Sickle Cell

Disease
Case Study
Samantha Howard
 What is Sickle Cell Disease
A genetic condition presented at birth

shows the inheritance of one abnormal hemoglobin gene from each

parent

Red blood cell disorder that affects the shape and productivity of
hemoglobin, the protein that carries oxygen through your body

Normal Red Blood Cell Sickle Cell

Disc-shaped and flexible Crescent or “sickle” shaped
Allows for free flowing does not allow them to bend or move
movement through our blood easily, they become more sticky and often
vessels can get stuck attached to capillaries
 Common Complications
1. Vaso-Occlusive Crisis (pain crisis)
- When Sickled cells travel through small blood vessels, they can get stuck and
block blood flow causing severe pain
2. Acute Chest Syndrome
- may occur when sickled cells block blood and oxygen from reaching the lungs or
may be caused by a viral or bacterial infection
- Chest pain, coughing, difficulty breathing, fever
3. Anemia
- Since sickled cells die early, it results in an overall lack of healthy red blood cells to
carry sufficient oxygen through the body
- Slowed growth, dizziness, tiredness, a fast heart rate
4. Avascular Necrosis (Death of Bone Tissue)
- When sickled cells block blood/oxygen flow to the bones in our body, the bone
tissue can die
- Common in the hip joint
Types Of Sickle Cell Disease
HbSS
Sickle cell anemia
created from 2 inherited genes, one from each parent
Hemoglobin S
Most severe form

HbS Beta Thalassemia HbSC

Hemoglobin S gene from one parent Hemoglobin S gene from one
Beta thalassemia from the other one parent and hemoglobin C
Beta thalassemia “zero” - more severe gene from the other
Beta thalassemia “plus” - more mild Usually milder
 Treatment
1. Bone Marrow Transplant
a. Replaces the abnormal cells in the bone
marrow with healthy cells from a donor
that is a genetic match
Lifestyle Prevention:
2. Blood Transfusions Stay hydrated
a. For severe anemia Avoid extreme temperatures
b. Most common for severe splenic crisis Avoid places with low oxygen
levels
c. Have to be careful with overloading iron
Receive vaccines
3. Pain management Avoid places with high risk of
a. IV fluids germs/infection
b. Pain reducing medications
 BRITTANY LARK
Age: 5 years old

Sex: Female

Allergies: None

Preferred language: English

Diagnosis: Sickle cell disease with

suspected vaso-occlusive event of the
tibia
 Past Medical History
- Diagnosed with Sickle Cell Disease at birth
- At age 3 she was admitted to the hospital for a fever of unknown origin which
resolved on its own
- At age 4 she was admitted to the hospital for a vaso-occlusive crisis of the spleen
- She was placed on prophylactic daily antibiotic therapy until she was 5 years
old
- Patient was admitted to Hillside Pediatric Hospital for evaluation and treatment of
a suspected sickle cell related vaso-occlusive event
- She presented with a fever and significant pain in her lower right leg, along the
distal tibia starting three days ago
Nutrition Focused Physical Exam/Subjective Information
- Mother reports
- They live with her grandmother, she provides most of her meals
- Takes a daily folic acid supplement
- Does not take any other vitamin/mineral supplements or oral nutritional
supplements
- Patient is a picky eater
- Usually consumes healthy, organic foods
- Over the past 3 days patient has been eating less than normal due to the pain
- Experiencing nausea
- 24 Hour Recall Assessment
- Calories ~1,060 calories
- Protein ~ 28 grams
- Fluid ~ 1200 ml
 Additional Diet Information
Diet Prescription: did not have one

Diet PTA: consumes healthy, organic foods

Tolerance to diet: usually tolerates her normal diet well, but with
the pain she has been consuming few oral fluids and less food

GI Symptoms: nausea

IVF: 1,000 ml Dextrose

 Medications
DEXTROSE 5% IN 0.45% NACL 1,000 ML IVF, nutrient and electrolyte replenisher

Morphine Sulfate 1 MG/ML Injectable Solution For pain

Morphine Sulfate 0.5 MG/ML Injectable For pain based on the patients level of consciousness
Solution and respiratory rate

