Test Review Exam 3- New for Spring 2022 Eating disorders 2 Anorexia Nervosa: o General: Eating disorder characterized by fear of gaining weight or becoming overweight Typical age of onset is late adolescence Results from a combo of physiological and psychological factors Societal pressures and ideals Genetic components o Risk Factors: Adolescence Perfectionist personality Family htx of eating disorder Low self-esteem Female gender anxiety depression suicidal thoughts low body mass index dehydration lanugo (fine, soft, unpigmented hair) cold intolerance hypotension crying compulsive exercising Anorexia o s/s: Severely underweight Cachectic appearance Dry sallow skin Thinning hair Sparse body hair Nail pitting VS: ↓ o Decreased temp, bradycardia, hypotension Murmur o 1/3 will have mitral valve prolapse o Also known as mitral valve regurgitation o Valves do no not close properly between each pump causing backward flow through the valve back to left atrium Anorexia Nervosa Tx: o Nutritional support o Clinical therapy (inpatient or outpatient) o Treat fluid and electrolyte imbalances and monitor CV involvement o Treat with multidisciplinary team (pediatricians, behavioral health specialists, registered dietician nutritionists) o Involve family in counselling o Focus on gradual weight gain of 2-3 lb per week o May need NG feedings Anorexia Nervosa Management: o Outpatient or inpatient tx (requires monthd of tx) o Nutritional consultation o Hospitalize if: FOOD REFUSAL Severe wt loss Unstable VS Require parenteral nutrition Arrested puberty (Real. Back when I was a teen I lost my period for months whenever I was starving myself to lose weight) o Frequent monitoring labs, VS (including ortho hypotension, irregular and decreased HR, hypothermia) o Food journal noting intake, binging, purging episodes o Journal: moods, behaviors, exercise o Therapy include eval for anxiety, depression o Support group Bulimia Nervosa o General: Eating disorder characterized by periods of binge eating followed by periods of purging Purging = self-induced vomiting, diuretics, and/or laxatives o Risk factors: Poor self-image Societal pressures normal weight or only slightly underweight food rituals o s/s: compulsive ways in which a person interacts with food that produces anxiety when not followed ex: taking abnormally small bites, tearing food apart labile mood repeated fad dieting diets for “fast weight loss” thinning hair brittle nails hypokalemia (vomiting, diuretics, lax) anemia Russell sign: calluses on backs of hands and fingers bradycardia esophageal irritation poor dentition o Treatment: Cognitive behavioral therapy Teaches children to change reactions so that automatic negative thought patterns are replaced w. alternative ones SSRIs Metabolic disorders- especially PKU- 2 Phenylketonuria (PKU) (info on slide) o Rare, inherited, metabolic disorder o Inborn error of metabolism detected thru newborn screening o Follows and autosomal recessive inheritance pattern o Cause: Deficiency of a liver enzyme Body is unable to process phenylalanine (amino acid-think protein) o Result: Irreversible brain damage from high levels of phenylalanine o S/S: Irritability Vomiting of protein feedings Musty body smell Urine odor Increased reflex action Seizures Skin rashes/eczema Microcephaly Developmental delays Behavior abnormality o Tx: Meal plans low in phenylalanine (low protein) Avoid protein foods: meat, milk, eggs, beans, nuts, aspartame (in diet soda) Level testing Genetic disorders and genes 3 General info: o Caused by abnorm in individual’s genetic material o Complex: Categorized into: monogenic (Mendelian) o can be autosomal dominant, autosomal recessive, x-linked dominant, or x-linked recessive multifactorial o caused by multiple gene and environmental factors nontraditional inheritance o do not follow typical patterns chromosomal disorders o do not follow straightforward pattern of inheritance (error in sperm or egg) o can be structural or numeric abnormalities o increased risk of health complications (especially cardiac) o hearing and vision problems common Inheritance: o x-linked (general) altered gene on X chromo male inherits altered x-linked gene they develop condition; female heterozygous or homozygous expression similar to autosomal disorders o x-linked recessive affects more males than females (males only have 1 x) no male to male transmission (hemophilia and Duchene MD) girls need 2 abnorm genes to have, 1 gene daughter, carrier o autosomal recessive: 2 copies of abnormal gene in homozygous state required to produce phenotype Fragile X syndrome: o Inherited gene condition and most common cause of intellectual disability o Boys experience more severe s/s (they only have 1 X chromo) o X-linked dominant inheritance pattern o s/s: minor dysmorphic features (deformity) developmental delay large head long face prominent ears flat feet enlarged testes flexible joints sensory processing troubles behavioral problems o Dx: genetic testing o Tx: Developmental therapies and interventions No cure but normal life span Trisomy 13 o AKA Patau Syndrome o Non-inherited