Colleagues, I rise today to speak to Bill S-280, An Act respecting a national framework on sickle cell disease.

This national framework must include measures to increase awareness, address training needs, create a national research network and a national registry, ensure equitable access to neonatal screening and consider an analysis of the financial impact on patients and families.

I will be speaking as the critic of this bill and, colleagues, this is my first attempt at being a critic.

I want to thank my leadership team and my Conservative colleagues for facilitating this outcome. It is especially significant since this is Black History Month in Canada, and sickle cell disease affects largely — but not solely — the Black community. Furthermore, February 29, 2024, is Rare Disease Day, and this coincides with the International Decade for People of African Descent now extending to 2028, as recently announced by Prime Minister Justin Trudeau.

It has garnered wide support from persons with lived experiences, advocacy organizations and the research and academic community, as outlined by the bill’s sponsor, Senator Mégie.

I wish to thank Senator Mégie and her team for bringing this important bill forward, and for allowing us, as the Senate of Canada, to truly put into action and bring to life the words in our Constitution and the Canadian Charter of Rights and Freedoms — creating an inclusive Canada, where all its citizens can have the opportunity to live to their highest potential.

This bill continues the legislative path started by MP Kirsty Duncan when she tabled Bill C-221, which was meant to implement a comprehensive national awareness for sickle cell disease and thalassemic disorders, which only made it to first reading — and to Senator Cordy’s introduction of Bill S-211 in this chamber, designating June 19 as National Sickle Cell Awareness Day. That bill received Royal Assent in December 2017. Thank you for that, Senator Cordy, and also for reminding us that it was the Honourable Dr. Jean Augustine who first spoke about sickle cell disease in the other place many years ago.

Senator Mégie has thoroughly discussed what sickle cell disease is, as well as its prevalence, complications, the lack of awareness and the lack of research funding. She has also reviewed important treatments that reduce hospitalizations and emergency room visits, and improve health outcomes and potential cures for the disease with gene editing and bone marrow transplants.

I also want to thank Senator Gerba, Senator Ravalia and Senator Cordy, as mentioned, for their insightful and supportive speeches.

I wondered what I could add to this already comprehensive review and compelling case for the need for such a bill, but I also wondered what questions I had on how the committee process might further shed light on this matter. This, colleagues, is how I believe I can fulfill my role as the official critic of the bill.

I decided, again, to look at my own community and dig into my own experiences as a physician — when, early on in my career, I treated a few children with sickle cell disease — and use this to infuse my inquiry.

I want to focus on the following: the development of new quality standards in Ontario; one serious and debilitating complication — stroke — and its prevention in order to show the importance of raising awareness; put this bill in a larger context regarding what this bill does for all Canadians, especially those living with hemoglobinopathies — genetic disorders — and rare diseases; and what could be considered during the committee process that might improve this bill.

Having not experienced the ravages of sickle cell disease in my nuclear or extended family, I, too, even though I trained as a pediatrician over 30 years ago, had limited exposure to the social, economic and psychosocial impact on patients and families — until I practised in my community of Windsor-Essex, and had a few of my own patients who had sickle cell disease. I saw the suffering, disparity in availability of comprehensive health services and lack of protocols, especially related to pain management in vaso-occlusive crises, as already has been discussed. There was a lack of coordinated psychosocial support, and a lack of awareness of the economic impact on patients and the acute on chronic disability experienced by many patients.

Today, Windsor-Essex County is fortunate to have two hematologists who have set aside time to care for patients with sickle cell disease and thalassemia within our community. Dr. Mohammad Jarrar, a pediatric hematologist/oncologist; and Dr. Andrea Cervi, an adult hematologist, both provide care to children and adults in our community, mostly through their private offices. I want to thank Dr. Jarrar for providing me with an update on care in the Windsor-Essex community, and the pending proposal to the Ministry of Health in Ontario to provide more comprehensive care clinics in smaller communities across Ontario. Dr. Melanie Kirby-Allen, co-director of the Hemoglobinopathy Program at The Hospital for Sick Children in Toronto, whom you met last week in the chamber, provided me with a medical update on sickle cell disease, and pointed me to the work of Health Quality Ontario which provides quality standards for care for various diseases across Ontario — in this case, sickle cell disease.

