Hematologic Conditions

Question 1

Hematologic Overview

Answer 1

Hematologic conditions affect normal functioning of red blood cells and clotting. Assessment and nursing interventions are geared toward preventing and controlling the problems associated with the alteration of blood cells and promoting normal function.

Question 2

Blood composition

Composition and function of blood

Answer 2

Formed elements (cells):
* erythroctes (RBCs)
* thrombocytes (platelets or PLTs)
* five types of leukocytes (WBCs)
1. neutrophils (immature cells are “bands”, mature cells are “segs”)
2. lymphocytes
3. monocytes
4. eosinophils
5. basophils

1. All blood cells are formed in the bone marrow originating from the hematopoietic stem cell.
2. Early in utero, blood cells are made by the liver and psleen; these organs retain their ability to make blood cells throughout life.
3. Before birth, the bone marrow becomes the main producer of blood cells.

Question 3

Plasma

Composition and function of blood

Answer 3

1. Approximately 90% of plasma is composed of water.
2. Approximately 10% of plasma is solutes such as electrolytes, proteins, dissolved nutrients, clotting factors, anticoagulants, and antibodies.

Question 4

Functions of blood

Composition and function of blood

Answer 4

Blood cells and plasma play a major role in the following:
1. Oxygenation of body tissues
2. Cellular nutrition
3. Excretion via transport of wastes to other organs
4. Maintenance of acid-base balance
5. Regulation of body temperature
6. Defense against foreign antigens
7. Transport of hormones

Question 5

Red blood cell functioning: and overview

Composition and function of blood

Answer 5

1. RBCs carry oxygenated hemoglobin from the lungs to the tissues of the body and deoxygenated hemoglobin from the tissues to the lungs.
2. RBC production is stimulated by two mechanisms that stimulate the bone marrow:
   a. Tissue hypoxia
   b. Renal production of erythropoietin (which is stimulated by hypoxia)
   c. The driving factor of RBC release from the bone marrow is based on the tissues’ need for oxygen rather than the number of RBCs circulating.
3. Normal RBCs live 120 days.
   a. When an RBC dies or is destroyed (hemolysis), most of the iron is conserved.
   b. Unconjugated bilirubin (also called indirect bilirubin) is one of the byproducts of RBC hemolysis.
   c. Byproducts of the breakdown of RBCs include iron and bilirubin. Iron is reabsorbed and reused. Bilirubin, in its unconjugated form, is present in the systemic circulation for transport to the liver. In the liver it is conjugated and becomes direct bilirubin that is removed from the body through the bile and ultimately through the stool.
   d. Liver enxymes convert unconjugated bilirubin into conjugated bilirubin in the liver for excretion in the bile.
4. Reticulocytes are immature RBCs that comprise 0.5% to 1.5% of the total RBCs circulating. Their quantity in the blood serves as an indicator of hematopoiesis (formation of blood) from the bone marrow.
   a. An increase in reticulocytes indicates an increased rate of production of RBCs from the bone marrow, whereas a decreased number of reticulocytes in the blood indicates a decreased production of RBCs from the bone marrow as seen in bone marrow suppression.
5. Excess RBC production is called polycythemia; it results in increased blood viscosity.
6. The newborn normally has a high RBC count (4.8 to 7.1 million/mm3).
   a. A normal RBC count is 4.5 to 5.5 million/mm3
7. RBC characteristics
   a. RBCs are described using laboratory values called RBC indices. These RBC indices describe size and hemoglobin content of the RBC that affects its color.
   (1) Mean corpuscular volume (MCV) indicates the size of the RBC; cells are normocytic, macrocytic (folate or vitamin B12 deficiency), or microcytic (iron deficiency anemia, lead poisoning, thallasemia).
   (2) Mean corpuscular hemoglobin (MCH) indicates the average amount of hemoglobin per RBC; it reflects the color of the cell (normochromic, hypochromic [pale], or hyperchromic).
8. RBC hemolysis
   a. There are a variety of conditions that can result in RBBC hemolysis, including sickle cell disease, hereditary spherocytosis, hemolytic disease of the newborn, blood transfusion reaction, and others.

Question 6

Hemoglobin functioning: An overview

Composition and function of blood

Answer 6

1. It is made up of two different pairs of globin molecules. The type of globin molecules depends ont he stage in life and any abnormalities of the genes that regulate hemoglobin formation.
2. There are different types of hemoglobin, including some that can result in disease. Two types of normal functioning hemoglobin include:
   a. Hemoglobin F: This is the type of hemoglobin made during fetal life. It absorbs oxygen at a lower tension; at birth, 75% of a newborn’s hemoglobin is HbF.
   b. Hemoglobin A: This type of hemoglobin is made in postnatal life and slowly replaces HbF during the first year of life. It is composed of 2 α and 2 ß globin chains.
   (1) The lowest point of HbA and HbF is 4 to 6 months of age, which is when iron should be added to the diet; infants who are solely breast fed need exogenous iron
3. Normal hemoglobin levels in the child are 11.5 to 14.5 g/dl with variability based on age and gender. Anemia is the term used to describe a low hemoglobin count.
4. Clinical instances with an increase in the hemoglobin level include situation in which there are more cells or less fluid, such as congenital heart disease, chronic hypoxia, high altitudes, and fluid loss (dehydration).
5. Clinical instances with a decrease in the hemoglobin and hematocrit levels include situations in which there are fewer cells or more fluid, such as aplastic anemia, renal disease, iron deficiency, bone marrow supporession, sickle cell disease, hereditary spherocytosis, hemorrhage, and fluid volume overload.

