Understanding the disorder/disease – Sickle cell anemia. Complications, Survival rate, Crisis prevention

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Sickle cell anemia is a lifelong blood disorder inherited from immediate parents. This inherited abnormality causes a pathological condition that can lead to severe complications and death. Sickle cell anemia is present at birth, but many infants do not show any signs until after 4 months of age.  Know and promptly report the incidence of sickle cell anemia for easy management.

Sickle cell anemia is a blood disease that alters the oxygen carrying capacity of hemoglobin and the shape of red blood cells. Hemoglobin is a protein inside the red blood cells and it carries oxygen to body cells and tissues.  Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin S changes the shape of red blood cells. The red blood cells become shaped like crescents or sickles. The fragile and sickle-shaped red cells deliver less oxygen to the body’s tissues. They can also form plaques easily in small blood vessels as well as break into pieces that can interrupt healthy blood flow in the blood vessels of the limbs and organs. These problems decrease the amount of oxygen flowing to the relevant body organs for metabolic functions. Also blocked blood flow can promote pain, organ damage and risk of infections. A peculiar concern is that women with this disease and use hormonal birth control may have their blood vessels blocked by blood clots or have more bone pains.

UNDERSTAND THE DISEASE- SICKLE CELL ANEMIA

Sickle cell anemia is a life-threatening blood disorder inherited directly from parents with sickle hemoglobin. People who inherited a sickle hemoglobin 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 are sickle cell carriers who do not have the symptoms of sickle cell anemia or disease.

People who have the disease are born with it. They inherit two genes of sickle hemoglobin—one from each parent. Sickled red blood cells assume an abnormal, rigid, sickle shape which do log-jam, stick and accumulate at the branching point inside the vein. The sickling occurs because of genetic mutation in the hemoglobin.  Sickle cell anemia therefore, is a genetically inherited disease from parents and it presents red blood cells in an abnormal sickle or crescent shape. If one parent has sickle cell anemia (SS) and the other has sickle cell trait (AS), then there is a 50% chance of a child having sickle cell disease and a 50% chance of a child having sickle cell trait. When both parents have sickle cell trait a child has a 25% chance of sickle cell disease.

COMPLICATIONS OF SICKLE CELL DISEASE

The term disease is applied, because this inherited abnormality causes a pathological condition that can lead to severe complications and death. Sickling decreases cells’ flexibility, results in increased risk of various complications and decreases life expectancy.  Sickle cell disease may lead to various acute and chronic complications such as stroke, renal and heart failure, abortion, retinopathy, priapism, hypertension and episodes of syncope; several of which have high mortality rate. This type of anemia also causes bone pain known as sickle cell pain crises. Most episodes of sickle cell crises last between five and seven days. Recurrent crises can damage the bones, kidneys, lungs, eyes, heart or liver especially in adults, and severe abdominal pain especially in children.

SICKLE CELL DISEASE OCCURRENCE AND SURVIVAL RATE

Sickle cell disease is a global health burden which occurs more commonly in people (or their descendants) from parts of tropical and sub-tropical sub-Saharan regions where malaria is or was common. In areas where malaria is common, there is a fitness benefit in carrying only a sickle cell trait.

In the past, sickle cell patients die between ages 20 and 40 as a result of organ failure and infections. The better understanding and management of the disease today make patients live into their 50s or beyond. In most instances, about 90% of patients survive to age 20, and close to 50% survive beyond the fifth decade with improved quality of life and reasonable health outcome.

SIGNS AND SYMPTOMS OF SICKLE CELL ANEMIA

The signs and symptoms of sickle cell anemia vary. Some people have mild signs and symptoms. Others have very severe signs and symptoms which often lead to hospitalization for treatment. Sickle cell anemia is present at birth, but many infants do not show any signs until after about 16 weeks of age. The most common signs and symptoms are associated with anemia, pain and sickle cell anemia complications. They include fatigue (feeling and appearing tired or weak), shortness of breath, rapid heart rate, dizziness and coldness in the hands and feet. There may be headaches, pale skin or mucous membranes (tissues that line the nose, mouth and other organs and body cavities) and jaundice (a yellowish colour of the skin or whitish colour of the eyes). Other signs and symptoms are sudden pains throughout the body (sickle cell crises) which often affect the bones, lungs, abdomen and joints, and acute pain (sudden and can range from mild to very severe, usually lasts from hours to a week or more).

There are signs and symptoms of chronic pain (especially in the bones, and can be unbearable, mentally draining and may restrict daily activities for weeks or months), painful crises in the bones of the back, the long bones and the chest (once a month or more or once a year, lasting from hours to days).  Other signs and symptoms of  sickle cell anemia include painful and prolonged erection (priapism), poor eyesight or blindness, problems with thinking or confusion caused by small strokes and ulcers on the lower legs (in adolescents and adults). Bone infection (osteomyelitis), gallbladder infection (cholecystitis), lung infection (pneumonia), urinary tract infection, painful joints caused by arthritis, and delayed growth and puberty are commonly observed.

SICKLE CELL DISEASE/CRISIS -PREVENTIVE MEASURES

It is very important for people to avoid these: marriage to a partner without laboratory confirmation of a partner’s sickle cell status. Sicklers should avoid been dehydrated (lack of enough fluids in the body) so as to minimize the risk of sickle cell crisis that could cause multiple organ failure or damage. Injuries that can result to ulcers which persist for years or come back after healing should not be permitted so as not to compound sickle cell anemia painful episodes.

MANAGEMENT OF SICKLE CELL DISEASE

The goal of treatment protocol for sickle-cell disease is to manage and control its signs and symptoms, and to limit the number of crises. Sickle cell anemia has no widely available cure presently. However, treatments that positively improve the anemia outcome and lower complications can help to check the symptoms and complications of the disease in both children and adults of all races. Patients with sickle cell disease need ongoing treatment, even when they are not having painful crisis. It is best to receive care from expert healthcare providers and clinics that major on taking care of many patients with sickle cell disease. Sickle cell patients should join sickle cell anemia – support group where members share common experiences which help to relieve the stress related to caring for this chronic and burdensome inherited disease. Make an early call to or contact with your healthcare provider if you notice the signs and symptoms of sickle cell disease especially in children. Carriers of sickle cell disorder should use hormonal contraceptive pills under medical supervision and undergo genetic counseling for informed choices before they have children. Sicklers living in malaria endemic countries should receive anti-malaria chemoprophylaxis for life.

Ultimately, there is need to be armed with the knowledge of these basic health information so as to know what to do in order to ameliorate the health challenges associated with sickle cell anemia.

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