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IRON DEFICIENCY ANEMIA-Inadequate intake of Iron-Low iron=low reticulocyte count-W/o iron, hemoglobin can’t be produced=ANEMIA-Highest Risk --6-36 months --adolescent- Depleted fetal store of iron at 6 months- Premature or multiple birth babies: Risk relatedTo decreased fetal iron stores and rapid growthASSESSMENT- General signs (all r/t lack of oxygen) --Irritability --Anorexia --lethargy- Infant may be overweight --“milk babies” --excessive milk intake(get lots of calories), but don’t get enough iron --cows milk causes hemmorragic bowel problems- Toddler usually underweightSEVERE OR LONG-STANDING ANEMIA- Behavior, Cognition, Developmental --watch for behavioral issues due to lack of O2 to brain- Growth retardation- Cardiac enlargement- Exercise intolerance-May exhibit PICA: eating dirt and shitINTERVENTIONS- Iron fortified food or cereal-Iron enriched foods- inadequate by itself --greens, vegetable, whole grains- Iron supplements --PO: Fer in SOL; should see an ^ in H/H if it works --give between meals unless child vomits-IM --Z track in large muscle -- No more than 1 mL per site --Don’t massage siteTEACHING-If iron is adequate, you’ll see green tarry stools-Liquid preparation can stain teeth; use straw- Don’t admin iron with cows milk/antacid: binds to iron and blocks absorption: give w/vit c, OJ, apple juiceSICKLE CELL ANEMIA- is a GENETIC DISEASE-both parents must have trait to pass on to child (autosomal recessive)- Carrier State --Sickle Cell Trait --8% of AA are positive for sickle cell trait- Disease State --Sickle cell anemiaASSESSMENT- Prenatal --Chorionic Villus Sampling -- Amniocentesis -- Placental Tissue is taken- After birth -- Mandatory Newborn Screening -- Hemoglobin Electrophoresis --gives hemoglobin shock --Sickledex for mass screening --Dose not differentiate between carrier state and Disease.-FETAL HEMOGLOBIN: produced @ birth and for a short period after. Does not sickle. (this is why hemoglobin won’t show up at first) --generally don’t show signs until middle to late infancy --General S/S -- Across childhood, tend to be smaller in ht and wt than peers --possible delay in sexual maturation --Chronic Anemia --RBC life span is 120 days (normal) --Life span of RBCs that sickle: 6-20 daysCRISIS-Disease is characterized by Crisis1)Vaso Occlusive2)Sequestration 3)AplasticSickle cell anemiaVASO OCCLUSIVE CRISIS (PAIN CRISIS)-cause by obstructionPatho1. Sickled cells block the microcirculation (LOG JAM)2. Absence of blood flow distal to obstruction3. Hypoxia of tissues distal to obstruction4. Tissue ischemia and infarction5. Symptoms are a result of obstructionTRIGGERS-Lack of oxygen KEEP OXYGENATED- Hypoxemia --can be caused by exercise, anything that uses O2.- Acidosis- Dehydration KEEP HYDRATED- Cold; - InfectionACUTE SIGNS- Hand-foot syndrome --painful, bilateral swelling of hands/or feet- ABD resembling surgical condition- Acute Chest Syndrome: lungs become under oxygenate b/c of log jam in vessels-Stroke-PriapismCHRONIC SIGNS- Cardiomegaly b/c heart is working harder- Inability to concentrate urine, enuresis, or both- Hepatomegaly- Splenomegaly; --eventually spleen stops functioning- Blindness: retinal detachmentPAIN MANAGEMENT FOR VASO-OCCLUSIVE-Acetaminophen or Ibuprofen-Add codeine-Morphine on PCA pump-Oxycodone-Hydromorphone-At above average risk for Demerol-induced seizures (don’t use in kids)SEQUESTRATION CRISIS- POOLING OF BLOOD IN THE SPLEEN AND LIVER --which means there is not enough blood in periphery-Splenomegaly (very ominous sign) & Hepatomegaly- Circulation Collapse --b/c not enough blood in peripheryAPLASTIC CRISIS- DECREASED RBC PRODUCTION-bone marrow suppressed by infection, decreased folic acid= can’t build hemoglobin- Profound Anemia --*Probably need a blood transfusionTEACHING- Seek early treatment for signs of illness --101.5 temp; call doctor- Importance of prophylactic medication --oral penicillin by 2 months --pneumococcal, meningococcal and influenza vac- KEEP HYDRATED- Avoid cold or warm compress --especially cold b/c cold causes vasoconstrictionOTHER INTERVENTIONS FOR SICKLE CELL ANEMIA- Medications --Hydoxyurea: increases Fetal Hemoglobin (which ^O2 production) --reduces production of abnormal blood cells --SE: Bonemarrow suppression-KEEP HYDRATED, LYTES REPLACE- Short Term O2 therapy for respiratory difficulty (crisis) --Will not reverse sickling --Prolonged use can depress bone marrow-Transfusion program after a stroke --so brain can stay oxygenated --reduce amount of abnormal cells (short term fix)- Transfusions for reasons other than a strokePROGNOSIS- Variable depending upon severity of disease --w/good care, can live into 50’s-Stem Cell Transplantation --mortality rate is high-if they die before age 5, its b/c of overwhelming infection B-THALASSEMIA/ COOLEY’S ANEMIA/ MEDITERRANEAN ANEMIA (24:17)PATHO1. Normal HGB composed of 2 alpha & 2 beta polypeptide chains2. With B-Thalassemia, beta polypeptide chain is decreased or absent3.Fragile, damaged RBCs result4.Severe Anemia-PROBLEM is in the RBCs-NO PROBLEM with hemoglobin --Therefore, body can still produce reticulocytes. Will see increased reticulocyte count b/c its trying to make more RBCs.*NO OCCLUSIVE PROBLEMS b/c they are small-Thalassemia Major-Disease State-Thalassemia Minor- Carrier StateDIAGNOSTICS-Prenatal --Amniocentesis --Chorionic Villus Sampling-After Birth --Hemoglobin ElectrophoresisASSESSMENTS- Asymptomatic First 6 Months -- b/c of Fetal Hemoglobin- Symptoms occur due to --Defective --Structurally impaired RBC --Shortened erythrocyte lifespan-Major Consequence --CHRONIC HYPOXIA --Fe OVERLOAD from blood transfusion. WILL SEE:-Hepatosplenomegaly: due to iron depositing in tissues-Growth abnormalities: Hyperplasia of bone marrow cavity -RISK for fractures-Cardiac- heart will develop CHF-Endocrine- can get a fibrotic pancrease-Skin: iron can spill into skin gives a bronze color --kids will look darkerGOAL: Maintain adequate Hgb levels- transfusion program-Transfuse --slow body’s production of defective hemoglobin --prevent bone changes --provide sufficient RBCs for Growth-Hemoglobin >9.5 gm/dl is what you want to get hgb to --Generally every 3-5 weeksSPLENECTOMY- Treats severe splenomegaly-Increases life span of transfused RBC-Increases susceptibility to overwhelming infection-Interventions --Antibiotics --Pneumococcal & Meningococcal Vaccines b/c no spleen --Regular Immunizations-Risk for sepsis **Call physician, go to ER if fever develops**HEMOSIDEROSIS-Iron overload 2ndary to transfusions --deposited in tissues (spleen, heart)-Treatment --Desferal (Deferoxamine)- binds to iron --Chelating Agent - will cause urine to be red, - Check input/output, KEEP HYDRATED, - AVOID IRON RICH FOODS --Administration IV or SQ Portable infusion pump for 8-24 hours 5-7 days a weekLEAD POISONING (PLUMBISM)HYPOCHROMIC, MICROCYTIC ANEMIASOURCES- Ingestion -paint chips are sweet tasting-Inhalation -older houses may have lead in them-Lead binds to sites that normally bind to ironTHOSE AT RISK- Less than 6 years old- Poverty- Minority children- Urban setting- MedicaidASSESSMENT QUESTIONS- Does the child… --live in or regularly visit a house build before 1987? --live in or regularly visit an older home undergoing renovation? --have a sibling or playmate who has or had lead poisoning?ASSESSMENT- Universal screening recommended by CDC at 1 and 2 years of age.