Supporting Students with Rheumatologic Disorders CHKD’s 15th Annual Chronic Illness Conference November 13, 2010 Anna Carmela Sagcal-Gironella, MD, MS Pediatric Rheumatology – Children’s Specialty Group Children’s Hospital of the King’s Daughters Objectives • Recognize in children the classic presentation of: – Juvenile Idiopathic Arthritis (JIA) – Juvenile Primary Fibromyalgia Syndrome (JPFS) – Systemic Lupus Erythematosus (SLE) • Discuss the approach to the treatment and management of children with these rheumatologic diseases. • Identify the primary care and school-related issues in children with these rheumatologic diseases. Juvenile Idiopathic Arthritis • 7 year old girl • Difficulty running and keeping up with friends lately • Appears well except for a minor limp when walking • Warm and swollen left knee • Stiff left knee in the morning, after a nap, or after prolonged sitting Juvenile Idiopathic Arthritis Limping Refusal to play or use Weakness extremity Joint Immobile Discomfort extremity Irritable Psychological regression Juvenile Idiopathic Arthritis • Most common rheumatologic disease in children • US incidence: 4-14 cases per 100,000 population/ yr. • Race: Caucasians > African lineage. Less common among Japanese, Filipinos, and Samoans • Gender: girls > boys • Age: < 16yo Diagnosis of JIA Persistent arthritis in at least JIA one joint Onset < 16 years old > 6 weeks duration Other diseases are excluded < 4 inflamed joints Any number of inflamed joints > 5 inflamed joints Fever (> 39oC) daily/ twice daily Large joints Large + small joints Rash (knee, ankle elbow, wrist) (fingers, toes) Large liver, spleen, lymph nodes Serositis Oligoarticular JIA Polyarticular JIA Systemic-onset JIA International League of Associations for Rheumatology (ILAR) Quotidian Fever of Systemic-Onset JIA Intermittent Fever • Nocturnal • Duration of at least 2 weeks • Recurrent spikes for at least 3 days Complications of JIA • Generalized growth disturbance (6-20%) – Linear growth – Weight – Sexual maturation • Localized growth disturbance – Limb length discrepancy • Decreased bone density – Higher risk for osteoporosis Complications of JIA • Macrophage Activation Syndrome • Active disease for > 10 years after onset (24-50%) • Psychosocial disruption – Adjustment and coping issues – Self-esteem – Dependence/independence – Adherence Psychosocial Burden of JIA • Diagnosis of JIA is a life-changing event for a family • Initial diagnosis a time of emotional turmoil for family • Families grieve the loss of the “healthy child” • Stages of grieving often encountered • Most families eventually adapt to diagnosis • Siblings often feel resentful, neglected, and angry • May also feel worried, uninformed, fearful, and guilty • May feel embarrassed if arthritis is visible • May impact the amount of time available for family and peer activities Psychosocial Burden of JIA • Children with JIA may feel isolated from peers • Loss of self-esteem • Negative body image • Primary concern: “not to be different” School and JIA Bowyer • unable to attend full school regularly 6% oligoarthritis, 28% polyarthritis, 44% systemic Ruperto • 20 % missed school Minden • comparable or higher than age matched controls (20-35 y/o) Oen • secondary education ↓ in women, compared to general population Treatment Goals of JIA • Control pain • Suppress inflammation • Restore/preserve function • Prevent/restore range of motion • Promote normal growth and development • Promote self-management Approach to JIA Treatment Experi- mental Sulfasalazine Methotrexate Plaquenil, auranofin Prednisone (intra-articular, oral) Non-Steroidal Anti-inflammatory Drugs Education Approach to JIA Treatment • Paradigm shift in 1990s Manage JIA more aggressively by giving treatment early in the disease course with the goal of preventing joint damage and slowing the progress of joint damage. Approach to JIA Treatment Education Non-Steroidal Anti-inflammatory Drugs Methotrexate Biologic: Enbrel, Humira, Anakinra, etc. (Prednisone