rotate_FB_master
Education
ABC OF SCD

What is Sickle Cell Disease (SCD)?
Sickle Cell Disease (also called Sickle Cell Anemia) is a group of red blood cell disorders you have inherited and are born with. SCD is caused by an abnormal form of Hemoglobin. This is the part of your red blood cell that carries oxygen around the body and keeps your vital organs working. In SCD, the abnormal Hemoglobin (HbS) is not able to work properly. The red blood cells become stiff and block up the blood vessels in your body, causing pain and damage, and they also get destroyed quickly, leading to anemia and other complications. Currently there is no universal cure for SCD and approximately 2500 Ontarians have the disease. 

What makes the red cell sickle? There is a substance in the red cell called hemoglobin. It carries oxygen from the air in our lungs to all parts of the body including the blood cells. One little change in this substance causes the hemoglobin to form long rods in the red cells when it gives away oxygen. These rigid rods change the red cells into a sickle shape instead of the normal round shape

Normal red blood cells contain hemoglobin A. Hemoglobin S and hemoglobin C are abnormal types of hemoglobin. Normal red blood cells are soft and round and can squeeze through tiny blood tubes (vessels). Normally, red blood cells live for about 120 days before new ones replace them.

People with sickle cell conditions make a different form of hemoglobin A called hemoglobin S (S stands for sickle). Red blood cells containing mostly hemoglobin S do not live as long as normal red blood cells (averagely about 16 days).

What does SCD mean to me? It is something you will have your whole life, and need to learn how to live with it, so that you can continue living as normally as possible, with schooling, work, and family life. As it is inherited, you also need to know your partner’s sickle cell status as you could pass it onto your future children too.

Types of SCD There are several types of SCD. The most common are: Sickle Cell Anemia (SS), Sickle-Hemoglobin C Disease (SC), Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia.

What is Sickle Cell Trait (SCT)? SCT (AS) is an inherited condition in which both hemoglobin A and S are produced in the red blood cells, always more A than S. Sickle cell trait is not a type of sickle cell disease. People with sickle cell trait are generally healthy. A carrier has one normal and one sickle hemoglobin gene. A carrier does not have, and will not develop, sickle cell disease. All races should be screened for this hemoglobin at birth.

What does it mean to have SCT? A carrier of sickle cell disease is also said to have the sickle cell trait. This person has inherited a sickle cell gene from a parent, but does not have SCD and is not more likely to get sick than any other person. They do not need special medical care and will not develop SCD  at any time in their life.

Why is it helpful to know that I am a carrier of SCD, or whether my child is a carrier? Knowing whether you or your child has the sickle cell trait is important for several reasons. When you are ready to have a family, your partner can have carrier testing so that you know, as a couple, if there is a chance of having a child with SCD. By having this information, you can let your child know if he or she is a carrier of SCD in the future.

What does my child’s carrier result mean? When a child is a carrier of SCD, it is very likely that one of the parents is also a carrier of SCD. More rarely, when a child is a carrier of SCD:

  • Both parents are carriers
  • One parent actually has SCD
  • One parent is a carrier and the other has SCD
If I have more children, could they have SCD? Most often when a child is a carrier of SCD, only one parent is a carrier and the chance to have a baby with SCD is very low

When both parents are carriers, each pregnancy they have has:

  • 1 in 4 (25%) chance of having SCD
  • 1 in 2 (50%) chance of being a carrier (but not having SCD)
  • 1 in 4 (25%) chance of not having SCD or being a carrier

You can have a blood test to find out if you are a carrier of SCD. If you want to have this test, talk to your health care provider. A carrier does not have, and will not develop, sickle cell disease.

SCT in Ontario: A significant percentage of Ontarians of African descent may carry the sickle cell gene and in some cases, depending on area of origin, the trait rate may be as high as 25%1. People with SCT do not normally experience symptoms of SCD and do not need special medical care. Knowing your Sickle Cell status matters!

How does someone get SCD? Anyone can have SCD or trait including Caucasians but it is seen more in people from Africa, the Mediterranean, Caribbean, Middle East, South East Asia, Western Pacific Region, South America, and Central America. SCD runs in families and is caused by a problem with the hemoglobin gene. Genes are the instructions that tell our bodies how to grow and develop. Most people have two normal copies of the hemoglobin gene – one from their mother and one from their father. A person with SCD has two sickle hemoglobin genes, one from each parent. For a couple to have a child with sickle cell disease, both parents must be carriers. A carrier of SCD has one normal hemoglobin gene

What are the complications to look for? SCD causes problems in 2 ways, by the breakdown of the red blood cell (hemolysis) and by blocking the flow of blood in the blood vessels (vaso-occlusion). This most commonly causes pain, especially in the bones. Other painful complications include priapism (see below), damage to shoulder and hip joints (avascular necrosis), chest pain (acute chest syndrome). There are also a lot of problems that may not be painful. These include damage to the lungs, heart (heart failure, pulmonary hypertension), kidney, liver and eyes, and also stroke, leg ulcers, infections. Because it can damage all of these body “systems”, sickle cell disease is an example of a “multisystem disorder”.

