Sickle Cell Basics
What is sickle cell disease?
It’s something you were born with caused by an abnormal gene that impacts your red blood cells. This makes some of your red blood cells shaped like a half moon. The job of the red blood cell is to carry oxygen throughout the body. Sometimes, the sickle cells can clump together because their shape make it harder to flow throughout the body. When this happens, it can cause pain or other problems because it blocks the flow of oxygen.
What is acute chest syndrome?
The sickled red blood cells become trapped in the blood vessels of the lungs.
What temperature is a fever? What should you do if you have a fever?
Fever is a temperature of 101.5 degrees or higher. Fevers happen when your body is fighting an infection.
Go to the Emergency Room. Do not take Tylenol or ibuprofen.
Children with sickle cell disease have an increased risk of infection because their spleen does not work normally. The spleen is an important part of the body’s defense against infection and works by removing bacteria from the bloodstream.
How does sickle cell cause pain? What is happening in your body that causes pain?
When sickle cells clump together, it causes a “traffic jam” in your blood vessels and the red blood cells carrying oxygen cannot get through as easily.
Your friends see you using a heating pad and stress ball in the middle of class. How can you explain why you are allowed to do this?
Share what you are comfortable sharing.
What is a genotype?
The kind of sickle cell you have:
Hemoglobin SS Disease
Hemoglobin SC Disease
Hemoglobin Sickle Beta Plus Thalassemia (SB+) Disease,
Hemoglobin Sickle Beta Zero Thalassemia (SB0) Disease
Hemoglobin S-other Diseases
What are some symptoms of acute chest syndrome?
Coughing
Difficulty breathing/fast breathing
Chest pain
Fever
What is a spleen and where is it located in your body?
The spleen is a small organ located on the upper left side of the abdomen under the rib cage. It acts as part of the body’s defense system by removing bacteria from the blood.
What are some coping skills you use to manage pain?
deep breathing, guided imagery, massage, use heating pad/packs, squeeze a stress ball, rest, distract yourself
It is the summer, and you are at band practice. Your coach tells the group, “No water breaks until the end of practice!” What do you do? Why is it important for your coach to know about your sickle cell?
Talk with your coach and tell your family
Keep a water bottle with you
Make a plan for you to be able to take breaks
What is hemoglobin?
Hemoglobin is a protein inside red blood cells and that carries oxygen throughout the body. When your hemoglobin is very low, you may need a blood transfusion.
What is avascular necrosis?
This is a bone condition that occurs in joints when sickled red blood cells block the flow of blood to the joints. When the joint does not receive enough blood supply, it can become weak and die. This can lead to persistent pain in the joint. AVN may cause in persistent hip, knee or shoulder pain.
What is splenic sequestration?
Sickle cells can get trapped in the blood vessels of the spleen, causing a blockage in blood flow. When blood stays inside the spleen instead of flowing back out into the body, this is called splenic sequestration. During splenic sequestration, the blood cell count falls, and the spleen becomes enlarged.
What are things that you can do to help prevent pain/ take care of yourself?
Drink lots of water.
Try not to let your body get too hot or too cold.
When it’s hot outside, take breaks from the heat to rest and drink water.
When it’s cold outside, dress in warm clothing and a hat when you’re outside.
Take medicine that your doctor tells you to take.
Take breaks during activities like sports or swimming.
Get plenty of rest.
You’re at school and you need to use the bathroom. Your teacher tells you need to wait until after class. What do you do?
Explain why it’s important for you not to wait when you need to go to the bathroom
“I have sickle cell and cannot hold it because that is bad for my kidneys”
Go to the bathroom
What is a blood transfusion?
A blood transfusion is a common, safe medical procedure in which healthy donated blood goes through an IV (straw) into your vein. A single transfusion may be given when a child is sick and their red blood cell count is too low. Some kids need transfusions every month to help keep them healthy.
What is a stroke?
A Stroke happens when part of the brain isn’t getting enough blood flow/oxygen. Red blood cells carry oxygen throughout the body. Sickled red blood cells can form an obstruction in the bloodstream of the brain that prevents oxygen from being delivered to parts of the brain.
What is jaundice?
Jaundice is a condition where the skin and whites of the eyes turn yellow. Bilirubin is normally made as the body breaks down hemoglobin which is in the red blood cells. The yellow color that you see in the eyes is caused by a high level of bilirubin from all the sickle cells being broken down.
What are common triggers of sickle cell pain?
Exposure to extreme temperatures (hot or cold)
Stress
Dehydration (need to encourage fluids to prevent)
High altitudes
Over-exertion
Shivering (if wet or cold)
Infection
You are coming back to school after few days in the hospital. Your teachers know you were hospitalized, but you have not told any of your friends. When you get to class, your classmates ask you why you haven’t been at school. How do you respond to your classmates?
You share what you are comfortable sharing.
Name 3 common complications of sickle cell
Pain
Acute chest syndrome
Splenic sequestration
Aplastic crisis
Avascular necrosis
Fever/infection
Sickle cell retinopathy
Gallstones
What are some signs and symptoms of a stroke?
Inability to move part of the body
Weakness or numbness in part of the body
Seizures
Strange or abnormal behavior
Sudden blurry vision
Severe vomiting
Severe headaches that are not relieved with medicine
What is sickle cell retinopathy?
Your retina is the back part of your eye. When not enough oxygen flows to the retina, that tissue can get damaged. This is called sickle cell retinopathy.
What does "vaso-occlusive" mean?
It means that there is a blockage in the blood vessels, usually caused by the sickle cells clumping together.
What is a 504 Plan?
Section 504 of the Rehabilitation Act of 1973 is a civil rights law that protects against disability discrimination in the school setting. It give people with certain chronic illnesses, like sickle cell, accommodations at school that are protected by law.