June 12, 2020- Toronto, Ontario: These last few weeks have become increasingly challenging
as we saw social injustice with the tragic killing of George Floyd, Briana Taylor, and so many others; sparking global unrest and demonstrations. The unnecessary deaths triggered discussions on how much, or how little, has changed when it comes to racism, and the demonstrations that started in Minneapolis where George Floyd died in police custody have spread all over the world, and even to our own country, Canada.
The killings only demonstrate the inequalities faced by the racialized communities - especially Black communities - in every area of life, including inadequate access to health care, food, housing and jobs, and of course the unfair target by the police bullet.
It is our duty as a community to come together to take steps towards reforming and advocating for equality in access to healthcare and to also ensure that no one should be discriminated against based on the color of their skin, ancestry background, sexual orientation, health challenges, and where they live.
According to PM Trudeau in a statement issued on June 2nd, Canadians must be aware of the
challenges facing Black Canadians and other minorities and take steps to address them. There is
systemic discrimination in Canada, which means our systems treat Canadians of color, Canadians who are racialized differently than they do others.
In sickle cell disease, systemic racism has always been very real as individuals with the disease presenting in Canadian hospitals with vaso-occlusive crisis (painful episode representing the hallmark of the disease) are treated as drug seekers compared to their non-racialized counterparts presenting with pain.
This led SCAGO in 2019 to write Dr. Rueben Devlin who leads the Ontario Premier’s council on improving health and ending hallway medicine that systemic racism is not only costing the system unnecessary health care dollars, it is costing many their lives and it is no longer acceptable! Currently, a working group comprising of representatives from different racialized communities is working on the next steps on this important health advocacy initiative. Racial injustice in health care is unhealthy and must be stopped!
In healthy individuals, red blood cells are round and flexible and move freely through blood vessels to distribute oxygen to all parts of the body. Individuals with SCD have red blood cells which are “sickle” in shape (C-shaped), sticky, and inflexible. These structurally modified red blood cells have difficulty moving through the blood vessels and instead adhere to vessel walls, causing clots within the vessels, inflammation and limiting oxygen distribution to critical organs. Persons with SCD often experience significant pain, and other complications such as infections, strokes, and acute chest syndrome.
About the Sickle Cell Awareness Group of Ontario (SCAGO)
Since 2005, SCAGO has been creating and providing evidence-based supports and services to enhance the health and the quality of life of families with SCD. Its broader vision is for every Ontarian with SCD to have equitable access to comprehensive, standard care regardless of place of residence in the province.
Twitter: @SCAGO1; Instagram: @SCAGO.1 Website: www.sicklecellanemia.ca
Visit our website to learn more about sickle cell disease.
There is currently no data that specifically speaks to how persons with SCD will be affected by this virus. Persons with SCD are however considered immunocompromised and should therefore take every precaution to protect themselves from exposure.
There is currently no vaccine to prevent COVID-19. The best way to prevent infection is to avoid being exposed to this virus.
WE URGE YOU TO LISTEN TO REPUTABLE NEWS SOURCES AND AVOID UNRELIABLE SOCIAL MEDIA INFORMATION WITHOUT FACT CHECKING THE SOURCE FIRST.
Here are some reputable websites with regular updates:
World Health Organization (WHO)
Centre for Disease Control (CDC)
Twitter – Toronto Public Health
Wearing a face mask or covering may not protect you from COVID-19, but it may protect others from your respiratory droplets and germs. The best protection is to stay home, keep six feet from others, and wash your hands often. However, if you are unable to maintain a two-meter (six feet) distance from others, such as on transit, in an elevator, grocery shopping, or entering and leaving your apartment building, a face mask or covering can be used.
You can make your own mask with materials you already have at home (e.g. cotton t-shirt or pillowcase) or use a scarf or bandana to cover your face.
How to Safely Wear a Mask or Face Covering
How to Safely Take a Mask Off
Who Should Not Wear a Mask or Face Covering
Wearing a Mask at Work
Follow instructions provided by your employer regarding the option of choosing to wear a non-medical mask or face covering.
Do Not Use Medical-Grade Masks
It is extremely important that we keep the supply of medical masks for healthcare workers where they are urgently needed for medical procedures, and to care for individuals who have COVID-19. Healthcare workers need medical masks, including N95 and surgical masks.
STROKES IN SCD
Dr. Kevin Kuo is an adult hematologist at Toronto General Hospital. He has given many hours to patient education. He provided in-depth teaching on Strokes in Sickle Cell Disease to both the Medical and the patient community.
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What is Iron Overload?
Toxic iron overload is an inevitable consequence of regular blood transfusion. Excess iron cannot be excreted by individuals living with sickle cell disease hence it is deposited in parenchymal tissues and toxicity results in organ damage.
Are You At Risk for Iron Overload?
Anyone who has received more than 10 transfusions is at risk for iron overload.
Every unit of blood contains about 200-250mg of iron. A single transfusion of two units would provide over 100 times what you would normally get from your diet in a day and is equivalent to 1-2 years’ worth of iron intake!
If you receive frequent blood transfusion, you should be discussing iron overload with your doctor
What is Hydroxyurea?
Hydroxyurea is a drug (capsule) that was originally used to treat some forms of blood cancer (Leukemia) and blood disorders that can later turn into Leukemia. It has been used for over 15 years to treat sickle cell disease.
Hydroxyurea works in a few different ways, but the main way is to increase your body’s production of Fetal (baby) Hemoglobin.
We know that this then protects you against sickle cell disease. It is a safe drug and there is no evidence to suggest increases the risk of cancer in patients with sickle cell disease. Like all drugs, it does have some side effects, and you need regular blood test monitoring. Also, unlike painkillers which you take when required, Hydroxyurea must be taken every day to be effective.
Hydroxyurea is the only drug therapy available for sickle cell disease and has been shown to prolong survival, reduce pain episodes and some of the complications. Unless you have been told by a specialist that you have very mild Sickle Cell Disease, you should consider going on the drug.
Why am I being offered this treatment?
You are most likely to be offered Hydroxyurea because you have lots of painful crisis or Acute Chest Syndrome. It may also be offered if there is concern about damage to any of your body organs from the sickle cell disease, or as an alternative to blood transfusion treatment.
Other available treatments for sickle cell disease
Many individuals with sickle cell disease may also be offered continuous blood transfusion or blood exchange. These therapies will also help to increase the level of the non-sickling blood in the system.
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 and approximately 4000 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 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 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 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 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:
If I have more children, could they have Sickle Cell Disease?
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:
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 healthcare provider. A carrier does not have, and will not develop sickle cell disease.
Sickle Cell Trait in Ontario:
A significant percentage of Ontarians of African descent may carry the sickle cell gene and in some cases, depending on the 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 Sickle Cell Disease?
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 the 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 the 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 are 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.