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Sickle Cell Disease Pain Management in Adolescents: A Literature Review
Review Article Sickle Cell Disease Pain Management in Adolescents: A Literature Review ---
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From the *Virginia State University, Petersburg, VA; †Indiana State University, Terre Haute, Indiana. Address correspondence to Bridget Wilson, PhD, MS, RN, Virginia State University, Petersburg, VA. E-mail: [email protected] Received December 10, 2013; Revised April 29, 2014; Accepted May 1, 2014. The authors report no financial interests or potential conflicts of interest with the submission of this manuscript. 1524-9042/$36.00 Ó 2015 by the American Society for Pain Management Nursing http://dx.doi.org/10.1016/ j.pmn.2014.05.015
Bridget H. Wilson, PhD, MS, RN,* and Jessica Nelson, DNP, RN†
ABSTRACT:
Sickle cell disease (SCD) pain continues to emerge in adolescents. More than 98,000 individuals are believed to have SCD in the United States. In fact, 1 in 500 Black infants will be affected by SCD. Identifying standards of care for this unique population can improve pain management and treatment. A significant effect of vaso-occlusive crisis is a decrease in the quality of life in children. Therefore, pain management is multidimensional and includes pharmacologic, physical, and psychological strategies. A review of the literature was conducted to identify best practices regarding pain management in adolescents with sickle cell anemia. Key words such as pain, pain management, adolescent sickle cell anemia, and acute sickle cell pain were entered into databases to reveal qualitative and quantitative studies from 2009 to the present. Many of the research articles identified poor SCD pain management. Studies showed that acute SCD pain management is essential and should be evaluated and robustly managed to achieve optimum pain relief for patients. Acute SCD pain usually occurs as a result of vaso-occlusive crisis. Untreated acute SCD pain can result in morbidity and mortality in adolescents. Nursing knowledge is critical to reducing the stigma and improving management of SCD pain. Nurses play a vital role in the introduction of evidence-based practice within the clinical setting. In an effort to educate nurses and other health care professionals about SCD, this article is a literature review of studies concerning SCD and pain management in emergency rooms. Ó 2015 by the American Society for Pain Management Nursing
BACKGROUND Sickle cell disease (SCD) is characterized as the most prevalent genetic blood disorder affecting red blood cells worldwide (Myers & Eckes, 2012). SCD is believed to affect more than 98,000 individuals in the United States alone and 1 in 500 Black infants (Porter, Feinglass, Artz, Hafner, & Tanabe, 2012; Vijenthira et al., Pain Management Nursing, Vol 16, No 2 (April), 2015: pp 146-151
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2012). SCD is characterized by recurrent, acute severe pain episodes due to vaso-occlusive crisis (VOC). The acute severe pain episode is caused by ischemic tissue resulting from blood vessel occlusion preventing blood flow (Vijenthira et al., 2012). VOC has been shown to decrease children’s quality of life. Therefore, pain management is multidimensional and includes pharmacologic, physical, and psychological strategies. According to Vijenthira et al. (2012), treating VOC pain should occur early and be aggressive to prevent morbidity. SCD patients seen in the emergency room (ER) wait at least 4 hours before receiving their first dose of pain medication (Haywood, Tanabe, Naik, Beach, & Lanzkron, 2013). SCD patients can have acute or chronic pain or a combination of both resulting in malformed sickle-shaped red blood cells. Sickle-shaped red blood cells cannot flow properly through the blood vessels and capillaries causing significant pain (Myers & Eckes, 2012). Both acute and chronic pain episodes require timely treatment. SCD patients often are seen as drug seekers once they enter the ER (Haywood et al., 2013). Many of these patients manage their pain at home; however, an average of 197,000 ER visits occur each year, with a hospital admission rate of 29% (Porter et al., 2012). The cost for SCD patients seen in the ER for VOC has been estimated to be more than $356 million. Elkins, Johnson, and Fisher (2012) indicated that pain is a ‘‘significant and ubiquitous health problem, costing productivity, employment, income, reducing quality of life, degrading relationships, and negatively affecting physical and psychological well-being’’ (p. 294). Even with the guidelines and recommendations for pain management in patients experiencing VOC episodes there continues to be a gap with providing pain control among this group. In part, 63% of nurses working with these patients believed addiction was a factor, and 30% were reluctant to administer a high dose of analgesic (Porter et al., 2012). Therefore, undertreatment of pain occurs in patients with SCD experiencing a VOC. Research by Meier and Miller (2012) suggests that although opioids were used for SCD patients with painful VOC episodes, drug dependency in SCD does not differ from other individuals in the general population without SCD. Dampier, Haywood, and Lantos (2011) indicated that drug seeking is often assumed due to the excessive use of opioids; however, it is somewhat uncommon among pediatric patients with SCD. In part, hyperalgesia from frequent opioid withdrawal or excessive use is more than likely the medical reason. Often, SCD patients are not drug seekers but are seeking care due to the lack of ‘‘psychosocial support,
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poor coping skills, and inappropriate therapeutic expectations’’ (Dampier et al., 2011, p. 128). SCD pain management should be determined and will require multiple medications in which responses are made on a variety of neurochemical pathways. Wang, Kavanagh, Little, Holliman, and Sprinz (2011) found that 6% of patients with SCD die during childhood. Wang et al. (2011) suggested there is evidence between gaps and variations in quality of care that contributes to mortality in children affected with SCD. Also, poor outcomes are a factor in the quality of care in SCD.
PURPOSE Nursing knowledge is critical to reducing the stigma and management of SCD pain. The purpose of this article is to investigate the best practices related to the management of SCD pain in adolescents. Identifying standards of care for this unique population can improve pain management and treatment. Nurses play a vital role in the introduction of evidence-based practice within the clinical setting. In part, nurses will serve as a catalyst providing scientific evidence to help them inform peers to facilitate change in adolescent SCD pain management.
