Sickle Cell Anemia

November 16, 2017
By , Amery, WI, WI

Sickle Cell Anemia is a severe hereditary form of anemia in which a mutated form of hemoglobin distorts the red blood cells into a crescent shape at low oxygen levels. It is most common among those of African descent. I am just 1 out of 100,000 in the world that have this disease.

This disease is often inherited, which means that the disease is passed by genes from parents to their children. Sickle Cell Disease is not contagious, which means you cannot catch it like a cold or an infection. You are born with it.

People who have this disease have inherited two abnormal hemoglobin genes, which means one from each parent. One of the two abnormal genes then causes a person’s body to make hemoglobin S. When a person has two different types of hemoglobin, the disease then becomes Sickle Cell Anemia.

 

Hemoglobin is a red protein responsible for transporting oxygen in the blood of vertebrates. There are 6 different types of hemoglobin which means that there are 6 different forms of Sickle Cell. Hemoglobin SS, Hemoglobin SC, Hemoglobin SB thalassemia, Hemoglobin SB+ thalassemia, Hemoglobin SD, and Hemoglobin SE.

Hemoglobin SS disease is the most common type of sickle cell, It occurs when you inherit two cells that are S genes from both parents. 

Symptoms of this disease can be very hard to locate because they are common with many other diseases. Symptoms include pain in the joints, sudden pain the chest area, dizziness, fatigue, low oxygen in the body, or malaise. There is a chance that you might have the inability to create urine or have blood in the urine.  Some other symptoms could be delayed development, inflamed fingers or toes, shortness of breath, or yellow skin and eyes.

There are not many things you can do to treat this disease. Depending on the severity of the case you can get certain medications, blood transfusions, and sometimes, but this is very rare, bone-marrow transplants. 

The only device that you can use to control this diseases yourself. It is important to know your own body.

Having this disease is not an easy task so having many different doctors that can help you manage the disease is important. Upon getting diagnosed with Sickle Cell, you should be having yearly checkups with a hematologist, cardiologist, pulmonologist, neurologist, pediatrician, primary care provider, and Emergency medicine doctor. 

A hematologist is someone who has studied the science of blood the many different diseases that it can create. At appointments they will be checking to make sure your hemoglobin level is where it should be and give you blood transfusions if needed.

A cardiologist is a specialist who specializes in the study or treatment of heart diseases and heart abnormalities. Having a cardiologist on hand is important because one serious complication of sickle cell is Acute Chest Syndrome. This is when the sickle cell shaped red blood cells block the tiny vessels of the lungs. These doctors are important to have on hand, as these syndromes can come at any time, and are ongoing.

As a child growing up with this disease, some of these symptoms, doctors, and treatments seem all too familiar.
Being adopted by parents and being brought into a family where no one had this disease made it very challenging for my parents. They knew nothing about it, and have to learn how this disease worked so they could help their child that has it.

This disease is most common in African Americans, and as my parents adopted two African American children, they never knew the lifelong effects this disease has. Luckily, my brother Charlie does not have Sickle Cell so that has made it easier to help me mange my disease. 

In 4th grade, I had my first serious episode with Sickle Cell. Over the period of a month, I had been ill, and didn’t feel well. In that time my body was fighting the infection, and my hemoglobin went down severely because the red blood cells that had sickled were causing a blockage in my spleen. This blockage is referred to splenic sequestration, where the spleen enlarges and doesn’t function like it normally should, and causes an extreme amount of pain.

Before making the decision to do the splenectomy, I had two blood transfusions in the hopes that putting “good” blood into my body would bring my hemoglobin to an acceptable level. This unfortunately did not work and so I had my spleen removed.

Sickle Cell patients are often times prescribed penicillin, which is used to treat common infections such as strep throat, bronchitis, and pneumonia. For patients with a comprised immune system because of Sickle Cell these illness can have serious medical complications and taking prophylactic penicillin can prevent this.


Staying well hydrated is essential for patients with sickle cell. Dehydration can trigger painful trigger crisis.
Overall I have learned to live with sickle cell. I drink a lot of water, I do my best to stay as healthy as possible.          

 

I also have asthma, a common complication with Sickle Cell patients. I use inhalers daily to keep my lungs functioning optimumally. It is important to be physically fit, and be well rested. Annual checkups with my hematologist is very important as well as checkups with an optometrist and ophthalmologist. Finally when I do get ill I follow up with my doctor if my symptoms last more than 2 days, to make sure my hemoglobin level has not decreased to the level of a sickle crisis.  


Sickle Cell can be a death sentence. I have been fortunate to have many doctors and specialists with me to manage my disease and have had few complications. Living with Sickle Cell may not be easy, but it is possible.






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