Transcript Assessing and Managing Symptoms and Co-Morbidities

ASSESSING AND MANAGING
SYMPTOMS AND COMORBIDITIES
In Children with Complex Medical Conditions
Melody Brown Hellsten MS PNPPC-BC
Texas Children’s Hospital
Advanced Practice Providers Conference
Pediatrics
Objectives
1) Identify 5 common symptoms and co-morbidities
in children with complex medical conditions
2) Evaluate symptom assessment tools
for children with complex medical conditions
3) Discuss pharmacologic and non-pharmacologic
management strategies for symptoms and comorbidities for children with complex medical
conditions
The Population
• Children with Complex Chronic Conditions (CCC)
• Chronic, irreversible condition requiring ongoing medical care
• Life-threatening/Life Limiting Illnesses
• Medically Fragile
• Technology Dependent
• Increased risk of symptoms and suffering due to medical condition,
treatment
• Probability of premature death in childhood, adolescents or early
adulthood
Primary Diagnoses
• Cancer and Hematologic conditions
• Congenital Anomalies
• Static and Progressive Neurologic disorders
• Neuromuscular disorders
• HIV
• Metabolic Disorders
• End-stage organ failure
• Neurological Devastation/Trauma
• Cystic Fibrosis
• Rare/Orphan Conditions
Symptom Burden in CCC
• Cancer
• pain, fatigue, sleep disturbance, loss of
energy, nausea and vomiting, hair loss,
and behavior and mood changes
• Report up to 11 symptoms per week
• Higher severity associated with lower
health related QOL
Symptom Burden in CCC
• Cystic Fibrosis (Dellen et al 2010)
• Dyspnea (100%)
• Fatigue (96%)
• Anorexia (85%)
• Anxiety (74%)
• Cough (56%)
• Symptom control ‘somewhat good’ 71%
• Medications/treatments caused discomfort but were felt to be
necessary
Symptom Burden in CCC
• Metabolic Disease (Malcolm, C 2011)
• Batten; Sanfilippo; Morquio
• Pain, cold hands/feet, joint stiffness, disturbed sleep
• Agitation, repetitive behaviors, nausea/vomiting,
constipation, diarrhea
• Cough, choking, drooling, muscle spasms, seizures,
breathing difficulties, secretions, sleep problems,
• Pain, spasms, seizures and breathing were most
difficult to control
• Sanfilippo had most symptom frequency and severity,
followed by Batten, then Morquio
Symptom Management - Parents
• Families employ numerous pharmacologic and
non-pharmacologic strategies to provide
symptom relief for their children
• Parent intuition, knowledge, experience; home
management; flexibility in medication management;
expertise in condition/management over time
• Worry and distress about uncontrolled symptoms
creates a sense of helplessness that leads to seeking
medical attention
• Parents value advice from providers who understand
the disease and their child
Challenges for Providers
• Difficulty assessing symptom due to
communication challenges
• Most challenging symptoms
• Behavioral; seizure
• Relentless nature of symptoms as disease
progresses leads to sense of helplessness
SYMPTOM
ASSESSMENT
Challenges in Symptom Assessment and
Management
• Children are living longer with complex medical conditions
• Worsening with disease progression
• Cognitive and communication impairments
• Numerous care providers
• Ambulatory Care; Inpatient Care; Community based care
• Wide range of disease management options
• Pharmacological
• Technological
• Supportive
• Multidimensional/Inter-related nature of distress
Multidimensional Distress
Physical
Spiritual
Child
Family
Social
Emotional
Multidimensional Distress
Pain;
Dyspnea;
Fatigue
Spiritual
Mortality;
Faith;
Hope
Cancer
CF
Sickle Cell
Social
Isolation;
withdrawal
Emotional
Coping;
Sadness;
Worry
Inter-related Distress
Disease
Complication
s/Side
Effects
Child
Symptoms
Co/Multi
morbidity
Inter-related Distress
HIE
VP Shunt
Complications
Gastrostomy
complications
Medication SE
Child
Feeding
Difficulties
Vomiting
Constipation
Seizures
Gastroparesi
s
Symptom Assessment
• Understand pathophysiology, progression of disease
• Hunt – Three forms of knowing (disease, patient, science)
