Children and adolescents with sickle cell anemia who received the adenosine diphosphate–directed antiplatelet agent prasugrel had no significant reduction in painful vasoocclusive crises compared with those who received placebo, found a trial conducted in 13 countries (Heeney MM et al. N Engl J Med. doi:10.1056/NEJMoa1512021 [published online December 8, 2015]). Previous studies had suggested that prasugrel reduced markers of platelet activation and, consequently, the rate and intensity of vasoocclusive pain in sickle cell anemia.
The Tyler sisters are tight. 11-year old Amatullah is the tomboy. 10-year old Khadeejah, the princess, who loves pinks. And 6-year old Hajar just loves to dance and sing. Watching them play Uno together at their Hampton home, the girls look like the picture of health.
But, the sisters were born with the cards stacked against them. All three, have with sickle cell disease. At the AFLAC Cancer and Blood Disorders Center at Children’s Healthcareof Atlanta, hematologist Dr. Fola Adisa and her team treat about 1,700 GeAnd at 7, Khadeejah had a stroke.
“I was scared for her, I was petrified,” says her mother Mapillar Dahn.
“It was on the left side of my brain,” Khadeejah says, “So they had to hook me up to another transfusion.”
Khadeejah was hospitalized. This spring, she’ll undergo a neurosurgery surgery to repair some damaged blood vessels in her brain. She’s recovered, but is still catching up. Even with the challenges, the girls are learning how to manage their disorders, even thrive with them.
“They’re pretty aware for kids, and that’s on purpose,” says their mom. “Because I want them to know what they have. Life is not guaranteed for anybody. Mommy is not always going to be here. I need them to be able to take care of themselves like Mommy is taking care of them.”Mapillar Dahn says, “We are blessed.”
Because the Tyler girls have something stronger than sickle cell: each other.
“It teaches you to be a little more grateful for what you have, especially for your health,” says Mapillar. Mapillar Dahn recently founded MTS Sickle Cell Foundation, Inc, a non-profit organization dedicated to helping raise awareness and provide support to the sickle cell community. To learn more about the organization and about sickle cell disease, please visit www.MyThreeSicklers.org.
A index of sickle cell articles with the latest research.
Sickle cell in the Medical Literature
1. West Indian Med J. 2015 Sep 22;65(1). doi: 10.7727/wimj.2015.492. [Epub ahead of print]
Newborn Screening for Sickle Cell Disease: Jamaican Experience.
Mason K1, Gibson F2, Gardner R1, Warren L1, Fisher C3, Higgs D3, Happich M4, Kulozik A4, Hambleton I5, Serjeant BE1, Serjeant GR1,6.
To review the history of newborn screening for sickle cell disease with especial reference to Jamaica.
A summary was done of the history, the development of associated laboratory technology and the implementation of newborn screening for sickle cell disease in Jamaica.
Screening was initiated at Victoria Jubilee Hospital, Kingston from 1973-1981, reactivated in 1995 and extended to the University Hospital of the West Indies in 1997 and to Spanish Town Hospital in 1998. From August 2008, there was a progressive recruitment of 12 hospitals in the south and west of Jamaica which has raised the frequency of islandwide newborn coverage from 25% in 1973 to 81%. The results of this extended programme in southwest Jamaica are presented. Dried blood spots collected from the umbilical cord proved stable, cheap and efficient; mean sample collection rates were 98%, maternal contamination occurred in < 1% and caused diagnostic confusion in < 0.1%. By March 31, 2015, a total of 54 566 births have been screened, detecting 161 with homozygous sickle cell (SS) disease, 125 with sickle cell-haemoglobin C (SC) disease and 36 with sickle cell-beta thalassaemia. Of the 327 babies with clinically significant sickle cell syndromes, all except five who died within seven days of birth were confirmed by four to six weeks and recruited to local sickle cell clinics.
Early detection of sickle cell disease and recruitment to clinics is known to reduce its morbidity and mortality. The methods currently detailed provide an effective and economic model of newborn screening which may be of value elsewhere.
PMID: 26901597 [PubMed – as supplied by publisher]
2. Int J Hematol Ther. 2015;1(1). doi: 10.15436/2381-1404.15.003. Epub 2015 Sep 6.
Exploring Adult Care Experiences and Barriers to Transition in Adult Patients with Sickle Cell Disease.
Bemrich-Stolz CJ1, Halanych JH2, Howard TH1, Hilliard LM1, Lebensburger JD1.
Young adults with sickle cell anemia are at high risk for increased hospitalization and death at the time of transition to adult care. This may be related to failure of the transition system to prepare young adults for the adult healthcare system. This qualitative study was designed to identify factors related to transition that may affect the health of adults with sickle cell anemia.
Ten patients currently treated in an adult hematology clinic participated in semi-structured qualitative interviews to describe their experience transitioning from pediatric to adult care and differences in adult and pediatric healthcare systems.
