Nx care of a child with hematologic disorder 2

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Nx care of a child with hematologic disorder 2
2015-01-31 19:36:37

part 2
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  1. Anemia: Nutritional deficiency
    Megaloblastic anemia
    • folic acid deficiency
    • Pernicious anemia/vit b deficiency
  2. megaloblastic anemia
    • def: anemia with larger than normal RBC's. Uncommon in children (mostly seen in foriegn countries bc we get a source of folic acid)
    • causes: nutritional deficiency- folic acid and vit b12
    • S/s: vary due to deficiency
    • change in skin color, pallor, diarrhea, headaches (not enough o2), anorexia, sore mouth or tongue, beef red smooth tongue (VB12 def), tingling numbness of hands or feet, weak muscles, fatigue
    • complications: mild CHF (uncommon)
    • Dx: labs: decr VB12, folic acid def= neutropenia, thrombocytopenia, incr MCV, and MCH normal MCHC
  3. Megaloblastic anemia
    Folic Acid deficiency
    • Folic acid def combined with  Vit C def= large RBC's, neutropenia, and thrombocytopenia, 
    • - MCV and MCH increas
    • - MCHC normal
    • - incre megaloblast in bone marrow (immature cell big) 
    • megaloblast arrest occurs in 1st yr of life from continued use of infant food with decre FA
    • s/s sores or mouth and tongue, poor growth
    • treatment: FA supplements, incre foods with folic acid in it
  4. folic acid foods
    • green leafy veggies spinach
    • citrus oranges, grapefruit
    • beans
    • beets, carrots, avocado
    • sunflowers seeds
    • almonds
    • diet that has alot of colors
  5. megaloblastic anemia
    Pernicious anemia (vit b12 deficiency)
    • causes:
    • - deficient or unable to use VB12
    • - lack or deficient of intrinsic factor in gastric mucosa (if we don't have intrinsic factor in our gut then we can't use VB12)
    • Dietary: source: food of animal orgin, beef, shellfish, eggs, cheese, milk
    • cow milk, breast milk
    • vegetarian at incr risk
    • s/s: occurs in first 2 years of life, pale, anorexia, chronic diarrhea, smooth beefy red tongue, ataxia, hyporeflexia, parathesis
    • labs: low serum vb12
    • treatment: for deficient diet- give injection
    • for lack of intrinsic factor give lifelong every month IM injection
  6. Hemoglobinopathies
    • sickle cell anemia
    • thalassemia 
    • RBC destroy within 10-20 days 
    • problem with hemoglobin in rbc
  7. Increase RBC destruction: 
    • hemoglobinopathy: a condition in wihch abnormal hemoglobin is present (problem with hemoglobin)
    • Normal newborn Hgb have hgb f and this type doesn't sickle
    • then over the next few months (6) this changes over to hgb s which becomes dominant 
    • but in these babies it turns into hgb S
    • cause: genetic
    • sickle cell disease
    • beta thalassemia cooley's anemia
    • alpha thalessemia (don't need to know)
  8. Sickle cell disease
    • two parents with the trait can pass this to their children. 50% of getting the trait. 25% of getting the disease
    • sickle cell disease: a group of inherited hemoglobinopathies in which the RBC's do not carry the normal adult hemoglobins
    • In US most common types: Hgb SS disease (sickle cell anemia), hemoglobin SC (not quite as sick) and hemoglobin sickle beta thalassemia 
    • sickle cell: severe chronic blood disorder. affects 2000 infants born in US annually
    • does not go away but we can manage the symptoms 
    • - most commons in Africans, mediterranean, middle eastern, and indian descent
    • - found in 1:500 african american 
    • Characterized by: RBC hemolysis, vasooculusive crisis and increase risk bacterial infection 
    • RBC breakdown and they go in the spleen (immune system) your spleen gets backed up bc the breakdown is happening too fast..so that puts u at risk for infection