rarely) Pharmaco- genomics Chronic Inflammation Anti- Pro- inflammatory inflammatory Anti-TNF Etanercept (Enbrel) Adalimumab (Humira) Infliximab (Remicade) Anti-IL-1 Anakinra (Kineret) Rilonacept (Arcalyst) B-cell depletion therapy Rituxan (Rituximab) Imbalance between mediators of inflammation Interdisciplinary Approach Psychosocial care – Self-management – Education – Family care Nutrition – Bone mineralization (calcium supplements) – Monitor growth parameters Interdisciplinary Approach Ophthalmologic management Physical management (Physical/Occupational Therapy) – Exercise – Rest – Heat/Cold or Hydrotherapy – Ultrasound – Devices (splints, orthotics, etc.) Psychology support/services – Cognitive behavioral therapy – Counseling Juvenile Primary Fibromyalgia Syndrome (JPFS) • 15 y.o. female • Junior high school • Frequent absences (total of 4 weeks) • Complains of “aching all over” • Is tired “all the time” • Complains of headaches and abdominal pain • Not sleeping well, needing frequent naps • Symptoms are worse with cold weather Common Symptoms of JPFS Symptoms Frequency* Poor sleep 96% Chronic headaches 71% Fatigue 62% Pain associated with physical activity 42% Subjective tissue swelling 40% Irritable bowel syndrome 38% Pain modulation by weather 36% Numbness/ tingling of extremities 24% Chronic anxiety or tension 22% Pain associated with stress or anxiety - *Siegel DM, Janeway D, Baum J. Fibromyalgia syndrome in children and adolescents: clinical features at presentation and status at follow-up. Pediatrics. Mar 1998;101(3 Pt 1):377-82. Other Common Symptoms of JPFS Symptoms Frequency* Morning stiffness 53% Depression 43% Dysmenorrhea 36% Abdominal pain** 17% Joint hypermobility** 14% Raynaud’s phenomenon 13% *Siegel DM, Janeway D, Baum J. Fibromyalgia syndrome in children and adolescents: clinical features at presentation and status at follow-up. Pediatrics. Mar 1998;101(3 Pt 1):377-82. **Gedalia A, Garcia CO, Molina JF; Bradford NJ; Espinoza LR. Fibromyalgia syndrome: experience in a pediatric rheumatology clinic. Clin Exp Rheumatol. May-Jun 2000;18(3):415-9. JPFS Frequency distribution of common tender points Tender Points Frequency Upper back (trapezius) 82% Medial side of the knee 76% Lateral elbow (lat. epicondyle) 67% Front neck muscles (SCM ) 61% Upper nape (occiput) 58% Medial elbow 55% Upper buttock (gluteus) 45% Normal PE no arthritis, weakness, or inflammation JPFS Distribution • 2/100 of US population • Gender: girls: boys 3:1 to 7:1 – adults: F:M 7:1A • Race: Caucasians > African Americans • Age: – Mean age of onset: 12 years old – Peak age of diagnosis: 13-15 y.o.B,C – Earliest age diagnosed is 5 y.o. • 25-40% of musculoskeletal pain syndromes AYunus MB. Semin Arthritis Rheum. Jun 2008;37(6):339-52. BYunus MB, Masi AT. Arthritis Rheum. Feb 1985;28(2):138-45. CSiegel DM, Janeway D, Baum J. Pediatrics. Mar 1998;101(3 Pt 1):377-82. Cycle Maintaining JPFS Anthony KK, Schanberg LE. Juvenile primary fibromyalgia syndrome. Curr Rheumatol Rep. 2001 Apr;3(2):165-71. Psychosocial Impact of JPFS Compared to normal patients and/ or JIA patients – Low health-related quality of life (due to pain)1 – Increased temperamental instability and higher levels of depression and anxiety 2 – Use of maladaptive coping strategies (emotion focused avoidance)3 – Emotion focused avoidance (strongly related to depression) 3 – Children and parents self-reported higher levels of anxiety/depression and poorer psychological adjustment 2 – Less family cohesion2 JPFS and School • Decreased concentration due to pain – Decreased learning ability – Branded as lazy, slacker, unmotivated, or emotionally disturbed – Lower school popularity and leadership qualities – School phobia – Frequent school absences • Fatigue – Difficulty with catching up with missed assignments JPFS and School Outcome - ↑ anxiety Academic Social problems: Failure - ↑ social withdrawal - Isolation JPFS > peers - ↑ emotional distress - Peer rejection -↑ sensitivity - Depression Kashikar-Zuck S, et al. “Social functioning and peer relationships of adolescents with juvenile fibromyalgia syndrome”. Arthritis & Rheumatism 2007;57:474-480. Treatment Goals for JPFS • Reduce pain • Reduce depression • Decrease sleep disturbances • Promote physical activity (mainstay of treatment) Treatment Approach for JPFS • Cognitive behavioral therapy (CBT) – Goals: • Decrease pain and disability • Improve ability to cope with pain and stress – Skills education: • progressive muscle relaxation • activity pacing • distraction techniques • reframing of negative thoughts • problem solving Golenberg DL, Burckhardt C, Crofford L. Management of Fibromyalgia Syndrome. Journal of the American Medical Association, 2004; 292 (19): 2388-2395. Burkhardt CS, Goldenberg D, et al. Guideline For The Management Of Fibromyalgia Syndrome Pain in Adults and Children, APS Clinical Practice Guidelines Series, 2005, No. 4. Treatment Approach for JPFS • Cognitive behavioral therapy (CBT) – Studies showed CBT reduced anxiety, depression, mood instability, pain sensitivity and improved family support1,2 – CBT, exercise and activity has better outcome compared to medication alone3 1Conte PM, Walco GA, Kimura Y. Arthritis Rheum. Oct 2003;48(10):2923-30. 2Walco GA, Ilowite NT. J Rheumatol. Oct 1992;19(10):1617-9. 3Rossy LA, et al. Ann Behav Med. Spring 1999;21(2):180-91. Treatment Approach for JPFS • Counseling and Support – Educate1 and reassure the patient and the family – Encourage school attendance – Encourage normal activity participation – Modify attendance or activity 1G Degotardi PJ, et al. J Pediatr Psychol. Aug 2006;31(7):714-23. Treatment Approach for JPFS • Pharmacologic (No pediatric trials. Adopted from adult studies.) – Nonsteroidal Anti-Inflammatory Drugs (NSAIDs ) • ibuprofen (Advil, Motrin) • naproxen (Naprosyn) – Antidepressants • amitriptyline (Elavil), fluoxetine, duloxetine • decrease pain, improve function and sleep – Skeletal muscle relaxants • cyclobenzaprine (Flexerie) • relieve painful muscle spasms – Corticosteroids and strong opioids are not recommended! Carville SF, et al. EULAR evidence-based recommendations for the management of fibromyalgia syndrome. Ann Rheum Dis. 2008 Apr;67(4):536-41. Treatment Approach to JPFS • Improve sleep hygiene – Avoid napping – Avoid caffeine (coffee, tea, soda, energy drinks) – Establish bedtime routine – Avoid aerobic exercise before bedtime – Sleep analysis to identify treatable sleep disorders1 – Low-dose antidepressant2 1Tayag-Kier CE, Keenan GF, Scalzi LV. Pediatrics. Nov 2000;106(5):E70. 2Gedalia A, et al. Clin Exp Rheumatol. May-Jun 2000;18(3):415-9. Burkhardt CS, et al. Guideline For The Management Of Fibromyalgia Syndrome Pain in Adults and Children, APS Clinical Practice Guidelines Series, 2005, No. 4. Treatment Approach to JPFS • Prescribed graded exercise (from adult studies) – Heated pool treatment with or without exercise is effective for fibromyalgia – Tailored aerobic exercise program* – Customized strength training program* *Can be beneficial to some patients with fibromyalgia Carville SF, et al. EULAR evidence-based recommendations for the management of fibromyalgia syndrome. Ann Rheum Dis. 2008 Apr;67(4):536-41. Prognosis for JPFS Treatment outcomes for 50 JPFS patients after 18 months of follow-up Gedalia A, et al. Clin Exp Rheumatol. May-Jun 2000;18(3):415-9. Childhood-Onset Systemic Lupus Erythematosus (cSLE) • 16 y.o. African American female • Fever (101-102oF) for 3 weeks • Always feels tired • Gradual decline in school performance • Recurrent headaches • Oral ulcers • Rash on cheeks and nasal bridge • Body pains for 4 weeks • Swollen feet Nature Medicine 9, 1337 (2003) http://www.nature.com/nm/journal/v9/n11/images/nm1103-1337a-I1.jpg cSLE • Lifelong • Episodic • Multisystem • Autoimmune disease • Widespread inflammation of blood vessels and connective tissues • Presence of antinuclear antibody (particularly dsDNA) • Variable manifestations • Unpredictable natural history cSLE Distribution • Prevalence: 12-50/ 100,000 individual1 • Common age of onset: 7-16 years old2 • More common among Native Americans, Asian Americans, Latin Americans, and African Americans compared to Caucasians • Girls:boys 4:1 (pre-puberty); 5:1 (after puberty)1 1Textbook of Pediatric Rheumatology 5th Ed. Cassidy, Petty, Laxer and Lindsley. Elsevier Saunders, 2005. 