Sickle Cell Anemia

Also known as Sickle Cell Disease

A Little Bit Of History

Even though, Sickle Cell Anemia or SCD, has been present in Africa for thousands of years going by many tribal names, SCD was first observed by James B. Herrick in 1910. A man named Walter Clements Noel came to Dr. Herrick complaining about episodes of pain and symptoms of anemia. Herrick was a cardiologist and didn't have any interest in his case. So, he assigned Dr. Ernest Irons to the case. Dr. Iron looked at Noel's blood under a microscope, and noticed the "sickles" in his blood. When Herrick saw it on the patient's chart, he became interested in it. He saw it as a new undiscovered disease to investigate.


There have been many people who have contributed to the research of SCD. For example, Hahn & Gillespie found out that red blood cells turn into sickles when you take away oxygen. Dr. Linus Pauling & his colleague found out that the hemoglobin in SCD patients had different chemical structures. The disease gets its name from the sickle or crescent moon shaped red blood cells.

The Info Spot

SCD is an autosomal recessive disease which means you have to get it from both parents. In other words, it is an inherited disease. If you inherit only one copy of the disease, you have a condition called sickle cell trait. SCD is diagnosed by a blood test called Hemoglobin Electrophoresis. It detects the different types of hemoglobin in your blood. Hemoglobin is the protein molecule found in red blood cells.

Newborns are also tested for SCD or sickle cell trait. Doctors take blood from a heel prick, and collect it in spots on special paper. A pregnant woman can also do a prenatal screening for SCD. It is done by taking a sample of amniotic fluid or tissue taken from the placenta. Amniotic fluid is the fluid surrounding the embryo. This test is used to identify the sickle cell trait, not SCD.

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Sickle Cell Disease

Symptoms

If you have or know someone who has SCD, you may know some of these symptoms.


  • Pain Crises (Episodes of Pain that happen suddenly)
  • Acute Chest Syndrome (Severe chest and abdominal pain, fever, cough, and trouble breathing)
  • Jaundice (Yellow color in the whites of the eyes and skin)
  • Leg Ulcers (A sore that develops in the tissue of an open wound)
  • Swelling in Hands and Feet
Most people who have SCD have some degree of anemia. They will probably get tired faster than others, fast pulse, shortness of breath, and paleness of skin. People with SCD will also have frequent infections because of their immune system is weak.

Communication of Complications

Almost every disease or condition has some complications, and SCD is no exception. SCD comes with a host of complications. These complications include:


  • Severe Anemia
  • Hand-Foot Syndrome (Redness, pain, and swelling on the palms of hands and/or feet)
  • Splenic Sequestration (Acute condition that involves large amounts of blood pooling within the spleen)
  • Eye Problems (Blurry vision, Blindness, etc.)
  • Priapism (Classified as pain in the groin area; Only happens to males)
  • Gallstones (Hardened deposits in the gallbladder)
  • Organ, Bone, and Joint Damage

Prognosis and Treatment.....

In 1973, the average lifespan for a person with SCD was 14 years. A short time isn't it? Thanks to the new and aggressive treatments, a person with SCD can reach 50 years and beyond. Unfortunately, there is not a cure for SCD, but there are different treatments you can consider. Keep in mind the treatments you can have depend on the severity of your SCD. You could get a blood transfusion, chemotherapy, vitamins, narcotics(or prescribed drugs), or you could see a specialist. Supplemental oxygen and bone marrow transplants are also possible treatments, as well as antibiotics are given. All of these treatments help out with the condition. For example, narcotics help out with the pain crises.

Current Events

There is a new procedure for stem cell transplants. Stem cells produce new healthy blood cells. The University of Illinois Hospital and Health Sciences System has been able to cure 12 adult patients with SCD. The success rate is eight-seven percent. The technique used eliminates the need for chemotherapy to prepare the patient. The patient's way of life improves greatly improves in just one month. In the procedure, patient receive immunosuppressive drugs just before the transplant, and a very low dose of total body irradiation. Irradiation is a more gentle treatment than chemotherapy, and it has less side effects. Donor cells come from a healthy and tissue-matched sibling. After the procedure is finished, the patient is supposed to keep taking the immunosuppressant drugs for at least a year.

This is considered to be a cure for SCD. Gene therapy is still a long distance away, but there are some researchers who are hopeful.

Our Story: Living with and Managing Sickle Cell Disease (Nicholas H.)

Impact on the Patient and Family

Having a child or family member can have an impact on family dynamics. There will be additional emotional strain and conflicts in a family. A parent or sibling may not be able to do certain things because of the child affected with SCD. The diagnosed person may have behavioral and emotional problems. They may also have low self-esteem and feeling of resentment towards unaffected people. A person with SCD will face many limitations such as not being able to participate in some activities. Even with the limitations, people live happy and meaningful lives.

Facts about the Sickle...

  • Sickle Cell Disease primarily affects black people, but it is present in people of Grecian, Mediterranean, Hispanic, South Asian, Italian, and South american descent.
  • It is not contagious. You have to inherit the disease.
  • The shape of a sickled red blood cell is not very flexible, and can get stuck in the circulatory system.
  • It is a disease of the blood.
  • Sickle red blood cells are destroyed quickly so the body does not have enough time to make new healthy ones.
  • There are several types of sickle cell disease, but the most common types are Sickle Cell Anemia (SS) and Sickle-Hemoglobin Disease (SC)