Johnson J., Keogh J. Pediatric Nursing Demystified: A Self-Teaching Guide
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Nursing alert Use Z-track injection to prevent staining of the skin.
䊋
Request stool softener as indicated to treat constipation from iron.
Client and Family Teaching
䊋 Teach proper nutrition as per treatment plan.
䊋 Inform family of dietary sources high in iron such as green leafy vegetables.
䊋 Administer with juices because vitamin C enhance absorption.
䊋 Stress the importance of follow-up blood testing to determine if hemoglobin
and hematocrit are adequate and iron administration is effective.
䊋 Teach family to administer iron properly:
• Oral medication with straw if liquid form is administered; avoid
substances that impair absorption (tea, antacid, milk).
• Teach the Z-track method for IM injections.
䊋 Caution family and client that stool will be dark green to black due to
iron content.
Nursing alert Avoid administering iron with tea, antacid, or milk to maximize
absorption.
SICKLE CELL ANEMIA
What Went Wrong?
In sickle cell anemia (SCA)/Hgb SS cell disease, an abnormal gene results
in production of an irregular red blood cell called hemoglobin (Hgb) S
that replaces some of the normal hemoglobin A. The red blood cells
collapse into a crescent shape (sickling) when stressed such as during
dehydration, hypoxemia, or acidosis. When cells sickle, clumping is
noted that obstructs small blood vessels and blocks blood flow. These
cells also have a short lifespan, resulting in early destruction due to dam-
aged cell membrane and low blood count: anemia. This condition is an
autosomal recessive condition requiring the gene from both parents. Some
clients inherit one gene and may exhibit the sickle cell trait, which may
or may not be symptomatic under severe conditions such as hypoxia dur-
ing exertion in low-oxygen settings (high altitude). Clients of African
descent have a high incidence of sickle cell anemia. Sickle cell anemia is
a chronic illness with distress resulting from blocked and inadequate cir-
culation and tissue/organ damage that cause pain and over time organ
failure and death.
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Signs and Symptoms
䊋 Acute pain due to blocked blood vessels and tissue ischemia, found in
• Extremities: swelling of hands, feet, and joints—dactylitis (hand-
foot syndrome)
• Abdomen
• Chest: pain and pulmonary disease
• Liver: jaundice and hepatic coma
• Kidney: hematuria and impaired function
• Brain: stroke
• Genitalia: painful erection (priapism)
䊋 Crisis episodes due to
• Vasoocclusion: most common crisis due to blocked blood flow from
sickling
• Sequestration
• Aplastic crisis due to extreme drop in red blood cells (RBC) (often
viral trigger)
• Megaloblastic anemia with excess need for folic acid or vitamin B
12
resulting in deficiency
• Hyperhemolytic crisis—rapid RBC destruction—anemia, jaundice,
and reticulocytosis
䊋 Sickling episodes have exacerbation with remissions after effective treat-
ment.
䊋 Fatigue secondary to the anemia.
䊋 Fever during a sickling episode possibly due to infection that provoked
distress.
䊋 Pooling of blood (sequestration) in organs resulting in enlargement:
• Splenomegaly
• Hepatomegaly
䊋 Organ damage due to vessel blockage:
• Heart (cardiomegaly) with weakened heart valves and heart murmur
• Lungs, kidneys, liver, and spleen malfunction and failure
• Extremities: avascular necrosis due to vascular blockage resulting
in skeletal deformities (hip, shoulder, lordosis, and kyphosis) and pos-
sible osteomyelitis
• Central nervous system (seizures, paresis)
• Eyes: visual disturbance, possible progressive retinal detachment
and blindness
䊋 Growth retardation may also be noted.
Test Results
䊋 Low RBCs.
䊋 Sickled cells noted per stained blood smear.
䊋 Sickle-turbidity test (Sickledex).
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䊋 Hemoglobin, hematocrit, and platelets.
䊋 Hemoglobin electrophoresis: separation of blood into different hemo-
globins to determine the form of hemoglobinopathies (hemoglobin
defects).
䊋 Newborn screening for SCA: detects hemoglobin defects early.
䊋 Pulse oximetry and blood gases may reveal hypoxia in severe anemia.
䊋 Acidosis may result in a decreased serum pH level.
䊋 Electrolyte imbalance may be noted due to acidosis.
Treatment
䊋 Hydration to thin blood and decrease sickling and vascular blockage.