Participants were generally unprepared for the adult healthcare system. Negative issues experienced by participants included physician mistrust, difficulty with employers, keeping insurance, and stress in personal relationships. Positive issues experienced by participants included improved self efficacy with improved self care and autonomy.
In the absence of a formalized transition program, adults with sickle cell anemia experience significant barriers to adult care. In addition to medical history review and identification of an adult provider, transition programs should incorporate strategies to navigate the adult medical system, insurance and relationships as well as encouraging self efficacy.
PMID: 26900602 [PubMed]
3. JACC Cardiovasc Imaging. 2016 Feb 10. pii: S1936-878X(15)00859-1. doi: 10.1016/j.jcmg.2015.05.013. [Epub ahead of print]
Cardiomyopathy With Restrictive Physiology in Sickle Cell Disease.
Niss O1, Quinn CT2, Lane A3, Daily J4, Khoury PR4, Bakeer N2, Kimball TR4, Towbin JA4, Malik P5, Taylor MD4.
The aim of this study was to identify a unifying cardiac pathophysiology that explains the cardiac pathological features in sickle cell disease (SCD).
Cardiopulmonary complications, the leading cause of adult death in SCD, are associated with heart chamber dilation, diastolic dysfunction, elevated tricuspid regurgitant jet velocity (TRV), and pulmonary hypertension. However, no unifying cardiac pathophysiology has been identified to explain these findings.
In a 2-part study, we first examined patients with SCD who underwent screening echocardiography during steady state at our institution. We then conducted a meta-analysis of cardiac studies in SCD.
In the 134 patients with SCD studied (median age 11 years), significant enlargement of the left atrial volume was present (z-score 3.1, p = 0.002), shortening fraction was normal (37.6 ± 4.7%), and lateral and septal ratios of mitral velocity to early diastolic velocity of the mitral annulus (E/e’) were severely abnormal in 8% and 14% of patients, respectively, indicating impaired diastolic function. Both TRV and lateral E/e’ correlated with enlarged left atrial volume in SCD (p = 0.003 and p = 0.006, respectively). Meta-analysis of 68 studies confirmed significant left atrial diameter enlargement in patients with SCD compared with controls, evidence of diastolic dysfunction and enlarged left ventricular end-diastolic dimension with normal shortening fraction. The majority of patients with catheter-confirmed pulmonary hypertension had mild pulmonary venous hypertension consistent with restrictive cardiac physiology.
Patients with SCD have a unique form of cardiomyopathy with restrictive physiology that is superimposed on hyperdynamic physiology and is characterized by diastolic dysfunction, left atrial dilation, and normal systolic function. This combination results in mild, secondary, pulmonary venous hypertension and elevated TRV. Sudden death is common in other forms of restrictive cardiomyopathy. Our finding of this unique restrictive cardiomyopathy may explain the increased mortality rates and sudden death seen in patients with SCD with mildly elevated TRV.
Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
PMID: 26897687 [PubMed – as supplied by publisher]
4. J Racial Ethn Health Disparities. 2016 Mar;3(1):176-82. doi: 10.1007/s40615-015-0142-7. Epub 2015 Jul 21.
Development of a New Adult Sickle Cell Disease Center Within an Academic Cancer Center: Impact on Hospital Utilization Patterns and Care Quality.
Andemariam B1, Jones S2.
A national shortage of specialized centers with expertise in the management of adults with sickle cell disease (SCD) remains a concerning public health disparity. Yet, there is an abundance of cancer centers whose operational infrastructure is not only suited to the treatment of the oncology patient, but also can provide medical and procedural care essential to the management of the patient with SCD. Our adult SCD center was formally embedded within an academic hospital-based cancer center in 2009. An evaluation of the impact of this new center has been performed.
A retrospective chart review was conducted of all SCD encounters occurring 5 years pre- and post-SCD center establishment. Demographic, clinical, as well as hospital utilization and care quality data were compared.
The SCD population grew from 22 to 165 patients. Following establishment of the SCD center, patients experienced greater average annual outpatient preventative visits for chronic disease management (1 vs. 4.1) and fewer average hospitalizations yearly (2.4 vs. 1). There was a decrease in hospitalization rates for management of acute pain (50 vs. 23 %), average hospitalization length of stay (12 vs. 6 days), and the proportion of hospital discharges resulting in readmission within 30 days (60 vs. 40 %). Hydroxyurea use among eligible patients increased from 30 to 90 %.
Findings suggest that embedding adult SCD centers within existing cancer centers can positively impact patterns of health care utilization and improve the quality of care.
PMID: 26896118 [PubMed – in process]
5. Pediatr Blood Cancer. 2016 Feb 19. doi: 10.1002/pbc.25944. [Epub ahead of print]
Initial Evaluation of the Pediatric PROMIS® Health Domains in Children and Adolescents With Sickle Cell Disease.
