1042 e3 SICKLE CELL PAIN ASSESSMENT Determine type of pain (onset, duration, frequency) Acute or chronic? Acute Chronic (frequently occurring acute or mixed acute superimposed on chronic) Brief or per[.]
1042.e3 SICKLE CELL PAIN ASSESSMENT Determine type of pain (onset, duration, frequency) Acute or chronic? Acute Chronic (frequently occurring acute or mixed acute superimposed on chronic) Brief or persistent? Conduct comprehensive assessment Brief Treatment history Persistent - Frequency of painful episodes in previous year Determine characteristics, location, and intensity based on self-report Determine characteristics, location, and intensity based on self-report and observation - Number of ED visits in past year - Frequency and duration of hospitalizations - Pain medication history - Current medication regimen Determine related symptoms Physical factors Demographic/ psychosocial factors Dimensions of pain - Intensity of 0– 10 scale: - Blood pressure - Age - Heart rate - Gender Mild (0–3) - School/work - Respiration - Developmental level Moderate (4–6) - Social activities - Oxygen saturation level - Chest/ abdomen - Family factors Severe (7–10) - Characteristics: - Cultural factors Location - Pain sites - Adaptation to SCD Quality - Tenderness - Coping styles - Warmth - Cognitive abilities - Swelling - Lab/x-ray data Precipitating factors - Mood - Level of distress Determine probable cause(s) Related to SCD? YES Treat based on characteristics of episode NO Conduct complete work-up to determine etiology of pain Impact of pain on functioning Summarize, assimilate, and prioritize profile Identify appropriate interventions based on comprehensive assessment • eFig 88.8 Pain assessment approach to sickle cell pain ED, Emergency department; SCD, sickle cell disease (From Center for Children with Special Needs Sickle Cell Disease—Critical Elements of Care 5th ed Seattle: Seattle Children’s Hospital; 2012.) - Self-care - Relationships - Parent ability (adults) CHAPTER 88 Hemoglobinopathies 1043 particularly difficult, as there are numerous etiologies (e.g., vasoocclusive events [VOEs], dactylitis, avascular necrosis, ACS, priapism, splenic sequestration and infarctions, hepatic crisis, gallstones, and leg ulcers) and pathways involved (e.g., vasoocclusion, inflammation, and alterations in pain processing) leading to acute and chronic pain.24–33 VOEs, defined as the acute onset of severe pain due to ischemia and reperfusion injury, are a hallmark of SCD The complexities of SCD pain are best summarized by Shapiro and Ballas: “Vasoocclusion is a physiologic process, but the resultant pain is a biopsychosocial phenomenon Psychosocial issues such as coping skills, social context, personality, mood, and interactions with the health care system mingle with the biologic factors and contribute to the expression of the illness.”34 In the intensive care unit (ICU), there must be an awareness of potential alterations in pain processing as well as awareness of the acute, neuropathic, inflammatory, psychologic, and sociocultural components of pain Pain can be acute, recurrent, or chronic, and it is complicated by coexisting chronic disease and racial overlays.23–28,35 Too often, racial attitudes and concerns of drug seeking prevent sufficient medication delivery to patients in excruciating pain.35,36 The need for aggressive and rapid treatment is critical and well documented in guidelines of the American Pain Society and British Society of Hematology and is essential for humane care and physiologic improvements (e.g., improving respiratory mechanics when having rib infarction pain).24,37–39 Pathophysiology, Diagnosis, and Presentation I ndividual risk factors for VOE pain include a higher WBC count, lower HbF, coexisting a-thalassemia, and older age.33 Physiologic factors that may lead to VOE include Hb polymerization, rheology of RBCs, cellular dehydration, RBC deformability and fragility, whole blood viscosity, WBC activation, endothelial factors, adhesion of RBCs to the endothelium, hemostatic factors, altered NO metabolism, and vascular factors.4,12–14,29–33,40 Vessel occlusion results in ischemic/reperfusion injury and the release of multiple inflammatory mediators that activate nociceptors, evoking a pain response Recurrent episodes lead to altered pain processing, making assessment and management more complex Diagnosis is based on a detailed qualitative description (typically, two components: a deep fatiguing, unrelenting ache with a component of biting, gnawing, or throbbing) and location (eFig 88.9) The description is essential to help rule out other etiologies of pain, as there is no definitive test to differentiate sickle cell pain from other sources of pain Physical findings can include swelling, warmth, erythema, and tenderness However, most commonly, examination of the involved area may be entirely normal and, despite excruciating pain, patients can appear in no apparent distress There is no laboratory or radiologic study that can validate the existence or absence of a VOE Therefore, one must trust the patient’s report of pain It is essential to