A Plan to Support Students Living with Sickle Cell Disease and

Recognize The United Nations World Sickle Cell Day- June 19th

In Canadian Schools

In Partnership with the SCDAC/AAFC’S member organizations

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

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What is Sickle Cell Disease (SCD)?• People with sickle cell disease have a type of haemoglobin (called

haemoglobin S (HbS) or sickle haemoglobin) which differs from normal adult haemoglobin (haemoglobin A 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, haemoglobin SC 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

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Sickle Cell Disease

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In Canada

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• 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.

Sickle Cell in Canadian Schools• Children and Youths with sickle cell disease attend Canadian

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.

• Most provincial education system in Canada 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 6

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

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Recommendations to combat Inadequate Support for Students with SCD in Canadian 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.

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Recommendations to combat Inadequate Support for Students with SCD in Canadian 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.

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Recommendations to combat Inadequate Support for Students with SCD in Canadian 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 studentsRef: http://www.sicklecelldisease.ca/wp-content/uploads/2013/10/School-Policy_Sickle-Cell-and-Thalassaemia_Version_1_1_Electronic.pdf

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Recommendations to combat Inadequate Support for Students with SCD in Canadian 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

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Recommendations to combat Inadequate Support for Students with SCD in Canadian 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

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Recommendations to combat Inadequate Support for Students with SCD in Canadian 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 could contribute to the re-modification of program.

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Recommendations to combat Inadequate Support for Students with SCD in Canadian Schools

• Stroke and silent cerebral infarcts: Individuals with SCD including children many a times suffer from silent stroke and cerebral infarcts that could cause subtle 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.

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Recommendations to combat Inadequate Support for Students with SCD in Canadian 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.

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Recommendations to combat Inadequate Support for Students with SCD in Canadian 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.

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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

patient support groups to learn more about SCD and provide some information to the teachers on the subject

• SCD Fact Chart: SCDAC will provide a one-page fact sheet for quick reference /use by educators

• Videos and resources: SCDAC has a library of videos and resources readily available on its website to educators-

http://www.sicklecelldisease.ca/media-room/videos/http://www.sicklecellanemia.ca/education.php

• Training/Webinars/Seminars: SCDAC in partnership with local sickle cell organizations and treatment centres could provide more support and information on the subject via in-school training, webinars and seminars.

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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 anaemia in particular.

• Canadian schools can raise more awareness around the disease by partnering with SCDAC; by including June 19th in 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 19th of 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’s

MOH Document18

Advocating for Individuals with SCD

Bill C-221, An Act respecting a Comprehensive National Strategy for Sickle Cell Disease and Thalassemic Disorders•In June 2011, Member of Parliament Kirsty Duncan introduced Bill C-221 to address the challenges faced by children and adults living with these inherited blood disorders. This Bill supports each of the key activities which the SCDAC is calling on the federal government to help establish.

•Sickle Cell Disease Association of Canada is holding its Advocacy on the Parliament Hill on May 5th, 2015 to drum support for Bill C221 and to advocate for the establishment of a comprehensive National Strategy for Sickle Cell and Thalassemia Diseases that would place Canada in a leadership position and provide a model to be emulated by other countries.

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How to Contact us

Sickle Cell Disease Association of Canada (SCDAC/AAFC)

Unit 34, 260 Adelaide St. E, Toronto, ON M5A 1N1.Website: www.sicklecelldisease.ca Email: president@sicklecelldisease.ca

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