Sickle cell disease is one of the most commonly inherited red blood cell disorders. It leads, of course, to serious health conditions, including infection, stroke, organ damage and acute and chronic pain. It is most prevalent in regions of Africa, the Mediterranean, the Middle East and India, although the sickle cell trait is found in all racial and ethnic groups. As a result of global migration, there has been an increase in the prevalence of sickle cell disease among people of African, Arab and Indian racial/ethnic backgrounds in Canada.

About 3,500 people in Ontario and 6,500 people across Canada have sickle cell disease. According to Newborn Screening Ontario, the incidence of sickle cell disease in Ontario in some populations is about 1 in 400 compared to cystic fibrosis — another gene disorder — where it’s 1 in 3,600. Of course, Senator Mégie spoke about the disparities in funding. This is something we will explore.

As well, I want to mention the health quality standards, which I alluded to in my opening remarks. This health quality standard in Ontario reflects the care that children, youth, adults and families should expect from health care providers, and the standard of care that providers are expected to adhere to.

There are eight such standards or quality statements about the care that patients and families should receive: care free from racism, discrimination and stigma; comprehensive assessments annually from an interprofessional team at a dedicated sickle cell centre; timely assessments of painful vaso-occlusive crises; treatment within 30 minutes of triage and 60 minutes of presentation; referral to a health care professional who’s knowledgeable about chronic pain and sickle cell disease; psychosocial assessments and social supports; and a designated health care provider when transitioning from youth to adult services.

Colleagues, patients and families in the Windsor-Essex region still have to travel to London, which is two and a half hours away, and sometimes to Detroit in the United States, as it is only 30 minutes across the bridge or through the tunnel.

Unfortunately, little has changed in 30 years. This is not good information, but this is why this bill is important.

The hematologists in my community do not have the funding for an interprofessional team to be able to provide comprehensive care that is readily available in these specialized centres. Of course, this often leads to extended periods in emergency rooms, which are already overcrowded, and prolonged hospitalizations.

In my previous statements and speeches, I have noted that a national strategy or framework would allow for standards, sharing of best practices, research and collaboration across jurisdictions at the municipal, provincial, territorial and federal level. It would improve accountability, allow for the collection of data, improve research and help set better policies that could ultimately lead to cost savings and better outcomes. This national framework would give the federal government and Canadians data and an accountability framework necessary to ensure that Canadians receive value for the health care dollars spent.

I would like to salute the efforts of the Sickle Cell Awareness Group of Ontario’s president and chief executive officer Lanre Tunji-Ajayi and the Thalassemia Foundation of Canada’s president Riyad Ebard for their tireless work in raising awareness and working to improve the lives of Canadians, especially those suffering from sickle cell disease and thalassemia.

Honourable senators, I have spoken about the situation in my community in Ontario and some health quality standards that have recently been developed. At this time, I want to focus on ischaemic strokes in childhood. Colleagues, the most devastating neurological consequence is stroke. The fact is that they are more common in children than adults, and the peak age for them to occur is between the ages of two to five years. This bears repeating: The peak age for strokes to occur is between two and five years, and the recurrence risk is up to 93% if untreated.

Thankfully, with newborn screening and early detection of stroke risk by transcranial doppler ultrasonography done in specialized centres, preventive treatment can be provided. This is one area where a national framework on sickle cell disease would be life-saving for many children across Canada. Many of these children would have cognitive and learning problems as a result of even micro-strokes, many of which go undetected.

As Senator Cordy noted in her speech, newborn screening is, however, not universally available across Canada. This is another reason for us to be able to move this bill forward so that, as senators, we have a role to ensure the universality principle and equitable access as outlined in the Canada Health Act.

Honourable senators, I want to share some thoughts on the broader implications of Bill S-280, An Act respecting a national framework on sickle cell disease. Connecting the dots, as it were. You are going to get used to hearing me say that. That is one of my favourite phrases.

