1.
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Hemoglobin. 2015 Jun 26:1-6. [Epub ahead of print]
nada.
Abstract
Chronic red blood cell (RBC) transfusion is employed for a wide range of sickle cell disease complications, ranging from primary and secondary stroke prophylaxis to prevention of painful vaso-occlusive episodes. Currently different methods are employed by centers for chronic transfusion that include simple, automated and partial manual RBC exchange transfusion. A retrospective cohort study of two different manual RBC exchange transfusion methods was conducted between two comprehensive care centers in Toronto, ON, Canada and London, United Kingdom in 19 and 21 sickle cell disease adults, respectively. London used a weight-based protocol, while Toronto used a unit-based method. Our results indicated that sickle cell disease patients utilizing a weight-based method are more often unable to achieve the prescribed Hb S (HBB: c.20A > T) target compared to the unit-based method (90.0 vs. 53.0% in the weight-based and unit-based methods, respectively, p = 0.0123). On multivariable logistic regression, none of the covariates examined was found to influence the ability to achieve the prescribed Hb S target after accounting for the exchange transfusion method. Mean interval of exchange sessions, session duration, total units of packed RBC, volume of blood used by body weight each year, the mean post exchange hematocrit [or packed cell volume (PCV)] and ferritin change were similar in both cohorts. In conclusion, the unit-based method was more effective at maintaining the prescribed Hb S target.
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PMID: 26114740 [PubMed - as supplied by publisher]
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2.
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Hemoglobin. 2015 Jun 26:1-5. [Epub ahead of print]
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Abstract
The quality of care for sickle cell disease patients hospitalized with a vaso-occlusive crisis (VOC) is poor, resulting in staggeringly high healthcare resource utilization. To evaluate in-patient care for VOC, we conducted a mixed-methods study of all adult sickle cell disease patients admitted with a VOC from 2010-2012. We quantitatively assessed the quality of care for all patients, and qualitatively studied a subset of frequently admitted patients. In total, there were 182 admissions from 57 unique patients. The median length of stay was 6 days and the 30-day readmission rate was 34.0%. We identified red blood cell transfusion and patient controlled analgesia use as predictors of increased length of stay. Interestingly, unlike prior findings, younger patients (18-30 years old) did not have increased healthcare resource utilization. Moreover, older age appeared to increase readmission rate and enhance the effect of patient controlled analgesia use on length of stay. Interviews of high healthcare resource utilizers revealed significant deficiencies in pain management and a strong desire for individualized care. This is the first study to examine in-patient predictors of acute healthcare resource utilization in sickle cell disease patients and to correlate them with qualitative perspectives of high healthcare resource utilizers.
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PMID: 26114739 [PubMed - as supplied by publisher]
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3.
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Br J Haematol. 2015 Jun 24. doi: 10.1111/bjh.13570. [Epub ahead of print]
Abstract
HbA2 , a tetramer of α- and δ-globin chains, provides a diagnostic clue to the presence of β-thalassaemia trait. This minor haemoglobin, which forms about 2-3% of the total, has no known physiological role, but has the interesting property of preventing polymerization of deoxy-sickle haemoglobin. If it were possible to increase the level of HbA2 sufficiently it could have a benefit in sickle cell disease similar to that of foetal haemoglobin. Moreover, HbA2 is present in all erythrocytes, an advantage not found with foetal haemoglobin, which is heterocellularly expressed. The molecular basis of HbA2 gene (HBD) expression is partially understood, and with new molecular tools, it might be possible to induce levels of HbA2 that could be clinically important. However, high concentrations of this positively charged haemoglobin might damage the erythrocyte membrane; also, the reciprocal relationship of δ- and γ-globin gene (HBD and HBG1/2, respectively) expression might negate any benefit of increasing transcription of the former.
© 2015 John Wiley & Sons Ltd.
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PMID: 26104837 [PubMed - as supplied by publisher]
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4.
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Clin Trials. 2015 Jun 17. pii: 1740774515590811. [Epub ahead of print]
Abstract
BACKGROUND/AIMS:
The registry ClinicalTrials.gov was created to provide investigators and patients an accessible database of relevant clinical trials.
METHODS:
To understand the state of sickle cell disease clinical trials, a comprehensive review of all 174 "closed," "interventional" sickle cell trials registered at ClinicalTrials.gov was completed in January 2015.