  9. SCD what happens?
    • sickle cell recessive gene is passed from both parents who have sickle gene or trait
    • sickle cell contains abnormal Hgb SS 
    • membranes of Hgb s are fragile and easily destroyed. changed into crescent shape
    • - crescent shape cant easily pass thru small vessels and they clump
    • - results in thrombosis (clot) occuldes vessels causing infarcts or areas of dead tissue (distal to clot)
    • common sites of infarcts:
    • - spleen, brain (stroke), heart, lungs (oxy ex), gi tract, kidneys, bones/joints (pain)
    • infants: asymptomatic until 4-6m of age bc of protective hgb F
  10. Sickle Cell disease cont
    • Complications:
    • - recurrent vaso-occulusive crisis pain
    • -stroke
    • - sepsis (at risk for infection)
    • - acute chest syndrome (clots in lungs block off blood vessels in lungs SOB)
    • - spelnic splenomegaly and abd pain
    • - low visual acuity
    • - leg ulcers
    • - gallstones
    • - decre G&D
    • - decre puberty
    • - priaprism (prolong erection)
    • - enuresis bed wetting
    • - multi-organ dysfunction
    • - dactylitis- swollen hands/feet

    • Vaso-occlusive Crisis triggers:
    • - stress/traumatic events
    • - infection
    • - fever
    • - acidosis
    • - dehydration
    • - physical exertion
    • - excessive cold/hot
    • - hypoxia (not enough oxygen which can cause triggers)
  11. SCD Hemoglobin AS (just the trait)
    • carriers for this disorder
    • 1:12 african americans have trait
    • minimal health problems
    • does not develop into sickle cell disease
    • no treatment necessary
    • genetic counseling important
    • if this person is under stress they can possible get symptoms but not as severe.
  12. Assessing child in Sickle cell crisis
    • Labs: hgb labs low 6-8
    • Cells: look under microscope u will see sickle
    • WBC: incres 12,000-20,000
    • Bilirubin: increa
    • retic count: increase
    • trigger factor: fever from infection
    • could have a yellow scelera bc of hemolysis (too fast), stroke, seizure, could die, assess spleen, liver, backpain (kidney problems), abd pain and tenderness), acute cough and chest pain 
    • always identify where the pain is so we can start intervention on this.
  13. SCD nursing assessment
    • HX: G&D, frequency of vaso-occulusive crisis, how often are u having these crisis, hospitalization, treatment of pain crisis, immunizations, blood transfusion in past, current medications, current/recurrent infections or illness
    • HPI: of current illness that resulted in crisises, pain assessment
    • PE H- toe assessment, vital signs
    • - inspect: sclera, conjuctivae, skin, palms, soles, oral mucosa, baseline neurologic function, priaprism (jaundice, lesion, ulcers, pallor)
    • Auscultate heart, lungs (acute chest syndrome murmur, respiratory infection)
    • Palpate joints and abdomen
    • (hepatosplenomegaly, tenderness)
  14. SCD nursing assess. continues
    • Lab and Dx testing-
    • - newborn screenings: sickledex or sickle cell prep
    • - hgb electrophoresis: confirms diagnosis
    • - hgb baseline 7-10 mg/dl & decre with crisis
    • - reticulocyte count incre
    • - peripheral blood smear: will show sickle cell
    • - Erythocyte sedimentation rate: incre
    • - LFT: abnormal (incre bilirubin)
    • - x-rays/or other scans to determine organ or tissue damage
  15. Vaso occuslive crisis how to manage
    • HOP
    • Hydration- IV
    • Oxygenate 
    • Pain management
    • prevent complication
    • administer a blood transfusion
    • bone marrow transplant
  16. SCD nursing management
    • Goal: prevent vaso-occulsive
    • educate: on their level, genetics, disease process, prophylactic antibiotics, seek medical advice for any febrile illness, complications
    • Managing a pain crisis:
    • - use stanardized pain scale, provide frequent pain assessment
    • - pain medication:
    • * moderate to severe pain= opiods's provide around the clock then change to prn
    • * Patient controlled analgesia (PCA)
    • * NSAID's and acetaminophen
    • non pharm measures: relaxation, hypnosis, music, massage, play, guided imagery, therapeutic touch
  17. SCD management
    • Prevent infection"
    • - prophylactic PO penicillian VK:
    • * start by 2 months contin to age 5 yrs
    • * if allergic then erythromycin
    • Immunization: 
    • * routine childhood vaccine
    • * 23 valent pneumoccal annual (at risk for overwhelming pneumoccal sepsis)
    • * menigoccal vaccine
    • * influenza yrly after 6m
    • * so they get all vaccine plus additional ones
  18. SCD teaching guidelines
    • hydrate everyday substance
    • avoid extreme temps
    • do not over exert yourself
    • assess 24hr to medical facility
    • seeks medical attention immediately:
    • febrile
    • pale and listless
    • cough, SOB, chest pain (acute chest syndrome)
    • increasing fatigue
    • unusual headache, loss of feeling, sudden weakens (stroke)
    • sudden vision changes
    • painful erection that wont go away
  19. Increased RBC destruction: Thalassemia