Dampier C1, Barry V1, Gross HE2, Lui Y3, Thornburg CD4, DeWalt DA2,5, Reeve BB2,6.
The Patient Reported Outcomes Measurement Information System (PROMIS®) has developed pediatric self-report scales measuring several unidimensional health attributes (domains) suitable for use in clinical research, but these measures have not yet been validated in sickle cell disease (SCD).
A convenience sample of SCD children, aged 8-17 years, from two sickle cell programs was recruited at routine clinic visits, including some for hydroxyurea monitoring or monthly transfusions. Children completed PROMIS pediatric items using an online data collection platform, the PROMIS Assessment Center Web site.
A total of 235 participants (mean age 12.5 ± 2.8 years, 49.8% female) participated in the study. Adolescents (ages 12-17 years) reported significantly higher pain interference and depressive symptoms, and worse lower extremity physical functioning domain scores compared to younger children (ages 8-11 years). Female participants reported significantly higher pain interference, fatigue, and depressive symptoms, and worse lower extremity physical functioning domain scores compared with their male counterparts. Participants with hip or joint problems that limited usual activities reported significantly higher pain, fatigue, and depressive symptoms scores, and worse upper/lower extremity physical functioning scores as did participants who had experienced sickle pain in the previous 7 days.
PROMIS pediatric measures are feasible in a research setting and identify expected differences in known group comparisons in a sample of SCD children. The large domain score differences between those with or without SCD-related complications suggest the potential usefulness of these measures in clinical research, but further validation studies are needed, particularly in clinical practice settings.
© 2016 Wiley Periodicals, Inc.
PMID: 26895143 [PubMed – as supplied by publisher]
6. J Pain. 2016 Feb 15. pii: S1526-5900(16)00517-4. doi: 10.1016/j.jpain.2016.01.475. [Epub ahead of print]
An evaluation of central sensitization in patients with sickle cell disease.
Campbell CM1, Moscou-Jackson G2, Carroll CP3, Kiley K3, Haywood C Jr4, Lanzkron S4, Hand M3, Edwards RR5, Haythornthwaite JA3.
Central sensitization (CS), nociceptive hyper-excitability known to amplify and maintain clinical pain, has been identified as a leading culprit responsible for maintaining pain in several chronic pain conditions. Recent evidence suggests that it may explain differences in the symptom experience of individuals with sickle cell disease (SCD). Quantitative sensory testing (QST) can be used to examine CS and identify individuals that may have a heightened CS profile. The present study categorized patients with SCD based on QST responses into a high or low CS phenotype and compared these groups on measures of clinical pain, vaso-occlusive crises, psychosocial factors, and sleep continuity. Eighty-three adult patients with SCD completed QST, questionnaires, daily sleep and pain diaries over a three-month period, weekly phone calls for three months, and monthly phone calls for 12 months. Patients were divided into CS groups (i.e. No/Low CS [n=17] vs. High CS [n=21]), based on thermal and mechanical temporal summation and aftersensations, which were norm-referenced to 47 healthy controls. High CS subjects reported more clinical pain, vaso-occlusive crises, catastrophizing, and negative mood, and poorer sleep continuity (p’s<0.05) over the 18-month follow-up period. Future analyses should investigate whether psychosocial disturbances and sleep mediate the relationship between central sensitization and pain outcomes.
In general, sickle cell disease patients with greater central sensitization had higher clinical pain, more crises, worse sleep, and more psychosocial disturbances compared to the low CS group.
Copyright © 2016 American Pain Society. Published by Elsevier Inc. All rights reserved.
PMID: 26892240 [PubMed – as supplied by publisher]
7. Clin J Pain. 2016 Feb 17. [Epub ahead of print]
Multiple Levels of Suffering: Discrimination in Health-Care Settings is Associated With Enhanced Laboratory Pain Sensitivity in Sickle Cell Disease.
Mathur VA1, Kiley KB, Haywood C Jr, Bediako SM, Lanzkron S, Carroll CP, Buenaver LF, Pejsa M, Edwards RR, Haythornthwaite JA, Campbell CM.
People living with sickle cell disease (SCD) experience severe episodic and chronic pain and frequently report poor interpersonal treatment within health-care settings. In this particularly relevant context, we examined the relationship between perceived discrimination and both clinical and laboratory pain.
Seventy-one patients with SCD provided self-reports of experiences with discrimination in health-care settings and clinical pain severity, and completed a psychophysical pain testing battery in the laboratory.
Discrimination in health-care settings was correlated with greater clinical pain severity and enhanced sensitivity to multiple laboratory-induced pain measures, as well as stress, depression, and sleep. After controlling for relevant covariates, discrimination remained a significant predictor of mechanical temporal summation (a marker of central pain facilitation), but not clinical pain severity or suprathreshold heat pain response. Furthermore, a significant interaction between experience with discrimination and clinical pain severity was associated with mechanical temporal summation; increased experience with discrimination was associated with an increased correlation between clinical pain severity and temporal summation of pain.