RESULTS:
The majority of registered sickle cell disease clinical trials listed an academic center as the primary sponsor and were an early phase trial. The primary outcome for sickle cell disease trials focused on pain (23%), bone marrow transplant (BMT) (13%), hydroxyurea (8%), iron overload (8%), and pulmonary hypertension (8%). A total of 52 trials were listed as terminated or withdrawn, including 25 (14% of all trials) terminated for failure to enroll participants. At the time of this review, only 19 trials uploaded results and 29 trials uploaded a manuscript in the ClinicalTrials.gov database. A systematic review of pubmed.gov revealed that only 35% of sickle cell studies completed prior to 2014 resulted in an identified manuscript. In comparison, of 80 thalassemia trials registered in ClinicalTrials.gov, four acknowledged failure to enroll participants as a reason for trial termination or withdrawal, and 48 trials (60%) completed prior to 2014 resulted in a currently identified manuscript.
CONCLUSION:
ClinicalTrials.gov can be an important database for investigators and patients with sickle cell disease to understand the current available research trials. To enhance the validity of the website, investigators must update their trial results and upload trial manuscripts into the database. This study, for the first time, quantifies outcomes of sickle cell disease trials and provides support to the belief that barriers exist to successful completion, publication, and dissemination of sickle cell trial results.
© The Author(s) 2015.
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PMID: 26085544 [PubMed - as supplied by publisher]
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5.
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J Health Psychol. 2015 Jun 15. pii: 1359105315588215. [Epub ahead of print]
Author information:
2Brigham and Women's Hospital, USA.
3Yale School of Medicine, USA.
Abstract
This study investigated coping with chronic illness in the adult patient-caregiver relationship for sickle cell disease, marked by debilitating acute and chronic pain. One-on-one interviews (N = 16) were conducted with eight primary caregivers of eight adults with extremely high hospital use, severe sickle cell disease with hospital admissions several times monthly over successive years. Caregivers were predominantly parents; two were romantic partners. Caregivers attributed disruptions to the disease's variability, tensions in how much support to give, and adults' inability to fulfill parental obligations. Both groups expressed fears of patients' increasing age, declining health, and early death. Targeted counseling and resilience training is recommended.
© The Author(s) 2015.
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PMID: 26078296 [PubMed - as supplied by publisher]
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6.
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Br J Haematol. 2015 Jun 5. doi: 10.1111/bjh.13520. [Epub ahead of print]
Abstract
Ex-vivo gene transfer of autologous haematopoietic stem cells in patients with monogenic diseases of the bone marrow has emerged as a new therapeutic approach, mainly in patients lacking a suitable donor for transplant. The encouraging results of initial clinical trials of gene therapy for primary immunodeficiencies were tempered by the occurrence of genotoxicity in a number of patients. Over the last decade, safer viral vectors have been developed to overcome the risk of insertional mutagenesis and have led to impressive clinical outcomes with considerably improved safety. We review the efforts in specific immunodeficiencies including adenosine deaminase deficiency, X-linked severe combined immunodeficiency, chronic granulomatous disease and Wiskott Aldrich syndrome. Major recent progress has also been made in haemoglobinopathies, such as beta-thalassaemia, sickle cell disease and Fanconi anaemia, and also specific lysosomal storage diseases, which, although not strictly bone marrow specific conditions, have been effectively treated by bone marrow-based treatment. The success of these recent studies and the advent of new technologies, such as gene editing, suggest that gene therapy could become a more generally applied treatment modality for a number of haematopoietic disorders.
© 2015 John Wiley & Sons Ltd.
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PMID: 26044877 [PubMed - as supplied by publisher]
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7.
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Stem Cell Res Ther. 2015 May 29;6:110. doi: 10.1186/s13287-015-0105-2.
Abstract
INTRODUCTION:
Stem cell therapy with bone marrow-derived mononuclear cells (BMMCs) is an option for improving joint function in osteonecrosis of the femoral head (ONFH). Bone marrow-derived mesenchymal stromal cell (MSC) numbers and their osteogenic differentiation are decreased in patients with ONFH. However, whether this decrease also extends to the early stages of ONFH in sickle cell disease (SCD) is still unclear.
