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Sickle Cell Anemia

Sickle cell anemia is a hereditary condition characterized by destruction of red blood cells in the body. It is noticed that sickle cell anemia affects people of African ancestry and those of Mediterranean and Middle Eastern descent. The soft round red blood cells develop an abnormal shape that resembles a sickle or crescent moon. Their shape stops them from flowing easily and they get clogged up inside smaller blood vessels. This stops the blood from flowing properly. Important organs like the brain, heart, or kidneys need constant blood flow to stay healthy. Although our body attacks and destroys these sickle cells, it can't make new blood cells fast enough to replace the older ones. This results in the decrease in number of red blood cells leading to anemia. Sickle cell anemia is generally caused by genetic defects or disease. A child with sickle cell anemia has inherited a defective hemoglobin gene.


Sickle shaped blood cells get stuck within the small blood vessels and obstruct blood flow to some parts of the body, thereby leading to pain. Symptoms of sickle cell anemia include fatigue, breathlessness, rapid heart rate, delayed growth and puberty, susceptibility to infections, excessive penis pain, chest pain and decreased fertility. Since the spleen is damaged by the sickle cells, the resistance to infections is reduced considerably. Hands and feet tend to swell (Hand-foot syndrome) and the body faces stunted growth. Retinal damage can occur due to the obstruction of blood flow to the eyes. In about 10% of the cases, a stroke can occur if the sickle cells block blood vessels in the brain. Acute chest syndrome is another complication of this form of anemia. In this condition, the patient suffers symptoms similar to pneumonia and will need to be treated in a hospital.


There is no cure for sickle cell anemia but treatment can be followed to prevent complications and improve the quality of life of the patient. The patient will need to be under the guidance and care of a hematologist or genetic counselor. Blood test for hemoglobin 5 gives an indication as to the presence of this disease. A microscopic analysis of a blood sample will exhibit the distinctly shaped sickle cells. Bone marrow transplant can offer potential cure for this form of anemia, provided the right donor is found. Red blood cells from a donor can be given intravenously to a patient. But it carries inherent risks. Penicillin is given to keep young affected kids from life-threatening infections. Painkillers like acetaminophen and ibuprofen can relieve the pain associated with sickle cell anemia. Children diagnosed with sickle cell anemia must be given regular childhood vaccinations as well as Hib (Hemophilus Influenzae B) vaccine and the pneumococcal vaccine.


Anemia

Anemia stands for 'without blood' in Greek; When the number of red blood cells (RBC) falls below normal, Anemia is a resultant condition. Hemoglobin is an important constituent of RBC. Hemoglobin usually occurs in the range of 12 and 18 g/dL (grams per deciliter of blood). If the hemoglobin levels show a decrease, anemic conditions set in. Consequently, the various organs and tissues of the body do not receive adequate oxygen on account of the diminished oxygen carrying capacity of the blood. This impairs their normal functioning. Usually women have smaller stores of iron than men. Besides, they also lose blood during menstruation making them primary targets for anemia.

Red Blood Cells

World Health Organization (WHO) defines anemia as a hemoglobin level lower than 13 g/dL in men and lower than 12 g/dL in women. It is essential to be familiar with the typical symptoms of anemia. Often anemia is misdiagnosed and left untreated. An anemic person is likely to feel extremely tired and weak. This is accompanied with dizziness and breathlessness. A person suffering from anemia tends to appear pale and experience feelings of depression. In some cases, anemia can lead to heart ailments too.

Causes of Anemia


  • Serious disease or infection such as hookworm infection, bleeding piles, esophageal var ices and peptic ulcers.
  • Hemorrhagic - Excessive blood loss due to surgery, menstruation or injury.
  • Genetic defects lead to sickle cell anemia, Thalassemia anemia and aplastic anemia.
  • Hemolytic - Excessive intravascular blood destruction where red blood cells are destroyed prematurely.

Types of Anemia

Iron deficiency Anemia - Nearly 20% adult women tend to suffer from this form of anemia. Loss of blood due to menstruation is not compensated with an iron-rich diet. Pregnancy and breast feeding can also deplete iron stores. Iron deficiency anemia is also noticed during growth spurts or internal bleeding.


Aplastic anemia - When the bone marrow does not produce sufficient quantities of blood cells, aplastic anemia is noticed. Childhood cancers such as leukemia are often responsible for this form of anemia. Other possible causes of aplastic anemia are radiation, cancer or antiseizure medications and chronic diseases such as thyroid or kidney malfunction. Treatment for aplastic anemia involves blood transfusions and bone marrow transplant. This is done to replace malfunctioning cells with healthy ones.