Question 7

Hematocrit overview

Composition and function of blood

Answer 7

1. Hematocrit is the percentage of packed RBCs to whole blood.
2. Hematocrit expresses the relationship of cells to fluid.
3. Hematocrit exists in a constant relationship to hemoglobin, which is approximately three times the hemoglobin concentration.
   a. The hematocrit level rises and falls in the same direction and for the same reasons as dose the hemoglobin level.
4. Normal hematocrit levels for the child are 35% to 45% with variability based on age and gender.

Question 8

Anemia

Conditions affecting the red blood cell

Answer 8

Overview
1. Anemia constitutes a symptom rather than a diagnosis.
2. It is classified according to morphology (RBC characteristics) and etiology.

Etiology
1. Anemia may result from a decrease or impairment in production, size, or lifespan of RBCs or from a reduction in the amount of hemoglobin.
2. Causes of anemia include:
a. Nutritional deficiencies
b. Increased hemolysis (RBC destruction)
c. Impaired or decreased rate of production by the bone marrow or due to decreased erythropoietin release from the kidneys.
d. Excessive blood loss (acute or chronic)

Clinical manifestations
1. Anemia can be either acute or chronic.
a. Chronic anemia: usually well tolerated by the body because of the body’s compensatory mechanisms. Clinical manifestations include growth retardation, delayed sexual maturation, increased heart rate and cardiac output, and cardiac murmur.
b. Acute anemia: clinical manifestations usually a result of acute tissue hypoxia. These include muscle weakenss, fatigue, pallor, headache, lightheadedness, increased heart rate, and heart murmur.

Assessment
1. Take the child’s diet history; document the nutrients needed to make RBCs (iron, folic acid, vitamin B12).
2. Note malnutrition or anorexia.
3. Check for medications that interfere with RBC production, such as phenytoin (Dilantin) and sulfonamides.
4. Check urine, stool, and emesis for blood.
5. Assess skin color for pallor from tissue hypoxia.
6. Check hemoglobin and RBC indices.
7. Note skin integrity for breakdown or poor wound healing from poor tissue oxygenation.
8. Check for jaundice and pruritus from large amoungs of unconjugated bilirubin in the blood related to hemolysis of the RBCs; check bilirubin level.
9. Note increased pulse and respiratory rates as the body compensates for hypoxia.
10. Note altered neurologic status or behavioral changes from poor oxygenation to the brain.
11. Assess for hepatomegaly and splenomegaly from sequestered RBCs related to the hematopoietic and phagocytic functions of the liver and spleen.
12. Note weakness or low exercise tolerance.
13. Note growth retardation or failure to achieve developmental milestones.

Interventions
1. Determine and eliminate the causes of anemia.
2. Decrease oxygen demands for acute anemia: plan activities, provide passive stimulation, allow frequent rest, give small frequent feedings with softer foods, and elevate the head of the bed.
3. Implement proper hand washing and mouth care.
4. Maintain the child’s normal body temperature.

Question 9

Iron deficiency anemia

Conditions affecting the red blood cell

Answer 9

Overview
1. One of the most prevalent nutritional disorders in the U.S.
2. Iron is a necessary component of normal hemoglobin formation. When iron is insufficient, the hemoglobin concentration in the RBC is low, causing RBCs to be microcytic (small) and insufficient for carrying oxygen.

Etiology
1. Common causes of iron deficiency anemia:
a. Inadequate dietary intake: Excessive whole milk intake in a child over 12 months of age is a common causative factor. Whole milk does not contain sufficient iron, such as found in iron-fortified cereals and formula.
b. Impaired absorption of iron
c. Blood loss
d. Excessive demands for iron required for growth
2. Risk factors
a. Premature infants
(1) Anemia is secondary to decreased fetal iron supply because most iron is transferred from the mother to the fetus in the last trimester of pregnancy.
b. Multiple pregnancy
(1) Anemia results from this risk factor because of the sharing of iron from the maternal iron source during pregnancy.
c. Low-income children 6 to 24 months
(1) Due to food insecurity
d. Adolescents
(1) Anemia is secondary to an adolescent’s increased growth rate accompanied by poor eating habits.
(2) Females with menorrhagia may also be at risk for anemia.

Assessment
1. Complete a dietary history and history of present illness.
2. Assess for signs of acute anemia such as tachycardia, pallor, and lethargy.
3. Obtain blood for complete blood cell count.