Perceived discrimination within health-care settings was associated with pain facilitation. These findings suggest that discrimination may be related to increased central sensitization among SCD patients, and more broadly that health-care social environments may interact with pain pathophysiology.This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially. http://creativecommons.org/licenses/by-nc-nd/4.0.
PMID: 26889615 [PubMed – as supplied by publisher]
8. J Pediatr Hematol Oncol. 2016 Feb 15. [Epub ahead of print]
Assessing the Immunogenic Response of a Single Center’s Pneumococcal Vaccination Protocol in Sickle Cell Disease.
Santoro JD1, Myers L, Kanter J.
Sickle cell disease (SCD) is the most common inherited hematologic disorder in the United States. Patients with SCD are at increased risk of invasive pneumococcal disease and are reliant on both early penicillin prophylaxis and antipneumococcal vaccination for prevention of infection. Although studies examining vaccine response have demonstrated a drop-off of titer response after 3 years, an optimal vaccination regimen has not been identified. Our study sought to assess the immunogenicity of our center’s pneumococcal vaccination strategy, which included Prevnar (PCV-7) (before the introduction of PCV-13) followed by Pneumovax (PPV-23) given routinely at 2 and 5 years of age and then every 5 years thereafter. Our goal was to assess vaccine response in a population of patients with SCD who had received vaccines according to this regimen using multiplex bead analysis. Our study demonstrated a significant percentage of persons with SCD do not maintain a sufficient vaccination response to PPV-23 for 5 years. Our study revealed that only 36% of patients had protective levels of antipneumococcal antibody titers at an average of 37 months after vaccination. Most alarmingly, within the group of patients with subtherapeutic titers, 64% demonstrated vaccine response to <25% of the tested serotypes. These findings were significantly associated with duration of time since last vaccine administration, but the mean age of lack of response was below the 3-year window where vaccine response was previously reported to wane. Our results indicate antipneumococcal immunity may not be optimally maintained using this vaccination strategy in patients with SCD leaving them vulnerable to invasive pneumococcal disease. Many pediatric hematologists stop prophylactic penicillin at 5 years of age making these results alarming. We recommend further investigation into an optimal vaccine schedule and monitoring of antipneumococcal titers in at-risk patients.
PMID: 26886376 [PubMed – as supplied by publisher]
9. Cochrane Database Syst Rev. 2016 Feb 16;2:CD011199. [Epub ahead of print]
Conjugate Haemophilus influenzae type b vaccines for sickle cell disease.
Allali S1, Chalumeau M, Launay O, Ballas SK, de Montalembert M.
People affected with sickle cell disease are at high risk of infection from Haemophilus influenzae type b. Before the implementation of Haemophilus influenzae type b conjugate vaccination in high-income countries, this was responsible for a high mortality rate in children under five years of age. In African countries, where coverage of this vaccination is still extremely low, Haemophilus influenzae type b remains one of the most common cause of bacteraemias in children with sickle cell disease. The increased uptake of this conjugate vaccination may substantially improve the survival of children with sickle cell disease.
The primary objective was to determine whether Haemophilus influenzae type b conjugate vaccines reduce mortality and morbidity in children and adults with sickle cell disease.The secondary objectives were to assess the following in children and adults with sickle cell disease: the immunogenicity of Haemophilus influenzae type b conjugate vaccines; the safety of these vaccines; and any variation in effect according to type of vaccine, mode of administration (separately or in combination with other vaccines), number of doses, and age at first dose.
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group’s Haemoglobinopathies Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also contacted relevant pharmaceutical companies to identify unpublished trials.Date of last search: 23 November 2015.
All randomised and quasi-randomised controlled trials comparing Haemophilus influenzae type b conjugate vaccines with placebo or no treatment, or comparing different types of Haemophilus influenzae type b conjugate vaccines in people with sickle cell disease.
DATA COLLECTION AND ANALYSIS:
No trials of Haemophilus influenzae type b conjugate vaccines in people with sickle cell disease were found.
There is an absence of evidence from randomised controlled trials relating to the subject of this review.
There has been a dramatic decrease in the incidence of invasive Haemophilus influenzae type b infections observed in the post-vaccination era in people with sickle cell disease living in high-income countries. Therefore, despite the absence of evidence from randomised controlled trials, it is expected that Haemophilus influenzae type b conjugate vaccines may be useful in children affected with sickle cell disease, especially in African countries where there is a high prevalence of the disease. The implementation of childhood immunisation schedules, including universal Haemophilus influenzae type b conjugate vaccination, may substantially improve the survival of children with sickle cell disease living in low-income countries. We currently lack data to evaluate the potential effect of Haemophilus influenzae type b vaccination among unvaccinated adults with sickle cell disease. Further research should assess the optimal Hib immunisation schedule in children and adults with sickle cell disease.
