Transcript Pediatric Idiopathic Chronic Pain Disorders

Pediatric Idiopathic Chronic
Pain Disorders
Lucinda M Brown MSN, RN, CNS
Dr. Daniel Lacey MD, PhD
January 2015
“ Pain is an unpleasant sensory and emotional experience
associated with actual or potential tissue damage or
described in terms of such damage.”
- International Association for the Study of Pain
“Pain is an inherently subjective multi-factorial
experience and should be assessed and treated as
such.”
- American Academy of Pediatrics and American Pain Society
What is the Purpose of Pain?
Acute pain serves as a protective mechanism
against impending tissue injury or death
Chronic pain in contrast serves no such
physiologic role and is itself not a symptom, but a
disease state.
Acute vs. Chronic Pain
Characteristic
Acute Pain
Chronic Pain
Cause
Generally known
Often unknown
Duration of pain
Short,
well-characterized
Persists after healing,
3 months
Treatment
approach
Resolution of
underlying cause,
usually self-limited
Underlying cause and pain
disorder; outcome is often
pain control, not cure
Defining Pain
Acute Pain Classification
Somatic Pain: Result of activation of nociceptors (sensory
receptors) sensitive to noxious stimuli in cutaneous or deep
tissues. Experienced locally and described as constant,
aching and gnawing. The most common type in cancer
patients.
Visceral Pain: Mediated by nociceptors. Described as deep,
aching and colicky. Is poorly localized and often is referred
to cutaneous sites, which may be tender. In cancer patients,
results from stretching of viscera by tumor growth.
Defining Pain
Chronic Pain Classification
Nociceptive pain: Visceral or somatic.
stimulation of pain receptors by tissue inflammation, mechanical
deformation, ongoing tissue injury. Responds well to common
analgesic medications and nondrug strategies.
Neuropathic Pain:
Involves the peripheral or central nervous system. Does not
respond predictably to conventional analgesics. May respond to
adjuvant analgesic drugs. Visceral pain also neuropathic.
Mixed or undetermined pathophysiology:
Treatment is unpredictable; requires various approaches.
Psychologically based pain syndromes:
Traditional analgesia is not indicated, doesn’t work. Uncommon.
Pediatric Chronic Pain
 In
a large series of 8-16 year-olds, 37.3% had
chronic pain, but only 5.1% had moderate or
severe chronic pain; percent increased with age
 They had a worse quality of life, missed more
days of school, were more likely to miss school
 Of those initially reporting chronic pain, 58%
still suffered at one year follow-up
 Peer relationships are often disrupted, deficient
Huguet A, Miro J. The Severity of Chronic Pediatric Pain: An epidemiological
Study. J Pain. 2008;9(3):226-236
Chronic Pain in Children
 Pain
that lasts at least 1, 3-6, >6 months
(contrast chronic from recurrent)
 Must be viewed within developmental,
ecobiopsychosocial domains
 Prematures, neonates fully capable of pain
perception and establishing pain “memory”
 Objective signs may be absent, in contrast to
acute pain
Am Pain Soc Bulletin Jan-Feb. 2001, pp10-12
Misconceptions That Can Lead to Under
Treatment of Pain in Children
 Children,
especially infants do not:
– Feel pain the way adults do
– Remember pain
 Lack of assessment for presence of pain
 Lack of knowledge of pediatric analgesics
– Use
– Dosing
– Adverse effects
 Preventing pain takes too much time
Pediatrics 2001; 108(3): 793-797
Identifiable Causes of Chronic Pain
Cancer
 Sickle cell disease
 HIV, pancreatitis, tumor-related,
neuropathies
 Cystic fibrosis
 Cerebral palsy
 Metabolic disorders
 Autoimmune/inflammatory disorders (JRA)

Idiopathic Chronic Pain in Children
Headaches, Migraine
 Recurrent Abdominal Pain (RAP)
 Musculoskeletal- neck, leg, back, arm,
chest
 Primary Juvenile Fibromyalgia
 Neuropathic, CRPS