How can I prevent developing these complications? Many complications can be prevented or reduced by either regular blood transfusions or Hydroxyurea tablets. However, both of these have side effects and there are simple lifestyle changes that can make a big difference. These include regular exercise, a good diet, not smoking and not drinking too much. Reducing the amount of stress in your life is also important, as is avoiding things that can provoke a painful crisis. Making sure you are up to date with vaccinations can help to prevent serious infections.

By seeing your doctor in clinic, when not in crisis, you have an opportunity to discuss these ways of coping with SCD, and reducing complications

Treatment of complications: Treatment of complications often includes antibiotics, pain management, intravenous fluids, blood transfusion and surgery all backed by psychosocial support. Like all patients with chronic disease patients are best managed in a comprehensive multi-disciplinary program of care. Screening newborns for SCD is perhaps the first step to effective treatment of the disease. NBS will grant the patient, early enough access to penicillin prophylaxis, vaccination against pneumococcus bacteria and folic acid supplementation.

1. Reference: Tunji-Ajayi, Lanre. “Sickle Cell Disease within the At-Risk Communities.” Scago, www.sicklecellanemia.ca/research.php#gpm1_5

SCD in Ontario

SCHOOLS

The Sickle Cell Awareness Group of Ontario (SCAGO) in collaboration with the Sickle Cell Disease Association of Canada (SCDAC) continues to support the schools in Ontario. Working closely with schools is essential given that students living with sickle cell disease must be encouraged to excel academically. This is doubly important as many of the students are ill-cut for hard manual work.

  • Resources available for schools
  • In-school trainings
  • in-school support for staff, educators, parents and students
  • Online videos and webinars
  • conferences and seminars

Links:
School Chart on Sickle Cell Disease

About Kids Health

Monthly Update from the Board "June 2014"

TCDSB-Recommendation

Peel Public Health reference guide for schools

School Health Reference Guide

Management Plan for SCD- Prepared by the HWCDB

Video Links

  1. https://www.youtube.com/watch?v=GDOs8fppF-s-

This video provides more awareness and education on the subject of sickle cell disease. It is important to use this video when training school educators and staff.
2.  https://www.youtube.com/watch?v=ZI3KJg3jvLc

This video provides a great insight on how sickle cell disease affects the students living with the disorder and how their peers can support them.

For educational videos on various topics relating to Sickle Cell Disease, visit:

https://www.youtube.com/user/scagovids
http://sicklecellanemia.ca/education/video/

 Poster

UNESCO Poster

WSCD-JUNE 19

The Sickle Cell Awareness Group of Ontario (SCAGO); Sickle Cell Association of Ontario (SCAO) and the Thalassemia Foundation of Canada (TFO) called for the recognition of June 19 as the World Sickle Cell Day in Ontario. The private member’s Bill 175 introduced in the Legislature on March 19th 2014 seeks the proclamation of June 19 of every year as the day to celebrate as well as promote awareness of the Sickle Cell Disease in the Province of Ontario.

The Sickle Cell Awareness Group of Ontario asks you to reach 6 people in your community with the message " Get tested for sickle cell trait". For those on twitter, spread the word with hashtag #EachReachSix. Thank you.

Schools: World Sickle Cell Day Message 2016 

10 Tweetable Facts about SCD: Tweet a Fact/Day

1. #SickleCellDisease is 1 of the most common inherited diseases with > 5000 people with #SCD in #Canada + that number is growing
2. Walter C Noel, a Grenadian dentist, 1st person described with #SickleCellDisease > 100yrs ago. A #BloodTest can diagnose carriers of #SCD
3. Persons with #SickleCell #trait carry abnorm hemoglobin #genes but have no symptoms. Get tested-check ur risk of passing #SCD to ur child
4. #SickleCellDisease affects O2 carrying red cells. Red bl cells become rigid, #sickle shaped + break easily causing #anemia + #jaundice
5. Sickled cells block blood vessels and causes severe #pain + #suffering in persons with #SickleCellDisease. Pain may last for days or wks
6. #SickleCells block blood vessels affecting blood flow + O2 delivery. This can cause permanent damage to organs inc brain + kidney #SCD
7. In #SickleCellDisease blood can become trapped in the spleen/liver causing shock and death. An urgent #BloodTransfusion will #SaveALife.
8. Children with #SickleCellDisease have high risk for stroke. Early exchange #BloodTransfusions can #SaveALife + reduce disability in kids
9. Stroke in high risk children with #SickleCellDisease can be prevented with regular #BloodTransfusions – happy bright #HealthyKids!
10. #Pregnancy can increase problems in women with #SickleCellDisease. Transfusions can save both the mother’s + baby’s life #SaveALife

Video links
1. https://www.youtube.com/watch?v=8O_oWWD0zFU
2. https://www.youtube.com/watch?v=GDOs8fppF-s
3. https://www.youtube.com/watch?v=ZI3KJg3jvLc
4. https://www.youtube.com/watch?v=pTlkyXkx2L8

For educational videos on various topics relating to Sickle Cell Disease, visit:
https://www.youtube.com/user/scagovids

School-WSCD-June 19th-flyer.jpg