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

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Presentation on theme: "Pediatric Idiopathic Chronic Pain Disorders Lucinda M Brown MSN, RN, CNS Dr. Daniel Lacey MD, PhD January 2015."— Presentation transcript:

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 CharacteristicAcute PainChronic Pain CauseGenerally knownOften unknown Duration of painShort, 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

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 u 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 u They had a worse quality of life, missed more days of school, were more likely to miss school u Of those initially reporting chronic pain, 58% still suffered at one year follow-up u 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 u Pain that lasts at least 1, 3-6, >6 months (contrast chronic from recurrent) u Must be viewed within developmental, ecobiopsychosocial domains u Prematures, neonates fully capable of pain perception and establishing pain “memory” u Objective signs may be absent, in contrast to acute pain Am Pain Soc Bulletin Jan-Feb. 2001, pp10-12

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10 Misconceptions That Can Lead to Under Treatment of Pain in Children u Children, especially infants do not: –Feel pain the way adults do –Remember pain u Lack of assessment for presence of pain u Lack of knowledge of pediatric analgesics –Use –Dosing –Adverse effects u Preventing pain takes too much time Pediatrics 2001; 108(3):

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

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

13 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

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 u Previous pain experiences u Social deprivation u Physical or sexual abuse u Parental modeling of chronic pain behaviors u Sleep disturbances u Decreased fitness, limited exercise u Stressors- school difficulties, poor test taking, bereavement

16 Intrinsic Factors for Chronic Pain u Low pain thresholds u Female gender u Hypermobility of joints u Poor perceived control over pain u Maladaptive coping strategies u Difficult temperament u 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 Transduction Transmission Transmission Modulation Modulation Perception Perception Interpretation Interpretation Behavior Behavior Injury Descending Pathway Peripheral Nerve Dorsal Root Ganglion C-Fiber A-beta Fiber A-delta Fiber Ascending Pathways Dorsal Horn Brain Spinal Cord Adapted with permission from WebMD Scientific American ® Medicine. 17

18 Volume + Brain and Spinal Influences on Pain Processing Substance P Glutamate and EAA Serotonin (5HT 2a, 3a ) Neurotensin Nerve growth factor Descending analgesic pathways –Norepinephrine – serotonin (5HT 1a,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”.

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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…..

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!

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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 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 Reassure that the appropriate medical evaluations have been done, we will not keep “fishing” or “shot- gunning” unless symptoms change - relaxation, hypnosis, encourage “well” behaviors, ignore and discourage “sick” behaviors (PADS), biofeedback Behavioral- relaxation, hypnosis, encourage “well” behaviors, ignore and discourage “sick” behaviors (PADS), biofeedback Medication- tricyclics, pregabalin; specific GI meds +/- Medication- tricyclics, pregabalin; specific GI meds +/-

32 Pediatric Low Back Pain 40% of teens report low back pain (LBP) 40% of teens report low back pain (LBP) LBP plus other pain 46% LBP plus other pain 46% LBP plus whole body pain 9% LBP plus whole body pain 9% Boys more common if LBP only Boys more common if LBP only Girls more common if LBP plus other pain 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 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 ;163(1):65-71 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) Young age (particularly younger than 4 years) Fever Fever Weight loss Weight loss Severe or constant pain Severe or constant pain Nocturnal pain Nocturnal pain Progression over the course of time Progression over the course of time Hx of acute or repetitive trauma Hx of acute or repetitive trauma Hx of malignancy Hx of malignancy Bowel or bladder dysfunction Bowel or bladder dysfunction Interference with activity (self limitation) 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 Neuropathic PainMuscle/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

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%). Spontaneous burning or stinging pain (81%). Electrical sensations or shooting pain Electrical sensations or shooting pain Allodynia, hyperalgesia, hyperesthesia Allodynia, hyperalgesia, hyperesthesia Vasomotor autonomic disturbance (87% color, 79% temperature). Vasomotor autonomic disturbance (87% color, 79% temperature). Sudomotor symptoms : sweating asymmetry (53%). Sudomotor symptoms : sweating asymmetry (53%). Trophic changes (altered skin, nail, or hair growth patterns) Trophic changes (altered skin, nail, or hair growth patterns) Notable limb edema (80%) and associated stiffness. Notable limb edema (80%) and associated stiffness. Differences often present between “warm” and “cold” Differences often present between “warm” and “cold” Often a prior and/or family history of migraine Often a prior and/or family history of migraine

38 Pediatric CRPS 90% in girls, mean age 11.8 years 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 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 Frequently initiated by minor trauma, pain can occur immediately or weeks to months after injury Mean time to diagnosis 13.6 weeks Mean time to diagnosis 13.6 weeks 70% required adjuvant medication (amitriptyline, gabapentin) 70% required adjuvant medication (amitriptyline, gabapentin) Early mobilization and physical therapy are the mainstays of treatment, kids respond better to non-invasive treatment 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 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 ;22(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: Give one point each, if yes, for: 1. Pain feels like pins and needles 1. Pain feels like pins and needles 2. Pain feels hot and burning 2. Pain feels hot and burning 3. Pain feels numb 3. Pain feels numb 4. Pain is like an electric shock 4. Pain is like an electric shock 5. Pain is worse if touched by clothes or bed linen 5. Pain is worse if touched by clothes or bed linen Pain is limited to joints (subtract one point if yes) 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:

41 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!!!

42 BRAIN PNS SPINAL CORD

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

44 TREATMENT MODALITIES EDUCATION EDUCATION PHARMACOLOGICAL PHARMACOLOGICAL PHYSICAL PHYSICAL BEHAVIORAL BEHAVIORAL PSYCHOLOGICAL PSYCHOLOGICAL COMPLEMENTARY THERAPIES COMPLEMENTARY THERAPIES

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

47 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

48 FAMILY THERAPY To observe and alter family contributors to pain perception 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 participate in development & implementation of behavioral plan (e.g. how to get child to go to school) To address family stress& problems To address family stress& problems To improve family communication To improve family communication To provide support& improve family coping To provide support& improve family coping

49 CAM and OTHER PAIN TREATMENTS Acupuncture Acupuncture Distraction Distraction Muscle Relaxation/Breathing Muscle Relaxation/Breathing Meditation Meditation Hypnotherapy Hypnotherapy Iyengar Yoga Iyengar Yoga Biofeedback Biofeedback Massage Therapy Massage Therapy Art Therapy Art Therapy

50 PAIN-ASSOCIATED DISABILITY SYNDROME “PADS” DOWNWARD SPIRAL OF INCREASING SYMPTOMS AND DISABILITY

51 (PADS) Pain-Associated Disability Syndrome (PADS) l Described in 1998 as “a spiral of increasing pain- related disruption of function” in children l Seen in all types of pediatric chronic pain disorders, head, visceral, musculoskeletal, etc. l 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 l Must assess functional limitations at home, school, etc., not just focus on pain as the only dimension l Sole treatment focus on medications often does not result in functional restoration l Best treatment program is multimodal with emphasis on non-medical therapies, including cognitive behavioral l 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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