• Comprehensive history & exam
• Pertinent diagnostic evaluations
• Symptom management plan
• Determine child and family’s priority symptoms
• Clarify goal of intervention from family perspective
• Thinking outside the box
Symptom Assessment
• Use a standardized patient/parent assessment tool
when possible
• Provides consistency in assessment
• Allows for evaluation of symptom management
• Symptom tools vs QOL tools
• Tools for research vs clinical use
• Pain/Symptom Diaries
• Symptom, frequency, intensity
• http://www.partnersagainstpain.com/trackingpain/management.aspx Pain Management Log
• Multiple symptom templates available online
• Easily created on MS Word or Excel tables
Multidimensional Pain Assessment
• Self report/parent report
• Intensity
• Quality
• Pattern
• Aggravating / alleviating factors
• Medication history
• Meaning
Pain – SelfWong
Report
Baker, 1998
• Faces Scales
• Visual-Analog
l------------------------l
0
no pain
10
worst pain
• 0-10 Verbal Report Scale
0 = no pain, 10=worst pain ever
Multivariate Tools – Self Report
• www.partnersagainstpain.com
• Initial Pain Assessment Tool
• Brief Pain Inventory
• Parent / Child Total Quality Pain Instruments
• Foster & Varni 2002
• Children 8-12 and parents
• Neuropathy Pain Scale Pain EDU.org
• Measures of quality and intensity of neuropathic pain
• Adolescent Pediatric Pain Tool )
(Savedra et al., 1993)
• Good for ages 8 and up, chronic and acute pain
Pain – Non Verbal Proxy
• Non-Communicating Children’s Pain Checklist-
Revised(Breau and others, 2002)
• Validated in children age 3-18
• Non-communicating children
• Proxy reporter of child in past 2 hrs
• 7 domains w/ total of 30 observations
• Vocal, social, facial, activity, body/limbs, physiological,
eating/sleeping
• 0-3 scale of severity of behavior per obs
• Total score of 7 or more indicates child has pain
• http://www.aboutkidshealth.ca/Pain
Pain – Non Verbal Proxy
• Paediatric Pain Profile
•
•
•
•
(Hunt and others, 2004)
Validated in children age 3-18
Non-communicating children
Proxy reporter of child in past 2 hrs
7 domains w/ total of 30 observations
• Vocal, social, facial, activity, body/limbs, physiological,
eating/sleeping
• 0-3 scale of severity of behavior per obs
• Total score of 7 or more indicates child has pain
• http://www.aboutkidshealth.ca/Pain
Pain – Non Verbal Proxy
• Revised FLACC pain tool(Voepel-Lewis et al 2002)
• 5 domains, 0-2pts per domain
• Original scale for infant assessment
• Revised scale adds behavioral cues characteristic of NI children,
parents able to add individual behaviors
• Good reliability, validity
(REVISED) FLACC Scale
Scoring
Categories
0
1
2
Face
No particular expression or
smile
Occasional grimace or frown,
withdrawn, disinterested, Sad,
appears worried
Frequent to constant quivering chin,
clenched jaw, distressed looking face,
expression of fright/panic
Legs
Normal position or relaxed,
usual tone & motion to
limbs
Uneasy, restless, tense, occasional
tremors
Kicking, or legs drawn up, marked increase
in spasticity, constant tremors, jerking
Activity
Lying quietly, normal
position, moves easily,
regular, rhythmic
respirations
Squirming, shifting back and forth,
tense, tense/guarded movements,
mildly agitated, shallow/splinting
respirations, intermittent sighs
Arched, rigid or jerking, severe agitation,
head banging, shivering, breath holding,
gasping, severe splinting
Cry
No cry (awake or asleep),
Moans or whimpers; occasional
complaint, occasional verbal
outbursts, constant grunting
Crying steadily, screams or sobs, frequent
complaints, repeated outbursts, constant
grunting
Consolability
Content, relaxed
Reassured by occasional touching,
hugging or being talked to,
distractible
Difficult to console or comfort, pushing
caregiver away, resisting care or comfort
measures
Each of the five categories (F) Face; (L) Legs; (A) Activity; (C) Cry; (C) Consolability is scored from 0-2, which results in a total
score between zero and ten.