METHODS:
We conducted a phase I/II, non-controlled study to determine efficacy and safety of BMMC implantation using a minimally invasive technique in SCD patients with ONFH. Eighty-nine patients were recruited and followed up for 60 months after surgery. Clinical and radiographic findings were assessed, and data were completed by in vitro analysis.
RESULTS:
At the final follow-up (60 months) there was a significant improvement in clinical joint symptoms and pain relief as measured by the Harris Hip Score (P = 0.0005). In addition, after the BMMC implantation procedure, radiographic assessment showed disease stabilization and only 3.7 % of the treated patients did not achieve a satisfactory clinical result. The amount of fibroblast colony-forming units was 28.2 ± 13.9 per 1 million BMMCs after concentration. Flow cytometry analysis showed a significantly higher number of hematopoietic stem/endothelial progenitor cell markers in concentrated BMMCs when compared with bone marrow aspirate, indicating an enrichment of these cell types. Isolated MSCs from SCD patients with pre-collapse ONFH maintained the replicative capacity without significant loss of their specific biomolecular characteristics, multi-differentiation potential, and osteogenic differentiation activities. Cytokines and growth factors (interleukin-8, transforming growth factor-beta, stromal cell-derived factor-1alpha and vascular endothelial growth factor) that mediate endogenous bone regeneration were also produced by expanded MSCs from SCD patients.
CONCLUSION:
The autologous BMMC implantation with a minimally invasive technique resulted in significant pain relief and halted the progression of early stages of ONFH in SCD patients. MSCs from SCD patients display biological properties that may add to the efficiency of surgical treatment in ONFH. In summary, our results indicate that infusion of BMMCs enriched with stem/progenitor cells is a safe and effective treatment for the early stages of ONFH in SCD patients.
TRIAL REGISTRATION:
PMCID: PMC4465459 Free PMC Article
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PMID: 26021713 [PubMed - in process]
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8.
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Br J Haematol. 2015 May 27. doi: 10.1111/bjh.13526. [Epub ahead of print]
Abstract
Sickle cell disease (SCD) affects approximately 100 000 people in the US, 12 500 in the UK, and millions worldwide. SCD is typified by painful vaso-occlusive episodes, haemolytic anaemia and organ damage. A secondary complication is infection, which can be bacterial, fungal or viral. Universal newborn screening, routine use of penicillin prophylaxis, availability of conjugated vaccines against S. pneumoniae and comprehensive care programmes instituted during the past few decades in industrialized countries have dramatically reduced childhood mortality and improved life expectancy. Yet patients with SCD remain at increased risk of infection. Unfortunately, the treatment of most bacterial infections that are common in SCD is not based on the results of randomized controlled clinical trials. In their absence, treatment decisions are based on consensus guidelines, clinical experience or adapting treatment applied in other diseases. This leads to wide variation in treatment among institutions and even between treating physicians in a single institution. Prevention of infection, when possible, is most important and we focus on prevention through targeted prophylaxis and vaccination. We will share our management strategies for managing the more common infections in SCD and provide the rationale for our recommendations.
© 2015 John Wiley & Sons Ltd.
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PMID: 26018640 [PubMed - as supplied by publisher]
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9.
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Pain Pract. 2015 May 26. doi: 10.1111/papr.12313. [Epub ahead of print]
Abstract
BACKGROUND:
Sickle cell disease (SCD) is a worldwide distributed hereditary red cell disorder. The principal clinical manifestations of SCD are the chronic hemolytic anemia and the acute vaso-occlusive crisis (VOCs), which are mainly characterized by ischemic/reperfusion tissue injury. Pain is the main symptom of VOCs, and its management is still a challenge for hematologists, requiring a multidisciplinary approach.
METHODS:
We carried out a crossover study on adult SCD patients, who received two different types of multimodal analgesia during two separate severe VOCs with time interval between VOCs of at least 6 months. The first VOC episode was treated with ketorolac (0.86 mg/kg/day) and tramadol (7.2 mg/kg/day) (TK treatment). In the second VOC episode, fentanyl buccal tablet (FBT; 100 μg) was introduced in a single dose after three hours from the beginning of TK analgesia (TKF treatment). We focused on the first 24 hours of acute pain management. The primary efficacy measure was the time-weighted-sum of pain intensity differences (SPID24). The secondary efficacy measures included the pain intensity difference (PID), the total pain relief (TOTPAR), and the time-wighted sum of anxiety (SAID24).