perform pain assessments every several hours using a developmentally appropriate pain scale, adjusting the pain plan as necessary to provide adequate analgesia As pain becomes more chronic, goals of treatment should shift toward maintaining function and maximizing quality of life Management S ee eFig 88.6 for a detailed care plan and eFigs 88.7 and 88.8 for overviews of acute pain management.22 An effective management strategy considers whether the pain is acute or chronic Factors include the underlying tissue damage and nociception and the history of pain episodes, doses of medications required to achieve acceptable analgesia, baseline pain medications, history of tolerance, mental state, how the patient processes pain, coexisting depression, and the presence of anxiety or fear A multimodal approach is outlined in eFigs 88.7 and 88.8, but essential components include (1) environmental manipulation; (2) complementary methods; (3) addressing pathophysiology and triggers (fluids to maintain euvolemia, warmth, and NSAIDs to decrease CTT); and (4) adjunctive interventions, such as physical therapy, ambulation, and incentive spirometry to maintain blood flow and prevent atelectasis and ACS After these issues have been addressed, providers may focus on opiates and additional medications Initial pharmacologic management of severe acute pain episodes includes the initiation of around-the-clock scheduled NSAIDs (many children will have resolution of pain with intravenous [IV] ketorolac41) followed by rapid and repeated doses of opiates (dosed every 20 minutes), transitioning to continuous infusion or patient-controlled analgesia (if developmentally appropriate).24,37–39,42 Although the absence of pain is not a goal, the aim is to relieve pain and suffering and allow rest and health-promoting activities (incentive spirometry and ambulation to reduce the risk of ACS) while avoiding oversedation, which can contribute to the development of pulmonary complications As cannabinoids have multiple effects on inflammation and neuropathic components of pain and can attenuate hyperalgesia, there is strong rationale for its use in SCD, although this remains controversial socially.43 Use of l-arginine (a precursor of NO) has resulted in a 50% reduction in overall opiate use in SCD,44,45 and there is increasing evidence for the use of glutamine.46 Sepsis. Detailed sepsis care plans are presented in eFigs 88.10 and 88.11.22 Sepsis has historically been a major cause of morbidity and mortality in SCD In the 1970s, 20% of patients with SCD in the United States died before age years, primarily from sepsis with encapsulated organisms, particularly Streptococcus pneumoniae.8 Since that time, the initiation of universal newborn screening in the United States has facilitated identification of affected infants, allowing for early initiation of prophylactic penicillin and the delivery of anticipatory guidance to families regarding fever management and immunizations Because of these interventions, deaths from sepsis in children with SCD have plummeted.47,48 Currently, the presence of a central line and a history of surgical splenectomy are two of the biggest risk factors for bacteremia.49,50 Pathophysiology and Etiology ocalized and recurrent infarctions in the spleen lead to the develL opment of functional splenia at an early age in children with SCD Functional asplenia, a finding observed as early as months of age, places the child at markedly increased risk of overwhelming sepsis, particularly from encapsulated organisms Reduced clearance of encapsulated organisms results from defects in cellular immunity, the alternate complement pathway, a decrease in memory cells, and opsonizing antibodies Children with SCD have a 100 to 400 times increased risk of bacteremia from encapsulated pathogens Management e pathophysiology, diagnosis, and management of sepsis are Th presented in Chapter 110 Several aspects of sepsis that are unique to SCD are noted in eFig 88.11 Acute Chest Syndrome Table 88.2 and eFig 88.12 summarize detailed care plans.22 Although definitions of acute chest syndrome vary, the most general one is a new nonatelectatic infiltrate on chest radiograph in a patient with SCD, though more stringent definitions include the requirements of fever, full lung segment involvement, and respiratory 1043.e1 • eFig 88.9 Self-depiction of a 6-year-old’s sickle cell pain (From Center for Children with Special Needs Sickle Cell Disease—Critical Elements of Care 5th ed Seattle: Seattle Children’s Hospital; 2012.) 1043.e2 Fever and sepsis Signs/symptoms Work-up Temp 38.3°C (101.0°F) or greater, or a temp greater than 38.0°C (100.4°F) for more than hours Physical exam, vital signs, evidence of systemic or localized infection, cardiopulmonary assessment, spleen size, and neurologic exam Blood culture Poor appetite CBC, retic count, platelets Fussy Low threshold for a chest X-ray Lethargic Culture other bodily fluids as clinically indicated A Obvious infection; or A Not Ill-appearing B Ill-appearing; or B Lab evaluation lacks high-risk features C WBC >30,000 or 39°C (102.2°F) E Age