Nutrition General Dietary Guidelines for Children with JRA – Well balanced diet that includes variety of foods – Meals should be planned using Food Pryamid – Serving Sizes appropriate for age.
Download ReportTranscript Nutrition General Dietary Guidelines for Children with JRA – Well balanced diet that includes variety of foods – Meals should be planned using Food Pryamid – Serving Sizes appropriate for age.
Nutrition General Dietary Guidelines for Children with JRA – Well balanced diet that includes variety of foods – Meals should be planned using Food Pryamid – Serving Sizes appropriate for age Nutritional Risk Growth retardation and impaired bone demineralization occur during periods of active disease, & are exacerbated by steroid administration, anorexia, or decreased physical activity. Anorexia very common Dietary inadequacies Limitations in physical activity Mechanical feeding difficulties Susceptibility to food fads and quackery Common Growth Problems in JRA Poor linear growth Low lean body and muscle mass Undernutrition and wasting Excessive weight gain & obesity Leg length discrepancy Mechanical Feeding Problems Arthritis of TMJ – Pain while chewing – Limitation of opening of mouth Mechanical Feeding Problems Arthritis in wrists, elbows, fingers: difficulty using utensils – Embarrassment – Try to hide this – Refusal to eat Solutions: – Cut up food – Use easy to grasp cups – OT Unproven Remedies 70% of JRA patients have tried 1 - 8 unconventional remedies 43% tried dietary changes No evidence that JRA is caused by food allergies No evidence that JRA is cured by anti-allergy diets Approach to School Attendance Absences: due to – Morning stiffness and pain – Flare of disease activity – Hospitalizations – Medical appointments (MD, OT/PT) Frequent absences Any prolonged absence or frequent 1 or 2 day absences may indicate possible secondary gain – Using illness for increased attention, increased control or avoidance of difficult situations at school – Or developing a school phobia Our Approach to School Attendance Identify the school issues – School checklist – Interview child and parent – Talk with school nurse if needed Send letter listing special needs Instruct parents to meet with school principal, counselor, & school nurse 504/IEP staffing if needed Special School Concerns: Homebound schooling Intermittent Schooling: safety net Full homebound – reserved for the unusual situations – a temporary measure – evaluated regularly by the physician – for situations of prolonged evaluation & diagnosis, or flare which kept child out of school for extended period of time causing child to be behind in school work. – Should be a last resort Special School Concerns: Mobility problems Difficulty getting to school or waiting for bus. – Special transportation – Arrange to be last on bus route or change bus stop – Schedule least important classes in AM Mobility problems Difficulties physically getting from class to class or climbing stairs – Allow extra time to get from class to class – Elevator pass – Schedule classes close together or progressively to minimize walking distances – Plan for extra assistance during fire or disaster drills or when going on field trips. – Wheelchair for special circumstances Stiffness -Sitting in one position too long – Allow to move frequently – Wiggle – Ask teacher to give errands (hand out papers) Special School Concerns: Problems with manual dexterity Messy, slow handwriting Painful, stiff fingers, hands – Rubber build-ups on pens / pencils to facilitate a better grip – Felt tip pens – Extra time for completion of assignments & tests – Modification of assignments requiring less writing – Use of tape recorders, computers – Aide to fill in bubbles Special School Concerns: Problems with manual dexterity Difficulty opening locker Difficulty reaching upper lockers – Alternative location for storage of books – Key lock (easier to open than combination) – Books kept in each classroom Difficulty eating or toileting – Allow extra time – Assistant Special School Concerns: Fatigue Tires more easily “it feels like I have the flu all the time” Trouble keeping up – Schedule rest period into middle of day – Allow extra time for completion of assignments – Assign a tutor Special School Concerns: Pain or limited movement in arms/shoulders/hands Extra set of books Rolling back pack Buddy system Alternative signaling plan Keep books in each classroom Special School Concerns: Physical Education Letter from MD to specify PE restrictions Adaptive PE “Dressing out” - may have difficulty changing, need extra time and assistance – Loose fitting clothes – Velcro closures Schedule in afternoon when not as stiff Special School Concerns: OT/PT May need OT/PT evaluation – in AM when joints are worse May need special desk or chair Can evaluate for adaptive equipment Special School Concerns: Psychosocial development Many children don’t want peers to know about their disease Many make up excuses to explain why they can’t run or walk as fast as peers Teasing – Classroom discussion – Counseling Arthritis Foundation Focus: RA, Osteoarthritis and JA Supports research, public