2Caeiro F, et al. Ann Rheum Dis. 1981 Aug;40(4):325-31. Common cSLE Symptoms Symptoms at any time Frequency (%) Malar (butterfly) rash 72 Arthritis 66 Fatigue 60 Renal disease 59 Other rashes 48 Fever (>100oF) 46 Weight loss 36 Nasal/ oral ulcers 36 Alopecia 31 Textbook of Pediatric Rheumatology 5th Ed. Cassidy, Petty, Laxer and Lindsley. Elsevier Saunders, 2005. Less Common cSLE Symptoms Symptoms at any time Frequency (%) Anorexia 22 Pleuritis/ carditis 24 CNS disease 21 Photosensitivity (UV radiation) 19 Raynaud’s phenomenon (purple/pale/red 18 digits from cold or stress Lymphadenopathy 15 Digital ulcers 6 Myocarditis (chest pain) 3 Myositis (muscle pain) 3 Thrombocytopenic purpura 2 Textbook of Pediatric Rheumatology 5th Ed. Cassidy, Petty, Laxer and Lindsley. Elsevier Saunders, 2005. Common CNS Symptoms in cSLE Symptoms Frequency (%) Recurrent headache 71 Cognitive disorder 55 Isolated seizure 47 Migraine 36 Acute confusional state 35 Major depressive episode 32 Depressive features 23 Anxiety disorder 21 Textbook of Pediatric Rheumatology 5th Ed. Cassidy, Petty, Laxer and Lindsley. Elsevier Saunders, 2005. ACR 1997 Criteria for SLE Classification 1. Malar (butterfly) rash 2. Discoid-lupus rash (+) Antibody to dsDNA or 3. Photosensitivity (+) Antibody to Smith 4. Oral ulcers (or nasal ulcers) nuclear antigen or 5. Non-erosive arthritis Positive finding of antiphospholipid 6. Serositis (pleuritis or carditis) antibodies based on: 7. Renal disorder (proteinura >0.5g/day 1. IgG or IgM anticardiolipin antibodies, or cellular casts) or 2. Lupus anticoagulant, or 8. Encephalopathy 3. False positive serologic 9. Cytopenia test for syphilis for at least 6 months 10. Immunologic disorder 11. Positive antinuclear antibody (ANA) Textbook of Pediatric Rheumatology 5th Ed. Cassidy, Petty, Laxer and Lindsley. Elsevier Saunders, 2005. Lupus Nephritis • 60-80% of cSLE patients develop renal disease • Occurs within 2 years of diagnosis of SLE • Risk of occurrence of lupus nephritis decreases 5 years after diagnosis of cSLE • Hypertension, dark foamy urine, edema around eyes, legs, ankles or fingers Mycophenolate mofetil Dialysis, Corticosteroid Cyclophosphamide Transplant Perfumo F, Martini A, Lupus. 2005;14(1):83-8 Central Nervous System Disease • #2 cause of morbidity and mortality in SLE • 20-95% of cSLE patients develop CNS disease – 50% is present at onset of cSLE – 40% will be present during the 1st year of cSLE – Some develop CNS disease years after cSLE diagnosis • Headache, dizziness, seizure, psychosis, cognitive dysfunction (confusion, poor memory, difficulty expressing thoughts) Duzova A, Bakkaloglu A. Curr Pharm Des. 2008;14(13):1295-301. Textbook of Pediatric Rheumatology 5th Ed. Cassidy, Petty, Laxer and Lindsley. Elsevier Saunders, 2005. Brunner, HI, University of Toronto, 2001 Psychosocial Impact of cSLE • Depression • Anxiety disorders • Feeling of uncertainty about illness, pain & fatigue • Poor physical functioning associated with: – helplessness, disease activity and disease damage (Hispanics) – fatigue and pain (African-Americans and Caucasian) • Self-esteem status is related to mental health Seawell AH, Danoff-Burg S. Psychosocial research on systemic lupus erythematosus: a literature review. Lupus. 