SEARCH CRITERIA A comprehensive literature review was performed using multiple databases including Medline, PubMed, Cochrane Database, EBSCOhost, CINAHL, and Health Source: Nursing Academic Edition. The original search yielded 27 appropriate articles. Key terms included sickle cell disease, pain, adolescent, treatment, vasoocclusive crisis, nursing, and emergency room. Due to the limited number of retrieved studies related to SCD, other databases such as ProQuest, ProQuest Nursing and Allied Health Source were added to search areas of sickle cell pain in adolescents. This generated 4,134 studies and articles to collect the needed data for this literature review. Key terms were narrowed to include sickle cell pain, nursing, pain, management, adolescents, and vaso-occlusive crisis. These terms were entered into ProQuest and ProQuest Nursing and Allied Health databases. The results were narrowed by date of publication, to include articles published from 2009 to the present. The final nine studies selected included quantitative, qualitative, cross-sectional, randomized control trials, and comparative analysis dealing with SCD and pain management and were analyzed and selected based on sickle cell pain management.
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LITERATURE REVIEW Many of the research articles acknowledged the need for pain management for clients with SCD in a VOC. According to Wang et al. (2011), efforts have been limited in improving quality care for children with SCD in comparison with other childhood chronic illnesses. The research study focused on developing ‘‘a set of quality-of-care indicators for the management of children with sickle cell disease who are cared for in a variety of settings by addressing the broad spectrum of complications relevant to their illness’’ (p. 484). Wang et al. (2011) indicated that quality of care might contribute to poor outcomes for children with SCD. Due to the lack of quality measures for children with SCD, a limited comprehensive and interdisciplinary system of care is developed. The overall goal of the Wang et al. study was to develop quality-of-care indicators to help children with SCD when they present to the ER or outpatient and inpatient clinics, by addressing the complications associated with the disease. Wang et al. (2011) used a Delphi method to generate the final set of quality-of-care indicators. The Delphi method consisted of one round of anonymous ratings by a panel of experts, and a face-to-face panel discussion. Using this method showed the appropriateness and quality-of-care indications for patients with SCD (Wang et al., 2011). As indicated by Wang et al., children diagnosed with SCD are at higher risk for acute and long-term impediments leading to early mortality. The effort to improve and assess quality care for children diagnosed with this disease has been limited compared with children with other chronic conditions. Therefore, as clinical evidence is made available, quality-of-care indicators will change. Haywood et al. (2013) conducted research to determine wait times for patients with SCD in the ER compared with patients with long bone fracture and those with other medical conditions. They used a cross-sectional, comparative analysis of data from the National Hospital Ambulatory Medical Care (NHAMC) survey from 2003 to 2008. The research used a generalized liner model to examine the ratios of patients with SCD wait time in the ER before pain treatment. Haywood et al. (2013) indicated that more than 200,000 patients with SCD are seen in the ER each year for VOC. Their research reported that patients with SCD who are seen in the ER are dissatisfied with the quality of care received. Moreover, these patients are subject to delays and feel they wait longer to be seen by the doctor (Haywood et al., 2013). Haywood et al. pointed out that these patients wait as long as 4 hours before receiving their first dose of pain medication compared with patients with renal colic.
Haywood et al.’s (2013) research indicated that SCD-related visits totaled 171,789 that met the NHAMCS research criteria. The research revealed that patients with SCD waited longer than the general patient group. The severe pain scale used during triage showed 54% of patients with SCD rated their pain between 7 and 10 on an 11-point scale. Patients with SCD experienced longer wait times in the ER than other patients (Haywood et al., 2013). Research suggests that longer wait times are the result of both the race of patients with SCD and their social economic status (Haywood et al., 2013). The study also showed that because SCD primarily affects Blacks, the impact of quality of care is limited. Dampier et al. (2013) used a randomized controlled trial to compare two patient-controlled analgesia (PCA) dosing strategies in adults and children with acute SCD pain. The study looked at the experiences related to protocol development, implementation, and analysis to assess the value of pain before treatment. The research used 31 clinical sites for the IMPROVE PCA (Improving Pain Management and Outcomes with Various Strategies of PCA) trial. The research study used a ‘ randomized, single blind, two-arm inpatient analgesic clinical trial randomization’’ (p. 321). Participants were 22 adults (ages $18) and 12 pediatric patients (ages 10-17) using two PCA dosing strategies. PCA opioid used during the clinical trial included morphine or hydromorphone with a higher dose demand using a low constant infusion or a lower demand using a higher constant infusion (Dampier et al., 2013). The goal for opioid use was to provide hypothetically ideal pharmacokinetics in patients with SCD. Dampier et al. used a visual analog scale to measure the duration of the patient hospitalization, using other pain measures as secondary value. Dampier et al. (2013) argued the need to optimize PCA dosing strategies for patients with SCD based on the effect of the ‘ chronic anemia on opioid pharmacokinetics’’ (p. 321). Chronic anemia increases cardiac output, which causes an increase in hepatic and renal flow. Several analgesics such as morphine use a metabolic pathway to include hepatic and renal excretion, causing accelerated plasma clearance in patients with SCD. Dampier et al. (2013) argued that conventional drug dosing in patients with SCD may result in subtherapeutic drug levels when providing continuous infusion. Therefore, continuous infusions may compensate for pharmacokinetic abnormalities in this patient population. The research recommends maintaining a steady drug level, which can be maintained by a demanddose PCA strategy. According to Dampier et al. (2013), PCA dosing is frequently used to deliver parenteral opioid analgesics and appears more efficacious than intermittent injections in SCD pain. Dampier et al.
