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Maria, a 3-year-old Caucasian female, was brought to the emergency room after two weeks of a dry cough that became productive and bloody, with occasional post-tussive bloody emesis. She was pale and lethargic with tachycardia, hypotension, and dry mucous membranes, and had significant bruising on her bony prominences.
Past Medical History
Maria's past medical history re vealed a ventricular septal defect (VSD) diagnosed at birth that closed without intervention. Her weight was 14.1 kg, which is at the 90th percentile for weight on the plotted Centers for Disease Control and Prevention (CDC) growth chart, as is her height. She has seasonal allergies, which are treated with loratadine (Claritin) for symptomatic relief. She appeared well nourished and well developed, and her mother reported Maria had met all of her developmental milestones. She lives with her parents in an apartment without pets, and nobody smokes. Both her 8-year-old sister and 2-month-old brother are healthy. Her immunizations are up to date, and she has routinely received well child care.
Assessment Findings in the Emergency Room
Vitals: Afebrile; HR - 160; BP - 64/40; RR - 40; O2 sat. - 100% on room air.
HEENT: Oropharynx is pale, slightly dry, and without lesions; cervical and sub-mandibular lymph nodes are en larged at approximately 1 cm.
Cardio-Respiratory: Breath sounds are clear and equal bilaterally; heart sounds are normal S1/S2 split without murmur; distal pulses are weak; capillary refill is slow at 4 seconds.
Abdomen: Abdomen is tender and distended with 48 cm abdominal girth; spleen is palpable.
Neurologic: Pupils are equal and reactive; Maria is irritable and combative with interventions but easily consoled by her mother.
Initial Management Plan
After initial laboratory studies, placement of a peripheral IV, and fluid boluses with normal saline, Maria was admitted to the Pediatric Intensive Care Unit for further stabilization. Her complete blood count (CBC) results were striking, with a white blood count (WBC) of 164,000, hemoglobin (Hgb) of 4, and hematocrit (Hct) of 16. Her platelet count was 17,000. In addition, the laboratory reported 16 nucleated red blood cells (RBCs), 2 atypical lymphocytes, 2 metamyelocytes, 3.6 myelocytes, 5 promyelocytes, and 8 immature blasts.
Because of the significant anemia, Maria's blood was typed and cross-matched. She subsequently received a transfusion of packed RBCs at 20 cc/kg with additional platelets. Until blood products were available, dopamine was started at 10 mcg/kg/minute and titrated to 20 mcg/kg/minute for hypotension. Maintenance fluids were started at D5 ½ NS at 30 cc/hour and increased to 75 cc/hour. Oxygen was administered per nasal cannula to keep saturations greater than 92%. An indwelling catheter was placed for urine output measurement. To make an accurate diagnosis, a plan was developed to sedate Maria so that a bone marrow aspirate and lumbar puncture could be safely performed. Prior to the procedure, Maria was hemodynamically stable with a HR of 100 and BP of 90/40. What medications and what doses would you expect to be ordered to appropriately sedate Maria for these procedures?
Continuing Management Plan
Maria received midazolam (Versed) 2 mg, fentanyl (Actiq, Duragesic) 10 mcg, and ketamine (Ketalar) 5 mg IV in preparation for the bone marrow aspiration and spinal tap. The desired sedation was not achieved with these initial doses. Maria was extremely agitated, crying, and thrashing. An additional 10 mcg of fentanyl and 5 mg of ketamine were added, but Maria continued to cry and thrash about in the bed. These failed attempts to sedate Maria prevented timely diagnostic testing. Therefore, her physicians decided to complete the procedures while physically restraining Maria. Much to the distress of those who assisted, Maria thrashed and moaned throughout both the lumbar puncture and bone marrow aspirate. At the end of the procedure, her mother was crying and talked about removing Maria from the hospital. The staff was also distressed by failing to provide adequate sedation and analgesia.
