysis, indicating that the cellular fraction of blood mediates resistance to t-PA. Sickle trait whole blood clots demonstrated an intermediate phenotype in response to t-PA. RBC exchange in SCD patients had a long-lasting effect on normalizing whole blood clot contraction. Furthermore, RBC exchange transiently reversed resistance of whole blood sickle clots to fibrinolysis, in part by decreasing platelet-derived PAI-1. These properties of sickle clots may explain the increased risk of VTE observed in SCD. Copyright © 2019 American Society of Hematology.

PMID: 30952675 

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6.  J Pediatr Hematol Oncol. 2019 Apr 3. doi: 10.1097/MPH.0000000000001477. [Epub ahead of print] Effect of Sickle Cell Anemia Therapies on the Natural History of Growth and Puberty Patterns. Nagalapuram V1, Kulkarni V, Leach J, Aban I, Sirigaddi K, Lebensburger JD, Iyer P. Abstract As pediatric patients with sickle cell anemia (SCA) have impaired growth and puberty patterns, we studied the effect of disease-modifying therapies on growth and puberty patterns for patients with SCA receiving hydroxyurea (HU), transfusions, or no therapy. We performed a retrospective study of children with SCA in whom anthropometric measurements and therapy type were recorded. Penalized smoothing splines were fitted to estimate growth curves and growth velocity, and linear mixed models were used to examine differences across treatment groups. Across group analyses were divided into early childhood (4.0 to 7.9 y) and peripubertal (8.0 to 12.0 y). We analyzed growth data on 157 SCA patients. From 8.0 to 12.0 years, girls on transfusion therapy were significantly taller than girls on HU (range, 5.7 to 7.2 cm; P-value range 0.002 to 0.01). From 10.0 to 12.0 years, boys on transfusion therapy were significantly taller than boys on HU (range, 4.1 to 9.4 cm; P-value range <0.0001 to 0.04). In addition, boys on transfusion therapy had an earlier peak height velocity as compared with boys on either HU or no therapy. In conclusion, children receiving transfusions tended to be taller than children on HU or no therapy. Children on HU did not demonstrate superior growth pattern when compared with children on no therapy in the peripubertal years.

PMID: 30951025 

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7.  Pediatrics. 2019 Apr 3. pii: e20182218. doi: 10.1542/peds.2018-2218. [Epub ahead of print] Improving Care for Sickle Cell Pain Crisis Using a Multidisciplinary Approach. Balsamo L1, Shabanova V1, Carbonella J1, Szondy MV2, Kalbfeld K2, Thomas DA1, Santucci K1, Grossman M1, Pashankar F3. Abstract OBJECTIVES:  Frequent hospitalizations for sickle cell disease (SCD) vaso-occlusive crises (VOCs) are associated with school absenteeism, emotional distress, and financial hardships. Our goal was to decrease hospital days for VOC admissions by 40% over a 5-year period. METHODS:  From October 2011 to September 2016, a multidisciplinary quality-improvement project was conducted with a plan-do-study-act methodology. Five key drivers were identified and 9 interventions implemented. Interventions included individualized home pain plans, emergency department and inpatient order sets, an inpatient daily schedule, psychoeducation, and a biofeedback program. High users (≥4 admissions per year) received an individualized SCD plan and assigned mental health provider. We expanded the high-use group to include at-risk patients (3 admissions per year). Data were analyzed for patients ages 0 to 21 years admitted for VOC. Hospital days were the primary measure; the 30-day readmission rate was the balancing measure. RESULTS:  A total of 216 SCD pediatric patients were managed in 2011 with a 14% increase over 5 years. A total of 122 patients were admitted for VOC. Hospital days decreased by 61% from 59.6 days per month in the preintervention period to 23.2 days per month in the postintervention period (P < .0001). Length of stay decreased from 4.78 (SD = 4.08) to 3.84 days (SD = 2.10; P = .02). Among high users, hospital days decreased from 35.4 to 15.5 days per month. The thirty-day readmission rate decreased from 33.9% to 19.4%. Overall savings in direct hospital costs per year were $555 120. CONCLUSIONS:  A dedicated team effort with simple interventions can have a significant impact on the well-being of a patient population and hospital costs. Copyright © 2019 by the American Academy of Pediatrics.