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argued that there are only a few studies related to PCA optimal dosing to treat in postoperative settings. They said that many physicians will use PCA to treat severe vaso-occlusive pain in patients with SCD. Thus, use of PCA dosing is determined based on the influence of chronic anemia opioid pharmacokinetics in SCD. Dampier et al. (2013) concluded that maintaining a frequent demand-dose PCA strategy will provide a steady-state drug level in patients with SCD. Dampier et al.’s research confirmed the feasibility of two PCA dosing strategies in both adults and pediatric patients. Although the adults showed some improvement, pediatric participants showed slower improvement of pain control. Dampier et al. (2013) suggested the need for future studies on dosing guidelines to include participants’ ages, weights, and prior use of oral opioids. The research study provided data for issues regarding pain management in patients with SCD. Vijenthira et al.’s (2012) research examined VOC in children with SCD. The purpose of the study was to benchmark pain outcomes including process and clinical outcomes. As indicated by Vijenthira et al. (2012), managing acute pain in patients with SCD does not effectively ‘‘translate into every day practice’’ (p. 292). The researchers used a retrospective cohort from electronic patient charts (EPC) of children with SCD who met the research criteria. A convenience sample of 50 admissions for SCD VOC was used. Thus, data were analyzed utilizing SPSS software version 17.0 (Chicago, IL). The results of the research revealed that 51% of patients experienced pain in one site. The locations included the following: 41% had pain in more than one location, 37% had leg pain, 33% chest pain, 22% pain in the abdomen, and 12% reported arm pain (Vijenthira et al., 2012). Of the patients presenting to the ER, 43% had not taken pain medication before arriving at the ER, 27% had taken some form of analgesic, and the remaining 57% had taken a combination of opioids and acetaminophen, nonsteroidal anti-inflammatory drug before arrival (Vijenthira et al., 2012). Vijenthira et al.’s study demonstrated that current practice in VOC needs to improve and that a benchmark is needed when evaluating children with SCD for pain management. Using a pain assessment and documentation strategy, as well as clinical outcomes, will help identify areas for improvement in the clinical setting for hospitalized children with SCD. The research revealed a need to further investigate the practices and effect on improving pain outcomes in SCD. Often, pain remains untreated throughout the patients’ hospitalization. The research findings suggest the need to activity manage vaso-occlusive pain in children. Vijenthira et al. (2012) suggest ‘ to better manage the VOC and
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improve clinical outcomes; pain intensity, length of stay, there are three broad pain process areas to target for improvement’’ (p. 295). The research findings showed that 24% of patients with SCD pain seen in the ER had no documentation after being triage. More than 24,707 pediatric patients were seen in the ER, 55.5% of who had no documented pain score (Vijenthira et al., 2012). To combat pain in children with SCD, a pain assessment and documentation are necessary to decrease episodes. The study results indicated that adequate relief for sickle cell pain is not being achieved in children experiencing VOC during hospitalization. In an important research study, Granados and Jacob (2009) evaluated the pain in hospitalized adults with SCD using a multidimensional pain assessment tool and the amount of relief from pain medications. Clients were excluded from the study if pain episodes were not related to SCD. Participants in the research study included 8 men and 11 women with SCD, with a mean age of 33.3 years. Research data were collected over a 1-year period and clients were asked about their SCD pain every day until discharged from the hospital. Clients were asked to describe their pain using a numeric rating scale from 0 (no pain) to 10 (worst possible pain) before and after analgesic treatment. Additionally, the participants were asked to indicate on a diagram of a body where they have pain. Pain medication treatments received by the SCD patients were recorded each day of hospitalization until discharge. Granados and Jacob (2009) used descriptive statistics to describe the client’s ‘ pain location, pain quality, pain intensity, and pain relief’’ (p. 163). Pain medications included morphine 1 mg on day 1 and a loading dose of 3 mg continuous infusion per hour by PCA, and hydromorphone for 7 days using a setting of 0.4 to 2.5 mg continuous infusing per hour, and 0.2 to 3 mg demand dose with a lock interval of 5 minutes (Granados & Jacob, 2009), with a maximum dose of 2 to 7.5 mg per hour. The client’s pain relief was measured using a numeric rating scale from 0 (no relief) to 100 (complete relief). Patients who received the analgesics reported their pain was relieved. Elkins et al. (2012) conducted randomized controlled trials to determine the efficacy in the treatment of chronic pain in patients with cancer, osteoarthritis, fibromyalgia, temporomandibular disorders, noncardiac chest pain, and SCD. The study reviewed clinical techniques surrounding cognitive hypnotherapy for managing pain. According to Elkins et al. (2012), hypnotherapy involves a hypnotic induction to change one’s perception, behavior, and coping mechanism for pain management. Hypnotherapy is used to
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reduce pain in a postsession for an easy return to a more comfortable state. Hypnotherapy often provides patients with analgesia, reduces pain-related stress, relieves procedural anxiety, improves sleep and mood, and reduces patients’ need for opioids during a painful medical-related procedure (Elkins et al., 2012). Elkins et al argued the need for both clinical practice and cognitive-behavioral interventions to manage pain. Before using hypnotherapy it is important to assess the patient’s pain to determine a treatment plan. Additionally, psychologic and social factors should be assessed.
