1. Chapter 28 Alterations in Hematologic Function
Child Health Nursing
Kristine Ruggiero CPNP, MSN, RN
Chapter 28
Alterations in
Hematologic Function
© 2006 Pearson Education, Inc.
Pearson Prentice Hall
Upper Saddle River, NJ 07458

2. Structure and Function of Blood Componenets
Review of Hematologic System
Blood formation
Red Blood Cells
White Blood Cells
Platelets

3. FIGURE 28–1 Types of blood cells.
Jane W. Ball and Ruth C. Bindler Child Health Nursing: Partnering with Children & Families
© 2006 by Pearson Education, Inc. Upper Saddle River, New Jersey All rights reserved.

4. Hematologic System
Bone marrow contains the essential element in the hematologic system….
The STEM CELL aka the pluripotential stem cell, meaning it has the ability to transform into more than one type of blood cell.
Remember, every blood cell in the body arises from a stem cell.
Although it’s fluid, blood is one of the body’s major tissues.

5. Blood Formation
In utero, the process of blood formation, called hematopoiesis, occurs in the liver and spleen. These organs retain some hematopoietic ability throughout life.
After birth, the red bone marrow becomes the main site of hematopoiesis.
The stem cells contained in the red marrow create blast cells. These are precursors to
RBCs, WBCs and PLTs
In infants and young children, all bones contain red bone marrow. And are therefore, capable of hematopoiesis. However, as the child approaches adolescence and bone growth stops, the bone marrow in many bones can’t form blood cells because the marrow has transformed to yellow bone marrow (fat deposits), although it can usually revert to red bone marrow during times of increased blood cell demand. Only the ribs, sternum, vertebra and pelvis continue to contain red bone marrow and produce blood cells.

6. Blood Components Blood is composed on plasma and cells
90% water
10% solutes, such as proteins, electrolytes, albumin, clotting factors, anticoagulants, antibodies and dissolved nutrients.
3 main cell types
RBCs or erythrocytes
WBC or leukocytes
Platelets, or thrombocytes
See Table 28-1 for normal pediatric values

7. Red Blood Cells Carry O2 to the tissues, and CO2 away from tissues
During times of hypoxia, a hormone from the kidneys (erythropoietin) stimulates the bone marrow to produce more RBCs.
Synthetic forms now available
Life of RBC= 120 days
An important waste product of RBC death is bilirubin
Bilirubin binds with albumin for transport to the liver cells to conjugate with glucuronide, forming direct bilirubin. Because unconjugated bili is fat-soluble and can’t be excreted in urine or bile, it may escape to extravascular tissue, especially fatty tissue and the brain, restulting in hyperbilirubinemia
O2 Is carried in the cell in a protein (globin) and iron (heme) structure known as hemoglobin. If adequate amounts of Iron aren’t available, the protein structure can’t be formed and RBCs cant carry their normal amount of oxygen.

8. White Blood Cells
Fight different types of infection in our body; each type has it’s own role
2 main categories of WBC’s
Granular leukocytes (granulocytes)
Neutrphils, decour invading microorganisms by phagocytosis
Eosinophils, act in allergic rxns, defend against parasites and lung and skin infections
Basophils, release heparin and histamine, involved in inflammatory and infectious rxns, aka mast cells in body tissues
Nongranular leukocytes (agranulocytes)
Lymphocytes, which are the main cells that fight infections and include B and T cells
Monocytes, work with neutrophils to help devour invading organisms

10. Platelets
Adhere to one another and plug holes in vessels or tissues where there’s bleeding.
This action is part of a larger coagulation process
PLTs also release serotonin at injury sites
Serotonin is a vasoconstrictor, decreases blood flow to injured areas

11. Iron Deficiency Anemia
A disorder of O2 transport in which the production of hgb is inadequate.
Without sufficient iron, the body can’t produce the Hgb molecure, b/c the heme component is primarily iron

12. Iron Deficiency Anemia
The cause?
Inadequate intake of iron in the diet, malabsorption of iron through the GI tract, or chronic blood loss
Last trimester of pregnancy, the fetus draws what iron it needs for the next 6-12 months
If mother is deficient in iron or
Baby is more than 4 weeks premature (32 weeks) may not have sufficient iron intake
Anemia will usually present in 2nd year of life

