Sickle cell disease can be fearsome but …

Dear Readers,

TODAY, I look at the problems encountered by children with sickle cell disease. It is imperative to point out at the outset that to help a child manage sickle cell disease parents should visit a doctor and share any concerns or new symptoms.

Parents should make sure their child takes all prescribed medicines. Tanzania has the third highest child birth rate of sickle cell anaemia in Africa after Nigeria and the Democratic Republic of Congo (DRC). But, few studies describe the severity of complications.

Reports on available medical treatments are also scanty. The sickle gene is most prevalent around Lake Victoria mainly in Mwanza and Kagera regions. The stark reality is that sickle cell disease (SCD) is one of the worst genetic causes of childhood mortality globally.

Statistics also show that Tanzania has one of the highest annual births of SCD individuals in the world, estimated to reach a worrisome total of 11, 000. If timely medical intervention misses, between 50 and 90 percent of the children die in childhood.

It has also been determined that children with SCD, who receive care in Tanzania are diagnosed late, hospitalized frequently and have severe complications. The effects of SCD range from causing high morbidity and mortality to reducing the quality of life.

Fortunately, cost-effective interventions have the potential to reduce childhood mortality by up to 70 percent. The disease also imposes a high socio-economic burden on individuals, families and health systems.

In the past 12 years, the SCD programme in Tanzania has developed, with local and global partnerships, a systematic framework for comprehensive research. The effort is integrated into providing healthcare, training and advocacy in SCD. 

Interventions with daily oral penicillin and pneumococcal conjugate vaccine (PCV) have significantly reduced mortality due to infections in SCD. As mentioned before, sickle cell disease is the single most important genetic cause of childhood mortality globally.

In Tanzania, blood transfusion is available for the management of severe anaemia, acute stroke and acute chest syndrome (ACS), but there is no chronic transfusion programme. Surgical services for spleen damage and other complications are provided.

An ailing child should be helped to avoid pain crisis triggers, such as extreme temperatures or stress. Adult patients should not smoke, drink alcohol, or use drugs because these can cause pain and other problems. Patients should drink lots of liquids and get enough rest.

Sickle cell disease is a condition in which red blood cells are not shaped as they should be. Red blood cells look like round discs. But in sickle cell disease, they are shaped like sickles, or crescent moons, instead. Unfortunately, these sickle shaped cells get stuck together.

Consequently they block small blood vessels. This stops blood from moving as it should, which can lead to pain and organ damage. Children (including adults) with sickle cell anemia may have jaundice (skin and whites of the eyes look yellow).

This happens because the sickle-shaped red blood cells break down faster than normal cells. People with sickle cell disease can have problems that need immediate care by a medical doctor, such as acute chest syndrome.

This health complication is caused by inflammation, infection, and blockages of small blood vessels of the lung. Signs include chest pain, coughing, trouble breathing, and fever.

A plastic crisis is another health problem which occurs when the body temporarily does not make enough red blood cells, and can cause severe anemia. Signs include paleness, extreme tiredness and a fast heartbeat.

Hand-foot syndrome is a critical health impairment which involves painful swelling of the fingers and toes. This anomaly is the first sign of sickle cell anemia in some infants. Children with sickle cell disease are at risk for some bacterial infections.

It is important for parents, guardians and others to watch for fevers of 101°F (38°C) or higher, which can be signs of an infection. Medical care should be sought right away if a fever happens.

Males, especially adults, with sickle cell disease can have painful, long-lasting erections. If it is not treated quickly, damage can cause problems with getting erections later on. The spleen traps the abnormal red blood cells and gets very large.

This can lead to a serious, quick drop in the number of red blood cells in the bloodstream. Signs include paleness, weakness or extreme tiredness, an enlarged spleen, and belly pain. Sickle-shaped cells can block small blood vessels in the brain, causing a stroke.

Signs include headache, seizures, weakness in the arms and legs, speech problems, a facial droop, or loss of consciousness. People with sickle cell disease are also at risk for problems such as leg ulcers, bone or joint damage, gallstones, kidney damage, eye damage, and delayed growth.

Sickle cell disease is a genetic condition. People who have it inherited certain hemoglobin genes from their parents. Hemoglobin is the protein inside of red blood cells that carries oxygen. Abnormal hemoglobin makes the red blood cells sickle shaped.

Someone who inherits a sickle cell gene from each parent has sickle cell disease. Someone who inherits a sickle cell gene from one parent and a normal hemoglobin gene from the other has sickle cell trait rather than sickle cell disease.

Most people with sickle cell trait don’t have symptoms, but can pass the gene to their children. Someone who inherits a sickle cell gene from one parent and another kind of abnormal gene from the other parent may have a different form of sickle cell disease.

This may be hemoglobin SC disease or sickle beta thalassemia. Sickle cell disease usually is found at birth with a blood test during routine newborn screening tests. A second blood test (called a hemoglobin electrophoresis) will confirm the diagnosis.

Sickle cell disease also might be diagnosed before a baby is born with a test on the amniotic fluid or with a sample of tissue from the placenta. Stem cell transplant (also called bone marrow transplant) is the only known cure for sickle cell disease.

Transplants are complex and risky, and for now they are an option only for some patients. Scientists are studying gene therapy for sickle cell anemia. One day, it’s hoped that doctors can stop the disease by changing or replacing the abnormal gene that causes it.

But even without a cure, children with sickle cell disease can lead fairly normal lives if they follow their treatment plan. The treatment plan might involve: Immunizations and daily doses of penicillin to help prevent infection.

Kids with sickle cell disease should get all recommended vaccinations, including the pneumococcal, flu, and meningococcal vaccines. Folic acid supplements, which can help kids make new red blood cells.

It is also imperative to mention here that signs and symptoms of sickle cell anemia vary from person to person and change over time. They include anemia. Sickle cells break apart easily and die, leaving the patient without enough red blood cells.

Red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells (anemia). Without enough red blood cells the body can’t get the oxygen it needs. This causes fatigue.

Sickle cells that block blood flow through blood vessels immediately deprive the affected organ of blood and oxygen. In sickle cell anemia, blood is also chronically low on oxygen. Chronic deprivation of oxygen-rich blood can damage nerves and organs.

The organs that are affected readily include kidneys, liver and spleen. Spleen complications occur in as many as 30 percent of patients at less than six years of age. Acute spleen damage may cause major or minor episodes.

Major episodes are life threatening, with rapid enlargement of the spleen and circulatory collapse requiring transfusion. Minor episodes also involve rapid enlargement of the spleen, but the haemoglobin reduction is less severe.

A decreased mortality rate from spleen damage may be achieved with repeated education about the problem for parents of affected infants during comprehensive clinic visits. Organ damage can be fatal. Sickle cells can block the tiny blood vessels that supply eyes.

Over time, this can damage the portion of the eye that processes visual images (retina) and lead to blindness. Acute chest syndrome is a life-threatening complication of with peak incidence in early childhood.

Sickle cell anemia can cause open sores, called ulcers, on a patient’s legs. It is also critical to know that the breakdown of red blood cells produces a substance called bilirubin. A high level of bilirubin in the body can lead to gallstones.

As hinted before, men with sickle cell anemia can have painful, long-lasting erections, a condition called priapism. As occurs in other parts of the body, sickle cells can block the blood vessels in the male sexual organ. This can damage the organ and lead to impotence.


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