    • Def: genetic disorder in which one of the two proteins that make up hemoglobin in red blood cells is deficient (if we don't have enough hgb we cant make enough rbc)
    • - involves problem with alpha or beta chain of hemoglobin
    • - pt's body cant produce sufficient adult hgb A
    • two main forms alpha and beta
    • - meterrian heritage
    • - african and asian decent
  20. Beta- Thalassemia: overview
    • RBC's are abnormal in shape and are destroyed rapidly= severe chronic hemolytic anemia and chronic hypoxia (rbc breaking apart and cant carry enough oxygen)
    • 3 categories:
    • Thalessemia minor (trait): mild microcytic anemia, no treatment
    • Thalessemia intermedia: requires blood transfusion to maintain quality of life
    • Thalessemia major (cooley's anemia): severe form: to survive need ongoing medical attention, regular transfusions, and iron removal (chelation
  21. Beta Thalassemia (cooley's anemia) Nursing assessment
    • Hx:
    • healthy infant at birth
    • between 6-12 months of age the infant, develops severe anemia, bone abnormalities, Failure to thrive, frequent infections, bleeding tendancies, and life threating complications
    • PE: starts to exhibit pallor, jaundice around 3-6m 
    • hepatosplenomegaly (too many red cells breaking apart getting stuck in spleen) with abdominal enlargement, bruising, anorexia
    • small body with large head/frontal bossing and maxillary prominence, possible mental retardation
    • features similar to down syndrome
    • Bone marrow is trying to make RBC but it can't so it is over working. Bone marrow is found in the flat bones (frontal and maxillary) will grow. large head and mouth
    • Liver store excess iron so it gets enlarged
  22. Anemia Beta Thalassemia (cooley's anemia) 
    • No treatment for minor forms
    • Transfusion: regular schedule transfusion PRBC
    • Absolutely no iron supplements: contraindicated in all forms. no iron rich foods
    • drug therapy: deferoxamine (desferal) for chelation therapy- treats hemosiderosis (iron toxicity)
    • Surgery: splenctomy or bone marrow transplant (hematopoietic stem cell transplant)
    • avoid strenuous activity 
    • Complication: iron overload (hemosiderosis), cardiac arrhythmias, liver, and or heart failure frequent infection, death
  23. beta thalassemia (cooley's anemia)
    nursing management
    • nursing management: support family, minimize effects of illness, educate family, administer blood transfusion
    • Blood transfusions: follow hosp policy
    • - given to maintain adequate level of hgb for O2 delivery and suppose erythrocytosis of the bone marrow
    • - monitor for transfusion reactions
    • administer chelating agent PO or SC
    • Educate family: adherence to blood transfusion and chelation therapy regimen (essential for survival)
    • referral for genetic counseling
  24. Blood administration
    • Determines client:
    • - allergies
    • - previous transfusion and reaction to transfusion
    • Administer immediately after receiving from blood bank
    • - cross match last 48-72hr
    • Check cross match record with 2 nurses: abo group, Rh type, clients name, hosp #, expiration
    • Do not warm up unless hypothermic response- if then use a special warming equipment
    • Infuse each unit over 3-4 hr but no longer than 4 hrs
    • key points:
    • check MD's order
    • check labels on blood bag and client's id
    • 18 gauge needle 
    • normal saline
    • baseline VS before infuse: check VS q 15 mins and than 30 mins
    • severe reactions most likely first 15min and 1st ml
    • blood administration set with filter
    • change blood tubing and filter with each unit
  25. Transfusion reactions
    • Mild:
    • Facial flushing, hives, rash
    • Severe: incre anxiety, wheezing dyspnea, decre BP
    • Allergic: headache, tachycardia, tachyapnea
    • Febrile: Fever, chills, anxiety
    • Hemolytic: hemoglobulinuria, chest pain, apprehension, low back pain, chills, fever, tachycardia, decr bp, incre RR
  26. Clotting disorder:
    • hemophilia
    • idiopathic thrombocytopenic purpura
  27. Clotting disorder: overview
    • clotting normally occurs after injury
    • our clotting system requires FACTORS in the blood and platelets to perform adequately
    • individuals with deficiences of factos and platelets tend to bleed, because it is more difficult for clot to form
    • factors involved in problems with clotting include: factor VIII, IX, X1
    • platelets also have a role in clotting cascade and clot formation
    • Bleeding times prolong in clotting disorder
  28. Labs done to check clotting time
    • PT, PTT, INR 
    • how long it takes for them to clot
  29. Clotting disorder: hemophilia
    • def: hereditary group of x-linked recessive bleeding disorder
    • x- comes from mom...mom passes this to her sons
    • cause: deficiency of clotting factor
    • Types:
    • - hemophilia A deficient in factor VIII
    • - hemophilia B (christmas disease) deficient factor IX
  30. hemophila: Therapeutic Management
    • Goal: prevent bleeding!!
    • Avoid contact sports or activities with incr risk of injury
    • encourage low contact sports: swimming, running, tennis
    • bleeding episode:
    • - administer factor at home or in outpt facility (with slow IV push)
    • Administer factor replacement
    • - prior surgery or other procedures
    • - blood transfusion, if necessary
    • - before dental procedure
  31. hemophilia: nursing assessment
    • health Hx: bleeding or hemorrhagic episode length of bleeding (how long did it take bleeding to stop)
    • Physical examination: focus on bleeding/injury severity
    • - assess CV status, ID chest pain, abdominal pain
    • - report abnormal finding immediately
    • - laboratory and Dx testing: decre HGB HCT, factor deficiencies, prolonged PTT, normal plt count, normal bleeding time and PT
  32. hemophilia nursing management
    • Preventing injury and bleeding episode:
    • - encourage regular excerise: keep muscle and joints strong= fewer bleeding episode
    • - refer severe hemophilia to pediatric hematologist or hemophilia treatment center
    • Managing a bleeding episode:
    • - admin factor VIII replacement slow IV push
    • -document: product name, number of units, lot number, and expiration date
    • (dose base on severity and weight of child)
    • if external bleed apply pressure
    • if joint: apply ice or cold compresses, elevate extremity
    • all bleeding cases should have follow up wih HCP
  33. Teaching guildelines: preventing bleeding in the child with hemophilia
    • protect toddlers with soft helmets, padding on the knees, carpet in the home and softened or covered corners
    • children should stay active: encourage low contact sports
    • avoid contact sports, trampoline ATV
    • for oral surgery: premedicate with amicar (inhibits fibrinolysis) 
  34. hemophilia education
    • health alert bracelet
    • family works closely with school nurse
    • teach family and friends to give IV factor VIII at home with VNA or at local er for bleeding episode
    • involve children in their care
    • provide support:
    • offer resources- different foundations 
  35. Clotting disorders: ITP
    • Idiopathic thrombocytopenia purpura:
    • Def: acquired hemorrhagic disorder, results from immune response following viral infection that produce antiplatelet antibodies. Destroys platelets. leads to development of petechiae, purpura or excessive bleeding
    • complications: severe hemorrhage/bleeding rare
    • Nursing assessment: see nextslide for complete list s/s
    • - hx: healthy child with recent viral infection recent MMR vaccine, or meds taken that cause can thrombocytopenia
    • - labs: CBC platelet count less than 50,000 (normal 150,000-400,000)
    • Nursing management: possibly no treatment ordered
    • - avoid asprin, NSAID's and antihistamine. use acetaminophen for pain
    • prevent trauma, educate on s/s of bleeding
  36. Idiopathic thrombocytopenia purpura s/s
    • petecheia: pinpoint hemorrhage occurring on the body, non blanch with pressure
    •  purpura: larger purplish aread of hemorrhage in which blood collects 
    • epixtaxis
    • oral bleeding
    • blood in stool or urine
    • excessive menstrual bleeding