Sickle Cell Disease is a common disease that affects the blood. It leads to blood cells destruction leaving those with the condition with very low blood levels and pain crisis as a result of the abnormal shaped, sticky and rigid red blood cells.
It runs in families. Parents with the abnormal gene ’S’, can pass it on to their children. The symptoms are worse when a child inherits one abnormal sickle cell gene from each parent hence becomes ‘SS’. This is normally referred to as sickle cell anaemia.
Sickle cell disease is a major health problem in Sub-Saharan Africa. About 80% of all children born with sickle cell disease in the world are found in Sub-Saharan Africa. In Ghana, 2% of newborns have sickle cell disease.
The most commonly known symptoms of sickle cell disease are pain in the bones, which occurs from as early as four months of age with painful, swollen fingers and low blood levels as they grow requiring multiple blood transfusions.
Sickle cell disease also affects all organs of the body: brain, eye, heart, lungs, liver, spleen, kidneys, bones and skin with wounds on the legs which are very difficult to heal.
They are also prone to frequent infections which result in frequent hospitalization and poor quality of life.
Due to the frequent hospitalization children tend to miss school and this affects their academic performance, which is associated with stigmatization and depression. The economic burden on parents in having a child with sickle cell disease is enormous as a result of the frequent hospitalization and medications.
Parents may have to skip work to attend to their admitted children and this may also affect their income. The economic and psychological burden tends to put pressure on such couples and this may lead to divorce and broken homes which further worsens the psychological trauma of children with sickle cell disease.
With all the complications and frequent hospitalizations, patients with sickle cell disease mostly have shorter lifespan as compared to the general population, but this has currently improved with the identification of patients with sickle cell at birth, establishment of sickle cell clinics for regular follow-ups to prevent crisis, folic acid supplementation, adequate fluid intake and the prescription of some medications such as Hydroxyurea that have been shown to decrease pain crisis, transfusion rate and hospitalization in patients with sickle cell disease and prolongs life.
The challenge currently is that the medication is not widely used across the country due to unavailability and cost. With all the devastating complications associated with sickle cell disease, kidney damage contributes significantly to the death rate especially as they grow into adulthood.
The kidneys’ structure and function can be affected in patients with sickle cell disease in so many ways throughout their lives. Right from infancy, they have kidneys with decreased ability to concentrate their urine which worsens with time. Some children even after five years still urinate on their beds at night due to this defect. They may pass small amounts of protein in their urine in childhood which eventually increases as they grow. This affects the kidney functions leading to kidney failure and death in the long run.
The frequent use of some pain medications in pain crises also has the potential to affect the kidneys. The frequent infections can also lead to kidney damage as well as some antibiotics used for the management of the infections.
Sickle cell patients do not have the ability to adequately concentrate their urine and hence need to take a lot of fluids every day. There can be kidney damage with inadequate fluid intake with the risk of bone pain crisis for which they take pain medications and thus cause more damage. Leaking of large amounts of protein in the urine causes more destruction to the kidney and eventually kidney failure. By 30 years of age, 27% of sickle cell disease patients will have protein in their urine.
Kidney disease also occurs when sickle cell patients develop heart failure, mostly from the sickle cell disease and the chronic low blood levels, and hence cannot pump enough blood to the kidneys. Sickle cell disease patients are also at risk of red blood cell destruction, develop jaundice and pass cola-like urine. The passage of cola-like urine also destroys the kidneys. When the blood cells form sickle shape, it becomes rigid, sticky and blocks small vessels decreasing blood flow to parts of the kidney and causing death of the cells of the kidney. The dead tissue can dislodge into the urine causing obstruction or blood to be seen in the urine. The obstruction can also lead to infection in the urinary tract which may further lead to kidney disease when not promptly treated.
Blood in urine in patients with sickle cell disease is a sign of kidney disease. Passage of blood in the urine by patients with sickle cell is mostly invisible but can be visible in severe cases. Rarely older patients, blood in urine may be due to cancer of the kidneys which leads to death within two years. Kidney disease accounts for 18% of deaths in patients with sickle cell disease.
In a study among children in Ghana, 26.4% of sickle cell patients had protein in their urine and 39.6% of them had chronic kidney disease.
When kidney disease progresses to kidney failure, dialysis is required. Unfortunately, death rate in patients with sickle cell disease on haemodialysis is three times higher than those without sickle cell disease. It is therefore imperative to monitor the kidney functions of patients with sickle cell disease from childhood to prevent damage and manage them appropriately to increase their survival.
Kidney damage should be prevented as much as possible. This can be done by;
Early diagnosis of patients with sickle cell disease at birth and regular monitoring at a dedicated sickle cell clinic
Patients should be encouraged to take in adequate amounts of fluid and much more during the hot weather in the tropics
Report any bone pain or fever promptly to your doctor to prevent the use of unnecessary painkillers that can further destroy the kidneys
Patients with sickle cell disease as part of regular reviews should have their urine, blood pressure and kidney functions checked at least twice a year for any sudden changes in kidney functions.
Medications are required to decrease the rate of damage of the kidney when there is protein in the urine.
Be careful about the use of some antibiotics that have the potential to destroy the kidneys as a healthcare professional when treating sickle cell disease patients
Avoid herbal medications and medications that increases your risk of red cell destruction as they will further worsen your kidney functions
When you report on any admission, ensure the kidney functions are checked so sudden deterioration can be salvaged promptly
The use Hydroxyurea has been used potentially to decrease crisis, hospitalization and decrease protein in the urine and improve survival
When you develop cola-like urine ensure you see your healthcare provider and drink a lot of water to ensure urine is clear to prevent kidney damage.
Written by Dr. Elliot Koranteng Tannor
Senior Specialist Physician/Nephrologist – KATH
Senior Atlantic Fellow for Health Equity
Source: Daily Graphic
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