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Increasing hydroxyurea dose helps to keep young sickle cell patients out of the hospital

St. Jude Children’s Research Hospital investigators have shown that using the drug hydroxyurea to boost average fetal hemoglobin levels above 20 percent in children and teenagers with sickle cell anemia was associated with at least a two-fold reduction in hospitalization for any reason.

Results of the federally funded HUSTLE study—Hydroxyurea Study of Long-Term Effects—appeared online this week in the American Journal of Hematology.

The findings should help settle the debate about how to optimize hydroxyurea for treatment of sickle cell disease in young people. Rather than calculating a standard dose of hydroxyurea based on patients’ weight, researchers used a dose-escalation approach to determine the maximum tolerated dose for each of the 230 St. Jude patients enrolled in the study.

“Our analysis showed that using this approach, hospitalizations for the average patient fell to less than one every couple of years rather than four to six annually,” said lead author Jeremie Estepp, M.D., an assistant member of the St. Jude Department of Hematology. “This frees children from the fevers, pain and other symptoms of this disease and gives them and their families more chances to enjoy childhood and adolescence.”



University of Cincinnati College of Nursing Faculty Founds Sickle Cell Clinic in Sierra Leone

Cheedy Jaja, PhD, associate professor at the University of Cincinnati (UC) College of Nursing, is once again taking on a major health problem in his native country of Sierra Leone. Two years after establishing an orphanage for children who lost their parents to the Ebola epidemic, Jaja has opened up a clinic for patients with sickle cell disease (SCD).

During a visit to Sierra Leone in late 2014 and early 2015 while establishing the orphanage called Dream Home, Jaja was approached by Sickle Cell Carers Awareness Network (SCCAN), a patient advocacy group. SCCAN asked if Jaja could help provide care and medication for a group of approximately 2,000 people who showed signs of SCD. At the time, no facility existed in Sierra Leone to make a definitive determination on whether people had SCD.

Upon his return to UC from Sierra Leone, Jaja received approval for a plan to use his faculty start-up funds to launch a clinical research program in his native country. He then worked out an agreement with Jericho Road, a Boston-based non-governmental organization, to use a large community health center in the Kono District of Sierra Leone as a medical home for his clinical program.

Another key piece of the puzzle came from a North Carolina-based company, BioMedomics. The company developed a point of care testing device, Sickle SCAN, that determines if a patient has SCD.

“It’s a finger prick blood test, just a little drop of blood,” says Jaja. “It provides immediate results and is less expensive than other testing methods.”

The next step for Jaja was hiring a clinical team consisting of a physician’s assistant, a registered nurse and a public health worker. The team created a clinical protocol for which aspect of SCD they planned to examine.

“Each patient will come to the clinic once a month, and they will have a free comprehensive medical screening,” says Jaja. “They will be examined for different aspects of sickle cell disease and they will be provided with medication.”

Jaja hopes that in the next 12 to 24 months, the program will collect definitive data on whether or not the intervention made a difference in patient’s lives. If the research finds that the intervention had a positive impact on SCD patients, the team will use this as a blueprint to show the Sierra Leone government how care for SCD patients can be provided.  He says the clinical staff is also research staff, and everyone will be involved in data collection.

“I’m going to create a database at UC with the help of the Center for Clinical and Translational Science and Training that will monitor real-time results in Sierra Leone from here,” says Jaja. “What we’re doing in Sierra Leone is promoting the mission of the university by encouraging both faculty and students to be global citizens and to provide care to marginalized patient populations globally.”



New Resource: “Sickle Cell and Thalassaemia Screening – What Parents Think”

Parent’s Stories’ is a new report that has been launched containing experiences from parents of African, Caribbean, Middle-Eastern, Asian and Mediterranean origin who have been through the United Kingdom’s National Health Service (NHS) Sickle Cell and Thalassaemia Screening Programme and who were at increased risk of having a baby with either sickle cell or thalassaemia, two serious genetically inherited blood conditions. The report stems from a successful collaborative project between the Screening Programme and two patient representative organisations – the Sickle Cell Society (SCS) and United Kingdom Thalassaemia Society (UKTS). The project aimed to determine any barriers affecting the timeliness of the offer of screening and prenatal diagnosis (PND) to couples and recommend ways of improving screening services.  As part of the project,  ‘at –risk’ couples who each carried a gene for sickle cell or thalassaemia who had been through antenatal screening within the last five years were interviewed and their shared stories made up the rich evidence contained in the ‘Parent Stories’.