13. Iron Deficiency Anemia
Who knew? About 80% of iron used in building Hgb is actually reabsorbed in the GI tract from dead RBCs that have broken up.
Therefore, problems w/ GI absorption causes iron deficiency
Cow’s milk allergy (common in Blacks and Asians) causes inflammation of GI tract
In adolescents- fad diets

14. Iron Deficiency Anemia
Clinical Manifestations:
Range from mild to severe
Pale appearance and decreased activity
Toddlers may have h/o prematurity and poor weight gain
Other sxs include
Fatigue, inability to concentrate, palpitations, dyspnea on exertion, craving for nonnutritive substances such as ice, tachycardia, dry brittle nails, concave or “spoon-shaped fingernails

15. Iron Deficiency Anemia lab values
Tests: Hgb levels are routinely screened, and a CBC is typically done at 9-12 months and 24 month well-baby check-ups and at-risk populations
Iron deficiency is a microcytic, hypochromic anemia, meaning the RBC’s are small and pale. RBCs w/ decreased iron appear bleached out
B/c the cells are small, the Mean corpuscular volume (MCV), the Mean corpuscular hemoglobin (MCH) and the Mean corpuscular hemoglobin concentrations are low.
Serum iron levels are decreased

16. Iron deficiency anemia lab values
Hemoglobin
Hematocrit
Reticulocyte count=
Hemoglobin g/dL= Mild iron deficiency
Hemoglobin g/dL= Moderate iron deficiency anemia
Hemoglobin less than 8 g/dL= severe iron deficiency anemia
Reticulocyte count= helps distinguish a hypoproductive anemia from a destructive process
decrease= indicates bone marrow disorders or aplastic crisis
increase= indicates hemolytic process or active blood loss

17. Iron Deficiency Anemia
So what is the greatest nutritional risk factor for developing iron deficiency anemia?
Intro of cow’s milk in first year of life

18. Iron Deficiency Anemia Complications
Untreated, anemia can cause stress on all body tissues, w/ decreased oxygenation, especially respiratory and cardiovascular systems
Decreased ability to concentrate
Poor muscle development
Decreased performance on developmental tests

19. Iron Deficiency Anemia Treatment
The AAP recommends if Hct less than 34% or Hgb less than 11.3 g/dL begin iron supplementation
Main treatment:
Treat underlying problem
GI bleeding, chronic blood loss
Lack of iron from diet
Iron Supplementation
ORAL ferrous sulfate at 3-6 mg/kg/day for 4 weeks, then repeat Hgb/Hct
Administer through a straw, nipple
Administer on empty stomach
Administer with source of vitamin C
SES of iron administration include-
black tarry stools
Constipation
GI discomfort
Foul aftertaste

20. Iron Deficiency Anemia
Iron Rich Foods
Iron fortified cereal and formula
Enriched bread
Dark green vegetables
Legumes (kidney and pinto beans)
Figs, raisins
Meats, fish, poultry
Dried appricots

21. Iron Deficiency Anemia Evaluation
With tx, reticulocyte count increases w/in 3-5 days. Indicates + therapeutic response
Hgb should normalize w/in 4-8 weeks
When lab values are nml, wean from iron supplements
Repeat labs in 6 months, monitor wt/ development

22. Sickle Cell Anemia

23. Sickle cell disease
Sickle cell anemia (SS) is an inherited, autosomal recessive genetic disease that affects the RBC’s, which become acutely sickle-shaped.
Involves RBCs and their ability to carry oxygen
Pathophysiology of the disease
Results from a single amino acid substitution (valine for glutamine) in position 6 of the beta globin chain of hemoglobin
What does this mean?...an unstable RBC w/ a shortened survival that under stress becomes sickle shaped
Background: Sickle cell disease is an inherited disorder of hemoglobin synthesis. The resulting abnormality produces a normocytic, hemolytic anemia with multiple diversely shaped RBCs that are susceptible to morphologically changing into a sickle shape. The sickle cells produce thrombosis and obstruction in small vessels, leading to ischemia and necrosis of distal tissue.
Pathophysiology: Sickle cell disease results from a single amino acid substitution (valine for glutamate) in position 6 of the beta-globin chain of hemoglobin. This genetic alteration yields an unstable RBC with a shortened survival that under stress becomes sickle shaped.

