Implementing ASH’s guidelines for acute medical care for incarcerated children and adults with sickle cell disease Open Access

The ASH special panel deliberated implementation strategies for ASH systematic review panel guidelines focusing on 3 time-sensitive medical management emergencies in individuals with SCD: acute neurological events, acute pain, and fever (Table 1).

For children or adults with SCD and acute neurological deficits, including transient ischemic attack, the ASH guideline panel recommends prompt blood transfusion. The transfusion should be given immediately upon recognizing symptoms, without delay, ideally within 2 hours of acute neurological symptom presentation. Acquiring imaging should not delay blood transfusion in the setting of new focal neurological deficits suggestive of a stroke or a transient ischemic attack. The type of transfusion (simple, modified exchange, or apheresis) depends on individual patient factors and local transfusion resources (strong recommendation).² 

The ASH special panel recommends that for individuals with SCD held in custody with acute focal neurological findings, the facility personnel should immediately refer the patient to the nearest medical center for emergency care, either by calling 911 or by providing prompt (ie, within 1 hour) nonmedical transport to the ED, where timely evidence-based medical care acute treatment of stroke for individuals with SCD can be delivered.

A patient with SCD who has previously experienced an acute neurological defect should have a correctional facility placement prioritized to a location geographically close to a medical center equipped to care for a patient with SCD. Access to a higher level of care can be limited in rural areas. Many jails and prisons contract with other correctional facilities to admit individuals with acute care requirements. Thus, we recommend prioritized admission to precontracted correctional acute health facilities with SCD expertise.

For adults and children with SCD presenting to an acute care setting with acute pain related to SCD, the ASH guideline panel recommends rapid (within 1 hour of ED arrival) assessment and administration of analgesia with frequent reassessments (every 30-60 minutes) to optimize pain control.² 

The ASH special panel recommends that for children and adults with acute SCD pain (ie, acute-onset pain not immediately attributable to a cause other than SCD), immediate notification and assessment should be conducted to include vital signs and pulse oximetry assessment, with a prompt notification and involvement of the licensed medical personnel on site. If no licensed medical staff are available on site, the patient should be evaluated by telemedicine or emergency medicine services or transferred promptly with the assistance of security staff to a medical facility for assessment. After prompt evaluation of the clinical history, vital signs, and clinical examination, communication should occur with the licensed medical provider overseeing the patient’s status to determine the management and ongoing assessment plan. If the acute vaso-occlusive pain event is complicated (associated with fever, decreased oxygen saturation of <94% or at least 3% below a baseline <94%, difficulty breathing, increased respiratory rate, increased heart rate, or other clinical manifestations of immediate concern such as priapism or signs of organ injury), prompt referral to a medical facility is required for a full assessment and further management.

1. Any individual with SCD held in custody should have access to the guidance of a community hematologist or clinical SCD specialist (ideally their continuity provider). If a patient does not currently have a community hematologist or SCD specialist, the ASH special panel recommends that medical services within a correctional setting ensure access to an SCD specialist in a timely fashion (within 2 hours, perhaps with telemedicine) to provide guidance. These SCD specialists should be able to provide a patient-specific comprehensive assessment, which should include additional questions related to common pain triggers (exposure to cold and hot environmental temperatures, dehydration, psychological stress, menstruation, and infections) as well as strategies to avoid those triggers and reduce pain in carceral settings.^17,18 The hematologists or SCD specialists should share the pain management plan with the facility medical providers and unit staff responsible for administering the pain management plan.

2. The Wong-Baker faces scale should be used to assess acute pain episodes in children, and a numeric rating or visual analog scale in adolescents and adults, with a corresponding acute pain treatment plan for each pain level (mild, moderate, and severe). The acute pain treatment plan should be reassessed and updated annually.

For adults and children with SCD presenting to an acute care setting with acute pain related to SCD for whom opioid therapy is indicated, the ASH guideline panel suggests tailored opioid dosing based on consideration of baseline opioid therapy and prior effective treatment.

The ASH special panel recognizes that the administration of opioids may or may not be available for individuals with SCD held in custody. For this reason, an individual with SCD held in custody with unremitting severe acute vaso-occlusive pain should be promptly transferred to an outside medical facility for prompt medical treatment with appropriate SCD expertise, as would be the case if they were not in custody.

