SCD and Race

When you look at the numbers of those affected by sickle cell disease, it is clear that individuals of African descent are affected the most. Why is this? Sickle cell disease being a "black" disease is a common misconception made by those who haven't done enough digging on the matter! 

Believe it or not, sickle cell disease evolved as a resistance to malaria. According to the American Anthropological Association's webpage, www.understandingrace.org, sickle cell disease developed "In the malaria belt regions of Africa, the Middle East, southern Europe and South Asia, this gene variant flourished because the benefits of malaria resistance outweighed the negative impact of sickle cell disease." Therefore, the sickle cell gene is related to malaria, not race!

Here is a map showing the relationship between malaria, sickle cell, and skin color:

Because of immigration, it's safe to say that races associated with these regions will be more susceptible as we look at prevalence numbers in the United States; however, it is important to understand that if malaria was heavily present in any other part of the world, the ethnic groups and races associate with those geographic locations would undoubtedly be affected.






Now, we know that for our students, sickle cell disease can be painfully debilitating, so how in the world does the sickle cell trait do good to the body and ward off malaria? To better understand how the malaria parasite works, click on the video below!

So the malaria parasite with evolve to infect red blood cells. Once in a red blood cell, it can hide from the bodies immune system. Here it begins to destroy the round disc-like cell. Individuals with the sickle cell trait, however, allow for the parasite to be filtered out through the spleen and destroyed.

Source:
American Anthropological Association

SCD in the Classroom

As educators, here are a few ways we can support students with sickle cell disease (from CDC Tips for Supporting Students with Sickle Cell Disease, section 2):

Make sure your student has plenty of water.

Always make sure your student has access to unlimited water throughout the day. This will help to prevent pain episodes and other health complications by keeping him/her hydrated. According to the Center for Disease Control, "frequent, small amounts of water are better than trying to drink a large amount of fluid at one time." So allow your students to carry and fill water bottles at all times. You may even consider keeping a supply of water bottles handy!

Frequent bathroom breaks may be necessary.

"Children with SCD produce large amounts of dilute urine even when they are dehydrated," (CDC).
With this in mind, it is imperative that we do not restrict sickle cell students from using the restroom. One accommodation you could make would be to provide a special bathroom pass so the student can excuse him or herself as often as is necessary. By doing so, you minimize attention drawn to the student as well as classroom disruptions usually associated with students asking the use the restroom frequently.

Accommodate your student when temperatures get extreme.

Since cold or hot weather can trigger pain crises, teachers should be mindful of where students with SCD are seated. Avoid drafty locations, fans, and air conditioning vents. Encourage layered clothing in the class and remind the students with SCD to wear a jacket outside during cold/rainy weather. Also, be aware that children with SCD should not exercise in extreme conditions (i.e. avoid cold, high heat, and humidity).

Accommodate your student during physical activities.

While most children with SCD  can engage in moderate exercise (i.e. running, swimming, bike riding), some students may need modifications. Teachers can modify curricula so that a child experiencing health problems related to SCD can participate in physical education roles that are less strenuous (i.e. teacher's "assistant," "scorekeeper," "umpire"). Admitting fatigue may be embarrassing or draw unwanted attention to a child with SCD, so it is important that we make necessary modifications and accommodations for the individual. He or she may also need frequent breaks or brief periods of rest. And as stated early, allow for frequent hydration. It is always a good idea to consult with the child's parent or even the student him/herself to get an understanding of the level of activity he or she can tolerate.

Pay extra attention to injuries.

The Center for Disease Control stresses to "never apply a cold pack to an injury or pain site if a child with SCD is injured during the school day." You may, however, use other first aid measures instead. These include: applying direct pressure for bleeding, wrapping with an ace bandage, or elevating a hurt limb.

Watch for signs of a stroke.

Strokes may be hard to detect when they affect such small portions of the brain, but they are extremely important to watch out for because they are relatively common in young children with sickle cell disease.

