Learning Objective #2 (Identify 2020 National Health Goals related to children with

hematologic disorders that nurses could help the nation achieve.)

TASK: Discuss the 2020 National Health Goals related to hematologic disorders in
children.

2020 National Health Goals speak to ways to improve children’s health. Both iron-
deficiency and sickle-cell anemia are seen worldwide, hence, improving care in
these areas could have a dramatic effect on both national and world health.

 Reduce the incidence of iron deficiency among children aged 1 to 2 years

from a baseline of 15.9% to a target level of 14.3%; in children aged 3 to 4
years, from 5.3% to 4.3%.

 Reduce the incidence of iron deficiency among adolescents 12 to 18 years of

age from 10.4% to 9.4%.

 Reduce the proportion of persons with hemophilia who develop reduced joint
mobility due to bleeding into joints from 82.9% to 74.6%.

 (Developmental) Reduce hospitalization due to preventable complications of

sickle-cell disease yearly among children aged 9 years and under.

 Increase the proportion of children with special healthcare needs who have
access to a medical home from 47.1% to 51.8%.

How can nurses help the nation achieve these goals?

 Educating parents about the importance of women taking an iron

supplement during pregnancy,
 Encouraging iron-rich food sources for young children,
 Educating adolescents about healthy diets

Being certain that parents are well informed about preventive measures for children
with all types of hematologic disorders could help reduce hospital admissions.

Learning Objective #3 (Assess a child with a hematologic disorder such as sickle-

cell anemia.)

TASK 1: Review the steps of the nursing process.

The nursing process functions as a systematic guide to client-centered care with 5
sequential steps. These are assessment, diagnosis, planning (and outcome
identification), implementation, and evaluation.

 Assessment: The first step and involves critical thinking skills and data
collection; subjective (involves verbal statements from the patient or
caregiver) and objective (measurable, tangible data).
 Diagnosis: In this phase, there is formulation of a nursing diagnosis by means
of employing clinical judgment. This assists in the planning and
implementation of patient care. A nursing diagnosis is a clinical judgment
about responses to actual or potential health problems on the part of the
patient, family or community. It encompasses Maslow's Hierarchy of Needs
and helps to prioritize and plan care based on patient-centered outcomes The
North American Nursing Diagnosis Association (NANDA) provides nurses with
an up to date list of nursing diagnoses.
 Planning: This is the stage where goals and outcomes are formulated that
directly impact patient care. The goals should be SMART. Nursing care plans
are essential in this phase of goal setting. These provide a course of direction
for personalized care tailored to an individual's unique needs.
 Implementation: involves the actual carrying out of nursing interventions
outlined in the plan of care. This phase requires nursing interventions.
 Evaluation: This final step of the nursing process is vital to a positive patient
outcome. Whenever a healthcare provider intervenes or implements care,
they must reassess or evaluate to ensure the desired outcome has been met.

NURSING PROCESS FOR A CHILD WITH HEMATOLOGIC DISORDER:

ASSESSMENT: Many of the symptoms of hematologic disorders begin insidiously,

with symptoms such as pallor, lethargy, and bruising. These are such minor
symptoms that parents may not bring their children to a healthcare facility for some
time. When they do, they are surprised to learn such subtle symptoms signify the
presence of a serious illness. Children with iron deficiency, for example, aside from
appearing pale and irritable, look plump and “healthy.” It takes careful history taking
to reveal the possibility of an iron deficiency.

NURSING DIAGNOSIS: When a child is diagnosed with an inherited disorder,

parents may feel guilty or blame themselves or their partner for their child’s disease.
This can make it difficult for a family to act together to support a child during an
illness when members need intensive support themselves. Examples of nursing
diagnoses that address the entire family include:
 Deficient knowledge related to the cause of the child’s illness;
 Imbalanced nutrition, less than body requirements, related to family pattern of
not eating iron-rich foods;
  Anxiety related to frequent blood-sampling procedures;
 Pain related to tissue ischemia (inadequate blood supply to a local area due
to blockage);
 Compromised family coping related to long-term care needs of child with a
chronic hematologic disorder.

OUTCOME IDENTIFICATION AND PLANNING: The outcomes planned should be

realistic for both the child and family. It may not be possible to reduce the number of
blood-sampling procedures, for example, but fear, pain, and anxiety related to the
procedures can be addressed with distraction techniques. If the patient refuses to
take the medicine, nurses may help parents administer such by helping them
disguise the taste. If parents often forget administering a prescribed long-term
medication, the nurse may devise ways to help the parents by having them install
alarms on their phone and put notes on home appliances. If a child will be restricted
in activity for long periods because the immune system is compromised as a part of
the illness, planning must include ways to keep the child engaged with friends to
promote development.

