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6 Delivering High-Quality Sickle Cell Disease Care with a Prepared Workforce
Pages 249-302

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From page 249...
... Chapter Summary • Providing high-quality care for sickle cell disease (SCD) requires identifying evidence-based services for individuals with SCD and the availability of a trained and willing multidisciplinary workforce.
From page 250...
... Some health care providers may be uncomfortable with providing SCD care because of a lack of knowledge and understanding about the clinical condition and the affected population. Clinical practice guidelines are an effective way of standardizing care and informing health care providers (especially non-experts)
From page 251...
... (CMS, 2020) There are currently two measures for SCD care that have been endorsed by the National Quality Forum (NQF)
From page 252...
... . The resulting guidelines, EvidenceBased Management of Sickle Cell Disease, were published in 2014 (Yawn et al., 2014)
From page 253...
... Management of Acute Complications • Use an individualized prescribing and monitoring protocol for SCD pain episodes or an SCD-specific protocol whenever possible • For severe pain, rapidly initiate around-the-clock parenteral analge sics, reassess frequently, titrate to relief • Immediate evaluation of all fevers > 101.3°F (38.5°C) and prompt administration of antibiotics in the case of affected children •  not give transfusions to treat priapism or acute renal failure unless Do there are other indications for transfusions • Evaluate for acute chest syndrome in the setting of acute onset of respiratory symptoms irrespective of the absence of fever; hospital ize for further management if an infiltrate is seen on chest X-ray or if oxygenation is subnormal Management of Chronic Complications • Use a combination of patient-reported pain relief, adverse effects, and functional outcomes to guide the use of long-term opioids for chronic pain • Treat avascular osteonecrosis with analgesics and consult physical therapy and orthopedics for assessment and follow-up • Obtain echocardiogram only in patients with signs or symptoms sug gestive of pulmonary hypertension • Initiate angiotensin-converting enzyme inhibitor therapy for adults with microalbuminuria or proteinuria and no other apparent cause Hydroxyurea and Transfusion Therapies • Treat all adults with hydroxyurea who have a history of three or more moderate to severe pain episodes in a 12-month period • Treat adults with severe or recurrent acute chest syndrome with hydroxyurea continued
From page 254...
... . Recommended services with low-quality evidence represent areas where some variation in care may be acceptable because the services may be appropriate for only a subset of the SCD population.
From page 255...
... : 1. NQF measure #2797, "Transcranial Doppler Ultrasonography Screening among Children with Sickle Cell Anemia: The percent age of children ages 2 through 15 years old with sickle cell anemia (Hemoglobin SS)
From page 256...
... SS) who were dispensed appropriate antibiotic prophylaxis for at least 300 days within the measurement year." The evidence on adherence to NQF-endorsed measures in SCD care is not robust.
From page 257...
... Some strategies for promoting uptake of measures are discussed later in the chapter. Recommendations Adapted from Other Stakeholder Groups Several other associations for health professionals have developed evidence-based recommendations for managing care for adults and children that are relevant for the SCD population.
From page 258...
... and the Adult Sickle Cell Disease Program at Johns Hopkins Hospital (JHH) from January 1, 2014, to December 31, 2015, to assess quality of care.
From page 259...
... PATIENT-CENTERED DIMENSIONS OF HIGH-QUALITY CARE The available guidelines for management of SCD focus primarily on managing the disease and associated complications and thus miss other important dimensions of high-quality care that pertain to the impact of the disease on an individual's health-related quality of life (HRQOL) or their experience with the health care system, especially in care transitions for children from pediatric care into adult care.
From page 260...
... . In response to this need, efforts began to develop SCD-specific tools as part of an NHLBI-sponsored Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me)
From page 261...
... (2013) developed the Sickle Cell Disease Pain Burden Interview–Youth for use in youth and young adults aged 7–21 years.
From page 262...
... . SDM in SCD care is critical but evolving.
From page 263...
... . Given the paucity of research in this area specific to SCD, there are ample research opportunities to examine SDM and the effectiveness of decision aids in improving patient knowledge and decision making in SCD care.
From page 264...
... Pain management for individuals living with SCD must be seen in the context of the current opioid crisis. Sinha et al.
From page 265...
... One available source of guidance, for example, is Got Transition™, discussed in Chapter 5. NHLBI provides guidance on adolescent health care and transitions in its report The Management of Sickle Cell Disease.