chromosomal condition o 3 copies of chromosome 13 present in each cell o s/s: cleft lip and cleft palate extra digits clenched hands close set eyes hernias low set ears scalp defects single palmar crease limb abnormalities microcephaly undescended testes o Complications: Apnea Deafness Heart failure Seizures Intellectual disability Vision problems o Prenatal testing can diagnose condition o Management most infants do not survive past 1st month of life symptomatic care for infant and supportive care for family Trisomy 18 o AKA Edwards syndrome (Edward from twilight is forever 18-(not really he turned at 17 but whatevs lol)) o Chromosomal condition typically not inherited Partial trisomy 18 can be inherited, but the person is typically asymptomatic o 3 copies of chromosome 18 in each cell o S/S: Intrauterine growth restriction Low birth wt Small head Small jaw Low set ears Short eyelid fissures Clenched and overlapping digits Hypotonia Short sternum Narrow hips with limited abduction o Management most infants do not survive past 1st month of life symptomatic care for infant and supportive care for family Trisomy 21 o AKA Down syndrome o Chromosomal condition resulting from random events that occur during formation of reproductive cells o 3 copies of chromosome 21 in each cell o Degree of intellectual disability and degree of developmental delay in severity o S/S: Flattened bridge of the nose Almond shaped eyes Cardiac defects Hearing and vision impairments Increased mobility of cervical spine o Complications: Heart defects GI abnormalities Soft-tissue and skeletal changes Hypothyroidism Atlantoaxial instability of first and second cervical vertebrae low muscle tone autoimmune disorders infectious dz Prenatal tests: o Dx: Ultrasound Amniocentesis Chorionic villus sampling Percutaneous umbilical blood sampling Children: Echocardiogram Abdominal ultrasound Lab testing Sleep apnea test Cervical x-ray Thyroid hormone levels o Tx: Assess for developmental delay using appropriate milestones based on children with trisomy 21 Refer to therapies, early intervention, and services (speech, occupational, PT) Turner Syndrome o Affects only females o Chromosomal condition caused by complete or partial absence of second sex chromosome o s/s: difficulty with nonverbal communication skills and executive function wide or weblike neck broad chest with widely spaced nipples slowed growth cardiac defects short fingers and toes o complications: chronic middle ear infections hearing loss autoimmune disorders heart, liver, and kidney abnormalities o Tx: Treat with growth hormone and estrogen therapy Klinefelter Syndrome o Most common sex chromosomal abnormality o Affects only males o Karyotype and phenotype male XXY o 1 or more extra X chromosomes present o Presentation: Dx delay until adolescence or adulthood Lack of secondary sex characteristics (physical features) ↓ facial hair ↓ pubic hair Hypogonadism (gonads (sex glands) produce little to no hormones) Underdeveloped testes Infertility Gynecomastia (overdevelopment or enlargement of breast tissue in males) Taller than average Long legs Short torso Learning disabilities Motor delay Speech/lang difficulty ADD o Management: No cure Focus on enhancing masculine characteristics and minimizing female characteristics Testosterone replacement Cosmetic surg o Nursing management: Supportive Education Hemoglobin r/t pediatrics and iron Fetal Hgb (Hgb F) o Until 4-6 mos of life o Shorter cell life and higher quantities o Increase risk of anemia and O2 carrying capacity o RBC transfer from liver to bone marrow and balance between oxygenation and production affected Hgb A: o Predominant type of Hgb 6 months after birth Heme o The part of Hgb that has iron and carries O2 Iron o Fetus: Receives iron thru placenta o Preterm infant: Misses final wks/mo of transplacental iron transfer so risk increases for iron deficiency anemia o Term infant: Physiologic anemia occurs between 2-6 mo d/t rapid growth and maternal iron stores (in milk) depletion between 4-6 mo o Adolescents: Rapid growth and starting period (females) lead to increased risk of anemia, must increase iron intake o Iron intake: Critical for appropriate development of hemoglobin and RBCs Iron fortified cereal Breast milk might need iron supplement Iron deficiency Anemia: o Causes: Not enough iron to produce Hgb Decreasing maternal stores and rapid growth times (infants/adolescents) Hgb levels decrease, o2 carrying capacity of blood decreases, results in weakness and fatigue o Associated with: Delayed growth Cognitive delays Behavior changes o Assessment: Health htx: Irritability HA Dizziness Weakness SOB Pallor Dietary intake of iron Health issues o Risk Factors: Gestational diabetes Prematurity Maternal anemia Low iron formula Breastfed-no iron supplements Low socioeconomic status Antacids o Exam: Lethargy Fatigue Inspect skin for pallor Spooning of nails Pulse ox HR-tachy Murmur Splenomegaly o Tx: OTC iron supplements Severe: may need transfusion o Nursing management: Safety: neurologic function r/t low O2 w/risk for fatigue decrease eating difficulties with