Pathophysiology of Procedural Sedation and Pain Management
Children undergoing painful procedures, such as bone marrow aspiration and spinal taps, need to have aggressive sedation and analgesia for safe and effective control of pain, anxiety, and motion. Without adequate sedation and analgesia, these procedures are traumatic for the child, witnessing family, and staff. Additionally, when a painful stimulus is repeated over time, sensitization may occur. Sensitization is an increased reaction to subsequent painful stimulus and/or a lowered pain threshold (von Baeyer, Marche, Rocha, & Salmon, 2004). Sensitization effects have been reported to be so dramatic with bone marrow aspirations and lumbar punctures that use of opioids was ineffective for future procedures in children younger than 8 years of age (Weisman, Bernstein, & Schechter, 1998). The age and developmental status of the child are critical components of the plan to manage painful procedures. Younger children who have limited cognitive ability to understand what is being done and the rationale for it experience increased fear and anxiety. Therefore, the plan for younger children should include anticipated use of maximum levels of medications as well as use of non-pharmacological supports.
Combinations of midazolam, ketamine, and fentanyl are deemed by many professionals to be ideal for conscious sedation due to their rapid onset and short duration of action (Borker, Ambulkar, Gopal, & Advani, 2006; Evans et al., 2005; Mason, 2008; Slonuim & Ognibene, 1998). Midazolam is a short-acting benzodiazepine that provides sedation, amnesia, and anxiolysis, but no analgesia (Borker et al., 2006). Its main side effects are respiratory depression and hypotension, which may occur in patients with hemodynamic instability. The recommended dose for midazolam is 0.1 to 0.2 mg/kg (Taketomo, Hodding, & Kraus, 2003). Ketamine is a dissociative anesthetic that induces a trance-like state accompanied by sedation, amnesia, analgesia, and immobilization (Borker et al., 2006). Ketamine can cause undesired hallucinations and respiratory depression, and must be used carefully in patients with increased intracranial pressure. Initial recommended dose for ketamine is 1 to 2 mg/kg (Taketomo et al., 2003) Fentanyl is a short-acting synthetic opioid with sedative effects used for pain control. Rapid IV infusion can cause skeletal muscle and chest wall rigidity, impairing ventilation. It can also cause respiratory depression. Fentanyl is dosed at 1 to 3 mcg/kg (Taketomo et al., 2003)
Appropriate initial dosing of medication will usually produce sedation along with anxiolysis and pain control. For Maria, an initial dose of 2 to 3 mg of midazolam, 14 to 42 mcg of fentanyl, and 14 to 28 mg of ketamine would be appropriate. Doses lower than these recommendations were given to Maria. Midazolam, ketamine, and fentanyl should be given slowly according to each medication manufacturer's directions. It is also important to allow at least 3 to 5 minutes for initiation of desired effects before the procedure begins. Safety and efficacy should always be considered no matter how rushed the atmosphere is (Mason, 2008). These 5 minutes can seem like a long time in an urgent situation. However, rushing to perform the procedure before the medications are working prevents the needed analgesia and sedation. Inadequate sedation resulting in the cancellation of a procedure or use of restraints in a crying child may occur as frequently as 30% of cases depending on technique, provider, and medications used (Blike & Cravero, 2001).
Underdosing of medications or failing to allow time for the medications to take effect results in failure to meet the goals of sedation, including ideal positioning for the procedures, managing the child's anxiety and stress, and pain control. Although initial doses are recommended, additional medication may be needed to achieve sedation. Nursing staff should have these doses immediately available. Dosing to effect and considering each child's unique physiology and risk factors constitute the art of sedation management. In addition, the nurse must be prepared for repeated dosing should the procedure extend beyond the time of maximal effect of the medications. Nurses should also calculate and have immediately available the appropriate reversal agents for all sedative medications used.
