2What is Sickle Cell Anemia? A serious condition in which red blood cells can become sickle-shapedNormal red blood cells are smooth and round. They move easily through blood vessels to carry oxygen to all parts of the body.Sickle-shaped cells don’t move easily through blood. They’re stiff and sticky and tend to form clumps and get stuck in blood vessels.The clumps of sickle cell block blood flow in the blood vessels that lead to the limbs and organs. Blocked blood vessel can cause pain, serious infection, and organ damage.
3Normal and Sickled Red Blood Cells in Blood Vessels Figure B shows abnormal, sickled red blood cells clumping and blocking the blood flow in a blood vessel. The inset image shows a cross-section of a sickled red blood cell with abnormal strands of hemoglobin.Figure A shows normal red blood cells flowing freely in a blood vessel. The inset image shows a cross-section of a normal red blood cell with normal hemoglobin.Source from
4Sickle Cell Anemia vs. Sickle Cell Trait People who have sickle cell anemia are born with it; means inherited, lifelong condition.They inherit two copies of sickle cell gene, one from each parent.Sickle cell trait is different from sickle cell anemia. People with sickle cell trait don’t have the condition, but they have one of the genes that cause the condition.People with sickle cell anemia and sickle cell trait can pass the gene on when they have children.Sickle cell anemia is an inherited, lifelong condition. People who have sickle cell anemia are born with it. They inherit two copies of the sickle cell gene, one from each parent. People who inherit a sickle cell gene from one parent and a normal gene from the other parent have a condition called sickle cell trait.Sickle cell trait is different from sickle cell anemia. People with sickle cell trait don’t have the condition, but they have one of the genes that cause the condition. Like people with sickle cell anemia, people with sickle cell trait can pass the gene on when they have children. To learn more about sickle cell trait, see the section on causes of sickle cell anemia.
5Inheritance of Sickle Cell Anemia If one parent has sickle cell anaemia (HbSS) and the other is completely unaffected (HbAA) then all the children will have sickle cell trait.None will have sickle cell anemia.The parent who has sickle cell anemia (HbSS) can only pass the sickle hemoglobin gene to each of their children.Source from
6Inheritance of Sickle Cell Anemia If both parents have sickle cell trait (HbAS) there is a one in four (25%) chance that any given child could be born with sickle cell anemia.There is also a one in four chance that any given child could be completely unaffected.There is a one in two (50%) chance that any given child will get the sickle cell trait.
7Why Anemia?Anemia is a condition in which a person’s blood has a lower than normal number of red blood cells, or the red blood cells don’t have enough hemoglobin. Hemoglobin is an iron-rich protein that gives blood its red color and carries oxygen from the lungs to the rest of the body.Normal red blood cells last about 120 days in the bloodstream and then die. Their main role is to carry oxygen, but they also remove carbon dioxide (a waste product) from cells and carry it to the lungs to be exhaled.In sickle cell anemia, a lower-than-normal number of red blood cells occurs because sickle cells don’t last very long. Sickle cells die faster than normal red blood cells, usually after only about 10 to 20 days. The bone marrow can’t make new red blood cells fast enough to replace the dying ones. The result is anemia.AnemiaAnemia (uh-NEE-me-uh) is a condition in which a person’s blood has a lower than normal number of red blood cells, or the red blood cells don’t have enough hemoglobin (HEE-muh-glow-bin).Hemoglobin is an iron-rich protein that gives blood its red color and carries oxygen from the lungs to the rest of the body.Red blood cells are made in the spongy marrow inside the large bones of the body. Bone marrow constantly makes new red blood cells to replace old ones. Normal red blood cells last about 120 days in the bloodstream and then die. Their main role is to carry oxygen, but they also remove carbon dioxide (a waste product) from cells and carry it to the lungs to be exhaled.In sickle cell anemia, a lower-than-normal number of red blood cells occurs because sickle cells don’t last very long. Sickle cells die faster than normal red blood cells, usually after only about 10 to 20 days. The bone marrow can’t make new red blood cells fast enough to replace the dying ones. The result is anemia.OutlookSickle cell anemia affects millions of people worldwide. There are excellent treatments for the symptoms and complications of the condition, but in most cases there’s no cure. (Some researchers believe that bone marrow transplants may offer a cure in a small number of cases.)Over the past 30 years, doctors have learned a great deal about the condition. They know what causes it, how it affects the body, and how to treat many of the complications. Today, with good health care, many people with the condition live close to normal lives and are in fairly good health much of the time. These people can live into their forties or fifties, or longer.
