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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.


Pulmonary Infarction

Infarction means 'tissue death' in medical terminology. It is caused by obstruction of blood supply to the tissue leading to lack of oxygen. Infarct, which refers to the resulting lesion is derived from Latin, 'infarctus' which means 'stuffed into'. Pulmonary infarction refers to the death of a small area of lung resulting from pulmonary embolism. It occurs in a small, dead end pulmonary artery.


How does pulmonary infarction occur ?

Pulmonary infarction results from free floating thrombus, when many material substances including fat, tumor, septic emboli, air, and amniotic fluid and injected foreign material may form an emboli and move to the pulmonary circulation. In other words, plugging of a branch of the pulmonary artery by a clot (thrombosis) or by a piece of clot carried by the blood stream to the lung from a thrombus located elsewhere can result in pulmonary infarction. The involved area of the lung ceases to function and complication of the thrombosed veins leads to heart disease.


Causes of pulmonary infarction

The most common cause of pulmonary infarction is pulmonary embolism, but there are other conditions which can cause pulmonary infarction including cancer, and autoimmune diseases such as lupus. Sometimes, this condition may occur following a surgery. Other underlying conditions especially in children with pulmonary emboli include sickle cell disease, nephrotic syndrome, chemotherapy and Inherited hyper coagulable state and Vasculitis.


Symptoms of pulmonary infarction

Symptoms associated with pulmonary infarction include shortness of breath, chest pain, and blood sputum or hemoptysis. Sudden piercing pain in the chest which often radiates to the shoulder is noticed. Difficulty in breathing, irritating cough and blood tinged sputum are other signs. Persistent hiccups are present. Most often the patient is anxious with a rapid pulse, sweats profusely and has an elevated body temperature. In some severe cases, the patient may be in a state of shock.


Diagnosis of pulmonary infarction

Reflex broncho constriction is often associated with pulmonary embolism. Increased breathing and decreased pulmonary compliance with diminished surfactant levels may occur due to pulmonary infarction. This contributes to increased work of breathing and diminished oxygen levels. In sickle cell disease, there is sickling of RBCs within the small blood vessels of the lungs due to dehydration as a result of fever, Tachypnea (rapid breathing) and decreased intake, which can precipitate in a cycle of relative de oxygenation that further exacerbates the sickling tendency. Many also suffer a component of reactive airway disease and oxygenation is further decreased due to this factor.


Morbidity may include pulmonary hypertension, right ventricular failure and Cor Pulmonale, paradoxical embolization in patients with intracardiac defects, and sometimes side effects of medications used to treat pulmonary embolism. If pulmonary embolism is large, there could develop right ventricular strain and right heart failure as there is sudden increase in pulmonary artery pressure leading to right heart failure. A sudden pressure in the right ventricle can cause a leftward shift of the intraventricular septum, which may result in a classic obstructive shock, thereby impairing left ventricular filling.


Treating pulmonary infarction

It is timely treatment that is vital. If symptoms of pulmonary infection develop while at home, consult a physician at once. In case, shock develops, it is essential to get first aid treatment. Many times, patients are already in the hospital when pulmonary infarction occurs. Administration of oxygen, use of anticoagulants and prevention of infection are some other suggested line of treatment. Surgery may also be indicated.


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Collection of Pages - Last revised Date: November 22, 2019