2004;13(12):891-9. Psychosocial Impact of cSLE • Diagnosis of SLE is a life-changing event for a family • Initial diagnosis a time of emotional turmoil for family • Stages of grieving often encountered • Siblings often feel resentful, neglected, and angry – May also feel worried, uninformed, fearful and guilty • May feel embarrassed if skin changes prominent & visible → isolation and withdrawal Psychosocial Impact of cSLE • Children with SLE may feel isolated from peers • Loss of self-esteem • Negative body image • Primary concern: “not to be different” Goal of Treatment of cSLE • Control disease progression – Minimize disease exacerbation – Prevent serious organ damage • Prevent medication side effects – Growth – Puberty – Development – Fertility • Treatment compliance and frequent re-evaluation Pharmacologic Treatment for cSLE • Hydroxychloroquinolone (Plaquenil) • Immunosuppressants – Cyclophosphamide (Cytoxan) - for severe renal/ CNS dse. – Mycophenolate mofetil (CellCept) - off label use for lupus nephritis – Azathioprine (Imuran) – Cyclosporine (Sandimmune) • Corticosteroids Pharmacologic Treatment for cSLE • Nonsteroidal anti-inflammatory drugs – Muscle and joint pains – Low-dose aspirin for anti-phospholipid syndrome – Naproxen, tolmetin, diclofenac – Some studies link Ibuprofen1-4 or naproxen4 to higher risk for aseptic meningitis • Calcium and vitamin D supplements 1RodríguezSC, et al. Medicine (Baltimore). 2006 Jul; 85(4): 214-20. 2Østensen M, Villiger PM. Lupus. 2001;10(3):135-9. 3Mandell BF, Raps EC. Am J Med. 1987 Apr;82(4):817-20. 4Weksler BB, Lehany AM. DICP. 1991 Nov;25(11):1183-4. Alternative Therapy for cSLE • Monoclonal antibodies (MAbs) – Synthetic proteins that targets B cells • Intravenous immunoglobulins – Blood product. Antibodies from B cells • Dehydroepiandrosterone (DHEA) – Synthetic equivalent of steroid produced by adrenal gland • Autologous stem cell transplant • Plasmapheresis – Extraction of and replacement with new plasma volume School Adjustments Mobility Possible Solutions • May have difficulty with stairs or steps • Arrange for help getting on the bus • Arrange to leave class a few minutes early or to arrive five minutes late; if necessary as many classes as possible on one floor • Keep classes close together to avoid walking long distances • Arrange for shortest time possible to ride on the bus. This gives more time for bathing, dressing, breakfast, exercises, etc. in the morning • Make special arrangements for gym class or P.E. teacher to adapt activities (swimming, scorekeeper, coach, timer, etc.) • Make plans ahead of time if necessary during field trips, fire drills, or special class activities. School Adjustments Stiffness Possible Solutions • ↑ with periods of inactivity, especially in AM’s or after sitting • Arrange with the teacher to allow student move about the class frequently (handing out or collecting papers, erasing chalkboards, etc.) • Arrange to have student sit in the back, or to one side of the class so they can stand without being noticed • Arrange for late arrival to school • If knee or hip problems, then discourage sitting cross- legged or “Indian-style” on the floor School Adjustments Fatigue Possible Solutions • Most pronounced in systemic & polyarticular & fibromyalgia •↑ fatigue and naps especially with flare and ↑ pain • May need altered schedule i.e., late arrival/early dismissal; rest during day; pacing of activities especially in fibromyalgia • May need altered length of assignments • May need pain med if fatigue related to pain FIBROMYALGIA: FOCUS ON FUNCTION NOT PAIN AND FATIGUE School Adjustments Activities of Daily Possible Solutions Living • 2 sets of books • Computer for writing assignments—needs wrist rest • Back pack for carrying items (if no shoulder involvement • Elevator pass/key, especially if flared or trouble w/ stairs • Pencil build-ups • Extra time to complete tests; obtain notes from teacher or classmate • Plan ahead for standardized tests (i.e., SAT, ACT, proficiency) for extra time; needs to be months before test • Permission for shorter homework assignments or computer when appropriate School Adjustments Physical Education / Possible Solutions Competitive Sports & Safety Issues JIA: • Allow to perform PT exercises in gym; altered PE program • Self-limit activities • Substitute as