disease have red blood cells that contain mostly hemoglobin S, an irregular type of hemoglobin, unlike normal red blood cells that contain hemoglobin A. Usually, red blood cells live around 120 days before new ones replace them; but red blood cells containing mostly hemoglobin S only live about 16 days. Normal red blood cells are round and flexible and can squeeze through the smaller blood vessels. While blood cells that contain too much hemoglobin S tend 
to become stiff, distorted in shape (like a sickle, hence the name) and have difficulty passing through the body’s small blood vessels. Because of the difficulty of passing through the blood vessel they start to cause a blockage in the small blood vessels, resulting in less blood reaching that part of the body. When tissues do not receive the proper amount of blood flow it ultimately becomes damaged. This is where the complication of Sickle Cell appears.Symptoms:
Infants. In infants, symptoms do not usually appear until late in the baby's first year. Usually they include:
- Fever
- Swelling of the hands and feet
- Pain in the chest, abdomen, limbs, and joints
- Nosebleeds and frequent upper respiratory infections
Children. Pain is the most common complaint. It can be acute and severe or chronic, usually from orthopedic problems in the legs and low back. Other symptoms include:
- Fatigue and shortness of breath (signs of anemia)
- Irritability
- Jaundice (yellowish discoloration of the skin and eyes)
Additional Symptoms in Adolescence or Adulthood. Symptoms of childhood continue in adolescence and adulthood. In addition, they experience:
- Delayed puberty (in young teenagers)
- Severe joint pain
- Progressive anemia
- Leg sores
- Gum disease
- Vision problems
Gene:
Mutations in the HBB gene cause sickle cell disease. Hemoglobin consists of four protein subunits, typically, two subunits called alpha-globin and two subunits called beta-globin. The HBB gene provides instructions for making beta-globin. Various versions of beta-globin result from different mutations in the HBB gene. One particular HBB mutation produces an abnormal version of beta-globin known as hemoglobin S (HbS). In people with sickle cell disease, at least one of the beta-globin subunits in hemoglobin is replaced with hemoglobin S.
This condition is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.
Diagnosis:
Blood tests can determine whether an individual has sickle cell trait or sickle cell disease.
Screening Tests for Newborns
In the United States, hospitals routinely screen newborn babies for sickle cell disease. To perform the test, a blood sample is taken from the baby's heel using a simple needle prick. Early detection of sickle cell disease can help reduce the risk for life-threatening infections and increase the odds for survival. Babies who are diagnosed with sickle cell disease are given daily antibiotics to help prevent infections.
Prenatal diagnosis is also possible through amniocentesis. The amniotic fluid is tested for the presence of the sickle cell gene.
The Environment and Sickle Cell:
The environment can have effects on patients with Sickle Cell. The biggest being the air we breath in. Smoking, factories, car, toxins, and many others pollute our air. Some people are more prone to feel the effects than others because of where they live.
I believe that by getting rid of smoking, harmful toxins in products, and harmful energy sources, such as coal. By doing this we can make the environment cleaner for those with not only Sickle Cell, but many other diseases.