9Signs and SymptomsIndividual signs and symptoms varies. Some have mild symptoms, others have very severe symptoms and may be hospitalized for treatmentPresent at birth, many infants doesn’t show signs until after 4 months of ageAnemia: Fatigue (tiredness), pale skin and nail beds, jaundice, and shortness of breathPain (Sickle Cell Crisis): Sudden episode of pain throughout the body. Common sites: bones, lungs, abdomen, and joints. Lack of blood flow can cause pain and organ damage.The signs and symptoms of sickle cell anemia are different in each person. Some people have mild symptoms. Others have very severe symptoms and are often hospitalized for treatment. Although sickle cell anemia is present at birth, many infants don’t show any signs until after 4 months of age.What Are the Signs and Symptoms of Sickle Cell Anemia?The most common signs and symptoms are linked to anemia and pain. Other signs and symptoms are linked to some of the complications of the condition.AnemiaPain (Sickle Cell Crisis)The general signs and symptoms of anemia are fatigue (tiredness), pale skin and nail beds, jaundice (yellowing of the skin and eyes), and shortness of breath.Sudden episodes of pain throughout the body are a common symptom of sickle cell anemia and are often referred to as “sickle cell crises.”A sickle cell crisis occurs when the red blood cells sickle (become “C” shaped) and stick together in clumps. The clumps block the flow of blood through the small blood vessels (capillaries) in the limbs and organs.Sickle crises can cause acute or chronic pain. Acute pain is the most common type. This is sudden pain that can range from mild to very severe. The pain usually lasts from hours to a few days. Chronic pain usually lasts for weeks to months. Chronic pain can be hard to bear and mentally draining. This pain may severely limit daily activities.Almost all people with sickle cell anemia have painful crises at some point in their lives. Some have a crisis less than once a year. Others may have 15 or more crises in a year.Many factors can contribute to a sickle cell crisis. Often, more than one factor is involved and the exact cause can’t be identified. Factors that occur in your body and aren’t under your control can cause a sickle cell crisis, such as an infection.Factors that you can control also can affect whether you have a sickle cell crisis. For example, dehydration (when your body doesn’t have enough fluid) can increase your chances of having a sickle cell crisis. Drinking plenty of fluids so your body is hydrated can often help decrease the chance of a crisis.The most common sites affected by sickle cell crises are the bones, lungs, abdomen, and joints. The blocked blood flow can cause pain and organ damage.
10Complication of Sickle Cell Anemia Hand-Food SyndromeSplenic CrisisInfectionsAcute Chest SyndromeDelayed growth and puberty in childrenStrokeEye problemPriapismGallstoneUlcers on the legsPulmonary Arterial HypertensionMultiple Organ FailureComplications of Sickle Cell AnemiaComplications of sickle cell anemia come from the effects of sickle cell crises on different parts of the body.Hand-Foot SyndromeWhen sickle cells block the small blood vessels in the hands or feet, pain and swelling along with fever can occur. One or both hands and/or feet may be affected at the same time. Pain may be felt in the many bones of the hands and feet. Swelling usually occurs on the back of the hands and feet and moves into the fingers and toes. This may be the first sign of sickle cell anemia in infants.Splenic (Sequestration) CrisisThe spleen is an organ in the abdomen that filters out abnormal red blood cells and helps fight infection. Sometimes, the spleen traps many cells that should be in the bloodstream and it grows large. This causes anemia. Blood transfusions may be needed until the body can make more cells and recover. If the spleen becomes too clogged with sickle cells, it can’t work normally. It begins to shrink and stop working.InfectionsBoth children and adults with sickle cell anemia have a hard time fighting infections. Sickle cell anemia can damage the spleen.Infants and young children with a damaged spleen are more likely to get infections that can kill them within hours or days. Pneumonia is the most common cause of death in young children who have sickle cell anemia. Meningitis, influenza, and hepatitis are other infections that are common in people with sickle cell anemia.Acute Chest SyndromeAcute chest syndrome is a life-threatening condition linked to sickle cell anemia. It's similar to pneumonia and is caused by an infection or by sickle cells trapped in the lungs. People with this condition usually have chest pain, fever, and an abnormal chest x ray. Over time, lung damage may lead to pulmonary arterial hypertension (see below).Delayed Growth and Puberty in ChildrenChildren with sickle cell anemia often grow more slowly and reach puberty later than other children. A shortage of red blood cells (anemia) causes the slow growth rate. Adults with sickle cell anemia often are slender or small in size.StrokeSickle-shaped red blood cells may stick to the walls of the tiny blood vessels in the brain. This can cause a