From page 266...
... Enrolling in and maintaining health insurance, particularly public health insurance, can be a challenging and time-consuming process, as discussed in Chapter 2. This lack of or delay in obtaining insurance coverage is a significant obstacle in accessing care and may seriously affect quality and continuity of care as young adults may avoid regular care and become more reliant on urgent or emergency care.
From page 267...
... . To better prepare young adults with SCD for the transfer to adult care, there is a need to help them develop increased confidence in their life skills.
From page 268...
... . PROMOTING UPTAKE OF RECOMMENDATIONS FOR SCD CARE There are a number of beneficial and effective services recommended for SCD care; two of these recommended services are endorsed by NQF as quality measures.
From page 269...
... said CMS had "missed a historic opportunity to definitively address a national shame, the poor state of care provided to children with sickle cell disease," because the Pediatric Measure Application Partnership (P-MAP) had recommended certain changes, such as to include the two NQF-endorsed measures in the core measure set.
From page 270...
... This will require developing measure sets, similar to the ones in Figure 6-2, that incorporate core metrics relevant for all adults and children in addition to core metrics specifically relevant for individuals with SCD and SCT. Developing and accrediting comprehensive care centers will facilitate adherence to quality indicators and existing and future quality measures, because these centers' accreditation and performance can be tied to providing recommended services for SCD.
From page 271...
... SUMMARY The evidence supporting the guidelines for SCD care is highly variable, relying on expert consensus but highlighting research gaps. The evidence for a comprehensive, systematic approach to quality care delivery for SCD is lacking, and the science of quality in SCD care appears to lag behind other diseases in several areas: • The majority of SCD quality indicators are supported with poor quality evidence, often with consensus expert recommendation as the highest level of evidence available.
From page 272...
... However, access to these services is often institution dependent and further reduced in adult care. Such services often require specialized clinical experience and technical expertise and are harder to access outside of comprehensive care centers.
From page 273...
... The committee employed a variety of methods in determining team members. First, the committee identified specialists and clinical services historically involved in SCD care.
From page 274...
... First Level Core Team Members with SCD and disease complication screening follow-up. Nurse care Knowledge of evidence-based Conduct initial assessment in clinics, There is no formal training with coordinator and management of individuals assist with appointment scheduling, the SCD population (negative nurses with SCD with SCD address patient-specific insurance provider biases and attitudes can expertise issues, administer infusions in influence care)
From page 275...
... predominantly Caucasian. Psychologist/ Comfortable discussing Provide psychological support for Knowledge gaps about psychiatrist evidence-based management mental issues, such as depression, SCD and negative provider of individuals with SCD anxiety, pain management, attitudes contribute to poor grief counseling, transition to communication.
From page 276...
... Dentist/dental Knowledge of evidence-based Assess and provide treatment There is limited formal training hygienist management of individuals for dental complications, which with the SCD population. with SCD include caries or cavities, tooth hypomineralization, orofacial pain, neuropathy, facial swelling, pallor of oral mucosa, malocclusions, infections, pulpal necrosis, cortical erosions, medullary hyperplasia, Second Level Core Team Members and abnormal trabecular spacing.
From page 277...
... process when patients reach their early teens; coordinate transfer to adult providers. Community health Basic knowledge of Assist with medication adherence, There are no national standards worker evidence-based management transitions of care, patient for training, which is designed of individuals with SCD education, and education/vocation by the organizations that employ assistance.
From page 278...
... Physical therapist Basic knowledge of Provide therapies to reduce There is no formal training with evidence-based management stress and pain and improve range the SCD population. of individuals with SCD of motion, which can include aerobic and breathing exercises, massages, or transcutaneous electrical nerve stimulation to disrupt pain sensors and relax muscles.
From page 279...
... NOTE: ACS = acute chest syndrome; CHW = community health worker; HU = hydroxyurea; OB/GYNs = obstetricians and gynecologists; PCP = primary care provider; QOL = quality of life; RBC = red blood cell; SCD = sickle cell disease. SOURCES: Informed by Agrawal et al., 2019; CFF, n.d.; Farooq and Testai, 2019; McIntosh, 2016; Mulimani et al., 2016; Okpala et al., 2002; Scott, 2016; Simon et al., 2016; Wilkie et al., 2010; and National Academies SCD committee judgment.
From page 280...
