Understanding Sickle cell anemia: Complications, Survival rate, Crisis and prevention

INTRODUCTION.

Sickle cell anemia is a lifelong blood disorder inherited from immediate parents. This abnormality causes a pathological condition that can lead to severe complications and death. Sickle cell anemia 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.

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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. Their broken pieces can interrupt healthy blood flow in the vessels of the limbs and organs. These problems decrease the amount of oxygen flow to relevant body organs for metabolic functions.  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. They can have more bone pains. 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.

 UNDERSTAND THE DISEASE- SICKLE CELL ANEMIA

Sickle cell anemia is a life-threatening disorder inherited directly from parents with sickle hemoglobin. People who inherited sickle hemoglobin gene from their father and normal gene from mother have sickle cell trait. People with sickle cell trait are sickle cell carriers who do not express symptoms of sickle cell anemia.

Sickle red blood cells assume abnormal, rigid, sickle shape which log-jam, stick and accumulate at the branching of the veins. The sickling occurs because of genetic mutation in the hemoglobin.  Sickle cell anemia therefore, is a genetically inherited disease from parents. It presents red blood cells in abnormal sickle or crescent shape. Parents with sickle cell (SS) and trait (AS), have 50% chance of having children of sickle cell disease and 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

This inherited abnormality causes pathological condition that can lead to severe complications and death. The disease decreases cells’ flexibility and life expectancy.  Various acute and chronic complications include stroke, renal and heart failure, abortion and retinopathy. Others are priapism, hypertension and episodes of syncope; several of which have high mortality rate. This anemia also causes bone pain known as sickle cell pain crises especially in children. 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.

 SICKLE CELL DISEASE OCCURRENCE AND SURVIVAL RATE

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

In the past, sickle cell patients die between ages 20 and 40. Organ failure and infections were responsible the deaths. 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. Close to 50% survive beyond the fifth decade with improved quality of life and reasonable health outcome.

 SIGNS AND SYMPTOMSOF 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) and shortness of breath. Rapid heart rate, dizziness and coldness in the hands and feet are reported.

There may be headaches and pale skin or mucous membranes (tissues that line the nose, mouth and other organs and body cavities).  Jaundice (a yellowish colour of the skin or whitish colour of the eyes) is among. Other signs and symptoms are sudden pains throughout the body (sickle cell crises). This often affects the bones, lungs, abdomen and joints. There may be acute pain (sudden and can range from mild to very severe and lasts from hours to a week or more).

Chronic, unbearable and mentally draining pain especially in the bones are part of symptoms of this anemia. These may restrict daily activities for weeks or months.  Reported are painful crises in the backbones, the long bones and the chest (once a month or more or once a year, lasting from hours to days).  Other signs and symptoms include painful and prolonged erection (priapism) and poor eyesight or blindness. Observed are thinking problems or confusion caused by small strokes and ulcers on the lower legs of adolescents and adults. Infections of bone (osteomyelitis), gallbladder (cholecystitis) and lung (pneumonia) are common.  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 observe these measures against this disease.  Marry a partner with laboratory confirmed status of sickle cell anemia. Sickle cell patients should avoid been dehydrated (lack of enough fluids in the body). Enough fluid minimizes 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. This could compound sickle cell anemia painful episodes.

 MANAGEMENT OF SICKLE CELL DISEASE

The goals of treatment protocol for sickle-cell disease are to manage and control its symptoms, and limit the number of crises. Sickle cell anemia has no widely available cure presently. Treatments that positively improve the anemia outcome and lower complications can help people of all ages and races. Patient with sickle cell disease need ongoing treatment, even when there is no painful crisis. It is best to receive care from expert healthcare providers at clinics designated for patients with sickle cell disease.

Sickle cell patients should join  support group where members share common experiences. This helps to relieve the stress related to this chronic and burdensome inherited disease. Make early contact with your care provider if you notice symptoms of sickle cell disease especially in children. Carriers of sickle cell disorder should use hormonal contraceptive pills under medical supervision.  They should undergo genetic counseling for informed choices before they have children. Carriers living in malaria endemic countries should receive anti-malaria chemoprophylaxis for life. Ultimately, knowledge of these basic information is needful for ameliorating the health challenges associated with sickle cell anemia

 

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