Vitamin deficiency anemia - Low levels of folic acid lead to faulty absorption of iron. Anemia caused due to folic acid deficiency is called Megaloblastic anemia. Pregnancy doubles the body requirements of folic acid and it is imperative that pregnant women take folic acid supplements. Good dietary sources of folate are fresh fruits, green leafy vegetables, cruciferous vegetables, liver and kidney, dairy products and whole grain cereals. Vegetables should be eaten raw or lightly cooked.Folic acid anemia is also a common problem faced by alcoholics. Vitamin B-12 deficiency can lead to a condition of Pernicious anemia. Diseases such as thyroid malfunction or diabetes mellitus can affect the body's ability to absorb vitamin B-12. This vitamin is vital in the production of hemoglobin.

Vitamin C Deficiency Anemia is a rare form of Anemia that is the result of small red cells owing to prolonged dietary deficiency of the Vitamin C.


Sideroblastic Anemia: In this anemia, the body has sufficient iron but it fails to incorporate it into hemoglobin.


Hemolytic Anemia results from high rate of destruction of Red Blood Cells (RBC) at a rate faster than the rate bone marrow can replenish them.


Thalassemia anemia - Thalassemia or Cooleys Disease is a hereditary disorder found predominantly in people of South East Asian, Greek and Italian racial groups. This form of anemia is seen in differing degrees as Thalassemia encompasses a group of related disorders that affect the human body in similar ways. The most common occurrences of Thalassemia are alpha and beta thalassemia. Thalassemia anemia is characterized by symptoms like jaundice, enlarged spleen, shortness of breath and facial bone deformities.


Diagnosing Anemia

A complete blood count test will test for hemoglobin levels and display an anemic condition. But often anemia is a symptom whose cause lies deeper. The cause and type of anemia will determine the treatment that is needed. A stool test will help in detecting occult blood. Hemoglobin electrophoresis is a blood test that helps identify abnormal hemoglobins. Diagnosing thalassemia or sickle cell anemia becomes possible with this test.

Treating Anemia

Deficiency can be treated with supplements of iron, Vitamin B-12 and Vitamin C. Partaking an iron-rich diet can be beneficial for those suffering from nutritional deficiency anemia. Seafood, nuts, whole grains and dried fruits such as raisins, prunes and apricots are rich in iron. Ensure adequate consumption of Vitamin C as it aids and stimulates iron absorption. Try and combine citrus foods with iron-rich foods - add tomatoes to a turkey sandwich or chopped strawberries with iron-fortified breakfast cereals.


Gallstones

The gall bladder is a small pear shaped organ on the underside of the liver that is used to store bile. When partially digested food passes from the stomach into the small intestine, the gall bladder expels bile to aid digestion. While gallstones usually form in the gallbladder; they can be found in bile; in the intrahepatic, hepatic, common bile, and cystic ducts. Cholelithiasis as Gallstones are called, often do not cause any symptoms. Gallstones are hard masses formed in the gallbladder or its associated passages. They are often discovered when having a routine x ray, abdominal surgery, or abdominal ultrasound. Cholangiography - radiographic examination of the bile duct is performed after injecting with a special dye to check the condition of Cholangitis - infection or inflammation of the bile ducts. Cholangitis manifests in the form of severe abdominal pain, fever and Jaundice.


When gallstones move from the gallbladder into the cystic duct of common bile duct (Choledocholithiasis), it can lead to a severe cramping pain in the upper right abdomen which can radiate to the right shoulder as a result of the blocked bile flow. This can last from a few minutes to a few hours. Cholesterol gallstones are more often noticed in women who are obese, on HRT or with elevated blood triglyceride. Black pigment gallstones are usually formed when there is increased destruction of RBC. Others who are likely to suffer gallstones are those suffering liver cirrhosis and biliary tract infections or sickle cell anemia. Gallstones can form when the gallbladder does not empty bile properly. Blood tests to check bilirubin, liver function test and pancreatic enzymes are often ordered. In cases of severe painful episodes, Cholecystectomy is performed.


Gallbladder diet

People suffering from gall bladder disease should follow a low fat diet for healthy living. The bile from the gall bladder is necessary for proper digestion of fats. When there is no bile or not enough bile is produced by the gall bladder, fat from food cannot be properly digested and makes one feel ill or causes diarrhea. Hence a long-term dietary therapy is strongly recommended for people who have had gall bladder surgery. If your gall bladder is diseased, a low fat diet reduces stimulation of the organ and allows your gall bladder adequate rest. This eating plan also helps to prevent painful spasms of the bile duct.


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Collection of Pages - Last revised Date: October 19, 2017