Common signs of stroke in kids and teens include:

• seizures
• headaches, possibly with vomiting
• sudden paralysis or weakness on one side of the body
• language or speech delays or changes, such as slurring
• trouble swallowing
• vision problems, such as blurred or double vision
• tendency to not use one of the arms or hands

Teachers should be aware that lack of attentiveness, decline in academic achievement, mild delay in vocabulary, and lack of organization can all be caused by small damages to the brain caused by stroke. Teachers should also be aware that they are in a unique position to monitor the behavior of students affected by sickle cell disease. They should contact parents when changes in learning or a child's attentiveness are detected so that the child's doctor can be notified.

Sources:

KidsHealth

Center for Disease Control and Prevention

Common Traits & Indicators of SCD

People with sickle cell disease (SCD) begin showing signs during the first year of life, usually around 5 months of age. Infants won't show symptoms of SCD at birth because fetal hemoglobin protects the red blood cells from sickling. At around 4-5 months old, the fetal hemoglobin is replaced by sickle hemoglobin and the cells begin to sickle. Symptoms and complications of SCD are different for each individual affected and can range from mild to severe.

As I continue my research into sickle cell disease, I've noticed that there isn't much to do in the way of prevention and treatment other than blood transfusions. This seems to become just a part of normal everyday life for those individuals. These individuals must be watched closely for iron build up in the body, which can have life-threatening damage to the organs.

It is important to remember that children with sickle cell disease are usually smaller in frame, have delayed puberty, or experience jaundice (yellowing of the skin and eyes). Children with SCD may also experience difficulty playing with other children, and may hinder social interpersonal development. These outward signs may make children living with SCD  easy targets for bullying and the psychological damage done can be equally devastating. As educators, keep this in mind and try to include other avenues for social development that all students can benefit from.

Symptoms are unique to each individual and include the following (information directly provided from the Center for Disease Control and Prevention):

• Hand-Foot Syndrome- swelling in the hands and feet, usually the first symptom of SCD. This swelling is often accompanied by fever and is caused by the sickle cells getting stuck in the blood vessels and blocking the flow of blood in and out of the hands and feet.
• The most common treatments for swelling in the hands and the feet are pain medicine and an increase in fluids, such as water.
• Pain "Episode" or "Crisis" - Pain is the most common complication of SCD and the number one reason people effected go to the emergency room or hospital. When sickle cells get stuck in small blood vessels and clog the blood flow, this causes mild to severe pain that can last for any length of time.
• To prevent a pain crisis, people with SCD can: drink plenty of water, try not to become exposed to extreme heat or cold, avoid situations that expose you to high altitudes, or take medications (as seen fit by your doctor!)
• Most patients can be treated with over the counter pain medications such as ibuprofen or aspirin.
• Anemia - with SCD, the red blood cells die early, which means there are not enough healthy red blood cells to carry oxygen throughout the body. The following symptoms may occur when this happens: tiredness, irritability, dizziness/lightheadedness, pale skin color, jaundice, slow growth, delayed puberty.
• Blood transfusions are used in severe cases of anemia.
• Infection - people with SCD, especially infants and children, are more susceptible to infection. Pneumonia is one of the leading causes of death in children with SCD.
• Vaccines can protect against harmful infections along with other overall good hygienic practices (i.e. washing your hands with soap many times a day, safely prepared food, penicillin)
•  Acute Chest Syndrome - this can be life-threatening and should be treated in a hospital. Symptoms and signs are similar to pneumonia and include chest pain, coughing, difficulty breathing, and fever.
• Adults can take a medicine called hydroxyurea to help prevent acute chest syndrome, but need to be watched closely for side effects such as low white blood cell count which increases the risk of infection.
• Splenic Sequestration - this happens when a large number of sickle cells get trapped in the spleen and cuase it to suddenly get large. Symptoms include sudden weakness, pale lips, fast breathing, extreme thirst, abdominal pain on the left side of the body, and fast heartbeat.
• Treatment typically is a blood transfusion.
• Vision Loss - includes blindness and can occur when blood vessels in the eye become blocked with sickle cells and the retina gets damaged. Some patients develop extra blood vessels in the eye from the lack of oxygen. Laser treatment often can prevent further vision loss if the retina is damaged by excessive blood vessel growth.
•  Leg Ulcers - usually occurs on the lower part of the leg. They happen more often in males than females and usually appear from 10 - 50 years of age. A combination of factors cause ulcer formation, including: trauma, infection, inflammation, and interruption of the circulation in the smallest blood vessels of the leg.
• Stroke - a stroke can happen if sickle cells get stuck in a blood vessel and clog blood flow to the brain. About 10% of children with SCD will have a symptomatic stroke. Stroke can cause learning problems and lifelong disabilities.
• A special exam called, transcranial Doppler ultrasound (TCD), can help identify children who are at risk for stroke. Frequent blood transfusions can help to prevent stroke.
• Deep Vein Thrombosis & Pulmonary Embolism - sickling of red blood cells can increase blood coagulations and induce an increased risk of  blood clot in a deep vein (DVT), or in the lung (PE) if the clot moves from the deep vein. DVT and PE can cause serious illness, disability and, in some cases, death.
•  Other possible complications - damage to body organs (i.e. liver, heart, or kidneys), tissues, or bones because not enough blood is flowing to the affected area(s), gallstones, priapism, and renal medullary carcinoma (a very rare form of kidney cancer).

