Nursing care of the child with hemotalogic disorder

• Consists of blood and blood forming tissues of body (bone marrow tissue form blood cells)
• - three cell categories
•     * Erythocytes or red blood cells (RBCs)
•     * Leukocytes or white blood cell: type neutrophils, lymphocytes, monocytes, eosinophils, basophils

All blood cells originate from a single type of cell called a multipotent hematopoietic stem cell

• results from different causes:
• Nutritional deficiency: malabsorption of nutrients (vitamins: b12, folic acid, other iron)
• Injury: trauma (hemorrahage), ingestion/exposure of toxins, radiation or drug exposure
• Infections: can lead to autoimmune (immune system attack itself) response idiopathic thrombocytopenia purpura (clotting disorder)
• genetics: sickle cell, hemophilia thalassemia
• Chronic bleeding: GI, GU, heavy menses

• RBC production: begins at 8 weeks gestation, primarily from the liver but when the baby is born the kidneys take over
• - Erythopoieton (EPO):
•   * Derived from liver in fetus and kidney after birth
•   * Hormone that regulates RBC production
•   * chemotherapy can effect this production or is someone has kidney problems. can cause anemia 
• - Hemoglobin
• * three types: Hgb A, hgb F (fetal), Hgb A2
• * Hgb F is found in the fetus and over time it converts to hgb A
• * hgb A is the predominate type (after 6m of age)
• * Hbg F is protective for some people esp with sickle cell helps cells to not sickle
• * hgb A2- abnormal??
• mom has transferred iron to the baby so baby has a good supply but after 6m if baby not getting enough from food then they can have physiological anemia

• Fetus: iron received from the placenta
• Preterm: at risk for anemia as the missed final weeks or months of transplacental iron transfer from mother (nurse- make sure mom is getting enough iron)
• Term: physiologic anemia occurs between 2-6 mons. iron stores depleted about 4-6 of life
• Sufficient iron intake is critical:
• breast milk or iron fortified formula (iron rich foods)
• Possible iron supplements
• Adolescents: need iron due to rapid growth, menses, they dont eat very well
• if baby ends up with anemia assess how mom is feeding. make sure mom isn't watering milk down found mostly in poor families

• Require a physicians order:
• Blood transfusions
• - PRBCs Packed red blood cells (admin for severe anemia to get the blood up also sickle cell thalessmia)
• - Whole blood: acute hemmorhage nothing wrong with they way the manufacture blood it is just they had a terrible injury
• - Fresh frozen plasma: given to hemaphiliacs to help with clotting factors that these people are difficient in
• Supplement oxygen: bc hypoxia O2 stat low 
• Splenectomy: bc spleen enlarged and isn't working as well (kids with sickle cell, hemophillia)
• Bone marrow transplant: sickle cell, aplastic anemia, thalssemia 

• Iron supplements (ferrous sulfate): iron deficiency anemia (IDA)
• Deferoxamine: Iron toxicity (chelating agent)
• Chelation: that this medication will bind itself to a metal in the body and excrete it thru the kidneys)
• some people have multiple transfusions and this is how they get too much iron in the body.
• Succimer: chelating agents- binds to lead used for blood lead levels > 45 mcg/ml
• when u give these chelating agents monitor I&O
• Factor (VIII/IX) replacement: focusing more on factor VIII bc it is the clotting factor that is necessary to form a clot. hemophillacs are deficient in this clotting factor. replacement so they don't bleed
• Penicillin VK: used as infection prophylaxis. kids with sickle cell are prone to infection. kids will get this for the 1st 5-6 yrs of life. PO everyday
• folic acid: folic acid deficiency/some kids with sickle cell
• Hydroxyurea: sickle cell pt helps to stimulate and promote hgb F production. helps to reduce the incidence of sickle cell 

• iron supplements: 
• - no milk or milk products
• -give 1 hr before or 2 hr a meal better absorbed on an empty stomach
• - liquid iron: rinse mouth can stain teeth (use straw behind teeth, mix in juice)
• - stool/urine turn dark colors, dark green/black
• - give with Vitamin C to help absorb
• - constipation: fluid/fibers
• Deferoxamine: rotate site of injection given sc. sometimes they may have some reaction when u give this so put hydrocortizone on it
• Factor VIII/IX: 
• - glass vial. u have to use filter needle
• - admin IV: when pt is experiencing bleed
• - Admin at home or ER: or given prophylactically to help bleed not to happen
• Penicillian VK:
• - check for allergies
• - monitor renal and hematologic function
• Folic acid supplements: admin anytime no meal required
• - encourage foods high folic acid: asparus, brussel sprouts, spinach, dark leafy green

• Always begins with Hx
• chief concerns: kids are fatigue, platelet disorder u may have bruising, nose bleed
• Preterm baby? was Vitamin K given at birth? nutrition- picky eater?, Fhx genetics, taking in too much milk, pica (non food substances)
• ice cubes: if anemic
• Obese infant/fatigue- IDA, not much milk and not enough iron
• eye: retinal hemorrahage/yellow eye jaundice- sickle
• frontal/maxillary bone boss (enlarged) bc bone marrow is overworking and it causes changes in the bone
• mouth: mucus membrane- anemia it will be pale. bleeding gum: anemia and coagulation problems
• petechia: little red spots..they don't blanch
• echymosis:
• abdomen: maybe tenderness liver enlargements
• extremities: nails spooning/clubbing bc of lack of oxygen
• iron def weak muscles 

• Fatigue: decrease oxygen supplement, cluster care as a nurse, observe activity
• Pain: sickle cell, hemophilic, use approa pain scale
• Impaired physical mobility: sickle cell, hemophilliac, encourage mobility, manage pain
• ineffective health maintenance: education about iron rich food, cow milk kids should be taken more than 24oz/day. vitamin supple  
• anxiety: confusion bc of decr oxygenation to the brain 
• ineffective family coping: needs support
• risk for injury: aviod rectal temps, perforated rectum, becareful 

• Complete blood count (CBC): test the following
• RBC total count: if u have too much it is called polycethemia, erythrocytosis- if too much blood gets to thick puts u at risk for clotting. 
• sometimes if a person is dehydrated can look like they have this. just give fluids
•   - hbg: hem and globin in the blood
•   - hematocrit: indirect measure RBC
• both use together to evaluate anemia 
• RBC indices:
• - MCV: size of the RBC (microcystic cell if anemia, if deficient in B12, folic acid, macrocystic cell)
• - MCH: O2 carrying capacity of HGB
• - MCHC: concentration of hgb in the RBC
• - RDW: width of RBC (when bone marrow is making more blood cells u will see variability with different sizes)
• WBC total count: fight infection
• - neutrophils: fight bacterial infection
• -lymphocytes: fight viral infection
• - monocytes: kids with mono, eusophil: allergic/parasitic 
• PLT count: 150-450 normal range clot
• differential determines types and numbers of each WBC in 100 

• RBC value
• 2-6mon 5.6
• 6-1yr 5.2 drops might be them going into physiological anemia

• Blood type and cross match
• - determine ABO blood type and antigens
• - used for trauma or a person with suspected blood loss
• - nurse: only good for 48-72hrs expired
• Clotting studies
• - PT, INR, PTT all done for clotting to make sure person is clotting 
• Coagulating factor concentration
• - measure concentration of coagulating factors
• - indicated for: hemophillia, DJC
• Hemoglobin electrophoresis
• - measures normal and abnormal hemoglobin in blood
• - indicated for: confirms sickle cell anemia, thalassemia 

• iron
• - evaluates iron metabolism
• - indicated for: IDA, hemosiderosis, hemoglodbinpathies

• lead: 
• - lead in blood
• - screen or confirm lead poisoning

• Reticulocyte count
• - measures reticulocytes (immature RBCs)
• - indicate bone marrow function
• - use this to see if the iron therapy are working: a week 5-8 days u will see these cells increasing (incre in count)

• Serum ferratin
• - measures ferratin level (major iron storage protein)
• - most sensitive test for diagonosing iron deficiency anemia

• Iron Deficiency
• Lead Poisoning

• Anemia: level of RBC's and Hgb are lower than normal
• Causes:
• - nutrional deficiency
• - RBC structure
• - kids living in high altitude
• - other chronic condition cystic fribrosis
• - RBC structure is altered 
• - bone marrow malfunction
• - exposure to toxin
• - adverse reaction to medication
• - trauma or surgery
• - increased rbc dustruction- hemolysis breaking down really fast
• In children (pediatrics) the most commons cause is nutritional deficiency

• classification: according to change in number of RBC's configuration or cause
• s/s: symptoms are observed when hgb <7-8 g/100ml
• - baby normal: birth- 2wks 14.5-24.5
• - 1-6yr: 9.5-14.1
• - think about it we won't see symptoms until it drops way lower than it is suppose to be. overtime child will get paler
• Normocytic (normal size cell), normochromic (normal color) anemia
• - this type means decr production of bone marrow due to hemorrahage blood loss 
• Microcytic, hypchromic anemia: small pale rbc's due to stunted hgb
• - r/t iron deficiency anemia, lead poisoning 
• Macrocytuc anemia: rbc's abnormally large, immature
• - megaloblastic anemia's, folic acid, or vita b12 deficiency

• cause:
• - low iron diet (6-15ml needed a day)
• - diet too rich in milk or dairy
• - malabsorption (GI disease ie celiac disease)
• - lead poisoning
• - heavy menstruation
• Symptoms:
• - pallor/pale mucus membrane
• - poor muscle tone
• - decre activity/irritable
• - heart murmur- harder for the heart to pump blood due to the lack of oxygen
• - splenomegaly?
• - spoon shape/clubbing fingernails
• - pica
• Diagnostic lab
• Decreased: 
• - hgb and hct- hgb < 11, hct< 33
• - reticulocyte count
• - serum iron and ferratin levels
• - microcytic, hypochromic RBC's 
•  

• Hx/dietary hx: PMH, FHX, diet incr milk consumption, picky eater, 24hr diet hx
• Physical examination:
• - neuro: fatigue, lethargy, irritable
• - skin: pale skin, conjuctibae, oral mucosa, palms and soles Nails: spooning
• CV: tachycardia, or murmur cardiomegaly (enlarge heart)
• Abd: splenomegaly (enlarged spleen)
• MSK: poor muscle tone, activity decre
• Infants: may show muscle tome and reduced activity. AAP recommends screening at 9-12 mons of age. if child is high risk u can start 6m

• therapeutic management: PO iron supplements. dose depends on severity of iron deficiency 1-6 mg/kg/day
• severe cases: blood transfusion
• Reticult count will increase

• Nursing management:
• - Patient/fam education
• - Iron supplement admin
•    - correct measurement
•    - do not give w/milk or milk product
•    - admin behind teeth, with straw, dilute juice, rinse mouth
•    - control constipation: fiber, inc fluids
• Dietary intervention:
• - consume food high in iron
• - vitamin c

• For iron deficient infants:
• - breast fed: start iron supplements at 4-5 (bc of physiological anemia). encourage moms to increase iron rich foods in diet/supplements
• - formula fed- use only iron fortied formula/cereals

• For children over 1 year of age:
• - limit cow's milk to 24oz/day
• - limit fast food consumption
• - encourage iron rich foods. provide variety of foods

refer to wic if family is struggling

• red meats- most easiest to absorb
• tuna and salmon
• eggs
• tofu
• enriched grains
• dried beans, peas, fruits
• leafy green vegatables
• iron-fortified breakfast cereals

• Definition: increased level of lead (heavy metal) in the body (>5 ug/dl). due to chronic inhalation or ingestion of lead (can inhale this from dust in home if doing renovation) containing materials
• - results in hypochromic, microcystic anemia
• Complications: behavioral and learning difficulties, cognitive impairments, possible seizures, and brain damage
• treatment: for > 45 mg/dl Chelation therapy med succimer other than this get rid of exposure
• Symptoms: abd pain, headache, pallor (bc of anemia), irrability,
• severe cases: seizures, coma, death 

• Age: 1-5 infants and young children
• living in an older home: lead paint banned since 1978
• hobbies: use of lead solder, refinishing old funiture
• Country of origin: developing countries at high risk (adopted children from other country)

• Paint: homes built before 1978, dust from windowsill, walls, and plaster in older homes
• Pica or eating non food items
• soil: leaded gas or car battery recycling
• glazed pottery and stained glass products
• lead pipes sypplying water to the home
• on the clothing: of certain parents/caregivers that work in certain manufacturer jobs
• Folk remedies: greta and arazon (spanish meds fine red power taken for upset stomach and teething babies) need to find these out in hx
• old painted toys or funiture

• History: source of lead, inhaled or ingested, pica
• PE: Sx anemia, pallor, abd pain, vomiting, constipation, anorexia, headache, fever, lethargy
• CNS sign:
• - hyperactivity, impulsiveness, irrability, loss of developmental progress, hearing impairment, learning difficulties 

• well check ups:
• - start universal screening 9-12m 6m if high risk
• - ensure high risk children have blood lead levels measured
• Labs:
• - finger stick, confirm with venous blood sample
• - CBC, chemistry profile
• - radiology: abdominal x-ray, head CT or MRI
• lead will land in the bone

• Education and prevention is key
• screen for risk factos
• ensure lead is being removed from child's home
• administer chelating agents:
• - monitor I&O bc of kidneys
• - hydrate: PO or IV
• - Monitor VS
• - Have epinephrine available (just in case they go into a anaphylatic reaction
• If they have lead: Referral early intervention or further evaluation

start monitoring them after 5 anything more than 45 chelating agent

• Bone dysfunction
• def: bone marrow fails to produce blood cells. Pancytopenia: reduce RBC, WBC, platelets
• at risk for alot of different problems ie anemia, infection, bleeding

different types ie congential

• Acquired aplastic anemia: excessive exposure to radiation, drugs, or chemicals that are known to cause bone marrow damage. most causes unknown
• classified as severe or non severe
• Drugs: chloramphenicoL (lots of lawsuits), sulfonamides (to treat uti), arsenic, hydantoin, benezne, quinine, felbamate
• other: radiation, inspecticide, chemotherapy drugs and serious infections(menigococcal pneumonia)

• Hx: exposure to drugs or radiation therapy
• FHx: enviroment, infectious disease hx
• PE: pallor, fatigue, anorexia, easy bruising, petechiae, frequent nose bleeds, heavy menses, gi bleeding, incr infection
• CV: incr HR, incr RR, SOB, cyanosis

• comes on quickly 
• Therapeutic management: bone marrow transplant, PRBC's, and platelet transfusion, EPO (to help stimulate bone marrow to produce cell), corticosteroids 
• Note: bone marrow transplant offers possible complete recovery 
• any drug/chemical suspected of causing the bone marrow dysfunction be discontined at one and the child must never be expose to it again
• offer parental support

• safety: prevent injury, avoid hemorrhage, prevent re exposure to offending substance
• prevent infection
• stool softners: strain, develop rectal fissure which they can bleed 
• PRBC, or platelet transfusions: administer only irradiated and leukocyte depleted units bc u don't want any reaction, if they are having a bone marrow transplant they cant have any of these antibodies in their blood
• refer families:
• aplastic anemia and myelodysplastic syndrome international foundation