Some Key Points that emerged:

  • Most women had told their doctor (GP) about their pregnancy early on and already knew their sickle cell or thalassaemia status before becoming pregnant.
  • Parents said some healthcare professionals (including some GPs) did not have much knowledge of the conditions or the screening pathway and did not recognise the need for prompt referral to counselling and PND. Parents wanted prompt referral or ‘self-referral’ to specialist nurses and midwives at the Sickle Cell and Thalassaemia Centres as from their experience this sped things up.
  • Parents also wanted to meet individuals who were living successfully with sickle cell or thalassaemia and to be put in touch with patient organisations who could find someone in the community to support them.


What Happens Next?

These parent stories are an invaluable resource for people commissioning services and working in the Screening Programme and have contributed to a report with recommendations for community organisations, the public and screening service providers.

The full ‘Parent Stories’ report is available at:

For hard copies please email:



My Friend Jen: A Little Different

JLG Publishing

ISBN: 978-0995664104

Date of Publication: Aug 2016

Size: 21.59 x 21.59 cm

Format: Paperback, 24 pages

RRP: £5.99, $9.90, €7.99

Available from Amazon, Barnes & Noble and



“There is nothing that my friend Jen can’t do, but on the inside, she’s a little different to me and you.”

As Jen’s friend tells us a story, you will learn a few simple tips on how to stay well with Sickle Cell.

A Little Different is the first in the series of My Friend Jen books. The series of rhyming children’s books aims to create better understanding and awareness of the blood disorder sickle cell anaemia in a fun and informative way.



Award winning author, and publisher Jenica Leah is one of the UK’s leading sickle cell ambassadors. Living with sickle cell anaemia herself, she has battled with the many complications that come with the condition and has never let this hold her back.

After undergoing total hip replacement surgery at the age of 25, Jenica Leah vowed to help others living with the sickle cell condition by being more open about her health and her journey, and by doing more to create awareness and get people talking about it.

The My Friend Jen children’s book series is just one of the ways in which she does this.



  • Fun and easy to read story on an educational topic, encouraging children to learn about one of the most common genetic disorders in the world.
  • Story written from a real-life perspective with passion and the drive to inspire readers.
  • The simple yet factual information in the story makes it a great teaching tool.
  •  It’s rhyming and rhythmic writing style makes it easy to read and remember key facts



“An excellent book for young children affected by sickle cell disorders.” – Professor Dame Elizabeth N. Anionwu

“A great resource for families and health professional: encourages good self-care & education of all around to see the person beyond the difference.”  – Dr C J Wright, FRCP, FRCPath

“This book is well written particularly for young patients to understand more about their condition. It is indeed a useful reference and my firm view is that it should be easily accessible to all.” – Dr S Pancham, Specialist in Haematology.


Articles in the Medical Literature


Hemoglobin. 2017 Nov 28:1-5. doi: 10.1080/03630269.2017.1397017. [Epub ahead of print]

Perinatal Maternal Mortality in Sickle Cell Anemia: Two Case Reports and Review of the Literature.

Rizk S1, Pulte ED1, Axelrod D1, Ballas SK1.


As outcomes of patients with sickle cell anemia improve and survival into adulthood with good quality of life and expectation of long-term survival becomes more common, challenges have developed, including issues related to reproduction. Pregnancy is frequently complicated in patients with sickle cell anemia with mortality up to 4.0%. Here we report maternal perinatal mortality in two women with sickle cell anemia who died post-partum due to acute chest syndrome (ACS), caused by bone marrow fat embolism and review the literature pertinent to this subject. Patient A was a 28-year-old woman with sickle cell anemia with multiple complications. At 30 weeks’ gestation she developed hemolysis associated with poor placental function necessitating delivery by C-section. The fetus was delivered successfully but she died due to multi organ failure after delivery. Autopsy showed pulmonary and amniotic fluid embolization. Patient B was a 37-year-old woman with uncomplicated sickle cell anemia who presented with pre term labor and crisis, then ACS and fetal distress. The infant was delivered successfully but the patient died after cardiovascular collapse. Autopsy results showed fat and bone marrow embolization as the cause of death. Pregnancy continues to be high risk for patients with sickle cell anemia including those with mild disease. Maternal perinatal mortality could be unpredictable due to serious complications of sickle cell disease. More studies to assess maternal perinatal mortality are needed.

PMID: 29182088



Mol Genet Genomic Med. 2017 Nov;5(6):692-699. doi: 10.1002/mgg3.327. Epub 2017 Aug 23.

Sickle cell trait knowledge and health literacy in caregivers who receive in-person sickle cell trait education.

Creary S1, Adan I1, Stanek J1, O’Brien SH1, Chisolm DJ1, Jeffries T1, Zajo K1, Varga E1.



