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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. Sickle cell disease 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 sickle cell disease, 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 sickle cell 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 sickle cell disease 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 sickle cell disease There are several types of sickle cell disease. 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? Sickle Cell trait (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 the sickle cell trait? 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 sickle cell disease and is not more likely to get sick than any other person. They do not need special medical care and will not develop sickle cell disease at any time in their life.

Why is it helpful to know that I am a carrier of sickle cell disease, 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 sickle cell disease. By having this information, you can let your child know if he or she is a carrier of sickle cell disease in the future.

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

  • Both parents are carriers
  • One parent actually has sickle cell disease
  • One parent is a carrier and the other has sickle cell disease

If I have more children, could they have sickle cell disease? Most often when a child is a carrier of sickle cell disease, only one parent is a carrier and the chance to have a baby with sickle cell disease is very low

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

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

You can have a blood test to find out if you are a carrier of sickle cell disease. 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.

How does someone get sickle cell disease? Anyone can have sickle cell disease 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. Sickle cell disease 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 sickle cell disease 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 sickle cell disease has one normal hemoglobin gene

What are the complications to look for? Sickle cell disease 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 sickle cell disease, 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.

Treatment Screening newborns for sickle cell disease 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.

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

A Plan to Support Students Living with Sickle Cell Disease and Recognize The United Nations World Sickle Cell Day-June 19 th In Ontario Schools
In Partnership with the Sickle Cell Disease Association of Canada
What is Sickle Cell Disorder (SCD)?
Sickle cell disorder (SCD) is a collective name for a series of serious inherited chronic conditions that can affect all systems of the body.

It is one of the most common genetic conditions in the world. The percentage of people who are carriers of the sickle cell gene is as high as 25% in some regions of the world

The life span of persons with these disorders can be reduced by as much as 30 years

Of all hemoglobinopathies, sickle cell disease affects the largest number of patients approximating 250,000 out of about 300,000-400,000 children born annually worldwide with various hemoglobinopathies. The prevalence of this condition is of higher magnitude than other, well-resourced congenital conditions such as hemophilia or cystic fibrosis

What is Sickle Cell Disease (SCD)?
People with sickle cell disease have a type of haemoglobin(called haemoglobinS (HbS) or sickle haemoglobin) which differs from normal adult haemoglobin(haemoglobinA or HbA). This can cause red blood cells to change shape and become blocked in the blood vessels, causing acute pain.

The main types of sickle cell disease are sickle cell anaemia, haemoglobinSC disease and sickle beta-thalassaemia.

The two main characteristics of SCD are a longstanding anemia and recurrent episodes of vaso-occlusion

Anemia is a result of increased breakdown of red blood cells. Student may appear pale and have yellow eyes from time to time

Vaso-occlusive episodes are blockages of the blood vessels anywhere in the body by deformed red blood cells. This causes a lack of oxygen in the affected area of the body

Sickle Cell Disease
In Canada
Immigration is changing the face of these disorders in Canada. Approximately 2/3 of the 1% annual growth in Canada’s population is due to net migration; 20% of the Canadian population is now foreign-born

Canadians affected by sickle cell disorders are both immigrants and Canadian-born with diverse ethnic
backgrounds- African, Caribbean, Mediterranean, Middle East, South America, South Asia

It is one of the most common genetic conditions in Canada and affects approximately 3500-5000 Canadians, 2000 of which are Ontarians.

Sickle Cell in Ontario Schools

Children and Youths with sickle cell disease attend Ontario Schools. An important part of school inclusiveness is recognizing the importance of offering care to young people with long-standing illness, particularly since a major part of childhood is spent in attending school.

The Ontario education system supports healthy living for all students while ensuring health and safety of those with Diabetes, Asthma, and Allergies.

It is equally important for the school system to support health and safety of students with other medical conditions including sickle cell disease

Since those with sickle cell disease are ill-suited to hard manual work, it becomes doubly important for them to receive a good education and good support to excel.

Ref: www.edu.gov.on.ca/eng/healthyschools/links.html

Gaps in the current School System
Lack of Individual Health Care Plan for students with SCD

Inadequate Support for Students with SCD

Lack of Awareness and Inadequate understanding of the disease by teachers and other school employees

Recommendations to combat Inadequate Support for Students with SCD in Ontario Schools
Sickle cell disease is associated with episodes of severe, debilitating pain called sickle cell crisis.

It is imperative for educators to be cognizant of simple measures to help reduce the number of school environment induced crisis suffered by students with sickle cell disease
Recommendations include:

Registration: On entering the school system, the student registration form should provide information on students’ sickle cell status –either if a student has sickle cell disease or trait.

Recommendations to combat Inadequate Support for Students with SCD in Ontario Schools
Individual Health Care Plans (IHCP): All children with SCD should have individual health care plans carefully crafted within or separately from the Individual Education Plan (IEP), which should be reviewed yearly. As SCD have numerous possible complications affecting many systems of the body, it is important, where possible, to include a specialist sickle cell nurse or hematologist in drawing up this plan.

Our association is able to refer the school to such resources.