IMPLEMENTATION: Nursing interventions for children with hematologic disorders

range from obtaining blood specimens for testing to assisting with blood or bone
marrow cell transfusions. A fingertip puncture for blood is often as painful as a
venipuncture. Suggesting that blood be drawn by means of an intermittent device
and applying an anesthetic cream before finger punctures or venipunctures are
effective measures to help reduce pain and improve cooperation with these
procedures. Even so, children may need some therapeutic playtime with a syringe
and a doll to express their anger about constant invasion by needles.

OUTCOME EVALUATION An evaluation focuses on whether short-term outcomes

such as moderation of pain or elimination of anxiety in a child undergoing diagnosis
or treatment were achieved. The focus also includes assessing the achievement of
long-term outcomes such as improving the ability of the family to manage the stress
of raising a child with a chronic illness or dealing with frequently occurring health
crises. Examples of expected outcomes that suggest goals were achieved include:
 Parents correctly state the most frequent causes of iron-deficiency anemia;
 Child states she feels better able to cope with blood-sampling procedures
through the use of imagery
 Parents describe realistic plans to ensure adherence to long-term medication
administration
 Parents voice that they understand the importance of preventing dehydration
in their school-age child with sickle-cell anemia.

TASK 2: Discuss assessment of a child with a hematologic disorder.

 Note:
An assessment of
children with
hematologic
disorders
begins with a
complete
history,
including
family history,
to identify
inherited
disorders. For
a specific
diagnosis,
children
generally
require several
diagnostic
procedures
such as blood
cell or bone
marrow
analysis.

TASK 2 PART 1: Discuss assessment of disorders of red blood cells.

Most RBC disorders fall into the category of the anemias, or a reduction in the
number or function of erythrocytes. Anemia occurs when the rate of RBC production
falls below that of cell destruction, or when there is a loss of RBCs, causing their
number and the hemoglobin level to fall below that of production. Anemias are
classified according to the changes seen in RBC numbers or configuration, or
according to the source of the problem. Although any reduction in the amount of
circulating hemoglobin lessens the oxygen-carrying capacity, clinical symptoms are
not usually apparent until the hemoglobin level reaches 7 to 8 g/100 ml.
 Normochromic, Normocytic Anemias
Normochromic (normal color), normocytic (normal cell size) anemias
occur because of impaired production of erythrocytes by the bone marrow or
by abnormal or uncompensated loss of circulating RBCs, as with acute
hemorrhage. The RBCs appear normal in both color and size; however, there
simply are too few of them for effective oxygen transport.
 Acute Blood-Loss Anemia
Blood loss that is sufficient to cause anemia can occur from trauma
(e.g. an automobile accident with internal bleeding; acute nephritis in which
blood is lost in the urine; or in the newborn from disorders such as placenta
previa, premature separation of the placenta; maternal–fetal or twin-to-twin
transfusion, or trauma to the cord or placenta; in children, it can occur from
the action of long-term intestinal parasites such as a tapeworm or hookworm;
or, in small infants, bedbug bites).
With sudden blood loss, children immediately appear pale. Because
their heart must push the reduced amount of blood through their body more
rapidly than usual, tachycardia will occur. Children will also begin to breathe
rapidly because body cells are still not able to receive adequate oxygen.
Newborns may have gasping respirations, intercostal retractions, and
cyanosis. Children with rapid heart and respiratory rates due to this do not
respond well to oxygen therapy because they lack RBCs to transport and use
the oxygen. They become listless and inactive, dizzy, and, possibly,
comatose. This type of acute blood-loss anemia generally is transitory
because the sudden reduction in available oxygen stimulates the release of
erythropoietin from the kidney and a regeneration response in the bone
marrow. The reticulocyte count rises, which is evidence that the bone marrow
is trying to increase production of erythrocytes to meet the sudden shortage.
Treatment involves control of bleeding by addressing its underlying cause and
transfusing additional RBCs. Lay the child flat to provide as much circulation
as possible to brain cells. Keep the child warm with blankets; place the infant
in an incubator or under a radiant heat warmer. Until blood is available for
transfusion, a blood expander such as plasma or intravenous fluid such as
normal saline or lactated Ringer’s may be given to expand blood volume and
improve blood pressure. With such emergency steps, the situation should be
transitory with no long-term consequences.
 Anemia of Acute Infection
Acute infection or inflammation, especially in infants, can cause
increased destruction or decreased production of erythrocytes. Common
conditions that do this include osteomyelitis and ulcerative colitis.
Management involves treatment of the underlying condition. When the
condition is reversed, blood values will return to normal.
 Anemia of Renal Disease
Either acute or chronic renal disease can cause loss of function in
kidney cells, which causes an accompanying decrease in erythropoietin
production, resulting in a normocytic, normochromic anemia. Administration of
recombinant human erythropoietin can increase RBC production and correct
the anemia, but not the renal disease.
 Anemia of Neoplastic Disease
Malignant growths such as leukemia or lymphoma (common
neoplasms of childhood) result in normochromic, normocytic anemias
because the invasion of bone marrow by proliferating neoplastic cells impairs
RBC production. There may be accompanying blood loss if platelet formation
also is decreased. The treatment of such an anemia involves measures
designed to achieve remission of the neoplastic process and transfusion to
increase the erythrocyte count.
 Hypersplenism
Under usual conditions, blood filters rapidly through the spleen. If the
spleen becomes enlarged, however, blood cells pass through more slowly,
with more cells being destroyed in the process. The overactive spleen then
leads to increased destruction of RBCs which can cause anemia and may
lead to pancytopenia (deficiency of all cell elements of blood). Virtually any
 underlying splenic condition can cause this syndrome. Therapeutic
management consists of treating the underlying splenic disorder and includes
a possible splenectomy.
 Aplastic Anemias
Aplastic anemias result from depression of hematopoietic activity in the
bone marrow. The formation and development of WBCs, platelets, and RBCs
can all be affected (Ambruso et al., 2016). Congenital aplastic anemia
(Fanconi syndrome) is inherited as an autosomal recessive trait. A child is
born with several congenital anomalies, such as skeletal and renal
abnormalities, hypogenitalism, and short stature. Within 10 years, most of
these children develop bone marrow failure as well as thrombocytopenia and
neutropenia (Ambruso et al., 2016). Acquired aplastic anemia is a decrease
in bone marrow production, which occurs if a child is excessively exposed to
radiation, drugs, or chemicals known to cause bone marrow damage.
Exposure to insecticides and chemotherapeutic drugs temporarily causes this.
Other examples of drugs that cause acquired aplastic anemia include
chloramphenicol, sulfonamides, arsenic (contained in rat poison, sometimes
eaten by children), hydantoin, benzene, or quinine. A serious infection such
as meningococcal pneumonia might cause autoimmunologic suppression of
the bone marrow, which then also results in this condition.