What’s Causing Chronic Pain?
Autoimmune and
Inflammatory
Disorders
• e.g. rheumatoid arthritis, lupus
• 2 – 3 % of population
Mechanical or
“Wear-and-tear”
Disorders
• e.g. osteoarthritis
• prevalence very agedependant
Idiopathic Pain
Syndromes
• e.g. fibromyalgia,
headaches, irritable
bowel
• 15 – 20% of
population have sx.
severe enough to
seek medical
attention
• frequently co-exist
with inflammatory
and mechanical
disorders
The “Pain Vulnerable Child”
Both intrinsic and extrinsic factors predispose
child to develop more pain than peers under
similar circumstances
Whether patient develops “Pain Associated
Disability” is influenced by many factors,
including family behavior and cultural
expectations, access to health care and whether
certain kinds of health care are acceptable.
Extrinsic Factors for Chronic Pain
 Previous
pain experiences
 Social deprivation
 Physical or sexual abuse
 Parental modeling of chronic pain behaviors
 Sleep disturbances
 Decreased fitness, limited exercise
 Stressors- school difficulties, poor test taking,
bereavement
Intrinsic Factors for Chronic Pain
 Low
pain thresholds
 Female gender
 Hypermobility of joints
 Poor perceived control over pain
 Maladaptive coping strategies
 Difficult temperament
 Many of these are genetic
Malleson PN, Connell H, Bennett SM, Eccleston C. Chronic musculoskeletal and
other idiopathic pain syndromes. Arch Dis Child. 2001;84:189-192
Physiology of Pain Perception

