The Disease
Lesson 3.1 Review
Who is at risk for sickle cell disease?
 Sickle cell anemia is the most common form of SCD;
 It is most common in people whose families come from
Africa, South or Central America (especially Panama),
Caribbean islands, Mediterranean countries (such as Turkey,
Greece, and Italy), India, and Saudi Arabia.
 In the United States, it's estimated that sickle cell anemia
affects 70,000–100,000 people, mainly African Americans.
 The disease occurs in about 1 out of every 500 African American
births.
 Sickle cell anemia also affects Hispanic Americans. The disease
occurs in more than 1 out of every 36,000 Hispanic American
births.
 More than 2 million Americans have sickle cell trait.
 The condition occurs in about 1 in 12 African Americans.
Sickle Cell Disease
 A blood disorder.
 Autosomal recessive disorder- individual
must inherit an affected copy of the allele
from each parent to display symptoms.
 http://www.discern-
genetics.org/diagram61.php
 Homozygous for the gene controlling
hemoglobin S.
 Characterized by the destruction of red
blood cells and by “episodic blocking of
blood vessels by the adherence of sickle
cells to the vascular endothelium.”
 Red blood cells are sickle shaped.
 Sickle red blood cells have a shortened
lifespan.
 Low hemoglobin & low hematocrit.
Components of Blood
 Red Blood Cells
 Also called erythrocytes.
 Hemoglobin-containing
cells that carry oxygen
from the lungs to the
body’s tissues.
 Take carbon dioxide
back to lungs to be
exhaled.
 Flatten disc shaped.
 Lifespan approximately
4 months.
 Made in bone marrow.
 Responsible for the red
color of blood.
Components of Blood
 White Blood Cells
 Also called leukocytes.
 Blood cells that are colorless, lack
hemoglobin, and contain a nucleus.
 Larger than red blood cells.
 Made in bone marrow by stem
cells.
 Travel throughout the body and
destroy bacteria, some produce
antibodies against bacteria and
viruses, and others help fight
malignant diseases.
 Include the lymphocytes, monocytes,
neutrophils, eosinophils, and
basophils.
 High count indicates infection.
Components of Blood
 Platelets
 Also called thrombocytes.
 A minute (small), colorless,
anucleate, disc-like body of
blood.
 Fragments
 Assists in blood clotting by
adhering to other platelets and
to damaged epithelium/interact
with clotting proteins to stop or
prevent bleeding
 Impaired by aspirin.
Components of Blood
 Plasma
 The pale yellow fluid portion of whole
blood.
 Vehicle by which blood cells are carried
around the body.
 Consists of:
 92% Water
 7% Vital Proteins (Albumin, Gamma Globulin,
Anti-Hemophilic Factor, Other Clotting
Factors)
 1% Mineral Salts, Sugars, Lipids/Fats,
Metabolic Waste Products, Hormones, and
Vitamins.
Anemia
 A condition in which the blood is deficient in
red blood cells, in hemoglobin, or in total
volume.
 Diagnosed:
 Microscopically- looking for abnormal blood cells.
 Blood oxygen and complete blood count (CBC).
 Hematocrit
 Hematocrit
 The percent of the
volume of whole
blood that is
composed of red
blood cells as
determined by
separation of red
blood cells from the
plasma usually by
centrifugation.
Symptoms of Sickle Cell Disease
 Signs and Symptoms Related to Anemia Aspect of
Disease:
 The most common symptom of anemia is fatigue
(feeling tired or weak).
 Other signs and symptoms of anemia include:
 Shortness of breath
 Dizziness
 Headaches
 Coldness in the hands and feet
 Paler than normal skin or mucous membranes (the tissue that
lines your nose, mouth, and other organs and body cavities)
 Jaundice (a yellowish color of the skin or whites of the eyes)
Symptoms of Sickle Cell Disease
 Signs and Symptoms Related to Pain
 Sudden pain throughout the body is a common symptom of sickle
cell anemia – “sickle cell crisis.”
 Affect the bones, lungs, abdomen, and joints.
 Occur when sickled red blood cells block blood flow to the limbs and organs.
 Acute or chronic, but acute pain is more common.
Acute pain is sudden and can range from mild to very severe, lasts from
hours to as long as a week or more.
 Chronic pain often lasts for weeks or months and can be hard to bear and
mentally draining. (Chronic pain may limit your daily activities).
 Can damage the bones, kidneys, lungs, eyes, heart, and liver.

Complications of the Disease**
 Hand-Foot Syndrome
 Splenic Crisis- Loss of
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Spleen
Infections
Acute Chest Syndrome
Pulmonary Hypertension
Stroke
Eye Problems
Ulcers on the Legs
Multiple Organ Failure
Complications of the Disease**
 Splenic Crisis- Loss of
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Spleen
Infections
Acute Chest Syndrome
Pulmonary Hypertension
Stroke
Eye Problems
Ulcers on the Legs
Multiple Organ Failure
How could you connect blue/gray fingernails
to any/all of these complications?
 Hand-Foot Syndrome
**In your upcoming AR’s, look up complications
of the disease/topic for which we are focusing on. Try
to relate Anna’s present symptoms to the complications, and explain in depth.
Sickle Cell Treatments
• Treating Pain:
• Medications and Fluids- over the counter pain medication, heating pads,
plenty of fluids, and rest.
• Hydroxyurea- medicine that reduces painful events by stimulating the
body to produce fetal hemoglobin; but can reduce white blood cell count
and put you at risk for infections.
• Preventing Complications:
• Blood Transfusions - receive blood from other individuals to reduce the
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amount of sickle cells.
Infections- antibiotics and vaccinations.
Eye Damage- regular check-ups.
Strokes- scans and transfusions.
Acute chest syndrome- oxygen therapy, blood transfusions, antibiotics,
pain medicine, and balancing body fluids.
Leg ulcers (sores) - cleansing solutions and medicated creams or
ointments, skin grafts, bed rest, pain medications.
• New Treatments:
• Blood and Marrow Stem Cell Transplants
• Gene Therapy
• New Medications
Living with Sickle Cell Anemia
 If you have sickle cell anemia, it's important to:
 Adopt or maintain a healthy lifestyle
 Follow a healthy diet- includes a variety of vegetables and fruits,
whole grains, fat-free or low-fat dairy products, and protein
foods; low in sodium (salt), added sugars, solid fats, and refined
grains.
 Take folic acid (a vitamin) every day to help your body make new
red blood cells.
 Sleep, rest, exercise.
 No smoking.
 Take steps to prevent and control complications
 Avoid: certain medications, high altitudes, strenuous exercise.
 Vaccines, regular doctors visits.
 Learn ways to cope with pain
Associated Biomedical Professionals
 Phlebotomists: collect blood for donation or for
testing so the blood can be analyzed in a clinical
laboratory.
 Hematologists: a physician who specializes in the
diagnosis, treatment, prevention, and/or
investigation of disorders of the blood and blood
vessel wall.
 Doctors, Nurses, & Surgeons
 Physical Therapists & Orthopedist