Interventions
1. Provide short-term iron supplementation to correct the acute anemia nd long-term dietary modification to include foods rich in iron.
2. Promote iron-rich foods, such as clams, liver, spinach, and fortified cereals.
3. Administer iron supplementation as necessary.
a. Iron temporarily stains teeth. Use a dropper at the back of the mouth.
b. Iron is best absorbed between meals with citrus fruit or juice.
(1) Antacids, tetracycline, and histamine H2 receptor blockers may interfere with absorption of iron.
c. Stools should be a dark black or tarry green when iron levels are adequate.
d. Place iron out of the reach of children because of the risk of iron overdose and toxicity.
e. Brush teeth after administration of iron.

Question 10

Aplastic anemia

Conditions affecting the red blood cell

Answer 10

Overview
1. Aplastic anemia results from the bone marrow’s failure to produce RBCs and other blood components.

Eitiology
1. The condition is a congenital or acquired condition; it is commonly caused by an autoimmune process or certain medications, radiation, and benzene products, although 50% of cases are idiopathic.

Assessment
1. Prepare for and assist with bone marrow aspiration and biopsy in the iliac crest. (The diagnosis is confirmed by decreased or abnormal cells.)
2. Assess for symptoms of anemia, platelet deficiency, and WBC deficiency.

Interventions
1. Provide transfusion support.
2. Admin immunosuppressive medications (e.g., cortisone, cylosporine) to stop the autoimmune process.
3. Prepare for possible bone marrow transplantation as a curative measure.
4. If platelets and white blood cells are also affected, initiate interventions for hemostasis and decreased immunity to prevent bleeding and infection.

Question 11

b-Thalassemia

Conditions affecting the red blood cell

Answer 11

Overview
1. Impaired production of the ß globin chain of hemoglobin. The result is an unstable hemoglobin molecule that is more fragile, unstable to hold oxygen well, easily destroyed, and has a shortened RBC lifespan. HbF production increases to compensate.
2. Most prevalent among individuals of Mediterranean descent.
3. Also called thalassemia major or Cooley anemia.

Etiology
1. The condition occurs when a child receives a thalassemia gene from each parent

Assessment
1. As hemolysis increases, the child demonstrates chronic hypoxia and exercise intolerance. Note an abnormal hemoglobin level of 5 to 9 g/dl.
2. Hemosiderosis (excess iron storage in the tissues)/hemochromatosis (excess iron storage with resultant cellular damage) can occur with increased blood transfusions; iron is a byproduct of the breakdown of RBCs that occurs in RBC hemolysis.
a. Observe for bronze skin.
b. Assess cardiac status because of iron buildup on heart muscles causing heart failure.
3. As bone marrow attempts to compensate, hyperplasia of the bone marrow cavity may occur, resulting in thinning of the cortex and bone pain; assess for bone pain.
a. Assess for skeletal deformities and frontal bossing.
4. Assess for splenomegaly and hepatomegaly
5. Assess for delayed sexual maturation

Interventions
1. Administer regular transfusion therapy
2. Administer iron chelation therapy, such as deferasirox, to remove excess iron.
3. Be aware for the possibility of pathogic fractures.
4. Prepare the child and family for a possible splenectomy.

Question 12

Sickle cell disease: overview

Conditions affecting the red blood cell

Answer 12

1. Sickle cell disease (SCD) is a term used to describe a group of genetic disorders of hemoglobin production characterized by a predominance of the abnormal hemoglobin S.
2. SCD, an autosomal resessive hemolytic anemia, is one of the most common genetic diseases in the U.S.
   a. The child receives the affected gene from both parents; if only one gene is passed, the child is a carrier and will not usually have symptoms.
   b. There is a 25% chance with each pregnancy of 2 carriers producing a child with SCD; a 50% chance that the child will be a carrier; and a 25% chance that the child will not have the gene.
   c. One in 13 African Americans in the U.S. carries the trait for SCD; populations most commonly affected with SCD are those from Africa, the Mediterranean, India, and the Middle East.

Question 13

Sickle cell disease: Pathophysiology

Conditions affecting the red blood cell

Answer 13

1. The globin molecule contains a defect in SCD (referred to as HbS); there is an amino acid substitution in the sixth position of the hemoglobin gene.
2. RBCs containing HbS maintain a relatively normal shape when oxygenated, but change to a sickled shape after giving up oxygen to the tissues of the body.
3. RBC sickling is reversible (for a period of time) under conditions of adequate oxygenation and hydration).
4. Sickled RBCs are stiff and nonpliable and have difficulty passing through small vessels, resulting in RBC trapping in the narrow vasculature, which impedes blood flow and rsults in vaso-occlusion, tissue ischemia, and infarction.
5. RBC lifespan is reduced from 120 days to 20 days, resulting in anemia.
6. Individuals with sickle cell trait have one abnormal ß globin gene and one normal ß globin gene.
   a. Individuals with sickle cell trait experience RBC sickling only under extreme conditions such as extreme dehydration, severe hypoxia, extreme cold or heat.

Question 14

Sickle cell disease: newborn screening

Conditions affecting the red blood cell

Answer 14

The initial newborn screening is not a confirmatory test. Infants who have abnormal results on newborn screens should have follow-up confirmatory testing and parental screening to distinguish sickle cell trait from SCD.

