Speci \201 c content Th e nature of the discussion with the family about functional pain p roblems should be individualized based on the educational, social, and cultural characteris tics of the family. Regardless, the complex nature of pain and the biopsychosocial dimensio ns should be SECTION 4 pain in specific populations and diseases 234 Provider communication Because children with multiple pain problems typically see multi- pl e specialists (neurology for headache, rheumatology for joint and musculoskeletal pain, gas troenterology for abdominal pain, ortho- paedics for back pain, cardiologists for POTS) in addition to their primary care providers, it is imperative that a mechanism for com- munica tion among providers be developed. Specialists may not address or even ask about other pain sites which they perceive are out of their realm of expertise and assume those will be add ressed by others. Th ey may off er suggestions that they perceive are help- ful for the spe cifi c problem that they are asked to address but may in fact be detrimental to the whole c hild. Additionally, there is also oft en signifi cant variability of opinion among provider s as to the etiology, evaluation and treatment of chronic pain problems. Konenjiberg et al. ( 2004 ) gave 135 pediatricians a series of case scenarios involving chronic pain and aske d how clinicians would address them. Th ere was limited consensus on etiology, on spe- cifi c diagnostic testing, and on treatment. A similar situation likely occurs in clinical prac tice as these problems are oft en quite confus- ing and subject to multiple diagnostic opin ions. As a result, fami- lies may feel confused by the lack of clarity among their physicia ns and continue a futile search for the right answer. Tests may oft en be unnecessarily rep eated and agents prescribed that may interact negatively. It is imperative therefore in sit uations in which multiple pains exist that the clinical team be in communication and speak, as much as possible, in one voice. for pain or spasm oft en have a side eff ect of sedation which can be an added benefi t of their use if they are taken at bedtime. Physical activity It is not uncommon when one is in pain to limit physical activ- ity. Th i s may occur because of associated pain, anxiety about pain which may occur as a result, dep ression, or diminished interest in social activities but regardless, it oft en leads to dec onditioning which increases pain and disability. Decreased exercise and weight bearing can also promote osteopenia. It is oft en valuable to have a skilled physical therapist evaluate the child \200\231s mus culoskeletal system to determine whether formal physical therapy may be needed or whether a home exercise program will suffi ce. Th e therapist can also evaluate the need for desensi tization and can emphasize the importance of pacing and stretching. He or she can identify particularly tight or weak areas and if the child is hypermobile, can help with strengtheni ng specifi c muscles to com- pensate for ligamentous laxity and promote joint stability. If only core strengthening and building endurance are felt to be necessary, then physical the rapy may not be necessary and a graduated exercise pro- gramme may be substituted. Either w ay, increasing movement will promote a sense of well-being and an inevitable reduction of p ain. Addressing anxiety and depression As mentioned repeatedly, anxiety and depression are oft e n fellow travelers with chronic pain and may have a role in initiating it, maintaining it, or both. Clinicians should address this with families in the context of the numerous factor s involved with chronic pain, as opposed to the organic versus psychological paradigm. Th e re is an extensive literature on psychological strategies reducing the arousal in pain sens itive individuals and response to these strategies does not imply psychological causation ( Eccleston et al., 2009 ). Th e psycholo- gist can teach relaxation strategies, can address catastrophization, and explore the thoughts that might be promoting incapacitation from a n ociceptive stimulus. Most pediatric pain programs employ or are connected to pediatric psyc hologists and their evalu- ation should be a critical part of the assessment and treatment of pain in children. Many families may be resistant to this part of the evalu- ation and th ey oft en fear that they will be blamed for their child \200\231s pain. As a result, they m ay state (sometimes quite strongly) that that component of the evaluation is unnecessary as their child \200\231s pain is real. It is important to dispel the notion that the purpose of psycho- logical