DISCUSSION Due to the limitations of the research studies, a lack of concrete evidence was connected to the set of indicators used for improvement of pain management for children with SCD pain. Other limitations included a small sample size. Vijenthira et al.’s (2012) study used a convenience sample of 50 patients with SCD and Dampier et al.’s study (2013) had only 34 participants. In part, the limitations to the research should be considered as the researchers were unable to derive specific factors from the data. These factors caused ER delays and delays in timely pain management among patients with SCD. The strengths of the articles included the importance of managing sickle cell pain among this population to reduce morbidity, rapid triage, and adequate analgesia dosing. Of the literature review, all the researchers used either a PCA or some type of analgesia to manage pain in patients with SCD. A close re-examination of managing SCD pain is required to mitigate VOCs in adolescents. Implications for Nursing Practice The results of this literature review could help to promote optimum SCD pain management in adolescents. Adolescents with SCD are at risk for acute and longterm impediments that lead to early mortality if pain management is limited. Best-practice guidelines for nurses for the management of acute pain crisis in SCD will result in better pain assessment in these patients and rapid medication treatment. Nurses serve as a catalyst for the medical doctor and the patient with SCD. It is important that nurses understand the factors associated with SCD pain. As health care providers, an open mind is essential in providing quality care. The best-practice guidelines for nurses should include the following: rapid clinical assessment of VOC; use of an age-appropriate pain scale; pharmacologic management as prescribed by a medical doctor; monitoring patient’s pain every 20 minutes until pain is controlled and stable; and monitoring vital signs,
hydration, and oxygen saturations. The implications for best-practice guidelines will help nurses to assess the patient with SCD with an open mind and not readily identify adolescent patients as drug seekers. Nurses should be able to recognize and assess SCD pain. Best-practice guidelines will also help nurses learn to reassess SCD pain and plan ongoing pain management to reduce morbidity and mortality in this population. Development of best-practice guidelines using multidimensional pain tools and pain management algorithms will help guide key pain management decisions in adolescents with SCD. Zempsky (2010) suggested that developing and implementing clinical practice guidelines will improve the care of patients with SCD. Therefore, rapid assessment and treatment of SCD pain in children and adolescents using clinical practice guidelines will improve care and reduce bias in this population. Therefore, the review is needed and will add to the body of nursing knowledge to help improve treatment of SCD VOC. Recommendations for Practice Change Evidence-based practice guidelines should be developed for consistency among health care providers, especially nurses caring for patients with SCD. The recommended changes include providing a clinical protocol for nurses caring for patients with SCD experience VOC pain. A written clinical protocol would serve as a tool for the initial patient assessment and reassessment in which prompt safe analgesia administration can be administered in an SCD VOC episode. The best-practice guidelines for patients with SCD VOC pain should include the following: assessment, diagnosis, plan, implementation, and evaluation. The assessment component should start with the initial assessment of precipitating contributory factors in patients with SCD experiencing acute pain. During the assessment, the nurse should also gather information about the patient so as to write a personalized care plan. The nursing diagnosis should be deemed as ‘‘acute pain related to SCD VOC crisis.’’ The nursing plan should consist of a pain management algorithm, an age-appropriate pain scale, documentation of severity of pain, rapid pain control, intravenous fluids, and monitoring of vital signs. Implementation of the clinical protocol should provide rapid pain control in these patients. The initial treatment of SCD VOC pain should occur within 30 minutes of admission to the ER (Glassberg, Parekh, & Zempsky, 2011). After the initial dose of opioid for acute pain, these patients need to be reassessed every 15 minutes until pain has improved. Patients with SCD who experience mild or moderate pain should be treated with a nonopioid every 2 to 4
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hours after initial treatment. For those patients experiencing acute pain, opioids should be administered every 2 to 4 hours after initial treatment as prescribed by the physician. The pain management algorithm will help nurses assess and reassess the patient’s VOC pain, thus leading to the appropriate analgesic treatment. Medication regimens for these patients should also include laxatives and antipruritics as needed while receiving opioid treatment. The final component of the clinical guidelines is evaluation. Evaluating the patient’s pain management will help determine if the analgesic treatment is effective. Managing SCD VOC pain can be difficult. Therefore, the clinical practice recommendations will contribute to nursing knowledge, and improving health outcomes for patients with SCD and their family members.
CONCLUSION SCD VOC pain continues to play an integral part in increasing morbidity and mortality. Managing SCD pain often can be complicated due to the stigma surrounding the patients with the disease. To better
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manage SCD VOC pain, nurses need more education about the disease process and the importance of managing SCD pain. Early pain intervention can reduce morbidity and mortality in these patients. Establishing best-practice clinical guidelines will serve as a tool to promote wellness and provide standards of care for clients experiencing SCD VOC pain. Moreover, nurses may benefit from continuing education related to caring for patents with SCD VOC. In terms of the study’s limitations, more research information was available on treatment of adults than adolescents experiencing SCD VOC pain. Further evidence-based practice is essential for nurses to improve clinical practice and enhance care for treatment of adolescents experiencing VOC pain. Clinical practice guidelines for SCD VOC pain will provide nurses with the basis for managing SCD VOC pain in adolescents. Researchers can expand on the current academic literature to address the stigma associated with SCD VOC pain management. Finally, research indicated analgesia management in SCD VOC pain is effective when health care providers understand the nature of the disease.