PMID: 26881484 [PubMed – as supplied by publisher]
10. Cochrane Database Syst Rev. 2016 Feb 16;2:CD011130. [Epub ahead of print]
Folate supplementation in people with sickle cell disease.
Dixit R1, Nettem S, Madan SS, Soe HH, Abas AB, Vance LD, Stover PJ.
Sickle cell disease is a group of disorders that affects haemoglobin, which causes distorted sickle- or crescent-shaped red blood cells. It is characterized by anaemia, increased susceptibility to infections and episodes of pain. The disease is acquired by inheriting abnormal genes from both parents, the combination giving rise to different forms of the disease. Due to increased erythropoiesis in people with sickle cell disease, it is hypothesized that they are at an increased risk for folate deficiency. For this reason, children and adults with sickle cell disease, particularly those with sickle cell anaemia, commonly take 1 mg of folic acid orally every day on the premise that this will replace depleted folate stores and reduce the symptoms of anaemia. It is thus important to evaluate the role of folate supplementation in treating sickle cell disease.
To analyse the efficacy and possible adverse effects of folate supplementation (folate occurring naturally in foods, provided as fortified foods or additional supplements such as tablets) in people with sickle cell disease.
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group’s Haemoglobinopathies Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also conducted additional searches in both electronic databases and clinical trial registries.Date of last search: 07 December 2015.
Randomised, placebo-controlled trials of folate supplementation for sickle cell disease.
DATA COLLECTION AND ANALYSIS:
Four review authors assessed the eligibility and risk of bias of the included trials and extracted and analysed the data included in the review. We used the standard Cochrane-defined methodological procedures.
One trial, undertaken in 1983, was eligible for inclusion in the review. This was a double-blind placebo-controlled quasi-randomised triaI of supplementation of folic acid in people with sickle cell disease. A total of 117 children with homozygous sickle cell (SS) disease aged six months to four years of age participated over a one-year period (analysis was restricted to 115 children).Serum folate measures, obtained after trial entry at six and 12 months, were available in 80 of 115 (70%) participants. There were significant differences between the folic acid and placebo groups with regards to serum folate values above 18 µg/l and values below 5 µg/l. In the folic acid group, values above 18 µg/l were observed in 33 of 41 (81 %) compared to six of 39 (15%) participants in the placebo (calcium lactate) group. Additionally, there were no participants in the folic acid group with serum folate levels below 5 µg/l, whereas in the placebo group, 15 of 39 (39%) participants had levels below this threshold. Haematological indices were measured in 100 of 115 (87%) participants at baseline and at one year. After adjusting for sex and age group, the investigators reported no significant differences between the trial groups with regards to total haemoglobin concentrations, either at baseline or at one year. It is important to note that none of the raw data for the outcomes listed above were available for analysis.The proportions of participants who experienced certain clinical events were analysed in all 115 participants, for which raw data were available. There were no statistically significant differences noted; however, the trial was not powered to investigate differences between the folic acid and placebo groups with regards to: minor infections, risk ratio 0.99 (95% confidence interval 0.85 to 1.15); major infections, risk ratio 0.89 (95% confidence interval 0.47 to 1.66); dactylitis, risk ratio 0.67 (95% confidence interval 0.35 to 1.27); acute splenic sequestration, risk ratio 1.07 (95% confidence interval 0.44 to 2.57); or episodes of pain, risk ratio 1.16 (95% confidence interval 0.70 to 1.92). However, the investigators reported a higher proportion of repeat dactylitis episodes in the placebo group, with two or more attacks occurring in 10 of 56 participants compared to two of 59 in the folic acid group (P < 0.05).Growth, determined by height-for-age and weight-for-age, as well as height and growth velocity, was measured in 103 of the 115 participants (90%), for which raw data were not available. The investigators reported no significant differences in growth between the two groups.The trial had a high risk of bias with regards to random sequence generation and incomplete outcome data. There was an unclear risk of bias in relation to allocation concealment, outcome assessment, and selective reporting. Finally, There was a low risk of bias with regards to blinding of participants and personnel. Overall the quality of the evidence in the review was low.There were no trials identified for other eligible comparisons, namely: folate supplementation (fortified foods and physical supplementation with tablets) versus placebo; folate supplementation (naturally occurring in diet) versus placebo; folate supplementation (fortified foods and physical supplementation with tablets) versus folate supplementation (naturally occurring in diet).
One doubIe-blind, placebo-controlled triaI on folic acid supplementation in children with sickle cell disease was included in the review. Overall, the trial presented mixed evidence on the review’s outcomes. No trials in adults were identified. With the limited evidence provided, we conclude that, while it is possible that folic acid supplementation may increase serum folate levels, the effect of supplementation on anaemia and any symptoms of anaemia remains unclear.Further trials may add evidence regarding the efficacy of folate supplementation. Future trials should assess clinical outcomes such as folate concentration, haemoglobin concentration, adverse effects and benefits of the intervention, especially with regards to sickle cell disease-related morbidity. Trials should include people with sickle cell disease of all ages and both sexes, in any setting. To investigate the effects of folate supplementation, trials should recruit more participants and be of longer duration, with long-term follow up, than the trial currently included in this review.