Despite universal screening that detects sickle cell trait (SCT) in infancy, only 16% of Americans with SCT know their status. To increase SCT status awareness, effective education for patients and their families is needed. The objective of this study was to assess caregivers’ SCT knowledge before and after an in-person SCT education session.


A trained educator provides in-person SCT education to caregivers of referred infants with SCT at Nationwide Children’s Hospital. From August 2015 to July 2016, primarily English-speaking caregivers of infants with hemoglobin S-trait were recruited and completed a health literacy assessment and a SCT knowledge assessment (SCTKA) before and after receiving education. Caregivers repeated the SCTKA again after ≥6 months, if they could be contacted.


Thirty-eight (38.1%) percent of 113 caregivers had high SCTKA scores (≥75%) before education but 90.3% achieved high scores after education. Caregivers with low SCTKA scores after education had significantly lower health literacy (P = 0.029) and baseline SCTKA scores (P = 0.003) compared to those with higher scores after education. At ≥6 months, caregivers’ scores were significantly higher (P = 0.014) than baseline, but only 73.3% scored ≥75%.


Our results suggest that caregivers’ baseline SCT knowledge is low, improves with in-person education but may decline with time. Caregivers who do not achieve high SCT knowledge after education had lower health literacy and baseline knowledge. Future studies should determine if adapting in-person education to caregivers’ health literacy and knowledge levels results in high and sustained SCT knowledge among all caregivers and more individuals who know their SCT status.

© 2017 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.

Free Article

PMID: 29178654

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J Pediatr Hematol Oncol. 2017 Nov 23. doi: 10.1097/MPH.0000000000001018. [Epub ahead of print]

Feasibility and Acceptability of Internet-delivered Cognitive Behavioral Therapy for Chronic Pain in Adolescents With Sickle Cell Disease and Their Parents.

Palermo TM1,2, Dudeney J1, Santanelli JP3, Carletti A1, Zempsky WT4,5..


Pain is a clinical hallmark of sickle cell disease (SCD), and is rarely optimally managed. Cognitive-behavioral therapy (CBT) for pain has been effectively delivered through the Internet in other pediatric populations. We tested feasibility and acceptability of an Internet-delivered CBT intervention in 25 adolescents with SCD (64% female, mean age=14.8 y) and their parents randomized to Internet CBT (n=15) or Internet Pain Education (n=10). Participants completed pretreatment/posttreatment measures. Eight dyads completed semistructured interviews to evaluate treatment acceptability. Feasibility indicators included recruitment and participation rates, engagement and adherence to intervention, and completion of outcome measures. In total, 87 referrals were received from 9 study sites; our recruitment rate was 60% from those families approached for screening. Among participants, high levels of initial intervention engagement (>90%), and adherence (>70%) were demonstrated. Most participants completed posttreatment outcome and diary measures (>75%). Retention at posttreatment was 80%. High treatment acceptability was reported in interviews. Our findings suggest that Internet-delivered CBT for SCD pain is feasible and acceptable to adolescents with SCD and their parents. Engagement and adherence were good. Next steps are to modify recruitment plans to enhance enrollment and determine efficacy of Internet CBT for SCD pain in a large multisite randomized controlled trial.

PMID: 29176462

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Am J Hematol. 2017 Nov 23. doi: 10.1002/ajh.24986. [Epub ahead of print]

The clinical epidemiology of sickle cell anemia in Africa.

Macharia AW1, Mochamah G1, Uyoga S1, Ndila CM1, Nyutu G1, Makale J1, Tendwa M1, Nyatichi E1, Ojal J1, Shebe M1, Awuondo KO1, Mturi N1, Peshu N1, Tsofa B1, Scott JAG1,2,3, Maitland K1,4, Williams TN1,3,4.


Sickle cell anemia (SCA) is the commonest severe monogenic disorders of humans. The disease has been highly characterized in high-income countries but not in sub-Saharan Africa where SCA is most prevalent. We conducted a retrospective cohort study of all children 0-13 years admitted from within a defined study area to Kilifi County Hospital in Kenya over a five-year period. Children were genotyped for SCA retrospectively and incidence rates calculated with reference to population data. Overall, 576 of 18,873 (3.1%) admissions had SCA of whom the majority (399; 69.3%) were previously undiagnosed. The incidence of all-cause hospital admission was 57.2/100 person years of observation (PYO; 95%CI 52.6-62.1) in children with SCA and 3.7/100 PYO (95%CI 3.7-3.8) in those without SCA (IRR 15.3; 95%CI 14.1-16.6). Rates were higher for the majority of syndromic diagnoses at all ages beyond the neonatal period, being especially high for severe anemia (hemoglobin <50g/L) (IRR 58.8; 95%CI 50.3-68.7), stroke (IRR 486; 95%CI 68.4-3,450), bacteremia (IRR 23.4; 95%CI 17.4-31.4), and for bone (IRR 607; 95%CI 284-1,300) and joint (IRR 80.9; 95%CI 18.1-362) infections. The use of an algorithm based on just five clinical features would have identified approximately half of all SCA cases among hospital-admitted children with a number needed to test to identify each affected patient of fourteen. Our study illustrates the clinical epidemiology of SCA in a malaria-endemic environment without specific interventions. The targeted testing of hospital-admitted children using the Kilifi Algorithm provides a pragmatic approach to early-diagnosis in high-prevalence countries where newborn screening is unavailable. This article is protected by copyright. All rights reserved.