Another medication commonly used for procedural sedation is propofol (Lalwani & Michel, 2005). Propofol (Dipravan) is a short-acting intravenous general anesthetic. It should be avoided for children who are allergic to egg and soy products. Propofol at high doses or used for prolonged periods of time has been known to cause metabolic acidosis leading to cardiac failure. The onset of action is within 30 seconds after bolus infusion with duration of action from 3 to 10 minutes. Recommended method of administration includes giving a bolus of 1 to 2 mg/kg followed by an infusion of 8 to 12 mg/kg/hr (Taketomo et al., 2003). Propofol can cause hypotension; therefore, either an intravenous normal saline infusion accompanies propofol or a volume bolus is prepared and ready to administer. Propofol would not have been appropriate for Maria on her initial admission to the PICU due to her hemodynamic instability, but once stabilized with fluids and blood transfusions, it could be considered. Propofol is considered a safe appropriate medication for procedural sedation in critical care (Vespasiano, Finkelstine, & Kurachek, 2007).
Local anesthetics should also be considered in the plan for procedural sedation and analgesia. The effect of local anesthetics is to block nerve conduction, preventing the transmission of painful stimuli. Local anesthetics can be injected, or applied topically or by dermal iontophresis (McCaffrey & Pasero, 1999). Avoid use of agents to which the child is allergic and allow adequate time for the desired effect to occur.
Adequate monitoring of the child receiving medications for sedation and a plan to address potential side effects, such as hypoxia, change in vital signs, vomiting, dizziness, and emergence reaction, is always required. Reversal agents should be readily available with appropriate doses pre-calculated. Although over-sedation is a concern for using medications to obtain appropriate levels of sedation, these critical events are rare (Blike & Cravero, 2001). Standards to ensure patient safety include an interval of fasting, pre-sedation assessment, appropriate medical staff supervision, access to age and size-appropriate airway, and resuscitation equipment and medications, and continuous cardio-respiratory monitoring with pulse oximetry until the child returns to baseline. Skilled medical professionals must focus their exclusive attention to continuous patient monitoring while the procedure is performed. "Immediate rescue" equipment, such as a resuscitation bag, mask, and airway management equipment, is vital (Committee on Drugs & the American Academy of Pediatrics [AAP], 2002).
The extra minutes taken to prepare a child with simple explanations and practice techniques for distraction or relaxation, such as deep breathing, guided imagery, or art therapy, may make procedures more successful and compassionate (Favara-Scacco, Smime, SchilirĂ², & DiCataldo, 2001). Child life specialists in peri-operative settings, oncology clinics, emergency departments, and other health care settings have helped children anticipate and manage acute health care experiences. Interventions from child life specialists lower the child's anxiety, increase comfort levels, calm fears, and lead to higher patient/family satisfaction. A child life specialist can also enhance the parents' ability to support their child by providing direction or modeling. Use of child life staff allows the other health care team members to use their time more efficiently (American Academy of Pediatrics [AAP] Child Life Council and Committee on Hospital Care & Wilson, 2006).
The Rest of the Story
Maria was transferred to the Hematology Oncology Unit for an inpatient stay to begin her chemotherapy treatment for acute myelogenous leukemia (AML). Her parents were overwhelmed by the fast-paced diagnosis and treatment of AML, and were also very anxious about future painful tests and interventions for their daughter. Maria was too young to understand the meaning of her diagnosis or the work of the health team. She required constant comforting from her parents and the nursing staff. Child life specialists quickly became an important aspect of her treatment and care while in the hospital.
Lessons Learned
While techniques for procedural sedation can be described in a protocol, successful sedation and pain management for compromised children is an art that requires collaboration and coordination of the entire health care team. Constant vigilance and assertive nurse-physician collaboration are required. This is particularly true for a child who will undergo repeated cancer treatments. Maria's first experience with bone marrow aspiration and lumbar puncture set the tone for future frequent medical experiences. Nurses contribute to the art of procedural sedation by assuring that the child's psychosocial needs are met during a stressful or emergent medical situation. Working collaboratively with child life specialists, physicians, and the family, nurses can address the emotional needs of the child during initial painful procedures. Successful sedation, analgesia, and amnesia for initial painful procedures facilitate building a therapeutic, trusting relationship with health care personnel for subsequent painful experiences and hospitalizations.
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