Paediatric Palliative Care & Paediatric Palliative Pain

1. Paediatric Palliative Care & Paediatric Palliative Pain Disclaimer: Whilst every effort has been made to ensure that the information in this presentation is accurate and referenced the author does not accept any responsibility for the use by any third parties. Dr Emma Heckford May 2012 Registrar in Paediatric Palliative Medicine, University Hospital Wales

2. Introduction to Paediatric Palliative Care • Pain in Paediatric Palliative Care • Case Studies

3. Introduction to Paediatric Palliative Care

4. Palliative Care for Children • ACT/RCPCH definition (2009): • “an active and total approach to care, embracing physical, emotional, social and spiritual elements” • Active (not simply stopping treatment) • Total • ‘Best quality of life for patient and family throughout course of a life-limiting illness’

5. PPC - Since when? • Evolution over last 30 years • Various models of care • based on local needs and resources • Paediatric Palliative Medicine now a recognised subspecialty • Currently in UK: • 10 tertiary consultants • ~ 50 ‘paediatricians with a special interest’

6. Why PPC? • At least 50% of child deaths in UK caused by LLCs • ~ 20,000 children in England living with conditions likely to require PC input • But: • Symptom management suboptimal • Professional anxieties • Most children die in hospital, often intensive care

7. Making a difference • Offering (and enabling) child and family choices • Improving quality of life – ‘as well as possible for as long as possible’ • Supporting adjustment and goal setting • Improving experience of death • Improving bereavement outcomes

8. What can be done? • Good Clinical Care • Symptom Control • Pain, Nausea, Vomiting, Constipation, Dyspnoea, Seizures, Spasticity, Hiccup, Sialorrhoea, Pruritis • Palliative Care Emergencies • Bleeding, Pain, SCC, SVCO, Intestinal obstruction, Hypercalcaemia • Facilitating child and family choices • e.g. avoiding hospital admission, supporting care at home • Psychosocial support • EOL care planning and preparation • Bereavement support

9. Generic versus Specialist PPC Palliative Care Services for Children and Young People in England, DH 2007

10. PPM Competencies

11. Palliative Care Needs

12. Palliative Care Needs

13. How is it different to PC in adults? • Different diagnoses, timescales, symptoms • Development and growth • Education • Ethical issues e.g. autonomy and consent • Family dynamics and family-centred care

14. Causes of death in children (0-19yrs) likely to have required Palliative Care Neurological 36% Congenital heart disease 11% Muscle disorders 5% Cystic fibrosis 2% Chronic renal failure 1% Cancer 45%

15. Disease trajectories Normality Group II e.g. Duchenne MD Group 1 e.g. Cancer Group III e.g. Batten’s Group V e.g. Cerebral Palsy Death 0 10 20 Time (Years)

16. When should PC be initiated? No right or wrong answer Here? Here? Or here? Diagnosis Death ….. But important to actively think about it Ongoing treatment and palliative care not necessarily contradictory

17. Transitioning to Palliative Care • Benefits of early initiation • Sense of openness • Attention to child’s quality of life • Greater opportunity for families to make choices • In practice, will depend on: • Disease trajectory • Need to re-align goals • Readiness of child/family/professionals

18. Which symptoms? • Few studies • Symptoms and their management poorly documented • especially non-pain symptoms • and especially if non-oncology diagnoses

19. Which symptoms? • Probably pain, dyspnoea, fatigue, nausea/vomiting the most prevalent • Also fatigue, agitation, seizures, spasms, secretions, constipation, sleep disturbance, anxiety……

20. Balance of burden and benefit • A key principle • Includes balancing of physical and emotional/spiritual aspects • Needs careful thought – e.g. ‘prolonging life’ • No such thing as interventions that are always appropriate or inappropriate • Evidence-based (as far as possible)

21. Burden versus benefit Benefit Burden Partial gastrectomy? Major surgery Mortality/Morbidity Prolonged hospitalisation Not curative Possibly prolonged life Very small chance cure Avoids catastrophic bleed

22. Burden versus benefit Burden Benefit Partial gastrectomy? Major surgery Mortality/Morbidity Prolonged hospitalisation Not curative Possibly prolonged life Very small chance cure Avoids catastrophic bleed

23. Burden versus benefit Benefit Burden Partial gastrectomy? Possibly prolonged life Very small chance cure Avoids catastrophic bleed Major surgery Mortality/Morbidity Prolonged hospitalisation Not curative

24. Pain in Paediatric Palliative Care

25. Pain in Palliative Care • Often more than one cause • May have both acute and chronic features (but not the same as either) • Rarely only physical • Usually gets worse with time • Considerations: • Balance of burden and benefit • Route (inclbuccal, transdermal) • Practicality (often at home) • Acceptability

26. Pain in Palliative Care • Unpleasant sensory and emotional experience • Entirely subjective – ‘what the patient says hurts’ • Japanese study - 75% children with LLCs had pain in last weeks of life

27. Pragmatic Classification • Neuropathic • Disordered sensation • Responds to anticonvulsants and antidepressants • Bone • Intense and focal • Responds to NSAIDs and bisphosphonates • Musclespasm • Responds to muscle relaxants and antispasmodics • Cerebralirritation • Caused by brain injury • Signs of anxiety • Responds to benzodiazepines Opioid sensitive/insensitive/ partially insensitive

28. EMOTIONAL Low mood Anger Anxiety Fear Frustration Helplessness, loss of control Altered body image Adjustment to transition to PC PHYSICAL Disease Treatment (surgery, RTx) Immobility, debility Procedural Other symptoms (constipation…) All exacerbated by poor sleep TOTAL PAIN SOCIAL Social isolation Family and relationship issues Finances SPIRITUAL Why me? Why our family? What will happen to me?

