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Looking for more, scientific information? Check out the "Sickle Cell Resources" page!
If you're looking for a boring, clinical (zzzz....) analysis of Sickle Cell, there's plenty of other sites for that. As a person living with Sickle Cell, I wanted to put a lighthearted spin on the subject.
I'm not an authority on the disease but, let's see what we can learn together.
Here's some questions and my brief (but not official) answers.
What the heck is Sickle Cell?
Sickle-cell anemia is the result of a hereditary defect in the synthesis of hemoglobin. Huh?
How about I just tell you what they told me? Sickle Cell is a genetic disease that affects the production of hemoglobin in red blood cells. The disease causes the red blood cell to change its
shape and it becomes sickle shaped or crescent moon shaped. Get it?
Ok, So what are the effects of this disease?
Now, that I've got that off my chest, I'll try to be civil and explain further.
Because these red blood cells are "sickle" shaped, certain conditions can cause them to block blood flow. Those little suckers just weren't made to navigate the crooks and crannies of one's capillaries.
Well what causes the PAIN?
Sickle cell pain episodes occur when there is a lack oxygen supply to a specific part of the body. This happens when many red blood cells become sickle shaped, making it easy for them to get stuck together and block blood vessels. The body reacts to the oxygen depleted area and this is the source of the pain. It's like the affected area is saying "this ain't blood! What the *&#% am I supposed to do with this?"
What are the conditions that cause the production of sickle cells?
At the tender age of thirteen I was warned about five particular conditions that can affect the production of sickle cells. They are:
Stress 
(Me on my last job!)Dehydration
Extreme cold
temperaturesHigh Altitudes
Infection (Aunt Weezie always had a cold!)
So I became a water drinking, stress free (yeah right!) low land dweller that runs from people with colds or flu!
Actually, I worked hard to reduce the stress in my life (for instance, I sold both of my children. That eliminated at least 70% of my
stress!) and vigorously guard my need to rest. And I won't venture anywhere near high altitudes. Heck, I've never seen snow fall!
What is a Sickle Cell Crisis?
When one of the aforementioned conditions causes the increase in sickle cells, they block vessels as described. If enough sickle cells are produced this blockage can cause extreme pain where ever this blockage is severe. It's usually at this point I break out the Henessy and try knocking myself out!
No, honestly, the pain can range from moderate to severe. Since I'm an old veteran at this I'll compare the moderate pain to a throbbing toothache and the severe pain to having your leg severed off.
Ok, to the pain you'd feel post-surgical to your leg being severed off. Listen, this ain't for the faint at heart. This throbbing pain can extend to one or several joints and can get severe enough to require hospitalization to control the pain.
Hospitalization?
Yes, hospitalization. When it gets to where you're in a crisis there's more going on than just the pain. You've probably broken one of the cardinal sins, meaning you've acted a fool and disregarded one of those conditions I mentioned earlier.
In that case, you not only have to regulate the pain, but treat the underlying condition that brought the crisis on in the first place. Hospitals often don't have a protocol for treating a Sickle Cell Crisis, so doctors tend to get creative at this point.
Usually, unless you happen to be the one yokel that gets doctor Frankenstein, they administer an IV for hydration, oxygen to attempt raising the blood oxygen level and some pain medicine to help with the pain.
Sometimes a doctor can become a little sadistic (or ignorant, take your pick) and you end up in a tug-of-war with him over the pain medicine.
"You want what kind of pain medicine?"
Well, that's all of the questions for now, but if you're looking for more, check out the new "Sickle Cell Resources" page, now including:
Genetic Information
Blood Hemoglobin
And much more!