ambulation (sitting, walking, standing) Nutrition: Formula with iron Iron supplements at 4-5 mo for breastfed infants (iron fortified cereal) Limit fast food consumption Iron rich foods: Red meat Tuna Salmon Eggs Tofu Enriched grains Dried beans Peas Dried fruits Green leafy veggies Iron fortified cereal Iron supplement admin: Measure precisely Liquid placed behind teeth (iron stains) May cause constipation o Increase fluids and adequate fiber intake) Dark colored stool Follow up: Monitor iron levels and iron intake Math problems 2-see “math to know for exams” Allergic reaction Allergies result from exposure to environmental or food allergens Caused by cell damage from antigen-antibody rxns that release histamine and causes allergic s/s 3 steps to dx: o Personal and med htx o Physicial examination o Diagnostic testing May use allergy testing to diagnosis and identify allergens s/s: o itchy eyes o runny nose o stomach cramps o hives o swelling o cough o wheezing o SOB o Redness o Pain o Feeling faint o Throat closing Tx: o Antihistamines o Bronchodilators o Corticosteroids o Preventative inhaled meds Common allergens: o Food allergens: Cows milk Eggs Wheat Chocolate Citrus fruit o Inhalant allergens Mold Pollen House dust Pet dander o Drug allergens Oral and injectable o Contact allergens Plants Dyes Chemicals o Other: Animal serum/venom Insect stings o Food and latex allergies: Wheezing is sign of edema occurring in the airway Sign of resp complication Provide dietary teaching on how to read food labels for food allergies Children should avoid exposure to allergens 3 types of rxns to natural latex: IgE mediated Cell mediated contact dermatitis Irritant dermatitis o Children with spina bifida should never be exposed to latex anaphylaxis 2 Acute, immediate, and severe IgE-mediated response to allergen Most common triggers: o Nuts o Shellfish o Eggs o Insect stings o NSAIDs o Radiopaque dyes o Latex Usually occurs within 5-10 min of contact with allergen Tissue edema leads to vasodilation and circulatory collapse s/s: o pruritic urticaria o coughing o lip and tongue swelling o stridor o extreme anxiety o asthma attack o loss of consciousness Tx and management: o IV epinephrine o Corticosteroids o Antihistamines o Support airway with intubation and ventilation if lip and tongue swelling and airway compromise o Assess circulation and administer IV fluids to provide vol expansion o Monitor for at least 2 hrs after anaphylactic rxn o Use intramuscular epinephrine when in community or outpatient settings Send pt to ER after General eye and ear (such as otitis media and how to look in the ear) 2 Otitis media o Bacterial/viral infection of middle ear o ET position increase risk secondary to URI o Complications: Hearing loss Expressive speech delay Tympanosclerosis TM perforation Chronic suppurative (pus) OM Acute mastoiditis Bacterial meningitis and abcess o Bacterial Antibx (amoxicillin PO) if rapid onset s/s s/s: middle ear inflammation, ear pain, erythema of TM may resolve wo antibx just wait and see o Viral Resolves on its own o PE tubes: Equalize pressure Allow air in and fluid to drain Last 6 mo Fall on its own Do not prevent infections Keep ears dry w. earplugs in pool or lake o Otitis media with effusion Fluid in middle ear wo s/s infection Risks: Smoke Absence of breastfeeding Frequent URI Allergy Age (younger) Male adenoid hypertrophy ET dysfunction Congenital disorders Complications: Hearing loss Deafness AOM Children younger than 3: pull pinna down (short) and straight back Children older than 3: pull pinna upward (tall) and back High and low blood sugar signs and insulin –many Type 1 diabetes Autoimmune condition resulting in pancreatic damage and lack of insulin s/s: o wt loss o polydipsia o polyphagia o polyuria o fatigue o blurred vision o mood changes diagnose with lab testing (A1C, fasting glucose, plasma glucose) management: o insulin therapy o glucose monitoring o insulin education o maintaining proper nutrition o pt and fam education o Fever management Fever usually peaks in evening Antipyretics used for discomfort with fever o Acetaminophen: 10-15 mg/kg/dose q 4-6 hrs limit 5 doses/24 hrs o Ibuprofen: 5-10mg/kg/dose q 6 hrs; increase 6 months age; limit 4 doses/24 hr; never give on empty stomach Never use aspirin (ASA) for child w. fever d/t risk for Reye’s syndrome Monitor for s/s of dehydration Sponging: o Controversial; done after antipyretics; use tepid (not cold) water or alcohol; ensure sponging does not initiate shivering Call provider: o Less than 3 mos and increase T 100.4 (38) o Any child who is lethargic or listless no matter what temp is o Fever increase 3-5 days o More than 105 o Immunocompromised by illness w/ cancer/HIV Light clothing Remove blankets Cooling blanket Fan Do not induce shivering/discomfort Signs of dehydration (such as mild to severe) Sickle cell 2 Abnorm Hgb gene (Hgb S) Sickle-shaped RBCs (clump together) o Not clotting, just clumping Autosomal recessive disorder o Asymptomatic carriers Ethnically linked (African, Mediterranean, Hispanic, middle eastern, Asian) Hgb F is protectant for 1st 4-6 