1. Integrating Palliative Care Into The Care Of Children With Developmental Disabilities Professor and Section Head, Palliative Medicine, University of Manitoba Medical Director, WRHA Adult and Pediatric Palliative Care Mike Harlos MD, CCFP, FCFP Erin Shepherd RN, MN Clinical Nurse Specialist, WRHA Pediatric Palliative Care

2. The presenters have no conflicts of interest to disclose

3. Objectives To explore where palliative care may fit into the spectrum of care for children with developmental disabilities To consider potential barriers to the involvement of palliative care To review common issues of communication in palliative and end-of-life care To explore an approach to health care decision-making To review common symptoms at end-of-life and their management

4. http://palliative.info

7. WHO Definition of Palliative Care for Children Palliative care for children is the active total care of the child's body, mind and spirit, and also involves giving support to the family. It begins when illness is diagnosed, and continues regardless of whether or not a child receives treatment directed at the disease. Health providers must evaluate and alleviate a child's physical, psychological, and social distress. Effective palliative care requires a broad multidisciplinary approach that includes the family and makes use of available community resources; it can be successfully implemented even if resources are limited. It can be provided in tertiary care facilities, in community health centres and even in children's homes.

8. Thank you for giving me aliveness Jonathan – 6 yr old boy terminally ill boy Ref: Armfuls of Time; Barbara Sourkes

9. Common Trajectory Of Decline In Progressive Life-Limiting Illness In Children Functional Status Time Decline Crises ( Scary Dips ) Death From presentation by Joanne Wolfe at the 16 th International Congress on the Care of The Terminally Ill

10. Prognostic Uncertainty I dont know whether to buy a coffin or a tricycle Comment from a father during prenatal palliative care consult Addressing the comfort of children must require prognostic certainty or the acknowledgment of the likelihood of dying Palliative Care can follow in parallel with aggressive, cure-focused care, as one member of the team supporting patient and family

11. What if…? What would things look like? Time frame? Where care might take place What should the patient/family expect (perhaps demand?) regarding care? How might the palliative care team help patient, family, health care team? What would things look like? Time frame? Where care might take place What should the patient/family expect (perhaps demand?) regarding care? How might the palliative care team help patient, family, health care team? Palliative Care… The What If…? Tour Guides Disease-focused Care (Aggressive Care) Disease-focused Care (Aggressive Care)

12. Palliative Care for Children with Developmental Disabilities Range of conditions Prognostication difficult –These children would typically not meet the criteria required for adult programs => pediatric criteria required Specific needs are varied –Will depend on care environment, momentum of decline, needs of the family +/or health care providers Resource for symptom management or crises Assist with care planning and decision making Inform the range of options and possibilities Resource for the family in dealing with serious illness Bereavement resources, follow-up +/or support

14. Barriers to Integration of Curative and Palliative Care Among the Population of Children with Severe Neurodevelopmental Disabilities 1.Professional Attitudes Towards Severe Cognitive Disability –May inappropriately downplay the burden of technological intervention –May under treat pain –May limit the spectrum of care options –If working in an inpatient setting may not recognize their assessment of QOL is based on an incomplete clinical picture Graham, RJ & and Robinson, WM. (2005) Integrating Palliative Care into Chronic Care for Children with Severe Neurodevelopmental Disabilities, J Dev Behav Pediatr 26 (5): 261-365

15. Continued… 2.Reconfigured Parental Roles Towards Children with Complex Medical Care Needs – Special relationship with their child – intertwined identity – Feelings of guilt evoked at prospect of child s death – Reluctance to plan for end of life care – Optimal palliative care may be as important as any care decision made throughout the child s life – Integration of palliative care goals into the usual chronic care regime Graham, RJ & and Robinson, WM. (2005) Integrating Palliative Care into Chronic Care for Children with Severe Neurodevelopmental Disabilities, J Dev Behav Pediatr 26 (5): 261-365

16. Continued… 3.The Uncertainty of Prognosis and the Pressure of Past – Struggle to identify outcome indices and valid comparison group on which to base accurate prognostication – Without faith in prognostication, planning for end of life care can be postponed or ignored – Clinicians must find a way to overcome their sense of discomfort with being previously wrong and counter parents unrealistic expectations for the endless restorative prowess of medical care Graham, RJ & and Robinson, WM. (2005) Integrating Palliative Care into Chronic Care for Children with Severe Neurodevelopmental Disabilities, J Dev Behav Pediatr 26 (5): 261-365

