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Sickle Cell anemia is a group of inherited red blood cell disorders. Normal red blood cells are round like doughnuts, and move through small blood tubes in the body to deliver oxygen. Sickle red blood cells become hard, sticky and crescent shaped. When these cells go through the capillaries, they clog the flow and break apart. This can cause pain, damage, a low blood count or anemia.
There is a substance in the red cell called hemoglobin that carries oxygen. One little change in this substance causes the hemoglobin to form long rods in the red cell when it gives away oxygen. These rigid rods change the red cell into a sickle shape.
There are three common types of sickle cell disease in the United States. - Hemoglobin SS or sickle cell anemia
- Hemoglobin SC disease
- Hemoglobin sickle beta-thalassemia
Each of these can cause sickle pain episodes and complications, but some are more common than others. All of these may also have an increase in fetal hemoglobin which can protect the red cell from sickling and help prevent complications. The medication hydroxyurea also increases fetal hemoglobin.
Incidence; Causes & Development
Sickle cell occurs in many ethnic groups including African Americans, Arabs, Greeks, Italians, Latin Americans and Native Americans. All races should be screened for this hemoglobin at birth.
One inherits the abnormal hemoglobin from both parents who may be carriers with the sickle cell trait or have sickle cell disease. One is born with the sickle cell hemoglobin and it is present for life.
Sickle cell trait occurs when a person carries one sickle hemoglobin producing gene inherited from their parents and one normal hemoglobin gene. Normal hemoglobin is called type A. Sickle hemoglobin is called type S. Sickle cell trait is diagnosed by the presence of hemoglobin AS on the hemoglobin electrophoresis. This will not cause sickle cell disease. Sickle cell trait does not adversely affect an individual's life expectancy.
Signs & Symptoms
Complications from the sickle cells blocking blood flow and early breaking apart include:Diagnosis & Tests
A simple blood test called the hemoglobin electrophoresis can be done by a doctor or local sickle cell foundation. This test will indicate whether a person is a carrier of the sickle cell trait, or if they have the disease. Most states now perform the sickle cell test when babies are born. The simple blood test will detect sickle cell disease or sickle cell trait.
Treatment & Prevention
Sickle cell patient should be under the care of a medical team that understands sickle cell disease. All newborn babies detected with sickle cell disease should be placed on daily penicillin to prevent serious infections. All of the childhood immunizations should be given plus the pneumococcal vaccine. Parents should know how to check for a fever because this signals the need for a quick medical checkup for serious infection.
The following are general guidelines to keep the sickle cell patient healthy:- Taking oral supplements of the vitamin folic acid (folate) daily to help make new red cells
- Daily penicillin until age six to prevent serious infection
- Drinking plenty of water daily (8-10 glasses for adults)
- Avoiding too hot or too cold temperatures
- Avoiding overexertion and stress
- Getting plenty of rest
- Getting regular check-ups from knowledgeable health care providers
Hydroxyurea, a drug used to treat other blood disorders, may help reduce the recurrence of acute attacks (crises). During an attack, a doctor may also administer pain relievers, fluid to prevent dehydration or oxygen to boost the level in the bloodstream. The doctor also may prescribe antibiotics to deal with or prevent infections associated with sickle cell anemia. Blood transfusions may reduce the body's level of abnormal hemoglobin. If the anemia is the result of autoimmunity, managing it may include avoiding suspect medications, treating related infections and taking immunosuppressive drugs, such as corticosteroids. Sometimes, removal of the spleen is called for.
Prognosis; Seek medical attention if
Sickle cell trait does not adversely affect an individual's life expectancy.
Patients and families should watch for the following conditions that need an urgent medical evaluation:- Fever
- Chest pain
- Shortness of Breath
- Increasing tiredness
- Abdominal swelling
- Unusual headache
- Any sudden weakness or loss of feeling
- Pain that will not go away with home treatment
- Priapism (painful erection that will not go down)
- Sudden vision change
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Signs, symptoms & indicators of Sickle Cell Trait / Disease:
Conditions that suggest Sickle Cell Trait / Disease: | | | | | Organ Health |  Retinopathy | The sickle-shaped blood cell reduces blood flow, potentially leading to sickle cell retinopathy. Patients will not have visual symptoms early on in the disease and need to be followed closely in case neovascularization occurs. |
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KEY | | Weak or unproven link |
GLOSSARY
Abdomen (Abdominal)
That part of the body between the chest and the hips that contains the stomach, intestines, liver, bladder, pancreas and other organs.
