Pediatrics

ASSESSING AND MANAGING SYMPTOMS AND CO-

MORBIDITIESIn Children with Complex Medical Conditions

Melody Brown Hellsten MS PNPPC-BC

Texas Children’s Hospital

Advanced Practice Providers Conference

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 co-morbidities 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

Child

Family

Physical

Emotional

Social

Spiritual

Multidimensional Distress

Cancer

CF

Sickle Cell

Pain; Dyspnea; Fatigue

Emotional

Coping; Sadness;

Worry

Social

Isolation; withdrawal

Spiritual

Mortality; Faith; Hope

Inter-related Distress

Child

Disease

Co/Multi morbidity

Symptoms

Complications/Side Effects

Inter-related Distress

Child

HIE

Gastroparesis

Feeding Difficulties Vomiting

ConstipationSeizures

VP Shunt Complications

Gastrostomy complications

Medication SE

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/tracking-pain/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 – Self Report• Faces Scales

• Visual-Analog

l------------------------l 0 10

no pain worst pain

• 0-10 Verbal Report Scale 0 = no pain, 10=worst pain ever

0No Hurt

2Hurts

Little Bit

4Hurts

Little More

6Hurts

Even More

8Hurts

Whole Lot

10HurtsWorst

Wong Baker, 1998

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 (Solodiuk & Curley, 2003)

Happy

exp

ress

ion

Res

tless

, squ

irmin

g

Dis

torte

d fa

ce, i

ncre

ased

tone

Cry

ing,

trem

ors

0 1 2 3 4 5 6 7 8 9 10

RepositionCheck tubes/equipChange loctionCuddle/ComfortTylenol/Ibuprofen

BatheMassageHeating PadCombo opioid

DistractionERPure opioid

MILD MODERATESEVERE

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 Morbidity Attitude Disease effect – Cure High Win Eradicate – Prolong life Moderate Fight Response– Prolong life Minimal Live with it Arrest growth– End of life Mild Surrender 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

• HIE

• Cerebral Palsy

• Neurodegenerative

• Symptoms• Seizures• Temperature

irregularity• Choking/

Aspiration• Resp

infections• UTIs• Constipation• Scoliosis• Pain

• Comorbidity• Epilepsy

• Dysautonomia• Dysphagia• Chronic lung

Dz• Neurogenic

bowel/bladder• Dystonia

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

• Symptoms• Tachypnea• Tachycardia• Dyspnea• Cough• Sleep disturbance

• Headaches• Irritability• Fatigue

• Comorbidity

• Chronic Infections

• Obstructive

Sleep Apnea

• Hypo ventilation

• Chronic respiratory failure

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

• Diseases• Neuro and

neurodegenerative

• Metabolic disorders

• Cystic Fibrosis

• Solid Tumors

• Symptoms• Nausea/

Vomiting

• Constipation

• Anorexia/ Cachexia

• Pain

• Comorbidity• Immobility

• Feeding Intolerance

• Gastroparesis

• Bowel Obstruction

• Polypharmy

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

References

Collins JJ, Byrnes ME, Dunkel IJ, et al. The Measurement of Symptoms in Children with Cancer. Journal of pain and symptom management. 2000;19(5):363-377.

Collins JJ, Devine TD, Dick GS, et al. The Measurement of Symptoms in Young Children With Cancer: The Validation of the Memorial Symptom Assessment Scale in Children Aged 7–12. Journal of pain and symptom management. 2002;23(1):10-16.

Miller E, Jacob E, Hockenberry MJ. Nausea, Pain, Fatigue, and Multiple Symptoms in Hospitalized Children With Cancer. Oncology Nursing Forum. 2011;38(5):E382-393.

Hockenberry MJ, Hooke MC, Gregurich M, McCarthy K, Sambuco G, Krull K. Symptom clusters in children and adolescents receiving cisplatin, doxorubicin, or ifosfamide. Oncology Nursing Forum. 2010;37(1):E16-27.

Baggott CR. An evaluation of the factors that affect the health-related quality of life of children following myelosuppressive chemotherapy. Supportive care in cancer. 2011;19(3):353-361.

Pritchard M, Burghen EA, Gattuso JS, et al. Factors that distinguish symptoms of most concern to parents from other symptoms of dying children. Journal of pain and symptom management. Apr 2010;39(4):627-636.

Fakhry H, Goldenberg M, Sayer G, et al. Health-Related Quality of Life in Childhood Cancer. Journal of developmental and behavioral pediatrics : JDBP. July/August 2013;34(6):419-440.

Woodgate RL, Degner LF. Expectations and beliefs about children's cancer symptoms: perspectives of children with cancer and their families. Oncology Nursing Forum. 2003;30(3):479-491.

Woodgate RL, Degner LF, Yanofsky R. A different perspective to approaching cancer symptoms in children. Journal of pain and symptom management. 2003;26(3):800-817.

Baggott C, Cooper BA, Marina N, Matthay KK, Miaskowski C. Symptom cluster analyses based on symptom occurrence and severity ratings among pediatric oncology patients during myelosuppressive chemotherapy. Cancer nursing. Jan-Feb 2012;35(1):19-28

Hockenberry MJ. Sickness behavior clustering in children with cancer. Journal of Pediatric Oncology Nursing. 2011;28(5):263

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