of experts discussing the new guidelines. The potential harms associated with regular blood transfusion therapy have been quantified in controlled clinical trials for primary and secondary prevention strokes in children with HbSS or HbS0 thalassemia. The panels strong recommendation for screening for developmental delay and cognitive impairment in children with SCD was based on 3 factors. The approach is described in detail in a previous article describing the methods of development.30. The quality of evidence on the benefits of cognitive rehabilitation specifically in individuals with SCD was low. Given the increased overall survival of adults with SCD into middle and old age with the cumulative effect of traditional cardiovascular risk factors leading to stroke, offering emergent treatment with tPA to older adults with SCD presenting with acute ischemic strokes within 4.5 hours of symptom onset is advised. Clipboard, Search History, and several other advanced features are temporarily unavailable. Draft recommendations were reviewed by all members of the panel, revised, and then made available online on 24 September 2018, or for external review by stakeholders including allied organizations, other medical professionals, patients, and the public. jointly chaired the panel, with content knowledge of guidelines and systematic review expertise; and M.R.D. Better documentation of the prevalence and progression of cognitive impairments in adults with SCD is needed. Some patients will be difficult to transfuse effectively to keep the HbS <30% on a consistent basis.67,68 If the HbS cannot be kept consistently <30% with either simple transfusion, manual exchange transfusion, or automated exchange transfusion, higher HbS-level thresholds of 35% to 40% are acceptable provided that the patient is consistently transfused at 3- to 4-week intervals. The panelists received no other payments. The secondary stroke incidence of 7 events per 100 person-years in the hydroxyurea group was significantly lower than the 28 per 100 person-years in the nontreatment group (P = .001; odds ratio [OR], 3.80; 95% CI, 1.55-9.31).96. In the general population and in children and adults with SCD, identification of children and adults with developmental or cognitive delays increases likelihood of access to remediation services. In several health care settings, the potential for reimbursement is increasing; for example, the American Medical Association Current Procedural Panel has developed a new billing code (96127) for behavioral health screening that can be used to screen for cognitive impairment.117 Physicians, nonpsychologist staff, and practitioners have also seen a dramatic increase in cognitive screenings conducted, indicating that surveillance and screening are possible in settings without ready access to specialists in psychological assessment.118 Both the American Psychological Association and the National Academy of Neuropsychology have acknowledged that in many health care settings there is a need for medical doctors or nonpsychologist staff or practitioners to provide wider access to cognitive or behavioral health screenings, and these organizations have provided clarification on the distinction between screening and a more comprehensive psychological assessment requiring a specialist.119,120, The specific tests for cognitive screening in children and adults with SCD, and the optimal strategies to screen and implement rehabilitation for cognitive impairments, have not been established. How many people have sickle cell disease in the UK? On occasion, a strong recommendation is based on low or very low certainty in the evidence. These services are provided to enhance the development of the child. Associate Professor of Pediatrics Director, Comprehensive Sickle Cell Center Children's Hospital Medical Center 3333 Burnett Avenue, OSB 4 Unfortunately, those around the affected individuals may label such impairments as problem behaviors or lack of motivation. The management of acute and chronic pain for individuals living with sickle cell disease (SCD) is a clinical challenge. Rather, a commitment to time with occupational therapists is required for patients to receive appropriate services. Therefore, a low certainty in benefit but high certainty of harm (particularly from other populations) justified a strong recommendation.141 When considering patient values and preferences, the panel members, including the 2 patient representatives, reported values with little variation in favor of screening. Unauthorized use of these marks is strictly prohibited. The review identified 48 studies that evaluated cognitive screening without a comparison group and reported outcomes of IQ score, the prevalence of cognitive impairment, developmental delay, or school performance. Furthermore, in this cohort, there was progressive vasculopathy on MRA imaging, with recurrent overt or silent cerebral infarct (RR, 12.7; 95% CI, 2.65-60.5; P = .01). The clinical experience of the panel, coupled with evidence from the literature, indicates that regular blood transfusion therapy is not a feasible option for the majority of children living in Africa or other low-middleincome settings. Despite endorsement from multiple different professional societies, implementing cognitive-screening strategies in high-risk children and adults with SCD will be challenging to implement without basic research strategies to identify barriers and facilitators within SCD care centers. Cognitive impairment in SCD characteristically affect specific mental processes such as memory, attention, executive function, processing speed, and visual-spatial function. The EtD table addressed effects of interventions, resource utilization (cost-effectiveness), values and preferences (relative importance of outcomes), equity, acceptability, and