MINOR CASE STUDY
SICKLE CELL ANEMIA IN PEDIATRICS
Michelle Smith | 2013
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Table of Contents
Introduction .................................................................................................................................................. 3
Social History................................................................................................................................................. 3
Normal Anatomy and Physiology of Applicable Body Functions .................................................................. 3
Present Medical Status and Treatment ........................................................................................................ 3
Theoretical Discussion of Disease Condition ............................................................................................ 3
Usual Treatment of the Condition ............................................................................................................ 5
Nutrition Treatment.............................................................................................................................. 5
Medical Treatment................................................................................................................................ 5
Patient’s Symptoms upon Admission Leading to Present Diagnosis ........................................................ 6
Laboratory Findings and Interpretation.................................................................................................... 6
Medications .............................................................................................................................................. 7
Observable Physical and Psychological Changes in Patient ...................................................................... 7
Treatment ................................................................................................................................................. 7
Medical.................................................................................................................................................. 7
Surgical .................................................................................................................................................. 7
Medical Nutrition Therapy ............................................................................................................................ 8
Nutrition History and Analysis of Previous Diet (24 hour recall) .............................................................. 8
Current Prescribed Diet & Responses ..................................................................................................... 10
Nutrition-Related Problems .................................................................................................................... 10
Evaluation of Present Nutritional Status and other Nutrients to Address ............................................. 10
Goals, Interventions, Monitoring, and Evaluation .............................................................................. 11
Patient’s Nutrition Education Process .................................................................................................... 11
General Conditions upon Discharge ....................................................................................................... 12
Prognosis ..................................................................................................................................................... 12
Summary & Conclusion ............................................................................................................................... 12
Appendix A - Medications ........................................................................................................................... 13
Bibliography ................................................................................................................................................ 14
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Introduction
The patient chosen for this case study, EV, is a 16 year old African American male who
has been diagnosed with sickle cell crisis pain. The height of the patient is 5 feet 5 inches (165.1
cm) and he weighs 115.5 pounds (52.5 kg). The patient was chosen for the pediatric case study
because of his sickle cell anemia diagnosis which has an impact on nutrition. The patient was
admitted to the hospital on February 27th and was discharged March 3rd.
Social History
This is a single male who is currently in juvenile detention but usually lives at home with
his mother. He is Christian with no special religious dietary guidelines to follow.
Normal Anatomy and Physiology of Applicable Body Functions
Normally, red blood cells have an iron-rich protein called hemoglobin inside that carries
oxygen to the body. Normal red blood cells have a disc-like shape allowing them to move freely
through blood vessels without getting stuck or easily breaking apart (1, 2). Normal red blood
cells live about 120 days in the bloodstream and then die (2).
Present Medical Status and Treatment
Theoretical Discussion of Disease Condition
Sickle cell anemia is an inherited disease that is named for the abnormal sickle or
crescent shape of the red blood cells (1-3). Sickle cell anemia is caused by an abnormal type of
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hemoglobin, hemoglobin S, that changes the shape of the red blood cells to the sickle shape
(1,2,4). These sickle-shaped cells are fragile and only live about 10 to 20 days and the bone
marrow cannot make new red blood cells fast enough to replacing the dying ones (2, 4). These
abnormal cells also deliver less oxygen to the cells of the body. These abnormal blood cells are
also sticky causing them to get stuck more easily in small blood vessels and often break into
pieces. These pieces can disrupt blood flow causing oxygen flow to cells to decrease even more
(1,4). This block in blood flow can cause pain and organ damage, increasing the risk for infection
(2).
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People can carry the sickle cell trait but show no symptoms of sickle cell anemia when
they inherit the sickle cell gene from only one parent. Sickle cell disease is much more common
in people of African and Mediterranean descent but is also seen in people from South and
Central America, the Caribbean, and the Middle East (1,2). One of every 12 African Americans
carry the sickle cell trait while 1 in 500 African Americans have the disease (2).
Symptoms of sickle cell anemia are not usually seen until after a person is 4 months old
(1,2). There are painful episodes called crises that almost all patients will experience. These
crises can last from hours to days and cause pain in the bones of the back, long bones, and
chest (1,2). When the anemia becomes more severe, the patient will experience fatigue,
paleness, rapid heart rate, shortness of breath, and yellowing of eyes and skin called jaundice
(1,2,4).