stroke. This type of stroke occurs mainly in children. The stroke can cause learning disabilities or more severe problems.Eye ProblemsThe retina, a thin layer of tissue at the back of the eye, takes the images you see and sends them to your brain. When the retina doesn’t get enough blood, it can weaken. A weak retina can cause serious problems, including blindness.PriapismMales with sickle cell anemia may have painful and unwanted erections called priapism (PRI-a-pizm). This happens because the sickle cells stop blood flow out of an erect penis. Over time, priapism can damage the penis and lead to impotence.GallstonesWhen red blood cells die, they release their hemoglobin, which the body breaks down into a compound called bilirubin. When there is too much bilirubin in the body, stones can form in the gallbladder.Gallstones can cause steady pain that lasts for 30 minutes or more in the upper right side of the belly, under the right shoulder, or between the shoulder blades. The pain may happen after eating fatty meals. People with gallstones may have nausea, vomiting, fever, sweating, chills, clay-colored stool, or jaundice (yellowish color of the skin or whites of the eyes).Ulcers on the LegsSickle cell ulcers (sores) usually begin as small, raised, crusted sores on the lower third of the leg. Leg sores occur more often in males than in females and usually appear between the ages of 10 and 50. The cause of leg ulcers is not clear. The number of ulcers can vary from one to many. Some heal rapidly, but others persist for years or come back after healing.Pulmonary Arterial Hypertension (High Blood Pressure)Damage to the small blood vessels in the lungs makes it hard for the heart to pump blood through the lungs. This causes blood pressure in the lungs to increase. This condition is called pulmonary arterial hypertension. Excessive shortness of breath is an important symptom linked to this problem.Multiple Organ FailureMultiple organ failure is rare, but serious. It happens when a person has a sickle cell crisis that causes two out of three major organs (lungs, liver, or kidney) to fail. Symptoms linked to this complication are a fever and changes in mental status such as sudden tiredness and loss of interest in your surroundings.
11TreatmentsEffective treatments are available to help relieve the symptoms and complications of sickle cell anemia, but in most cases there’s no cure.The goal is to relieve the pain; prevent infections, eye damage, strokes and control complications if they occur.Pain medicine: acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs), and narcotics such as meperidine, morphine, oxycodone, and etc.Heating padsHydroxyurea, Folic AcidBlood TransfusionsTreating PainMild painful crises can be managed with treatments such as over-the-counter medicine and heating pads. However, severe pain may need to be treated in a hospital. Painful crises are the leading cause of emergency room visits and hospitalizations of people with sickle cell anemia.The usual treatments for acute (short-term) pain crises are pain-killing medicines and fluids, given either by mouth or through a vein, to prevent dehydration (a condition in which your body doesn’t have enough fluids). The pain-killing medicines most often used are acetaminophen, nonsteroidal anti-inflammatory drugs (NSAIDs), and narcotics such as meperidine, morphine, oxycodone, and others.The treatment of patients who have mild-to-moderate pain usually begins with NSAIDs or acetaminophen. If pain continues, a narcotic may be added. Moderate-to-severe pain is often treated with narcotics. The narcotic may be used alone or together with NSAIDs or acetaminophen. Narcotic abuse and addiction are pain management issues that must be considered in any pain control plan.A medicine called hydroxyurea may be given to adults and older adolescents with severe sickle cell anemia to reduce their number of painful crises. This medicine is used only to prevent these crises, not to treat them when they occur. Given daily, hydroxyurea can reduce the frequency of painful crises and of acute chest syndrome. People taking the medicine also may need fewer blood transfusions.People taking hydroxyurea must be watched carefully because the medicine can cause serious side effects, including an increased risk of dangerous infections. Some evidence suggests that long-term use of hydroxyurea can cause tumors or leukemia. Because of these risks, the medicine is usually only used in adults and older teenagers with severe sickle cell anemia. Although hydroxyurea is being tested in infants and children at this time, it won’t be approved for use in children until its long-term effects can be more closely studied.The information provided in this powerpoint is for educational purposes and should not be substituted for doctor’s advice, so consult your doctor
12PreventionIdentify what can trigger the “Crisis” such as stress, avoid extremes of heat and cold weather, don’t travel airplane that is not cabin pressurizedMaintain healthy lifestyle habitsEating healthyAvoid dehydrationExercise regularlyGet enough sleep and restAvoid alcohol and don’t smokeRegular medical checkups and treatment are important