Sources:

Centers for Disease Control and Prevention

What is Sickle Cell Anemia?

According to the U.S. Department of Education and IDEA 2004, this is what they have to say on Sickle Cell Disease:

Regulations: Part 300 / A / 300.8 / c / 8
(8) Orthopedic impairment means a severe orthopedic impairment that adversely affects a child's educational performance. The term includes impairments caused by a congenital anomaly, impairments caused by disease (e.g., poliomyelitis, bone tuberculosis), and impairments from other causes (e.g., cerebral palsy, amputations, and fractures or burns that cause contractures).

Regulations: Part 300 / A / 300.8 / c / 9 / i
(i) Is due to chronic or acute health problems such as asthma, attention deficit disorder or attention deficit hyperactivity disorder, diabetes, epilepsy, a heart condition, hemophilia, lead poisoning, leukemia, nephritis, rheumatic fever, sickle cell anemia, and Tourette syndrome...

Sickle cell disease (SCD) refers to a group of genetic red blood cell disorders. Individuals with SCD have abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in their red blood cells. In other words, in SCD, red blood cells become hard and sticky and have a crescent-like "C" shape to them.

Hemoglobin is the protein in red blood cells that carries oxygen through out the entire body. In order for cells in tissues to receive a steady supply of oxygen, the protein in the hemoglobin takes up oxygen in the lungs and carries it to these tissues in the rest of the body.

Red blood cells with normal hemoglobin look like a disc; however, a red blood cell that has a sickle hemoglobin are more crescent shaped.

The following image shows where normal red blood cells flow freely through a blood vessel, while sickle cells will stick together and cause blockage:

According to the National Heart, Lung, and Blood Institute:

"People who have SCD inherit two abnormal hemoglobin genes, one from each parent. In all forms of SCD, at least one of the two abnormal genese causes a person's body to make hemoglobin S. When a person has two hemoglobin S genes, Hemoglobin SS, the disease is called sickle cell anemia. This is the most common and often most severe kind of SCD."

There is no widely available cure for sickle cell disease; however, there are many emerging new treatments that are currently being evaluated. Younger patients with severe SCD can undergo a stem cell transplant involving the transplant of bone marrow of a healthy donor to the SCD patient. Although this can cure a small number of patients, it can have life-threatening side-effects in other patients. Other treatments include gene therapy, and new medications to stimulate production of fetal hemoglobin in order to prevent the red blood cells from sickling.

Sources:

• IDEA 2004
• National Heart, Lung, and Blood Institute
• Health Care Corner
• Centers for Disease Control and Prevention