Acute
An illness or symptom of sudden onset, which generally has a short duration.
Anemia (Anaemia, Anemias)
A condition resulting from an unusually low number of red blood cells or too little hemoglobin in the red blood cells. The most common type is iron-deficiency anemia in which the red blood cells are reduced in size and number, and hemoglobin levels are low. Clinical symptoms include shortness of breath, lethargy and heart palpitations.
Autoimmune Disease (Autoimmune, Autoimmunity)
One of a large group of diseases in which the immune system turns against the body's own cells, tissues and organs, leading to chronic and often deadly conditions. Examples include multiple sclerosis, rheumatoid arthritis, systemic lupus, Bright's disease and diabetes.
Checkup (Check-up, Checkups, Check-ups)
A thorough physical examination that includes a variety of tests depending on the age, sex and health of the person.
Corticosteroid (Corticosteroids)
Steroid hormone produced by the adrenal cortex.
Folic Acid
A B-complex vitamin that functions along with vitamin B-12 and vitamin C in the utilization of proteins. It has an essential role in the formation of heme (the iron containing protein in hemoglobin necessary for the formation of red blood cells) and DNA. Folic acid is essential during pregnancy to prevent neural tubular defects in the developing fetus.
Gallstone (Biliary Calculus, Gall Stone, Gall Stones, Gallbladder Attack, Gallbladder Attacks, Gallstones)
Stone-like objects in either the gallbladder or bile ducts, composed mainly of cholesterol and occasionally mixed with calcium. Most gallstones do not cause problems until they become larger or they begin obstructing bile ducts, at which point gallbladder "attacks" begin to occur. Symptoms usually occur after a fatty meal and at night. The following are the most common ones: steady, severe pain in the middle-upper abdomen or below the ribs on the right; pain in the back between the shoulder blades; pain under the right shoulder; nausea; vomiting; fever; chills; jaundice; abdominal bloating; intolerance of fatty foods; belching or gas; indigestion.
Hemoglobin
The oxygen-carrying protein of the blood found in red blood cells.
Jaundice
Yellow discoloration of the skin, whites of the eyes and excreta as a result of an excess of the pigment bilirubin in the bloodstream.
Kidneys (Kidney, Renal)
Bean-shaped organs, each about the size of a fist. They are located near the middle of the back, just below the rib cage. The kidneys are sophisticated reprocessing machines, each day handling about 50 gallons of blood to sift out about half a gallon of waste products and extra water. The waste and extra water become urine, which flows to the bladder through tubes called ureters. The actual filtering occurs in tiny units inside the kidneys called nephrons. Every kidney has about a million nephrons. In a nephron, a glomerulus -- which is a tiny blood vessel, or capillary -- intertwines with a tiny urine-collecting tube called a tubule. A complicated chemical exchange takes place, as waste materials and water leave your blood and enter your urinary system. The kidneys recycle chemicals such as sodium, phosphorus, and potassium and thus regulate their levels. Renal: Pertaining to the kidneys.
Liver (Hepatic)
The largest and one of the most complex organs of the body, the liver is responsible for much of the metabolism of fats, proteins and carbohydrates. It is the site of much of the body's detoxification. It is connected very closely with digestion and the regulation of blood sugar, among many other functions. Found behind the ribs on the right side of the abdomen, it has many important functions such as removing harmful material from the blood, making enzymes and bile that help digest food, and converting food into substances needed for life and growth. Hepatic: Pertaining to the liver.
Lung (Lungs, Pulmonary)
Organ of the body, located in the chest cavity which is designed to bring oxygen from the air into the blood stream, while also expelling carbon dioxide and other waste gases out of the body. Pulmonary: Related to the lungs.
Red Blood Cell (Erythrocyte, Erythrocytes, RBC, Red Blood Cells)
Any of the hemoglobin-containing cells that carry oxygen to the tissues and are responsible for the red color of blood.
Retinopathy
An eye disorder that does not cause inflammation but results from changes in the eye (retinal) blood vessels.
Spleen (Splenectomy)
The largest organ of the lymphatic system in the body, located between the stomach and the diaphragm (the muscle below the lungs which provides the motion for breathing). It destroys old red blood cells, produces lymphocytes and plasma cells, and stores blood. It also has other as yet unknown functions. A splenectomy is the surgical removal of the spleen.
Ulcer (Ulceration, Ulcers)
Lesion on the skin or mucous membrane.
Last updated: Sep 28, 2008
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