Pediatric Idiopathic Chronic Pain

Disorders

Lucinda M Brown MSN, RN, CNS

Dr. Daniel Lacey MD, PhDJanuary 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, ³3 months

Treatmentapproach

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 ClassificationNociceptive 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?

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

Mechanical or “Wear-and-tear” Disorders • e.g. osteoarthritis • prevalence very age-dependant

Autoimmune and Inflammatory Disorders• e.g. rheumatoid arthritis, lupus• 2 – 3 % of population

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

Descending Pathway

PeripheralNerve

Dorsal RootGanglion

C-Fiber

A-beta Fiber

A-delta Fiber

AscendingPathways

Dorsal Horn

Brain

Spinal Cord

Adapted with permission from WebMD Scientific American® Medicine.17

Volume

+

Volume

Brain and Spinal Influences on Pain Processing

• Substance P• Glutamate and EAA • Serotonin (5HT2a, 3a)

• Neurotensin• Nerve growth factor

• 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, infectionAcute, recurrent- migraine or equivalents in

younger childrenChronic, 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 suicideTeens 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 migraineHave 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 TreatmentsUrgency 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

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 “shot- gunning” 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 Pain40% of teens report low back pain (LBP)LBP plus other pain 46%LBP plus whole body pain 9%Boys more common if LBP onlyGirls more common if LBP plus other

painFunction 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 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 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 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):567-572

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 (70% of cases)

NEED to find single diagnostic test, not yetEarly recognition through educationEarly diagnosis equals BETTER prognosis Need more effective treatments for CRPSResearch 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 rate6 months to 2 years 70-80%, after 2 years: 20%

PREVENT PADS!!!

BRAIN

PNS

SPINALCORD

Treatment Goals for Chronic Pain

Minimize physical pain and

discomfort

Alleviate anxiety

Prevent potentially deleterious

physiologic responses due to

pain

PREVENT PADS!!!!!

TREATMENT MODALITIES

EDUCATIONPHARMACOLOGICALPHYSICALBEHAVIORALPSYCHOLOGICALCOMPLEMENTARY THERAPIES

EDUCATION 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

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

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

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

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 pain-related 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!!

Chronic Pain Treatment Impediments

CatastrophizationHypervigilanceFocusing only on pain severity (0-10) and

reductionFocusing only on mediation treatmentNot 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 plan-parents 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, CNSbrownl@childrensdayton.org937-641-3000 X8934

Thanks for your interest in pain management!