- Target Screening: if kid has elevated levels, check family- check for s/s of anemia b/c anemic kids are at higher riskGREATEST CONCERNS- Damage to the developing brain and nervous system of young children; will see learning/behavioral problems-With Massive Levels… --Cerebral Edema --Encephalopathy -- DeathDIAGNOSTICS- Lead Serum level greater than/equal to 10 mcg/dL- Abdominal X-Ray: will show paint chips- “Lead Lines” at Epiphyseal line of long bonesINTERVENTIONS- Remove child from the environment- All flat surfaces need to be wet, wiped off so dust won’t settle- Chelating therapy at 45mg/dL; --Medication binds with lead and leaves body -- Need more than one dose -- Equilibrium Process --Lead moves between blood, soft tissues, organs, bone, teethINTERVENTIONS CONTINUED- Protect kidneys with adequate hydration during Chelating Treatment-Succimer (Chemet) --Oral for 19 days --Capsule or sprinkle- Calcium disodium edentate (EDTA) --Very painful IMHIV(40:00)-Destroys T cells, eliminates immunityVERTICAL TRANSMISSION- When? -- Late in Pregnancy --I&D --Breastfeeding- Zidovudine (AZT): Inhibits action of viral transmission --Mom: While pregnant --Infant: for 6 weeks- PREVENTION FOR VERTICAL TRANSMISSION --prevent exposure to baby by: - Avoid breastfeeding - C section - Wash baby before giving injectionsHORIZONTAL TRANSMISSION- Transfused before 1987- Adolescents- High risk behavior --oh, you know… the booty do.ASSESSMENT-Maternal antibodies persist up to 18 months --PCR test to detect proviral DNA in babies younger than 18 months. Has a greater sensitivity, really expensive- ELISA or Western blot test used for 18 months or older -antibody test; defines HIV infection- Immature immune system --AIDS- Fulminant Course in 5-10 Months- Failure to thrive? - will see decreased adipose tissue, lack of appetite, small for age- Recurrent infections? --opportunistic infections present, body can’t fight it-Neurodevelopmental AlterationsTREATMENT GOALS- Slow growth of HIV virus- Prevent opportunistic infections- Provide nutritional supportTHERAPY- Antiretroviral Meds -Bactrim- prevention for Pneumocystis carinni- Immunizations -can’t give live vaccine if immunocompromisedITP: IDIOPATHIC THROMBOCYTOPENIC PURPURA- Most common bleeding disorder in children- Cause is unknown, but usually follows a viral illness- causes excessive destruction of platelets- WILL SEE: bruising, petchiae, DIAGNOSIS- by history - have they had a viral illness lately? -platelet count decreased-Bone marrow: must r/o leukemia- Come in looking bruised and abusedMANGEMENT-WinRHO- Steroids, Immunoglobulin- Splenectomy after 1 year of treatment if s/s continue -b/c spleen eats up platelets-ITP should resolve on ownHEMOPHILIA (4:36 II)Deficiency in clotting factorTYPES-Hemophilia A -- Classic --Factor VIII Deficiency -- 80-85% of all hemophilia-Hemophilia B -- Christmas Disease -- Factor IX Deficiency- The less clotting factor, the more severe problemsBLEEDING- Hemarthrosis- bleeding into a joint capsule --S/S occur at 6 months of age when activity increases -Swelling -Redness -Pain -Loss of movement -Repeated episodes of damage joint**Hallmark sign of Hemophila is Joint and Muscle Bleeding-Subcutaneous Hematoma -Left alone- unless can cause damage -Neck, mouth, throat- can obstruct airway-Epistaxis (Nose Bleed) -TX: apply ice, keep calm-Retroperitoneal Cavity -Hazardous b/c of large space -will see s/s of early shock b/c of massive blood loss-Spinal Cord - Hematoma can cause paralysis-Intracranial Hemorrhage PREVENT BLEEDING-Exercise and PT --regular program strengthens muscles around joints-Age- appropriate activity: may need to modify --NO contact sports-Teeth: need very good dental care-IM injections are to be avoided if possible --If need IM injection, hold pressure for 15 minutes-Venipuncture instead of heel sticksRECOGNIZE AND CONTROL BLEEDING- Believe child- RICE: Rest, Ice, Compression, ElevationFactor Replacement per Medical Protocol --FYI: Give all of factor in the bottle (expensive)-Genetically engineered recombinant factor is recommended over factor concentrate from pooled plasma b/c decreased risk of transmitting HIV and such -- Starting at 2-3 years of age - Family to start venipuncture or access central line And administer replacement factor -8-12 years of age - child will begin self-administration - prompts release of stored Factor VIII (DDAVP) DDAVP:desmopressin- Tylenol --some literature states ibuprofen may be used with Caution-Morphine.