mo of life o Hgb F doesn’t behave same way o Once we switch to Hgb A we start to have problems Occlusion and hemolysis o RBCs are fragile and break down faster Premature RBC death o Low RBC counts o Hemolytic anemia Sickle cell crises: o Triggered by hypoxia, acidosis, fever, dehydration, and hypothermia o Vaso occlusive crisis: Clumped up sickle cells are occluding vascular circulation Severe pain from ischemia Dactylitis (swelling of fingers and toes) o Splenic sequestration: Blood gets trapped in spleen o Silent cerebral infarct: Neuro assessments Stroke or silent cerebral infarct o Acute Chest syndrome: Pulm vessel occlusion Mimics pneumonia but infarcts and not infiltrations Sickle Cell Tx: o Address triggers: Isotonic fluid for dehydration/fluid bolus Warm compresses for pain Prevent infection (hand washing, antibx, vax) Treat fevers quickly: give antipyretic o Pain management: Nonpharm interventions Non-steroidal meds adjunct Opioids when needed o Bone Marrow transplant: Autologous stem cell therapy w. human leukocyte antigenmatched sibling Cord blood transplants o Hydroxyurea: Increases level of fetal Hgb Reduces amount of crises and promotes splenic function Can cause neutropenia o Deferoxamine: Used if hemolysis results in high levels of free iron Binds with the excess free iron that are released by the hemolyzed RBCS Subq infusion: Chelating agent to bind iron Use with vitamin C Monitor for SE o Transfusion of packed RBCs o Psychosocial support: Sickle cell foundation Family Management: o Early identification and prevention of crisis o Family education, genetic testing, importance of follow ups o Prophylactic penicillin: prevent infection o Folic acid: helps make RBCs Prevention and early detection of vaso-occlusion o Any febrile illness: seek immediate attn o Vax and penicillin prophylaxis o Encourage adequate fluid intake o Limit exposure to temp extremes o Avoid stress and overexertion o Contact HCP at first sign of pain crisis o Seek tx immediately if: Pale and listless Abd pain Limp or swollen joints Cough SOB Chest pain Unusual HA Loss of feeling Sudden weakness Priapism (sickling in penis/ prolonged erection) Sickle cell labs: o Newborn screening: Law in all states indicates possibility of sickle cell dz requiring further eval by Hgb electrophoresis or sickle cell turbidity test o Hgb: Baseline usually 7-10 mg/dL (12-17 norm) Significantly lower w. splenic sequestration, acute chest syndrome, or aplastic crisis o Hgb electrophoresis: Blood test to look at Hgb and see types o Reticulocyte count (immature RBC) Greatly elevated Body is having to make lots of RBCs to replace the ones that are bad General cancer and neutropenic precautions Oncological principles: o Proto-oncogenes: Normal genes that can mutate into cancer cells Can regulate cell growth, division, death o Can mutate to form oncogenes which create unchecked cellular growth and resulting malignancy o Tumor suppressor gene involved in cell growth and death (apoptosis) o Neoplasm: results from uncontrolled regulation of tumor suppressor cells (malignant or benign) o Process of oncogenesis is the same in children and adults o Some genetic mutations may contribute to cancer development Peds vs adults cancer: o Origin Peds: Embryonic cells, blood cells, CNS Adults: epithelial cells o Part of body affected Peds: tissues, bone marrow components Adults: solid organs o Cause Peds: not well understood Adults: mainly dt environmental toxins o Prevention Peds: human papilloma vax; most not preventable Adults: most preventable thru screening and limiting exposure to toxins o Response to tx Peds: highly responsive Adults: less responsive Chemo SE/risks o Infection: Pts are at high risk for infection bc myelosuppression dt chemotherapy Monitor WBC and practice neutropenic precautions (masks and appropriate ppe when in pts room, no fresh fruits/veggies, no raw/rare meat, only pasteurized dairy products, no live/active cultures in yogurt) (this is from google and my basic knowledge from working at the oncology unit-could not find in PowerPoint) Admin granulocyte colony stimulating factor o Anemia RBC suppression Treat anemia with iron supplementation, epoetin alfa, blood transfusion o Hemorrhage platelet suppression Treat platelet suppression by monitoring for s/s of internal bleeding, avoiding high impact activity, avoiding rectal temps and other procedures o Nausea: Administer antiemetics before infusion and around the clock 24-48 hrs after Nonpharm therapies: ginger, peppermint oil. Acupuncture, and craniosacral therapy Cluster care to minimize interruptions o Mucositis White patches, ulcer, redness Suck on ice chips/ice pops for pain or meds Avoid spicy, acidic, salty, hot foods Eat moist and soft foods Rinse mouth 5-6x daily Use soft toothbrush o Pain: Admin pain meds Oncological emergencies: o Tumor lysis syndrome Metabolic abnormalities from release of intracellular contents after malignant cells are destroyed hyperkalemia, hyperphosphatemia, hypocalcemia, hyperuricemia hydrate with IV fluids b4 chemo treat with allopurinol: for uric acid buildup o Sepsis Progression