24. Sickle cell disease How is the individual affected?
Short Hgb life span
Chronically anemic
Sickle cells risk being destroyed by the spleen…? Implications
Damage to the spleen
w/o normal functioning spleen at risk for infections
Sickle cells only live for about 15 days, while normal hemoglobin can live up to 120 days. Also, sickle cells risk being destroyed by the spleen because of their shape and stiffness. The spleen is an organ that helps filter the blood of infections and sickled cells get stuck in this filter and die. Due to the decreased number of hemoglobin cells circulating in the body, a person with sickle cell disease is chronically anemic. The spleen also suffers damage from the sickled cells blocking healthy oxygen carrying cells. Without a normal functioning spleen, these individuals are more at risk for infections. Infants and young children are at risk for life-threatening infections.

25. Sickle cell disease Age:
Hematologic changes evident as early as 10 weeks, though usually delayed until age 6-12 months--? Why do you think this is
Beta-chain (adult) hemoglobin is not prominent until the age of 3 months
Age:
Hematologic changes indicative of the disorder are evident as early as the age of 10 weeks, though symptoms are usually delayed until the age of 6-12 months….why do you think this is?? because of high levels of circulating fetal hemoglobin.
Beta-chain (adult) hemoglobulin is usually not prominent until the age of 3 months.
After infancy, erythrocytes of patients with sickle cell anemia contain approximately 90% hemoglobin S (HbS), 2-10% hemoglobin F (HbF), a normal amount of minor fraction of adult hemoglobin (HbA2), and no hemoglobin A (HbA).

26. Difference in Hgb Normal Hgb cells Live for 120 days Round Smooth
Flexible, like a letter “o” so they can move through vessels easily
Sickle Hgb cells
Live for about 15 days
Stiff
Sticky
Form into the shape of a sicle, or the letter “C”, when they loose Oxygen
Cluster together…what would this lead to in the body?
Sickle cell disease involves the red blood cells, or hemoglobin, and their ability to carry oxygen. Normal hemoglobin cells are smooth, round, and flexible, like the letter "O," so they can move through the vessels in our bodies easily. Sickle cell hemoglobin cells are stiff and sticky, and form into the shape of a sickle, or the letter "C," when they lose their oxygen. These sickle cells tend to cluster together, and cannot easily move through the blood vessels. The cluster causes a blockage and stops the movement of healthy, normal oxygen-carrying blood. This blockage is what causes the painful and damaging complications of sickle cell disease.

27. FIGURE 28– The clinical manifestations of sickle cell anemia result from pathologic changes to structures and systems throughout the body.
Jane W. Ball and Ruth C. Bindler Child Health Nursing: Partnering with Children & Families
© 2006 by Pearson Education, Inc. Upper Saddle River, New Jersey All rights reserved.

28. Sickle Cell Anemia Sickling
Triggered by fever, emotional stress, physical stress
States of hypoxia
High altitudes
Hypoventilation
Poorly pressurized aircrafts
Dehydration
Cold

29. Sickle Cell Crisis SS crisis are acute exacerbations of the disease
Vary in severity and frequency
Three most common types
Vaso-occlusive crisis
Sequestration crisis
Aplastic crisis

30. Vaso-occlusive Crisis “Pain Crisis”
Aka thrombotic crisis
Most common type of crisis
Precipitated by dehydration, exposure to cold, acidosis or localized hypoxemia
Extremely painful
Caused by stasis of blood w/ clumping of cells in the microciruclation, ischemia and infarction
Thrombosis and infarction of tissue may occur if crisis not reversed
Clinical manifestations include fever, pain, tissue engorgement, swelling of joints, hands and feet, priapism and severe abdominal pain

31. Splenic Sequestration
Life-threatening crisis: death can occur w/in hours; high mortality (up to 50%)
Caused by pooling of blood in the spleen
Spleen can hold up to 1/5th of body’s blood supply at one time—leads to CV collapse
Clinical manifestations include profound anemia, hypovolemia and shock
Occurs b/t 4 months-3 years
Tx- blood transfusions, emergent splenectomy

32. Aplastic Crisis
Diminished erythropoiesis and increased destruction of RBCs
(bone marrow depression resulting from a viral infection)
Triggered by viral infection or depletion of folic acid
Clinical manifestations include profound anemia, pallor, fatigue

33. Acute Chest Syndrome
This is similar to pneumonia, with symptoms such as difficulty breathing, chest pain and fever.
It can be caused by an infection or by blocked blood vessels in the lung.
This potentially life-threatening disorder should be treated in the hospital.
Treatments may include antibiotics, blood transfusions, pain medications, oxygen and medicines that help open up blood vessels and improve breathing.

