Download Nursing Care Management

Hematologic/
immunologic
dysfunction
Objectives:
1. Define the pathophysiology of SCA
2. Identify the type of crisis
3. Prognosis and Nursing care management of Thalassemia
4. Nursing management of pediatrics with sickle cell anemia
5. Therapeutic Management
6. Diagnostic evaluation of Thalasemia and management of
Thalasemia
7. Nursing management of pediatric leukemia patients.
Is one of a group of diseases collectively termed
hemoglobinopathies in which normal adult Hgb (Hgb A [HbA]) is
partly or completely replaced by abnormal sickle Hgb (HbS).
Includes all those hereditary disorders whose clinical,
hematologic, and pathologic features are related to the presence
of HbS.
Even though the term SCD is sometimes used to refer to SCA, this
use is incorrect. Other correct terms for SCA are SS and
homozygous SCD.
The clinical features of SCA are primarily the result of
(1) Obstruction :caused by the sickled RBCs,
(2) Vascular inflammation, and
(3) Increased RBC destruction .
 The abnormal adhesion, tangling, and trapping of rigid
sickle-shaped cells accompanied by the inflammatory
process intermittently blocks the microcirculation causing
vaso-occlusion .
 The resultant absence of blood flow to adjacent tissues
causes local hypoxia, leading to tissue ischemia and
infarction (cellular death). Most of the complications
seen in SCA can be traced to this process and its impact
on various organs of the body
Clinical manifestations of SCA:
Vary greatly in severity and frequency. The most acute symptoms of the
disease occur during periods of exacerbation called crises.
Types of episodic crises:
1. Vasoocclusive.
2. Acute splenic sequestration
3. Aplastic
4. Hyperhemolytic
5. Cerebrovascular accident
6. Chest syndrome
 The crises may occur individually or concomitantly with one or more
other crises.
1. Vasoocclusive Crisis (VOC): Preferably called a “painful
episode,” is characterized by ischemia causing mild to
severe pain that may last from minutes to days.
2. Acute splenic sequestration: Is apooling of a large
amount of blood usually in the spleen and infrequently in
the liver that causes a decreased blood volume and
ultimately Shock.
3. Aplastic crisis: Is diminished RBC production usually
caused by viral infection that may result in profound
anemia.
4. Hyperhemolytic: is an accelerated rate of RBC
destruction characterized by anemia, jaundice, and
reticulocytosis.
5. Acute chest ndrome (ACS): Which is clinically similar to
pneumonia. It is the presence of a new pulmonary infiltrate
and may be associated with chest pain, fever, cough,
tachypnea, wheezing, and hypoxia.
6. Cerebrovascular Accident (CVA, stroke): Sickled cells block the
major blood vessels in the brain,resulting in cerebral
infarction, which causes variable degrees of neurologic
impairment.
 The current treatment for SCD children who have experienced a
stroke is chronic transfusion therapy.
 Repeat CVAs causing progressively greater brain damage occur
in approximately 70% of untreated children who have
experienced one stroke
Diagnostic Evaluation
 Newborn screening for SCA so infants can be identified before
symptoms occur.
 At birth, infants have up to 80% of HbF, which does not carry the
defect.
 Because levels of HbS are low at birth, Hgb electrophoresis or other
early diagnosis (before 3 months of age) enables initiation of
appropriate interventions to minimize complications.
 If SCA is not diagnosed in early infancy, it is likely to manifest
symptoms during the toddler and preschool years.
 SCA is occasionally first diagnosed during a crisis that follows an
acute respiratory tract or GI infection.
Nursing Care Management:
 Educate the Family and Child about:
1. Seek early intervention for problems, such as fever of
38.5° c (101.3° F) or greater
2. Give penicillin as ordered
3. Recognize signs and symptoms of splenic sequestration,
as well as respiratory problems that can lead to hypoxia
4. Treat the child normally. (The child is normal but can get
sick in ways that other children cannot).
5. Adequate hydration to prevent sickling and to delay the
adhesion–stasis–thrombosis–ischemia cycle.
6. Parents need instructions on how many daily glasses or bottles
of fluid are required. foods are also a source of fluid for
example, soups, ice cream, and puddings.
7. Increased fluids combined with impaired kidney function
result
8. Enuresis: Parents who are unaware of this, frequently use the
usual measures to discourage bedwetting, such as limiting
fluids at night, and may resort to punishment to force bladder
control.
 Promote Supportive Therapies During Crises
1. In choosing and scheduling analgesics, the goal should be
prevention of pain.
2. Any pain program should be combined with psychologic
support to help the child deal with the depression, anxiety, and
fear that may accompany the disease.
 If blood transfusions are given, the nurse must observe for signs
of reaction, hypervolemia, and signs of cardiac failure.
 Be aware of spleen size by palpation, splenomegaly is an
ominous sign.