RESULTS:
SPID24 was significantly higher in TKF than in TK treatment. All the secondary measures were significantly ameliorated in TKF compared to TK treatment, without major opioid side effects. Patients satisfaction was higher with TKF treatment than with TK one.
CONCLUSIONS:
We propose that VOCs might require breakthrough pain drug strategy as vaso-occlusive phenomena and enhanced vasoconstriction promoting acute ischemic pain component exacerbate the continuous pain of VOCs. FBT might be a powerful and feasible tool in early management of acute pain during VOCs in emergency departments.
© 2015 World Institute of Pain.
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PMID: 26009799 [PubMed - as supplied by publisher]
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10.
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Emerg Med J. 2015 Jun;32(6):e14. doi: 10.1136/emermed-2015-204980.7.
Abstract
INTRODUCTION:
Some ambulance services in the UK use the Medical Priority Dispatch System (MPDS) to triage 999 calls and determine the response allocated to patients. When MPDS was developed in the 1970s Sickle Cell Disease (SCD) was not common in the UK and was therefore not included in the system. As ethnic diversity in the UK increased so did the prevalence of SCD, yet ambulance services were still unable to identify this group of patients and assess their specific needs. Patients with SCD usually contact the ambulance service when they experience a Sickle Cell Crisis (SCC), which is characterised by severe pain. It is important ambulances arrive quickly to allow patients' pain to be managed in a timely manner. However, not having a way to identify these patients at the time of the 999 call means that patients potentially receives variable responses.
METHOD:
To determine the level of care provided, a retrospective clinical audit was undertaken for patients who reported SCC. A questionnaire was sent to patients with SCD which was used to inform the development of an override to MPDS allowing Emergency Medical Dispatchers to allocate a faster response to patients reporting SCC at the time of the 999 call. Following the introduction of the over-ride, a re-audit was conducted.
RESULTS:
The re-audit showed the majority of patients (62%) received an equal or a faster response in line with the updated MPDS protocol. This increase resulted in reduced waiting time for patients; from 11 minutes and 20 seconds to 9 minutes, an average of 2 minutes and 20 seconds faster than before the over-ride.
CONCLUSION:
The over-ride resulted in a quicker ambulance response which reduced the chances of the patient's condition worsening before the arrival of an ambulance. This project also provided evidence and allowed for the SCC override to be adopted nationally.
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PMID: 25991787 [PubMed - in process]
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11.
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J Thromb Thrombolysis. 2015 Aug;40(2):182-5. doi: 10.1007/s11239-015-1230-6.
Author information:
Abstract
Sickle cell disease (SCD) is associated with a significant hypercoagulable state and several hemostatic anomalies have been identified in this disease state. Of interest, SCD patients can become iron overloaded after transfusion, and iron can enhance fibrinogen as a substrate for thrombin, resulting in thrombi that commence coagulation quickly and form rapidly. We hypothesized that SCD patients would display hypercoagulable plasma coagulation kinetics and an iron enhancement of coagulation. After obtaining IRB approval, we assessed coagulation kinetics and iron enhancement with viscoelastic methods in archived, citrated plasma obtained from ambulatory or hospitalized SCD patients (n = 20). All SCD patients had plasmatic hypercoagulability, and 65 % were positive for iron enhancement of coagulation. In conclusion, continuing investigation correlating such viscoelastic data with clinical symptoms may provide insight into the role played by iron in the setting of SCD, including complications such as vaso-occlusive crisis.
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PMID: 25986992 [PubMed - in process]
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12.
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J Opioid Manag. 2015 May-Jun;11(3):243-53. doi: 10.5055/jom.2015.0273.
Abstract
BACKGROUND:
Although opioid prescribing in sickle cell disease (SCD) can be controversial, little is published about patterns of opioid use.
OBJECTIVE:
To report on home opioid use among adults with SCD.
DESIGN:
Cohort study.
PARTICIPANTS:
Adults with SCD (n = 219) who completed daily pain diaries for up to 6 months and had at least one home pain day.