REFERENCES Dampier, C., Haywood C. Jr., & Lantos, J. (2011). A ‘‘narcotics contract’’ for a patient with sickle cell disease and chronic pain. Pediatrics, 128(1), 127–131. Dampier, C. D., Smith, W. R., Wager, C. G., Kim, H., Bell, M. C., Miller, S. T., Weiner, D. L., Minniti, C. P., Krishnamurti, L., Ataga, K. I., Eckman, J. R., Hsu, L. L., McClish, D., McKinlay, S. M., Molokie, R., Osunkwo, I., Smith-Whitley, K., & Telen, M. J. (2013). IMPROVE trial: A randomized controlled trial of patient-controlled analgesia for sickle cell painful episodes: Rationale, design challenges, initial experience, and recommendations for future studies. Clinical Trials, 10(2), 319–331. Elkins, G., Johnson, A., & Fisher, W. (2012). Cognitive hypnotherapy for pain management. American Journal of Clinical Hypnosis, 54(4), 294–310. Glassberg, J., Parekh, S., & Zempsky, W. (2011). Evidencebased management of sickle cell disease in the emergency department. Emergency Medicine Practice, 13(8), 1–24. Granados, R., & Jacob, E. (2009). Pain experience in hospitalized adults with sickle cell disease. MedSurg Nursing, 18(3), 161–182. Haywood, C., Tanabe, P., Naik, R., Beach, M. C., & Lanzkron, S. (2013). The impact of race and disease on sickle
cell patient wait times in the emergency department. The American Journal of Emergency Medicine, 31(4), 651–656. Myers, M., & Eckes, E. J. (2012). A novel approach to pain management in persons with sickle cell disease. Medsurg Nursing, 21(5), 293–298. Porter, J., Feinglass, J., Artz, N., Hafner, J., & Tanabe, P. (2012). Sickle cell disease patients’ perceptions of emergency department pain management. Journal of the National Medical Association, 104(9), 449–454, Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/23342819. Wang, C. J., Kavanagh, P. L., Little, A. A., Holliman, J. B., & Sprinz, P. G. (2011). Quality-of-care indicators for children with sickle cell disease. Pediatrics, 128(3), 484–493. Vijenthira, A., Stinson, J., Friedman, J., Palozzi, L., Taddio, A., Scolnik, D., Victor, C., Kirby-Allen, M., & Campbell, F. (2012). Benchmarking pain outcomes for children with sickle cell disease hospitalized in a tertiary referral pediatric hospital. Pain Research & Management: The Journal of the Canadian Pain Society, 17(4), 291–296. Zempsky, W. (2010). Evaluation and treatment of sickle cell pain in the emergency department: Paths to a better future. Clinical Pediatric Emergency Medicine, 11(4), 265–273.
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From the *Virginia State University, Petersburg, VA; †Indiana State University, Terre Haute, Indiana. Address correspondence to Bridget Wilson, PhD, MS, RN, Virginia State University, Petersburg, VA. E-mail: [email protected] Received December 10, 2013; Revised April 29, 2014; Accepted May 1, 2014. The authors report no financial interests or potential conflicts of interest with the submission of this manuscript. 1524-9042/$36.00 Ó 2015 by the American Society for Pain Management Nursing http://dx.doi.org/10.1016/ j.pmn.2014.05.015
Bridget H. Wilson, PhD, MS, RN,* and Jessica Nelson, DNP, RN†
ABSTRACT:
Sickle cell disease (SCD) pain continues to emerge in adolescents. More than 98,000 individuals are believed to have SCD in the United States. In fact, 1 in 500 Black infants will be affected by SCD. Identifying standards of care for this unique population can improve pain management and treatment. A significant effect of vaso-occlusive crisis is a decrease in the quality of life in children. Therefore, pain management is multidimensional and includes pharmacologic, physical, and psychological strategies. A review of the literature was conducted to identify best practices regarding pain management in adolescents with sickle cell anemia. Key words such as pain, pain management, adolescent sickle cell anemia, and acute sickle cell pain were entered into databases to reveal qualitative and quantitative studies from 2009 to the present. Many of the research articles identified poor SCD pain management. Studies showed that acute SCD pain management is essential and should be evaluated and robustly managed to achieve optimum pain relief for patients. Acute SCD pain usually occurs as a result of vaso-occlusive crisis. Untreated acute SCD pain can result in morbidity and mortality in adolescents. Nursing knowledge is critical to reducing the stigma and improving management of SCD pain. Nurses play a vital role in the introduction of evidence-based practice within the clinical setting. In an effort to educate nurses and other health care professionals about SCD, this article is a literature review of studies concerning SCD and pain management in emergency rooms. Ó 2015 by the American Society for Pain Management Nursing
BACKGROUND Sickle cell disease (SCD) is characterized as the most prevalent genetic blood disorder affecting red blood cells worldwide (Myers & Eckes, 2012). SCD is believed to affect more than 98,000 individuals in the United States alone and 1 in 500 Black infants (Porter, Feinglass, Artz, Hafner, & Tanabe, 2012; Vijenthira et al., Pain Management Nursing, Vol 16, No 2 (April), 2015: pp 146-151
SCD Pain in Adolescents
2012). SCD is characterized by recurrent, acute severe pain episodes due to vaso-occlusive crisis (VOC). The acute severe pain episode is caused by ischemic tissue resulting from blood vessel occlusion preventing blood flow (Vijenthira et al., 2012). VOC has been shown to decrease children’s quality of life. Therefore, pain management is multidimensional and includes pharmacologic, physical, and psychological strategies. According to Vijenthira et al. (2012), treating VOC pain should occur early and be aggressive to prevent morbidity. SCD patients seen in the emergency room (ER) wait at least 4 hours before receiving their first dose of pain medication (Haywood, Tanabe, Naik, Beach, & Lanzkron, 2013). SCD patients can have acute or chronic pain or a combination of both resulting in malformed sickle-shaped red blood cells. Sickle-shaped red blood cells cannot flow properly through the blood vessels and capillaries causing significant pain (Myers & Eckes, 2012). Both acute and chronic pain episodes require timely treatment. SCD patients often are seen as drug seekers once they enter the ER (Haywood et al., 2013). Many of these patients manage their pain at home; however, an average of 197,000 ER visits occur each year, with a hospital admission rate of 29% (Porter et al., 2012). The cost for SCD patients seen in the ER for VOC has been estimated to be more than $356 million. Elkins, Johnson, and Fisher (2012) indicated that pain is a ‘‘significant and ubiquitous health problem, costing productivity, employment, income, reducing quality of life, degrading relationships, and negatively affecting physical and psychological well-being’’ (p. 294). Even with the guidelines and recommendations for pain management in patients experiencing VOC episodes there continues to be a gap with providing pain control among this group. In part, 63% of nurses working with these patients believed addiction was a factor, and 30% were reluctant to administer a high dose of analgesic (Porter et al., 2012). Therefore, undertreatment of pain occurs in patients with SCD experiencing a VOC. Research by Meier and Miller (2012) suggests that although opioids were used for SCD patients with painful VOC episodes, drug dependency in SCD does not differ from other individuals in the general population without SCD. Dampier, Haywood, and Lantos (2011) indicated that drug seeking is often assumed due to the excessive use of opioids; however, it is somewhat uncommon among pediatric patients with SCD. In part, hyperalgesia from frequent opioid withdrawal or excessive use is more than likely the medical reason. Often, SCD patients are not drug seekers but are seeking care due to the lack of ‘‘psychosocial support,
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poor coping skills, and inappropriate therapeutic expectations’’ (Dampier et al., 2011, p. 128). SCD pain management should be determined and will require multiple medications in which responses are made on a variety of neurochemical pathways. Wang, Kavanagh, Little, Holliman, and Sprinz (2011) found that 6% of patients with SCD die during childhood. Wang et al. (2011) suggested there is evidence between gaps and variations in quality of care that contributes to mortality in children affected with SCD. Also, poor outcomes are a factor in the quality of care in SCD.