Transduction

Transmission

Modulation

Perception

Interpretation

Behavior
Injury
Brain
Descending
Pathway
Peripheral
Nerve
Dorsal
Root
Ganglion
Ascending
Pathways
C-Fiber
A-beta Fiber
A-delta Fiber
17
Dorsal
Horn
Spinal Cord
Adapted with permission from WebMD Scientific American® Medicine.
Brain and Spinal Influences
on Pain Processing
Volume
•
•
•
•
•
Substance P
Glutamate and EAA
Serotonin (5HT2a, 3a)
Neurotensin
Nerve growth factor
Volume
+
• Descending analgesic
pathways
– Norepinephrine –
serotonin (5HT1a,b)
– Opioids
• GABA
• Cannabanoids
• Adenosine
Central Sensitization
 Nociceptive neurons in CNS develop lowered thresholds and increase
in suprathreshold responses. This also results from dysfunction of
endogenous descending pain control systems. Initially protective,
thresholds should return to baseline if tissue injury is absent. Instead,
they respond more to non-nocuous stimuli and outlast an initiating
trigger.
 Hyperalgesia- excessive sensitivity to a normally painful stimulus
 Allodynia- painful sensation to a normally non-painful stimulus. This is
an easy clinical sign of sensitization.
 Expansion of the receptive field- pain beyond the area of peripheral
nerve supply. After-stimulus unpleasant quality of pain- burning,
throbbing, tingling, numbness, etc.
 Chronicity- pain is no longer coupled to tissue injury, a sensory
“illusion”.
Idiopathic CS Syndromes “Family”
• Fibromyalgia syndromes
(FMS)
• Chronic headaches
• Irritable bowel syndrome
(IBS), RAP
• Chronic fatigue
syndromes (CFS)
• Orthostatic Intolerance
(OI), POTS
• Myofascial pain
syndromes (MPS)
• Posttraumatic stress
disorder (PTSD)
• Depression, anxiety
• Neuropathic, central pain
• Noncardiac chest pain
• Restless legs syndromes
(RLS)
• Periodic limb movement
disorder (PLMD)
• Temporomandibular
disorder (TMD)
• Multiple chemical
sensitivity (MCS) ?
• Female urethral
syndromes (FUS)
• Interstitial cystitis
• Primary dysmenorrhea
(PD), pelvic pain,
vulvodynia
• Sleep disorders
Daniel Lacey, MD
CSS Symptoms That Overlap
The neurologist sees chronic headache; the gastroenterologist
sees IBS; the dentist sees TMD; the cardiologist sees chest
pain/syncope; the rheumatologist sees fibromyalgia; the
gynecologist sees pelvic pain; the orthopod sees…etc…..
Headaches in Children
 Acute- trauma, infection
 Acute, recurrent- migraine or equivalents in
younger children
 Chronic, progressive- increased intracranial
pressure, degenerative disease, vascular,
hydrocephalus
 Chronic, stable- tension, medication
overuse, new daily persistent headaches
(NDPH), transformed migraine, pseudotumor
cerebri
Teens with Chronic Headaches
 Often not diagnosed and treated for many years!
 Are at a significantly greater risk for suicide
 Teens who have migraines with aura are 6 times more likely to
have a high suicide risk than those without aura.
 Are 3.5 times more likely to have a psychiatric disorder than
those without migraine
 Have at least a 50% chance of having at least one psychiatric
disorder if their headaches are daily. Abut 20% have major
depression and/or panic and anxiety disorders.
 Have a higher frequency of previous physical and/or sexual
abuse (30%)
CDH/Migraine Treatments
 Urgency and aggressiveness depends on whether
child is going to school, participating in normal
activities of daily living.
 May need inpatient admission for IV meds if has
been in “status migrainosus”, to ED many times.
Unfortunately, a common occurrence.
 Often a mixture of acute, abortive and preventive
medications and non-medical treatments is the most
successful regimen.
 Long-term headache freedom rate: 30%, many CDH
patients return to being episodic migraneurs
CDH/Migraine Treatment (2)
1. Amitriptyline, start 0.5-1mg/kg @ bedtime
(25mg maximum), increase to 1-3mg/kg
2. Topiramate, start 0.5mg/kg @ bedtime
(25mg maximum), increase to 50-100mg BID
3. Propranolol, start 1mg/kg divided BID
4. Consider valproate, tizanidine, gabapentin,
clonidine, venlafaxine, BOTOX, fluoxetine, ? opioids
5. “Alternatives”, riboflavin, Coenzyme Q10,
magnesium, butterbur, massage, Vitamin D
6. Biobehavioral, relaxation, imaging, SLEEP!
The current status of Recurrent
(RAP)Abdominal Pain
Definition of RAP
• Derives from the seminal description by Apley of
children between the ages of 4 and 16 years that
persists for more than 3 months and affects
normal activity.
• RAP is not a diagnosis !!!!!
• It may be the predominant clinical manifestation
of a large number of precisely defined organic
disorders, but in the majority of cases, RAP is
due to a ‘functional’ bowel disorder. Often see
IBS in patients with inflammatory bowel
diseases.
Prognosis of RAP in Children
• Pain resolves completely in 30% to 50% of patients by 2
to 6 weeks after diagnosis.
• This suggests that child and parent accept reassurance
that the pain is not organic and that environmental
modification is effective.
• Nevertheless, more long-term studies suggest that 30% to
50% of children who have functional abdominal pain in
childhood experience pain as adults, especially IBS.
• Thirty percent of patients who have functional abdominal
pain develop other chronic complaints as adults.
Treatment of RAP
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Reassure the family and patient that we believe the
pain is real and will treat accordingly
Reassure that the appropriate medical evaluations
have been done, we will not keep “fishing” or “shotgunning” unless symptoms change
Behavioral- relaxation, hypnosis, encourage “well”
behaviors, ignore and discourage “sick” behaviors
(PADS), biofeedback
Medication- tricyclics, pregabalin; specific GI meds
+/-
Pediatric Low Back Pain
40% of teens report low back pain (LBP)
 LBP plus other pain 46%
 LBP plus whole body pain 9%
 Boys more common if LBP only
 Girls more common if LBP plus other pain
 Function better if only have LBP, worse if
have LBP plus other pain, worst if have LBP
plus widespread pain