PMID: 29168218

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MMWR Morb Mortal Wkly Rep. 2017 Nov 24;66(46):1269-1271. doi: 10.15585/mmwr.mm6646a2.

CDC Grand Rounds: Improving the Lives of Persons with Sickle Cell Disease.

Hulihan M, Hassell KL, Raphael JL, Smith-Whitley K, Thorpe P.


Approximately 100,000 Americans have sickle cell disease (SCD), a group of recessively inherited red blood cell disorders characterized by abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in the red blood cells. Persons with hemoglobin SS or hemoglobin Sß0 thalassemia, also known as sickle cell anemia (SCA), have the most severe form of SCD. Hemoglobin SC disease and hemoglobin Sß+ thalassemia are other common forms of SCD. Red blood cells that contain sickle hemoglobin are inflexible and can stick to vessel walls, causing a blockage that slows or stops blood flow. When this happens, oxygen cannot reach nearby tissues, leading to attacks of sudden, severe pain, called pain crises, which are the clinical hallmark of SCD. The red cell sickling and poor oxygen delivery can also cause damage to the brain, spleen, eyes, lungs, liver, and multiple other organs and organ systems. These chronic complications can lead to increased morbidity, early mortality, or both. Tremendous strides in treating and preventing the complications of SCD have extended life expectancy. Now, nearly 95% of persons born with SCD in the United States reach age 18 years (1); however, adults with the most severe forms of SCD have a life span that is 20-30 years shorter than that of persons without SCD (2).

Free Article

PMID: 29166365 [Indexed for MEDLINE]

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Stem Cells Transl Med. 2017 Nov 21. doi: 10.1002/sctm.17-0066. [Epub ahead of print]

A Universal Approach to Correct Various HBB Gene Mutations in Human Stem Cells for Gene Therapy of Beta-Thalassemia and Sickle Cell Disease.

Cai L1,2,3, Bai H2,3, Mahairaki V3,4, Gao Y2,3, He C2,3, Wen Y1,5, Jin YC3,6, Wang Y3,7, Pan RL3,8, Qasba A3,9, Ye Z2,10, Cheng L2,3.


Beta-thalassemia is one of the most common recessive genetic diseases, caused by mutations in the HBB gene. Over 200 different types of mutations in the HBB gene containing three exons have been identified in patients with β-thalassemia (β-thal) whereas a homozygous mutation in exon 1 causes sickle cell disease (SCD). Novel therapeutic strategies to permanently correct the HBB mutation in stem cells that are able to expand and differentiate into erythrocytes producing corrected HBB proteins are highly desirable. Genome editing aided by CRISPR/Cas9 and other site-specific engineered nucleases offers promise to precisely correct a genetic mutation in the native genome without alterations in other parts of the human genome. Although making a sequence-specific nuclease to enhance correction of a specific HBB mutation by homology-directed repair (HDR) is becoming straightforward, targeting various HBB mutations of β-thal is still challenging because individual guide RNA as well as a donor DNA template for HDR of each type of HBB gene mutation have to be selected and validated. Using human induced pluripotent stem cells (iPSCs) from two β-thal patients with different HBB gene mutations, we devised and tested a universal strategy to achieve targeted insertion of the HBB cDNA in exon 1 of HBB gene using Cas9 and two validated guide RNAs. We observed that HBB protein production was restored in erythrocytes derived from iPSCs of two patients. This strategy of restoring functional HBB gene expression will be able to correct most types of HBB gene mutations in β-thal and SCD. Stem Cells Translational Medicine 2017.

Free Article

PMID: 29164808

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J Pediatr Psychol. 2017 Nov 16. doi: 10.1093/jpepsy/jsx141. [Epub ahead of print]

Cognitive Function, Coping, and Depressive Symptoms in Children and Adolescents with Sickle Cell Disease.