Question 15

Sickle cell disease: Assessment

Conditions affecting the red blood cell

Answer 15

1. Clinical manifestations of SCD are due to either
   a. Obstruction by sickled RBCs
   b. Destruction of RBCs
2. Symptoms rarely appear before age 4 months because of the predominance of HbF, which prevents excessive sickling.
3. Assess for signs and symptoms of anemia (hemoglobin is typically between 6 and 9 g/dl).
4. Assess for pain caused by vaso-occlusive crisis or episode (VOC or VOE) using developmentally appropriate pain scales.
5. In infants, assess for dactylitis (hand-foot syndrome), a painful swelling and redness of the hands and feet cause by vaso-occlusion and infarction in teh small vessels. It can be confused with cellulitis, but it is not from an infectious process.
6. Assess for priapism: a painful and prolonged erection of the penis caused by vaso-occlusion.
7. Assess for signs of acute chest syndrome. Acute chest syndrome is clinically similar to pneumonia but has more serious complications associated with it. This includes marked acute anemia caused by RBC sickling and marked respiratory distress. Pneumococcal pneumonia is common. Assess respiratory status.
8. Assess for signs of infection. Susceptibility to infection begins about 6 months of age with congestion/infarction of the vessels within the spleen causing splenic failure. [A healthy spleen protects against infection by filtering bacteria and abnormal cells.]
   a. Fever is the first sign of bacteremia in the child with SCD. A temperature of 101.5° F or greater requires immediate medical evaluation and treatment because of the risk for developing overwhelming sepsis.
   b. Assist in collection of laboratory studies (blood cultures, CBC, urine, X-ray).
9. Assess for splenic sequestration. The spleen traps the altered RBCs, possibly resulting in hypovolemic shock. It occurs most frequently in children 2 months to 5 years with HbSS and may occur into adolescence in children with heterozygous (HbSC) SCD due to the phagocytic filtering properties of the spleen.
10. Assess for signs of aplastic crisis. Aplastic crisis in the child with SCD is the cessation of RBC production form the bone marrow resulting in profound anemia. Viral infeciton (particularly human parvovirus) is typically the cause of aplastic crisis in the child with SCD. This is commonly assess with a low reticulocyte count.
11. Assess for signs of stroke; VOC/VOE can cause occlusion in the brain.
12. Assess for delayed growth.
13. Assess for poor wound healing related to decreased peripheral circulation of oxygenated blood.
14. Assess vision related to potential for retinal infarction.
15. Assess for signs and symptoms of stroke. Sickling and inflammation of the cerebral vessels can obstruct blood flow resulting in ischemia and infarction of brain tissue.

Question 16

Sickle cell disease: Interventions

Conditions affecting the red blood cell

Answer 16

Supportive care for pain/VOC
1.Provide analgesics as ordered, which commonly includes a nonsteroidal anti-inflammatory drug and an opioid.
2. Provide nonpharmacologic pain management (e.g., heating pads, massage, distraction).
3. Increase hydration (IV+PO) for hemodilution to decrease blood viscosity and reverse VOC.
4. Promote oxygenation, especially if pulse oximetry shows oxygen level < 95% or 2% below baseline, to decrease ongoing sickling (will not reverse sickled cells).
5. Hydroxyurea is a drug that increases the amount of HbF. The drug reduces complications of SCD related to VOC and acute chest syndrome.

Supportive care for acute chest syndrome
1. Provide IV antibiotic therpay to treat bacterial infection as a potential cause.
2. Promote pulmonary hygiene (chest physiotherapy, cough, deep breathing, mobility) - promotes aeration of the lung and decreases sickling and consequent respiratory distress.
3. Administer packed RBCs for severe anemia (< 5-6 g/dl) - anemia in acute chest syndrome can be profound. An exchange transfusion may be considered for severe or progressive acute chest syndrome.
4. Carefully monitor IV fluids - although IV fluids promote thinning of blood and decreased sickling of RBCs, fluid overload will increase respiratory effort.
5. For chest pain, promote splinting of the chest when coughing.
6. Promote frequent use of incentive spirometry and mobility to promote lung expansion.

Supportive care for infection
1. Fever requires immediate attention in SCD. Provide IV antibiotics; this is required for children wtih SCD who have a temperature of >101.4° F.
a. Encourage the child to receive the pneumococcal and meningococcal vaccines as well as annual influenza vaccine.
b. Most children with SCD are on daily oral antibiotics (PCN).

Supportive care for splenic sequestration
1. Monitor BP and assess for hypovolemia.
2. Admin IV fluids and packed RBCs to correct hypovolemia and anemia.
3. Consider splenectomy for repeated episodes of splenic sequestration to reduce or eliminate hemolysis and for profound anemia.

Supportive care for stroke
1. Stabilize the patient and preform an exchange transfusion to prevent further brain damage and decrease the percentage of sickled cells/HbS.
2. Provide supportive care including circulation and oxygenation.
3. Provide stroke rehabilitation as needed.
4. Children with a history of a stroke or with a high % HbS levels should recieve regular transfusion therapy to keep HbS level < 30%. In children who receive mulitple transfusions, chelation therapy should be included to decrease risks of iron overload and its complications.