MAIN MEASURES:
Use of long-acting or short-acting opioids, other analgesics, or adjuvants; the proportion of home days, home pain days, and home crisis days with opioid use; these two outcomes according to patient characteristics.
KEY RESULTS:
Patients used opioids on 12,311 (78 percent) of 15,778 home pain days. Eighty-five patients (38.8 percent) used long-acting opioids with or without short-acting opioids and 103 (47.0 percent) used only short-acting opioids. Twenty-one (9.6 percent) patients used only non-opioid analgesics and 10 (4.6 percent) used no analgesics. Both pain intensity and pain frequency were higher among opioid users (analysis of variance [ANOVA], p < 0.0001). Opioid users used hydroxyurea more often than nonusers, even when controlling for mean pain on pain days. Among all patients, significant relationships were found between any opioid use and somatic symptom burden, SCD stress, negative coping, and physical and mental quality of life (QOL); the relationship with SCD stress and physical QOL remained when controlled for mean pain. Among opioid users, similar associations were found between frequency of opioid use and some disease-related and psychosocial variables.
CONCLUSIONS:
In this adult SCD sample, opioids were used by the majority of patients. Pain was the overwhelming characteristic associated with use, but disease-related and psychosocial variables were also associated.
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PMID: 25985809 [PubMed - in process]
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13.
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J Pediatr Hematol Oncol. 2015 Jul;37(5):e308-15. doi: 10.1097/MPH.0000000000000355.
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Abstract
Suboptimal vitamin D (vit D) status (<32 ng/mL) is ubiquitous among African American children with type SS sickle cell disease (SCD-SS). The vit D supplemental dose to normalize vit D status is unknown. Five to 20-year-old African American children with (n=21) and without (n=23) SCD-SS were randomized to vit D3 supplementation (4000 or 7000 IU/d) and evaluated at 6 and 12 weeks for changes in vit D and SCD status. A dose was considered unsafe if serum calcium was elevated associated with elevated serum 25 hydroxyvitamin D (25(OH)D). At baseline 95% of subjects with SCD-SS and 87% of healthy controls had suboptimal vit D status (mean±SD, 19.2±7.2 and 22.3±9.3 ng/mL, respectively). After 12 weeks supplementation, both D3 doses were safe and well tolerated. Neither group achieved the a priori efficacy criterion of 25(OH)D≥32 ng/mL in >80% of subjects (45% in SCD-SS and 63% in controls). However, for both subjects with SCD-SS and healthy subjects by 12 weeks, deficient (<20 ng/mL) vit D status was eliminated only in those receiving 7000 IU/d. For subjects with SCD-SS, by 12 weeks there was a significant (all P<0.05) increase in fetal hemoglobin, decrease in high-sensitivity C-reactive protein, and reduction in the percentage of subjects with a high platelet count.
PMCID: PMC4474739 [Available on 2016-07-01]
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PMID: 25985241 [PubMed - in process]
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14.
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Semin Perinatol. 2015 Apr;39(3):238-251. doi: 10.1053/j.semperi.2015.03.008.
Abstract
Sickle cell disease is a group of disorders, the majority of which are detected through state newborn screening programs. There is limited knowledge of disease prevalence in the U.S.
POPULATION:
We report 20 years of case finding and laboratory data for sickle cell disease and trait to assist in: planning for health services delivery; providing data for researchers; aiding in tracking health outcome trends; and assessing sickle gene prevalence in the newborn population. During the 20-year period, there were 39,422 confirmed cases of sickle cell disease among 76,527,627 newborn births screened (1:1941) and 1,107,875 laboratory reports of probable sickle trait among 73,951,175 newborn births screened (1:67). The highest sickle cell disease incidence during the 20 years was in the District of Columbia (1:437) followed by Mississippi (1:683) and South Carolina (1:771). For sickle cell trait, the highest incidences were in the District of Columbia (1:22), Mississippi (1:26), and South Carolina (1:31).
Copyright © 2015 Elsevier Inc. All rights reserved.
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PMID: 25979783 [PubMed - as supplied by publisher]
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15.
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Blood Cells Mol Dis. 2015 Jun;55(1):48-55. doi: 10.1016/j.bcmd.2015.03.014. Epub 2015 Mar 31.