Prussien KV1, DeBaun MR2, Yarboi J1, Bemis H1, McNally C1, Williams E1, Compas BE1.



The objective of this study was to investigate the association between cognitive functioning, coping, and depressive symptoms in children and adolescents with sickle cell disease (SCD).


Forty-four children (M age = 9.30, SD = 3.08; 56.8% male) with SCD completed cognitive assessments measuring working memory (Wechsler Intelligence Scale for Children-Fourth Edition) and verbal comprehension (Wechsler Abbreviated Scale of Intelligence-Second Edition). Participants’ primary caregivers completed questionnaires assessing their child’s coping and depressive symptoms.


Verbal comprehension was significantly positively associated with secondary control coping (cognitive reappraisal, acceptance, distraction), and both working memory and secondary control coping were negatively associated with depressive symptoms. In partial support of the primary study hypothesis, verbal comprehension had an indirect association with depressive symptoms through secondary control coping, whereas working memory had a direct association with depressive symptoms.


The results provide new evidence for the associations between cognitive function and coping, and the association of both of these processes with depressive symptoms in children with SCD. Findings provide potential implications for clinical practice, including interventions to improve children’s cognitive functioning to attenuate depressive symptoms.

PMID: 29155970

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Glob Pediatr Health. 2017 Nov 7;4:2333794X17739194. doi: 10.1177/2333794X17739194. eCollection 2017.

Disease Knowledge, Illness Perceptions, and Quality of Life in Adolescents With Sickle Cell Disease: Is There a Link?

Asnani MR1, Barton-Gooden A1, Grindley M1, Knight-Madden J1.


Disease knowledge, illness perceptions, and quality of life (QOL) were examined in 150 adolescents (mean age = 16.1 years, SD = 1.9; 49.3% males) with sickle cell disease (SCD). Females had higher knowledge (P = .004), lower QOL (P = .02), and perceived their illness to be more unpredictable (P = .03). Those with more severe disease perceived their illness to be unpredictable with worse outcomes. Those with higher knowledge scores perceived their illness to be chronic, made more sense of their illness, and perceived greater personal and treatment control. Final hierarchical regression model showed that secondary education as compared to primary education level (P < .001) was positively correlated whereas disease severity (P < .001), perceived unpredictability (P = .024), and negative emotions (P < .001) were negatively correlated with QOL. Health practitioners should assess adolescents’ illness perceptions and encouraging continuing schooling and addressing emotional/psychological problems could improve their QOL.

PMCID: PMC5680938 Free PMC Article

PMID: 29152543

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Conflict of interest statement

Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.



Ann Hematol. 2017 Nov 16. doi: 10.1007/s00277-017-3182-8. [Epub ahead of print]

Simultaneous point-of-care detection of anemia and sickle cell disease in Tanzania: the RAPID study.

Smart LR1, Ambrose EE2,3, Raphael KC2, Hokororo A2,3, Kamugisha E4, Tyburski EA5,6, Lam WA5,6, Ware RE1, McGann PT7.


Both anemia and sickle cell disease (SCD) are highly prevalent across sub-Saharan Africa, and limited resources exist to diagnose these conditions quickly and accurately. The development of simple, inexpensive, and accurate point-of-care (POC) assays represents an important advance for global hematology, one that could facilitate timely and life-saving medical interventions. In this prospective study, Robust Assays for Point-of-care Identification of Disease (RAPID), we simultaneously evaluated a POC immunoassay (Sickle SCAN™) to diagnose SCD and a first-generation POC color-based assay to detect anemia. Performed at Bugando Medical Center in Mwanza, Tanzania, RAPID tested 752 participants (age 1 day to 20 years) in four busy clinical locations. With minimally trained medical staff, the SCD POC assay diagnosed SCD with 98.1% sensitivity and 91.1% specificity. The hemoglobin POC assay had 83.2% sensitivity and 74.5% specificity for detection of severe anemia (Hb ≤ 7 g/dL). Interobserver agreement was excellent for both POC assays (r = 0.95-0.96). Results for the hemoglobin POC assay have informed the second-generation assay design to be more suitable for low-resource settings. RAPID provides practical feasibility data regarding two novel POC assays for the diagnosis of anemia and SCD in real-world field evaluations and documents the utility and potential impact of these POC assays for sub-Saharan Africa.

PMID: 29147848

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Br J Haematol. 2017 Nov 16. doi: 10.1111/bjh.15021. [Epub ahead of print]

Fetal haemoglobin induction in sickle cell disease.

Paikari A1, Sheehan VA1.


