Sickle cell disease (SCD), also called sickle cell anemia, is a serious health condition that runs in families. Sickle cell disease is a group of inherited red blood cells disorders. Symptoms of sickle cell disease vary. In some people, they are mild, in others severe. But many people are able to have a very good quality of life by learning to manage the disease.
Sickle cell disease changes normal, round red blood cells into cells that can be shaped like crescent moons. The name “sickle cell” comes from the crescent shape of the cells. (A sickle is a tool with a crescent-shaped blade.)
Normal red blood cells move easily through your blood vessels. They take oxygen to every part of your body. But cells with sickle shape can get stuck and block blood vessels. This stops the oxygen from getting through causing a lot of pain. But, once oxygen is able to reach your cells, they return to their normal round shape.
There are several forms of SCD. They vary based on the sickle cell and also hemoglobin (Hb) genes you inherit from your parents. However, HbSS is the most common form. It’s also is the most severe form of SCD. People who have HbSS get 2 sickle cell genes, 1 from each parent.
What are the signs and symptoms of sickle cell anemia?
Sickle cell anemia is present at birth, but most infants don’t show any signs until they are more than 4 months old. Sickle cell disease symptoms range from mild to severe. Signs and symptoms of sickle cell disease include:
Anemia is a condition in which blood has a lower than normal number of red blood cells. People with anemia do not have enough red blood cells, which deliver oxygen.
Also, sickle cells die earlier than normal red blood cells. Red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells. Due to this, they may feel tired or weak.
Fatigue is one of the most common symptoms of sickle cell disease. Other symptoms of anemia includes;
- extreme tiredness
- shortness of breath on exertion
- yellowing of whites of the eyes.
- dizziness and lightheadedness
- increased heart rate
- pale skin
Dactylitis, painful swelling in your hands or feet. Mostly, it is the first symptom babies and young children with sickle cell disease exhibit. Your child may have sickle cell disease if you notice symptoms such as:
- increased fussiness
- swelling, tenderness, or redness in their hands or feet
- avoiding grabbing or holding items
- avoiding walking or standing
Pain also known as crisis occurs when there’s low oxygen flow through tiny blood vessels to your chest, abdomen and joints. Skeletal pain can also occurs i.e. in your bones. It is a common complaint among older children and adults.
The pain varies in intensity and can last for a few hours to a few weeks. But if it’s severe, you’ll need to seek emergency care at a hospital. Some people have only a few pain episodes. But for others, a dozen or more crises a year.
You may have pain anywhere in your body and in more than one place. These include common areas such as:
- Lower back
Most children who have sickle cell disease are pain-free between crises. But adolescents and adults may also suffer with ongoing pain.
Sickle cells can damage an organ that fights infection such as spleen. This leaves you more vulnerable to infections. Doctors commonly give infants and children with sickle cell anemia vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia.
Red blood cells provide your body with the oxygen and nutrients you need for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.
Tiny blood vessels that supply your eyes may become plugged with sickle cells. This can damage the retina — the portion of the eye that processes visual images, leading to vision problems.
Tips for you
Besides, environmental conditions also influence your symptoms. Sickle cell disease can worsen in extreme conditions, such as:
- High altitude
- Sleep apnea
- Exposure to cold temperatures
- Intense exercise
Over a lifetime, sickle cell disease can harm your spleen, brain, eyes, lungs, liver, heart, kidneys, penis, joints, bones, or skin. There are effective treatments that can reduce symptoms and prolong life.
Regular check-ups are also an important part of life with this disease. People with sickle cell disease need a good working relationship with a doctor who is an expert in treating it.