Abstract
Chronic inflammation and reduced blood levels of omega-3 fatty acids (n-3) are known characteristics of sickle cell disease (SCD).The anti-inflammatory properties of n-3 fatty acids are well recognized. Omega-3 treated (n=24), hydroxyurea (HU) treated (n=18), and n-3 untreated (n=21) homozygous SCD patients (HbSS) and healthy (HbAA) controls (n=25) matched for age (5-16years), gender and socioeconomic status were studied. According to age (5-10) or (11-16) years, two or three capsules containing 277.8mg docosahexaenoic (DHA) and 39.0mg eicosapentaenoic (EPA) or high oleic acid placebo (41%) were assigned to n-3 treated and n-3 untreated groups, respectively. Hydroxyurea treated group was on dosage more than 20mg/kg/day. The effect of supplementation on systemic and blood cell markers of inflammation was investigated. The n-3 treated group had higher levels of DHA and EPA (p<0.001) and lower white blood cell count and monocyte integrin (p<0.05) compared with the n-3 untreated. No difference was detected between the two groups regarding C-reactive protein, granulocytes integrin and selectin, plasma tumour necrosis factor-α and interleukin-10. The n-3 treated group had lowered nuclear factor-kappa B (NF-κB) gene expression compared to n-3 untreated and HU treated groups (p<0.05). This study provides evidence that supplementation with n-3 fatty acids may ameliorate inflammation and blood cell adhesion in patients with SCD.
Copyright © 2015 Elsevier Inc. All rights reserved.
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PMID: 25976467 [PubMed - in process]
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16.
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Pediatr Blood Cancer. 2015 May 13. doi: 10.1002/pbc.25574. [Epub ahead of print]
Author information:
Abstract
Novel insights into the neurobiology of sickle cell disease (SCD) pain have recently been discovered. We systematically reviewed the literature focusing on original research that examined the biology of pain in SCD and/or addressed assessment or treatment of neuropathic pain in SCD. This review of 15 articles that met inclusion criteria provides epidemiological, basic, and clinical data that support central and/or peripheral nervous system abnormalities likely contribute to sickle cell pain. Continued basic and clinical investigation into pain neurobiology is imperative to translate these discoveries into novel ways to assess and treat neuropathic pain and decrease patient suffering. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc.
© 2015 Wiley Periodicals, Inc.
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PMID: 25976161 [PubMed - as supplied by publisher]
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17.
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Stroke. 2015 Jul;46(7):1793-9. doi: 10.1161/STROKEAHA.115.008721. Epub 2015 May 12.
Kawadler JM1, Kirkham FJ2, Clayden JD1, Hollocks MJ1, Seymour EL1, Edey R1, Telfer P1, Robins A1, Wilkey O1, Barker S1, Cox TC1, Clark CA1.
Author information:
Abstract
BACKGROUND AND PURPOSE:
Sickle cell anemia is associated with compromised oxygen-carrying capability of hemoglobin and a high incidence of overt and silent stroke. However, in children with no evidence of cerebral infarction, there are changes in brain morphometry relative to healthy controls, which may be related to chronic anemia and oxygen desaturation.
METHODS:
A whole-brain tract-based spatial statistics analysis was carried out in 25 children with sickle cell anemia with no evidence of abnormality on T2-weighted magnetic resonance imaging (13 male, age range: 8-18 years) and 14 age- and race-matched controls (7 male, age range: 10-19 years) to determine the extent of white matter injury. The hypotheses that white matter damage is related to daytime peripheral oxygen saturation and steady-state hemoglobin were tested.
RESULTS:
Fractional anisotropy was found to be significantly lower in patients in the subcortical white matter (corticospinal tract and cerebellum), whereas mean diffusivity and radial diffusivity were higher in patients in widespread areas. There was a significant negative relationship between radial diffusivity and oxygen saturation (P<0.05) in the anterior corpus callosum and a trend-level negative relationship between radial diffusivity and hemoglobin (P<0.1) in the midbody of the corpus callosum.
CONCLUSIONS:
These data show widespread white matter abnormalities in a sample of asymptomatic children with sickle cell anemia, and provides for the first time direct evidence of a relationship between brain microstructure and markers of disease severity (eg, peripheral oxygen saturation and steady-state hemoglobin). This study suggests that diffusion tensor imaging metrics may serve as a biomarker for future trials of reducing hypoxic exposure.