PURPOSE Nursing knowledge is critical to reducing the stigma and management of SCD pain. The purpose of this article is to investigate the best practices related to the management of SCD pain in adolescents. Identifying standards of care for this unique population can improve pain management and treatment. Nurses play a vital role in the introduction of evidence-based practice within the clinical setting. In part, nurses will serve as a catalyst providing scientific evidence to help them inform peers to facilitate change in adolescent SCD pain management.
SEARCH CRITERIA A comprehensive literature review was performed using multiple databases including Medline, PubMed, Cochrane Database, EBSCOhost, CINAHL, and Health Source: Nursing Academic Edition. The original search yielded 27 appropriate articles. Key terms included sickle cell disease, pain, adolescent, treatment, vasoocclusive crisis, nursing, and emergency room. Due to the limited number of retrieved studies related to SCD, other databases such as ProQuest, ProQuest Nursing and Allied Health Source were added to search areas of sickle cell pain in adolescents. This generated 4,134 studies and articles to collect the needed data for this literature review. Key terms were narrowed to include sickle cell pain, nursing, pain, management, adolescents, and vaso-occlusive crisis. These terms were entered into ProQuest and ProQuest Nursing and Allied Health databases. The results were narrowed by date of publication, to include articles published from 2009 to the present. The final nine studies selected included quantitative, qualitative, cross-sectional, randomized control trials, and comparative analysis dealing with SCD and pain management and were analyzed and selected based on sickle cell pain management.
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LITERATURE REVIEW Many of the research articles acknowledged the need for pain management for clients with SCD in a VOC. According to Wang et al. (2011), efforts have been limited in improving quality care for children with SCD in comparison with other childhood chronic illnesses. The research study focused on developing ‘‘a set of quality-of-care indicators for the management of children with sickle cell disease who are cared for in a variety of settings by addressing the broad spectrum of complications relevant to their illness’’ (p. 484). Wang et al. (2011) indicated that quality of care might contribute to poor outcomes for children with SCD. Due to the lack of quality measures for children with SCD, a limited comprehensive and interdisciplinary system of care is developed. The overall goal of the Wang et al. study was to develop quality-of-care indicators to help children with SCD when they present to the ER or outpatient and inpatient clinics, by addressing the complications associated with the disease. Wang et al. (2011) used a Delphi method to generate the final set of quality-of-care indicators. The Delphi method consisted of one round of anonymous ratings by a panel of experts, and a face-to-face panel discussion. Using this method showed the appropriateness and quality-of-care indications for patients with SCD (Wang et al., 2011). As indicated by Wang et al., children diagnosed with SCD are at higher risk for acute and long-term impediments leading to early mortality. The effort to improve and assess quality care for children diagnosed with this disease has been limited compared with children with other chronic conditions. Therefore, as clinical evidence is made available, quality-of-care indicators will change. Haywood et al. (2013) conducted research to determine wait times for patients with SCD in the ER compared with patients with long bone fracture and those with other medical conditions. They used a cross-sectional, comparative analysis of data from the National Hospital Ambulatory Medical Care (NHAMC) survey from 2003 to 2008. The research used a generalized liner model to examine the ratios of patients with SCD wait time in the ER before pain treatment. Haywood et al. (2013) indicated that more than 200,000 patients with SCD are seen in the ER each year for VOC. Their research reported that patients with SCD who are seen in the ER are dissatisfied with the quality of care received. Moreover, these patients are subject to delays and feel they wait longer to be seen by the doctor (Haywood et al., 2013). Haywood et al. pointed out that these patients wait as long as 4 hours before receiving their first dose of pain medication compared with patients with renal colic.
Haywood et al.’s (2013) research indicated that SCD-related visits totaled 171,789 that met the NHAMCS research criteria. The research revealed that patients with SCD waited longer than the general patient group. The severe pain scale used during triage showed 54% of patients with SCD rated their pain between 7 and 10 on an 11-point scale. Patients with SCD experienced longer wait times in the ER than other patients (Haywood et al., 2013). Research suggests that longer wait times are the result of both the race of patients with SCD and their social economic status (Haywood et al., 2013). The study also showed that because SCD primarily affects Blacks, the impact of quality of care is limited. Dampier et al. (2013) used a randomized controlled trial to compare two patient-controlled analgesia (PCA) dosing strategies in adults and children with acute SCD pain. The study looked at the experiences related to protocol development, implementation, and analysis to assess the value of pain before treatment. The research used 31 clinical sites for the IMPROVE PCA (Improving Pain Management and Outcomes with Various Strategies of PCA) trial. The research study used a ‘ randomized, single blind, two-arm inpatient analgesic clinical trial randomization’’ (p. 321). Participants were 22 adults (ages $18) and 12 pediatric patients (ages 10-17) using two PCA dosing strategies. PCA opioid used during the clinical trial included morphine or hydromorphone with a higher dose demand using a low constant infusion or a lower demand using a higher constant infusion (Dampier et al., 2013). The goal for opioid use was to provide hypothetically ideal pharmacokinetics in patients with SCD. Dampier et al. used a visual analog scale to measure the duration of the patient hospitalization, using other pain measures as secondary value. Dampier et al. (2013) argued the need to optimize PCA dosing strategies for patients with SCD based on the effect of the ‘ chronic anemia on opioid pharmacokinetics’’ (p. 321). Chronic anemia increases cardiac output, which causes an increase in hepatic and renal flow. Several analgesics such as morphine use a metabolic pathway to include hepatic and renal excretion, causing accelerated plasma clearance in patients with SCD. Dampier et al. (2013) argued that conventional drug dosing in patients with SCD may result in subtherapeutic drug levels when providing continuous infusion. Therefore, continuous infusions may compensate for pharmacokinetic abnormalities in this patient population. The research recommends maintaining a steady drug level, which can be maintained by a demanddose PCA strategy. According to Dampier et al. (2013), PCA dosing is frequently used to deliver parenteral opioid analgesics and appears more efficacious than intermittent injections in SCD pain. Dampier et al.