... . The SCD care team members are classified as core or supplementary based on their level of involvement in managing the clinical and psychosocial complications of SCD (see Chapter 4)
From page 281...
... Third-year fellows have described benign hematology as more "complicated" and "overwhelming" than malignant hematology or medical oncology (Bernstein et al., 2017)
From page 282...
... . Compounding the problem, many of these physicians specialize in benign hematology, and there is no pool of new fellows to replenish them.
From page 283...
... Easing the financial burden from student loans and offering financial incentives for research on and treatment of the SCD population is another way to attract health care professionals to specialize in SCD care. The current federal loan repayment programs (LRPs)
From page 284...
... . These opportunities need to be disseminated to students and health professionals who may be interested in providing care to the SCD population but who have concerns regarding student loan debt.
From page 285...
... The workshop assessed pre- and post-workshop knowledge and had its greatest impact on shifting FIGURE 6-3  Elements of improving the emergency department experience. NOTE: ED = emergency department; FMECA = failure mode, effects, and criticality analysis; IV = intravenous; NHLBI = National Heart, Lung, and Blood Institute; PCA = patient-controlled analgesia; PDSA = plan-do-study-act; QI = quality improvement; SCD = sickle cell disease.
From page 286...
... The program has advanced the treatment of SCD-related pain in the ED, leading to more rapid pain relief. The multidisciplinary team of physicians, nurses, social workers, and individuals living with SCD works to increase awareness and decrease negative provider attitudes about SCD patients.
From page 287...
... . In 2020, HRSA, in collaboration with the Office of Minority Health at HHS, launched a 6-month pilot of a national SCD ECHO, the Sickle Cell Disease Training and Mentoring Program (HHS, n.d.)
From page 288...
... . Other members of the SCD medical treatment team include, but are not limited to, nurses and nurse practitioners, physician assistants, blood bank and transfusion specialists, neurologists, radiologists, dentists, ophthalmologists, obstetrician/ gynecologists, genetic counselors, integrative medicine specialists, nephrologists, orthopedists, pulmonologists, psychologists, and social workers (see Table 6-1)
From page 289...
... CONCLUSIONS AND RECOMMENDATIONS Despite national efforts to improve quality in SCD care, that quality remains poor for most individuals in the United States. National quality metrics have not been developed, resulting in significant variations.
From page 290...
... Access to providers and the availability of a trained, prepared workforce are key issues that have not been adequately addressed in SCD care. The lack of adult hematologists and other providers further contributes to variations in care.
From page 291...
... Strategies are needed to enhance the evidence base to support the treatment of SCD. Conclusion 6-2: The evidence for a comprehensive, systematic approach to the delivery of high-quality care for SCD is lacking, and the science of quality in SCD care lags behind other diseases in several areas: SCD clinical guidelines are supported with poor quality evidence; there are multiple sets of guidelines, but none are universally accepted or cover the services needed for comprehensive care; adherence to clinical guidelines and quality indicators is not measured, reported, required, incentivized, or included in public transparency reports; and there is evidence of a lack of funding and priority for the development of quality metrics in SCD.
From page 292...
... Food and Drug Administra tion should work together with and fund researchers and professional associations to develop and track a series of indicators to assess the quality of sickle cell disease care, including the patient experience, the prevention of disease complications, and health outcomes. Recommendation 6-2: The Centers for Medicare & Medicaid Services and private payers should require the reporting of expert consensus driven sickle cell disease (SCD)
From page 293...
... n.d. ASH clinical practice guidelines on sickle cell disease.
From page 294...
... 2015. Exploring adult care experiences and barriers to transition in adult patients with sickle cell disease.
From page 295...
... 2014. Transition from paediatric to adult care for patients with sickle cell disease.
From page 296...
... 2010. Transition from pediatric to adult care for patients with sickle cell disease.
From page 297...
... 2013. Systematic review of transition from ado lescent to adult care in patients with sickle cell disease.
From page 298...
... 2019. American Society of Hematology 2019 guidelines for sickle cell disease: Cardiopulmonary and kidney disease.
From page 299...
... 2018. Sickle cell disease pain management.
From page 300...
... 2015. An evaluation of the transition to adult care for young patients with sickle cell disease.
From page 301...
... 2017. Development of quality indicators for transi tion from pediatric to adult care in sickle cell disease: A modified Delphi survey of adult providers.
From page 302...
... 2018. Transition from pediatric to adult care: Sickle cell disease.


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