34. Acute chest syndrome
The acute chest syndrome (ACS) in sickle cell disease (SCD) can be defined as:
a new infiltrate on chest x-ray
associated with one or more NEW symptoms: fever, cough, sputum production, dyspnea, or hypoxia..
A past history of an ACS is associated with early mortality compared to those who have never had an episode.
The disorder is most common in the 2 to 4 year age group and gradually declines in incidence with age.
ACUTE CHEST SYNDROME - when sickling is in the chest. This can be a life-threatening complication of sickle cell disease. It often occurs suddenly, when the body is under stress from infection, fever, or dehydration. The sickled cells stick together and block the flow of oxygen in the tiny vessels in the lungs. It resembles pneumonia and can include fever, pain, and a violent cough. Multiple episodes of acute chest syndrome can cause permanent lung damage.
The acute chest syndrome (ACS) in sickle cell disease (SCD) can be defined as:
a new infiltrate on chest x-ray
associated with one or more NEW symptoms: fever, cough, sputum production, dyspnea, or hypoxia.
Recent data from the Clinical Course of Sickle Cell Disease Cooperative Study indicate that this complication occurs with an incidence of 10,500/100,000 patients/year(2). ACS occurs most often as a single episode, but certain patients have multiple episodes. A past history of an ACS is associated with early mortality compared to those who have never had an episode. The disorder is most common in the 2 to 4 year age group and gradually declines in incidence with age. It is believed that Hb F exerts a protective effect on those less than 2 years of age. Furthermore, the decline observed in older age groups is partially related to at least two other factors: (a) excess mortality in the group which had an ACS, and (b) fewer viral episodes in adults due to acquired immunity.

35. Nursing Dx: Pain r/t sickling of RBCs
Pain can occur in any organ or joint in the body
Pain can be reversed
Oxygenation
Hydration
Pain Management
Rest
Mild pain episodes can be treated w/ OTC pain meds (tylenol, ibuprofen) and heating pads
More severe episodes require hospitalization and IV pain meds
Hydroxyurea
1998 FDA approved this drug for use in tx of SCD
Pain episodes. This is the most common symptom of sickle cell disease. Some affected individuals have one or fewer pain episodes a year, while others may have 15 or more.1,2 Pain episodes usually last a few hours to a few days, but they may sometimes last for weeks. Pain can occur in any organ or joint in the body, wherever sickle-shaped cells pile up and block blood vessels. Mild pain episodes can be treated at home with over-the-counter pain medications (such as acetaminophen and ibuprofen) and heating pads. But some pain episodes may be severe and need to be treated in the hospital with strong pain-killing drugs given intravenously (in a vein).  
Until recently, there was no effective treatment to prevent the sickling that causes a pain crisis other than blood transfusions. A 1995 study reported that treatment with a drug called hydroxyurea reduced the number of pain episodes in some of the severely affected adults by about 50 percent.3 In 1998, the Food and Drug Administration (FDA) approved the use of this drug in patients over 18 years of age who have had at least three painful episodes in the previous year. A 2003 study that followed the same patients for nine years also found that treatment with hydroxyurea reduced deaths by 40 percent.4
Several smaller studies have found that hydroxyurea is effective and well tolerated in children in the short term. However, the drug is not yet routinely recommended in children because it is not known whether it has any adverse effects on growth and development. Researchers continue to study the long-term safety of the drug in children.