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argued that there are only a few studies related to PCA optimal dosing to treat in postoperative settings. They said that many physicians will use PCA to treat severe vaso-occlusive pain in patients with SCD. Thus, use of PCA dosing is determined based on the influence of chronic anemia opioid pharmacokinetics in SCD. Dampier et al. (2013) concluded that maintaining a frequent demand-dose PCA strategy will provide a steady-state drug level in patients with SCD. Dampier et al.’s research confirmed the feasibility of two PCA dosing strategies in both adults and pediatric patients. Although the adults showed some improvement, pediatric participants showed slower improvement of pain control. Dampier et al. (2013) suggested the need for future studies on dosing guidelines to include participants’ ages, weights, and prior use of oral opioids. The research study provided data for issues regarding pain management in patients with SCD. Vijenthira et al.’s (2012) research examined VOC in children with SCD. The purpose of the study was to benchmark pain outcomes including process and clinical outcomes. As indicated by Vijenthira et al. (2012), managing acute pain in patients with SCD does not effectively ‘‘translate into every day practice’’ (p. 292). The researchers used a retrospective cohort from electronic patient charts (EPC) of children with SCD who met the research criteria. A convenience sample of 50 admissions for SCD VOC was used. Thus, data were analyzed utilizing SPSS software version 17.0 (Chicago, IL). The results of the research revealed that 51% of patients experienced pain in one site. The locations included the following: 41% had pain in more than one location, 37% had leg pain, 33% chest pain, 22% pain in the abdomen, and 12% reported arm pain (Vijenthira et al., 2012). Of the patients presenting to the ER, 43% had not taken pain medication before arriving at the ER, 27% had taken some form of analgesic, and the remaining 57% had taken a combination of opioids and acetaminophen, nonsteroidal anti-inflammatory drug before arrival (Vijenthira et al., 2012). Vijenthira et al.’s study demonstrated that current practice in VOC needs to improve and that a benchmark is needed when evaluating children with SCD for pain management. Using a pain assessment and documentation strategy, as well as clinical outcomes, will help identify areas for improvement in the clinical setting for hospitalized children with SCD. The research revealed a need to further investigate the practices and effect on improving pain outcomes in SCD. Often, pain remains untreated throughout the patients’ hospitalization. The research findings suggest the need to activity manage vaso-occlusive pain in children. Vijenthira et al. (2012) suggest ‘ to better manage the VOC and
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improve clinical outcomes; pain intensity, length of stay, there are three broad pain process areas to target for improvement’’ (p. 295). The research findings showed that 24% of patients with SCD pain seen in the ER had no documentation after being triage. More than 24,707 pediatric patients were seen in the ER, 55.5% of who had no documented pain score (Vijenthira et al., 2012). To combat pain in children with SCD, a pain assessment and documentation are necessary to decrease episodes. The study results indicated that adequate relief for sickle cell pain is not being achieved in children experiencing VOC during hospitalization. In an important research study, Granados and Jacob (2009) evaluated the pain in hospitalized adults with SCD using a multidimensional pain assessment tool and the amount of relief from pain medications. Clients were excluded from the study if pain episodes were not related to SCD. Participants in the research study included 8 men and 11 women with SCD, with a mean age of 33.3 years. Research data were collected over a 1-year period and clients were asked about their SCD pain every day until discharged from the hospital. Clients were asked to describe their pain using a numeric rating scale from 0 (no pain) to 10 (worst possible pain) before and after analgesic treatment. Additionally, the participants were asked to indicate on a diagram of a body where they have pain. Pain medication treatments received by the SCD patients were recorded each day of hospitalization until discharge. Granados and Jacob (2009) used descriptive statistics to describe the client’s ‘ pain location, pain quality, pain intensity, and pain relief’’ (p. 163). Pain medications included morphine 1 mg on day 1 and a loading dose of 3 mg continuous infusion per hour by PCA, and hydromorphone for 7 days using a setting of 0.4 to 2.5 mg continuous infusing per hour, and 0.2 to 3 mg demand dose with a lock interval of 5 minutes (Granados & Jacob, 2009), with a maximum dose of 2 to 7.5 mg per hour. The client’s pain relief was measured using a numeric rating scale from 0 (no relief) to 100 (complete relief). Patients who received the analgesics reported their pain was relieved. Elkins et al. (2012) conducted randomized controlled trials to determine the efficacy in the treatment of chronic pain in patients with cancer, osteoarthritis, fibromyalgia, temporomandibular disorders, noncardiac chest pain, and SCD. The study reviewed clinical techniques surrounding cognitive hypnotherapy for managing pain. According to Elkins et al. (2012), hypnotherapy involves a hypnotic induction to change one’s perception, behavior, and coping mechanism for pain management. Hypnotherapy is used to
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reduce pain in a postsession for an easy return to a more comfortable state. Hypnotherapy often provides patients with analgesia, reduces pain-related stress, relieves procedural anxiety, improves sleep and mood, and reduces patients’ need for opioids during a painful medical-related procedure (Elkins et al., 2012). Elkins et al argued the need for both clinical practice and cognitive-behavioral interventions to manage pain. Before using hypnotherapy it is important to assess the patient’s pain to determine a treatment plan. Additionally, psychologic and social factors should be assessed.