17. A Model for End-of-Life Care in Children with Neurodevelopmental Disabilities 1.Acknowledge that children with disabilities have quality-of-life 2.Acknowledge that children with disabilities are entitled to end-of-life care 3.Begin discussions regarding interventions, treatment options, quality-of-life, and end-of-life care early and as part of a medical home model 4.When considering technology support options, present both benefits and potential difficulties, while also acknowledging that technology is not irrevocable Graham, RJ & and Robinson, WM. (2005) Integrating Palliative Care into Chronic Care for Children with Severe Neurodevelopmental Disabilities, J Dev Behav Pediatr 26 (5): 261-365

18. Continued… 5.Utilize a multidisciplinary approach, including physicians, nurses, social workers, psychologists, teachers, friends and others 6.Engage non-acute care providers at times of medical crisis 7.Equate end-of-life care with quality care that is not inconsistent with chronic care 8.Acknowledge the parent-child relationship, expertise, and longitudinal perspective 9.Model effective and compassionate care of children with neurodevelopmental disabilities for trainees and colleagues Graham, RJ & and Robinson, WM. (2005) Integrating Palliative Care into Chronic Care for Children with Severe Neurodevelopmental Disabilities, J Dev Behav Pediatr 26 (5): 261-365

19. Silence Is Not Golden Dont assume that the absence of question reflects an absence of concerns As families watch a loved one decline, it would be very unusual for them not to wonder what to expect, and over what time frame Waiting for such questions to be posed may result in missed opportunities to address concerns; consider exploring preemptively

20. Be Clear 20 Make sure youre both talking about the same thing Theres a tendency to use euphemisms and vague terms in dealing with difficult matters such as death & dying… this can lead to confusion The single biggest problem in communication is the illusion that it has taken place. George Bernard Shaw

21. Planning For Predictable Challenges

22. functional decline occurs food/fluid intake decrease.; feeds may not be tolerated enteral medication route may be ineffective symptoms develop: dyspnea, congestion, delirium family will need support & information

23. A foundational component of effective communication is to connect / engage with that person… i.e. try to understand what their experience might be –If you were in their position, how might you react or behave? –What might you be hoping for? Concerned about? This does not mean you try to take on that person's suffering as your own Must remain mindful of what you need to take ownership of (symptom control, effective communication and support), vs. what you cannot (the sadness, the unfairness, the very fact that this person is dying) Connecting

24. 1.Acknowledge/Validate and Normalize Thats a very good question, and one that we should talk about. Many people in these circumstances wonder about that… 2.Is there a reason this has come up? Im wondering if something has come up that prompted you to ask this? 3.Gently explore their thoughts/understanding Sometimes when people ask questions such as this, they have an idea in their mind about what the answer might be. Is that the case for you? It would help me to have a feel for what your understanding is of your condition, and what you might expect 4.Respond, if possible and appropriate If you feel unable to provide a satisfactory reply, then be honest about that and indicate how you will help them explore that Responding To Difficult Questions

25. Day 1 Final Day 3 Day 2 The Perception of the Sudden Change Melting ice = diminishing reserves When reserves are depleted, the change seems sudden and unforeseen. However, the changes had been happening. That was fast!

26. Helping Families At The Bedside: Physical Changes physical changes of dying can be upsetting to those at the bedside: –skin colour – cyanosis, mottling –breathing patterns and rate –muscles used in breathing reflect inescapable physiological changes occurring in the dying process. may be comforting for families to distinguish between who their loved one is - the person to whom they are so connected in thought and spirit - versus the physical changes that are happening to their loved one's body.

27. Decisions

28. Helping Family And Other Substitute Decision Makers In situations where death will be an inescapable outcome, family may nonetheless feel that their choices about care are life-and-death decisions (treating infections, hydrating, tube feeding, etc.) It may be helpful to say something such as: I know that youre being asked to make some very difficult choices about care, and it must feel that youre having to make life-and-death decisions. You must remember that this is not a survivable condition, and none of the choices that you make can change that outcome. We are asking for guidance about how we can ensure that we provide the kind of care that he would have wanted at this time.

29. An Approach To Decision Making The health care team has a key role in providing information related to technical or medical issues, and physiological outcomes… reviewing/explaining details about the condition, test results, or helping explore treatment options indicating when a hoped-for outcome or treatment option is not medically possible Patient/family must have a central role in considerations relating to value/belief systems (such as whether life is worth living with a certain disability) or to experiential outcomes (such as energy, well-being, quality of life)

30. Goal-Focused Approach To Decision Making Regarding effectiveness in achieving its goals, there are 3 main categories of potential interventions: 1. Those that will work: Essentially certain to be effective in achieving intended physiological goals (as determined by the health care team) or experiential goals (as determined by the patient) goals, and consistent with standard of medical care 2. Those that wont work: Virtually certain to be ineffective in achieving intended physiological goals (such as CPR in the context of relentless and progressive multisystem failure) or experiential goals (such as helping someone feel stronger, more energetic), or inconsistent with standard of medical care 3. Those that might work (or might not): Uncertainty about the potential to achieve physiological goals, or the hoped-for goals are not physiological/clinical but are experiential