With time, the spleen will stop working causing bone, gallbladder, lung, and urinary
tract infection. Other symptoms include delayed growth, delayed puberty, and painful joints
caused by arthritis (1).
Usual Treatment of the Condition
Nutrition Treatment
Basal energy requirements are higher in adolescents with sickle cell anemia than in
healthy control subjects therefore energy intake may need to be increased. Energy deficits are
common and nightly tube feedings can help improve nutritional status (3).
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Medical Treatment
Patients with sickle cell anemia need ongoing treatment to limit the number of crises.
Folic acid supplements should be taken to make new red blood cells. Treatment for sickle cell
anemia crisis includes blood transfusions, pain medications, antibiotics to prevent bacterial
infections, and fluids. Medical treatments for complications of sickle cell anemia include dialysis
or kidney transplant for kidney disease, counseling for psychological complications, gallbladder
removal with gallstone disease, hip replacement for avascular necrosis of the hip, surgery for
eye problems, treatment for overuse or abuse of narcotic pain medications, and wound care for
leg ulcers (1,4).
Sickle cell anemia can be cured by bone marrow or stem cell transplants. This is not
currently an option for most patients as they are unable to find well-matched donors (1, 2).
Patient’s Symptoms upon Admission Leading to Present Diagnosis
The patient was admitted to the hospital with sickle cell anemia-related pain crisis
occurring in his right leg. On a scale of 1 to 10, he rated his pain an 8.
Laboratory Findings and Interpretation
Clinical indicators to diagnose and monitor patients with sickle cell anemia include
bilirubin, blood oxygen, complete blood count, hemoglobin, serum creatinine, serum
potassium, and sickle cell test (1,3).
Below is a list of the lab values out of the normal range for the patient that are related
to his diagnosis.
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Bilirubin
RBC
Hemoglobin
Hematocrit
Sickle cells
Creatinine
Lab Value
1.6
3.05
9.2
25.8
1+
0.36
Normal Value Range
0.1-1.5
4-5.65
12.6-16.7
36.9-48.5
Negative
0.6-1.2
Medications
A list of the medications the patient was on when at the hospital is provided in Appendix
A. Also listed are the uses and possible side effects that relate to the patient.
Observable Physical and Psychological Changes in Patient
The major physical change seen in the patient was that he seemed very small for his
age, both in height and weight.
Treatment
Medical
The only medical treatment for this patient has been providing him with pain
medication as needed for his pain.
Surgical
No surgeries have been needed for this patient.
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Medical Nutrition Therapy
Nutrition History and Analysis of Previous Diet (24 hour recall)
A 24 hour recall was obtained from the patient relating to what he eats when not in
juvenile detention.
Breakfast
4 waffles with butter
and syrup
4 slices bacon
2 c OJ
Snack
Ham & cheese
sandwich on white
bread with mayo
Snack bag of potato
chips
Can of pepsi
Lunch
2 McChicken
sandwiches
10 piece nuggets with
sweet chili sauce
Small fries
Hi-C
Below is the nutrient analysis of his 24 hour recall.
Nutrients
Average Eaten
Total Calories
3968 Calories
Protein (g)***
172 g
Protein (% Calories)***
17% Calories
Carbohydrate (g)***
483 g
Carbohydrate (% Calories)***
49% Calories
Dietary Fiber
22 g
Total Fat
34% Calories
Saturated Fat
9% Calories
Monounsaturated Fat
13% Calories
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Dinner
2 drumsticks
White rice with corn
and carrots
Kool-aid
Polyunsaturated Fat
9% Calories
Linoleic Acid (g)***
34 g
Linoleic Acid (% Calories)***
8% Calories
α-Linolenic Acid (g)***
2.8 g
α-Linolenic Acid (% Calories)***
0.6% Calories
Omega 3 - EPA
28 mg
Omega 3 - DHA
84 mg
Cholesterol
443 mg
Minerals
Average Eaten
Calcium
1340 mg
Potassium
4325 mg
Sodium**
7287 mg
Copper
1860 µg
Iron
21 mg
Magnesium
389 mg
Phosphorus
2212 mg
Selenium
214 µg
Zinc
17 mg
Vitamins
Average Eaten
Vitamin A
341 µg RAE
Vitamin B6
3.3 mg
Vitamin B12
3.2 µg
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Vitamin C
303 mg
Vitamin D
2 µg
Vitamin E
10 mg AT
Vitamin K
92 µg
Folate
744 µg DFE
Thiamin
2.8 mg
Riboflavin
2.7 mg
Niacin
53 mg
Choline
476 mg
Current Prescribed Diet & Responses
This patient is currently on a solid, regular, adolescent diet order. He is eating great and
loves the food.
