Addressing Pain in Children with Serious Illness

1. Addressing Pain in Childrenwith Serious Illness Jamie Sharpe, APRN, ACHPN Palliative Care NP Kapi‘olani Medical Center for Women and Children

2. At the completion of this module, the participant will be able to: 1. Describe prevalence of pain, challenges and barriers to identifying pain in pediatric populations 2. Describe tools and techniques for assessing pain in children 3. Identify pharmacologic and non-pharmacologic strategies for pain management Objectives CREATING A HEALTHIER HAWAIʻI

3. Palliative Care provides interdisciplinary services to prevent or relieve physical, psychological, social and spiritual distress from life-threatening or life-limiting conditions or their treatment; to help patients and families live as normally as possible; to provide them with timely and accurate information and to support decision making. But first…..what is Pediatric Palliative Care? CREATING A HEALTHIER HAWAIʻI

4. Palliative Care can be helpful at any point in an illness or condition that could be potentially life-threatening or life-limiting. It can help improve qualify of life for patients and families as they navigate through difficult serious illnesses. PC can be concurrent with curative or life-prolonging therapy. Palliative care is not limited to the imminently dying or terminal CREATING A HEALTHIER HAWAIʻI

5. Cure is possible but may fail (i.e. advanced cancer) • Premature death is likely but treatment can prolong quality and quantity of life (CF, sickle cell disease, etc) • Progressive condition without curative options where treatment is exclusively palliative in nature but may extend life (i.e. muscular dystrophy) • Conditions that are not progressive but render a child vulnerable to life-limiting complications (medically fragile, cerebral palsy, etc) Pediatric Populations Appropriate for Palliative Care CREATING A HEALTHIER HAWAIʻI

6. Now…. Let’s Talk About Pain

7. Pain is a subjective response • Pain in childhood can be acute, chronic or a combination of both • Children’s pain is influenced by many factors Pain and Children ELNEC 2016 CREATING A HEALTHIER HAWAIʻI

8. Children’s pain experience • 25-46% experience some level of pain <3 months duration • Up to 30% experience chronic or recurrent pain severe enough to interfere with daily functioning • Effect of pain on quality of life Scope of the Problem CREATING A HEALTHIER HAWAIʻI

9. Physical Well-Being Psychological Well-Being Social Well-Being Spiritual Well-Being Model of Quality of Life CREATING A HEALTHIER HAWAIʻI

10. Health professionals • Myths related to assessment of pain • Healthcare system • Related to parents/children • Personal/cultural beliefs • Lack of knowledge/fear Barriers to Pain Relief for Children CREATING A HEALTHIER HAWAIʻI

11. Risk of respiratory depression Opioid pain control leads to addiction Children become accustomed to pain Children will always tell you when they are having pain A child that is sleeping/or playing does not have pain Presence of pain indicates worsening of disease or approaching death Early use of opioids leaves no future options Myths Related to Pain and Pain Management in Children CREATING A HEALTHIER HAWAIʻI

12. Opioid addictions are rare Repeated exposure to painful procedures leads to increased anxiety and perception of pain Studies have shown that children as young as 3 years old can use self-report pain scales. There is no ceiling dose for opioids Children may be afraid to tell about pain Important to employ verbal and nonverbal cues Facts about Childhood Pain Carter et al., 2011; Collins et al., 2011; Goldman et al., 2012; Hockenberry & Wilson, 2011 CREATING A HEALTHIER HAWAIʻI

13. Psychological disorder with behavioral pattern • Not a pharmacological side effect • Risk factors for addiction to consider: • family dysfunction, family history of substance abuse • personal history of substance abuse including alcohol, illegal or prescription drugs • stressful life events, especially history of preadolescent sexual abuse • psychological disorders: ADD, OCD, bipolar, schizophrenia, depression • age 16 and up to 43 Addressing Fears – Addiction Risks CREATING A HEALTHIER HAWAIʻI

14. Pain acts as a natural opioid antagonist Concern about respiratory depression is usually out of proportion with actual outcomes – in reality it is rare in children Should not prevent appropriate/adequate pain management Frequent assessment/monitoring in hospital setting At home, appropriate teaching for patient and family including dose, managing overdoses, pain logs Patients most at risk: preexisting respiratory compromise, obesity, developmental delays and those receiving other sedating medications Respiratory Depression CREATING A HEALTHIER HAWAIʻI