Fetal haemoglobin (HbF, α2γ2) induction has long been an area of investigation, as it is known to ameliorate the clinical complications of sickle cell disease (SCD). Progress in identifying novel HbF-inducing strategies has been stymied by limited understanding of gamma (γ)-globin regulation. Genome-wide association studies (GWAS) have identified variants in BCL11A and HBS1L-MYB that are associated with HbF levels. Functional studies have established the roles of BCL11A, MYB, and KLF1 in γ-globin regulation, but this information has not yielded new pharmacological agents. Several drugs are under investigation in clinical trials as HbF-inducing agents, but hydroxycarbamide remains the only widely used pharmacologic therapy for SCD. Autologous transplant of edited haematopoietic stem cells holds promise as a cure for SCD, either through HbF induction or correction of the causative mutation, but several technical and safety hurdles must be overcome before this therapy can be offered widely, and pharmacological therapies are still needed.

PMID: 29143315

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Pediatr Blood Cancer. 2017 Nov 14. doi: 10.1002/pbc.26885. [Epub ahead of print]

Acute chest syndrome among children hospitalized with vaso-occlusive crisis: A nationwide study in the United States.

Takahashi T1, Okubo Y2,3, Handa A4.



Acute chest syndrome (ACS) is a common complication among pediatric inpatients with sickle cell disease and vaso-occlusive crisis (VOC). However, little is known about the factors associated with ACS complication. The present study assessed the epidemiological features of children hospitalized with VOC and ascertained factors associated with ACS complication.


Hospital discharge records of patients with VOC aged <20 years were obtained for the years 2003, 2006, 2009, and 2012 from the Kids’ Inpatient Database. Data were weighted to estimate the annual hospitalization rates with respect to gender and race/ethnicity in the United States. Multivariable logistic regression was conducted to ascertain factors associated with ACS complication after adjusting for patient and hospital characteristics.


The total annual hospitalizations for VOC increased from 22,511 in 2003 to 24,292 in 2012. Multivariable logistic regression analysis showed that children aged 5-9 years had 2.59 times higher odds of ACS than children aged 15-19 years (95% confidence interval [CI], 2.32-2.88). Comorbidity of asthma (odds ratio [OR], 1.42; 95% CI, 1.31-1.54) and obstructive sleep apnea (OR, 1.70; 95% CI, 1.31-2.20) were associated with ACS development. ACS was also associated with male gender and the summer and fall seasons.


We reported nationwide estimates of the annual hospitalization rate for childhood VOC in the United States and demonstrated the major risk factors associated with ACS complication. Vigilance is needed for ACS complications in high-risk VOC admissions.

© 2017 Wiley Periodicals, Inc.

PMID: 29134776

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Cochrane Database Syst Rev. 2017 Nov 7;11:CD003425. doi: 10.1002/14651858.CD003425.pub4. [Epub ahead of print]

Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease.

Owusu-Ofori S1, Remmington T.



Acute splenic sequestration crises are a complication of sickle cell disease, with high mortality rates and frequent recurrence in survivors of first attacks. Splenectomy and blood transfusion, with their consequences, are the mainstay of long-term management used in different parts of the world. This is a 2017 update of a Cochrane Review first published in 2002, and previously updated, most recently in 2015.


To assess whether splenectomy (total or partial), to prevent acute splenic sequestration crises in people with sickle cell disease, improved survival and decreased morbidity in people with sickle cell disease, as compared with regular blood transfusions.


We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, which comprises of references identified from comprehensive electronic database searches and handsearching relevant journals and abstract books of conference proceedings. We also searched clinical trial registries. Additional trials were sought from the reference lists of the trials and reviews identified by the search strategy.Date of the most recent search: 14 August 2017.


All randomized or quasi-randomized controlled trials comparing splenectomy (total or partial) to prevent recurrence of acute splenic sequestration crises with no treatment or blood transfusions in people with sickle cell disease.


No trials of splenectomy for acute splenic sequestration were found.


No trials of splenectomy for acute splenic sequestration were found.


Splenectomy, if full, will prevent further sequestration and if partial, may reduce the recurrence of acute splenic sequestration crises. However, there is a lack of evidence from trials showing that splenectomy improves survival and decreases morbidity in people with sickle cell disease. There is a need for a well-designed, adequately-powered, randomized controlled trial to assess the benefits and risks of splenectomy compared to transfusion programmes, as a means of improving survival and decreasing mortality from acute splenic sequestration in people with sickle cell disease.There are no trials included in the review and we have not identified any relevant trials up to August 2017. We will continue to run searches to identify any potentially relevant trials; however, we do not plan to update other sections of the review until new trials are published.