Pellise F, Balague F, Rajmil L, Cedraschi C, Aguirre M, Fontacha CG. Prevalence of
Low Back Pain and its Effect on Health-Related Quality of Life in Adolescents.
Arch Pediatr Adolesc Med. 2009;163(1):65-71
Red Flags
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Young age (particularly younger than 4
years)
Fever
Weight loss
Severe or constant pain
Nocturnal pain
Progression over the course of time
Hx of acute or repetitive trauma
Hx of malignancy
Bowel or bladder dysfunction
Interference with activity (self limitation)
Chronic Pediatric Chest Pain
•
•
•
•
•
Musculoskeletal 86%
Infectious (costochondritis) 9%
Asthma 3%
Gastrointestinal 0.6%
Cardiac 0.6%- more likely if occurs during
exertion
Rx- Effexor, NSAIDs
Reddy SRV, Singh HR. Chest Pain in Children and Adolescents. Pediatrics in
Review. 2010;33(1)e1-e9
Neuropathic Pain is Different
from Muscle/skeletal Pain
Neuropathic Pain
Muscle/Skeletal Pain
Chronic pain (months/years)
Acute pain (hours or days)
Caused by injury or disease to
nerves
Caused by injury or inflammation that
affects both the muscles and joints
Mild to excruciating pain that can last
indefinitely
Moderate to severe pain that
disappears when the injury heals
Causes extreme sensitivity to touch –
simply wearing light clothing is
painful
Causes sore, achy muscles
Sufferers can become depressed or
socially withdrawn because they see
no relief in sight and may experience
sleep problems
Sufferers can become anxious and
distressed but optimistic about relief
from pain
Wall PD. Textbook of Pain. 4th ed; 1999; Jude EB. Clin in Pod Med and Surg.1999;16:81-97;
Price SA. Pathophysiology: Clinical Concepts of Disease Processes. 5th ed; 1997: Goldman L.
Cecil Textbook of Medicine. 21st ed; 2000
Complex Regional Pain Syndrome
• COMPLEX- A combination of neuropathic
and sensory/neurovascular abnormalities
required
• REGIONAL- Often involves one or more
limbs, generalizes distally, contralateral
spread is also possible
• PAIN- Can be spontaneous and/or provoked,
not dermatomal in distribution
CRPS Symptoms
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Spontaneous burning or stinging pain (81%).
Electrical sensations or shooting pain
Allodynia, hyperalgesia, hyperesthesia
Vasomotor autonomic disturbance (87% color, 79%
temperature).
Sudomotor symptoms : sweating asymmetry (53%).
Trophic changes (altered skin, nail, or hair growth patterns)
Notable limb edema (80%) and associated stiffness.
Differences often present between “warm” and “cold”
Often a prior and/or family history of migraine
Pediatric CRPS
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90% in girls, mean age 11.8 years
Lower limbs 85%, especially the foot (75% of all cases) whereas
in adults, uppers twice as frequent
Frequently initiated by minor trauma, pain can occur
immediately or weeks to months after injury
Mean time to diagnosis 13.6 weeks
70% required adjuvant medication (amitriptyline, gabapentin)
Early mobilization and physical therapy are the mainstays of
treatment, kids respond better to non-invasive treatment
Most recover completely, 40% need inpatient stay, 20% relapse
Low AK, Ward K, Wines AP. Pediatric Complex Regional Pain Syndrome. J Ped Ortho. 2007;27(5):567572
Wilder TR. Management of Pediatric patients with Complex Regional Pain Syndrome. Clinical J Pain.
2006;22(5):443-448
Screening for Neuropathic Pain
Give one point each, if yes, for:
1. Pain feels like pins and needles
2. Pain feels hot and burning
3. Pain feels numb
4. Pain is like an electric shock
5. Pain is worse if touched by clothes or bed linen
Pain is limited to joints (subtract one point if yes)
If score is three or higher, pain is likely neuropathic
CRPS FACTS
 When not caught early, CRPS can be progressive
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(70% of cases)
NEED to find single diagnostic test, not yet
Early recognition through education
Early diagnosis equals BETTER prognosis
Need more effective treatments for CRPS
Research is desperately needed
In 40-60% of patients, pain is unrelieved
Cherny NI. The treatment of neuropathic pain: From hubris to humility. Pain.
2007;132:225-226
EARLY DIAGNOSIS CRITICAL
 Early diagnosis ( <3 mo.) with PROPER treatment, success
rate is highest, the best prognosis
 If left untreated, can lead to lifetime of severe, intractable,
chronic pain
 First 3-6 months after onset: 80-90% recovery rate
 6 months to 2 years 70-80%, after 2 years: 20%
PREVENT PADS!!!
BRAIN
SPINAL
CORD
PNS
Treatment Goals for Chronic Pain
 Minimize
physical pain and
discomfort
 Alleviate
 Prevent
anxiety
potentially deleterious
physiologic responses due to
pain
 PREVENT
PADS!!!!!
TREATMENT MODALITIES
 EDUCATION
 PHARMACOLOGICAL
 PHYSICAL
 BEHAVIORAL
 PSYCHOLOGICAL
 COMPLEMENTARY
THERAPIES
EDUCATION
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Reassurance: pain is real and biological
Reason for pain: dysregulation in pain neural
signaling system (ascending/descending)
Reason for failure of medical tests: looking in
the wrong places
Avoid mind-body split
Review how other factors influence pain:
anxiety, depression, beliefs, attention, memory;
hypervigilance, catastrophizing
PHYSICAL THERAPY
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Especially for patients who have
 chronic musculoskeletal pain
 complex regional pain syndrome
 become deconditioned due to inactivity
Requires specific expertise by PT
Exercise has specific benefits related to muscle
strengthening/functioning & posture, and
generalized benefits related to improved body
image, body mechanics, somatic self-efficacy,
sleep, and mood
PSYCHOLOGICAL INTERVENTIONS
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Cognitive-Behavioral Therapy (CBT)
Social Skills Training
Psychotherapy: child or family or both
Academic interventions
Treatment aimed at PTSD or unresolved
grief or trauma
FAMILY THERAPY
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To observe and alter family
contributors to pain perception
To participate in development &
implementation of behavioral plan
(e.g. how to get child to go to school)
To address family stress& problems
To improve family communication
To provide support& improve family
coping
CAM and OTHER PAIN TREATMENTS
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Acupuncture
Distraction
Muscle Relaxation/Breathing
Meditation
Hypnotherapy
Iyengar Yoga
Biofeedback
Massage Therapy
Art Therapy
PAIN-ASSOCIATED
DISABILITY SYNDROME
“PADS”
DOWNWARD SPIRAL
OF INCREASING
SYMPTOMS AND DISABILITY
Pain-Associated Disability Syndrome
(PADS)
Described in 1998 as “a spiral of increasing painrelated disruption of function” in children
 Seen in all types of pediatric chronic pain
disorders, head, visceral, musculoskeletal, etc.
 Preventing or addressing this should be the
primary goal of early pediatric pain management