䊋 Minimize infection; antibiotics may be ordered, vaccines recommended
to avoid meningitis, pneumonia, and other infections.
䊋 Oxygen supplement to decrease tissue ischemia.
䊋 Pain medication: oral or intravenous analgesics such as opioids.
䊋 Electrolyte replacements may be ordered to correct imbalances.
䊋 Blood replacement with packed cells if anemia is severe.
䊋 Bed rest with mild range of motion during episodes.
Nursing Intervention
䊋 Pain control; fear of addiction is not the issue during a crisis.
䊋 Fluid intake: Monitor intravenous fluids closely to avoid fluid overload.
䊋 Intake and output to regulate volume and monitor kidney function.
䊋 Rest periods during the day to avoid fatigue.
䊋 Mild range of motion to retain mobility.
Nursing alert Avoid cold and cold compresses with increased vasoconstriction
and pain.
Family Teaching
䊋 Teach proactive care to prevent episodes/crisis:
• Adequate fluid intake to prevent dehydration
• Avoiding infection or early treatment
• Moderate activity and adequate rest to avoid fatigue and hypoxia
䊋 Early signs of impending crisis: splenic palpation to detect sequestra-
tion
䊋 Stress need for immediate care if there are signs of crisis.
䊋 Genetic testing and counseling:
• Explain that SCA is an autosomal recessive condition requiring the
gene from both parents.
• Encourage testing of siblings to allow for childbearing planning.
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• Explain that each pregnancy when both parents are carriers presents
a 25% chance a child will be born with the disease and a 50% chance
the child will have the sickle cell trait.
• Refer for counseling and family planning if additional childbearing
is desired.
• Discuss alternative parenting options (insemination, adoption, etc.).
䊋 Support child and family with emotional responses, grieving, and coping:
• Allow ventilation of anger, concerns, fears, and questions.
• Support during depression over chronic illness.
• Provide honest responses regarding care during episodes.
• Use positive terms and avoid words like “crisis” when discussing
vasoocclusive or other problem episodes with the child and family.
• Encourage child in control of condition and lifestyle needed to avoid
episodes and promote maximum development.
CHAPTER 7 / The Hematologic System
135
Parents:
Offspring:
XY XX
XX XX XY XY
Father with abnormal gene
on X sex chromosome
Mother with normal
X sex chromosomes
FIGURE 7-3
HEMOPHILIA
Hemophilia is a group of congenital bleeding disorders due to a deficiency of
specific coagulation proteins. This condition occurs most commonly in per-
sons of African descent, possibly as a genetic adaptation in trait carriers as
protection from malaria.
What Went Wrong?
Hemophilia results most often from a genetic defect and most commonly a
deficiency of factor VIII (hemophilia A) or factor IX (hemophilia B, or Christ-
mas disease). However, a third of hemophilia cases occur from gene mutation.
The X-linked form of the condition is passed when an affected male (XhY)
mates with a female carrier (XhX) producing a 1 in 4 chance of the offspring
having a girl or having a boy with the disease, having a female carrier, or hav-
ing a child without the disease or trait. The female carrier can also be symp-
tomatic.
Signs and Symptoms
䊋 Bleeding of varied degrees depending on severity of deficiency:
• Spontaneous bleeding
• Bleeding with trauma
• Bleeding with major trauma or surgery
䊋 Hemarthrosis (bleeding into the joints) in the knees, elbows, and ankles
begins with stiffness, tingling, or ache as early sign of bleeding, pro-
gressive damage.
䊋 Warmth, redness, swelling, and severe pain and loss of movement.
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1. Why should a client with sickle cell anemia be concerned if he or she marries a
person with the sickle cell trait?
Answer:
2. What defect is most commonly caused by anemia?
a. Increased red blood cell count and blood viscosity
b. Depressed hematopoietic system and hyperactivity
c. Increased presence of abnormal hemoglobin
d. Decreased capacity of blood to carry oxygen
Answer:
✔
ROUTINE CHECKUP 1
䊋 Epistaxis (not most frequent bleed).
䊋 Hematomas may cause pain at the site due to pressure.
䊋 Intracranial bleeding can cause changes in neurostatus and progress to
death.
Nursing alert Bleeding from the mouth, throat, or neck could result in airway
obstruction and warrants immediate attention.
Test Results
䊋 History of bleeding with X-linked inheritance evidenced is diagnostic.
