1. Managing Refractory Symptoms in the Child with Severe Neurological Insult: The Role of the Pediatric Acute Care Nurse Practitioner
Maria Rugg, RN, MN, ACNP, CHPCN(c), &
Sherri Adams RN MSN CPNP
The Hospital for Sick Children
Toronto, Canada

2. TORONTO

4. The hospital for sick children is a tertiary and quaternary care academic pediatric hospital, with 300 beds.

6. Objectives
Examine a framework to understand refractory symptoms in the pediatric patient at the end of life using problem based learning
Describe the health care team’s application and use of the framework within a tertiary/quaternary academic health care centre setting
Describe a potential body of research to evaluate the role of the acute care nurse practitioner within this setting

7. Case History
7 year old female – previously well child had near drowning incident in while at camp
Prolonged resuscitation on site
Suffered severe neurological damage secondary to hypoxia
Initially in PICU then transferred to General Pediatric Ward
No CPR plan established – team told in handover from ICU that the family did not wish to discuss this any further
Primary caregiver – Mother (parents separated), Father rarely visited

8. Case History Child’s Condition Severely neurologically impaired
Vegetative state
Non communicative, no suck or swallowing ability
Dependent for all ADL’s
Severely opisthotonic, rigidity
Constantly sweating, moaning
Grimacing, repetitive facial movements
Very disturbing for caregivers to observe

9. Case History Initial Plan Form a relationship with the family
Ongoing stabilization of patient
Insert Gastrostomy Tube
Manage “perceived” pain and symptoms
Teach family care of child and counsel on prognosis
Discharge child home or to an institution depending on family’s needs and ability to do caregiving for child

10. Case History Problems with initial plan
Rigidity and opisthotonus was refractory to medical management
Severe GERD, did not tolerate NG or NJ feeds (reflux/aspiration/pneumothorax)- consider - GJ or PICC
No good scale to quantify pain in neurologically impaired children
Tried to titrate medications for comfort – a “comfort level” was not reached
Mother “shouldering” family and making all care decisions

11. Case Summary Severe neurologic injury Prognosis: no improvement
Quality of life perceived as poor by family and health care team
Mother just wanted patient to be comfortable
Multiple specialists/professionals involved
Symptoms becoming unmanageable by traditional methods

12. Framework For Symptom Management Dodd et. al
Framework For Symptom Management Dodd et. al. (2001) JAN, 33(5):

13. Framework For Symptom Management Dodd et. al
Framework For Symptom Management Dodd et. al. (2001) JAN, 33(5):
Assumptions of The Model
Gold standard is self-report
Do not have to experience symptom, just be at risk
Nonverbal patients experience symptoms-caregiver report assumed accurate
Management may be targeted at the individual, group, family or work environment
Symptom Management is a dynamic process

14. Framework for Symptom Management, Dodd et. al
Framework for Symptom Management, Dodd et. al. (2001) JAN, 33(5):

15. Framework For Symptom Management
Symptom Experience
Perception-changes from the norm
Response-physiological, psychological, sociocultural and behavioral
Evaluation-judgments on severity, cause, treat ability and effect on lives

16. Framework for Symptom Management, Dodd et. al
Framework for Symptom Management, Dodd et. al. (2001) JAN, 33(5):

17. Framework For Symptom Management
Symptom Management Strategies
Patient
Family
Healthcare system
Healthcare Provider

18. Framework for Symptom Management, Dodd et. al
Framework for Symptom Management, Dodd et. al. (2001) JAN, 33(5):

19. Framework For Symptom Management
Symptom Outcomes
Functional Status
Emotional Status
Mortality
Morbidity&Co-morbidity
Quality of Life
Self Care
Costs

20. Discussion Points - Symptom Outcomes
What is an Refractory Symptom? JOP 12(3):40-45(1996)
What is the symptom and how is it manifested?
For whom is it difficult?
What are the child’s preferences and/or capacity to tolerate the symptom?
What are the family or caregivers preferences and/or capacity to tolerate the perceived distress?
Have various approaches and alternative trails been fully explored?

21. Discussion Points - Symptom Experience/Management
What is the most comfortable way to live and die?
GJ tube/PICC: Prolonged life with severe neurologic injury with multiple medical interventions and eventual death from secondary complications
NG feeds: Respiratory failure secondary to aspiration
Withdrawal of fluid with sedation: up to 2-3 weeks of sedation, dehydration and eventual death

22. Discussion Points – Symptom Experience
Moral Distress: Is it ethically appropriate to treat or withdraw
Sanctity of life
Child should not be denied life-saving treatment because of any degree of mental of physical disability, nor because of the presence of overwhelming suffering
Quality of life
Life is not always better than death
When life is felt to be worse than death, then death is the treatment of choice
Social utility
Refusal to allow the extreme needs of one patient to outweigh the competing needs of others
Greatest good for the greatest number

23. Discussion Points – Symptom Experience/ Management
Withdrawal of Fluids and Nutrition
Few controlled studies
Case reports
Emerging consensus
Seriously ill or dying patients experience little if any discomfort upon the withdrawal of tube feedings, TPN, or IV hydration

24. Comfort Care for Terminally Ill Patients JAMA 272:16. 1994
Prospective case series in inpatient setting
Determine frequency of symptoms of hunger, thirst and determine whether these symptoms could be palliated without forced feeding, forced hydration, or parental nutrition
Adults with terminal illnesses
32 patients monitored over 12 month period
20 patients (60%) never experienced any hunger, 11 (34%) experienced hunger only initially
20 patients (62%) experienced no thirst or thirst only initially during their terminal illness
In all patients symptoms of hunger, thirst, and dry mouth could be alleviated with small amounts of food, fluids and/or by application of ice chips and lubrication of lips
Patients who are terminally ill did not experience hunger and those who did needed only small amounts of food for alleviation
Food and fluid administration beyond the specific requests of patients may play a minimal role in providing comfort to terminally ill patients

25. What Happened?
Teams collaborated to provide effective symptom management and transition from active treatment to comfort care
Provided pain control through subcutaneous butterfly (morphine and methadone)-responded by reduction in hypertonia and mom able to hold in arms for first time since admission to hospital
Died comfortably (as per parent and healthcare provider report) 4 days after withdrawal of fluids and addition of round the clock sedation with family at bedside
Debrief sessions with team members identified changes in practice needed – Led by Palliative Care NP

26. APN Role in Pediatric Palliative Care
Who Does What?
Concern that palliative care practitioners were only used as “ symptomatologists”
By definition palliative care aims to manage the physical, emotional and spiritual needs of patients facing life threatening illness and their families
Pediatric APN is ideally positioned to support team and families through the complex dynamic of symptom experience, symptom management, and symptom outcomes

27. Areas for Further Research
The Palliative Care NP
Critical elements that characterize APNs and make these nurses uniquely qualified for an expanded role within this area include:
In depth knowledge of a specific patient population
Decision making capability
Leadership skill
Capacity to negotiate a complex integrated health network (Weggel,1997)
APN Role
Using the Sick Kids model the APN is well positioned to enhance and lead complex systems involved in the care of complex patients and their families

28. Summary Symptom management must consider the whole patient and team
Approach to care should be holistic and collaborative - Utilizing a model to guide your practice
Comfort and understanding with end of life care requires experience and support from expert consultants ( such as a Palliative care team)
APN can be leaders in this specialized area of care-managing symptoms, families and team’s experience of those symptoms and outcomes of that experience