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Sickle cell trait can take a sudden deadly turn

Many people who carry sickle cell trait don’t know it. Yet, it can be just as deadly as sickle cell anemia.
In the United States, about 80,000 people have sickle cell anemia, but more than 12 million have sickle cell trait. Both disorders target certain ethnic groups, including South and Central Americans, Cubans, Saudi Arabians, Asians, Mediterraneans, and American Indians, but the disorders disproportionately strike African Americans. About 1 in 400 African Americans have sickle cell disease. About 1 in 10 African Americans have sickle cell trait.

What causes sickle cell disorders
Both sickle cell anemia and sickle cell trait are genetic disorders. People with sickle cell anemia have inherited homozygous sickle hemoglobin genes (hemoglobin S) from both parents; their blood type is called hemoglobin SS. People with sickle cell trait have inherited a normal hemoglobin gene (hemoglobin A) from one parent and a sickle hemoglobin gene from the other parent; their blood type is called hemoglobin AS.
Sickle cell anemia is a general term for several genetic disorders caused by red blood cells (RBCs) that are sickle-shaped because of deformed hemoglobin. A chronic condition, sickle cell anemia causes life-threatening signs and symptoms, such as an enlarged spleen, muscle pain, liver scarring, pneumonia, and bone and joint pain. The typical lifespan for those with sickle cell anemia is 40 to 60 years.

Screening for sickle cells
Two laboratory tests, Sickledex and hemoglobin electrophoresis, are used to screen for sickle cell anemia and sickle cell trait.
Sickledex detects sickle cells if 25% or more of RBCs have a sickle shape. Hemoglobin electrophoresis is much more sensitive: It can detect a small number of sickle cells. It also determines if a person has one or two mutated genes, thus distinguishing sickle cell trait from sickle cell anemia. Because the less sensitive Sickledex is less expensive, it’s commonly used for screening.
If one or both parents have sickle cell trait, a newborn needs genetic testing because early diagnosis helps prevent complications.

New view of sickle cell trait
Until the 1980s, sickle cell trait was considered a carrier state and a
benign condition. But researchers who were investigating why young, healthy, African-American recruits were dying during military training discovered that 50% of them had sickle cell trait.
Today, people with sickle cell trait are considered to be at risk for death if they perform strenuous exercise without proper conditioning, become dehydrated, have an increased body temperature, participate in high-altitudes training, or exercise after an illness with a fever.
Sickle cell trait doesn’t cause obvious signs and symptoms, but in certain situations it may behave like sickle cell anemia with serious consequences. For example, some people with sickle cell trait have experienced abdominal pain at high altitudes and required a splenectomy. None of them had a clue they had sickle cell trait until they had this experience. Others learn they have sickle cell trait late in life when they have an illness that causes dehydration or fever. Still others find out at a health fair screening.

Why does sickle cell trait behave like sickle cell anemia?
People with sickle cell trait have both normal and abnormal RBCs, but usually both types are round. Round RBCs move easily through the tiny capillaries of the body. Unfortunately, when the abnormal RBCs are exposed to conditions that lead to hypoxia or dehydration, their shape changes from round to crescent or sickle-shaped. These sickle cells don’t transport as much oxygen as round cells, and they have difficulty moving through capillaries. Thus, body cells suffer from hypoxia both because of diminished oxygen transport and because of capillary blockage by the sickle cells. Unrelieved hypoxia progresses to cell death, excruciating pain (sickle-cell crisis), lactic acidosis, muscle and organ damage, and ultimately death.
When a person exercises, oxygen needs increase, and some cells may experience hypoxia. The hypoxia is more profound when a person exercises at high altitudes, where the atmospheric oxygen concentration is low. Any exercise causes perspiration and dehydration as the body attempts to cool itself. Perspiration is especially profound when a person is not conditioned, exercises strenuously, or exercises in a hot, dry environment.

How deadly rhabdomyolysis develops
A person with sickle cell trait who engages in strenuous exercise also can develop exertional rhabdomyolysis, the catabolism of muscle tissue—a potentially fatal condition. Rhabdomyolysis occurs when dehydration leads to sickle cells. When a person exercises, muscle protein breaks down and produces a waste product called myoglobin. If myoglobin production is excessive, and the body doesn’t have enough fluid to dilute it, myoglobin rises to toxic levels that damage the kidneys. Signs and symptoms of rhabdomyolysis include muscular swelling, muscular weakness, cramping, and pain. The urine looks like tea because it contains a large amount of myoglobin.
Treatment is based on symptom severity and is needed immediately to improve the chance of survival. The patient remains on bed rest and restricts all activity to decrease the workload on the affected body systems. The patient receives oral fluids and I.V. infusions to promote myoglobin excretion. For the same reason, a patient may receive a diuretic and sodium bicarbonate.

Preventing complications
No standard recommendations exist to decrease the risks for those with sickle cell trait, but certainly better screening and education would help. People need to know whether or not they have sickle cell trait. Because Sickledex can’t detect most cases of sickle cell trait, hemoglobin electrophoresis should be used for screening. When this test determines that a person has sickle cell trait, the person needs up-to-date information about the condition, including genetic counseling.
Athletic trainers, team physicians, coaches, and military drill instructors should be taught about sickle cell trait and the dangers of dehydration, the need to adjust to a new climate or higher altitude, the importance of physical conditioning, and the importance of rest during and immediately after an acute illness. Athletes who have sickle cell trait should inform coaches and trainers.
Many people have the trait and don’t know it, and those who do know may have been told it’s benign. But now we know better. We need more effective screening and patient teaching to manage the condition and prevent deadly complications.