Provide anticipatory guidance for the patient and family.
1. Vaccinations after one has had a splenectomy should include pneumococcal, meningococcal, and Hib vaccines.
2. Review signs and symptoms of acute anemia and when to call healthcare provider.
3. Review importance of prompt notification of healthcare provider with temperature > 101.5 °F.
4. Teach avoidance of dehydration by promoting fluid intake; avoidance of cold with use of ice packs on trauma, cold swimming pools, and extreme weather; avoidance of situation of low oxygen tension; and avoidance of infectious situations.

ALERT: In children under 5 years of age, splenectomy is generally aavoided because of the increased risk for infection in asplenic children.

Other
1. Provide good skin care.
2. Avoid aspirin, as it enhances acidosis and promotes sickling.
3. Implement relaxation techniques to decrease the child’s stress level.
4. Initiate genetic counseling.
5. Teach the child or teen to avoid activities that promote a crisis, such as excessive exercise, mountain climbing, or deep sea diving; avoid extreme heat or cold.
6. Teach the family to seek early treatment of illness.
7. Teach child to avoid wearing tight clothing that impedes circulation.

Question 17

Hyperbilirubinemia

Conditions affecting the red blood cell

Answer 17

Overview
1. This is a normal occurrence in the newborn related to a high rate of hemolysis of RBCs containing hemoglobin F in the days following delivery.
2. The liver is not usually mature at birth and produces fewer enzymes.
3. Note: if jaundice appears in the first 24 hours of life or lasts >1 week in a term infant, contact a physician to assess for biliary atresia or hemolytic disease.

Assessment
1. Note jaundice of skin and eyes.
2. Assess serum indirect bilirubin level, which may rise above 12 to 15 mg/dl.
a. When blood is drawn, turn off phototherapy lights and transport blood in a covered tube (light will falsely lower the bilirubin blood sample).
3. Monitor for and prevent the development of kernicterus, which occurs when serum indirect bilirubin content is so hight that it deposits in the brain cells, resulting in permanent brain damage.
a. Assess neurologic status.
4. Assess hydration status and stooling pattern while infant is in phototherapy.
5. Assess body temperature when infant is receiving phototherapy.

Interventions
1. Initiate phototherapy; use bilirubin reduction lights to decompose unconjugated bilirubin beneath the skin, promoting excretion.
2. During phototherapy, reposition frequently.
3. Maintain normal body temperature and adequate hydration.
4. If using conventional bilirubin lights
a. Keep the lights at least 12 in. away from the infant.
b. Keep the child’s eyes shielded from the light at all times.
c. Keep the child naked.
d. Infant may be removed only for feeding.
5. If using a fiberoptic blanket, keep the infant wrapped in it at all times, including during feeding; eye patches are not necessary but may be used if the family is concerned.
6. Assist with an exchange transfusion if the child does not respond to phototherapy or if the bilirubin level is concerning for kernicterus.

Question 18

Altered clotting function

Disease conditions affecting the clotting cascade

Answer 18

Platelets (thrombocytes) overview
1. Platelets are the smallest of the formed elements in the blood.
a. They are not nucleated.
b. They live 4 to 10 days.
c. Normal platelet values in children are between 150,000 and 400,000/mm3.
2. Platelets cause capillary hemostasis by adhering to the inner surface of a vessel and sticking to each other.
a. They produce a temporary mechanical plug via a process called adhesion when they recognize damage to a blood vessel.
b. They repair breaks in small blood vessels and capillaries, especially in the skin, mucous membranes, and internal organs.
c. Heparin, aspirin, guaifenesin (Robitussin), indomethacin (Indocin), chemotherapies, penicillins, amphotericin, and phenylbutazone (Butazolidin) interfere with platelet function.

Normal clotting mechanism
1. Multiple clotting factors (including calcium and antihemophilic factor VIII) activate thromboplastin formation; generation of thromboplastin is measured by partial thromboplastin time (PTT).
2. Prothrombin and vitamin K (with the help of thromboplastin) result in thrombin production, which is measured by prothrombin time (PT).
3. Fibrinogen, thrombin, and factor VIII result in a fibrin clot, which is measured by thrombin time.

Question 19

Idiopathic/immune thrombocytopenic purpura (ITP)

Disease conditions affecting the clotting cascade

Answer 19

Overview
1. Idiopathic/immune thrombocytopenic purpura (ITP) is an acquired hemorrhagic disorder that results in the autoimmune destruction of platelets in the spleen.
2. ITP is typically proceeded by an upper respiratory tract infection or other viral illness.

Assessment
1. Clinical manifestations are consistent with those found in profound thrombocytopenia because the platelet count in ITP is typically < 20,000/mm3
a. Assess for petechial rash.
b. Note bruises and other signs of bleeding such as blood in the urine or from gums.

