The Disease Lesson 3.1 Review

Who is at risk for sickle cell disease? Sickle cell anemia 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. 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, disklike 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.

Symptoms of Sickle Cell Disease Signs and Symptoms Related to Anemia 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.

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.

Complications of the Disease Hand-Foot Syndrome Splenic Crisis- Loss of Spleen Infections Acute Chest Syndrome Pulmonary Hypertension Stroke Eye Problems Ulcers on the Legs Multiple Organ Failure

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 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

How does sickle cell disease affect daily life? Loss of spleen – Sickle cells can block the blood flow to spleen and cause it to necrosis (die), which leads to increase susceptibility to infections Strokes are prevalent due to blood clots to the brain Breathing is often difficult due to blood movement impediment through the lungs Anemia since sickle cells die quickly Pain, especially in the joints due to lack of blood flow

Treatments Hydroxyurea – medicine that can reduce painful events and help with breathing Blood transfusions – Receive blood from other individuals to reduce the amount of sickle cells Bone marrow transplant – this can cure sickle cell if done early since blood is made from the marrow Surgeries – spleen may need to be removed and older sickle cell patients may need joint replacements