CRITICAL THINKING

CASE STUDY #18:

SICKLE-CELL ANEMIA

Liwalug, Johara M.
 What is sickle-cell anemia?

Sickle cell disease, also

called sickle cell anemia, is
a genetic red blood cell
 one of a group of disorders known as sickle cell disease. disorder that causes blood
cells to take on a sickle or
 is an autosomal recessive inherited disorder carried on the beta chain crescent shape.
of hemoglobin; the amino acid valine takes the place of the normally
appearing glutamic acid.
 is an inherited red blood cell disorder in which there aren't enough
healthy red blood cells to carry oxygen throughout your body.
 Hemoglobin

 Hemoglobin is found in all red blood cells and carries oxygen from the lungs to tissues and
organs throughout the body. Normal red blood cells are soft, smooth, round and can move
easily through the body. When affected by sickle cell disease, the red blood cells become
rigid, sticky, and sickle shaped. This results in periodic plugging of blood vessels, thereby
preventing delivery of oxygen to tissues and organs.
Scenario:

Joey is a 7-year-old child with sickle-cell anemia

you see in the emergency room for a vaso-occlusive
crisis.
Thin, early school-age child with weight at 5th percentile for age. Was screened and
diagnosed with sickle-cell anemia at birth. Described as “picky eater”; has eaten little
since mother was deployed in the Middle East because he doesn’t like father’s
cooking. Has a history of two former vaso-occlusive crises. Missed last regularly
scheduled health assessment 2 weeks ago because father had difficulty taking off from
work. Was playing “tag” with older brothers this afternoon. Sclera was jaundiced and
child was crying from pain by time father returned from work. Hemoglobin 6g/100
mL; hematocrit 31%.
Study Guide Questions
2. During the history taking, you discovered that while playing “tag” together with his brothers he fell
down and sustained bruising in his right knee and that’s why he’s crying from pain. Why should
children with sickle-cell anemia not engage in contact sports or playing too hard in the sun?

 Children with sickle-cell anemia have to follow what seems like a

great many rules to avoid clotting or bleeding episodes, such as not
playing contact sports or playing too hard in the sun.
3. Since Joey is still a child what is the appropriate assessment tool that you can use to
assess for pain? Why did Joey suffer from extreme pain?

 The appropriate assessment tool that can be use to assess the

child’s pain by use of FACES Pain scale. Joey suffers from
extreme pain because of a new vaso-occlusive crisis, and his
right knee is discolored by a large brush burn.
4. You administer oxygen by nasal prongs to keep oxygen saturation above 95%.
Why is it very important to monitor the oxygen saturation of Joey?

 Oxygen saturation decreases because sickled cells are unable to

carry a full complement of oxygen.
5. You ask Joey’s father whether the child takes his folic acid and hydroxyurea
religiously at home. What are the actions of these medications?

Folic acid – help to make new RBCs.

 Folic acid and hydroxyurea help to build
Hydroxyurea- is an antineoplastic agent, has the
new RBCs to replace those that have been potential to increase the strength and oxygenation
hemolyzed. capacity of sickled cells.
 6. You notice that Joey is thin and his father tells you that Joey is a “picky eater”.
What dietary regimen can you recommend to the father?

 Provide nutrient-rich, high energy foods including dried fruit, nuts and nut butters, or smoothies if Joey
doesn't have much of an appetite. Sauces, gravies and sources of fat may also be added to meals and
snacks for extra calories.
 Get plenty of calcium-rich foods and beverages such as milk, yogurt, and cheese. Other sources of
calcium include leafy green vegetables and calcium-fortified products such as soymilk, as well as some
types of breakfast cereals and 100% fruit juices.
 Eat from a rainbow of fruits and vegetables and pair them with grains, and proteins (such as eggs, fish,
chicken, meats, beans or tofu and nuts or seeds).
 Also, the folic acid helps to replace rbc’s. Natural sources of folic acid include leafy
green vegetables, fruits, dried beans and peas.
7. Why is it very important for Joey to be well-hydrated?

 Joey should be well-hydrated to prevent dehydration. Drinking water promotes

healthy blood flow and reduces the chance of our red blood cells sickling and
sticking together. It keeps our blood cells supple so they can move more freely
within our blood vessels
8. What will be your recommended activities for Joey?

 Joey should attend regular school to maintain normal childhood and

should be allowed to participate in all school activities except contact
sports, such as football, which could result in rupture of an enlarged
spleen or liver.
Evidence-based practice related to the
management of sickle-cell anemia

The sickle trait (hemoglobin AS) occurs in about 8% of Blacks, and although
typically benign, there is some concern that intense physical exercise in such
individuals could lead to cardiac deaths from occlusive crises. It has been reported
that patients with sickle-cell disease are more prone to kidney and renal failure as
well as rhabdomyolysis during times of extreme exertion. Controversies as to
whether testing of athletes for sickle cell should be mandatory continue to exist.
Although occurrences of death associated with sickle cell and exertion are rare, the
National Collegiate Athletic Association (NCAA) has mandated testing for athletes
(Lawrence, 2014)
Author: Silbert-Flagg, JoAnne
(Source:Maternal and Child Health Nursing Care, Care of the Childbearing Family 8th edition vol.2 p.1244)