feasibility. Professional illustration by Patrick Lane, ScEYEnce Studios. The quality of the studies does not allow for a pooled analysis nor an evidence-based recommendation-specific procedure with clear benefit, hence the reason for prospective standardized protocols and long-term outcome tracking. However, in SCD, the principles of management of acute ischemic strokes and TIAs, coupled with observational studies, provide evidence for best practices. For children with HbSC, the risk of abnormal TCD measurements and stroke is less than for those with HbSS.46. SCD is a rare disease, with few guidelines developed for prevention, screening, and treatment of CNS manifestations in children and adults with SCD. Outcomes prioritized by the ASH Guideline Panel on Cerebrovascular Disease, Researchers at the Mayo Clinic Evidence-Based Practice Center conducted new systematic reviews or updated existing systematic reviews to answer the main 10 PICO questions. For children (ages 2-16 years) with HbSS or HbS0 thalassemia or compound heterozygous SCD, who have abnormal TCD screening, and live in low-middle-income settings (where regular blood transfusion therapy and chelation therapy are not available or affordable), the ASH guideline panel suggests hydroxyurea therapy with at least 20 mg/kg per day at a fixed dose or the maximum tolerated dose (conditional recommendation based on low certainty in the evidence about effects ). Generally, red blood cell exchange with apheresis is not recommended for patients with SCD and hemoglobin levels <5.0 g/dL, in part because of concerns of lowering the hemoglobin level during priming of the apheresis machine. Part E summarizes ASH decisions about which interests were judged to be conflicts and how they were managed, including through recusal. The review identified 1 randomized controlled trial that compared academic tutoring with memory rehabilitation vs academic tutoring, 1 observational study that compared academic tutoring and specific learning and memory strategies with academic tutoring, and 1 observational study that compared computer-based cognitive training completers with noncompleters. The potential harms associated with regular blood transfusion therapy have been quantified in controlled clinical trials for primary and secondary prevention strokes in children with HbSS or HbS0 thalassemia. The primary premise for our PICO #3 recommendation (see Table 1) is based on the observation that 95% of children with HbSS live in low-middleincome settings,7 coupled with the urgency to prevent strokes in children, in the absence of a randomized controlled trial conducted in low-middleincome settings. Sickle Cell Disease: A Review of Nonpharmacological Approaches for Pain. If feasible, screening for silent cerebral infarcts in children and adults with HbSS in a low-middleincome country should be done for the same reason that the screening occurs in high-income settings. For researchers: The recommendation is supported by credible research or other convincing judgments that make additional research unlikely to alter the recommendation. The most common clinical manifestation of SCD is vaso-occlusive crisis. There are 5 independent reasons to justify screening for silent cerebral infarct in children and adults with HbSS or HbS0 thalassemia: Silent cerebral infarcts are prevalent; 39% of children3 and 50% of young adults will have a silent cerebral infarct.4. The panel did not include the role of HSCT for primary and secondary stroke prevention, an emerging treatment strategy in high-income settings. Although most studies show that opioids provide short-term relief, they are associated with side effects including constipation, opioid-induced hyperalgesia, and risk of . Treatment with regular blood transfusion commonly requires iron chelation therapy to attenuate excessive stores of iron that accumulate with blood transfusion. Several studies have compared the incidence of strokes before and after revascularization surgery; however, such studies are intrinsically limited. The rate of clinical adverse events during the pretreatment phase (2 months) was compared with the treatment phase (mean, 29 months). Alternative options for primary stroke prevention other than initial regular blood transfusion therapy for a year for some, then followed by maximum tolerated dose of hydroxyurea therapy, should be identified for children living in high-income settings. After the hemoglobin has reached 10.0 g/dL with simple transfusion or if the baseline hemoglobin level is greater than 8.5 g/dL, the panel recommends an automated-exchange red blood cell transfusion (apheresis).79 This procedure will require a timely dialogue, initiated soon after presentation to the medical facility, between the transfusion service, the hematologist, and intensivist to ensure a multidisciplinary approach to management. Statements about the underlying values and preferences as well as qualifying remarks accompanying each recommendation are its integral parts and serve to facilitate more accurate interpretation. TCD screening coupled with regular blood transfusion therapy for those with an abnormal TAMMV TCD measurement is associated with a 92% reduction in stroke incidence compared with observation alone.38 The threshold for regular blood transfusion therapy is 2 nonimaging TCD measurements 200 cm/s or a single TAMMV measurement >220 cm/s in the proximal portion of the middle cerebral artery or the distal portion of the internal carotid artery (Figure 1 in supplemental File 5). For children who have compound heterozygous SCD other than HbSC, who have evidence of hemolysis in the same range as those with HbSS, an abnormal TCD velocity, and live in a high-income setting (where regular blood transfusion therapy is feasible), the ASH guideline panel suggests regular blood transfusion for