Managing Sickle Cell Anemia

If you or a loved one are one of the 1-3 million people who live with Sickle Cell Anemia, you know that the complications related to the disease are often painful, inconvenient, difficult to manage and largely misunderstood. If you don’t live with the condition or have a loved one who does, then chances are you know very little about the disease and how it affects the day-to-day life of those afflicted by it. That is why awareness and understanding are so important. Our intent with this article is to educate and inform those without knowledge on the disease; with that said, let’s get started.

What Is Sickle Cell Anemia?

One common misconception is to broadly blanket Sickle Cell Anemia in with iron-deficiency anemia, a much more common form of anemia which, according to the Mayo Clinic, is related to the body’s shortage of healthy red blood cells due to- as the name implies- a shortage of iron in the body, often for dietary reasons or because of a pregnancy. Sickle Cell Anemia, on the other hand, is a genetic condition that describes a package of inherited red blood cell disorders that affects hemoglobin, a protein molecule in blood that carries oxygenated blood from the lungs into the body’s tissue.

An individual with Sickle Cell Anemia has red blood cells that become hard, sticky, and shaped like the letter “C”, or like a sickle- a farming tool with a C-shaped blade- which is where Sickle Cell Anemia gets its namesake. According to the CDC, this lack of hemoglobin and unusual shape causes the sickle cells to die early, leading to a near-constant shortage of red blood cells, and blood flow clogging in smaller blood vessels.

What are the Symptoms and Complications of Sickle Cell Anemia?

Symptoms and complications vary significantly from person-to-person, ranging from mild to severe, and usually begins to manifest at around 5-6 months old. Early symptoms can include signs of jaundice, fatigue or fussiness, and swelling of the hands and/or feet. As those with the condition age, more complications can arise; according to the National Heart, Lung, and Blood Institute (NHLBI), those complications may include:

• Acute Chest Syndrome, a very serious condition in which sickling in blood vessels causes lung damage, depriving the lungs of oxygen.
• Acute Pain Crisis, a severe pain possible anywhere in the body caused by a lack of oxygen flow, sometimes triggered by altitude change, dehydration, illness, stress or change in temperature.
• Brain complications, caused by a stroke.
• Chronic pain caused by organ damage.
• Retinal damage, caused by injured injured blood vessels in the eye, which can lead to damage in vision or outright vision loss.
• Other organ complications, including in the liver, kidney, and heart.
• Joint pain and damage, caused by lack of oxygen flow to the joints.

How is Sickle Cell Anemia Treated?

 Sickle Cell Anemia is usually treated with a number of medications and procedures, including:

• Penicillin, for young children and adults who are at risk of developing infections caused by complications from the condition.
• Hydroxyurea, an oral medication that has been shown to reduce pain episodes caused by acute chest syndrome and acute pain syndrome. Hydroxyurea has also been shown to reduce anemia and decrease the need for blood transfusions and hospital visits.
• Blood transfusions, which can help reduce or prevent complications, particular prior to surgeries and those at risk of stroke.
• Blood and bone marrow transplants, which can actually cure Sickle Cell Anemia, but are most often options for children. These transplants are much riskier for adults, and finding the proper genetic match is not always an option.

How Can I Manage My Sickle Cell Anemia?

If you are experiencing Sickle Cell Anemia, you know that it can be a very difficult and frustrating condition to manage, but remember: you aren’t alone. Depending on the severity of your condition, follow-up appointments with your doctor could be as often as four times a year; keeping up a routine appointment schedule with your doctor is an important step in staying on top of any complications you may be having, and finding solutions for those problems. Your doctor may recommend a number of screenings and vaccinations that can act as both proactive steps in catching future problems before they arise and finding an existing issue before it worsens.

Living as healthy of a lifestyle as possible can also be an aid in limiting complications; while it is no guarantee, it can help! Getting consistent physical activity without overexerting yourself, maintaining a good sleep schedule, eating healthy foods, and staying away from tobacco products are all positive lifestyle decisions that can help manage symptoms. Making sure that you are taking any prescribed medications at the frequency recommended, and avoiding crisis triggers such as extreme temperatures or abrupt changes in altitude, can also be a good management tool.

It’s also important to keep a careful eye on how you are feeling. If you feel as though something unusual or excessively painful is occurring in your body, don’t hesitate to contact your doctor immediately. It’s best to be extra-cautious when it comes to even the slightest sign of a serious complication developing. Also, don’t be afraid to lean on friends, family, and the community if you are feeling poorly–either physically or mentally. Local support groups and loved ones can help. Try to remember often that, when you are in any kind of pain, you are not alone; there is help, both professionally and interpersonally! If you are trying to find health care providers and Sickle Cell Anemia support groups in your area, the CDC directory is an excellent resource to point you in the right direction.