Do you know someone with Sickle Cell Disease? You may not realize it, but you actually probably do.
Thanks to Ryan Clark, a Pittsburgh Steelers player, this disease has gotten some attention in the media lately. Though Clark does not have Sickle Cell Disease, he has the Sickle Cell trait, which, in him, becomes aggravated at higher elevations. This suffrage was brought to the forefront after the Steelers played the Denver Broncos in Denver back in 2007, when he became very sick and ended up having to have both his spleen and gall bladder removed as a result of the strain and stress of the elevation on his body. This is something that is common for anyone who suffers the disease.
So what exactly is Sickle Cell Disease (SCD)?
It is a condition which mostly affects African-Americans, though others can suffer it as well. It is a condition which affects the red blood cells of a sufferer. Whereas a normal red blood cell is circular in shape, a sickle cell is sickled or crescent-shaped, as seen in the pictures below. (Wikipedia really is good, it gave us that picture!)
As can also be seen in the first image, normal red cells flow easily through our vascular system, bending to fit through capillaries, allowing for the normal exchange of oxygen and carbon dioxide between our vasculature and tissues. Unfortunately, sickle cells do not bend as easily (they're also sticky, unlike RBCs) and, as a result, clog up the capillary space. This causes intense pain, as tissues are not able to get rid of carbon dioxide nor receive the vital oxygen they need to survive. (Who knew oxygen was SO important?!)
Now, if these cells are not so prominent, then they don't cause so many problems, but if they are, the pain the clogging causes is referred to as a Sickle Cell Crisis, or SCC in the hematology world. So what do we do to treat this? As there are no cures at this time, we treat the symptoms and try to make the blood flow better. This is done first by giving pain medication - the pain is very intense. We also give plenty of fluids, both orally and intravenously. Depending on hemoglobin and hematocrit levels, we may or may not give blood as well. At my facility, we don't do this until the hemoglobin is less than 5 (normal is 12-17) or the patient is symptomatic (signs/symptoms of low oxygenation, shortness of breath, etc.). Another treatment is heat to the affected area (via heat packs or a heating pad), as this helps to open up the blood vessels and allow for easier passage of blood through the vasculature; it also helps with the pain. We encourage early ambulation to promote the blood flow and also offer oxygen if the oxygen saturation level is low (a common place for the SCC to occur is in the chest).
So how does one prevent a SCC and stay out of the hospital? It's not entirely easy to say. There are things SCD sufferers can do to help, but they still almost always end up with the occasional hospitalization.
- First off, it's recommended to avoid alcohol (isn't that recommended for almost every disease prevention/treatment?).
- Also, it is important to not smoke - while smoking has it's own normal repercussions, in SCD, the effects are heightened, especially in that smoking restricts blood flow. In SCD, we want to encourage blood flow.
- Adding to this, we say to stay warm - cold weather and even air conditioning can cause constriction of the blood vessels, again restricting flow. Though we can't control the weather, we can control how we dress, so carry a sweater inside and stay bundled outside.
- Next, drink lots and lots of water. One site recommends drinking at least 96 ounces (that's more than 2 liters!) of water a day during warm weather. Again, this helps to promote blood flow. - One teacher and great mentor I had in my elementary through high school days would carry around a liter bottle with her and make sure to drink at least that much during the day and then drink that much at home/outside of school. (This is something I, a person with normal RBCs, have done as well. Water is good for all of us to drink.)
- As always, it is recommended to get exercise. Not so much that the body is stressed, but enough that the body is healthy.
- Mentioning stress, now's a good time to mention that SCD sufferers should avoid stress. While this isn't easy to do, it should be a goal. Daily stress is normal, but getting plenty of sleep helps to decrease stress on the body. (Another tip - enough sleep!)
- Avoid infection. Infection causes stress on the body as well. Some SCD patients are put on antibiotics as a preventative, especially in the younger years when the immune system is not yet fully developed.
That's definitely not an all-inclusive list. There is much more people can do to prevent a crisis, but these are the basics.
Want a little lesson? Free of charge! -- The Sickle Cell trait comes via a gene mutation that occurred as a result of malaria. This is why many of the sufferers of this disease are of African descent or from some other tropical climate where malaria is (or was) common. Therefore, those with Sickle Cell Disease are actually immune to malaria. Who knew?! There are actually different forms and, therefore, different severities of the disease. Those of African descent generally have the most severe form compared to those of Indian, Middle-Eastern, or South American descent. Milder forms don't generally result in hospitalization, although I have known of a person with the trait to be hospitalized during pregnancy because of the stress on the body. (Ryan Clark, he only has the trait, not the full-blown disease. It was the stress of the higher elevation and pushing his body through a professional football game which caused his problems.)
If you know someone with Sickle Cell Disease, encourage them. Learn from them. And support them.
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