In case you were wondering...

Here is a good explanation of sickle cell disease.

What is sickle cell disease?

Sickle cell disease is the name of a group of inherited blood disorders that is characterized by chronic anemia, periodic episodes of pain and other complications.
The disease affects the red blood cells. Sickle cell disease results when red blood cells make mostly hemoglobin S instead of the normal hemoglobin A. Normal red blood cells are smooth, round and soft; their shape and softness enable them to move easily through the blood vessels to carry oxygen to all parts of the body.
In sickle cell disease, the abnormal hemoglobin S causes the red blood cells to become hard, pointed and sticky and shaped like crescents or sickles. When these hard and pointed red cells go through the small blood vessels, they often get stuck and block the vessels. They also stick to and damage the lining of larger blood vessels. The damaged vessels become scarred, narrowed and sometimes completely closed. The blockage of blood flow through blood vessels can lead to pain, stroke and damage to many organs.
Sickle cells also break up too easily and survive for only 10 to 20 days compared to 120 days for normal red blood cells. The rapid destruction of sickle cells leads to anemia — a shortage of red blood cells and hemoglobin. It becomes difficult for the body to make new red blood cells fast enough to keep up with the needed supply.


Infections


Infections are a major complication of sickle cell anemia. In fact, pneumonia is the leading cause of death in children who have sickle cell anemia. Other common infections linked to sickle cell anemia include meningitis, influenza, and hepatitis.

If a child who has sickle cell anemia shows early signs of an infection, such as fever, seek treatment right away.

To prevent infections in babies and young children, treatments include:
Daily doses of penicillin. Treatment may begin as early as 2 months of age and continue until the child is at least 5 years old.

All routine vaccinations (such as a yearly flu shot) plus the meningococcal vaccine. Adults who have sickle cell anemia also should have flu shots every year and get vaccinated for pneumonia.


Eye Damage

Sickle cell anemia can damage the blood vessels in the eyes. Parents should ask their child's doctor about regular checkups with an eye doctor who specializes in diseases of the retina. The retina is a thin layer of tissue inside the back of the eye. Adults who have sickle cell anemia also should have regular checkups with an eye doctor.

Strokes
Stroke prevention and treatment is now possible for children and adults who have sickle cell anemia. Starting at age 2, children who have sickle cell anemia often get routine ultrasound scans of their heads. This is called transcranial Doppler ultrasound. These scans are used to check blood flow in the brain.
The scans allow doctors to find out which children are at high risk for a stroke. These children are then treated with routine blood transfusions. This treatment has been found to greatly reduce the number of strokes in children.

Treating Other Complications

Acute chest syndrome is a severe and life-threatening complication of sickle cell anemia. Treatment usually requires hospitalization and may include oxygen, blood transfusions, antibiotics, pain medicine, and checking the body's fluids.


How is sickle cell disease treated?

The goals of treatment are to prevent infections, relieve pain, and prevent or control complications. Infections are the leading cause of death in sickle cell disease. Pain attacks are the leading cause of emergency room visits and hospitalizations.


Because infections may lead to death, special measures are taken to prevent or reduce the severity of infections. This includes twice-daily penicillin for children for at least the first five years of life, special vaccines, and aggressive evaluation and treatment when patients develop fever or other signs of infection.

Vaccinations against certain dangerous bacteria (pneumococcus, haemophilus influenza type B, and meningococcus) and viruses (hepatitis B and influenza) are especially important for people with sickle cell disease. Blood transfusions are used to treat some acute complications and to prevent the occurrence or recurrence of other complications. Children who have had a stroke or are at high risk for initial stroke are given blood transfusions on a regular and long-term basis to prevent stroke.


Pain is most often treated with acetaminophen, nonsteroidal anti-inflammatory drugs (also known as NSAIDs) and opioids, which are the strongest pain medications and include substances such as morphine, oxycodone and hydrocodone.