Recommendations to combat Inadequate Support for Students with SCD in Ontario Schools
School absences: It is known that a student with SCD could miss days even weeks of schooling a year due to the disease. Most pupils with SCD do not feel supported by schools in catching up these absences. A minority have absences at or beyond levels defined by government as persistently absent.
It is important that such students are not mislabeled by educators as truant and their parents pressured to account for themselves if such absences are the result of serious episodes of illness.
We recommend that the school system have strong supportive frameworks to provide catch-up mechanism for these students
Recommendations to combat Inadequate Support for Students with SCD in Ontario Schools
Tiredness: The person with SCD may experience severe anaemia. This may mean they feel tired, lethargic and unable to concentrate. They may feel tired to the point where they feel they need to sleep.
It is important that teachers do not mistake serious medical symptoms of SCD for laziness.
Climbing several flights of stairs several times per day to get to and from the classroom is physically demanding for some young people with SCD. In some cases issuing a personal lift pass may be appropriate
Recommendations to combat Inadequate Support for Students with SCD in Ontario Schools
Water: Young people with SCD need to be well hydrated to reduce the likelihood of becoming ill. It is important to have a ready supply of fresh drinking water available and do not restrict drinking water in class. Ensure water fountains are working and kept in the highest state of cleanliness so young people with SCD are not put off using them and risk of infection is kept to a minimum.

Using the Toilet: People with SCD cannot concentrate urine as readily. They produce large quantities of dilute urine and need to go to the toilet more often. Do not restrict toilet breaks for children

Recommendations to combat Inadequate Support for Students with SCD in Ontario Schools
Physical Exercise:Avoid hard, physical exercise involving strenuous exertion that could precipitate a sickle cell crisis. Encourage moderate exercise. Listen to the young person who will come to know their own safe limits of physical activity. Do not refuse requests if a young person asks to be excused or stop activity because of tiredness or pain. For children with SCD, cold or wet weather, or exposure of the skin to cooling wind may all be a trigger to episodes of illness.
Obligatory sports and gym sessions out of doors in cold and wet weather is a potent stimulant to crisis for some children. It is important to listen to the child and parent, and follow advice from their specialist medical teams about this.

The GYM Program needs to be modified for children with SCD. SCDAC and SCAGO could contribute to the re-modification of the program.

Recommendations to combat Inadequate Support for Students with SCD in Ontario Schools
Stroke and silent cerebral infarcts: Individuals with SCD including children many a times suffer from silent stroke and cerebral infarcts that could causesubtle cognitive dysfunction and impair/impact their academic performance.

A drastic or steadily declining change in academic performance might be a warning sign for silent stroke. Hence having a record of students’ sickle cell status would aid the educator in identifying a child who might need further investigation of subtle cognitive dysfunction.

Recommendations to combat Inadequate Support for Students with SCD in Ontario Schools
Infection: Young people with SCD may have a damaged or missing spleen (the organ that helps to fight infections). All children with SCD should be on prophylactic antibiotics, and if they have an active infection resulting in fever, the caregiver should be contacted immediately. The school needs to understand that they may be absent from school more often than other children due to infection. The school also need to enable safe storage and dispensing of any antibiotic drugs prescribed for the young person with sickle cell disorder

Temperature: Avoid activities that require outdoor work in cold or damp conditions; avoid under-heating of classrooms, especially mobile classrooms; maintain good ventilation of study areas. Allow coats to be worn in class, and permit the child with SCD to stay inside at break in cold or wet and windy weather.

Recommendations to combat Inadequate Support for Students with SCD in Ontario Schools
In conclusion, It is important that: Students with sickle cell disease do not feel singled out or set apart by their disease. They should still be encouraged to participate actively within their reasonable restrictions. They truly understand their limits. Educators could help them excel by operating within it
Recommendations to Combat Lack of Awareness and Inadequate Understanding of the Disease by Teachers and Other School Employees
Improved education: To improve the understanding of teachers and other employees within the school system

We recommend that schools team up with local hospitals and SCAGO to learn more about SCD and provide some information to the teachers on the subject

SCD Fact Chart: SCAGO will supply a one-page fact sheet for quick reference /use by educators

Videos and resources: On its school page, SCAGO has a library of videos and resources readily available for use by educators- http://www.sicklecellanemia.ca/education.php

Training/Webinars/Seminars: SCAGO in partnership with the local treatment centrescould provide more support and information on the subject via in-school training, webinars and seminars

June 19th
In 2006, the World Health Organization passed a resolution calling for the development and implementation of national, integrated programs for the prevention and management of sickle-cell anaemiain particular.

Ontario schools can raise more awareness around the disease by partnering with SCDAC-SCAGO; by including June 19thin the school calendar, encouraging all tutors, staff, and students to watch one or more education video on sickle cell disease and by sending a notice home on June 19thof every year in recognition of the The United Nations Declared World Sickle Cell Day-June 19th

Ref: WHO 117th Session EB117.R3, Agenda item 4.8 25 January 2006: Sickle-cell anaemia (http://apps.who.int/iris/bitstream/10665/20668/1/B117_R3-en.pdf, accessed 2013-11-15)-Dr. Pendergrast’sMOH Document

Advocating for Individuals with SCD
Bill 165, An Act to establish Sickle Cell and Thalassemia Care Ontario and to proclaim Sickle Cell and Thalassemia Awareness Day

In June 2011, Member of Provincial Parliament Mike Colle introduced Bill 165 to address the challenges faced by children and adults living with Sickle Cell and ThalassemicDiseases in Ontario.

Sickle Cell Awareness Group of Ontario, a member organization under the umbrella of the Sickle Cell Disease Association of Canada led the Action Day on March 19th2014 to drum support for Bill 165 and advocate for the establishment of a comprehensive Provincial Strategy for Sickle Cell and Thalassemia Diseases that would place Ontario in a leadership position and provide a model to be emulated by other Provinces.

How to Contact us

Sickle Cell Awareness Group of Ontario (SCAGO)
235-415 Oakdale Rd. North York, ON. M3N 1W7.
Website: http://sicklecellanemia.ca/education/
Email: info@sicklecellanemia.ca

Sickle Cell Disease Association of Canada (SCDAC)
1030 MountCastleCres. Pickering, ON. L1V 5H9
Website: www.sicklecelldisease.ca
Email: president@sicklecelldisease.ca

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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