Signs Of Acute Coronary Syndrome
(ACS):
1. Fever of 38.5° C (101.3° F) or higher
2. Cough
3. Dyspnea
4. Declining oxygen saturation
(oximetry)
Signs Of Cerebrovascular Accident
(CVA):
1.Severe headaches
2.Severe vomiting
3.Seizures
4.Strange, abnormal behavior
5.Inability to move an arm or leg
6.Stutter or slurred speech
7.Changes in vision
β-Thalassemia
 β-Thalassemia is the most common of the Thalassemias and
occurs in four forms:
1. Thalassemia minor, an asymptomatic silent carrier.
2. Thalassemia trait, which produces a mild microcytic anemia
3. Thalassemia intermedia: which is manifested as splenomegaly
and moderate to severe anemia.
4. Thalassemia major (Cooley anemia), which results in a severe
anemia that would lead to cardiac failure and death in early
childhood without transfusion support
Pathophysiology:
 Normal postnatal Hgb is composed of two α- and two β-polypeptide
chains.
 In β-thalassemia, there is a partial or complete deficiency in the
synthesis of the β-chain of the Hgb molecule.
 Consequently, there is a compensatory increase in the synthesis of αchains, and γ-chain production remains activated, resulting in defective
Hgb formation.
 This unbalanced polypeptide unit is very unstable; when it
disintegrates, it damages RBCs, causing severe anemia.
 To compensate for the hemolytic process, an overabundance of
erythrocytes is formed unless the bone marrow is suppressed by
transfusion therapy.
Diagnostic Evaluation:
1. Hematologic studies reveal the characteristic changes in RBCs
(microcytosis, hypochromia).
2. Low Hgb and Hct levels are seen in severe anemia.
3. Radiographs of involved bones reveal characteristic findings.
Therapeutic Management :
1. Transfusions are the foundation of medical management that
may require transfusions as often as every 3 to weeks.
The advantages of this therapy include
 Improved physical and psychologic well-being because of the
ability to participate in normal activities
 Decreased cardiomegaly and hepatosplenomegaly,
 Fewer bone changes,
 Normal or near-normal growth and development until puberty,
 Fewer infections.
2. Deferoxamine (Desferal): To minimize the development of
hemosiderosis (iron overload ), which has been shown to
be a safe equivalent to In some children with severe
splenomegaly.
3. Splenectomy may be necessary to decrease the disabling
effects of abdominal pressure and to increase the life span
of supplemental RBCs
Prognosis of Thalassemia
 Most children treated with blood transfusion and early
chelation therapy (Desferal) survive well into adulthood.
 The most common causes of death are:
1. Heart disease.
2.Post splenectomy sepsis
3.Multiple-organ failure secondary to hemochromatosis.
Nursing Care Management
The objectives of nursing care are to:
1. Promote compliance with transfusion and chelation therapy,
2. Assist the child in coping with the anxiety-provoking
treatments and the effects of the illness,
3. Foster the child’s and family’s adjustment to a chronic illness.
4. Observe for complications of multiple blood transfusions.
As with any chronic illness, the family’s needs must be met for
optimal adjustment to the stresses imposed by the disorder .
IRON-DEFICIENCY ANEMIA:
Anemia caused by an inadequate supply of dietary iron
Pathophysiology:
 Can be caused by any number of factors that decrease the
supply of iron, impair its absorption, increase the body’s need
for iron, or affect the synthesis of Hgb.
 The iron stores in newborns are usually adequate for the first 5
to 6 months in a full-term infant but for only 2 to 3 months in
preterm infants and multiple births.
 If dietary iron is not supplied to meet the infant’s growth
demands after the fetal iron stores are depleted, iron-deficiency
anemia results.
Therapeutic Management:
 Supplemental iron: This is usually done through dietary
counseling and oral iron supplements (iron-fortified commercial
formula, iron-fortified infant cereal, oral iron supplements).
 Ascorbic acid (vitamin C) appears to facilitate absorption of iron
and may be given as vitamin C–enriched foods and juices with the
iron preparation.
 If the Hgb level fails to rise after 1 month of oral therapy, it is
important to assess for persistent bleeding, iron malabsorption,
noncompliance, improper iron administration, or other causes of
the anemia.
Prognosis :
Prognosis:
1. Very good.
2. However; some evidence indicates if iron deficiency anemia is
sever and longstanding.
3. Cognitive, behavior and motor impairment may result in iron
fortified cereals. It may be difficult for infant at first to accept
foods than milk.
4. Diet education of teenagers is especially difficult, emphasizing
the effect of anemia on appearance and energy level may be
useful.
Nursing Management:
1. Oral iron should be given as prescribed in two divided doses between
meals.
2. A citrus fruit or juice taken with the medication to help in absorption.
3. An adequate dosage of oral iron turns the stool into green color.
Absences of greenish black stool may be clue to poor administration of
iron, either in schedule or in dosage.