evaluation and treatment in chronic pain is to unearth subtle psychologi cal causation and blame parents for the child \200\231s problem. It is to identify every tr igger that may promote and maintain pain and to teach techniques to help ameliorate it. Occ asionally, psychophar- macological intervention may be necessary as well. Conclusion Th is chapter has attempted to review the commonalities and overlaps between the common pain problems that children experience, most of which are functional in nature. Th is area has had limited attention in the past, most likely due to the fact that pain proble ms are typically evaluated by experts in the specifi c organ system from which the pain see ms to stem and they may not have expertise in pain that stems from other areas. When one lo oks at these problems from a distance however, there are remarkable similarities in their t heoretical aetiolo- gies, in their impact on the child and family, in associated symptoms, and in their treatment. Th e adequate care of these problems demands an appreciation of the biopsychosocial model which looks at the child as a whole and on a multidisciplinary appro ach which allows for mul- tiple streams of information to impact on the child \200\231s car e. Case example Constance Payne is a 15-year-old young woman who was referred to the gastroenterology progr am by her primary care provider for longstanding abdominal pain which had recently escalate d in intensity. Constance has had periumbilical pain since the age of 12 but recently since high school entry her discomfort had increased. In the past, Constance had missed 1 to 2 d ays of school a month for her abdominal pain, but in the fall of ninth grade began missing at least one day per week. Attending to other symptoms Other symptoms may amplify the incapacitation associated with c hronic pain, regardless of site. Dizziness, nausea, and fatigue may result from pain but ma y also potentiate it. If at all possible, these symptoms should be addressed. Oft en POTS m ay be associated with these symptoms or with a general sensation of feeling unwell but it i s oft en not considered and orthostatic vital signs are not obtained. Because the treatment of POTS is oft en relatively straightforward and its amelioration can dramatically help th e child \200\231s pain and mood, obtaining blood pressure and heart rate measurements both lying down and then standing up should be a routine part of the evaluation of any type of c hronic pain. Th e gastroenterologist who evaluated her identifi ed no pat- tern to her symptoms and no o bvious triggers for it. Her physi- cal examination and subsequent laboratory investigation did not off er a specifi c pathological explanation for her pain nor did the endoscopy that was performed subsequently. A number CHAPTER 23 chronic pain syndromes in childhood: one trunk, many branches 235 Chang , L. , Sundaresh , S. , Elliott , J. , Anton , P. A. , Baldi , P. , Licudine , A. , e t al . ( 2009 ). Dysregulation of the HPA axis in irritable bowel syndrome . Neurogastroent erol Motil , 21 , 149 \200\223159. of medications were prescribed for Constance including hyo- scyamine and omeprazole which w ere both ineff ective. During the course of this evaluation, she reported that recently, she had developed an almo st continuous headache and she oft en felt dizzy. Additionally, she described her vision as \200\230blurry \200\231. She was subsequently referred to a neurologist who ordered a mag - netic resonance imaging scan of her head and sent her to an oph- thalmologist. None of th ese investigations yielded an explanation for her pain. Constance began taking ibuprofen to dampen her headache but stated that this medication aggravated her stom- ach pain. Concern was raised about her dizziness and she was referred to a cardiologist who identifi ed a si gnifi cant disparity between her heart rate when she changed position from lying to standin g. He suggested a trial of salt and increased fl uids. Chartier , M. J. , Walker , J. R. , and Naimark , B. ( 2007 ). Childhood abuse, adult healt h, and health care utilization: results from a representative community sample . Am J Epide miol , 165 , 1031 \200\2231038. Coakley , R. and Schechter , N. L. ( 2013 ). Chronic pain is like \200 :the use of analogy and metaphor in the treatment of chronic pain in children . Pediatr Pain Lett , 15 (1), 1 \200\2238. Chen , E. , Craske , M. G. , Katz , E. R. , Schwartz , E. , and Zeltzer , L. K. ( 2000 ). P ain-sensitive temperament: does it predict procedural distress and response to psychologica l treatment among children with cancer? J Pediatr Psychol , 25 , 269 \200\223278. Drossman , D. A. ( 1999 ). An integrated approach to the irritable bowel syndrome . Aliment Pharmacol Th er , 13 (Supplement s2), 3 \200\22314. Eccleston , C. , Crombez , G. , Scotford , A. , Clinch , J. , and Connell , H. ( 2004 ). Ad olescent chronic pain: patterns and predictors of emotional distress in adolescents with ch ronic pain and their parents . Pain , 108 , 221 \200\223229 Eccleston , C. , Palermo , T. M. , deWilliams , A. C. , Lewandowski , A. , and Morley , S. ( 2009 ). Psychological therap ies for the management of chronic and recurrent pain in children and adolescents . Cochrane Database Syst Rev , 2 , CD003968 . Because of these multiple complaints, Constance became increasingly inactive. Th is inactiv ity was aggravated by additional reports of achiness in her neck, knees, and back. She beca me increasingly incapacitated and was referred to an orthopedic surgeon and rheumatologist, neither of whom could explain her pain and deterioration. Her sleep became increasingly di s- ordered and she began napping during the day and having dif- fi culty falling asleep at night and awakening in the morning. She complained of crushing fatigue. Evans , S. , Keenan , T. R. , and Shipton , E. A. ( 2007 ). Psychosocial adjustment and phy sical health of children living with maternal chronic pain . J Paediatr Child Health , 43 , 262 \200\223270. Fichtel , A. and Larsson , B. ( 2002 ). Psychosocial impact of headache and comorbidity wit h other pains among Swedish school adolescents . Headache , 42 , 766 \200\2237758. Constance and her mother began appearing frequently in the emergency department because, ac cording to her mother, \200\230some- thing has to be done \200\231. Two of these visits en ded in hospitalization for observation and pain control. Constance began losing interest in friends and in activities that had previously brought her pleasure. Foulkes , T. and Wood , J. N. ( 2008 ). Pain genes . PLoS Genet , 4 , e1000086 . doi: 10.1371/journal.pgen.1000086. Geisser , M. E. , Glass , J. M. , Rajcevska , L. D. , Clauw , D. J. , Williams , D. A. , Ki leny , P. R. , et al . ( 2008 ). A psychophysical study of auditory and pressure sensitivit y in patients with fi bromyalgia and healthy controls . J Pain , 9 , 417 \200\223422. With all of these complaints and no explanation, Mrs Payne began to express her worry that her cadre of doctors was missing something essential that would explain this unique constel lation of fi ndings. She herself had a cousin who had similar symptoms and was eventually f ound to have a serious underlying disease. She stated that in September she had a healthy d aughter who did well in school and was an excellent athlete. Now, Constance can barely care for herself. Even though she herself has fi bromyalgia, Mrs Payne feels she has to stay ho me and care for Constance. She reports as well the fact that although Constance sees many d octors, her physi- cians never seem to talk to each other and she feels the overwhelm- ing burden of coordinating her daughter \200\231s care due to her fear that something will be m issed if she is not perpetually vigilant. Giesecke , T. , Gracely , R.H. , Grant , M. A. , Nachemson , A. , Petzke , F. , Williams , D. A. , et al . ( 2004 ). Evidence of augmented central pain processing in idiopathic chron ic low back pain . Arthritis Rheum , 50 , 613 \200\223623. Goldenberg , D. , Mayskiy , M. , Mossey , C. , Ruthazer , R. , and Schmid , C. ( 1996 ). A randomized, double-blind crossover trial of fl uoxetine and amitriptyline in the treatment of fi bromyalgia . Arthritis Rheum , 39 , 1852 \200\2231859. Goodman , J. E. , and McGrath , P. J. ( 2003 ). 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Rozen , T. D. , Roth , J. M. , and Denenberg , N. ( 2006 ). Cervical spine joint hypermobil ity: a possible predisposing factor for new daily persistent headache . Cephalalgia , 26 , 1182 CHAPTER 24 Non-in \202 ammatory musculoskeletal pain Jacqui Clinch Summary Non-infl ammatory musculoskeletal pain is common in chil- dren and adolescents, and when persistent or widespread, can have a negative impact on physical and psychological we ll-being. Diagnostic labels and criteria are not uniform in the current lit- erature, but m usculoskeletal pain may present as widespread pain or juvenile fi bromyalgia, complex regio nal pain syndrome, or in association with joint hypermobility. Chronic musculoskeletal pain , irrespective of its trigger, can bring persistent and recurrent distress, disability, and widespread family disruption. Once seri- ous medical causes have been excluded by history, examination, and relevant investigations, the focus should be on