DISCUSSION Due to the limitations of the research studies, a lack of concrete evidence was connected to the set of indicators used for improvement of pain management for children with SCD pain. Other limitations included a small sample size. Vijenthira et al.’s (2012) study used a convenience sample of 50 patients with SCD and Dampier et al.’s study (2013) had only 34 participants. In part, the limitations to the research should be considered as the researchers were unable to derive specific factors from the data. These factors caused ER delays and delays in timely pain management among patients with SCD. The strengths of the articles included the importance of managing sickle cell pain among this population to reduce morbidity, rapid triage, and adequate analgesia dosing. Of the literature review, all the researchers used either a PCA or some type of analgesia to manage pain in patients with SCD. A close re-examination of managing SCD pain is required to mitigate VOCs in adolescents. Implications for Nursing Practice The results of this literature review could help to promote optimum SCD pain management in adolescents. Adolescents with SCD are at risk for acute and longterm impediments that lead to early mortality if pain management is limited. Best-practice guidelines for nurses for the management of acute pain crisis in SCD will result in better pain assessment in these patients and rapid medication treatment. Nurses serve as a catalyst for the medical doctor and the patient with SCD. It is important that nurses understand the factors associated with SCD pain. As health care providers, an open mind is essential in providing quality care. The best-practice guidelines for nurses should include the following: rapid clinical assessment of VOC; use of an age-appropriate pain scale; pharmacologic management as prescribed by a medical doctor; monitoring patient’s pain every 20 minutes until pain is controlled and stable; and monitoring vital signs,
hydration, and oxygen saturations. The implications for best-practice guidelines will help nurses to assess the patient with SCD with an open mind and not readily identify adolescent patients as drug seekers. Nurses should be able to recognize and assess SCD pain. Best-practice guidelines will also help nurses learn to reassess SCD pain and plan ongoing pain management to reduce morbidity and mortality in this population. Development of best-practice guidelines using multidimensional pain tools and pain management algorithms will help guide key pain management decisions in adolescents with SCD. Zempsky (2010) suggested that developing and implementing clinical practice guidelines will improve the care of patients with SCD. Therefore, rapid assessment and treatment of SCD pain in children and adolescents using clinical practice guidelines will improve care and reduce bias in this population. Therefore, the review is needed and will add to the body of nursing knowledge to help improve treatment of SCD VOC. Recommendations for Practice Change Evidence-based practice guidelines should be developed for consistency among health care providers, especially nurses caring for patients with SCD. The recommended changes include providing a clinical protocol for nurses caring for patients with SCD experience VOC pain. A written clinical protocol would serve as a tool for the initial patient assessment and reassessment in which prompt safe analgesia administration can be administered in an SCD VOC episode. The best-practice guidelines for patients with SCD VOC pain should include the following: assessment, diagnosis, plan, implementation, and evaluation. The assessment component should start with the initial assessment of precipitating contributory factors in patients with SCD experiencing acute pain. During the assessment, the nurse should also gather information about the patient so as to write a personalized care plan. The nursing diagnosis should be deemed as ‘‘acute pain related to SCD VOC crisis.’’ The nursing plan should consist of a pain management algorithm, an age-appropriate pain scale, documentation of severity of pain, rapid pain control, intravenous fluids, and monitoring of vital signs. Implementation of the clinical protocol should provide rapid pain control in these patients. The initial treatment of SCD VOC pain should occur within 30 minutes of admission to the ER (Glassberg, Parekh, & Zempsky, 2011). After the initial dose of opioid for acute pain, these patients need to be reassessed every 15 minutes until pain has improved. Patients with SCD who experience mild or moderate pain should be treated with a nonopioid every 2 to 4
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hours after initial treatment. For those patients experiencing acute pain, opioids should be administered every 2 to 4 hours after initial treatment as prescribed by the physician. The pain management algorithm will help nurses assess and reassess the patient’s VOC pain, thus leading to the appropriate analgesic treatment. Medication regimens for these patients should also include laxatives and antipruritics as needed while receiving opioid treatment. The final component of the clinical guidelines is evaluation. Evaluating the patient’s pain management will help determine if the analgesic treatment is effective. Managing SCD VOC pain can be difficult. Therefore, the clinical practice recommendations will contribute to nursing knowledge, and improving health outcomes for patients with SCD and their family members.
CONCLUSION SCD VOC pain continues to play an integral part in increasing morbidity and mortality. Managing SCD pain often can be complicated due to the stigma surrounding the patients with the disease. To better
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manage SCD VOC pain, nurses need more education about the disease process and the importance of managing SCD pain. Early pain intervention can reduce morbidity and mortality in these patients. Establishing best-practice clinical guidelines will serve as a tool to promote wellness and provide standards of care for clients experiencing SCD VOC pain. Moreover, nurses may benefit from continuing education related to caring for patents with SCD VOC. In terms of the study’s limitations, more research information was available on treatment of adults than adolescents experiencing SCD VOC pain. Further evidence-based practice is essential for nurses to improve clinical practice and enhance care for treatment of adolescents experiencing VOC pain. Clinical practice guidelines for SCD VOC pain will provide nurses with the basis for managing SCD VOC pain in adolescents. Researchers can expand on the current academic literature to address the stigma associated with SCD VOC pain management. Finally, research indicated analgesia management in SCD VOC pain is effective when health care providers understand the nature of the disease.
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