Selected references
Asgharian A, Anie K. Women with sickle cell trait: reproductive decision-making. J Reprod Infant Psychol. 2003;21(1):23-34.
Cleary M. Sickle cell trait and exertional rhabdomyolysis. Athletic Therapy Today. 2003;8(5):66-77.
Kark J. Sickle cell trait. Available at: http://sickle.bwh.harvard.edu/sickle_trait.html. Accessed May 20, 2007.
Platt A, Sacerdote A. Hope and Destiny: A Patient’s and Parent’s Guide to Sickle Cell Disease and Sickle Cell Trait. Roscoe, Ill: Hilton; 2002.
For a complete list of selected references, visit www.AmericanNurse Today.com.

Phyllis Adams, EdD, RN, FNP, APRN, BC, NP-C, is Director of the Family Nurse Practitioner Program at the University of Texas at Arlington School of Nursing and a Family Nurse Practitioner at HEB School-Based Clinic in the John Peter Smith Health Network System, Euless, Texas.

9 thoughts on “Sickle cell trait can take a sudden deadly turn”

  1. S. Jewell says:

    Have sickle cell trait. Feel tired often.

    Looking for a doctor to treat me in AZ.

    I live in Yuma, full time employee, active, youthful senior, advanced degrees, travels often.

  2. Stacy Brownrpt says:

    Im so glad there’s info out now, got tired and frustrated about people thinking I was being silly. A health care professional colleague told me that it sounded silly and didnt make any sense.
    At about age 7 I had serious abdominal pains and the doctor queried SCD, thats when i knew I had the trait. Up to about age 8 to 14 I got moderate to severe hip and knee joint pains that sometimes were difficult to even differentiate which joint was affected. I had to use pain gels every time as the areas (right and left) would feel so inflamed.
    Pains sudsided as I got older but about 8 years ago i started cycling and the pain returned whenever I cycle for over 30 minutes non-stop. I tried continuing hoping it would just work its way in but the pain got severe so several occasions I restarted and stopped….SAME PAIN as a child.

  3. Quoia Robinson says:

    All of this information is true I am a Sickle cell trait Carrier an I’m in pain constantly ,always cold, constant migraines, My stomach hurts, Heavy bleeding. I didn’t know what was going on until I’ve gotten sick 😷 5years ago I was 8mons pregnant with my second Son I had to got to the hospital only to learn 😭😥 my baby passed away 😢💔 that’s when the doctor told me Ive got severe chronic anemia I ask what? He then explained I have Sickle Cell Trait he then explained to say half of my blood cells are normal 🌕 the other half are Sickles 🌙he said there’s no cure all you can do is take prescribed medication ,iron pills 💯try to get on a healthy diet an workout he finally said 💪🙌 that’s going to help you in any way but only to suppress it because there’s no cure sorry.

  4. Wanda Blount says:

    My left kidney was bleeding and doctors couldn’t understand why. Didn’t know I had the trait, I was 16 years old. Now at age of 63, both my legs clotted and had surgery on both leg three times. They say sickle cell trait had nothing to do with it. I am in pain everyday. I need help

  5. Marissa Dernbach says:

    Love to hear more a trait carrier as well.
    Thanks again Marissa

  6. Shantel says:

    I have sickle trait and have recently been in a
    SCD crisis.

  7. Marie says:

    I was about 24 maybe 25 and I end up in the hospital for 1week for my sickle cell trait and that was when I learned what it was and that I had just the trait. It started with me having a regular cold from what I thought then high fevers of 108 and a pain in my lower stomach area I thought I was constipated then I was getting sick but nothing was coming up so my kids grandma took me to the ER and the doctor told me he had never seen a person get sick from having just the trait and that is when I learned of what sickle cell was and my 10yr old has the full blown sickle cell and I am 34 now and I still remain having pains and sickness just off the trait.

  8. Cindy says:

    I have the sickle cell trait. As a teen I had episodes of fainting and severe stomach pain. But unfortunately, the doctors never told me I had the trait. I was always viewed as if I was lying. But now I am 41 years old I have a daughter and a son that happened to have the trait and they both suffer from the same pain I have. The same pain. At this age 41 I still have pain and the doctors as of now don’t understand my pain or my children pain.

  9. Emily Rodriguez says:

    I can verify that this information about Sickle Cell Trait is correct. My husband was born with it, but had no problems while deployed in the mountains of Germany as a young man in Army. One day when he was in mid fifties, he developed a pulmonary embolus, and was placed on Coumadin. Later, he complained of L hip pain, diagnosed as Avascular Necrosis. After his hip replacement, he was weaned from the Coumadin dosing when it was determined that he did not need to take it for life.
    He lived a normal life for two years, until he went on a long road trip and returned home. Within two days, he began having leg/chest pain and tests revealed that his heart, lungs, aorta, groin vessels and L leg all were clotted.
    tPA was done, but to no avail and he died two days after admission.
    The Sickle Cell Trait led to a Hypercoagulopathy which could not be reversed.

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