Sickle cell anemia is an inherited blood disorder. It makes abnormally shaped red blood cells that look like crescents, “or sickles.” Compared to healthy round red blood cells, they don’t last long enough to transport oxygen. The result is anemia.
Thanks to their shape, sickle-shaped cells can also cause blockages. They aren’t very flexible, so they stick to vessel walls. This may slow or stop blood flow.1
In America, about 100,000 people have sickle cell anemia. It’s most common in blacks and African Americans, with a diagnosis of 1 in every 365 births.2 Regardless, sickle cell disease is complicated, affecting each person in different ways. Treatment depends on management.
Types Of Sickle Cell Anemia
- Hemogobin SS: Two inherited two sickle cell genes (“S”), one from each parent. This is the most severe form.
- Hemoglobin SC: One inherited sickle cell gene from one parent, and an abnormal hemoglobin (“C”) from the other. This is a milder form.
- HbS beta thalassemia 0 or +: One inherited sickle cell gene (“S”) from one parent, and one gene for beta thalassemia, a type of anemia. HbS beta 0-thalassemia is more severe. Hbs beta +-thalassemia is milder.
- Hemoglobin SD, SE, or SO: One inherited sickle cell gene (“S”) and an abnormal hemoglobin (“D”, “E” , or “O”). These forms are rare.
Sickle Cell Trait
Hemoglobin AS: One inherited sickle cell gene (“S”) from one parent, and one normal gene (“A”) from the other. Symptoms are not present, but the person can pass on the sickle cell gene to their kids.3
Symptoms Of Sickle Cell Anemia
People with sickle cell disease are born with it, but symptoms usually show up after the child is five or six months old. Early signs include fatigue, yellowish skin, pain, and swelling of limbs. The more distinct symptoms are:
Acute Pain Crisis
When oxygen delivery decreases, pain develops. This is described as sharp, intense, and throbbing. Pain often shows up in the lower back, legs, arms, stomach, and chest, but it can affect more than one area at a time. Triggers include sickness, extreme temperature changes, stress, high altitudes, and poor hydration.
Chronic Pain
As a common complication of sickle cell anemia, chronic pain can make everyday life difficult.
Anemia
Mild to moderate anemia is typical. Fatigue, weakness, headaches, and shortness of breath are common symptoms. Severe anemia can be life-threatening.4
Can Sickle Cell Anemia Be Prevented?
As an inherited disease, sickle cell anemia can not be prevented. It’s not contagious, nor can it be avoided through diet. Again, the disease develops from mutated genes.
If you already have sickle cell anemia or trait, you might pass it on to your kids. A genetics counselor can determine your risk.5
Otherwise, complications can be prevented or limited.
Management Of Sickle Cell Anemia
1. Recognize Triggers
From stress to dehydration, various factors can spark pain. It’s different for everyone. By paying attention to your triggers, you can limit the risk of pain.
Granted, sometimes pain happens just because. Prepare yourself by having a home treatment plan. This may include drinking lots of water and taking anti-inflammatory pain medication, like ibuprofen.6
2. Prevent Infections
In sickle cell anemia, the spleen is impaired. This organ normally removes harmful microorganisms and old red blood cells from the body. But if it doesn’t work, common illnesses are a huge threat.7
To avoid infections, regularly wash your hands. Do it before and after cooking and eating. Hand washing is also a must after using the bathroom, blowing your nose, or taking public transportation. Can’t get to a sink? Always have antibacterial gel on hand. It’s better than nothing.
Immunizations and vaccines can also prevent infections like pneumococcus, influenza, and meningococcus. Until the age of 5, children are often required to take penicillin twice a day to avoid pneumococcus.8
Of course, it’s important for everyone to avoid infections. But if you have sickle cell anemia, it’s even more crucial.
3. Adopt Healthy Habits
As with most life-long diseases, a healthy lifestyle is the key for management. Every day, make nutritious food choices. Focus on fruits, vegetables, and whole grains. A dietitian can provide guidance for meal planning.
Stay hydrated. Remember, dehydration can spark a pain episode. Aim for 8 to 10 glasses of water each day. Physical activity will also keep you healthy. Don’t forget about hydration, and if you feel tired, take a break.
The only actual cure to sickle cell anemia is hematopoietic stem cell transplantation. However, a very close genetic match is needed. Most people are either too old or don’t have a relative that fits the bill.9
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