PMID: 26880182 [PubMed – as supplied by publisher]
11. Curr Pain Headache Rep. 2016 Mar;20(3):20. doi: 10.1007/s11916-016-0546-z.
Headache and Facial Pain in Sickle Cell Disease.
Vgontzas A1, Charleston L 4th2, Robbins MS3.
Children and adolescents with sickle cell disease (SCD) have a high prevalence of recurrent headaches (24.0-43.9 %). Acute presentation with headache can be diagnostically challenging, as the clinician must consider evaluation of several potentially devastating conditions including vascular diseases (stroke, hemorrhage, venous sinus thrombosis, moyamoya, posterior reversible encephalopathy syndrome), facial and orbital bone infarcts, dental pain, and osteomyelitis. Patients with SCD and primary headache disorders may benefit from comprehensive headache treatment plans that include abortive therapy, prophylactic therapy, and non-pharmacological modalities. Although there is limited data in adults, those with SCD are at risk for medication overuse headache secondary to frequent opioid use. Addressing headache in patients with SCD may help to reduce their use of opioids and disability and improve pain and quality of life.
PMID: 26879878 [PubMed – in process]
12. J Clin Apher. 2016 Feb 16. doi: 10.1002/jca.21447. [Epub ahead of print]
Safety, tolerability, and outcomes of regular automated red cell exchange transfusion in the management of sickle cell disease.
Tsitsikas DA1, Sirigireddy B1, Nzouakou R1, Calvey A1, Quinn J1, Collins J1, Orebayo F1, Lewis N1, Todd S1, Amos RJ1.
We report here our experience with regular automated red cell exchange transfusion for the management of chronic complications of sickle cell disease in 50 patients in our institution from June 2011 to December 2014. The mean sickle hemoglobin level was 44% and 8.5% pre- and post-transfusion, respectively. Platelets were reduced by a mean 70% during the procedure with a count of less than 50 × 109 /l in 6% of cases. The alloimmunization rate was 0.065/100 units of red cells with no hemolytic reactions. Patients with no iron overload at baseline showed no evidence of iron accumulation with a mean liver iron concentration of 1.6 mg/g dry tissue and 1.9 mg/g dry tissue at baseline and 36 months, respectively. All six patients with pre-existing iron overload and on chelation therapy, showed a gradual reduction of their liver iron concentration and two patients could discontinue chelation during the follow-up period. Seventy percentage of patients who were on the programme for recurrent painful crises showed a sustained reduction in the number of emergency hospital attendances; the mean number of days in hospital for emergency treatment was 103 in the year prior to commencing ARCET and reduced to 62 (40%) after the first 12 months, 51 (50%) after 24 months, and 35 days (66%) after 36 months. J. Clin. Apheresis, 2016. © 2016 Wiley Periodicals, Inc.
© 2016 Wiley Periodicals, Inc.
PMID: 26878828 [PubMed – as supplied by publisher]
13. J Wound Care. 2016 Feb;25 Suppl 2:S23-7. doi: 10.12968/jowc.2016.25.Sup2.S23.
Healing of chronic sickle cell disease-associated foot and ankle wounds using transdermal continuous oxygen therapy.
Massenburg BB1, Himel HN1.
Objective Sickle cell disease (SCD) is a complex cause of capillary stasis that can lead to lower extremity venous ulcers. Since SCD is characterised by impaired oxygen delivery that can be exacerbated by lower extremity venous stasis, we sought to determine if direct delivery of oxygen to a lower extremity ulcer associated with SCD could augment healing. Method We performed a pilot study of a portable device that delivers oxygen directly to the wound site to assess this possibility. The device was assessed in a single patient with three longstanding wounds of the lower extremities associated with SCD: the lateral right ankle, the medial aspect of the distal left leg, and the dorsal left foot. The left leg and left foot wounds received 15 weeks of treatment each. Results Both left lower extremity wounds healed, and neither wound has relapsed in the 42 months since the treatment was completed. At 36 months from the completion of treatment, the patient presented to the hospital with renal failure, and with the 27 days of complete bed rest and wound elevation, the right ankle wound shrunk to 55% of the original wound surface area. Transdermal oxygen appears to have assisted in healing and to have provided a sustained benefit that has delayed relapse of the two SCD-associated leg ulcers that received an extended course of treatment. Similarly, complete bed rest appears to aid in the healing of SCD-associated leg ulcers. Further study is needed to determine the mechanism of action and the optimal method of use of the transdermal continuous oxygen delivery device for SCD-associated lower extremity ulcers.
