Debilitating pain … monthly ER visits … pain medication … repeat.
It’s an exhausting cycle that is part of the reality thousands of Canadians face while living with sickle cell disease. Affecting millions worldwide, this rare disease affects the red blood cells – carrying oxygen around the body – and has, so far, primarily been treated with pain medication for symptom relief.
Richard Ward, collaborator with the Princess Margaret team at Toronto’s University Health Network (UHN) and medical director of the Red Blood Cell Disorders Clinic at Toronto General Hospital (TGH), explains that individuals with sickle cell produce a different form of red blood cells. As a result, the oxygen’s “form of carriage” is less effective. Over years and decades, patients are starved of oxygen – causing acute pain episodes from a build-up of acids and toxins in the body and potentially resulting in organ failure.
“If you’ve had a loved one who’s had a heart attack … that clutching of the chest … the agony … It’s almost the same sort of thing that happens to someone with sickle cell but it’s happening throughout their body.”
On May 25, UHN announced that it had joined the ranks of Calgary, Sick Kids and a few centres around the world in performing a “transformative” stem cell transplant from a sibling that can cure some adult sickle cell patients – thus “freeing the patient forever from constant blood transfusions and medical complications related to the illness.”
Serena Thompson, patient advocate, vice president and director of the Sickle Cell Association of Ontario, says this treatment is a step in the right direction.
For the adult patient, the transplant “has made the biggest difference for me. I can do so much more; I have a new sense of energy. I don’t feel sick. I have freedom from sickness,” the patient said in a press release announcing the transplant.
However, Thompson, who has sickle cell disease, notes that while it sounds “so great” to not have to take medication every day, this treatment is not available for most.
“You have to have a 100 per cent match, and that doesn’t always happen when you have a sibling,” says Thompson. “It’s great that some people can be cured but it’s a very small percentage … compared to who’s suffering from the disease.”
Thompson notes that the key step moving forward is to identify the need for a change in approach within the health-care system – developing comprehensive care approaches that rethink how the system manages, treats and medicates patients.
“I think at that point everything else will fall in place,” says Thompson. “There isn’t comprehensive care (now). That alone (would be) a step toward improving health outcomes for patients. And it improves their experience and improves their treatment.”
A big part of it has to do with the makeup of the disease, notes Lanre Tunji-Ajayi, president of the Sickle Cell Awareness Group of Ontario. With the vast majority of sickle cell patients being people of colour, there are racialized systemic barriers for patients trying to receive care in Canada.
“Sickle cell disease is still a disease that is not very much understood in Ontario and in Canada as a whole. It’s stigmatized. There’s a lot of discrimination especially when they present in the hospitals,” says Tunji-Ajayi. “Many times, they end up either not receiving the right care or not receiving the right care at the right time. Some have been turned away because (hospital staff) felt that they are not actually in pain. I could go on and on to talk about the systemic racism and the inequity in health care.”
Ward notes that he “sees the impact” of widespread racism that is “a huge aspect of the disease,” ultimately contributing to a number of preventative complications.
“Compared to individuals that are coming from a middle-class background and who’ve got resources, we see that the patients who are more marginalized and disadvantaged tend to have higher acute care utilization,” says Ward. “By that I mean they have more episodes of coming to the hospital with acute pain that they can’t manage at home, and it’s partly because they are just not able to access the system.
“The vast majority of individuals with sickle cell disease come from an African or a Caribbean background, not all, but the vast majority do, (and) that is not what the health-care system looks like on the physician side. So, you then have these barriers in terms of ‘can I as a middle-aged white guy fully understand … can I fully appreciate and be empathic towards them?’”
The key step moving forward is developing comprehensive care approaches that rethink how the system manages, treats and medicates sickle cell patients.
This issue hits close to home for Tunji-Ajayi. Her brother, Sunday Afolabi, did not receive timely intervention and died of preventable complications due to sickle cell disease in the 1990s.
Tunji-Ajayi estimates that about 65 per cent of individuals with sickle cell disease in Ontario are at the poverty level, affecting their mental health, access to nutritious food, ability to pay for medication, and it sometimes even results in moving away from expensive urban centres – areas that usually have specialized care to deal with rare conditions.
Racial biases are also too often left unchecked, says Thompson, leading to people being stereotyped.
“We’re now looking into anti-black racism in the health-care system where we’re stigmatized as drug seekers, hospital hopping, angry people,” says Thompson. “Yeah, most of the time we’re angry because we’re not being taken seriously.
“We’re just asking to be treated fairly … like a human being.”
Although the new curative treatment option has been exciting news, many, like Thompson, are careful to not get their hopes up. “When we’re comparing to other diseases that are predominantly in the white population, they’re getting better treatment, the research is there, funding is there, resources are there. Everything is there for them,” says Thompson.
“They might not find the universal type of stem cell therapy for everybody so I’m already coming to grips with the fact that that’s not going to happen for me. But hopefully, it leads to other treatments.”