Zeltzer LK, Tsao JC, Bursch B, Myers CD. Introduction to the Special Issue on Pain:
From Pain to Pain-Associated Disability Syndrome. J Pediatr Psychol.
2006;31(7):661-666
PADS Prevention
Must assess functional limitations at home,
school, etc., not just focus on pain as the only
dimension
 Sole treatment focus on medications often does
not result in functional restoration
 Best treatment program is multimodal with
emphasis on non-medical therapies, including
cognitive behavioral
 Functional improvement always precedes pain
reduction!!
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Chronic Pain Treatment Impediments
 Catastrophization
 Hypervigilance
 Focusing only on pain severity (0-10) and reduction
 Focusing only on mediation treatment
 Not focusing on function!!!
 Not emphasizing that restoration of normal function
almost always precedes pain reduction, not the other
way round
 For some patients, accepting that they may always
have pain will actually result in less pain (ACT)
Chronic Pain Service at Dayton Children’s
 Consult team includes Dr. Lacey, Cindy Brown MSN,
RN, CNS, Rehab therapist, Massage therapist by
referral, Psychologist, Dietician. A pharmacist is
consulted by the team as needed.
 Goal-To use a coordinated team approach to reduce
pain(NOT pain free) and to restore activities of daily
living.
 Available by referral through the Neurology Clinic
Treatment Goals
 Medications alone will not relieve the pain.
 Strategies that include exercise(up and out of bed
ambulating on a regular basis), massage, discussing
emotions, improving sleep, using relaxation and
deep breathing techniques/guided imaging and
distraction are utilized daily.
Important Do’s for our pain patients
 Do not re enforce the “sick role.”
 Be empathetic but firm regarding exercise, activities
of daily living.
 Do not use pain scales to “rate” pain(they were
developed for acute pain), instead focus on function
and daily activities.
Follow-up
 Patients continue to follow with Dr. Lacey and the
chronic pain team on an outpatient basis. Other
alternative therapies such as hydrotherapy,
acupuncture/acupressure, hypnosis may be initiated.
 Patients need to also follow a regular schedule at
home. School attendance may be limited during
acute exacerbations but school/activity involvement
is essential.
 Ongoing psychological counseling which focuses on
managing pain is crucial.
Follow-up
 Working with parents and other caregivers on an
outpatient basis is an important part of the planparents need to be coaches and not enablers.
 Goal to successful treatment is outpatient care;
repeat admissions should be limited.
 Key is to focus on multi-modal interventions and
again, to attend school/work and activities as much
as possible.
What’s new in 2014-15
 “Start Talking” Opioid Consent Requirement and the
use of OARRS.
 Support group for patients with chronic pain
 Education for the community providers and schools
regarding chronic pain
Questions
 Contact Cindy Brown MSN, RN, CNS
 [email protected]
 937-641-3000 X8934
 Thanks for your interest in pain management!