of infection in children w. neutropenia Neutrophil count <500 at higher risk Tx w. fluids, antibx, vent support o Superior vena cava syndrome Compression of vena cava s/s: dyspnea, cough, HA, pleural effusion, edema in neck and UE treat tumor to reduce pressure may need to intubate o Increased ICP From primary tumor, metastases, surg procedure May result in brainstem herniation wo intervention ALL (leukemia) Acute Lymphoblastic Leukemia Most common cancer in children in U.S Immature nonfunctioning WBC (lymphoblasts) dominate bone marrow production RBC and platelet suppression from WBC production s/s: o fatigue o pallor o fever o anorexia o petechiae 3 phases of chemo for Tx: o Induction phase (4 wks): induce remission o Consolidation phase (variable): Strengthen remission and CNS prophylaxis w. intrathecal chemo o Maintenance phase (2-3 yrs) Eliminate residual cancer cells w. intermittent chemo Ensure pain management for procedures Hodgkin’s lymphoma Characterized by presence of Reed-sternberg (RS) cells o B lymphocytes that have lost immune function Typically starts in lymph nodes and spreads thru lymph system (rarely occurs below the diaphragm) s/s: o swollen, painless, rubbery lymph nodes in cervical and supraclavicular region o fever o night sweats o cough and dyspnea (mediastinal mass) staged based on spread tx: combo of chemo and low dose radiation Ticks Use fine-tipped tweezers Protect fingers w. tissue, paper towel, latex gloves Grasp tick as close to skin as possible and gently pull upward w. slow steady, even pressure Do not twist or jerk tick After removal, clean site q. soap and water Wash hands Save tick for identification if illness occurs lice Type of parasite Pediculosis capitis o Aka head lice Pediculosis pubis o Pubic lice s/s o itching o nits o look like dandruff but do not comb away General psych Disorders result from genetics, physiological changes, and environmental exposures Mental health: o State of well-being in which every individual realizes his or her own potential, can cope with the normal stresses of life, can work productively and fruitfully, and is able to contribute to his/her community Cognition: o The process of thought and knowing that is acquired thru experiences and maturation Pediatric vs Adult Mental Health o Definitions consistent for children and adults o Anatomical changes to fetal brain yield cognitive and behavioral deficits o Children differ from adults in the scope, presentation, and progression of mental and cognitive disorders o Access to good nutrition, positive relationships, and safe housing affects development of mental illness and cognitive disability Gen Nursing Interventions: o Provide supportive care Play therapy Form of psychotherapy that encourages children to express feelings and emotions thru play Used for 3-12 yrs of age Therapeutic play Technique employed by child life specialists for hospitalized children Art therapy Incorporates creativity in healing and expressing childhood emotions (all ages) o Maintain safe environment Ensure safety from SH Environmental assessments No sharp objects or tubing Standard safety measures Adult supervision Assess the safety or home environment if outpatient and hospital environment if inpatient Special needs Learning Disabilities: o Difficulties in receiving and processing info and generating appropriate responses o Most common disorder: dyslexia (disorder of receptive lang that creates difficulty using letters to decode written lang) o Common s/s may not appear until child enters school o S/S: Slower acquisition of lang and math skills Difficulty recognizing letters and numbers Problems with reading comprehension o Identify thru standardized developmental eval Assess for sensory difficulties o Treat with an individualized plan (IEP) to foster appropriate growth and development Stem cell transplant Intense chemotherapy and radiation to create myelosuppression followed by infusion of stem cells Autologous transplantations use the child’s own stem cells Allogenic transplantations use donor stem cells Before transplant: o Myelosuppression to decrease potential rejection o Protective iso in positive pressure room o Prophylactic antibx, antifungals, and immunoglobulins After transplant complications: o Graft-versus-host dz (acute or chronic) o Growth retardation, hypothyroidism, and secondary cancers Death perception by age Infants/toddlers (birth to 3 yrs) o Have little to no concept of death o Egocentric thinking prevents their understanding death (toddlers) o Mirror parental emotions (sadness, anger, depression, anxiety) o React in response to changes brought about by being in hospital (change of routine, painful procedures, immobilization, less independence, separation from family) o Can regress to an earlier stage of behavior Preschool children (3-6 yrs) o Egocentric thinking o Magical thinking allows for the belief that thoughts can cause an event such as death (as a result, child can feel guilt and shame) o Interpret separation from parents as punishment for bad behavior o View dying as temporary bc of lack of a concept of time and