15. Previously treated for pain with injections or painful procedures Has been encouraged to “be brave” Lacks understanding that pain can be treated Afraid of medications side effects or addiction Worried that if still in pain they won’t be discharged as planned May believe that tubes (such as NG) won’t come out until pain medications stopped Why would a child deny pain? CREATING A HEALTHIER HAWAIʻI

16. Cancer pain • Disease, treatment & procedure related • Chronic non-malignant pain • Sickle cell disease, diabetes, rheumatoid arthritis, HIV, cystic fibrosis, neurological degenerative diseases • Musculoskeletal/rheumatic • Juvenile Primary Fibromyalgia • Juvenile Idiopathic Arthritis • Complex Regional Pain Syndrome Special Populations CREATING A HEALTHIER HAWAIʻI

17. Neurocognitive Impairment • Pain experience • Pain indicators • Effect of uncontrolled pain • Assessment • Knowing the child • Recognizing their patterns • Intersubjective process with health care providers It is important to assess the patient by developmental or cognitive stage, NOT chronological stage Special Populations CREATING A HEALTHIER HAWAIʻI

18. Self-report or parent/proxy report Behavioral Physiologic Intensity, Quality, Pattern, Aggravating/Alleviating factors Medication history Meaning Multidimensional Assessment CREATING A HEALTHIER HAWAIʻI

19. Pre-verbal / nonverbal (examples) • FLACC (Face, Legs, Activity, Cry, Consolability), Pain Observation Scale, Modified Objective Pain Score • Non Communicating Children's Pain Checklist (NCCPC) • Self-Report Pain Intensity Scales • FACES Pain Scale- Revised (FPS-R), Visual Analog Scale (VAS) Pain Assessment Tools See Supplemental Materials for more specific information CREATING A HEALTHIER HAWAIʻI

20. Motor • Rigidity/thrashing/arching • Fetal position/restlessness/ sleep • Communication • Intense crying (high-pitched), grunting • Facial expression • Grimacing, brows lowered and drawn together • Other behaviors • Hypersensitive/inconsolable, poor oral intake, shut down/ sleep • Age-appropriate assessment tool: FLACC Infants CREATING A HEALTHIER HAWAIʻI

21. FLACC CREATING A HEALTHIER HAWAIʻI

22. Motor • Clingy, restless/irritable, physically aggressive to others • Communication • Verbally aggressive, loud/fearful cry • Facial expression • Furrowed brow, tense • Other behaviors • Regressive behavior, uncooperative • Decreased periods of sleep or overall increased sleep time • Age-appropriate assessment tool: FACES, FLACC Toddlers CREATING A HEALTHIER HAWAIʻI

23. Motor • Rigid/guarding, can point to “hurt” • Communication • Verbalize pain occurrence, can describe intensity • Facial expression • Tense, furrowed brow • Other behaviors • May view pain as punishment, learn of secondary gain from pain • Age-appropriate assessment tool: FACES scale Preschoolers CREATING A HEALTHIER HAWAIʻI

24. Motor • Resist movement, localize pain • Communication • Verbalize pain, use cultural expressions, use objective measurements • Facial expression • May appear sad, tense • Other behaviors • Difficulty sleeping, nightmares • Age-appropriate assessment tool • Visual analog scale, verbal 0–10 numerical scale School-Age Children CREATING A HEALTHIER HAWAIʻI

25. Motor •  muscle tension or tremors, flinches • Communication • Very specific about pain qualities, may deny pain in front of peers • Facial expression • Frowning/grimacing, may avoid eye contact • Other behaviors • Anger/sadness, difficulty sleeping, regressive behavior with family • Age-appropriate assessment tool • Verbal 0-10 numeric scale Adolescents CREATING A HEALTHIER HAWAIʻI

26. Types of Pain Nociceptive Pain • - Normal pain transmission • Occurs in presence of tissue damage or inflammation • Serves to warn & protect individuals from further injury • Will typically respond to NSAIDs & opioids CREATING A HEALTHIER HAWAIʻI