The ASH special panel recommends that adults and children with mild acute pain related to SCD held in custody receive a short course (5-7 days) of nonsteroidal anti-inflammatory drugs (NSAIDs; eg, ibuprofen or naproxen), in alignment with the ASH systematic review panel recommendation. However, the ASH special panel preferred to exclude ketorolac as an NSAID because of the toxicity profile of the renal disease.¹⁹ For moderate to severe acute pain, the ASH special panel recommends that ibuprofen and naproxen can be given with opioids for acute pain management. However, the special panel notes that evidence of the added benefit of NSAID use for acute pain for those receiving morphine is lacking.²⁰ The ASH special panel also suggests additional nonopioid acute pain management approaches in custody, including the following strategies.

Offer extra water during meals and snacks. Ensure adequate hydration during physical activity and monitor environmental temperature because exposure to environmental extremes, such as being too hot or cold, may result in an acute pain episode or exacerbate acute pain.

Provide prompt access to a heating pad, such as a microwavable gel pack, for application to affected areas. If security concerns are present, heat therapy may need to occur in the medical area. Particular attention should be paid to avoiding cold packs or cold showers because they may initiate an acute vaso-occlusive pain episode.

Implement mindfulness strategies such as slow, controlled breathing while counting breaths; sensory exploration for things you see, hear, smell, and taste; and provide alone time in the room.

Encourage distraction exercises such as playing a security-approved board game, card game, or puzzle; journaling; reading; and using paper, nontoxic washable markers, and coloring supplies.

Increase communication with loved ones/family.

If pain prevents participation in activities, provide the patient with alternate activities that promote relaxation, such as listening to music or watching television, if accessible.

For patients in facilities in which they can earn privileges for positive behaviors but cannot participate because of pain, provide additional access to communicating with family and loved ones, and participate in other passive recreational activities.

Ensure the patient has access to spiritual support.

The patient should have a single room.

Avoid extreme cold or hot, humid conditions while in confinement, indoors or outdoors, because cold or moderate weather may result in acute vaso-occlusive pain or exacerbate an acute pain episode.^21-23 

The management of fever in children and adolescents was not included in the ASH systematic review panel guidelines but had recommendations in the 2014 National Heart, Lung, and Blood Institute (NHLBI) guidelines for SCD. The ASH special panel endorses the NHLBI guidelines for fever (≥38.5°C), which indicate that clinicians should respond in a timely fashion because fever in a child with SCD represents a potentially life-threatening event that may be associated with sepsis.²⁴ 

The ASH special panel recommends that fever in a child or adolescent with SCD requires rapid assessment of vital signs and pulse oximetry along with a triage call, per established facility procedures, that would result in communication with the licensed medical provider overseeing the patient’s care. The medical provider should address the patient’s disposition after the initial triage assessment. Children with SCD and fever should promptly receive a physical examination and have laboratory studies performed to include a complete blood count, reticulocyte count, and blood culture. Blood culture should be drawn before empirical administration of broad-spectrum antibiotics; standard care is to provide the antibiotics within several hours after the onset of the fever because of the concern of septic shock leading to death. The laboratory values must be returned and reviewed promptly to allow for a disposition based on clinical surveillance, laboratory data, and the medical provider's impression of the underlying cause. Acute management of fever associated with acute vaso-occlusive pain, abdominal pain with or without emesis, and increased respiratory rate should be managed at an ED or acute care medical facility outside of the custodial facility unless the custodial facility has physicians accustomed to managing acute care of individuals with SCD, and the ability to begin IV treatments including antibiotics, fluids, and pain medication.

The ASH special panel recommends the same approach for adults as for children regarding assessment, triage, laboratory evaluation with prompt, empirical administration of broad-spectrum antibiotics, preferable acute management in an ED or acute medical facility, and communication with the responsible licensed medical provider in the facility. However, the epidemiology of fever and its sequelae in adults is far less well-defined, but there is still a risk of life-threatening complications.^25,26 

A significant limitation of the ASH special panel’s recommendations is that during the intake process into the custodial facility, we assumed that the individual with SCD was aware of their diagnosis and would communicate the SCD diagnosis to the facility's health care provider. For a range of reasons, including the fact that the individual may not know of their SCD or that they are unwilling to disclose their diagnosis because of perceived stigma or vulnerability while in custody, the health care team in the facility may not be aware of the SCD. The committee recognized that most of the carceral care is beyond the control of the sickle disease health care providers.

The optimal implementation strategies for rendering time-sensitive medical care are beyond the scope of the ASH special panel’s charge. The committee also did not have a standardized approach to ensure that all individuals who entered a custodial facility were identified as having SCD without universal screening for anemia with additional laboratory tests to confirm SCD.