36. Nursing Dx: Risk for Infection
Infants and young children w/ SCD are especially vulnerable to serious bacterial infections
Major cause of death in children w/ SCD Daily prophylactic Pen VK 125 mg BID from 2 months- 5 years of age
Erythromycin for children w/ PCN allergies

37. Nursing Dx: Risk for Infection
Important to receive regular childhood vaccinations (Hib and PCV 7)
In addition children w/ SCD should also receive a yearly flu shot (influenza) beginning at 6 mos of age
Another type of pneumoccocal vaccine (PCV 23)—protects against additional bacteria b/t 2-5 years of age
Meningococcal vaccine (protects against meningitis at age 5 and beyond)
It is important for babies and children with sickle cell disease to receive regular childhood vaccinations. The Hib (Hemophilus influenzae b) vaccine and the pneumococcal vaccine (Prevnar) help protect against potentially life-threatening bacterial infections. These vaccines are recommended for all babies starting at 2 months of age. Children with sickle cell disease should receive additional vaccinations. These include a yearly flu (influenza) shot, beginning at 6 months of age; another type of pneumococcal vaccine (23-valent pneumococcal vaccine), which protects against additional types of bacteria at 2 and 5 years of age; and the meningococcal vaccine, which protects against meningitis, at age 5.1 

38. Treatment for Sickle Cell Anemia
Treatment consists of sx management
The primary focus being on prevention of sickle cell crisis
Education
Blood transfusions
Hydration: Drinking plenty of water daily
(8 to 10 glasses) or receiving fluid intravenously (to prevent and treat pain crises)
Pain Management
hydroxyurea (a medication recently developed that may help reduce the frequency of pain crises and acute chest syndrome; it may also help decrease the need for frequent blood transfusions. The long-term effects of the medication are unknown.)

39. Medications for SS anemia
Hydroxyurea mg/kg/day to start and increase until therapeutic response (not more than 35 mg/kg)
A chemotherapeutic drug used in CA tx
Shown to decrease the number and severity of crises
Increases production of Hemoglobin F
Side effects include bone marrow supression, HA’s dizziness, N/V

40. Clotting Disorders Hemophilia A (Factor VIII deficiency)
Von Willerbrand Disease
Disseminated Intravascular Coagulation (DIC)
Idiopathic Thrombocytopenic Purpura (ITP)

41. Hemophilia A
Hereditary bleeding disorder, that result from deficiency of specific clotting factors
Hemophilia A aka Factor VIII is most common type
80% of people w/ hemophilia
X-linked recessive traits, which manifests as affected males, and carrier females
30% of cases are new mutations
Range of manifestations of disease from mild to severe

42. Hemophilia A Clinical Manifestations
Children usually do not manifest sxs until after 6 months of age (begin moving around, loosing teeth)
Spontaneous bleeding
Hemarthrosis (bleeding into joint space)
Deep tissue hemorrhage
Nosebleeds
Easy bruising (ecchymosis)
Hematuria
Life-threatening bleeding includes:
Head/ intracranial
Neck and throat
Abdominal/GI
Iliopsoas muscle with decrease hip ROM

43. Hemophilia A Complications from bleeding include: Bone changes
Contractures
Disabling deformities result from immobility and from bleeding into joint spaces
Muscle contractures
Joint arthritis
Chronic pain
Muscle atrophy
Compartment syndrome
Neurologic impairment

44. Hemophilia A Treatment: Factor Replacement Therapy
Goal to control bleeding by replacing the missing clotting factor and prevent complications
Factor Replacement Therapy
On demand
Prophyllaxis
IV infusions consist of
Fresh frozen plasma
Cryoprecipitate
Factor VIII

45. Treatment of Hemophilia A
Prophylaxis:
Scheduled infusions of factor 2-3 X/ week
DDAVP (Desmopressin acetate)
An analog of vasopressin, causes a 2-4 fold increase in factor VIII
Not to be confused w/ DDAVP for nocturnal enuresis
Synthetic vasopressin
MOA: release of stores from endothelial cells raising factor VIII and vWD serum levels
Administered IV, sub-Q or nasally (stimate)

46. Treatment of Hemophilia A
Amicar (epsilon amino caproic acid)
Antifibrinolytic
Uses:
Mucocutaneous bleeding
mg/kg q 6 hours
Contraindications:
hematuria

47. Hemophilia A Complications of Treatment:
Inhibitors/antibody development
IgG antibody to infused factor VIII concentrates which occurs after exposure to the extraneous VIII protein
20-30% of pts w/ severe hemophilia A
Blood-borne illnesses
Hep A,B and C
HIV

