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Pediatric Idiopathic Chronic Pain Disorders

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1 Pediatric Idiopathic Chronic Pain Disorders
Lucinda M Brown MSN, RN, CNS Dr. Daniel Lacey MD, PhD January 2015

2 “ 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

3 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.

4 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 Treatment approach Resolution of underlying cause, usually self-limited Underlying cause and pain disorder; outcome is often pain control, not cure The causes of acute pain are often known, but the causes of chronic pain and its associated symptoms are not well understood.1 The pain experienced by patients with acute pain often can be alleviated. In general, the duration of acute pain is brief and has been well characterized.1 The time course of chronic pain, however, is usually indeterminate, and patients with chronic pain are often refractory to treatment.2 One definition of chronic pain is pain that has persisted beyond the time of normal healing; for research purposes, however, chronic pain is often defined as pain that has persisted at least 3 (sometimes 6) months.3 Because chronic pain can almost never be cured,4 optimal treatment usually involves helping the patient restore function and supporting a patient’s coping by utilizing approaches that minimize pain, maximize QOL, improve sleep, and enable patients to return to work and perform their regular activities.3,4 1. Galer BS, Dworkin RH. A Clinical Guide to Neuropathic Pain. Minneapolis, Minn: The McGraw-Hill Companies, Inc; 2000:7-8. 2. Rowbotham MC. Chronic pain: from theory to practical management. Neurology ;45(suppl 9):S5-S10. 3. Portenoy RK, Kanner RM. Definition and assessment of pain. In: Portenoy RK, Kanner RM, eds. Pain Management: Theory and Practice. Philadelphia, Pa: FA Davis Company. 1996:6. 4. Woolf CJ, Mannion RJ. Neuropathic pain: aetiology, symptoms, mechanisms, and management. Lancet. 1999;353:

5 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.

6 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.

7 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):

8 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


10 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):

11 Identifiable Causes of Chronic Pain
Cancer Sickle cell disease HIV, pancreatitis, tumor-related, neuropathies Cystic fibrosis Cerebral palsy Metabolic disorders Autoimmune/inflammatory disorders (JRA)

12 Idiopathic Chronic Pain in Children
Headaches, Migraine Recurrent Abdominal Pain (RAP) Musculoskeletal- neck, leg, back, arm, chest Primary Juvenile Fibromyalgia Neuropathic, CRPS

13 What’s Causing Chronic Pain?
Autoimmune and Inflammatory Disorders e.g. rheumatoid arthritis, lupus 2 – 3 % of population 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

14 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.

15 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

16 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:

17 Physiology of Pain Perception
Transduction Transmission Modulation Perception Interpretation Behavior Injury Brain Descending Pathway Dorsal Root Ganglion Peripheral Nerve Pain that manifests in diverse diseases may operate through common mechanisms. No pain mechanism is an inevitable consequence of a particular disease process. A given pain mechanism could be responsible for many different symptoms. More than one mechanism can operate in a single patient, and these may change over time. The main neurotransmitter in primary afferents is the excitatory amino acid glutamate. Activation of nociceptors causes the release of glutamate from central terminals; this release acts on the ionotropic glutamate receptor amino-3-hydroxy-5-methylisoxazole-4-proprionic acid postsynaptically to cause a rapid depolarization of dorsal horn neurons and, if threshold is reached, action potential discharge. Transduction: noxious stimuli cause ion channels in the membranes of thermal, mechanical, and chemical receptors located in the skin and tissue to open. Ions enter the receptor and depolarize it. Transmission: a wave of depolarization, or action potential, travels toward the spinal cord via A-beta (thinly myelinated) fibers and C (unmyelinated) fibers and up the ascending pathway. A-beta (light touch) fibers may become sensitized by CNS mechanisms to produce allodynia. Modulation/Perception: the ascending pain pathway carries impulses from the nociceptor to the sensory cortex; thus the sensation of pain is perceived. Interpretation: impulses are carried by 1st, 2nd, and 3rd order neurons. 1st order neurons carry impulses from the nociceptor to the dorsal horn of the spinal cord. 2nd order neurons carry impulses from the spinal cord to the thalamus, while 3rd order neurons carry the impulse from the thalamus to the primary sensory cortex. Crossman AR, Neary D. Neuroanatomy, 2nd ed. Churchill Livingstone, 2000. Galer B, Gammaitoni A, Alvarez N. 6. Immunology [XIV. Pain]. In: Dale DC, Federman DD, eds. WebMD Scientific American® Medicine. New York, NY:WebMD Corporation; 2003. Guyton AC, Hall J. Textbook of Medical Physiology, 10th Ed. Saunders, 2000. Woolf CJ, Mannion RJ. Neuropathic pain: aetiology, symptoms, mechanisms, and management. Lancet. 1999;353: Ascending Pathways C-Fiber A-beta Fiber Dorsal Horn A-delta Fiber Spinal Cord 17 Adapted with permission from WebMD Scientific American® Medicine.