PMID: 30944154 

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Conflict of interest statement POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.
8.  Pediatr Blood Cancer. 2019 Apr 2:e27740. doi: 10.1002/pbc.27740. [Epub ahead of print] Greater number of perceived barriers to hydroxyurea associated with poorer health-related quality of life in youth with sickle cell disease. Smaldone A1,2, Manwani D3, Green NS4. Abstract BACKGROUND:  Despite medical benefits, hydroxyurea adherence in adolescents is often poor. As part of a baseline assessment of 28 youth (10-18 years) parent dyads who participated in a 6-month feasibility trial to improve hydroxyurea adherence, we measured the relationship between greater barriers to adherence and health-related quality of life (HRQL) from youth and parent perspectives. PROCEDURE:  Barriers were measured using the Adolescent and Parent Medication Barriers Scales with nine hydroxyurea items added. Barriers reported by ≥25% of the sample were considered common. Generic and disease-specific HRQL were measured by PedsQL and PedsQL Sickle Cell Disease modules. Data were analyzed using descriptive statistics, Cronbach alpha, Spearman correlation coefficients, and paired t tests. RESULTS:  Fifty-six subjects (28 dyads) participated. Youth reported greater barriers compared with parents (5.0 ± 3.9 and 3.5 ± 3.2; P = 0.03), with >80% of respondents reporting ≥1 barriers. Twelve barriers were reported by ≥25% of adolescents, whereas six were reported by ≥25% of parents. Of these, only two were common to both dyad members. Approximately one-third of youth had generic and disease-specific HRQL scores that fell at or below cutoff scores, suggesting being at risk for impaired HRQL. Greater barriers were inversely associated with poorer generic (parent r = -0.43, P = 0.03; youth r = -0.44, P < 0.001) and disease-specific HRQL (parent r = -0.53, P = 0.005; youth r = -0.53, P < 0.001). CONCLUSIONS:  Hydroxyurea barriers were frequently reported but differed by dyad members’ perspective. Greater barriers were associated with poorer generic and disease-specific HRQL. To reduce barriers to hydroxyurea in youth with sickle cell disease, perspectives of both dyad members should be addressed. © 2019 Wiley Periodicals, Inc.

PMID: 30941907 

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9.  Pediatr Blood Cancer. 2019 Apr 2:e27733. doi: 10.1002/pbc.27733. [Epub ahead of print] Health literacy and knowledge of chronic transfusion therapy in adolescents with sickle cell disease and caregivers. Yee MEM1,2, Meyer EK3, Fasano RM4, Lane PA1,2, Josephson CD1,2,4, Brega AG5. Abstract BACKGROUND:  Patients with sickle cell disease (SCD) may require chronic transfusion therapy (CTT) for prevention of stroke or other complications. Limited health literacy (HL) is common and is associated with poor health-related knowledge and outcomes in chronic disease. We sought to assess HL and transfusion knowledge in patients with SCD on CTT and their caregivers. METHODS:  A cross-sectional study of patients was conducted in outpatient hematology clinics. Forty-five pairs of adolescent patients and caregivers and 20 caregivers of pre-adolescent patients completed the Newest Vital Sign HL assessment and answered questions assessing SCD and transfusion knowledge. Community-level median income and unemployment rates were estimated from Census data. We computed the correlation of HL with knowledge and compared each to Census variables, payor status, educational attainment, and stroke. RESULTS:  HL was inadequate in 22 (34%) caregivers and 31 (69%) adolescents. Adequate caregiver HL was associated with higher educational attainment but not community-level socioeconomics or payor status. Mean knowledge score was lower in adolescents than in caregivers and correlated with age in adolescents (r = 0.42, P = .004). HL correlated with knowledge (r = 0.46, P < .0001). There were no significant correlations of HL or knowledge between adolescents and their caregivers. Neither HL nor knowledge was associated with prior stroke. The greatest knowledge was demonstrated for iron overload and SCD genotype, whereas knowledge gaps existed in alloimmunization, indication for CTT, and SCD curative therapy. CONCLUSIONS:  Enhanced educational resources in transfusion therapy, alloimmunization, and curative therapy are needed for patients with SCD and caregivers of all HL levels. © 2019 Wiley Periodicals, Inc.

PMID: 30941859 

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Sickle Cell Conferences and Events

 Sickle Cell Regional Conference: New & Emerging Therapies & Approaches to Management will be held on Saturday, May 18, 2019 in Mobile, AL. This one-day conference, presented by the University of South Alabama Comprehensive Sickle Cell Center, will be held at the USA Student Center. For more information on the conference visit: , Email or call 251-470-5893. Register online at: http//

The Foundation for Sickle Cell Disease Research will be hosting its 13th Annual Symposium June 7-9, 2019 in Fort Lauderdale, FL. Registration is now open.  

Sickle Cell Disease Association of America’s 47th National Convention will be held October 9-12, 2019 in Baltimore, MD.

5th Annual Sickle Cell Symposium: Saturday November 9th, 2019  At

The Speedway Club, 5555 Concord Parkway South Concord, NC 28027