timer, scorekeeper, linesman, etc. if unable to do activity • Avoid activities putting stress on involved joints • Safety, as related to driving and C-spine involvement; special considerations (i.e., mirrors, stick shift vs. automatic); specific joints: neck, hands School Adjustments Physical Education / Possible Solutions Competitive Sports & Safety Issues SLE: • Noncompetitive/contact sports with spleen enlargement or low platelet count • Assess risk of bleeding • Safety w/ activities related to cognition, ↓ platelets operating automobile/vehicle issues/sports activities JPFS: • Need graduated aerobic exercise (often very deconditioned) School Adjustments Photosensitivity Possible Solutions • Indoor activity is advised • For outdoor activity, remind the use of high SPF sunscreen long sleeved clothes and broad-rimmed hat • Apply sunscreen at least 30 minutes before going out • Reapply sunscreen every 3 hours • Provide UV filter screen for computer monitors School Adjustments Puberty & Risk Possible Solutions Screenings • Possible delayed menarche, amenorrhea or anovulatory cycles with flares - mostly in JIA and SLE • Counseling regarding expectations/concerns related to puberty Screening for: • sexual activity/ alcohol (especially if taking methotrexate or biologic drugs) • smoking increases risk for clots; especially if + APA in SLE weight gain, acne, and BP secondary to corticosteroids • gingival hyperplasia if on cyclosporine increase brushing to 4x/day, if possible • May need support for body image issues • Be cognizant of possible mood changes if on corticosteroids School Adjustments Diet/ Meds Possible Solutions • Students on corticosteroids should limit salt intake • Monitor calorie intake to avoid excessive weight gain and diabetes • Supplement with calcium and vitamin D (to prevent osteoporosis) • NSAIDs may cause abdominal pain, easy bruising, headaches take after meals • Patients on corticosteroids may have weight gain, mood swings, decreased bone density, occasional concentration problems in school School Adjustments Immunization Possible Solutions JIA/SLE: Typically no contraindication UNLESS on immunosuppressives, steroids, or biologics --- then: • No live virus vaccines • May need to postpone immunization if on high-dose steroids JIA/ SLE/ JPFS Annual flu vaccine recommended (especially if on steroids, immunosuppressives, or biologics) • No intranasal vaccines if on above meds School Adjustments Common Illness Possible Solutions • Interim illnesses may cause mild flare of symptoms • Assess fevers, GI upset, infections • Avoid (if possible) sulfa medications if on methotrexate • EBV (mono) special consideration if on methotrexate, cyclophosphamide, CellCept, or biologics • Increased risk of infections if on methotrexate, cyclophosphamide, CellCept biologics (will need to hold biologic if febrile or develops significant infection such as pneumonia) • Notify family if classmate with varicella School Adjustments Lovell DJ, et al. , “School Attendance and Patterns, Special Services and Special Needs in Pediatric Patients with Rheumatic Diseases: Results of a Multicenter Study,” Arthritis Care and Research, Vol 3, No 4. 1990. School Adjustments Attendance & Possible Solutions Transition • ↑ absence (due to symptoms, medical consult, hospitalization) •May need assignment/schedule adjustments • May need gradual re-entry program • If has IEP/504 waiver of waiting time to receive assignments during absences • May need tutor or home teacher if out for extended time • WATCH GRADES (serve as indicator of cognition/ depression; Symptoms can be subtle! • Plan transitions and anticipatory changes and needs between schools (i.e., elementary/ middle/high school) • College transition issues • BVR assessment and vocational issues Acknowledgment Janalee Taylor, RN, MSN, CNS Associate Clinical Director Division of Pediatric Rheumatology Cincinnati Children’s Hospital Medical Center Thank you!
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