48. Hemophilia A Nursing Considerations
Factor replacement given on time
Lab monitoring as ordered
Increase metabolic states will increase factor requirements
Factor coverage for invasive procedures
Document- infusion and response to tx
NO NSAIDS
NO HEAT
NO IM injections
Utilize Hemophilia Center staff for ???s

49. Hemophilia A Nursing Considerations
Avoid taking temperatures rectally or giving suppositories
Check Bp by cuff as little as possible
Avoid IM or subcutaneous injections
Use only paper or silk tape for dressings
Perform mouth care w/ glycerin swab
Limit venipunctures
Do not give aspirin

50. Hemophilia A Psychosocial Issues
Guilt
Challenge of hospitalizations
Control issues
Financial/ insurance challenges
Feeling different/ unable to do certain activities
Counseling needs
Refer for genetic counseling after dx

51. Von Willebrand Disease
A hereditary bleeding disorder
vWF Involved w/ platelet adhesion
Most common form of disorder is autosomal dominant trait
Disease can occur in both males and females equally
Manifestations:
Easy bruising
Epistaxis

52. Von Willebrand Disease
Other clinical manifestations include:
Gingival bleeding
Ecchymosis
Increased bleeding w/ lacerations or during surgery and dental extractions
Menorrhagia (increased menstrual bleeding)
GI bleeding
Children w/ wWD usually do not have hemarthrosis

53. Von Willebrand Disease
Treatment:
Similar to Hemophila A
Restore clotting factor and prevent complications associated w/ bleeding
Infusion of vWB protein concentrate
DDAVP
Amicar for mucous membrane bleeding
Nursing Management:
Similar to Hemophilia A

54. Disseminated Intravascular Coagulation (DIC)
Life-threatening process which occurs as complication of other serious illnesses in infants and children
Most common cause of DIC is infection
An acquired pathologic process in which the clotting system is abnormally activated, resulting in widespread clot formation in the small vessels throughout the body.
These changes slow blood circulation, cause tissue hypoxia and results in tissue necrosis.
The circulating fibrin also interfere w/ clotting process and bleeding and hemmorrhage result

55. DIC The sequence of events for DIC Clinical Manifestations: Treatment:
– please see p 1027
Clinical Manifestations:
see chart on page 1028
Treatment:
Controlling bleeding, identifying and correcting the primary cause of the disorder, and preventing further activations of clotting mechanisms

56. DIC Nursing Assessment and Diagnosis:
Involves all body systems, so frequent thorough assessment of entire body is critical
Observe for petechiae, ecchymoses, and oozing every 1-2 hours
Observe for pooling of blood in dependent areas
Assess IV site Q 15 minutes for oozing
Examine stool for presence of blood
Assess extremities for cap refill, warmth and pulses
Frequently assess VS and LOC
I’s and O’s
Monitor O2 sat and ABG’s
ID family’s coping strategies and support systems

57. Idiopathic Thrombocytopenic Purpura (ITP)
Aka autoimmune thrombocytopenic purpura
Most common bleeding disorder in children
Occurs in children 2-10 years-old, peaks b/t 2-5 y.o
A disorder characterized by increased destruction of platelets in the spleen, even though plt production in the bone marrow is normal
Autoimmune
Plts are destroyed as a result of the binding of autoantibodies to PLT antigens
After a viral illness

58. ITP Clinical Manifestations: Multiple ecchymoses Petechiae
Purpura (purplish areas where blood has collected d/t bleeding from blood vessels)
Bleeding from gums
nosebleeds
Hematuria
heme in stools

59. ITP Dx is made by Hx and PE and lab findings
Tx: depends on PLT counts and clinical presentation
Corticosteroids
IVIG
PLT administration only if hemorrhage occurs
If no response to therapy in 6mos-1 year, splenectomy may be tx of choice
Spontaneous remission in 90% of cases

60. Nursing Care of the Child with a Hematologic Disorder
Based on the Disorder
RBC’s
Oxygenation
Circulation
Fluid
Nutrition
Pain Management

61. Nursing Care of a Child with a Hematologic Disorder
Based on the Disorder
WBC’s
Infection
Oxygenation
Nutrition
Platelets and bleeding disorders
Bleeding
Circulation
Injury Prevention

62. Collaborative Care for a Child with a Hematologic Disorder
Team Approach
Family Involved
Decisions w/ family and child