DECLARATION OF INTEREST:
No competing financial interests exist for any author. The content of this article was expressly written by the authors listed. No ghostwriters were used to write this article.
PMID: 26878371 [PubMed – in process]
14. J Pediatr Psychol. 2016 Feb 10. pii: jsw003. [Epub ahead of print]
Examining Biopsychosocial Factors in Relation to Multiple Pain Features in Pediatric Sickle Cell Disease.
Schlenz AM1, Schatz J2, Roberts CW3.
To examine biopsychosocial variables in relation to multiple pain features in pediatric sickle cell disease (SCD). METHODS: 76 children with SCD (M = 14.05, SD = 3.26), ages 8-19 years, and 70 caregivers completed measures of coping, mood, and family functioning and reported on multiple pain features via retrospective interviews during routine hematological visits. Sickle cell genotype and health care utilization were collected via medical record review. Using hierarchical regression, biological (genotype), child psychological (coping and mood), and social factors (caregiver coping and family functioning) were evaluated in relation to multiple pain features. RESULTS: Genotype was associated with pain intensity, and child psychological factors were associated with pain frequency. Multiple biopsychosocial factors were related to health care utilization. CONCLUSIONS: Biopsychosocial factors may have distinct relationships with pain features in pediatric SCD. Understanding these relationships may refine the biopsychosocial model and inform integrated medical and psychosocial approaches in SCD.
© The Author 2016. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: [log in to unmask]
PMID: 26869665 [PubMed – as supplied by publisher]
15. Cochrane Database Syst Rev. 2016 Feb 12;2:CD006111. [Epub ahead of print]
Piracetam for reducing the incidence of painful sickle cell disease crises.
Al Hajeri A1, Fedorowicz Z.
Sickle cell disease is one of the most common genetic disorders. Sickle cell crises in which irregular and dehydrated cells contribute to blocking of blood vessels are characterised by episodes of pain. Treatment is mainly supportive and symptomatic. In vitro studies with piracetam indicate that it has the potential for inhibition and a reversal of the process of sickling of erythrocytes. This is an update of a previously published Cochrane review.
To assess the effectiveness of piracetam for reducing the incidence of painful sickle cell disease crises.
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Last search of the Group’s Haemoglobinopathies Trials Register: 21 September 2015.
Randomised controlled trials comparing orally administered piracetam to placebo or standard care in people, of all ages and both sexes, with sickle cell disease.
DATA COLLECTION AND ANALYSIS:
Two authors independently assessed trial quality and extracted data. Trial authors were contacted for additional information. Adverse effects data were collected from the trials.
Three trials involving 169 participants were included in the review. A limited amount of data addressing some of the primary and some of the secondary outcomes were provided, but data were incomplete and based on un-validated assumptions used in the evaluation of outcomes. One trial reported a reduction in the number of pain crises and their severity with active intervention than placebo but presented no data to confirm these results. A second trial presented a monthly global pain score based on the number of sickle cell crises and severity of pain but included no separate data for these primary outcomes. Although there was no significant difference between the piracetam and placebo periods for the number of days of hospitalisation (P = 0.87) in one trial, inconsistencies in the criteria necessary for hospitalisation during sickle crises did not permit accurate conclusions to be drawn. Two of the trials reported participant satisfaction with piracetam but provided no details as to how this satisfaction had been assessed. There were no reports of toxicity or adverse effects with piracetam other than one participant who experienced dizziness.
The small number of included trials and their poor methodological quality provided insufficient reliable evidence to support the routine use of this medication for preventing the incidence of painful sickle cell disease crises.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: 26869149 [PubMed – as supplied by publisher]
16. World J Clin Pediatr. 2016 Feb 8;5(1):25-34. doi: 10.5409/wjcp.v5.i1.25. eCollection 2016.
Novel insights in the management of sickle cell disease in childhood.
Iughetti L1, Bigi E1, Venturelli D1.
Sickle cell disease (SCD) is a life-threatening genetic disorder characterized by chronic hemolytic anemia, vascular injury and multiorgan dysfunctions. Over the last few decades, there have been significant improvements in SCD management in Western countries, especially in pediatric population. An early onset of prophylaxis with Penicillin and a proper treatment of the infections have increased the overall survival in childhood. Nevertheless, management of painful episodes and prevention of organ damage are still challenging and more efforts are needed to better understand the mechanisms behind the development of chronic organ damages. Hydroxyurea (Hydroxycarbamide, HU), the only medication approved as a disease-modifying agent by the United States Food and Drug Administration and the European Medicines Agency, is usually under-used, especially in developing countries. Currently, hematopoietic stem-cell transplantation is considered the only curative option, although its use is limited by lack of donors and transplant-related toxicity. SCD symptoms are similar in children and adults, but complications and systemic organ damages increase with age, leading to early mortality worldwide. Experts in comprehensive care of young patients with SCD, especially those approaching the transition age to adulthood, are missing, leading people to rely on urgent care, increasing health care utilization costs and inappropriate treatments. It would be important to establish programs of comprehensive healthcare for patients with SCD from birth to adulthood, to improve their quality and expectancy of life.