䊋 Clotting factor function testing will reveal an abnormality in ability to
form fibrinogen or generate thromboplastin:
• Whole blood clotting time
•PT
• PTT
• Thromboplastin generation test (TGT)
• Prothrombin consumption test
• Fibrinogen level
䊋 Pulse oximetry and blood gases may reveal hypoxia in severe anemia.
䊋 Acidosis may result in a decreased serum pH level.
䊋 Electrolyte imbalance may be noted due to acidosis.
Treatment
䊋 Factor VIII concentrate to replace the missing clotting factor.
䊋 DDAVP (1-deamino-8-D-arginine vasopressin) for mild hemophilia
(type 1 or IIA) to increase production of factor VIII.
䊋 Corticosteroids for chronic hemarthrosis, hematuria, acute hemarthrosis.
䊋 Ibuprofen or other nonsteroidal antiinflammatory drug (NSAID) for pain
relief.
Nursing alert NSAIDs should be used cautiously because they inhibit platelet
function.
䊋 Epsilon aminocaproic acid (EACA, Amicar) blocks clot destruction.
䊋 Exercise and physical therapy with active range of motion as client tolerates
to strengthen muscles around joints.
Nursing alert After acute episode, avoid passive range of motion due to pos-
sible joint capsule stretching with bleeding. Client should control active range
of motion according to pain tolerance.
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CHAPTER 7 / The Hematologic System
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Nursing Intervention
䊋 Maintain protective environment to prevent injury to client.
䊋 Monitor closely for signs of bleeding.
䊋 Treat bleeding episodes promptly.
䊋 Apply pressure to nares if nosebleed is noted.
䊋 Minimize crippling due to contractures and joint damage from
bleeding:
• Promote complete absorption of blood from joints.
• Mild exercise of limbs during confinement to prevent disuse.
• Encourage regular exercise regimen at home.
Client and Family Teaching
䊋 Protective care to prevent injury: Child-proof rooms with rounded
corners, padding, and so on, to minimize injury to mobile infant or
toddler.
䊋 Noncontact sports and activities with minimum injury potential such as
golf, swimming.
䊋 Safety equipment to minimize injury.
䊋 Soft toothbrush with water irrigation for mouth care to prevent oral
bleeding.
䊋 Electric razor instead of blades for shaving.
䊋 Teach to recognize bleeding episode in early stages and early treatment:
• RICE (rest, ice, compression, and elevation) to control bleeding
䊋 Medical identification bracelet and notification of school nurse regard-
ing condition.
䊋 Teach child to control condition and lifestyle needed to avoid episodes
and promote maximum development.
䊋 Refer as needed for financial support if insurance ceases to cover client
when older than age 21 and is removed from parental insurance.
䊋 Provide support for emotional stress to patient and family related to
chronic condition.
䊋 Genetic counseling:
• Encourage testing of siblings to allow for childbearing planning.
• Explain that each pregnancy when both parents are carriers presents
a 25% chance a child will be born with the disease and a 50% chance
the child will have the sickle cell trait.
• Refer for counseling and family planning if additional childbearing
is desired.
• Discuss alternative parenting options (insemination, adoption, etc.).
Nursing alert Avoid aspirin compounds and substitute acetaminophen because
aspirin impairs platelet function.
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IDIOPATHIC THROMBOCYTOPENIC PURPURA
Idiopathic thrombocytopenic purpura (ITP), a hemorrhagic condition, is an
acquired disorder with an unknown cause that is possibly autoimmune in origin.
It occurs between 2 and 10 years of age with recovery within 6 months.
What Went Wrong?
Excessive destruction of platelets results in deficiency (thrombocytopenia)
leading to bleeding disorders. Bone marrow may be normal with large young
platelets noted. The disorder may be acute or chronic.
Signs and Symptoms
䊋 Petechiae, or bruising, due to bleeding in superficial skin surfaces.
䊋 Bleeding from mucous membranes.
䊋 Prolonged bleeding from wounds.
䊋 Fatal hemorrhage is rare.
Test Results
䊋 Platelet count <20.000 mm
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䊋 White cell count to rule out leukemia
䊋 Testing to rule out lupus or lymphoma
Treatment
䊋 Supportive treatment
䊋 Prednisone
䊋 Anti-D antibody (if client is >1 year of age or <19 years of age)
䊋 Intravenous immune globulin (IVIG)
䊋 Splenectomy (after 5 years of age) for clients with severe chronic ITP to
remove risk of hemorrhage followed by prophylactic penicillin and vac-
cines to prevent influenza, meningitis, or pneumonia
Nursing Intervention
䊋 Supportive care:
• Protective environment with padding to prevent injury.