-COMPLICATIONS- Inhibitors --Factor specific antibodies - If used factor VII or IX are used too long, antibodies are produced making the synthetic useless; will use Factor VII or Novo7 (fairly new and expensive) -- Develop after child has received factor replacement -- AIDS - esp those receiving factor replacement prior 1987 -- Hepatits B or C - will have to get transfusions if exposed -- Insurance: They will drop you off their plan like assholes, then you can’t pay for treatment. Then you die.NEUROBLASTOMA (16:12 II)- Cancer arising from the sympathetic nervous system --Anywhere along the SNS chain --originates in the adrenal gland -- 20% in the thorax ASSESSMENT-S/S are based on location of the tumor --ABD: may press on other organs, distention, bowel Issues, SOB, --Thorax: SOB, neck, facial edema, vena cava syndrome --Cranium- Assess for metastasis of boneTREATMENT- Surgical removal of tumor- Surgical removal of tumor and chemotherapy-Chemotherapy before and after surgical removal of tumor- Frequent radiation therapyLEUKEMIAS (18:32 II)- Most common form of Cancer in Children --onset: preschool- 1st grade --down syndrome 20x risk for developing acute lymphoid leukemia- Types found in children --Acute lymphoid leukemia (ALL): better cancer to have --Acute nonlymphoid (myelogenous) leukemia: BADPATHOPHYSIOLOGY of ALL1. Rapid increase in the number of immature WBC- lymphocyte cells (blast cells)2. Blast cells compete for space and nutrients in bone marrow (infiltration & competition)3. Anemia, infection, bleeding & eventually weak bone: - Will see bone pain as main symptom- Blast cells/immature lymphocyte migrate to other organs --liver, spleen, blood brain barrier-Diagnosed based on --history --CBC with differential -Low WBC count is good, kid will have better outcome -*Bone marrow aspiration: need for definitive diagnosis -- Lumbar puncture: checks if blast cells have crossed BBB; if there is any CNS involvementCONSCIOUS SEDATION (24:57 II)-depressed LOC but must maintain independent airway and airway reflexes, respond to stimuli- Close monitoring required- Resuscitation equipment at bedside- Advantages are: --Reduction of fear, anxiety --relief of pain --minimal risk & rapid recoveryPHASES OF CHEMOTHERAPYINDUCTION THERAPY- Goal- Remission, kill as many cells as possible- Time of high risk because medications cause myelosuppression (bone marrow suppression)CENTRAL NERVOUS SYSTEM PROPYLAXIS-Goal- Prevent leukemic cells in CSF-Intrathecal administration-Cranial irradiation reserved for high risk patients - INTESIFICATION THERAPY -Goal- Destroy any residual leukemia -After completion of remission therapy - MAINTENANCE THERAPY -Goal-Remain in remission -After successful completion of induction & Intensification therapy -Hematopoietic Stem Cell Transplantation as soon as they go into remission -AML child will need thisWILM’S TUMOR (29:53 II)- Type of NEPHROBLASTOMA (kidney)- Occurs most frequently at age 2-5S/S- Fast growing, asymptomatic, firm, lobulated mass located on one side of the abdomenDIAGNOSIS- CT, IVP, Nephrectomy(biopsy)TREATMENT- DO NOT PALPATE, will break of cancer cells if you do- surgery, chemo, possible radiation*High cure rate- 95% if caught earlyBRAIN TUMORS (34:40 II)GENERAL SIGNS- subtle or rapid changes- behavioral signs- neurological signs (as if you had increased ICP) -headache -vomiting -seizures -visual disturbancesBrain tumor continued above rightTYPESASTROCYTOMA- Cerebral or Cerebellar- 40% of brain tumors in children- can be subtle or rapid signs depending on location -SLOW growing=subtle s/sMEDULLOBLASTOMA- 20% OF BRAIN TUMORS IN CHILDREN- FAST growing=rapid onsetRETINOBLASTOMA (35:40 II)-Primarily in children less than 5 years old- Cancerous tumor arising out of the retina - may be inherited, if parents had it, child will be monitored for itSIGNS/SYMPTOMS- *Classic sign is white eyeTREATMENT- Radiation- Removal of the eyeTEACHING- Prepare the parents - they expect to see an empty