PMID: 29112240

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Arch Dis Child. 2017 Nov 5. pii: archdischild-2017-313213. doi: 10.1136/archdischild-2017-313213. [Epub ahead of print]

Evaluation of newborn sickle cell screening programme in England: 2010-2016.

Streetly A1,2, Sisodia R1, Dick M3, Latinovic R4, Hounsell K1, Dormandy E1.



To evaluate England’s NHS newborn sickle cell screening programme performance in children up to the age of 5 years.


Cohort of resident infants with sickle cell disease (SCD) born between 1 September 2010 and 31 August 2015 and followed until August 2016.


1317 infants with SCD were notified to the study from all centres in England and 1313 (99%) were followed up.


Early enrolment in clinical follow-up, parental education and routine penicillin prophylaxis.


Age seen by a specialist clinician, age at prescription of penicillin prophylaxis and mortality.


All but two resident cases of SCD were identified through screening; one baby was enrolled in care after prenatal diagnosis; one baby whose parents refused newborn screening presented symptomatically. There were 1054/1313 (80.3%, 95% CI 78% to 82.4%) SCD cases seen by a specialist by 3 months of age and 1273/1313 (97%, 95% CI 95.9% to 97.8%) by 6 months. The percentage seen by 3 months increased from 77% in 2010 to 85.4% in 2015. 1038/1292 (80.3%, 95% CI 78.1% to 82.5%) were prescribed penicillin by 3 months of age and 1257/1292 (97.3%, 95% CI 96.3% to 98.1%) by 6 months. There were three SCD deaths <5 years caused by invasive pneumococcal disease (IPD) sensitive to penicillin.


The SCD screening programme is effective at detecting affected infants. Enrolment into specialist care is timely but below the programme standards. Mortality is reducing but adherence to antibiotic prophylaxis remains important for IPD serotypes not in the current vaccine schedule.

© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Free Article

PMID: 29104181

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Conflict of interest statement

Competing interests: None declared.



Blood Cells Mol Dis. 2017 Oct 9. pii: S1079-9796(17)30322-4. doi: 10.1016/j.bcmd.2017.08.017. [Epub ahead of print]

Real-life experience with hydroxyurea in sickle cell disease: A multicenter study in a cohort of patients with heterogeneous descent.

Rigano P1, De Franceschi L2, Sainati L3, Piga A4, Piel FB5, Cappellini MD6, Fidone C7, Masera N8, Palazzi G9, Gianesin B10, Forni GL10; Italian Multicenter Study of Hydroxyurea in Sickle Cell Anemia Investigators.


We conducted the first nation-wide cohort study of sickle cell disease (SCD) in Italy, a Southern European country exposed to intense recent flux migration from endemic areas for SCD. We evaluate the impact of hydroxyurea on a total of 652 pediatric and adult patients from 33 Reference Centers for SCD (mean age 24.5±15years, 51.4% males). Hydroxyurea median treatment duration was 7years (range: <1year to 29years) at a mean therapeutic dose of 18±4.7mg/kg/day. Hydroxyurea was associated with a significant increase in mean total and fetal hemoglobin and a significant decrease in mean hemoglobin S, white blood and platelet counts, and lactate dehydrogenase levels. Hydroxyurea was associated with a significant reduction in the incidence of acute chest syndrome (-29.3%, p<0.001), vaso-occlusive crisis (-34.1%, p<0.001), hospitalization (-53.2%, p<0.001), and bone necrosis (-6.9%, p<0.001). New silent cerebral infarction (SCI) occurred during treatment (+42.4%, p<0.001) but not stroke (+0.5%, p=0.572). These observations were generally consistent upon stratification for age, descent (Caucasian or African), genotype (βS/βS, βS/β0 or βS/β+) and duration of treatment (< or ≥10years). There were no new safety concerns observed compared to those commonly reported in the literature. Our study, conducted on a large population of patients with different descent and compound state supports the benefits of hydroxyurea therapy as a treatment option. Registered at clinical (NCT02709681).

PMID: 29107441

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Cannabis Cannabinoid Res. 2017 Jul 1;2(1):197-201. doi: 10.1089/can.2017.0011. eCollection 2017.

The Use of Cannabis by Patients with Sickle Cell Disease Increased the Frequency of Hospitalization due to Vaso-Occlusive Crises.

Ballas SK1..