bc dead person can still have attributes of the living (sleeping, eating, breathing) School-age children (6-12 yrs) o Start to respond to logical or factual explanations o Begin to have an adult concept of death (inevitable, irreversible, universal) which generally applies to older school age children (912) o Fear often displayed thru uncooperative behavior o Can be curious about funeral services and what happens to body after death Adolescents (12-20 years) o Can have adult-like conept of death o Can have difficulty accepting death bc they are discovering who they are, establishing an identity, and dealing with issues of puberty o Rely more on peers than the influence of parents which can result in the reality of a serious illness causing adolescnets to feel isolated o Can be unable to relate to peers and communicate with parents o Can become increasingly stressed by changed in physical appearance due to medications or illness than the prospect of death o Can experience guilt and shame Autism (ASD) 3 Genetic basis combined with environmental factors (no link to vax) Implement standardized developmental screenings into well-child appointments (MCHAT, Ages and Stages, Denver developmental test, etc) s/s: o stereotypy (repetitive movements) o obsessive behavior o difficulty with sensory integration o echolalia (repetition of words w/out meaning) o avoidance of eye contact Impairments: o 3 specific areas: Communication Socialization interaction Repetitive & characteristic behavior Perseverative/stereotypy: o Rocking, flapping, lip smacking o + some have intellectual disability, others do not ASD tx: o Early identification and referral to early intervention programs are the cornerstones of effective tx o Plan educational activities that limit overstimulation of senses o Collab with school personnel to align child’s needs for maximal learning and limited distress o Consult with speech therapist to improve communication skills o Utilize multidisciplinary approach (developmental pediatricians, behavioral health specialists, occupational therapists) Idiopathic thrombocytopenia purpura (ITP) Clotting disorder-IMMUNE RESPONSE Immune response approximately 1-4 wks after viral infection causing production of antiplatelet antibodies causing platelet destruction More common in young children, most self-limiting, recover in a few months Presentation: o Bruising o Petechiae (pinpoint) o Purpura (larger areas) Complications: o Severe hemorrhage o Intracranial hemorrhage o Organ bleeding Platelet count & Tx: o <10,000 (norm 150,000-400,000): give corticosteroids (prednisone or prednisolone: po 2-3 wks or count >30,000) o IVIG o Platelet transfusions not indicated unless life threatening Assessment: o Previously healthy child with recent increase bruising, gum bleeding, epistaxis (nose bleeds) o Recent viral infection o MMR immunization o Meds with thrombocytopenia SE Note: petechiae/purpura and progression include lips and oral mucosa Management: o Observation o Re-eval o Avoid ASA and NSAIDs o Give acetaminophen for pain PRN o Avoid trauma (EX: CONTACT SPORTS) o Know s/s serious bleeding and when to call HCP Henoch Schoenlein Clotting disorder Develops in association with viral or bacterial infection (typically respiratory) Benign, good prognosis o Unless ongoing nephrotic syndrome d/t renal injury and possible HTN, pulm, cardiac, neurologic complications If renal injury: o Renal function tests o Eval BP o Tx as needed Presentation: o Vasculitis (leakage) with IgA(antibody) dominant immune deposits affecting small vessels o Skin (purpura rash) o kidney, gut, and joint pain Tx: o As needed with corticosteroids if severe joint/GI manifestations Management: o Treat s/s o Pain management o Hydration if norm renal function o I&O o Teach s/s renal injury to parents and when to notify HCP Beta thalassemia aka Cooley’s Anemia Abnorm Hgb beta chains lead to rapid RBC destruction Autosomal recessive condition Ethnically linked (Mediterranean, middle eastern, Asian) Hemolytic anemia with bone marrow hyperplasia d/t erythropoiesis (thin cortical bone w. new growths) o Bone marrow makes lots and lots of RBCs resulting in expansion of bone marrow Characteristics of dz: o Severe anemia o Cellular damage o Free iron buildup (hemosiderosis) o Bronze skin o Frontal bossing Manifestations: o Bone malformations (maxillary, frontal lobe) Caused by the bone marrow expanding trying to make more RBCs o Osteoporosis and osteopenia o Splenomegaly and hepatomegaly o Cardiomyopathy o Short statues o Yellow or bronze skin pigmentation Tx: o Transfusion therapy o Chelation therapy o Bone marrow transplantation o Monitor effects on growth and development Tx (2nd slide) o Frequent blood transfusions to replace deficient RBCs Infection control with central venous access devices Follow blood bank protocols and keep in blood refrigerator Monitor for transfusion rxns o Chelation therapy to reduce free iron Oral or parenteral agents Take with vit C Monitor for SE, kidney damage, liver damage Use a supervised care approach to improve compliance o Splenectomy o Growth hormone replacement