policy Juvenile Arthritis Alliance: annual conference Education and resources Blogs/groups (kids, tweens, teens, parents) Advocacy Summit (April 16-18, 2012 in Washington, DC Advocacy in Tallahassee Arthritis Foundation Camp Boggy Creek/Camp Funrise Regional Family Fun Days in Florida Jingle Bell Runs/Creaky Bones Walk/Runs Joints in Motion Systemic Lupus Erythematosus A systemic multi-system autoimmune disease characterized by the presence of autoantibodies and multiorgan system involvement. + ANA in >90% of patients with SLE +ANA is 1:160 or higher Etiology: combination of genetic susceptibility and environmental factors, including exposure to sunlight, infections, drugs, and chemicals. SLE in kids facts 15-20% of lupus cases begin before age 19 Incidence & prevalence rates vary by ethnicity and are higher in Hispanic, Black, North American First Nations, and SouthEast and South Asian populations Exposure to UV-B rays may exacerbate either skin or systemic disease. Female to male 2:1, 5:1 Mean age at diagnosis 12-13 years 1982 revised criteria for classification of SLE Malar rash SLE criteria Discoid rash: Erythematous raised patches with adherent keratotic scales and follicular plugging. Atrophic scarring. SLE criteria Photosensitivity: unusual reaction to sunlight Oral ulcers Arthritis SLE criteria Serositis (Pleuritis, documented by ECG or rub, or evidence of pericardial effusion Renal disorder (proteinuria or casts) Neurological disorder (seizures, psychosis, known metabolic derangement – uremia, ketoacidosis, electolyte imbalance Hematological disorder (hemolytic anemia, lymphopenia SLE criteria Immunological disorder (+DSDNA or +anti-SM nuclear antigen, or + antiPhospholipid antibody, or + Lupus anticoagulant ANA Management of SLE Calcium + Vitamin D Sunscreen NSAIDS Plaquenil Oral prednisone – sometimes IV Methotrexate – steroid sparing (folic acid) Azathioprine or Cellcept Cytoxan Cyclosporin Juvenile Dermatomyositis A multisystem disease resulting in chronic inflammation of striated muscle and skin. -- Juvenile Dematomyositis Criteria for diagnosis – Symmetrical proximal muscle weakness – Characteristic rash Heliotrope rash of eyelids & periorbital edema Erythematous papules over extensor surfaces of joints including MCPs and PIPs, elbows, knees, ankles: Gottron papules) – Increased CK, AST, ALT, LDH, Aldolase – EMG – Muscle biopsy – MRI of thigh muscles (preferred to EMG and muscle biopsy) Gottron’s papules Scaly erythematous rash, violaceous discoloration Incidence: 3.2 new cases per 1 million children Rates of blacks and whites = Hispanic rates slightly less Median age of onset: 7 years 25% are < 4 years of age Female to male ratio 2.1:1 Symptoms: present in 79 children Rash 100% Weakness 100/% Muscle pain 73% Fever 65% Trouble Swallowing 44% Hoarseness 43% Stomach pain 37% Arthritis 35% Calcifications 23% Blood in Stool 13% Levels of weakness at Diagnosis Mild: Can’t run as fast as friends in play and sports activities, Tires more easily Moderate: Difficulty combing hair, Loss of ability to dress self, Trouble getting in and out of car, C/O aches in muscles after walking Severe: Wants to be carried everywhere, Trouble swallowing, Trouble walking. Needs help going from lying down to sitting. Abnormally prominent nailfold capillaries and hemorrhage present in a patient with dermatomyositis. Changes of nailfold capillary patterns, characterized by loss of (drop-out) nailfold capillary loops that surround the remaining, enlarged dilated capillaries. Juvenile Dermatomyositis Treatment Corticosteroids IV and/or PO Methotrexate Plaquenil Azathioprine IVIG 2 gm/kg/mo Cyclosporin) Cyclophosphamide Mycophenolate mofetil Prograf (tacrolimus) Rituximab Anti-TNF (Enbrel or Remicade Dermatomyositis mortality 30% pre-steroid era (Bitnum et al) 10% post-steroids (Spencer et al) 2011: 2 out of 346 (<1%) UK cohort Prognosis Normal to good funtional outcome Minimal atrophy or contracture Calcinosis 40% Wheelchair dependence Death 65-80% 25-30% 205% 1-2% Juvenile Localized Scleroderma All types of Scleroderma are characterized by an excessive accumulation of collagen. Dermatologists call this morphea. Peds rheumatologists call it localized scleroderma. Limited to skin and subdermal tissues. Rarely internal organs. – Circumscribed morphea (oval or round areas of induration Linear scleroderma (lines of induration) can affect trunks and limbs or head: involves dermis, subQ tissue and sometimes muscle and underlying bone. Can affect the limbs and/or trunk. – Generalized: induration starting as individual plaques – 4 or more, and larger than 3 cm. And involve at least 2 sites Pansclerotic morphea – Circumferential involvement of limbs affecting the skin, subq tissue, muscle and bone. The lesion may involve other areas of the body without internal organs involved. Treatment/Prognosis Very little evidence on which to base treatment recommendations: – Solumedrol + SubQ methotrexate: most show response within 4 months – If no response: Cellcept – In study of the linear scleroderma: 50% showed skin softening of 50% or more OR had disease resolution by 3.8 years after dx.