Interventions
1. ITP is often self-limited and may resolve without treatment.
2. For those requiring interventions, initial treatments may include oral steroid therapy, IV immunoglobulins (IVIG), and anti-D globulin.
3. Platelet transfusions are not routinely indicated, as the pathophysiology is immune in nature, which results in platelet destruction.
4. Consider splenectomy for prolonged ITP unresponsive to treatment.
5. Provide for safety measures to prevent trauma and bleeding, such as avoiding contact sports.
6. Avoid using injections; if one is necessary, inject subcutaneously and hold pressure for 5 minutes.
7. Do not administer aspirin, which increases bleeding.

Question 20

Hemophilia

Disease conditions affecting the clotting cascade

Answer 20

Overview
1. Comprises a group of X-linked recessive genetic disorders that result from a deficiency in one of the clotting factors; it is not aplatelet deficiency.
2. The most common type (75% of all cases) is hemophilia A (also called factor VIII deficiency or classic hemophilia), a sex-linked recessive disorder.
3. Other common types are hemophilia B (also called factor IX deficiency or Christmas disease).
4. Hemophilia is classified according to the presence of factor activity. Each severity level is a predictor of the ease with which a child is prone to bleeding.

Severity: Factor activity %, bleeding tendency:
* Severe: 1%, spontaneous bleeding without trauma
* Moderate 1%-5%, bleeding with trauma
* Mild 5%-50%, bleeding with severe trauma or surgery

Genetics
1. Genetic counseling should be offered to affected parents.
a. If the father has the disorder and the mother does not, all daughters will be carriers, but sons will not have the disease.
b. If the mother is a carrier and the father does not have hemophilia, each son has a 50% chance of getting hemophilia and each daughter has a 50% chance of being a carrier.

Assessment
1. Assess for signs of bleeding, especially after circumcision, immunizations, or minor injuries.
a. Multiple bruises
b. Multiple episodes of hemarthrosis (joint bleeding)
c. Measure the joint’s circumference and compare with that of the unaffected joint.
d. Note swelling, pain, or impaired joint mobility.
e. Assess for joint degeneration from repeated hemarthrosis.
f. Assess for bleeding into the neck, mouth, and thorax, which can seriously threaten respiratory status.
g. Check for peripheral neuropatheis from bleeding near periopheral nerves.
h. Assess for decreased clotting factor levels and prolonged PTT.

Interventions
1. Interventions to prevent bleeding
a. Consider helmet use in toddler with moderate to severe hemophilia.
b. Remove or pad toys and other objects in child’s environment that could cause injury.
c. Recommended toothettes (instead of bristle toothbrushes) and stool softeners if needed.
d. Encourage activities that do not inolve contact or the potential for injury, such as chess, fishing, and theater. (Avoid contact sports.)
e. Discourage weight gain and obesity, which would increase the load of the joints.
f. Give factor replacement to maintain an acceptable serum level; this is usually done by the family at home.

Interventions when bleeding occurs
1. Instruct the family to give factor replacement as soon as possible to minimize bleeding.
2. RICE (Rest, Ice, Compression, Elevation).
3. Elevate the affeccted extremity above the heart.
4. Immobilize the site to facilitate the clot forming.
5. Avoid aspirin or other medications or treatments (sutures, cauterization) that may intensify or aggravate the bleeding.
6. Perform bilateral site assessments and circumference measurements when there is an unaffected site for comparison.

Interventions for hemarthrosis
1. Give factor as soon as possible to stop the bleeding.
2. Immobilize the affected extremity.
3. Promote vasoconstriction and pain relief by applying ice compresses and pressure.
4. Treat pain as needed; avoiding aspirin and ibuprofen.
5. Avoid excessive movement or weight bearing immediately following the bleed.
6. Once the bleed has stopped, the joint should be mobilized to keep the muscles around the joint strong, which helps support the joint and also facilitates absorption and prevents contractures.
7. Decrease anxiety to lower the child’s heart rate.

Question 21

Venous Thromboembolism (VTE)

Disease conditions affecting the clotting cascade

Answer 21

Overview
1. A venous thromboembolism is a blood clot within a deep vein that can compromise blood flow and has the potential to embolize to other areas of the body, including the lungs.
2. Risk factors for developing a blood clot include Virchow’s triad: venous stasis (blood pooling in the vein), endothelial injury, and hypercoagulability.
a. Additional factors that play a part in Virchow’s triad and clot formation are emerging in pediatric literature, and include presence of a central line, thrombophilia, trauma, surgery, acute inflammation or infection, and immobility, and other high-risk conditions/medications (e.g., malignancy, cyanotic heart disease).

Assessment
1. For VTEs in the extremities, assess for unilateral pain, swelling, disoloration, and altered blood flow/return
2. For blood clots in other anatomic locations symptoms correlate with the associated organ
a. Pulmonary embolism: hypoxia, cough, dyspnea, tachypnea, tachycardia, hemoptysis
b. Cerebral dural sinus thrombus: papilledema, neurologic alterations

Interventions
1. VTE prevention (promoting mobility and central line patency)
2. Administer anticoagulants

Question 22

Disseminated Intravascular Coagulation

Answer 22

1. Cause: Major trauma, transfusion reaction, cancer (especially leukemia)l can be life treatening.
2. Pathophysiology: consumption of clotting factors and platelets resulting in massive bleeding from multiple body sites and blood clots (microemboli) that can block blood flow to organs. Hypovolemia can result.
3. Treat underlying problem/condition. Prepare for transfusion of fresh frozen plasma and platelets.