18 Brain and Spinal Influences on Pain Processing
Volume Volume Substance P Glutamate and EAA Serotonin (5HT2a, 3a) Neurotensin Nerve growth factor Descending analgesic pathways Norepinephrine – serotonin (5HT1a,b) Opioids GABA Cannabanoids Adenosine +

19 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”.


21 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

22 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….. 17

23 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

24 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%)

25 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

26 CDH/Migraine Treatment (2)
1. Amitriptyline, start bedtime (25mg maximum), increase to 1-3mg/kg 2. Topiramate, start bedtime (25mg maximum), increase to mg 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!


28 The current status of Recurrent (RAP)Abdominal Pain

29 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.

30 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.

31 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 +/-

32 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

33 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)

34 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

35 Neuropathic Pain is Different from 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 There is a big difference between muscle aches and soreness and neuropathic pain. These types of pain differ in: How long you experience pain How the pain occurs The severity of symptoms The type of symptoms And the impact on your life 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

36 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

37 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

38 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): Wilder TR. Management of Pediatric patients with Complex Regional Pain Syndrome. Clinical J Pain. 2006;22(5):

39 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

40 CRPS FACTS When not caught early, CRPS can be progressive (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:

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!!!

42 BRAIN SPINAL CORD PNS Available drug treatments for chronic pain currently include simple analgesics such as acetaminophen, salicylates and other nonsteroidal anti-inflammatory drugs (NSAIDs), traditional opioid drugs, and adjuvant agents (eg, antidepressants, anticonvulsants). Typically, the choice of a drug is made by balancing the indications for treatment, the clinical efficacy of the drug, and its toxicity. An understanding of the mechanism of action of these drugs helps to establish their role in therapy. Better understanding of the pathophysiology of acute and chronic pain has led to numerous advances in pharmacologic management of painful disorders, including low back pain, migraine headache, fibromyalgia, postherpetic neuralgia, osteoarthritis, rheumatoid arthritis, and cancer-related neuropathic pain. Opioids mimic the actions of endogenous opioid peptides by interacting with mu, delta, or kappa opioid receptors. The opioid receptors are coupled to G1 proteins and the actions of the opioids are mainly inhibitory. They close N-type voltage-operated calcium channels and open calcium-dependent inwardly-rectifying potassium channels. This results in hyperpolarization and a reduction in neuronal excitability. They also decrease intracellular cAMP which modulates the release of nociceptive neurotransmitters (eg, substance P). Inhibition of prostaglandin synthesis by cyclooxygenase is the principal mode of the analgesic and anti-inflammatory actions of NSAIDs. Cyclooxygenase is inhibited irreversibly by aspirin and reversibly by other NSAIDs. The widespread inhibition of cyclooxygenase is responsible for many of the adverse effects of these drugs. NSAIDs also reduce prostaglandin production within the CNS. This is the main action of paracetamol. Argoff CE. Pharmacologic management of chronic pain. J Am Osteopath Assoc. 2002;102(suppl 3):S21-S27. Aronson MD. Nonsteroidal anti-inflammatory drugs, traditional opioids, and tramadol: contrasting therapies for the treatment of chronic pain. ClinTher. 1997;19:420-32; discussion Bovill JG. Mechanisms of actions of opioids and non-steroidal anti-inflammatory drugs. Eur J Anaesthesiol Suppl. 1997;15:9-15.

43 Treatment Goals for Chronic Pain
Minimize physical pain and discomfort Alleviate anxiety Prevent potentially deleterious physiologic responses due to pain PREVENT PADS!!!!!


45 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

46 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

Cognitive-Behavioral Therapy (CBT) Social Skills Training Psychotherapy: child or family or both Academic interventions Treatment aimed at PTSD or unresolved grief or trauma

48 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

Acupuncture Distraction Muscle Relaxation/Breathing Meditation Hypnotherapy Iyengar Yoga Biofeedback Massage Therapy Art Therapy


51 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):

52 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!!

53 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)

54 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

55 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.

56 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.

57 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.

58 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.

59 What’s new in “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

60 Questions Contact- Cindy Brown MSN, RN, CNS
X8934 Thanks for your interest in pain management!

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