Nursing Management o Blood transfusion, education, monitor for transfusion rxns o Chelation: Admin with transfusion Iron—ferrous; chelation products have fer in their name o IV Desferal (deferoxamine) Chelating agent Binds to iron and allows its removal via stool & urine Given subq at home by family via battery pack infusion pump o Exjade Oral deferoxamine Well tolerated Minimal SE Monitor renal and liver function Monitor for GI bleeding Dissolve in juice or water o Family teaching: Adherence to transfusion and chelation schedule Chelation therapy for iron and lead- drugs used Iron: o IV Desferal (deferoxamine) or subq o Exjade (oral deferoxamine) Lead: o EDTA-ethylene diamine tetra-acetic acid Autosomal recessive inheritance 2 copies of abnormal gene in homozygous state required to produce phenotype Both parents of affected person must be carriers of abnorm gene (25%) In other words, requires 2 copies of the mutated gene (one from each parent) to cause the disorder Humoral vs. cellular immunity Humoral immunity: o Specific immune function o Involves B cells, which recognize specific antigens and secrete antibodies o Training Cellular immunity: o Specific immune function o Involves T cells which attack antigens marked by B cells o Automatic Organ donation Open casket funeral usually possible for organ, eye, and tissue donors Thry entire donation process, body is treated with care, respect, and dignity and will not be disfigured There is no cost to donors or their families Federal law prohibits buying and selling organs in US o Violators punished with prison sentences and fines o Dang, plan b is out the window Majority of deceased organ donors are pts who have been declared brain dead National computer system matched donated organs to recipients Most major religions in US support organ donation and consider donation as the final act of love and generosity towards others Anyone, regardless of age or med htx, can be a donor Childhood infectious diseases (basic definitions and cause) Types of infections: o Bacterial: staph, strep, sepsis MRSA Staph infection resistant to certain antibx Acquired thru contact, resp droplets, blood, sharing personal items, contaminated surfaces Scarlet fever o Sandpaper rash and strawberry tongue Diphtheria Pertussis (Bordetella) Tetanus (lockjaw) Botulism Osteomyelitis Septic arthritis o Viral: in general (viral exanthems: a rash caused by virus) Mumps: inflammation of parotid glands; fever and parotiditis Spread by resp secretions Varicella Measles(rubeola) Highly contagious Spreads thru resp secretions Mild fever, conjunctivitis, coryza, cough, kolpik spots (clustered white lesions in oral mucosa), maculopapular rash Rubella German measles Spread by aerosolized particles Irregular macular rash, fever, malaise, HA, sore throat, red eyes, lymphadenopathy o Zoonotic: Cat scratch dz: painful swollen nodes rabies o Vector-borne: Lyme dz: from deer tick bite, ring like bull0eye rash around bite, remove tick, treat w. antibx West nile: mosquito bite, febrile illness, may lead to meningitis Rocky mountain spotted fever: dog and wood tick bite, tx with tetracyclines Malaria: mosquito bute; high fever and chills may have cyclic pattern Zika: mosquito bite; can cause microcephaly o Parasitic and helminthic: pediculosis (lice) scabies helminth worms pinworms (fecal-oral route-rectal night itchingscrotch tape) (everyone had this in cuba) roundworms, hookworms (pores, hair follicles, skin, eating) Chickenpox (varicella) Itchy red macules then papules-vesicles-scabs As initial lesions progress thru stages, new lesions form on trunk and extremities even as some scab dev 2 wks after exp Contagious 1-2 days before eruption Not contagious once all lesions scab and dry Extremely contagious herpes virus Prevented thru vax Can remain latent and reactivate later as shingles Transmitted thru contact with weeping lesions or nasopharyngeal secretions Children contagious 1-2 days before rash appears and until all lesions are crusted over s/s management: o acyclovir for severe cases Treat hypoglycemia 15 g of carbs Posterior pit gland disorder Not enough ADH Most common form No cure Life-long tx Characteristics: DI o Excessive thirst o Excessive urination not affected by decreasing fluid intake (polyuria/polydipsia) Cause: o Head trauma o Post cranial surg o Genetic mutation o Granulomatous dz (immune deficiency) o Infections (meningitis/encephalitis) o Vascular anomalies o Congenital malformations o Leukemia o Meds inhibiting vasopressin(adh) release (phenytoin) o Idiopathic/hereditary Antidiuretic hormone (ADH) is called vasopressin and comes from hypothalamus and posterior pit gland o In DI body does not produce enough adh o Pt is not able to hold on to urine or concentrate it o Pt will urinate until dehydrated o Drink excessively to compensate for water loss s/s: o weight loss o ftt o dehydration (dry MM, decreased tears, poor skin turgor) o increased UO o tachycardia o tachypnea Tx: o Daily replacement of ADH with DDAVP (desmopressin) which is vasopressin/ADH Nursing management: o Individualized care o I&O o Monitor for