Question 23

Blood Product Administration

Answer 23

1. Red Blood Cell Transfusions are generally indicated with a hemoglobin < 7-8 for acute anemia and < 6 for chronic anemia.
2. Take vital signs before administration, including blood pressure, temperature, pulse, and respirations, for baseline.
3. Verify consent and check for right patient, right blood type, compatible type and cross match between donor and patient, product expiration, product appearance, and unit identification utilizing two clinican independent verifications. Assess for religious objections to the administration of blood products and report to the team.
4. Utilize blood tubing with appropriate filter, rpimed with normal saline. Initiate nonemergent transfusion slowly; monitor the patient closely.
5. Assess for reaction.
   a. Hemolytic (flank pain, severe headache, dyspnea, signs of shock and renal failure, fever/chills, nausea/vomiting, dark or red urine)
   b. Allergic (hives/pruritis, flushing, wheezing, airway edema)
   c. Febrile (fever/chills)
   d. Transfusion associated circulatory overload (dyspnea, rales, cough, cyanosis, distended neck veins, chest pain)
6. For a reaction:
   a. Stop the transfusion.
   b. Maintain patient IV infusion using normal saline.
   c. Monitor the patient closely.
   d. Contact the provider and blood bank.

Question 24

A preschool-age child with sickle cell disease is admitted with vaso-occlusive crisis and fever. What is the nurse’s highest priority when caring for this child?

a. Providing fluids
b. Maintaining protective isolation
c. applying cool ompresses to painful areas.
d. Administering antibiotics, as prescribed.

Answer 24

d

The child with SCD is at increased risk for infection due to decreased or absent splenic unciton. Fever is the first and best sign of infection in a child with sickle cell disease and requires prompt medical attention and administration of an antibiotic.

Question 25

A toddler with hemophilia is hospitalized with multiple injuries after falling off a slide. X-rays reveal no bone fractures. When caring for this child, what is the nurse’s highest priority?

a. Administering platelets, as prescribed.
b. Taking measures to prevent infection.
c. Frequently assessing the child’s level of consciousness.
d. Discussing a safe play environment wiht the parents

Answer 25

c

A child with hemophilia must be monitored for bleeding sequelae after an injury. One of the greatest risks for children with hemophilia is a head injury; therefore, an important assessment for the chid with hemophilia will include monitoring neurologic status for evidence of a head bleed.

Question 26

What is the primary aim of treatment for a 4-year-old admitted with a vaso-occusive crisis secondary to sickle cell disease?

a. Decrease his fever
b. Prevent the spread of infection
c. Provide for his hydration, oxygenation, and pain management needs
d. Prevent necrosis and gangrene of the ischemic body part

Answer 26

c

The child with sickle cell disease in crisis requires methods to reduce RBC sickling such as hydration and oxygenation. Additionally, children with sickle cell disease crisis require pain relieving measures such as pharmacologic and nonpharmacologic pain management.

Question 27

Which of the following is a clinical manifestation of chronic anemia experienced by children with sickle cell disease?

a. Excessive weight gain
b. Cardiac murmur
c. Early sexual maturation
d. A ruddy complexion

Answer 27

b

Chronic anemia is characterized by signs of compensation. Symptoms of chronic anemia include growth retardation, cardiac murmur, delayed sexual maturation, and hyperbilirubinemia. Development of a baseline cardiac murmur is the result of the ongoing backflow of blood through the valves caused by baseline tachycardia and increased cardiac output.

Question 28

A normal platelet count is:

Answer 28

150,000 - 400,000

Question 29

Idiopathic thrombocytopenia purpura (ITP) is associated with a platelet count below:

Answer 29

20,000.

Question 30

The nurse is caring for 2-year-old Molly, who is being evaluated for anemia. Which of the following would the nurse expect to hear when obtaining Molly’s history?

a. Molly has been unusually hungry.
b. Molly has been scratching her arms and legs.
c. Molly has just had a growth spurt.
d. Molly has been complaining of pain in her legs.

Answer 30

b

The increased breakdown of RBCs, seen in some anemias, can lead to pruritus due to the presence of unconjugated bilirubin. The child is more likely to complain of pruritis than pain.

Question 31

The nurse is leading a support group for parents of children with sickle cell disease. The nurse knows that more education is needed when one parent states:

a. “I know that my child’s wounds may take longer to heal because of sickle cell disease.”
b. “Instead of Tylenol, aspirin helps with the pain of sickle cell disease.”
c. “I always send a bottle of water to school with my child.”
d. “We don’t let our child shovel the driveway.”