Acetaminophen 100 MG/ML Oral Suspension Given as needed

[Rx-Act Pain Relief] Tylenol, pain, aches, fever

Docusate sodium 100 MG per 10 ML Oral Laxative, given daily

Solution

Ibuprofen 100 MG per 5 ML Oral Suspension Advil, anti-inflammatory

Zofran 2 MG/ML Injectable Solution Given as needed

nausea and vomiting
 BUN 8 mg/dL normal

Labs CO2 26 mg/dL normal

Creatine 1.1 mg/dL normal

Glucose 84 mg/dL normal

Basic Serum Chloride 108 mEq/L normal
Metabolic
Panel Serum
Potassium
4.1 mEq/L normal

Serum Sodium 139 mEq/L normal

 Hemoglobin 9.8 g/dL Low

hematocrit 29.6% Low

Labs RBC’s

Platelet count
3.2 ml

120,000
Low

On lower side

WBC 10,800 cells Slightly high

CBC With Segmented

neutrophils
61 normal

Differential Band forms 3 normal

basophils 1 normal
Anemic Lab Values Eosinophils 0 normal
Hemoglobin < 12 g/dl
Hematocrit < 36 - 44% Lymphocytes 36 normal
RBC’s < 3.8 ml
Monocytes 3 normal
 Anthropometric Data
Height: 100 cm IBW: 18 kg (39.6 lbs)

Weight: 16 kg (35.2 lbs) UBW: 16 kg

Height Percentile: 5th percentile

%UBW: 100%
Weight Percentile: 22nd percentile
%IBW: 88%
BMI: 16.2
%weight change: N/A
BMI percentile: 73rd percentile
Time Frame: N/A
BMI class: normal, healthy
 Estimating Needs
Calories: Based on ABW and World Health Organization (WHO) Equation + stress
factor of 1.2-1.6

~ 970 - 1296 calories per day

Protein: Based on ABW and the DRI (0.95 g/kg/BW + stress factor 1.2-1.6)

~ 18 - 24 grams of protein per day

Fluid: Based on ABW and the DRI + stress factor

1,700 - 1,800 ml of fluid per day

 Meeting Needs?
- 24 Hour Recall Assessment (PTA, when feeling healthy)
- Calories ~1,060 calories (adequate)
- Protein ~ 28 grams (adequate)
- Fluid ~ 1200 ml (low)
- Over the past 3 days mother reports patient has not been eating
normally due to pain
- Consuming less food
- Consuming very little oral fluids
 Malnutrition

Patient does not meet criteria for

malnutrition
 Nutrition Diagnosis
PES # 1
Altered nutrition related lab values related
to diagnosis of anemia as evidenced by lab
Hemoglobin value of 9.8 g/dL, Hematocrit
29.6%, and a Red Blood Cell count of 3.2 ml.
PES #2
Inadequate energy intake related to severe
pain from a Sickle Cell vaso-occlusive crisis
as evidenced by patient’s mother reporting
a significant decline in her oral energy
intake since the pain started three days
ago.
 Nutrition Care Plan
Plan

Continue Folic Acid Supplement

Recommendations
Introduce Multivitamin - vitamin A, vitamin
D, zinc, B6, B12 Test for Sickle Cell Iron Deficiency

Introduce Omega - 3 Fatty Acid Supplement Have mother loosely monitor her oral
intake
Protein Supplement - ensure shake, ensure
apple juice, boost pudding, magic cup ice
cream
 Nutrition Care Plan
Monitor

Hemoglobin, hematocrit, and RBC lab values Goals

Continued Tolerance to Folic Acid Increase overall energy intake

Supplement
Increase hemoglobin, hematocrit, and
Tolerance to Multivitamin RBC lab values

Tolerance to Omega - 3 Fatty Acid Decrease pain

Supplement

Tolerance to protein supplement

Iron Lab Results

 Nutrition Care Plan
Diet Education

Sickle cell disease education for both her mother and

grandmother
Compliance expected
Patients mother has not received any effective education in the
past and seems ready to learn how to best help her daughter

Nutrition Risk

Medium
 Resources
https://www.cdc.gov/ncbddd/sicklecell/complications.html

https://www.cdc.gov/ncbddd/sicklecell/facts.html

https://www.cdc.gov/growthcharts/data/set1clinical/cj41c022.pdf

https://www.cdc.gov/ncbddd/sicklecell/facts.html

https://www.webmd.com/a-to-z-guides/sickle-cell-crisis