Merkel, S and others. The FLACC: A behavioral scale for scoring postoperative pain in young children, Pediatr Nurse 23(3):293-297,
1997. Copyright: Jannetti Co. University of Michigan Medical Center.
Malviya, S.., Vopel-Lewis, T, Burke, C., Merkel, S.., Tait, A.R. (2006) The revised FLACC ovservational pain tool: improved
reliability and validity for pain assessment in children with cognitive impairment. Pedatric Anesthesia 16:258-265
Pain – Non Verbal Proxy
• Individualized Numeric Rating Scale
0
1
2
MILD
Reposition
Check tubes/equip
Change loction
Cuddle/Comfort
Tylenol/Ibuprofen
3
4
5
(Solodiuk & Curley, 2003)
6
MODERATE
Bathe
Massage
Heating Pad
Combo opioid
7
8
9
SEVERE
Distraction
ER
Pure opioid
10
Symptom Assessment Tools
• Cancer
• SSPedi – Tomlinson et al (2014) not validated as yet
• 15 item screening tool with 5 point Likert
• Not at all bothered to extremely bothered
• MSAS 7-12, 10-18 – Collins (2000, 2002)
• 7-12 measures 8 common symptoms, 10-18 measures up to 30, Global
Distress Scale – 10 items
• Obtains presence of symptom, frequency, severity, distress
• Cancer Fatigue Scale 13-18 – Hinds et al (2007)
• 14 items related to fatigue, 11 items measuring causes of fatigue
Symptom Assessment Tools
• Muscle Tone
• Clinical measures of upper limb impairment
(Randall 2012)
• Modified Melbourne Assessment (2, 3, and 4 yrs)
• 16 items representative of main components of upper limb
movement: grasp, reach, release, manipulation
• Quality of Upper Extremity Skills Text (18mo-18 yrs)
• 4 domains: dissociated movements, grasp, weight bearing, protective
extension
• Hypertonia Assessment Tool
• Differentiates dystonia, spasticity, rigidity
Symptom Assessment Tools
• Dyspnea
• Breathlessness VAS (Tosca, 2011)
• Used in Asthma
• 10 cm line – 0 breathlessness, 10 no breathlessness
• Cut off value of 6 correlated with bronchial airflow limitation
• Modified Borg Scale – (Hommerding, 2010)
• Evaluated in Cystic Fibrosis patients
• Vertical scale 0-10; 0 no symptoms, 10 maximum symptom
• Used with 6 min walk test to provide information regarding patient level of
distress
• Pediatric Dyspnea Scale (Kahn, 2009)
• Asthma
• Picture of 7 faces smiling to crying with chest tightness represented by
lungs tied with rope successively tighter
• Dalhousie Dyspnea Scale (McGrath, 2005)
• Measures three factors of dyspnea: throat closing, chest tightness, effort
• Pictures with slide rule : boy running, lungs tied with rope, trachea tied
with rope
Symptom Management
• Inter disciplinary family-centered care is an
integral part of the symptom management for a
chronically ill child.
• Family shapes types of interventions
• Illness Experience
• QOL and Sources of Suffering
• Goals of Care
• Curative/Restorative
• Life Prolongation
• Quality EOL
Disease Trajectory
Goal

–
–
–
–
Cure
Prolong life
Prolong life
End of life
Morbidity
High
Moderate
Minimal
Mild
Attitude
Win
Fight
Live with it
Surrender
Disease effect
Eradicate
Response
Arrest growth
None
Original slide design – J. Kane MD
Anticipatory Guidance
• This is what parents want from us!