27. Neuropathic Pain - Abnormal pain transmission • May occur in the absence of tissue damage or inflammation • Believed to serve no purpose or warn against further injury • Best treated with co-analgesics (i.e., anticonvulsants & tricyclic antidepressants) • Seen in: complex regional pain syndrome, amputation, nerve trauma, Guillain-Barré syndrome Types of Pain CREATING A HEALTHIER HAWAIʻI

28. Rapid assessment and treatment of pain is imperative Close collaboration with providers and family is essential to optimum use of analgesic treatments. Assess pain regularly and change your plan accordingly Always integrate non-drug strategies in combination with medications to enhance pain control Pain Management CREATING A HEALTHIER HAWAIʻI

29. Opioid medications should be given on scheduled basis if possible • PLUS adequate PRN doses for breakthrough pain (not PRN only) • Designed to control baseline pain Around the Clock Dosing CREATING A HEALTHIER HAWAIʻI

30. Individualize to the child based on their level of pain, prior experience with opioids, and desired activity level Frequently assess pain level and adjust treatment plan as necessary In pain crisis - rapid titration to comfort is imperative Stay Ahead of Pain CREATING A HEALTHIER HAWAIʻI

31. Oral vs IV In toddler/early childhood compliance with oral effected by flavor Avoid rectal No IM ‘SHOTS’ Use Simplest and Least Invasive Route CREATING A HEALTHIER HAWAIʻI

32. Acetaminophen • Useful for mild pain, anti-inflammatory action • Works synergistically with morphine • q 4-6 hrs PO/PR, q6 hrs IV Non-opioids • Ibuprofen • Mild, moderate, acute, or chronic pain alone • For severe pain in combination with opioid + adjuvant • Treatment of bone pain, inflammation, mild to moderate pain and fever • q 6-8 hrs CREATING A HEALTHIER HAWAIʻI

33. Toradol (ketorolac) IV q 6-8 hrs • Recommended for no longer than 5 days Celebrex (celcoxib) PO q 12-24 hrs • If classical NSAIDs contraindicated • Safety and efficacy not established in children less than 2 years of age Non-opioids CREATING A HEALTHIER HAWAIʻI

34. Gastric irritation, heart burn, ulceration, and bleeding • Use gastroprotective for prolonged use (PPI, ranitidine) • Effect on platelet aggregation: short-acting, reversible • Renal effects: rare, insufficiency and nephrotoxicity can occur with prolonged high doses Management of NSAID Side Effects CREATING A HEALTHIER HAWAIʻI

35. Tramadol (age 12 and up) • Some may be rapid metabolizers leading to inc risk respiratory depression/death • Contraindicated pts <18 yo post tonsillectomy • Has ceiling effect. If using >8mg/kg/day change to strong opioid. Do not combine with strong opioids • Monitor for dizziness and hypoglycemia • Lowers seizure threshold or precipitate serotonin syndrome Treatment of Moderate Pain CREATING A HEALTHIER HAWAIʻI

36. Codeine – up to 34% of children gain no analgesic effect due to poor metabolism, on other hand rapid metabolizers can produce dangerously high levels Acetaminophen combination products such as Tylenol #3, Vicodin (hydrocodone/APAP) or Percocet (oxycodone/APAP) as dosing cannot be increased if increasing pain without risking associated liver toxicity with high doses of APAP Medications NOT recommended CREATING A HEALTHIER HAWAIʻI

37. Morphine • Variety of routes, formulations (IV, SC, PO/SL, PR) • Used for moderate to severe/intractable pain Fentanyl – for severe pain • Used in anesthesia, procedural sedation - IV • Bolus IV dosing or continuous infusion • Patch has been found useful in some cancer and chronic non-malignant pain (age 1 and older) Moderate to Severe Pain - Opioids CREATING A HEALTHIER HAWAIʻI

38. Hydromorphone • More potent than morphine • Good alternative if morphine or oxycodone cannot be used • Oxycodone • PO/SL • Long-acting approved for age 11 and older Opioids - Continued CREATING A HEALTHIER HAWAIʻI