at least a year (vs no transfusion) with the goal of keeping maximum HbS levels below 30% to reduce the risk of stroke (conditional recommendation based on very low certainty in the evidence about effects ). Results: The panel met in-person and via conference calls to generate possible questions to address. Similarly, the affected individual may be unaware of these limitations as well. Telerehabilitation for musculoskeletal pain - An overview of systematic reviews. The best strategies to partner with local, state, and federal health care authorities in low-middleincome settings to provide hydroxyurea therapy for primary stroke prevention programs should be determined. Time to first neurological event-stroke, seizure, TIA for children with normal or conditional TCD measurements (time averaged mean maximum velocity of <200 cm/s, nonimaging, or <185 cm/s imaging technique) and with (n = 68) and without SCIs on MRI (n = 353). The systematic review did not identify any studies that compared screening to no screening. Preexisting SCIs. The SIT Trial25 was published after the 2014 panel review and was not referenced in the 2014 NHLBI Expert Panel Report.162 The recent AHA/ASA statement on pediatric stroke management86 provides guidance for initial management of suspected or confirmed acute ischemic strokes in children with SCD, as well as primary and secondary stroke prevention with regular blood transfusion. Optimal transfusion targets and methods for secondary stroke prevention are needed. The EtD framework for each recommendation describes which individuals were recused from making judgments about each recommendation. Recusal was also used to manage conflicts of interest.14,34-36 During deliberations about recommendations, any panel member with a current, direct financial interest in a commercial entity that marketed any product that could be affected by a specific recommendation participated in discussions about the evidence and clinical context but was recused from making judgments or voting about individual domains (eg, magnitude of desirable consequences) and the direction and strength of the recommendation. The certainty of the net benefit of this recommendation was considered high. For children aged 12-59 months: If 0 doses or 1 dose of Hib vaccine was given before age 12 months, give 2 doses of Hib, 8 weeks apart. No dissent was observed in the panel regarding the importance of identifying the risk of an initial stroke and providing caregivers with the option to select the best treatment of primary stroke prevention for the child. The peak systolic TCD velocity has not been applied as a predictor of initial stroke in a randomized controlled trial. Overall, the ASH Guideline Panel on Cerebrovascular Disease placed a higher value on maintaining cognitive function than on being alive with reduced cognitive function (significantly less than baseline functioning). If new infarcts are present, then a discussion with the patient and family regarding the pros and cons of a step-up in therapy intensity to prevent cerebral infarct recurrence. For adults with SCD presenting with symptoms of acute ischemic stroke and being considered for IV tPA (age 18 years, no hemorrhage on CT scan, within 4.5 hours of onset of signs, symptoms, and without contraindications for thrombolysis), the ASH guideline panel suggests management using a shared decision-making approach that follows these principles: Patients may be evaluated for IV tPA based on its established inclusion and exclusion criteria detailed in stroke-management algorithms. Over a 6-year study period among 4775 children with HbSS or HbS0 thalassemia from 6 US states, 22% to 44% of children received TCD screening.69. Blood Adv. As described by the GRADE Guidance Group, good practice statements endorse interventions or practices that the guideline panel agreed have unequivocal net benefit yet may not be widely recognized or used.22 Good practice statements in these guidelines are not based on a systematic review of available evidence. Children evaluated for abnormal TCD measurement should not have a recent blood transfusion because of the known association between TCD velocities and transfusions.43 Typically, the TCD measurement should be done at least 3 months after the last transfusion and when the child is at their baseline state of health. The current number needed to treat is 7 (ie, 7 children with abnormal TCD measurements must receive at least monthly blood transfusion therapy for at least a year to prevent 1 stroke). 2019 Dec 10;3(23):3867-3897. doi: 10.1182/bloodadvances.2019000916. Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014. Patient preferences should drive clinical decisions. The .gov means its official. The evidence summary and EtD framework can be found online at: https://guidelines.gradepro.org/profile/a34d09131753ee2f22fcaec5b2510f11, https://guidelines.gradepro.org/profile/b9ab114a172cfa3dab8c60ae5d7ba0b0, and https://guidelines.gradepro.org/profile/fe67f197d72505e0299c9557312b83b9. Management of younger patients without these risk factors should emphasize early transfusion. Policymaking, including that by payers, will require substantial debate and input from stakeholders. As science advances and new evidence becomes available, recommendations may become outdated. Should IV thrombolysis with tPA vs no treatment with tPA be used for adults with SCD presenting with acute ischemic stroke and no hemorrhage on CT scan within 4.5 h of onset of symptoms? was chair of the panel, with CNS content knowledge expertise; M.H.M. When developing these recommendations, the ASH guideline panel acknowledged variability in knowledge about risks and benefits of treatments, as well as variability for patients, their family members, and provider perceptions of the balance between harms vs benefits. For each