Answer 31

b

In addition to the risk of Reye Syndrome, aspirin increases sickling by placing the body in a state of acidosis. Wounds often do take longer to heal due to a lack of adequate peripheral oxygenation. Keeping a child hydrated and avoiding excessive exercise and extreme temperature changes can help prevent sickling.

Question 32

Normal hemoglobin in children:

Answer 32

11.5-14.5

A hemaglobin of 7-8 in acute anemia would warrant a PRBC transfusion.

Question 33

Which symptoms would be expected in a 4-year-old admitted with acute lymphocytic leukemia?

a. Weight loss, petechiae, high platelet count
b. Weight gain, anemia, elevated WBC count
c. Petechiae, anorexia, anemia
d. Low platelet count, anemia, hunger

Answer 33

c

The child with acute lymphocytic leukemia would have suppression of any blood cells originating from the bone marrow (RBCs and hemoglobin, WBCs and platelets). As a result, you would expect a low platelet count and petechiae, lowered RBC, and anemia. In addition, the child will typically demonstrate malaise and anorexia.

Question 34

A child with leukemia is particularly susceptible to infection because:

a. Enlarged lymph nodes are not functional
b. Immature leukocytes are not capable of normal phagocytosis
c. Severe anemia conditions exist
d. The liver cannot detoxify the blood

Answer 34

b

The child with leukemia has bone marrow that is invaded by immature, non-functional leukocytes called blasts. These blasts invade the bone marrow and interfere with the production of normal blood cells, such as leukocytes, needed to fight infeciton. While lymph nodes may be enlarged with lymphatic system involvement, this is not the primary source of the body’s inability to fight infection. Severe anemia is also caused by the invasion of the bone marrow by the rapidly proliferating blasts. However, this is not a reason for the increased susceptibility to infection seen in leukemia.

Question 35

The nurse is reviewing information about pediatric cancer. Which of the following is an accurate statement?

a. Cancer is the leading cause of death in children
b. Central nervous system tumors are the most common type of pediatric cancer.
c. The incidence of cancer increases with age.
d. Childhood cancers grow slower than adult cancers.

Answer 35

c

The incidence of cancer increases with age. While cancer is second, accidents are the leading cause of death in children. Tumors of the CNS are the second most common type of pediatric cancer, after leukemia. Because the body tissues are in a state of rapid growth during childhood, pediatric cancers grow faster than adult cancers.

Question 36

Lisa is a 6-year-old who is receiving chemotherapy to treat childhood malignancy. She is currently anemic and is about to receive packed RBCs. Which of the following should be included in her plan of care?

a. Start a second large bore IV.
b. Administer Zofran prior to the administraiton of the blood.
c. Administer oxygen by nasal cannula.
d. Limit Lisa’s visitors and anticipate the need for protective isolation.

Answer 36

c

The administration of oxygen can assist the child with anemia by helping the RBCs meet the tissue’s need for oxygen. Unless the child needs to receive concurrent incompatible IV fluids for meds, there is not need to start a second IV. Zofran is used to treat nausea, a common side effect of cancer and chemotherapy, it is not indicated in the senario. Although neutropenia is a side effect of cancer and chemotherapy, it is not indicated in the scenario, therefore isolation is not necessary at this point.

Question 37

Which of the following would be expected in a 4-year-old who has just been diagnosed with acute lymphocytic leukemia (ALL)?

a. Recent history of enuresis
b. Frequent complaints of leg pain
c. Insomnia
d. Ruddy complexion

Answer 37

b

Increased proliferation of WBCs causes hypertrophy of the bone marrow which cna lead to bone pain. ALL is not associated with enuresis or insomnia.

Question 38

The nurse is working with a group of students in the pediatric cancer clinic. In reviewing a student nurse’s project on leukemia, the nurse correctly challanges which of the following statements?

a. Acute lymphocytic leukemia (ALL) is the most common type of leukemia in children.
b. ALL peaks in school-age children.
c. In adolesecents, acute myelogenous leukemia (AML) is more common than ALL.
d. Only about 65% of children diagnosed with AML will live 5 years.

Answer 38

b

ALL, which is the most common type of leukemia in children, peaks between the ages of 2 and 5 years. In adolescents, acute myelogenous leukemia (AML) is more common that ALL. Survival from AML increased to a national average of 65%.

Question 39

The nurse is caring for Gracie, a toddler with end stage cancer. Her parents ask the nurse if their daughter is likely afraid to die. Which of the following is the nurse’s best response?

a. “Gracie is most likely afraid that dying will hurt, so we will promise to keep her comfortable.”
b. “Gracie is most likely afraid to be separated from you, so we’ll keep reminding her that you are with her.”
c. “Gracie is too young to understand that anything unusual is going on, this is really much harder for you.”
d. “Gracie probably thinks that death is a punishment, so we need to keep telling her that she has done nothing wrong.”

Answer 39

b

Toddlers have a very limited concept of death and do not associate pain with death. Their greatest fear is separation from the parent. Although the toddler has little to no understanding of death, an ill child still comprehends that her routine has been altered and can sense the reactions of those around her. Interpreting death as a punishment is typical of the older preschooler who actively engages in magical thinking.