The ASH special panel’s directive focused on ASH’s SCD clinical practice guidelines that relate to acute medical care and not all acute or chronic manifestations of the disease. Frequent acute SCD complications that were omitted include priapism and acute vascular necrosis of the femoral head²⁷ or humerus²⁸ resulting in acute and chronic pain, acute cholecystitis, splenic sequestration, exacerbation of leg ulcers that may require acute and chronic management,²⁹ and headaches.

Regarding women’s health, women with SCD may have acute vaso-occlusive pain associated with the onset of their menstrual cycle,³⁰ and pregnancy is associated with a significant risk of morbidity and mortality.³¹ Women with SCD have a much higher incidence rate of acute vaso-occlusive pain than men with SCD.³² Furthermore, if pregnant, the woman and the fetus should be managed by a fetal-maternal specialist with expertise in managing SCD. Without evidence-based clinical practice guidelines, we would refer the clinician to references that describe the best available evidence for managing acute care and ongoing health maintenance for specific women’s health concerns while in custody.

Another limitation of the ASH guidelines that the ASH special panel could not address is the absence of primary care guidelines for maintaining continuity of care in custody and upon reentry. Without evidence of best practice, the ASH special panel believes that, at minimum, individuals with SCD should have an identified primary care provider in the community and a specialist in the care of SCD. The ASH special panel strongly endorses continuity with the primary care provider that follows the American Academy of Pediatrics Bright Futures, American College of Physicians, American Academy of Family Physicians, and US Preventive Services Task preventive care and health maintenance evidence-based guidelines. A failure to follow existing preventive care guidelines such as Bright Futures is below the standard of care in the community. The American Academy of Pediatrics’ Bright Future Guidelines should be applied to all children held in custody. As expected in the community for individuals with SCD and pregnant women with SCD, hematologists with SCD expertise and maternal-fetal specialists are expected to be engaged with their patients throughout the time the patients are held in custody. There should be a clear plan for continuity of care after discharge and managing obstetric emergencies that may occur while a patient is in custody. People in custody with SCD often require additional subspecialty care and rapid access to emergency care, and the distance to this care should help guide where they are incarcerated. The medical care of individuals with SCD continues to evolve rapidly, requiring ongoing review of recent evidence-based guidelines for its management.

The ASH special panel recognized that a range of professional responsibilities within each carceral facility may prohibit prompt ascertainment of vitals, pulse oximetry readings, and a physical examination. However, if acute medical complications, including stroke, acute pain with or without respiratory distress, and fever, are identified in a child or an adult with SCD, the ASH special panel recommended that an emergency response should be activated (ie, call 911 or immediately transfer by the custody team to an emergency room to triage the patient as quickly as possible at a medical facility outside the custody setting for acute care management with SCD expertise and equipment for prompt management). For all transfers of patients to ED for SCD clinical manifestations, the custody medical team must communicate to the medical center receiving the patient that the patient has SCD.

As would be the case for individuals not in custody, the ASH special panel strongly recommends that acute medical care is provided for all individuals with SCD held in custody that meets or exceeds community standards. Establishing a minimum care standard, that is, at least equivalent to the expected care in the community, also entails having a pediatric or adult SCD expert engaged in caring for the patient in custody who can facilitate: (1) acute management of any life-threatening emergency in a timely fashion, (2) prevention of complications, and (3) support of anticipatory guidance per the ASH guidelines. The standard care would include an acute and chronic pain management strategy implemented promptly when either or both are present. Furthermore, given the unique health and health care delivery challenges inherent to custody settings, the ASH special panel strongly recommends that future ASH clinical practice guidelines for SCD and other diseases address how the management should be different or triaged for people in custody.

Contribution: M.R.D. and E.S.B. conceived of and wrote the first draft of the manuscript; and all authors revised the manuscript, critically reviewed the final version of the manuscript, provided suggestions for improvement, and approved the content.

Conflict-of-interest disclosure: M.R.D. participated as a chair of the Novartis steering committee; this activity is not directly related to this work. N.S. is a consultant for Pfizer, Agios, bluebird bio, Emmaus, Vertex, and Akirabio; has served as a speaker for Alexion, Pfizer, and Emmaus; and is conducting research sponsored by Pfizer. E.S.B. serves as chair of the Juvenile Health Committee of the National Commission of Correctional Health Care and serves on the commission’s board of directors. The remaining authors declare no competing financial interests.

Correspondence: Michael R. DeBaun, Vanderbilt-Meharry Sickle Cell Disease Center of Excellence, Vanderbilt University Medical Center, 2525 West End Ave, Suite 750, Nashville, TN 37203; email: m.debaun@vumc.org.