recommendation, the panel took a population perspective and came to a consensus on the following: the certainty in the evidence, the balance of benefits and harms of the compared management options, and the assumptions about the values and preferences associated with the decision. RESULTS: Usable responses from 254 of 574 eligible respondents (44%) were received. As such, they completed a disclosure-of-interest form, which was reviewed by ASH and is available as supplemental Files 2 and 3. Research evaluating implementation practices that produce the best access to screening, surveillance, and assessment for developmental delays and cognitive impairments, or both, is needed. However, based on the extensive experience of the panel, blood transfusion therapy is acceptable to many caregivers and children with HbSS or HbS0 thalassemia phenotypes and abnormal TCD measurements. ASH does not warrant or guarantee any products described in these guidelines. eCollection 2023 Feb. American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease. When health care systems and health care providers adopt these guidelines, there will be a decrease in neurological morbidity in children and adults with SCD. The American Congress of Rehabilitation Medicine has formally endorsed evidence-based strategies to support individuals with executive dysfunction. There are no validated risk stratification or reliable age cutoff criteria to guide the choice of initial therapy. We did not identify a study reviewing the challenges of regular hydroxyurea therapy for families living in low-middleincome settings. The systematic review did not identify any comparative studies in SCD that compared cognitive rehabilitation to no rehabilitation. In some cases, the treatment with tPA may occur before the patient has been recognized as having SCD. Algorithm of management of acute suspected ischemic stroke in children with SCD. In an optimal implemented primary stroke program in high-income settings, <1% of the children who receive TCD screening coupled with regular blood transfusion therapy for abnormal TCD measurements will have strokes.2 For children with an abnormal TCD measurement, the risk of ischemic strokes is exceptionally high: 10.7 strokes per 100 patient-years.38. A hematologists diagnosis of only silent cerebral infarct in a child with HbSS or HbS0 thalassemia will misclassify 7% of the children as having a silent cerebral infarct, when in fact they had a stroke.25 This misclassification of a stroke as a silent cerebral infarct may result in a different clinical course in treated and untreated individuals. These guidelines were developed by an expert panel composed of health care professionals with expertise in family medicine, general internal medicine, adult and pediatric hematology, psychiatry, transfusion medicine, obstetrics . The document was revised to address pertinent comments, but no changes were made to recommendations. Formal assessments of cognition providing an objective evaluation of the patients cognitive capacities are often requested after cerebral injury is diagnosed by cerebral MRI scan. Following the GRADE approach, randomized trials and observational studies provide an initial level of certainty in evidence classified as either high or low, and this classification may be subsequently modified based on additional factors.32 Then, evidence-to-decision (EtD) factors are applied to make a recommendation. These studies provide a compelling rationale for the use of hydroxyurea for primary stroke prevention in children with abnormal TCD measurements in low-middleincome settings61 as additional trials with CNS complications as end points are conducted.39. No data exist for the other SCD phenotypes. Venous thromboembolism prophylaxis in sickle cell disease: a multicenter cohort study of adolescent inpatients. Their recommendations are similar to the ASH CNS panel recommendation. What are others saying and what is new in these ASH guidelines? Increased longevity of patients with sickle cell disease in high-income, middle-income, and low-income countries present unprecedented challenges for all settings; however, a globally standardised solution for patient transition from paediatric to adult sickle cell disease health care . Should screening with MRI for silent cerebral infarcts vs no screening be used for children and adults with HbSS or HbS0 thalassemia? Sickle cell disease (SCD) is a group of complex genetic disorders with multisystem manifestations. Please enable it to take advantage of the complete set of features! American Society of Hematology 2021 guidelines for sickle cell disease: stem cell transplantation. Given that children and adults with HbSS or HbS0 thalassemia have an increased prevalence of intracranial160 and extracranial3 vasculopathy and surgically correctable aneurysms,4,161 MRA of the intracranial and extracranial vessels should be added to the MRI of the brain. In summary, the results for STOP and STOP 2 demonstrated a clear benefit of regular blood transfusion compared with no transfusions (observation) (Figure 3). (D) Linear and punctate T2 hyperintensities that suppress on FLAIR are consistent with prominent perivascular spaces (Virchow-Robin spaces). 8600 Rockville Pike The panel followed best practices for guideline development recommended by the Institute of Medicine and the Guidelines International Network (GIN).12-15 The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach16-21 to assess the certainty in the evidence and formulate recommendations. Axial FLAIR and T2-weighted images show ill-defined symmetrical T2-weighted hyperintensity in the deep parietal white matter. Optimal timing of intervention with blood transfusion therapy and brain-imaging modality has not been rigorously studied. 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