31. Goal-Focused Approach To Decisions Goals unachievable, or inconsistent with standard of medical care Discuss; explain that the intervention will not be offered or attempted. If needed, provide a process for conflict resolution: Mediated discussion 2nd medical opinion Ethics consultation Transfer of care to a setting/providers willing to pursue the intervention Goals unachievable, or inconsistent with standard of medical care Discuss; explain that the intervention will not be offered or attempted. If needed, provide a process for conflict resolution: Mediated discussion 2nd medical opinion Ethics consultation Transfer of care to a setting/providers willing to pursue the intervention Goals achievable and consistent with standard of medical care Proceed if desired by patient or substitute decision maker Goals achievable and consistent with standard of medical care Proceed if desired by patient or substitute decision maker Uncertainty RE: Outcome Consider therapeutic trial, with: 1.clearly-defined target outcomes 2.agreed-upon time frame 3.plan of action if ineffective Uncertainty RE: Outcome Consider therapeutic trial, with: 1.clearly-defined target outcomes 2.agreed-upon time frame 3.plan of action if ineffective

32. Considerations In The Final Hours Are there preexisting medical conditions need attention in the final hours? not typically necessary to continue ongoing medical management of underlying illnesses, with the possible exception of seizure disorder What new symptoms exists of might arise (typically dyspnea, congestion, agitated delirium), and related medication needs? Uncommon for pain to arise as a new symptoms in final hours What are the available routes of medications administration? Anticipated concerns of family

33. Prevalence of Symptoms In Final Days Dyspnea: 80% + Congestion: reported as high as 92% Delirium: 80% + Families who would be grateful for support and information: must be near 100% When these issues arise at end-of-life, things havent gone wrong… they have gone as they are inclined to.

34. Role of the Health Care Provider Anticipate changes in functional status, loss of oral route, the development of symptoms Develop a care plan that prepares for predictable issues – LAD, ACP Preemptively address communications issues: food/fluid intake sleeping too much are medications causing the decline? how do we know he/she is comfortable? can he/she hear us? dont want to miss being there at time of death how long can this go on? what will things look like?

35. Management of Symptoms SymptomDrug Non-Oral Route(s) Dyspneaopioidsublingual (SL) – small volumes of high concentration; same dose as oral subcutaneous – supportable in most settings; same dose as IV = ½ po dose IV – limited to hospital settings intranasal – fentanyl – lipid soluble opioid; use same dose as IV to start. Painopioidsee above Secretionsscopolaminesubcutaneous transdermal (patches; compounded gel) glycopyrrolatesubcutaneous Agitated Delirium neuroleptic (methotrimeprazine ; haloperidol) SL– use same dose for all routes subcutaneous (most settings); IV (hospital) lorazepamSL – generally use with neuroleptic

36. Medications Needed 1.Opioid: pain, dyspnea 2.Antisecretory: congestion 3.Sedative (neuroleptic +/- benzodiazepine): agitated delirium Plus whatever condition-specific medications are needed (e.g. anticonvulsants)

37. Opioid Use in Final Days/Hours specific doses will depend on the degree of distress and existing opioid tolerance usually need to use short-acting opioid in order to respond quickly to changing symptoms (dyspnea) if patient is on long-acting morphine or hydromorphone, switch to equivalent daily dose of short-acting divided q4h (½ the oral dose if switching to subcutaneous or IV) if patient is on transdermal fentanyl, consider leaving this as is, and adding a q4h dose of morphine or hydromorphone, starting low and titrating up as needed. the interval between prn (as-needed) doses should not exceed one hour, regardless of route. The effect of a prn dose will be evident by 1h, and a longer interval will cause needless suffering

38. Medications Needed 1.Opioid: pain, dyspnea 2.Antisecretory: congestion 3.Sedative (neuroleptic +/- benzodiazepine): agitated delirium Plus whatever condition-specific medications are needed (e.g. anticonvulsants)

39. Opioids in Dyspnea Uncertain mechanism Comfort achieved before resp compromise; rate often unchanged Often patient already on opioids for analgesia; if dyspnea develops it will usually be the symptom that drives the need for titration Dosage should be titrated empirically May need rapid dose escalation in order to keep up with rapidly progressing distress