Humans are 99.9% similar in their DNA. This means that what we learn from a single gene disorder like sickle cell disease will invariably help with the treatment of other genetic disorders. In a recent program, Nobel Laureate in Chemistry Jennifer A. Doudna noted that gene editing techniques like those used in CRISPR, having recently become available as a potential cure for sickle cell disease, may likely have therapeutic implications for other genetic disorders, such as Duchenne muscular dystrophy, cystic fibrosis, Huntington’s disease and other rare disorders. Of course, I mentioned Rare Disease Day, which is on February 29.

I believe that my critique of the bill, which I will discuss below, will be explored during committee. My question to Senator Mégie was: Why was the bill specific to sickle cell disease and not inclusive of hemoglobinopathies or sickle cell disease and thalassemia, being the other hemoglobinopathy?

Hemoglobinopathies are a group of inherited disorders in which there is an abnormal production of the hemoglobin molecule. Both sickle cell disease and thalassemia have a different gene defect in the globin gene for hemoglobin, but are treated by the same clinicians.

She said to me there is a significant lack of awareness about sickle cell disease and, of course, there are the many acute and life-threatening unique features of sickle cell disease, including the frequent visits to the emergency room, pain medications and it being predominantly in the Black population and the acknowledged presence of anti-Black racism experienced in the health care system. This is one of the things we will be able to explore in our committee. Another area that could be more thoroughly explored is the economic and social impact of sickle cell disease on patients and their families.

In closing, I believe that the committee will investigate and explore ways that the bill could be improved and provide significant information to Canadians. It would be important to hear testimony from witnesses with lived experiences, advocacy organizations and the research community, including funding agencies, government, philanthropic organizations, clinicians and experts who could provide analysis of the economic, financial, educational and psychological impacts.

I want to salute Senator Mégie for her tireless efforts in bringing this bill forward, and I urge you, dear colleagues, to vote to send this to committee for further study.

Senator Burey, will you take a question?

Certainly.

Thank you for your speech, Senator Burey. You gave it like a veteran senator. It was a very good critic speech. And thank you to Senator Mégie for her excellent sponsorship work.

I want to come back to how you started the speech around Black History Month. You and I have something in common, we are people of Caribbean background and therefore we are among the folks most likely to inherit sickle cell anemia. You spoke of the stigma. I am aware in eastern Ontario of the Interdisciplinary Centre for Black Health through the University of Ottawa. They are doing a lot of awareness work.

I wonder if you could speak further about the stigma around sickle cell and what barriers the Black community would face because there is inherent racism in how we access services. I wonder if you could comment further on that.

Thank you so much, Senator Clement. This is a very important point. I want to return to something that Senator Cordy mentioned in her speech when it was the Honourable Dr. Jean Augustine who spoke about sickle cell disease and everyone said, “What is that?” not knowing that it affected so many Canadians.

The lack of awareness is one of the reasons why there is so much stigma associated with it. We spoke of the vaso-occlusive crisis, which are painful events when you come in and you are thought of as a drug-seeking person so you are stigmatized and not given appropriate pain medication. We already know from many research studies that when racialized people go to the hospital asking for pain medication, their pain score is thought to be not as relevant as someone from another ethnic group.

We know there is a lot of work to be done. I think that having this bill to raise awareness will help to decrease that stigma. It will also allow people to talk about it — I’m talking about people who are suffering from sickle cell disease — so that it will be able to enter the conversation. We all know about mental health and how, for a long time, we know that it came out of the Senate in Out of the Shadows at Last, the mental health report that was so important. Talking about it brings things out of the shadows. That is what is really important.

Thank you, Senator Mégie. In medicine, we learn of all the hemoglobinopathies. If you remember, a hemoglobinopathy is a disease related to the hemoglobin as they are a part of it. We learn about thalassemia disorders, and you will see that most clinics are called “hemoglobinopathy clinics,” but they service kids who have sickle cell disease and thalassemia.

Interestingly, there are different kinds of sickle cell disease. Senator Mégie alluded to that. There is one where you have the SS trait or one where you have the SC. You will have one where there is sickle cell and thalassemia, and then another one where you have sickle cell Punjab disease, so we learn about all of those things.

Senator Mégie, do you have another question?

Technically, you should be asking a question, but you can make your comment.

No, I have not asked Dr. Melanie Kirby-Allen about having the stand-alone with just sickle cell in that framework. I will ask her that question, and I believe we will be able to explore this further in the committee process.