39. Low dose naloxone infusion IV • 0.5 - 2 mcg/kg/hrto reverse opioid-induced side effects such as pruritis, nausea, etc (if moderate to severe: opioid rotation) • Naloxone IV/SC • 1-5 mcg/kg to reverse opioid-induced depressed respiratory rate • Consider for oversedation ONLY if conservative measures show no effect (tactile stimulation) • 10 mcg/kg to reverse opioid induced apnea and coma; titrate to effect Opioid antagonists and side effect management CREATING A HEALTHIER HAWAIʻI

40. Less research and EBP for peds due to less incidence in children than in adults • Neuropathic pain • Antidepressants: amitriptyline 0.5-2 mg/kg PO qhs • Anticonvulsants: gabapentin Adjuvants for Neuropathic Pain CREATING A HEALTHIER HAWAIʻI

41. Co-analgesics: medications used in combination with opioids to enhance analgesia or treat specific types of pain • Anesthetics: lidocaine, ketamine, propofol • Corticosteroids: dexamethasone (tumor edema, nerve compression in brain/bony mets) • Anxiolytics: lorazepam, diazepam, midazolam • Barbiturates: phenobarbital, pentobarbital Adjuvants for Pain CREATING A HEALTHIER HAWAIʻI

42. Common • Constipation: prevention is KEY! • Miralax, senna and docusate sodium, bisacodyl, mag citrate • Nausea/vomiting: Zofran, promethazine hydroxyzine • Pruritus: diphenhydramine, hydroxyzine • Sedation: tolerance usually within a few days Uncommon • Urinary retention, delirium, myoclonic jerks, seizures, respiratory depression Side Effects to Monitor CREATING A HEALTHIER HAWAIʻI

43. Part of multi-modal analgesia plan for complex pain if opioids alone not sufficient • Nerve block • Epidural infusion Anesthesia Interventions CREATING A HEALTHIER HAWAIʻI

44. Use in combination with medications to enhance pain control • Cuddling • Distraction • Relaxation techniques • Massage • Hypnosis • Aromatherapy Non-Drug Strategies CREATING A HEALTHIER HAWAIʻI

45. Addressing pain in children with serious illness requires addressing multiple factors that effect quality of life. Consider barriers to pain reporting as well as provider and family fears when planning strategies. Education is key to overcoming. Pain assessment needs to be age and developmental stage appropriate. Pain medication choice depends on type of pain, severity of pain and side effects and relief need to be closely monitored. Summary CREATING A HEALTHIER HAWAIʻI

46. Carter, B.S., Levetown, M., & Friebert, S.E. (2011). Palliative care for infants, children, and adolescents: a practical handbook, 2nd edition. Baltimore, MD: The Johns Hopkins Press. Collins, J.J., Berde, C.B., & Frost, J.A. (2011). Pain assessment and management. In: J. Wolfe, P.S. Hinds, & B.M. Sourkes (Eds), Textbook of interdisciplinary pediatric palliative care. Philadelphia, PA: Elsevier/Saunders. Goldman, A., Hain, R., & Liben, S. (Eds). (2012). Oxford textbook of palliative care for children, 2nd edition. New York, NY: Oxford University Press, USA. Hockenberry, M.J. & Wilson, D. (Eds.) (2011). Wong’s nursing care of infants and children, 9th edition. St. Louis, MO: Mosby. Hummel, P., Puchalski, M., Creech, S.D., & Weiss, M.G. (2008). Clinical reliability and validity of the N-PASS: neonatal pain, agitation, and sedation scale with prolong pain.Journal of Perinatology, 28(1), 56-60.. Krechel, S.W., & Bildner, J. (1995). CRIES: A new neonatal postoperative pain measurementscore. Initial testing and reliability. Pediatric Aneasthesia, 5(1), 53-61. doi: 10.1111/j.1460-9592.1995.tb00242.x. Lawrence, J, Alcock, D., McGrath, P., Kay, J., MacMurray, & S.B., Dulberg, C. (1993). The development of a tool to assess neonatal pain. Neonatal Network, 12 (6 September), 59 66. Stevens, B., Johnston, C., Petryshen, P., & Taddio, A. (1996). Premature Infant Pain Profile: Development and initial validation. Clinical Journal of Pain, 12(1), 13-22. References CREATING A HEALTHIER HAWAIʻI