40. AgentIntermittent DoseParenteral Infusion Dose CodeineEnteral0.5 – 1.0 mg/kg q4hNot recommended parenterally Morphine Sulfate Enteral0.2 – 0.3 mg/kg q 4h 0.05 mg/kg IV load over 10 min then 0.01 – 0.03 mg/kg/hr IV/SQ0.05 – 0.2 mg/kg q 2-4h Hydromorphone Enteral30 – 80 micrograms/kg q4h 10 – 20 micrograms/kg IV load over 10 min then 2 – 8 micrograms/kg/hr IV/SQ15 micrograms/kg q 2 – 4h Oxycodone0.05 – 0.15 mg/kg po q4hN/A Fentanyl Citrate0.5 – 2 micrograms/kg IV0.5 – 2 micrograms/kg/hr IV Lorazepam0.05 mg/kg IV/SL Midazolam IV 0.025 – 0.05 mg/kg titrated carefully, with 2-3 min. between fractions Infusion would be guided by prn doses …neither surgical anesthesia nor fatal intoxication is produced by benzodiazepines in the absence of other drugs with CNS- depressant actions; an important exception is midazolam, which has been associated with decreased tidal volume and respiratory rate (Goodman & Gilman) Nasal0.1 mg/kg in each nostril po/SL Child 1 month–18 years 0.5 micrograms/kg (max. 15 mg) 30–60 minutes before procedure Methotrimeprazine0.025 - 0.1 mg/kg q6h po/SQ0.1 - 0.4 mg/kg/24 hr IV/SQ Recommended Opioid And Sedative Doses For Dyspnea (> 6 Months Age)* * For infants < 6 months start with ¼ of the pediatric starting dose and titrate

41. Common Concerns About Aggressive Use of Opioids at End-Of-Life How do you know that the aggressive use of opioids for dyspnea doesn't actually bring about or speed up the patient's death? I gave the last dose of morphine and he died a few minutes later… did the medication cause the death?

42. 1.Literature: the literature supports that opioids administered in doses proportionate to the degree of distress do not hasten death and may in fact delay death 2.Clinical context: breathing patterns usually seen in progression towards dying (clusters with apnea, irreg. pattern) vs. opioid effects (progressive slowing, regular breathing; pinpoint pupils) 3.Medication history: usually the last dose is the same as those given throughout recent hours/days, and was well tolerated

43. Typically, with excessive opioid dosing one would see: pinpoint pupils gradual slowing of the respiratory rate breathing is deep (though may be shallow) and regular

44. COMMON BREATHING PATTERNS IN THE FINAL HOURS Cheyne-Stokes Rapid, shallow Agonal / Ataxic

45. Palliative Management of Secretions

46. Managing Secretions in Palliative Patients Factors influencing approach management: Oral secretions vs. lower respiratory Level of alertness and expectations thereof Proximity of expected death Death Rattle – up to 50% in final hours of life At times the issue is more one of creating an environment less upsetting to visiting family/friends Suctioning: If you can see it, you can suction it Suctioning Increased Secretions Mucosal Trauma

47. Secretions - Prevalence At Study Entry And In Last Month Of Life UK Childrens Cancer Study Group/Paediatric Oncology Nurses Forum Survey Goldman A et al; Pediatrics 2006; 117; 1179-1186

48. Atropine Eye Drops For Palliative Management Of Secretions Atropine 1% ophthalmic preparation Local oral effect for excessive salivation/drooling Dose is usually 1 – 2 drops SL or buccal q6h prn There may be systemic absorption… watch for tachycardia, flushing

49. Glycopyrrolate For Palliative Management Of Secretions Enteral: 40 – 100 micrograms/kg 3 – 4 times daily 2006 British National Formulary For Children IWK Health Centre (Halifax) Formulary Refs: Parenteral: 4 – 10 micrograms/kg 3 – 4 times daily (1/10 th the enteral dose) IWK Health Centre (Halifax) FormularyRef: Less sedating than scopolamine (doesnt cross the blood-brain barrier), longer acting, however not as effective Useful where patient is still alert; scopolamine will cause sedation and delirium in awake patients

50. Scopolamine For Palliative Management Of Secretions Transderm-V ® (Scopolamine) AgeDose 1 month – 3 yrs250 micrograms every 72 hours (1/4 patch) 3 – 10 yrs500 micrograms every 72 hours (1/2 patch) 10 – 18 yrs1 mg every 72 hours (one patch) Ref: 2007 British National Formulary For Children Intermittent SQ/IV: 6-10 micrograms/kg (max. 600 micrograms) q 4h Continuous SQ/IV: 40-60 microgram/kg/day (1.67 – 2.5 microgram/kg/h) 2006 Rainbow Hospice GuidelinesRef: