PEDS: HEME/IMMUNE

high risk groups for developing iron deficiency anemia:

- premature - infants/children with excessive milk intake (milk is bad for Fe absorption) - adolescents (growth spurts) - pregnant females - low-income children

interventions for an iron deficient patient include:

- rest - iron foods - oral supplements - vitamin c - restrict cow's milk

A nurse is educating a group of teenage girls about dietary sources of iron. Which statement by one of the teenagers indicates to the nurse the need for more instruction? a. "Fish is a good source of iron." b. "We need 8 mg of iron each day." c. "We need more iron during pregnancy." d. "Iron can be taken with 6 oz of orange juice."

b, adolescents need 18 mg

type of hemophilia: - most common - factor 8 deficiency & VW factor deficiency - autosomal dominant (can inherit 1 parent) - bleeding from skin & mucous membranes

von willebrand disease

patients with iron deficiency anemia present with:

- irritability - paleness - edema - infections - low albumin, serum iron, and decreased hbg hct

common symptoms of anemia:

- jaundice (RBC lysis) - pale, cool - SOB - weak - change of stool color - low BP, high HR - heart palpitations - spleen enlargement (collecting damaged RBCs)

what are common stressors for sickle cell anemia?

- low O2 - infection - dehydration - cold weather

What are the 4 symptoms of anemia, regardless of cause?

- pallor - tachycardia - fatigue - weakness

what are management interventions of a sickle cell crisis?

- bedrest - hydration - pain meds (morphine, dilaudid) - PCN/antibiotics - transfusions - splenectomy

what are the outcomes of thalassemia?

- bone marrow hyperplasia (enlarged bones) - hemolysis (anemia, hemosiderosis, hemochromatosis) - organ failure (from high Fe levels)

what foods would you give to an iron deficiency anemic patient?

- chicken - pork - egg yolk - shrimp - liver - beef - clams - broccoli - potatoes with skin - Fe fortified cereal - dried apricot - dried beans/green peas - spinach - raisins - watermelon

what are the major consequences of crises?

- damage to multiple organs - compromised immunity

list severe symptoms of anemia:

- fainting - chest pain (angina) - heart attack

at 6-12yrs., the child's hgb count is: (g/dl)

11.5-15.5

at 1-3 days, the infant's hgb count is: (g/dl)

14.5-22.5

what are the (3) causes of anemia?

1. decreased RBC production 2. increased RBC loss 3. increased RBC destruction

If both parents have the sickle cell trait (autosomal recessive), then what percentage does the child have of getting the disease?

25%

What should the plan of care include to minimize the potential for a sickling episode in a child with sickle cell anemia? 1 Providing an iron-rich diet 2 Ensuring hemoconcentration 3 Enforcing periods of quiet play 4 Promoting adequate oxygenation

4

screening for iron deficiency anemia is recommended at ______ months, ______ months, & in adolescence

9-12, 15-18

A 3-year-old child is admitted to the pediatric unit with a hemoglobin level of 6.4 g/dL (64 mmol/L). Which would the nurse's priority assessment be? A. manifestations of shock B. increased white blood cell count (WBC) C. presence of hemoglobinuria D. signs of cardiac decompensation

D

what is a common cause of hospitalization of a child with sickle cell disease?

acute chest syndrome

A child with sickle cell disease has a sequestration crisis. The parents ask how it differs from a painful episode (vaso-occlusive crisis)? Which answer explains the sequestration crisis? A. there is peripheral ischemia along with the pain. B. there is decreased blood volume and signs of shock. C. red blood cell production diminishes with severe anemia. D. red blood cell destruction is accelerated and jaundice appears

B

Which of the following directions would NOT be recommended for a parent giving iron supplements to their child? a. monitor for signs of constipation. increase use of fiber or stool softeners b. have child take medication at dinner time so they have a full stomach to increase absorption c. have child drink liquid supplement with a straw to prevent staining teeth d. do not give at the same time as calcium or antacid tablets

B

identify the hematology system lab: - # of RBCs - # of WBCs - concentration of hgb & hematocrit - # of platelets - size/shape of RBCs

CBC

what anemia is described as - pancytopenia (anemia, leukopenia, thrombocytopenia) - the bone marrow fails to produce blood cells - acquired autoimmune suppression of blood cell production - hereditary - Fanconi's anemia (rare)

aplastic anemia

what type of SCA crisis is described as: - decreased erythropoiesis - causes bone marrow inability to produce cells

aplastic anemia crisis

A child with beta-thalassemia (Cooley anemia) is admitted to the ambulatory care unit for a transfusion. What instruction should the nurse include in the plan of care? a. encourage fluids b. restrict activities c. protect from infections d. offer small meals frequently

b

which blood disorder has the highest incidence in people from the Mediterranean, Arabian peninsula, & southeast Asia?

thalassemia

What does it mean if a patient has heterozygous sickle cell anemia (SCD)?

they inherit the trait from 1 parent

T/F: anemia is a symptom, not a disease

true

T/F: clotting (bleeding) disorders can be either genetic or acquired

true

what live vaccines need to be held for a child with HIV?

MMR, Varicella, LIAV, Rota

T/F: if an infant's mother has HIV, the infant will be treated as if they are positive until they are confirmed negative

true

what prophylactic treatment would you give to a patient from 2mon-5yrs. if at risk for sickle cell anemia?

PCN

are RBCs/erythrocytes or WBC/leukocytes an acid base buffer?

RBCs/erythrocytes

A 2-year-old child is admitted to the pediatric unit with a diagnosis of thalassemia major (Cooley anemia). The parents are told that there is no cure but the anemia can be treated with frequent blood transfusions. The father tells the nurse he is glad that there is a treatment that "fixes" his child's problem. Before responding, the nurse should recall that blood transfusions: a. correct the anemia, but they may also cause other problems b. reverse the anemia, but they also present a risk of hepatitis c. are a supportive treatment; fewer will be needed as the child grows older d. are a replacement for defective red blood cells (RBCs); they are like giving insulin to a person with diabetes

a

Which is the priority nursing intervention to prevent thrombus formation in a child with sickle cell anemia? a. encouraging fluids b. encouraging bed rest c. administering O2 d. administering prescribed anticoagulants

a

list examples of what to look for in a hematology assessment:

- family member hx - ht./wt./growth chart - nutrition assessment - developmental milestones - skin: pallor, flushing, rash, bruising, bleeding - joints: edema, pain, inflammation, ROM - hx of fatigue/lethargy - frequent infections

What is the difference between homozygous and heterozygous sickle cell anemia? Which one is more severe?

- homozygous is a carrier with symptoms while heterozygous is carrier without symptoms - homozygous

what are the clinical presentations for someone with aplastic anemia?

- increased bruising - increased infections - anemia - delayed growth & development - increased risk of leukemia

management of a pt. with aplastic anemia

- monitor for infection - immunosuppressive therapy - transfusions - bone marrow transfusions

for hemophilias (A, B, VW), what are interventions for management?

- replace missing factor - avoid NSAIDS - corticosteroids for bleeding - injury prevention - treat injuries quickly (elevate, compression, ice)

what are the clinical manifestations of thalassemia?

- small stature - delayed sexual maturation - bronzed, freckled skin (iron overload) - enlarged head - enlarged maxilla - flat nose bridge - generalized osteoporosis

what are the continuum's of disease in children with HIV?

- thrush - hematosplenomegaly - FTT - encaphalopathy - opportunistic infections (especially pneumonia)

what are nursing management interventions for thalassemia?

- transfusions to maintain hgb >10 - protect from infection (rx of sepsis) - long-term prophylactic antibiotics - vaccines - splenectomy

treatment interventions for a patient with immune thrombocytopenic purpura (ITP)

- tx if platelets < 20,000 - restricted activity - immunosuppression therapy - splenectomy if longer than 1 yr

A nurse in the pediatric clinic is assessing an 11-month-old infant with iron-deficiency anemia. The infant's hemoglobin is 8 g/dL (80 mmol/L). What does the nurse expect to observe when assessing the infant? 1 Pallor 2 Tremors 3 Cyanosis 4 Spasticity

1

A nurse is teaching the parents of a school-aged child with sickle cell anemia about ways to prevent sickling. What should the nurse explain as the primary cause of sickling? 1Hypoxia 2Hemodilution 3Hypocalcemia 4Hemoglobin

1

in a child, their platelet count reach adult levels at __________.

1 week

adult hgb norm (g/dl)

10

at what age is a splenectomy common at (due to splenic sequestration crisis & decreased immunity)

10

how many negative HIV tests do you need to confirm a negative HIV status of a newborn to an infected mother

2

Which intervention would reduce the risk of perinatal transmission via vaginal birth in an adolescent who is diagnosed with HIV infection? 1. Using forceps during delivery 2. Using a fetal scalp electrode during delivery 3. Using antiretroviral during the intrapartum period 4. Administering zidovudine an hour before labor

3

Zidovudine (AZT) is given to a pregnant mother who has HIV after the 1st trimester & during L&D. AZT is given to an infant for the 1st ____ weeks to reduce transmission rates

6

at what age do infants start to produce hemoglobin a? what hemoglobin are infants born with?

6 months, hemoglobin f

Infants are screened at ___ months for sickle cell anemia. This is because hgb ___ doesn't sickle

6, f

A nurse concludes that the teaching about sickle cell anemia has been understood when an adolescent with the disorder makes which statement? A: "I'll start to have symptoms when I drink less fluid." B: "I'll start to have symptoms when I have fewer platelets." C: "I'll start to have symptoms when I decrease the iron in my diet." D: "I'll start to have symptoms when I have fewer white blood cells.

A

identify the hematology system lab: - asses # of cells in CBC - assess % of types of WBCs

CBC with differential

what supplements/element interferes with Fe absorption? what facilitates iron absorption?

Calcium, vitamin c

increased RBC loss is caused by:

acute blood loss (hemophilia) or hemorrhage (trauma)

what type of SCA crisis is described as: - common reason for hospitalization - caused by pulmonary infiltrate of abnormal blood cells - often confused with pneumonia - use incentive spirometer to prevent

acute chest syndrome

Which is most important for the nurse to emphasize during discharge planning for a child after a sickle cell vasooclusive crisis? a. a high-calorie diet b. a rigorous exercise regimen c. an increased intake of fluids d. an increase in hours spent sleeping

c

A nurse is preparing to discharge a school-aged child who has undergone splenectomy for β-thalassemia (Cooley anemia). What information should the discharge teaching include? a. the child should avoid participation in contact sports b. the hematological problems have been cured by the splenectomy c. a high fever should be reported to the child's health care provider d. blood transfusions will be needed more frequently than before the splenectomy

c, an increased risk of sepsis

patients with sickle cell might produce hemoglobin ___ or hemoglobin ____

c, s

why would a nurse need to give cow's milk to a patient 1 hour before or 2 hours after giving an Fe supplement?

calcium interferes with iron absorption

a procedure in which excess metals, such as iron, are removed from the blood

chelation therapy

identify the hematology system lab: - assesses how well a pt. clots - higher values = bleeding risk - lower values = risk for clot (DVT, PE)

clotting indices (PT, reticulocyte count, aPTT)

- pallor - increased HR - fatigue - SOB - weakness - systolic murmur - PICA if a patient presented with these symptoms, what is causing their anemia?

decreased RBC production

how do you technically diagnose thalassemia?

electrophoresis

T/F: RBC, hemoglobin, hematocrit, & WBC lab norms do not vary by age

false

T/F: anemia is not the most common heme alteration in children

false

what causes thalassemia?

genes for Hgb are missing/different and fewer/smaller RBCs than normal

bleeding into a joint that can cause permanent joint damage - common in pt. with hemophilias - skin is taught and shinny - medical emergency

hematrhosis

type of hemophilia: - "classic hemophilia" - factor 8 deficiency - X-linked - deep tissue bleeding

hemophilia A

type of hemophilia: - "Christmas Disease" - factor 9 deficiency - X-linked - deep tissue bleeding

hemophilia B

bronzing, freckled skin from iron overload is called:

hemosiderosis

what kind of diet should a patient with HIV be on?

high calorie, nutrient dense

- most common bleeding disorder in children - usually after viral infection which causes an autoimmune response to destroy platelets - thrombocytopenia - purpura/petichiae - antibodies destroy platelets

idiopathic thrombocytopenia purpura (ITP)

what is the BEST way to prevent acute chest syndrome?

incentive spirometer

- pallor - increased HR - fatigue - dark urine - splenomegaly - hepatomegaly if a patient presented with these symptoms, what is causing their anemia?

increased RBC destruction

- pallor - increased HR - fatigue - weakness - cool skin - decreased peripheral pulses - low BP (late sign) if a patient presented with these symptoms, what is causing their anemia?

increased RBC loss

increased RBC destruction is caused by:

intracorpuscular (sickle cell), extracorpuscular (immune, toxin)

how would you assess a patient with dark complexion for anemia?

look for pallor under eyelids

Decreased RBC production is caused by:

nutritional deficits & bone marrow alterations

what 2 vaccines would be encouraged for a pt. with thalassemia?

pneumoccoccal, meningococcal

what do platelets/thrombocytes secrete to cause vasoconstriction?

serotonin

- genetic mutation that lacks both humoral and cellular immunity (no b or t-cells) - low lymphocyte count (infected easily) - more common in males - 1st infection - thrush - signs: FTT, chronic diarrhea

severe combined immunodeficiency disease (SCID)

how would you want a patient to take oral iron supplements (Poly-vi-sol w/Fe)

sip through a straw to avoid eruption

what type of SCA crisis is described as: - hepatic & splenic pooling of blood resulting in shock - high mortality rate

splenic sequestration crisis

what type of SCA crisis is described as: - obstruction by sickled cells - has greater RBC destruction - intense pain that can be constant or intermittent

vaso-occlusive crises (VOCs)