• Limit activity until platelets 50,000 to 100,000/mm
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.
䊋 Client and family teaching:
• Teach to avoid all contact sports.
• Medical examination with any abdominal or head trauma to rule out
internal bleeding.
䊋 Premedicate with acetaminophen 5 to 10 minutes before anti-D antibody
infusion and monitor for reaction of fever, chills, or headache. Treat with
Benadryl and Solu-Cortef and observe client closely.
Nursing alert Teach client and family to avoid using aspirin or NSAIDs for pain
management due to effect on platelets.
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BETA-THALASSEMIA
Thalassemia is an inherited disorder involving deficiency in production of
globin chains in hemoglobin. The beta form of the disorder is the most com-
mon and is found most often in persons of Greek, Italian, and Syrian descent.
An alpha form of thalassemia is found in people of Chinese, Thai, African, and
Mediterranean descent possibly due to genetic mutation because of intermarriages
or spontaneous mutation.
What Went Wrong?
Thalassemia is an autosomal-recessive disorder in which the alpha or beta
polypeptide chains in hemoglobin A are impacted. In beta-thalassemia there
is a decreased synthesis of the beta chains with an increased synthesis of alpha
chains resulting in defective hemoglobin and damaged red blood cells (hemolysis)
and resulting anemia.
An overproduction of RBCs (immature cells) may result in compensation
for the hemolysis. Folic acid deficiency may result from increased demand
on bone marrow.
Pediatric Nursing Demystified
140
1. Common symptoms of hemophilia in children include (a) ––––––––––,
(b) ––––––––––, (c) ––––––––––, (d) ––––––––––.
Answers:
2. What data in a 6-year-old client’s history would alert the nurse most to watch the
client closely for signs of thrombocytopenia?
a. The client has reported frequent backache and weight loss.
b. The client reports urinating three times a day.
c. The mother mentioned blood on the pillow some mornings.
d. The school history indicates hyperactivity when doing class work.
Answer:
3. Idiopathic thrombocytopenic purpura (ITP) commonly results in what finding?
a. Blood clots
b. Pallor
c. Dry membranes
d. Bruising
Answer:
✔ ROUTINE CHECKUP 2
Signs and Symptoms
䊋 Anemia with accompanying:
• Pallor.
• Fatigue.
• Poor feeding.
• Progressive chronic anemia: Hypoxia, headache, irritability, precor-
dial and bone pain, and anorexia may be noted.
䊋 Thalassemia minor occurs with trait carrier condition and is nonsymp-
tomatic.
䊋 Thalassemia intermedia manifests with splenomegaly and moderate to
severe anemia.
䊋 Thalassemia major (Cooley anemia) is severe anemia.
䊋 Excessive iron storage in organs without organ damage (hemosiderosis)
or with cellular damage (hemochromatosis) may be noted.
䊋 Retarded growth; particularly delayed sexual maturation is commonly noted.
䊋 Bronzed complexion: iron-containing pigment may be noted due to
breakdown of RBCs and excess iron.
䊋 If untreated, bone changes such as enlarged head and other facial
changes may be noted.
Test Results
䊋 RBC count is low.
䊋 Hemoglobin and hematocrit levels are decreased.
䊋 Hemoglobin electrophoresis analyzes the hemoglobin variants and helps
distinguish the type and severity of thalassemia.
Treatment
䊋 Maintain adequate hemoglobin levels to reduce bony deformities and
expansion of the bone marrow.
䊋 Provide blood cells to promote growth and maintain activity tolerance:
• Transfusions of RBCs as needed to keep Hgb >9.5 g/dL
䊋 Deferoxamine (Desferal), an iron chelating agent, with oral vitamin C
may be administered to promote iron excretion (may help growth if
given early at 2 to 4 years of age).
䊋 Bone marrow transplantation may be done in some children.
䊋 Splenectomy may be done to decrease destruction of blood cells, if
severe splenomegaly is noted.
Nursing alert After splenectomy, client is at risk for infection and should
receive vaccines to prevent influenza, meningitis, and pneumonia in addition to
regular immunizations.
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