TASK 2 PART 2: Compare the presentation and symptoms of normochromic,

normocytic anemias.
Normochromic,
Cause Symptoms
normocytic anemias
Children immediately appear
pale, tachycardic, and
breathe rapidly. Because
they don’t respond well to
Blood loss that is
Acute blood-loss oxygen therapy, they
sufficient to cause
become listless and inactive,
anemia anemia can occur from
dizzy, and, possibly,
trauma.
comatose. Newborns may
have gasping respirations,
intercostal retractions, and
cyanosis.
Anemia of acute Acute infection or  Fatigue
inflammation (e.g.  Weakness
infection
  Pale or yellowish skin
osteomyelitis and  Irregular heartbeats
ulcerative colitis)  Shortness of breath
causes increased  Dizziness/
destruction or lightheadedness
decreased production  Chest pain
of erythrocytes.  Cold hands and feet
 Headaches
 fatigue or tiredness
 shortness of breath
Both acute and chronic  unusually pale skin
renal disease cause  weakness
loss of function in  body aches
Anemia of renal kidney cells which  chest pain
causes an
disease  dizziness
accompanying
decrease in  fainting
erythropoietin  fast or irregular heartbeat
production.  headaches
 sleep problems
 trouble concentrating
During malignant Common symptoms of
growths, the invasion anemia in addition to
Anemia of of bone marrow by symptoms of neoplastic
neoplastic disease proliferating neoplastic disease. In fact, anemia can
cells impairs RBC be considered a symptom of
production. the neoplastic disease itself.
Anemia is one of its symptoms
along with:
 Pain or fullness in the left
upper abdomen that may
spread to the left
shoulder.
 Feeling full without eating
An enlarged/overactive or after eating only a
spleen causes small amount from
Hypersplenism
destruction of more the enlarged
cells than normal. spleen pressing on your
stomach.
 Fatigue.
 Frequent infections.
 Easy bleeding.
 Or no symptoms in some
cases.
 Congenital. The child is born
with several congenital
anomalies, such as skeletal
and renal abnormalities,
hypogenitalism, and short
stature. Within 10 years,
most of these children
develop bone marrow failure
as well as thrombocytopenia
Results from
and neutropenia.
depression of
Acquired. can have no
hematopoietic activity in
symptoms otherwise these
bone marrow.
can be:
Congenital aplastic
 Fatigue
anemia is inherited,
 Shortness of breath
Aplastic anemias while acquired aplastic
 Rapid or irregular
anemia occurs if a child
heart rate
is excessively exposed
 Pale skin
to radiation, drugs, and
 Frequent or
other chemicals that
prolonged infections
cause damage to bone
 Unexplained or easy
marrow.
bruising
 Nosebleeds and
bleeding gums
 Prolonged bleeding
from cuts
 Skin rash
 Dizziness
 Headache
 Fever

