Sickle Cell Anemia

Sickle Cell Anemia General Information

Blood diseases are usually persistent, causing severe damages to organs and tissues as a result of the lack of oxygen and nutrients. A chronic blood disorder characterized by abnormal, rigid and sickle shape of the red blood cells is Sickle Cell Anemia, also known as hemoglobin SS disease (or Hb SS). A normal red blood cell is shaped like a biconvex disc and moves easily through the blood vessels carrying oxygen and other necessary substances to all parts of your body. The unusual appearance of the red blood cells decreases their flexibility and restricts their movement through blood vessels. The sickle cells get stuck in blood vessels, interfering in the blood flow; furthermore, they do not live as long they should, causing anemia and pain.

People diagnosed with this type of medical condition have often a good health status, but they are at high risk of periodic painful attacks and numerous severe complications. Sickle Cell Anemia is an inherited medical disorder (it represents an autosomal recessive trait) and occurs in persons (or in their descendents) who live in parts of sub-Saharan Africa where malaria is very common. This blood disease is caused by a genetic problem. A person suffering from this disease is born with two sickle genes (one inherited from each parent). Most people who have Sickle Cell Anemia can not be cured. However, treatment options aim to prevent further problems that are associated with this type of anemia and to relieve pain caused by damaged organs and blocked blood vessels.

Sickle Cell Anemia Symptoms

Sickle Cell Anemia is an inherited blood disorder that is present at birth, but its signs and symptoms usually do not occur until the newborn is five months of age. The manifestations of this medical condition are experienced by almost all the patients and range from mild and moderate (in the early stages) to severe (in the advanced stages). The ways these symptoms manifest depend a lot on the immune system of patient. Patients diagnosed with Sickle Cell Anemia will usually accuse one or more of the following: fatigue, bone pain, paleness, delayed growth and delayed puberty, attacks of abdominal pain, jaundice, breathlessness, fever, susceptibility to infections, rapid heart beat or ulcers on the lower part of legs. Other less common signs and symptoms are experienced only by few patients and include: pain felt in the chest area, blindness or poor eyesight, hematuria (presence of blood in urine), frequent urination, excessive thirst and priapism (painful erection).

Sickle Cell Anemia may become life threatening in time, if it is not discovered in the early stages. In the advanced stages of the condition the patient deals with acute painful episodes, called crises. There are three types of crises: hemolytic, splenic sequestrations and aplastic. These crises of pain can last hours or even days, affecting different organ systems. If you still have questions regarding this chapter, we recommend you to contact your health care provider or any other trained specialist.

Sickle Cell Anemia Treatment

Blood disorders, especially those caused by genetic abnormalities are harder to treat or even impossible to cure. The main aims of any treatment option are to relieve the painful signs and symptoms and to prevent further severe complications. So, an early diagnosis followed by the proper therapy can accelerate the process of healing and stop the disease from reaching more advanced and serious stages. Sickle Cell Anemia is easily detected after five months of age, when the signs and symptoms appear. In order to rule out other possible conditions and to confirm the diagnosis, health care providers must perform several tests, for example: sickle cell test, CBC (complete blood count), hemoglobin electrophoresis etc. Other additional exams may reveal decreased amounts of oxygen in the blood, elevated bilirubin, high serum potassium and creatinine and blood in the urine. If these tests show the patient is suffering from Sickle Cell Anemia, the health care provider must proceed immediately with the adequate treatment option.

All the treatments are applied to manage and control the symptoms and to try to minimize the risk of crises. When dealing with this type of blood disorder, the patients need to make regular visits to their health care providers to monitor the health status and to check the red blood cells count. Transplant of bone marrow is considered the only potential cure for Sickle Cell Anemia, but there are very few suitable donors for the transplant. People diagnosed with this medical disorder need continuous therapy, even if the painful crises are gone. Supplements with folic acid (an important element in producing the red blood cells) are absolutely necessary because of the rapid blood cells turnover. During the painful crises specialists will administrate sufficient IV fluids and certain medicines, especially analgesics. Over-the-counter pain-relieving medications are prescribed, as well as applications of heat to the affected areas.

Because the pain is often critical, patients may require narcotics (stronger painkillers). Antibiotics, for example Penicillin are prescribed to newborn babies until they are six years old. This helps prevent infections that can be very dangerous for an infant, for example pneumonia. Another prescription drug is Hydrohyurea, prescribed as Hydrea or Droxia. This medication is usually used to treat cancer, but it can also be administrated to adult patients with severe medical disorders. If it is taken day by day, the crises’ frequency is reduced and people do not need blood transfusions. Other treatment options used for Sickle Cell Anemia include blood transfusions and supplemental oxygen. Blood transfusions are extremely useful due to the fact that they increase the number of normal red blood cells in circulation and help correcting the anemia. These procedures carry some risks. Because blood contains iron, regular blood transfusions cause excessive amounts of iron in your organism. This can cause damage to the liver, heart and other major organs. The first oral medicine that was able to reduce the excessive levels of iron is Deferasinox, prescribed as Exiade; it was released in 2004. People with Sickle Cell Anemia need supplemental oxygen to prevent acute chest syndrome.