Nutrition-Related Problems
This patient did not have a nutrition-related problem. An example of a possible problem
could be inadequate protein-energy intake related to decreased ability to consume sufficient
protein and energy as evidenced by estimated energy intake less than recommended levels.
Evaluation of Present Nutritional Status and other Nutrients to Address
Patients with sickle cell anemia require 120-150% higher energy needs. EV had an EER of 2768
with an active activity factor. EER x 1.2-1.5 = 3320-4150 kcals. His protein needs were 1.1-1.4 g pro/kg
BW which was 58-74 g pro/day. Based on the analysis of his 24 hour recall plus a reported a 10# weight
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gain in the last month, the patient is getting enough calories and protein in his diet without the need for
supplements. His diet is low in vitamin D, therefore, he may want to have his vitamin D levels checked
and possibly take a vitamin D supplement if it is low.
Children with sickle cell anemia have lower vitamin B6 concentrations and are at risk for
nutritional deficiencies. Inadequate dietary intakes of folate are common whereas vitamin B 12
intakes are usually adequate. Suboptimal vitamin A intake, low serum vitamin D status, and zinc
deficiency are also common in children with sickle cell anemia. Supplementing the diet with
missing nutrients and correcting malnutrition are important objectives. A multivitamin-mineral
supplement should be recommended. Avoid one with excess iron if the patient is receiving
transfusions (3).
Goals, Interventions, Monitoring, and Evaluation
The goal for this patient is to consume > 50% of meals. The intervention for the patient
includes a general healthful diet and the patient will be monitored and evaluated by checking
the amount of their food intake.
Other possible interventions for children with sickle cell anemia may be to include a
liquid supplement if the patient isn’t able to eat enough calories and protein to sustain growth
or to add a multivitamin-mineral supplement to replace missing nutrients.
Patient’s Nutrition Education Process
Literature was provided on how to eat high calorie and high protein meals with
suggestions of adding butter and sauces to vegetables and sandwiches. The patient verbalized
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his understanding and had no barriers to learning. He denied having any other questions at the
time of the visit.
General Conditions upon Discharge
Upon discharge the patient was no longer experiencing pain crises from the sickle cell
anemia. His appetite was still good, eating 100% of meals.
Prognosis
The patient has a very good prognosis. His needs are very high but he is still able to eat
enough calories and protein to promote weight gain and growth.
Summary & Conclusion
I have learned a lot about sickle cell anemia with this case study. I was unaware just how
high the needs for these patients were. I didn’t know about the pain crises they experience
because of the disease. It was amazing to see how much a boy had to eat and yet only gained
10# in one month.
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Appendix A - Medications
Drug
Ketorolac
Use
Short-term relief
of moderately
severe pain
Tylenol
Pain relief
Side Effects
Headache, dizziness,
drowsiness, diarrhea,
constipation, gas, sores in
mouth, sweating
Rash, hives, itching,
hoarseness, difficulty
breathing or swallowing
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Bibliography
1. PubMed Health. The sickle cell anemia page. Available at:
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001554/. Accessed March 7, 2013.
2. National Heart, Lung, and Blood Institute. Explore sickle cell anemia page. Available at:
http://www.nhlbi.nih.gov/health/health-topics/topics/sca/signs.html. Accessed March 7, 2013.
3. Escott-Stump S. Nutrition and Diagnosis-Related Care. 7th ed. Baltimore, Md: Lippincott Williams
& Wilkins; 2012.
4. Centers for Disease Control and Prevention. Sickle Cell Disease page. Available at:
http://www.cdc.gov/ncbddd/sicklecell/facts.html. Accessed March 7, 2013.
5. Medline Plus. The drugs, supplements, and herbal information page. Available at:
http://www.nlm.nih.gov/medlineplus/druginfo/meds/a693001.html. Accessed March 7, 2013.
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