What Is Sickle Cell Anemia?

When Should You See a Doctor?


Although sickle cell anemia is usually diagnosed in infancy, if you or your child develops any of the following symptoms, see medical treatment immediately: 


·         Any signs or symptoms of stroke - If you notice any one-sided paralysis or weakness in the face, arms or legs, confusion, trouble walking or talking, sudden vision problems or numbness or headache call 91 1 or your local emergency number right away


·         Swelling in the hands or feet


·         Abdominal swelling – especially if the area is tender to touch


·         Fever - People with sickle cell anemia have an increased risk of infection, and fever can be the first sign of an illness.


·         PaIe skin or nail beds


·         Yellow tint to the skin or the whites of the eyes





Sickle cell anemia can lead to a host of complications, including:


·         Stroke - A stroke can occur if sickle cells block blood flow to an area of your brain. Stroke is one of the most serious complications of sickle cell anemia. Signs of stroke

      include seizures, weakness or numbness of your arms and legs, sudden speech

      difficulties, and loss of consciousness.  If your baby or child has any of these signs

      or symptoms, seek medical treatment immediately. A stroke can be fatal.


·         Acute Chest Syndrome - This life-threatening complication of sickle cell anemia causes chest pain, fever and difficulty breathing. Acute chest syndrome can be caused by a lung infection or by sickle cells blocking blood vessels in your lungs. It requires emergency medical treatment with antibiotics, blood transfusions and medication that open the patient’s lungs.  Recurrent attacks can cause permanent damage to the lungs. 




·         Pulmonary Hypertension -  About one-third of sickle cell anemia patients will

     eventually develop high blood pressure in their lungs (pulmonary hypertension).

     Shortness of breath and difficulty breathing are common symptoms of this condition,

     which can ultimately lead to heart failure.


·         Organ Damage - Sickle cells can block blood flow through blood vessels, immediately depriving an 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 in your body, including your kidneys, liver and spleen.

     Organ damage can be fatal. 


·         Blindness - Tiny blood vessels that supply your eyes can become blocked by sickle cells. Over time, this can damage the retina – the portion of the eye that processes visual images --and lead to blindness.


·         Gallstones - The breakdown of red blood cells produces a substance called bilirubin. Bilirubin is responsible for the yellowing of the skin and eyes (jaundice) in people with sickle cell anemia. A high level of bilirubin in your body can also lead to gallstones.


Tests and Diagnosis

A blood test can check for hemoglobin – S which is the defective form of hemoglobin that is the underlying cause of sickle cell anemia. In the United States, this blood test is part of routine newborn screening performed at the hospital. However, older children and adults can also be tested.


Assessing the Risk of Stroke

By using a special ultrasound machine called a Transcranial, Doctors can gain a better understanding of which children are at risk of having a stroke.


Blood Transfusions

Blood transfusions increase the number of normal red blood cells in circulation, helping to relieve anemia. Regular blood transfusions can reduce the risk of stroke in children with sickle cell anemia that are at high risk.   However, frequent blood transfusions also carry some risk. Blood contains iron. Regular blood transfusions can cause an excess amount of iron to build up in the body.  Excess iron in the body can cause damage to the heart, liver and other organs.  Sickle Cell patients that have regular blood transfusions must also receive treatment to reduce the excess iron levels in their body.  An oral medication called Deferasirox (Eiade) can help reduce excess iron levels in the body. It can be administered to patients older than 2 years of age. 


Supplemental Oxygen

Breathing oxygen through a mask assists in adding oxygen back into the blood.  Patients suffering from acute chest syndrome and sickle cell crisis use this form of treatment to assist in breathing.


Lifestyle and Home Remedies

Infants and children with sickle cell disease need to receive regular childhood vaccinations. Children and adults with sickle cell anemia also should have a yearly flu

shot and be immunized against pneumonia. If you or your child have sickle cell anemia,

follow these suggestions to remain healthy:


·         Take folic acid supplements daily

·         Eat a balance diet

·         Drink plenty of water.

·         Avoid extreme hot or cold temperatures

·         Reduce the amount of stress

·         Exercise regularly -  but don't overdo it

·         Use over-the-counter medications with caution

·         Fly in airplanes with pressurized cabins

·         Avoid high altitudes


Adults Struggling with Once Was a Child’s Blood Disorder

It can be said that the problems faced by sickle cell patients are a reflection of the

lack of healthcare.  Healthcare in the United States can be inadequate in meeting the needs of the poor and especially those with chronic diseases.  


In the past, the majority of Sickle Cell patients died at a young age which meant that research and treatment was focused on pediatrics.  However with the advancement of medical research, patients with Sickle Cell Disease are living much longer.  However   doctors that specialize in treating Sickle Cell Disease in infants far outweigh those that treat adults for the same disease.  Greater awareness of the disease and treatment protocols needs to be legislated by the government and implemented by healthcare professionals so that patients of all ages across the United States can receive the same level of treatment. 


If you have a child with sickle cell anemia, find a support group, educate yourself on the disease, and locate a doctor that specializes in the disease. Children with Sickle Cell Anemia Disease have special needs and require regular medical care.


Currently there are no Sickle Cell Centers, or Specialists focusing on Sickle Cell Anemia Disease in adults located in the state of Oklahoma.