29. Aims of assessment • To assess likely/possible causes: • Treat reversible causes • Identify most appropriate pain-relieving measure/s • To establish a baseline: • Can then judge improvements or not

30. Pain assessment • History • Examination • Tools BUT children may be…. Pre-verbal Non-verbal Cognitively impaired Frightened

31. Pain assessment tools • All children on regular analgaesics should ideally have routine assessment of their pain • The ideal tool: • Practical – easy, quick and fun to use • Validated • Appropriately applied • Developmentally and culturally appropriate • Special groups: • Neonates, infants, developmental delay

32. Pain assessment tools FLACC Behaviour Scale

33. Pain management • WHO approach • Current gold standard in PPM • 3 tiers plus adjuvants at each stage

34. WHO pain ladder Increasing levels of pain or persistent pain despite therapy on a previous step in the ladder STEP 3 Strong opioid for severe pain +/- nonopioid +/- adjuvant STEP 2 Mild opioid for moderate pain +/- nonopioid +/- adjuvant Morphine Other major opioids Paracetamol Codeine Tramadol Paracetamol STEP 1 Nonopioid +/- adjuvant Paracetamol (Aspirin) NB Adjuvants to be considered at each stage

35. Golden Rules • By the ladder – do not rotate, move up • By the clock – major opioids regular with breakthrough • By the route - avoid needles if possible • By the child

36. Adjuvant analgaesics • ‘Adjuvant’ = not primarily analgaesic but can improve pain in certain circumstances Neuropathic Anticonvulsants (CBZ, gabapentin) Antidepressants (amitriptyline) NMDA antagonists (methadone, ketamine) Cerebral irritation Benzodiazepines Phenobarbitone • Spasm • Benzodiazepines • Baclofen • Tizanidine • Botox Bone NSAIDs Bisphosphonates RTx Chemo Surgery Non-pharmacological Physiotherapy Psychology Family support Inflammatory/Oedema Steroids Chemo

37. Initiating strong opioid therapy • What drug? • Morphine - short acting formulation (Oramorph, Sevredol) • By mouth if possible • What dose? • 1mg/kg/day = total daily dose E.g. 30kg – 30mg/day • Then calculate 4 hrly dose = 5mg • And for breakthrough pain? • Give the same 4 hourly dose as required – 5mg

38. Myths about opioids • Is morphine addictive doctor? • If we start now, will we run out of options? • Will it shorten his/her life?

39. Adverse effects? • Constipation • Drowsiness • Nausea and Vomiting • Pruritis • Urinary retention • Respiratory depression

40. Titration phase • Aim – to match the amount of analgesia given with the degree of pain experienced • Add up all doses taken in 24 hours • Eg. 30mg + 15mg = 45mg • 45mg ÷ 6 = 7.5mg • Prescribe 7.5mg 4hrly and 7.5mg prn for breakthrough pain

41. Maintenance phase • More convenient opioid preparations • MST • E.g. If total daily Oramorph requirement: 45mg • Appropriate MST dose: 22.5mg bd • Breakthrough still 7.5mg oramorph prn • Other strong opioids • Patches – fentanyl, buprenorphine • S/C – diamorphine • Also – methadone, oxycodone, hydromorphone • Breakthrough usually still oramorph

42. Managing the maintenance phase • Keep reviewing need for breakthrough analgesia • Titrate background analgesia upwards (or downwards) as necessary • Keep thinking about adjuvants • Change route of drug delivery if necessary

43. Toxicity/Side effects • Symptoms and signs: • Myoclonus, Pinpoint pupils, Itch, Sickness, Reduced level of consciousness, Reduced RR • Think: • ?Dose too high e.g. post RTx • ?Reduced excretion e.g. renal impairment • ?Time to rotate to a different drug

44. Opioid rotation • Calculate equivalent dose for the new drug • Decrease the total daily dose for the new drug by 25% (incomplete tolerance) • Prescribe background and breakthrough • Titrate

45. Don’t forget….. • Adjuvants • Non-pharmacological interventions • Managing other symptoms too • Talking and listening • Explanation and understanding can go a long way • “the pain seemed to go by just talking”

46. Case Studies

47. Sophie • Teenager with severe cerebral palsy • Less well over last 6/12 – chest infections, spasms • Fewer smiles, more agitated • Scoliosis worse, history of hip dislocation • Upset on moving and handling

48. Regular oramorph titrated Then onto fentanyl patch Paracetamol • Codeine PRN • Helped but very constipating • Family reluctant to try major opioid Paracetamol PRN

49. Regular oramorph titrated Then onto fentanyl patch Paracetamol Adjuvants NSAIDs not tolerated Bisphosphonates not practical Optimised seizure and spasm mx Non-pharmacological Respite and family support from local hospice Family support from Pall Care CNS Palliative Care team home visits as required Advance care planning and making choices

50. Amina • 6 month old baby • Large family, Muslim faith • Large tumour left thorax • Delayed diagnosis • Poor response to chemotherapy • Respiratory compromise • Palliative radiotherapy • Hickman line in situ