I think all of us in healthcare have patients that we will always remember. Sometimes they are for good things, like the miracles we get to see every so often, sometimes they are for not so good things like child or elder abuse or a bad trauma case. But every once and awhile you remember someone because of their character in the face of daunting health conditions. The first person that I will remember forever because of that particular condition is a woman I took care of when I was still an LVN in the early 1990’s. That woman had sickle cell disease, and when a person is in sickle cell crisis, they have horrible pain. Her courage in the face of that pain, a pain she had experienced before so she knew exactly what was coming, will always stay with me.
June 19th is World Sickle Cell Day. World Sickle Cell Day is designed to increase awareness of sickle cell disease and to help recognize the courageous people who live with this condition: the people who suffer from sickle cell disease and their loved ones.
Sickle Cell Disease is a genetic disorder that affects red blood cells. Within red blood cells is a molecule called hemoglobin. This molecule is what oxygen attaches to as red blood cells carry that vital element around the body. There are several kinds of hemoglobin: hemoglobin A, hemoglobin C, hemoglobin D, hemoglobin R and hemoglobin S. The type of hemoglobin determines the shape of the blood cell. People without sickle cell disease produce mainly hemoglobin A.
Hemoglobin is such a big molecule and it is so tied to how red blood cells work that the kind of hemoglobin influences how the red blood cells are shaped. Cells with mostly hemoglobin A are soft, flexible, and disk shaped, those cells are referred to as “normal.”
Cells that have mostly other types of hemoglobin get very stiff and are more sickle shaped, if you’ve never seen a sickle, think about the shape of the crescent moon, almost a shallow C-shape.
This causes issues when those cells enter the tiniest of all blood vessels, the capillaries. Capillaries are so small that red blood cells must line up in single file and squeeze through. Because “normal” red blood cells are soft, disk shaped and flexible, they can squish and bend to get to get thought that tiny “tunnel” of a capillary.
The sickle shaped cells, however, get hung up. They can’t fit in the capillaries or they get stuck in the capillaries.
Think about it like this. On the walk down path in Carlsbad Caverns, there is a spot where the cave narrows and the roof drops to about 5 feet (I have to duck to go through and I am short!) only one person can fit through at time. If you go on a busy day, that spot can get backed up a bit because the crowd must line up and squeeze through that narrow space on person at a time. They do get through, but it takes a little effort and there is a slow-down of the flow.
That is kind of how red blood cells work when it comes to capillaries when everything is “normal.” But think about what would happen if someone got stuck in that narrow opening or was too big to pass through. That would stop everyone from getting through because there is no place to go around them.
That is exactly what happens with those sickle cells. This is so bad because that means with blood cells, everything downstream from that capillary that is blocked cannot get oxygen. So all the other cells in the body that are dependent on that capillary for their blood flow start starving and when cells start starving for oxygen first they start screaming for it.
A “scream” from a cell is pain. Multiply this problem by hundreds and thousands of times. If the lack of oxygen goes on long enough, the cells die. That causes much worse problems. Sickle cells also don’t live very long. “Normal” red blood cells live about 120 days, while sickle cells live only about 16 days, so people with sickle cell disease are usually anemic, which means they don’t have enough red blood cells in their system to supply the demand for oxygen. That means people with sickle cell disease tire easily, they are more susceptible to infections, and they heal injuries slowly.
I am sure you are asking, if that happens with sickle cell disease, how do people survive it at all? They survive because their bodies don’t produce those sickle shaped cells exclusively. People with sickle cell disease always make sickle-shaped cells, but stressors to the body make people with sickle cell disease produce more of those kinds of cells than “normal” cells. That condition is often referred to as a sickle cell crisis, which is very painful in all parts of the body, particularly the joints because of how those sickle shaped cells collect. It can cause tissue damage throughout the body because of the lack of oxygen, and because of the short lives of the cells, sickle cell crisis can also cause life-threatening anemias.
There is no cure at this time for sickle cell disease. People who live with this condition learn to manage symptoms and try to avoid the things that put them into crisis. A cure may be on the horizon because there has been some success in identifying the gene that causes sickle cell disease, but like all genetic research of this type, those kinds of cures are in their infancy at best. On June 19th, if you know someone with sickle cell disease, please recognize the courage they display every day as they live with this very challenging condition.