socket after surgeryHODGKINS AND NON-HODGKINS LYMPHOMA, SEE POWERPOINT. (31:00 II)RISK FOR INJURY R/T MALIGNANT PROCESS AND TREATMENT (37:49 II)EXTRAVAGATION OF CHEMOTHERAPY- Vesicants (sclerosing agents f’s up tissues)- Central Line- At the first sign of infiltration: STOP INFUSION IMMEDIATELYANAPHYLAXIS TO CHEMOTHERAPY-Prevention of - Thorough hx of allergies - preparation for: mask&bag, emergency meds- Recognition of - HTN, wheezing, Urticaria *IMMEDIATELY STOP INFUSION-Give aminophilineRADIATION THERAPY-Changes DNA so that it cannot reproduce- Scheduled over 1-6 weeks --Cells that are not susceptible one day will be Susceptible anotherSIDE EFFECTS - Radiation sickness -anorexia, nausea, vomiting -TX: rest, loose clothes on radiation site-Extreme fatigue- Skin reactionsTEACHING & RADIATION THERAPY- Site marked with indelible ink -do not try to wash-Do not expose irradiated area to direct sunlight or heat-No cream or lotion on site -Some contain metal which can misdirect radiationLATE EFFECTS r/t THERAPIESCHEMO & RADIATION - Secondary malignancy - Brain tumorRADIATION - Infertility - Gland dysfunctionCRANIAL IRRADIATION & INTRATHECAL CHEMO -Learning disabilities -Behavioral problemsRISK FOR INFECTION R/T DEPRESSED BODY DEFENSES (41:25 II)LEUKOPENIABone Marrow suppression -Hybrid Goals: 1. Recognize significance of absolute neutrophil count (ANC) in cancer care 2. Understand how to calculate (ANC) (SEGS+BANDS)xWBCx10=??? Below 500, ^ risk for Infection PREVENTION-**Prevent infection- private room- no one in room with an infection- assess sites of potential infection- monitor temperature-Prophylaxis against - Bacteria: give Bactrim - Fungi: give Nystatin - Virus: the herd effect, NO live immunizations-Adolescents - AbstinenceRISK FOR HEMORRHAGETHROMBOCYTOPENIA (43:56 II)Not enough platelets, decreased ability to clot- “Double Whammy” - Infection increases the tendency toward bleeding - bleeding sites are at high risk for infection-Oral care -soft toothbrush or cloth- Nose Bleeds - don’t disturb clots- No ASA containing products- Adolescent female -Birth control pills to suppress periods - No tampons- increases risk for infection -PlateletsALTERED SKIN INTEGRITY (44:54 II)PREVENTING ULCERATED MUCOSA- Mouth -Meticulous oral care, so they can eat - No alcohol based products-Perianal area -Wash after each BM. Sitz bath if ulcers present - Use protective barrier - Avoid rectal temps et suppositories - stool softeners - infant during chemotherapySTOMATITIS- Local Anesthetics -OTC Meds w/o alcohol - Chloraceptic lozenges - Orabase - Benadryl & Maalox- Prescription -Benadryl, Maalox & viscous Lidocaine - Viscous lidocaine in Not recommended for young Children- may depress gag reflex, ^ risk aspiration- Soft, Moist, bland dietALOPECIA- Hair regrows in 3-6 months - may grow back in different color and texture- Protect head from cold and sun- Scarves, Hats & Wigs - use cotton cap- polyester increases perspiration and causes itchingALTERED NUTRITION: LESS THAN BODY REQUIRES (III)BASICS- Avoid pressure to eat- do not let food become a control issue- small, frequent meals & nutritious snacks - may need to fortify foods or use supplementsNAUSEA & VOMITING-Serotonin- Receptor Antagonists before chemo -scheduled instead of PRN - Ondansetron (Zofran)- Avoid foods with strong odors- Psychogenic Vomiting - Conditioned response for developing nausea and or vomitingTUMOR LYSISBreakdown of malignant cells (ALL patients)- Release of intracellular components into blood- Hyperuricemia- Hyperkalemia- Hyperphosphatemia- Secondary Hypocalcemia- May result in cardiac arrhythmia, renal failure or acidosis- Seen most commonly in ALL and non-Hodgkin’s lymphoma patientsTREATMENT/MONITORING- Vigorous hydration 2-4 times maintenance- Strict I & O, daily weight, HYDRATION!- Urine specific gravity- Monitor Urine pH to determine what crap spills into blood- Mental Status ................
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