Introduction: The objective of this study was to determine if patients with sickle cell disease using cannabis had decreased frequency of acute vaso-occlusive crises (VOCs) that required hospitalization. Method: This was a retrospective study in which 270 urine drug screen tests were done on 72 patients: 40 males and 32 females. Results: Cannabinoids were found in 144 urine tests from 37 patients and were negative in 126 tests from 35 patients. Males who used cannabis were significantly younger (p<0.001) than males who did not. Patients who tested positive used benzodiazepines, cocaine, and phencyclidine significantly more often than patients who tested negative. There was no significant difference in the amounts of opioids consumed by users and nonusers of cannabis. The cannabis cohort was seen in the clinic significantly (p<0.05) less often than controls, but hospital admissions were significantly greater in the cannabis group than controls (p<0.05). Conclusion: These data show an unexpected negative effect of cannabis on the frequency of VOCs. This may be due to the effect of cannabis on the brain and/or the severity of the disease in the cannabis users. More controlled studies are needed to clarify these findings.

PMCID: PMC5627667 Free PMC Article

PMID: 29082316

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Conflict of interest statement

No competing financial interests exist.



Hematol Oncol Clin North Am. 2017 Dec;31(6):1061-1079. doi: 10.1016/j.hoc.2017.08.009.

Sickle Cell Disease in the Emergency Department.

Lovett PB1, Sule HP2, Lopez BL1.

Author information:

1 Department of Emergency Medicine, Thomas Jefferson University & Hospitals, 1020 Sansom Street, Suite 239, Thompson Building, Philadelphia, PA 19107, USA.

2 Department of Emergency Medicine, Thomas Jefferson University & Hospitals, 1020 Sansom Street, Suite 239, Thompson Building, Philadelphia, PA 19107, USA. Electronic address:


Acute painful episodes are the most common reason for emergency department visits among patients with sickle cell disease (SCD). Early and aggressive pain management is a priority. Emergency providers (EPs) must also diagnose other emergent diagnoses in patients with SCD and differentiate them from vaso-occlusive crisis. EPs should be aware of cognitive biases that may misdirect the diagnostic process. Administration of intravenous fluids should be used judiciously. Blood transfusion may be considered. Coordination of care with hematology is an important part of the effective emergency department and long-term management of patients with SCD.

PMID: 29078924

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Sickle Cell Conferences and Events


The Global Sickle Cell Disease Network (GSCDN) invites you to the GSCDN session on Sunday, December 10, 2017 at the Omni Atlanta Hotel, Room International F, during the American Society of Hematology (ASH) meeting in Atlanta, USA.

The theme for this year’s meeting is “Newborn screening for sickle cell disease in sub-Saharan Africa.” The meeting will include discussions around the current landscape and next steps of newborn screening for sickle cell disease in sub-Saharan Africa. If you wish to attend the meeting, kindly RSVP at by Monday, November 27, at the latest. For more information on the meeting please see attached invitation or email



The Sickle Cell Disease Association of America, Inc. will be hosting its third Community Health Worker Certification Training Program, with additional training on sickle cell disease, beginning January 29, 2018.

The seven week training will be held via webinar, Mondays and Wednesdays from 12PM-5PM EST. In addition to the online coursework, trainees must complete 80 hours of fieldwork (40 hours in a medical institution and 40 hours in a community organization). They must also pass a final exam. The training costs are estimated to be $1200 ($800 for the course plus additional fees and expenses). SCDAA now offers a Sickle Cell Disease Community Health Worker Certification Program. This one-of-a-kind certification allows individuals to learn about the fundamentals of community health workers, as well as sickle cell disease.

What is a community health worker?

According to the American Public Health Association, a community health worker (CHW) is, “A frontline public health worker who is a trusted member of and/or has an unusually close understanding of the community served.”

What qualities should a community health worker possess?

  • Connected to Community
  • Persistent, Creative and Resourceful
  • Empathic, Caring, Compassionate and Humble
  • Open-minded/Non-judgmental
  • Honest, Respectful, Patient, Realistic
  • Friendly, Engaging, Sociable
  • Dependable, Responsible, Reliable
  • Culturally sensitive, able to work with diverse communities

Who employs community health workers?

CHWs work in a variety of settings, which can include: hospitals, clinics, community-based organizations, health departments, payors, faith-based organizations, and many more.

How can community health workers help individuals with sickle cell disease?

Community health workers can assist individuals with sickle cell disease in a variety of ways. They may be asked to help identity and overcome barriers to care, such as transportation or childcare, navigate the healthcare system, assist with obtaining health insurance, conduct health education, reinforce healthy behaviors, or assist clients with other needs, as they arise.

How can I train to be a community health worker?

The Sickle Cell Disease Association of America offers a seven week sickle cell disease community health worker certification. The next training will begin on January 29, 2018. If you are interested you may download the application here

For any additional questions, please contact Jessica Suggs at or call 410-528-1555.