• Majority of parents prefer partnership, want information, but
ultimately feel responsible for final decision
• Most presenting co-morbidities will have more than one
potential intervention
• Align interventions with child/family goal for the symptom or
problem
• Difficult symptom management decisions
• Surgery
• Balancing disease directed therapy and comfort
• Technology
Symptom Management - Neuro
• Diseases
• CNS malformation
• Comorbidity
• Epilepsy
• Seizures
• Dysautonomia
• Temperature
• Dysphagia
• HIE
• Cerebral Palsy
• Symptoms
• Chronic lung
Dz
• Neurogenic
bowel/bladder
• Dystonia
•
•
•
•
•
•
• Neurodegenerative
irregularity
Choking/
Aspiration
Resp
infections
UTIs
Constipation
Scoliosis
Pain
Management
(Hauer, 2010)
• Dysautonomia (variable HR, HTN, temp instability, flushing/sweating/pallor; GI, posturing)
• Pharmacologic – gabapentin; cyproheptadine; clonidine; morphine
• Non-Pharm – related to presenting issue
• Dystonias (hypertonia, hypotonia, spasticity, rigidity)
• Pharmacologic – Benzodiazepines; baclofen, botox, gabepentin
• Non-pharmacologic – range of motion/therapies, bracing, massage,
warmth, swaddling
• Invasive – Rizotomy, rods/titanium ribs, baclofen pump
• Dysphagia
(reflux, choking, drooling, aspiration, +/-cough)
• Pharmacologic – PPIs/H2
• Non-pharm – oral motor therapy, neuromotor electrical stim;
thickened feeds
• Invasive – gastrostomy +/- fundoplication, G/Jujuneostomy
Symptom Management - Respiratory
• Diseases
• Fibrotic disease
• Neuro-
degenerative
• Obstructive lung
disease
• Metastatic
malignancy
• Thoracic
Insufficency
• Comorbidity
• Chronic
Infections
• Obstructive
Sleep Apnea
• Hypo
ventilation
• Chronic
respiratory
failure
• Symptoms
• Tachypnea
• Tachycardia
• Dyspnea
• Cough
• Sleep
disturbance
• Headaches
• Irritability
• Fatigue
Management - Respiratory
• Dyspnea, secretions, chronic respiratory failure
• Pharmacologic – morphine, anxiolytics, bronchodilators,
ipratropium,expectorants/mucolytics, steroids; antibiotics,
glycopyrolate (may cause mucous plugs)
• Non-pharm – circulating air, oxygen, cool environment, energy
sparing activities
• Advanced – BiPAP; cough assist, Interpulmonary Percussive
Ventilation
• Death by respiratory failure
• Frightening to most families
• Discuss ways to keep patient comfortable
• Role of hypercapnea in relaxing, sedating
• Prepare family for end-stage breathing patterns
Symptom Management - Gastrointestinal
• Comorbidity
• Diseases
• Immobility
• Neuro and
neurodegenerative
• Symptoms
• Nausea/
Vomiting
• Feeding
Intolerance
• Metabolic
disorders
• Constipation
• Gastroparesis
• Anorexia/
• Cystic Fibrosis
• Bowel
• Solid Tumors
• Polypharmy
Cachexia
Obstruction
• Pain
Management - Gastrointestinal
• Nausea/Vomiting
• Pharmacologic – based on underlying cause
• prokinetics, ondansetron, scopolamine, corticosteroids; cannabinoid
• Non-pharm – aroma therapy, relaxation breathing
• Constipation
• Opioid induced – stool softeners/laxitives, fluids as needed,
Methylnaltrexone
• Dysmotility – prokinetics, erythromycin, PEG
• Obstruction – steroids, decompression, surgical management
• Non-pharm – abdominal massage, LE ROM/Bicycle movements
• Anorexia/Cachexia
• Normal and expected symptom in advanced disease
• Familys worry about ‘starvation’ – patients do not report ‘starvation’
Summary
• Children with complex medical conditions experience
significant symptom burden throughout their disease process
• Anticipating, assessing and managing symptoms on a regular
basis is imperative
• Develop symptom management plans with parents/children
based on symptoms most distressful to them
• Clarify goals of care, child/parent hopes and potential for
symptom management interventions to achieve their goals
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