rehabilitation. Multidisc iplinary team management to facilitate cohesive working and the introduction of psychologic al and physical therapies can improve outcome. Further research is required to defi ne the role of pharmacological interventions. Epidemiology Th ere is limited data on the prevalence and incidence of muscu- loskeletal pa in in youth, and on the prevalence of pain-associated suff ering or disability. However, pa in is a common experience during childhood. One study showed that 83% of school-aged childr en had experienced an episode of pain during the preceding 3 months (Roth- Isigkeit et al., 2005 ). Pain is a normal sensation but becomes disabling when it persists and is associate d with suff ering. In this same study, 30.8% of the children and adolescents stated that th e pains had been present for over 6 months. Musculoskeletal pains accounted for 64% of all the pains that were reported (Roth-Isigkeit et al., 2005 ). Other studies support this fi n ding (Brattberg, 2004 ; Perquin et al., 2000 ). \231 Th e epidemiology of DIP (also refer red to as juvenile fi bromyal- gia or chronic widespread pain) is diffi cult to accurately assess. Th ere are no universally agreed diagnostic criteria, although some authors have su ggested using the term juvenile fi bromyalgia and proposed diagnostic criteria akin to the adult fi bromyalgia criteria (Kashikar-Zuck et al., 2006 ). Th e HUNT study (Hoft un et al. , 2008) showed 8.5% of a population of 7373 adolescents in Norway fulfi lled the criteria f or DIP; whereas a UK study showed the prevalence of chronic widespread pain in 17-year-old school children to be 4.3% (Deere et al., submitted). Introduction Th e most common chronic musculoskeletal pain conditions reviewed in pediatric rheumatology settings include diff use idi- opathic musculoskeletal pain (DIP; also called juvenile fi bromyal- gia or chronic widespread pain), chronic pain related to childhood jo int hypermobility, complex regional pain syndrome (CRPS), chronic back pain, and persistent joint pain following previous or controlled infl ammation (e.g. juvenile idiopathic arthri tis (JIA)) (Malleson and Clinch, 2003 ; O \200\231Sullivan et al., 2011 ). \231 Hypermobility, as currently defi ned, is present in 19.8% of the normal UK adolescen t population (Clinch et al., 2011 ). Th ere is a relationship between localized pain and si gnifi cant hypermo- bility (Beighton score 6 or above); this is discussed later in the chap ter (Tobias et al., 2013 ). For many young people presenting to clinic, regardless of whether the cause of their pain i s known, the chronic experience of pain has oft en had a large and wholly negative impact o n their physi- cal and psychological well-being, and their family (Malleson and Clinch, 200 3 ). Children who suff er persistent musculoskeletal pain and other symptoms also have a si gnifi cant chance of developing chronic widespread pain and pain-associated disability in a dult life (Jones et al., 2007 ). \231 Low-back pain (LBP) is commonly reported. A UK observational cohort study reported t he prevalence of LBP as 16.3% in 17-year- old adolescents (Deere et al., submitted). A cros s-sectional study of school-age children showed prevalence of LBP that lasted for at least 1 month was 24% (Watson et al., 2002 ), the HUNT study showed this to be 16.7% for girls an d 11% for boys (1 day a week for at least 3 months; Hoft un et al., 2011 ). Th is chapter will review: (1) the epidemiology of musculoskeletal pain in childhood, (2) e valuation of the impact of chronic pain, (3) the clinical features of common pain presentat ions and their relevance to diagnosis and treatment planning, and (4) rehabilitation interv en- tions aimed at the management of chronic musculoskeletal pain. \231 Th e prevalence of CRPS in adults is 26 per 100 000 person years (De Mos et al., 200 7 ). In children the epidemiology has not been robustly evaluated but recent studies have d emonstrated that approximately 90% of the cases reported are females in a range of 8 to 16 years (Sherry et al., 1999 ). Th ere tends to be a delay in SECTION 4 pain in specific populations and diseases 238 recognizing the diagnosis (Stanton Hicks, 2010 ). A complicating factor is the lack of clea r diagnostic criteria for children. \231 Perceived thermodysregulation \200\224this is observed more com- monly in adolescent girls. Limbs are particularly cool and mot- tled. Occasionally there will be areas that ar e very red and hot to touch on a background of the mottled skin. Th ere may also be an abno rmal perception of temperature, but an increase in ther- mal pain sensitivity has only been documented in adults (Geisser et al., 2003 ). \231 In JIA it is clear that persistent pain is a signifi cant problem for many children (Anthony et al., 2007 ). Approximately 40% describe pain 5 years aft er diagnosis (Lovell a nd Walco, 1989 ). A signifi cant minority progress to develop localized or diff use mus- cu loskeletal pain that is not directly related to the control of their infl ammatory conditio n (Haverman et al., 2012 ). \231 Autonomic dysfunction \200\224pain is a powerful stressor. Continuous pain signals, immobility, and fatigue act directly on the auto- nomic system (Cohen et al., 2001 ). In an environment of physi- cal and emotional anxiety, the sympathetic system is more active. Th is leads to tachycardia, hyperventilation (compounded with panic attacks), cold sweats, bl urred vision, abdominal pain and extreme pallor. Girls particularly complain of nausea, diz ziness, and episodes of feeling faint. Children look unwell during these episodes of increa sed pain. It is not unusual for attending paedia- tricians to investigate cardiovascular, n eurological, and gastroin- testinal systems in an attempt to elicit pathology. Looking generally across studies a number of features are clear: \231 Girls experience more musculoskeletal pain than boys (Groholt, 1994; Hoft un et al., 2011 ). \231 Children living in low-educated, low-income families have a 1.4-fold increase in the odds of having back pain (Groholt et al., 1994). \231 Th e incidence of chronic widespread musculoskeletal pain peaks in older adolescence (Hoft un et al., 2011 ). \231 Multiple common symptoms (including joint pain, headaches, abdominal pain) in childh ood are associated with a moderately increased risk of chronic widespread pain in adulthood (Jones et al., 2007 ). \231 Musculoskeletal disequilibrium \200\224these young people are still growing, oft en in their peripubertal growth spurt, and muscu- loskeletal pain can have lasting eff ects on the fi nal positioning (Lightman et al., 1987 ). Proprioceptive signals from the joints ar e reduced and the limb held in a rigid, fi xed position (Sherry et al., 1999 ; Walco et al. , 2010 ). Legs may \200\230give way \200\231. Knees and hips are held fl exed, feet are in verted, and hands are clenched with fl exed wrists. Th ese positions are oft en described a s the most comfort- able. Muscles and tendons quickly tighten and this complicates the pain and disability. Th e adaptive positioning of a young person with leg or abdominal pains pa rticularly aff ects the gait and resting positions and thus alters the loads on the spine a nd pelvis. Clinical features of chronic musculoskeletal pain As many features of musculoskeletal pain conditions are shared by most children, it is appropriate to describe the general presenta- tions at this stage, and then concentrate on specifi c pain-related conditions in their ow n right. General features It is not unusual for musculoskeletal pain to start in a localized area of the body (Buskila and Neumann, 2000 ). Th e pain can intensify and radiate to other areas. It is associated oft en with a reluctance to mobilize, and avoidance of movement. In adult s, avoidance and fear of pain and movement can reduce fi tness and further increase in pain (Elbaz et al., 2009 ), but in children this occurs on the background of a musculoskeletal system that is still develop- ing. Discomfort and pain intensity increase and become consta nt. As discrete pains multiply and continue, the young person may avoid contact with, or us e of, an area of the body aff ected, and this can then lead to muscular spasms, and abnorma lities of posture and gait. As gait alters, the resultant asymmetry can lead to other vulne rable sites (including lower lumbar, anterior knee, and lateral aspect of pelvis) taking an altered load and causing further discom- fort. Over time, aff ected limbs may adopt fl exe d positions, and ten- dons tighten, further exaggerating imbalance and pain. Th ere are a number of infl uences on physical behavior, including psychosocial factors. Wh at can fi rst appear as a structural anomaly or physical constraint may be better explained by fear of pain on movement, or postural compensation due to overprotection of a painful b ody part. Although an important area, there is very lit- tle research of the eff ects of a chronic stressor such as pain during critical periods of physical development, or in the me asurement of movement constrained by pain. Speci \201 c childhood musculoskeletal pain conditions Di \200 use idiopathic pain syndro mes (juvenile \201 bromyalgia/chronic widespread pain) Th is describes widespread pain, o ft en located over muscles and joints, and