4. If parenteral iron preparation are prescribed, iron dextran must be
injected deeply into a muscule mass using z-tract method, and the site
has been injected should not to massage to minimize skin staining &
irritation, because no more 1 ml should be given in one site.
5. The IV route should be considered to avoid multiple injections E.g.
Anphalaxis
LEUKEMIAS
 Leukemia, cancer of the blood-forming tissues, is the most
common form of childhood cancer.
 The annual incidence is 3-4 cases per 100,000 white children.
 It is more common in boys and whites, with the peak onset
between 2 and 5 years of age.
 It is one of the forms of cancer that has demonstrated dramatic
improvements in survival rates.
 Disease-free survival for children with Acute Lymphoid
Leukemia (ALL) approaches 80% , and Acute Non Lymphoid
Leukemia (ANLL) has a 50% to 65% survival rate.
Pathophysiology
 Leukemia is an overproduction of WBCs, most often in the
acute form, the leukocyte count is low (thus the term
leukemia).
 The immature cells do not deliberately attack and destroy the
normal blood cells or vascular tissues.
 Cellular destruction takes place by infiltration and subsequent
competition for metabolic elements.
 The proliferating cells depress the production of formed
elements of the blood in bone marrow by competing for and
depriving the normal cells of the essential nutrients for
metabolism.
Classification
 Leukemia is a broad term given to a group of malignant
diseases of the bone marrow and lymphatic system.
 Morphology, two forms are generally recognized in children,
1. Acute Lymphoid Leukemia (ALL).
2. Acute Non Lymphoid Leukemia (ANLL) or Acute
Myelogenous Leukemia (AML).
Diagnosis
1. History taking and physical Examination.
2. Peripheral blood smear may reveal immature forms of leukocytes.
3. Bone Marrow Aspiration or Biopsy.
4. lumbar puncture is performed to determine whether there is any
CNS involvement.
5. Chromosome studies—Children with trisomy 21 have 20 times the
risk of other children for developing ALL. Children with more than 50
chromosomes on the leukemic cells (hyperdiploid) have the best
prognosis.
6. Cell-surface immunologic markers—Cell-surface antigens have
permitted differentiation of ALL into three broad classes: B-cell ALL;
T-cell ALL; and common ALL antigen.
Signs and Symptoms:
1. Anemia from decreased rbcs.
2. Infection from neutropenia.
3. Bleeding from decreased platelet production.
4. Weakening of the bone and a tendency toward fractures.
5. Severe bone pain.
6. The spleen, liver, and lymph glands demonstrate marked
infiltration, enlargement, and eventually fibrosis.
7. Increased intracranial pressure
Therapeutic Management:
1. Induction therapy, which achieves a complete remission or less
than 5% leukemic cells in the bone marrow.
2. CNS prophylactic therapy, which prevents leukemic cells from
invading the CNS.
3. Intensification therapy (consolidation), which eradicates residual
leukemia cells, followed by delayed intensification, which prevents
emergence of resistant leukemic clones.
4. Maintenance therapy, which serves to maintain the remission
phase.
5. Hematopoietic stem cell transplantation (HSCT):
•
Is not recommended for children with ALL during the first
remission because of the excellent results possible with
chemotherapy.
• It can be from matched unrelated donors or mismatched
donors.
• It is accompanied by significant morbidity and mortality,
including graft-versus-host disease (GVHD), overwhelming
infection, or severe organ damage.
Prognosis:
•
The most important prognostic factors for determining long- term
survival for children with ALL (in addition to treatment) are
1. The Initial WBC count: Children with a normal or low WBC count who
are CALLA positive have a much better prognosis than those with a high
count or other cell types.
2. The child’s age at the time of diagnosis: Children diagnosed between 2
and 9 years of age have consistently demonstrated a better outlook
than those diagnosed before 2 or after 10 years of age.
3. The sex of the child: girls appear to have a more favorable prognosis
than boys.
4. The type of cell involved.
5. Karyotype analysis.
Late Effects of Treatment :
1. Pain: use analgesia.
2. Infection: secondary to neutropenia
3. Anemia: blood transfusion may be necessary.
4. Hemorrhage: Administration of platelet concentrate or platelet
rich plasma
5. Anorexia: Nasogastric tube feeding or total parenteral nutrition
my be implemented.
6. Mucosal ulceration: local anesthetics are effective in temporary
relieving the pain. Rectal ulcer are managed by meticulous
toilette Hygiene.
7. Neuropathy: administer laxatives for Sever constipation,
maintain good body alignment, carry out safety measure during
ambulation, and provide a soft or a liquid diet for sever jaw pain.
8. Hemorrhagic cystitis: promote a liberal fluid intake, frequent
voiding immediately after feeling the urge, administer the drug
early in the day.
9.
Alopecia: Inform the family that hair regrows in 3 to 6
months and my be of a different color and texture.
10. Moon Face: due to chemotherapy.
11. Mood change: Provide emotional support.