PMCID: PMC4737690 Free PMC Article
PMID: 26862499 [PubMed]
17. Pediatr Blood Cancer. 2016 Feb 8. doi: 10.1002/pbc.25940. [Epub ahead of print]
Nutrient Insufficiencies/Deficiencies in Children With Sickle Cell Disease and Its Association With Increased Disease Severity.
Martyres DJ1, Vijenthira A2, Barrowman N3, Harris-Janz S4, Chretien C4, Klaassen RJ4.
Sickle cell disease (SCD) is characteristically described as a disease of hemolytic anemia and vaso-occlusive crises (VOCs). However, patients suffer from a multitude of other problems including impaired development, chronic pain, and increased susceptibility to infection. Nutritional deficiency has been implicated as a contributor to these issues.
We reported the nutrition status with respect to vitamin D, zinc, B6, B12, folate, and homocysteine serum levels in Canadian children with SCD (n = 91). We also tested for associations between nutrients and markers of disease severity and growth.
Almost half the sample (42%) had multiple nutrient insufficiencies/deficiencies, and a further 27% had a single insufficiency/deficiency. The most common insufficiency/deficiency was zinc in 57% followed by calcidiol (25 dihydroxyvitamin D (25(OH)D)) (52%). Sixteen percent of patients had low vitamin B6 levels, while folate, calcitriol (1,25(OH)D), and homocysteine levels were normal. Increased number of vitamin insufficiencies/deficiencies was associated with increasing disease severity (P = 0.018). Zinc insufficiency/deficiency was significantly associated with an increased number of home pain crises (P = 0.001) and an increased incidence of hospitalizations for VOCs (P = 0.01).
Our findings show that patients with SCD commonly have multiple nutrient insufficiencies/deficiencies and support the growing evidence for the link between low zinc and increased VOC. It also indicates that increased nutrient insufficiencies/deficiencies are associated with increased disease severity in SCD. Prospective studies with larger samples are needed to further elucidate the relationship between nutrient deficiencies and SCD, and to determine whether nutrient supplementation can improve the disease course.
© 2016 Wiley Periodicals, Inc.
PMID: 26855061 [PubMed – as supplied by publisher]
New article in Sociology, the journal of the British Sociological Association. The reference is: Dyson, SM; Berghs, M and Atkin, K (2016) “Talk to me. There’s two of us”: Fathers and sickle cell screening Sociology 50(1): 178-194. [ISSN: 0038-0385] http://dx.doi.org/10.1177/0038038514560261
Sickle Cell Conferences and Events
Hemoglobinopathy Counselor Training Course will be held on April 7-8, 2016. The two-day course, presented by the Cincinnati Comprehensive Sickle Cell Center, will be held at Cincinnati Children’s Hospital Medical Center. The course registration fee is $150. The deadline to register is March 24, 2016 and registration is limited. For more information, including a course brochure, please email: [log in to unmask] Registration is also available online at www.regonline.com/2016scdcounselorcourse
Event: Sickle Cell in Focus (SCiF) 2016, National Institutes of Health, Bethesda, Maryland, USA
Date: Thursday 2nd – Friday 3rd June 2016
Venue: Natcher Conference Centre, National Institutes of Health, Bethesda, MD 20894 USA
The 10th Sickle Cell in Focus conference returns to the USA in June 2016. Sickle Cell in Focus is an internationally renowned educational update for sickle cell disease. It attracts a wide audience of clinicians, academics and other healthcare professionals involved in the disease from around the world.
6th International African Symposium on Sickle Cell Disease July 11th – 15th, 2016 Accra, Ghana
Call for Abstracts
Abstract submissions will be accepted for the following categories:
Public Health and Health Education
Blood Transfusion Services
Newborn Screening and Follow Up
More information regarding submission guidelines and deadlines will be available shortly.
The Comprehensive Sickle Cell Center at The Children’s Hospital of Philadelphia, the Sickle Cell Foundation of Ghana, and other international partners have been organizing a series of international symposia aimed at improving knowledge and practices in the management of SCD in Africa.
The target audience for this symposium are healthcare workers, public health officials, laboratory technologists, community-based organizations, and support groups for patients and families.
1. Explore strategies for development and sustainment of Newborn Screening programs for SCD in Africa
2. Explore strategies for simplified, rapid, and accurate diagnostic tests for SCD in Africa
3. Explore strategies for development of hydroxyurea treatment programs for SCD in Africa
4. Explore strategies for improvements in blood supply, transfusion safety, and practices in the management of SCD in Africa
5. Discuss genetic counseling strategies and their social impact in Africa
6. Discuss guidelines for management of SCD in Africa