TASK 2 PART 3: Compare the presentation and symptoms of hypochromic

anemias.
 Hypochromic Anemias
When hemoglobin production is inadequate, erythrocytes appear pale
(hypochromia) and are also usually reduced in diameter (microcytic).
 Iron-Deficiency Anemia
This is the most common anemia of infancy and childhood.
Without adequate iron, hemoglobin cannot be incorporated into RBCs.
This type of anemia occurs most often between the ages of 9 months
and 3 years from infants drinking more milk than they are eating iron-
rich foods. Its frequency rises again in adolescence, when iron
requirements increase, especially for girls who are menstruating.
 Chronic Infection Anemia
 Acute infection interferes with RBC production, producing a
normochromic, normocytic anemia. When infections are chronic,
however, anemia of a hypochromic, microcytic type occurs, which is
probably caused by impaired iron metabolism. The degree of anemia is
directly related to the severity of the underlying disease; treatment of
the underlying cause will usually result in increased hemoglobin levels
(Ambruso et al., 2016).

TASK 2 PART 4: Compare the presentation and symptoms of macrocytic anemias.

 Macrocytic anemia

A macrocytic anemia is one in which the RBCs appear abnormally large

(Wang, 2016). Such cells are actually immature erythrocytes or megaloblasts
(nucleated immature red cells), so these anemias are often also referred to as
megaloblastic anemias. Because these anemias are caused by nutritional
deficiencies, they occur most often in developing countries.

 Anemia of Folic Acid Deficiency

A deficiency of folic acid combined with vitamin C deficiency
produces an anemia in which the erythrocytes grow abnormally
large. There is often accompanying neutropenia (a condition that
means that you have lower-than-normal levels of neutrophils, a type
of white blood cell, in your blood) and thrombocytopenia ( a low blood
platelet count). Although the mean corpuscular hemoglobin
concentration will be normal, the mean corpuscular volume and
mean corpuscular hemoglobin will both be increased. Bone marrow
contains megaloblasts, indicating inhibition of the production of
erythrocytes at an early stage. Megaloblastic arrest, or inability of
RBCs to mature past this early stage, may occur in the first year of
life from the continued use of infant food containing too little folic
acid or from an infant drinking goat’s milk, which tends to be
deficient in folic acid. Treatment is daily oral administration of folic
acid. With this treatment, the response is dramatic.
 Pernicious Anemia (Vit B12 Deficiency)
Vitamin B12 is necessary for the maturation of RBCs. Pernicious
anemia results from a deficiency in vitamin B12, either from
inadequate intake or malabsorption (Sun, Chang, Wang, et al.,
2016).
In children, the cause is more often a lack of ingestion of vitamin
B12 rather than poor absorption. For vitamin B12 to be absorbed
from the intestine, an intrinsic factor must be present in the gastric
mucosa. If a child is born with an intrinsic factor deficiency,
symptoms occur as early as the first 2 years of life. The child
appears pale, anorexic, and irritable, with chronic diarrhea. The
tongue appears smooth and beefy red due to papillary atrophy. If not
identified and treated at that point, neuropathologic findings such as
ataxia (a group of disorders that affect co-ordination, balance and
speech), hyporeflexia (absent or diminished response to tapping),
paresthesia (burning or prickling sensation that is usually felt in the
hands, arms, legs, or feet, but can also occur in other parts of the body) ,
and a positive Babinski reflex (big toe bends up and back to the top of
the foot and the other toes fan out) will develop.
The rate and efficiency of absorption of vitamin B12 can be
tested by the ingestion of the radioactively tagged vitamin when a
dose of intrinsic factor is also measured. If the anemia is identified
as being caused by a vitamin B12–deficient diet, temporary
injections of vitamin B12 will reverse the symptoms. If the anemia is
caused by lack of the intrinsic factor, lifelong monthly intramuscular
injections of vitamin B12 may be necessary.