Icicles and a Crescent Moon: Get the facts and a few practicle tips on Sickle Cell Disease

September 1st, 2006

This could be the description of things you’d see on a crisp wintry night. But for many these words describe the shape of their red blood cells, a tell-tale sign of Sickle Cell Disease. Sickle Cell Disease is a hereditary condition, occurring in children whose parents both have the sickle cell trait. Sickle cell can be a very painful, sometimes debilitating illness. The red blood cells in individuals with sickle cell are less flexible, and can lose their usual rounded, disc-like shape, to become sickled-shaped, with jagged edges, kind of like an irregularly shaped icicle or a crescent moon. Because these oddly shaped cells do not travel easily through the smaller blood vessels, they tend to gather and cause blockages, which is why these individuals experience pain. The location of pain is directly related to where the ’sickled’ cells have gathered. When blood flow to the major organs is occluded, pain may feel more generalized, and life threatening complications may occur. During these acute, painful episodes, the condition is known as Sickle Cell Crisis (SCC).
Sickled cells also have a shorter life expectancy than normal red blood cells, which can lead to anemia, hence the term sickle cell anemia. The red blood cells carry oxygen, which the body needs to function, to all the tissues and every organ in the body. If there are too few red blood cells, the patient may experience symptoms of anemia: unusual fatigue and weakness, breathlessness, dizziness, rapid heartbeat, and possibly a host of other symptoms, depending on the severity of anemia.
Blood transfusions are given to patients with moderate to severe anemia. Other problems commonly associated with SCC are infection, and severe pain. The treatment includes: rest; antibiotics to fight infection; intravenous fluids for hydration and increased circulation; and narcotics for pain relief. Organ failure is a less frequent, but very dangerous complication, and it requires specific supportive care for the affected body system(s).
September is National Sickle Cell Awareness Month. The observance originated in 1975, but President Reagan signed the resolution to designate September as “National Sickle Cell Anemia Awareness Month” in August, 1983. Ironically, September is the perfect month to dedicate to learning about Sickle Cell disease; it’s the beginning of fall—the beginning of the cold season, the beginning of the holiday season…the beginning of the SCC season! In my experience as a nurse, I’ve noticed that the number of patients admitted to the hospital in sickle cell crisis increases dramatically during these times. Crises can be brought on by anything that leads to constriction (narrowing) of the blood vessels, circulatory problems, decreased oxygenation (too little oxygen in the blood), or decreased hydration (less liquid in the blood causes the blood to be thicker). People with sickle cell will never be disease free, but they can lead relatively normal, healthy lives if they take some steps to help prevent the actual crises. Here are some helpful hints to make this winter season as pain free as possible:

*Exercise regularly, but not to the point of exhaustion.
*Drink at least 8 glasses of water a day, especially during warm weather. And drink plenty of fluids during exercise.
*Reduce or avoid stress. Tip: yoga, exercise, daily meditation, and self-hypnosis are very good tools for relieving stress.
*Treat any infection fast, see your doctor ASAP.
*Always wear warm clothes outside in cold weather and inside in air-conditioned rooms during hot weather. Never swim in cold water.
*Take warm baths on a daily basis. Relaxing in a jacuzzi can be very beneficial, but always check with your doctor first. The temperature may be too warm for some individuals (i.e. pregnant women, men with a low sperm count, and other people with certain illnesses).
*Limit how much alcohol you drink.
*Don’t smoke. If you do smoke, quit.
*If you experience pain that is not relieved by mild analgesics or whatever the doctor has prescribed for you, see your doctor ASAP. You may be heading for crisis and require hospitalization, but your duration as an inpatient may be shortened by prompt medical attention.

Happy, Health Fall!