with signifi cant pain associated disability (Ma lleson et al., 2001 ). Th e term juvenile fi bromyalgia has been used, with diagnostic crit eria similar to those used in adults (Hoft un et al., 2011 ), including a history of widesp read pain for at least 3 months, and the presence of 11 of 18 bilateral tender points (occi put, low cervical, trapezius, supraspinatus, second rib, lateral epicondyle, gluteal, great er trochanter, and knees). It should be noted that these American College of Rheumatology c riteria have recently been revised and now do not include tender points as it is recognized that widespread pain and related symptoms are good markers for adult fi bromyalgia (Wolfe et al., 2010 ). Associated symptoms and signs \231 Hypervigilance and pain sensitivity that is greater than expected from a given physi cal trigger. Children oft en report a height- ened awareness of pain and pain associated cu es (American Pain Society, 2012 ; Malleson and Clinch, 2003 ; O \200\231Sullivan et al., 20 011). It is unclear whether this is caused by fear of pain, or increased sensitivity of pai n signaling. Clinically, this presents as young people describing unbearable pain on minima l skin contact, and heightened fear of being touched, for example, on examination. CHAPTER 24 non-inflammatory musculoskeletal pain 239 Th e onset of pain in DIP/juvenile fi bromyalgia is oft en gradual. Th ere may have been an initial insult or trauma but oft en there is no obvious trigger and only vague recollectio ns of the time of onset. Th e pain is generalized. Th ere may be areas of allodynia (profou nd hypersensitivity to light touch) and subjective hyperalgesia but there is oft en an abse nce of the autonomic changes (including reduced cutaneous perfusion, localized swelling, sh iny stretched appearance) seen in more localized pain conditions (O \200\231Sullivan et al. , 2011 ). Box 24.1 Budapest clinical diagnostic criteria for complex regional pain syndrome 1. Continuing pain, which is disproportionate to any inciting event. 2. Must report at least one symptom in three of the four following categories: a. Sensory: reports of hyperesthesia and/or allodynia. What is striking in the young people with diff use pain is the asso- ciated fatigue, poor s leep pattern, and extremely low mood. It is widely believed, however, that low mood in adol escents are reactive (to the pain-associated disability) rather than a primary depres- sion . Th is is in contrast to adults with fi bromyalgia where primary depression is frequently seen (Buskila et al., 1995 ). Menstrual cycling has been shown to aff ect fi bromyalgia-rel ated symptoms in nearly 50% of adult patients (Pamuk and Cakir, 2005 ), and this may be rel evant in the adolescent female population. b. Vasomotor: reports of temperature asymmetry and/or skin color changes and/or skin color asymmetry. c. Sudomotor/oedema: reports of oedema and/or sweating changes and/or sweating asymmetry d. Motor/trophic: reports of decreased range of motion and/ or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin). 3. Must display at least one sign at time of evaluation in two or more of the following categories: Complex regional pain syndrome Although it is almost 20 years since this condition fi rst r eceived attention in children, the diagnosis and treatment of CRPS contin- ues to be poorly understood. Many children receive investigations and rehabilitation for other presumed con ditions, resulting in a long journey before CRPS is recognized, and oft en a worsening of t he symptoms and associated disability (Berde and Lebel, 2005 ; Connelly and Schanberg, 2006 ). Th e diagnosis of CRPS remains a clinical one, and in adults is based on the Budapest C riteria ( Box 24.1 ) (Harden et al., 2010 ). Although there is frequently a precipitating t rauma, this is not present in around 10% of cases, and pain is usually out of proportion to the inciting event and accompanied by allodynia (Stanton Hicks, 2010 ). Children most oft en describe the pain as burning, with sensations akin to dysaesthesia. CRPS in pediatric pa tients is clinically distinct from the adult condition (Connelly and Schanberg, 2006 ; Stan ton Hicks, 2010 ): a. Sensory: evidence of hyperalgesia (to pinprick) and/or allo- dynia (to light touch and/o r deep somatic pressure and/or joint movement). b. Vasomotor: evidence of temperature asymmetry and/or skin color changes and/or asymmetry. c. Sudomotor/oedema: evidence of oedema and/or sweating changes and/or sweating asymmetry. d. Motor/trophic: evidence of decreased range of motion and/ or motor dysfunction (weakness , tremor, dystonia) and/or trophic changes (hair, nail, skin).