© 2015 American Heart Association, Inc.
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PMID: 25967572 [PubMed - in process]
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18.
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Cochrane Database Syst Rev. 2015 May 8;5:CD001916. [Epub ahead of print]
Author information:
1Haematology and Sickle Cell Centre, London North West Healthcare NHS Trust, Central Middlesex Hospital, Acton Lane, London, UK, NW10 7NS.
Abstract
BACKGROUND:
Sickle cell disease comprises a group of genetic blood disorders. It occurs when the sickle haemoglobin gene is inherited from both parents. The effects of the condition are: varying degrees of anaemia which, if severe, can reduce mobility; a tendency for small blood capillaries to become blocked causing pain in muscle and bone commonly known as 'crises'; damage to major organs such as the spleen, liver, kidneys, and lungs; and increased vulnerability to severe infections. There are both medical and non-medical complications, and treatment is usually symptomatic and palliative in nature. Psychological interventions for individuals with sickle cell disease might complement current medical treatment, and studies of their efficacy have yielded encouraging results. This is an update of a previously published Cochrane Review.
OBJECTIVES:
To examine the evidence that psychological interventions improve the ability of people with sickle cell disease to cope with their condition.
SEARCH METHODS:
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, which comprises references identified from comprehensive electronic database searches and the Internet, handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 17 February 2015.
SELECTION CRITERIA:
All randomised or quasi-randomised controlled trials comparing psychological interventions with no (psychological) intervention in people with sickle cell disease.
DATA COLLECTION AND ANALYSIS:
Both authors independently extracted data and assessed the risk of bias of the included studies.
MAIN RESULTS:
Twelve studies were identified in the searches and seven of these were eligible for inclusion in the review. Five studies, involving 260 participants, provided data for analysis. One study showed that cognitive behaviour therapy significantly reduced the affective component of pain (feelings about pain), mean difference -0.99 (95% confidence interval -1.62 to -0.36), but not the sensory component (pain intensity), mean difference 0.00 (95% confidence interval -9.39 to 9.39). One study of family psycho-education was not associated with a reduction in depression. Another study evaluating cognitive behavioural therapy had inconclusive results for the assessment of coping strategies, and showed no difference between groups assessed on health service utilisation. In addition, family home-based cognitive behavioural therapy did not show any difference compared to disease education. One study of patient education on health beliefs showed a significant improvement in attitudes towards health workers, mean difference -4.39 (95% CI -6.45 to -2.33) and medication, mean difference -1.74 (95% CI -2.98 to -0.50). Nonetheless, these results may not apply across all ages, severity of sickle cell disease, types of pain (acute or chronic), or setting.
AUTHORS' CONCLUSIONS:
Evidence for the efficacy of psychological therapies in sickle cell disease is currently limited. This systematic review has clearly identified the need for well-designed, adequately-powered, multicentre randomised controlled trials assessing the effectiveness of specific interventions in sickle cell disease.
Mtatiro, S. N., J. Mgaya, T. Singh, H. Mariki, H. Rooks, D. Soka, B. Mmbando, S. L. Thein, J. C. Barrett, J. Makani, S. E. Cox and S. Menzel (2015). "Genetic association of fetal-hemoglobin levels in individuals with sickle cell disease in Tanzania maps to conserved regulatory elements within the MYB core enhancer." BMC Med Genet 16(1): 4.
Wonkam, A., J. Makani, S. Ofori-Aquah, O. E. Nnodu, M. Treadwell, C. Royal, K. Ohene-Frempong and H. A. C. as members of the (2015). "Sickle cell disease and H3Africa: enhancing genomic research on cardiovascular diseases in African patients." Cardiovasc J Afr 26(2 Suppl 1): S50-55.
Makani, J., J. Mgaya, E. Balandya, K. Msami, D. Soka, S. E. Cox, A. N. Komba, S. Rwezaula, E. Meda, D. Muturi, J. Kitundu, G. Fegan, F. J. Kirkham, C. R. Newton, R. W. Snow and B. Lowe (2015). "Bacteraemia in sickle cell anaemia is associated with low haemoglobin: a report of 890 admissions to a tertiary hospital in Tanzania." Br J Haematol. http://www.ncbi.nlm.nih.gov/pubmed/26084722
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