hypernatremia (thirst, neuromuscular excitability, confusion, seizures) o hydration o promote activity o education o daily wt DDAV o Keep refrigerated o Clear nostril before giving o Proper dosage o Repeat dose if child sneezes o Measure specific gravity o Monitor s/s of overdose (siadh) Confusion HA drowsiness Rapid wt gain secondary to fluid retention SIADH Excessive secretion of ADH resulting from central nervous system changes Water is reabsorbed and urine output is decreased leading to decreased serum sodium from hemodilution s/s: o water intoxication o hyponatremia o jugular vein distension o increased BP o sudden wt gain o fluid in lungs o concentrated urine Tx: o Fluid monitoring o Restriction and demeclocycline (reduce reabsorption of water) Teach pts and families about water restriction Encourage child to wear medical alert bracelet Normal range for specific gravity 1.005-1.030 Lupus Chronic autoimmune dz consisting of remissions and exacerbations s/s: o butterfly rash on bridge of nose and cheeks (malar rash) o fever o joint inflammation/arthritis o fatigue o weight loss o splenic enlargement o vasculitis o leukopenia o anemia o nephrotic syndrome o Raynaud disorder o Alopecia o Photosensitivity o Stomatitis o Pleurisy o seizures Management: o Encourage rest during lfare ups o Help pts and caregivers identify signs of impending flare up o Protect against cold weather and ultraviolet light Medications based on severity: o Mild to moderate: Corticosteroids Antimalarial agents (chloroquinine) NSAIDs o Severe: High-dose corticosteroids and/or immunosuppressive drugs ADHD (Attention Deficit Hyperactivity Disorder) Neurobehavioral disorder characterized by inattentiveness with or w/out hyperactivity Pathophysiology is unclear, but appears to have genetic component s/s: o short attention span o impulsivity o difficulties with movement Rule out sensory and organic causes of s/s before diagnosing Diagnose with formal eval and psychological testing (include educators, caregivers, and child in eval process) ADHD Tx: o Caregiver education, development of individualized education plans, pharmacotherapy o Meds: Stimulants: Ritalin Adderall Focalin Controlled substances: These meds are controlled substances strict prescribing practices and restrictions SE: Weight loss Appetite suppression Tachycardia Hypertension Labs to monitor: Serum liver function levels Serum kidney function levels Sensory integration dysfunction Neurologic disorder where child cannot organize sensory input used in daily living Develops hyposensitivity or hypersensitivity to sensory input: o How brain processes multiple sensory modality inputs including: Proprioception (position awareness) Vision Auditory system (sound) Tactile (touch) Olfactory (smell) Vestibular system (balance) Interoception (internal sense- pain, hunger, emotions, etc) Taste Result: o No or limited or overreaction to diff textures, sounds, sensations, etc Risks: o Preterm infants o Low birth weight o Autism spectrum disorder Therapy: o OT and other therapies to ↑ ability to function, respond appropriately, & control responses Neurofibromatosis Neurocutaneous genetic disorders of nervous system Characterized by abnormalities of skin & CNS Primarily affect growth & development of neural cells Progressive & usually worsens over time Unpredictable Type 1: o Most common type o Tumors grow on nerves and produce abnormalities including skin changes & bone deformities Characteristics: Café-au-lait spots (light brown macules, often present at birth) ↑ size, #, and pigmentation Usually spare face Pigmented nevi (moles) Axillary freckling Slow growing cutaneous & subq or dermal neurofibromas (lumps) Complications: o Headache o Hydrocephalus o Scoliosis o Cardiac defects o HTN o Seizures o Vision/hearing loss o Neurocognitive deficits o Learning disabilities o ADD o Fine and gross motor delays o ASD o Behavioral and psychosocial issues o Speech abnormalities o ↑ risk of neoplasms (abnorm growth of tissue) Management: o No cure o Control symptoms o Manage complications Nursing management: o Supportive care o Early detection o Support and education Anxiety disorders Worry, fear, and anxiety extend past normal adaptive coping mechanisms and cause stress and significant impairment s/s: o Abdominal pain o Palpitations o Nausea o Vomiting o Dyspnea o Aggression o Defiance o Dizziness Categorized by causes and manifestations: o Generalized Anxiety Disorder o Separation Anxiety Disorder o Panic Disorder o Obsessive-compulsive disorder (OCD) Anxiety disorders tx: o Exposure-based cognitive behavioral therapy to treat separation anxiety disorder and school refusal Find out what’s causing school refusal and solve the problem o SSRIs often used for generalized anxiety disorders o SSRIs may increase risk of suicide in pediatric population Obsessive compulsive disorder Repeated behaviors (checking curling iron unplugged, washing hands, cleaning) done to ↓ anxiety from obsessions Growth and Development 3 questions from module 1 Neuro 2 questions from module 1, especially seizures, increased ICP, and meningitis page in Canvas