Manage Sickle Cell Pain Crises


Standardized order sets reduce medication errors and create efficiency for physicians, nurses, and pharmacists.1 A less recognized benefit may be that standardized orders promote equity in care and may help to mitigate disparities that occur along racial and ethnic lines. Patients admitted with an acute pain crisis secondary to sickle cell disease (SCD) are often suspected of drug-seeking behavior and may be treated in a cursory manner because they are “frequent flyers” on the medical service. In view of these factors, this patient group may benefit more than others from a standardized treatment protocol. Over the past year, hospitalists and other clinicians at Novant Health in Charlotte, N.C., have composed an admission order set for SCD pain crisis in an effort to improve care for this underserved group.

Novant Health

Novant Health is a multi-hospital, non-profit system serving 3.5 million people from Virginia to South Carolina. Presbyterian Hospital, a 531-bed facility in Charlotte, N.C., is the flagship hospital for Novant’s Southern Piedmont Region (SPR). Presbyterian Inpatient Care Specialists (PICS) is a 44-provider group that offers hospitalist services for adult patients at Presbyterian Hospital, two community hospitals in Matthews and Huntersville, and Presbyterian Orthopedic Hospital.2 In 2005, 180 adult patients were discharged from the Novant-SPR hospitals with a principal diagnosis of SCD, and most of these patients were managed by PICS. This cohort of 180 patients accounted for more than 600 hospitalizations for SCD-related illness at the Novant-SPR facilities in 2005.

Patients admitted with an acute pain crisis secondary to sickle cell disease are often suspected of drug-seeking behavior and may be treated in a cursory manner because they are “frequent flyers” on the medical service.

Process and Procedure

Standardized order sets have been in widespread use at the Novant-SPR facilities for a number of years. The process of order set development begins with patient needs as perceived by medical staff. Once a physician has drafted an order set, the draft is reviewed by the Order Set Subcommittee, a multidisciplinary team chaired by Susan Smith, RPh, PharmD, with nurses, physicians, and other support staff represented as needed. Once the subcommittee has approved the draft, it goes to the Pharmacy and Therapeutics Committee for final approval. In this fashion, more than 600 order sets have been developed and made available on the hospital’s intranet. The PICS team has helped to develop a number of disease management order sets and protocols, including the general medical admission orders. In addition, PICS has assisted in developing disease-specific orders for community-acquired pneumonia (CAP), CVA/TIA, and other common diseases admitted by the hospitalist service.

The PICS Sickle-Cell Disease Treatment Protocol p.1 click for large version

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The PICS Sickle-Cell Disease Treatment Protocol p.2 click for large version

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The PICS Sickle-Cell Disease Treatment Protocol p.3 click for large version

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In the case of the adult SCD pain crisis order set, Stephen Wallenhaupt, MD, chief medical officer for Novant Health, recalls, “We recognize that SCD patients have unique needs in terms of pain management and that a standardized order set which incorporates aggressive pain management options is the best way to meet their needs.”

Fran Davis, RPh, PharmD, researched the pertinent literature and consulted the anesthesiologists on staff in an effort to tailor the pain management options for SCD patients. Using the recommendations of these specialists, the order set reflects the consensus that patients with a severe SCD pain crisis require parenteral opiate analgesia on admission.3

Because many patients admitted by PICS can be categorized as having severe pain, the order set contains choices for patient-controlled analgesia (PCA), in keeping with the recommendation for opiate administration at a fixed interval rather than on an as-needed basis.4 And because many hospitalists may not be familiar with ordering PCA, the SCD orders specify the dose ranges, as well as the lockout interval for morphine and hydromorphone PCA. With PCA options readily available, hospitalists need not delay therapy while awaiting a consultation from the pain management service. For all patients receiving opiates, the order set contains an automatic order for naloxone for respiratory depression manifested by a respiratory rate less than nine.

For patients with less severe pain, options for PRN (pro re nata, or as needed) bolus dose morphine and hydromorphone offer dose ranges that reflect the narcotic tolerance frequently observed in these patients. The order set includes options for non-opiate agents, including ketorolac and acetaminophen. Adjuvant therapies, including promethazine, zolpidem, and diphenhydramine, are also contained within the order set. Prompts for intravenous fluid replacement, oxygen therapy, and other supportive measures are incorporated into the orders as well.

The Novant-SPR hospitals have adopted a separate order set for the administration of pneumococcal vaccine. This order set allows for the automatic administration of pneumococcal vaccine, without a specific physician order, to every patient who meets criteria and who desires the vaccine. On admission, every patient is screened by a nurse regarding their need for pneumococcal vaccine; as a result, SCD patients who have not been immunized in the previous five years are offered the vaccine.

Day Hospital Model

In order to provide SCD patients with immediate and aggressive analgesic therapy and fluid replacement, some centers have adopted the Day Hospital (DH) model. The DH is a specific site at which patients with SCD pain crisis are treated by staff familiar with the patients and with their special analgesic needs.5 The DH model has been shown to provide rapid pain relief in an outpatient setting and to reduce the need for unnecessary hospitalizations. In addition, length of stay and cost per case are lower for DH patients admitted to the hospital than for patients not followed by DH staff. It has been suggested that the DH model is especially appropriate for centers with an emergency department too busy to begin treatment in a timely fashion.6

Novant Health has adopted the DH model in the urgent care setting. Patients with pain crises can be managed in one of Novant’s urgent care centers with a standard order set that includes options for IV hydration as well as parenteral morphine or hydromorphone. This outpatient order set parallels that designed for inpatients, because it prompts the physician to order aggressive analgesic therapy as well as adjuvant treatments for nausea and anxiety. Practitioners from Novant’s Sickle Cell Clinic often meet the patient at the urgent care center to assist with evaluation and management. In the past two years, approximately two-thirds of patients with SCD pain crisis have been discharged to home with good pain relief. The remaining one-third are referred to the hospital—usually to be admitted and managed by the PICS team.

Hope for the Future

It has been more than 50 years since the molecular defect in hemoglobin S was first described by Linus Pauling and 50 years since the genetic defect was first elucidated.7,8 Despite these and subsequent advances in understanding the pathogenesis of SCD at the cellular and molecular level, our treatment for SCD remains inadequate. It is akin to a “halfway technology” described by Lewis Thomas as, “the kinds of things that must be done … in order to compensate for the incapacitating effects of certain diseases whose course one is unable to do very much about. It is a technology designed to make up for disease, or to postpone death.”9

At present a number of agents designed to inhibit the sickling process are under investigation, including omega-3 fatty acids, Gardos channel blockers, and anti-adhesion therapies.10 In time, perhaps, one of these rational approaches to treatment will bridge the chasm in the halfway technology currently at our disposal. TH

Dr. Gardella is a board-certified internist and pulmonologist who currently serves as vice president for clinical improvement for Novant Health—Southern Piedmont Region.

Julie Swanger works with both pediatric and adult sickle cell populations through Presbyterian Community Care Services and Blume Pediatric Hematology and Oncology Clinic in Charlotte, N.C.


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