Summary
A 5-year-old child and his parents boarded a plane heading for a new life. They were Syrian refugees, who fled conflict in their country and then lived a difficult life in a refugee camp. Now they were heading to Canada. But soon after arriving, there’s a problem. The child has hemophilia and due to hemophilia complications, a minor injury while traveling became a major concern. Add to this a language barrier and trying to understand a new culture. Dr. Robert Klaassen, a pediatric hematologist and lead of the Comprehensive Hemophilia Care Clinic at Children’s Hospital of Eastern Ontario in Ottawa, Canada, shares his experience working with a family who overcame incredible barriers and the lessons learned along the way.
Dr. Robert Klaassen
Dr. Klaassen is a Pediatric Hematologist/Oncologist at the Children’s Hospital of Eastern Ontario, Professor at the Department of Pediatrics, University of Ottawa and Clinical Investigator at the CHEO Research Institute. He is the hemophilia director for the Eastern Ontario/Western Quebec region and is the past president of the Association of Hemophilia Directors of Canada. His clinical focus is on the care of patients with non-malignant hematologic disorders and his research activities specialize in quality of life.
Transcript
DDx SEASON 9, EPISODE 6
How One Child Overcame Hemophilia Complications Despite Incredible Barriers
Dr. Raj Bhardwaj: This season of DDx is sponsored by Sanofi. The case study you’re about to hear is a real patient story.
A young Syrian boy displaced by conflict finds asylum in another country halfway around the world.
But soon after he arrives with his family, there’s a problem.
Dr. Robert Klaassen: My fellow contacted me because they were in a bit of a panic because this patient arrived in the emergency department and nobody really knew what to do.
Dr. Bhardwaj: This is DDx, a podcast from Figure 1 about how doctors think.
I’m Dr. Raj Bhardwaj.
This season, we’re taking you inside the minds of doctors who specialize in the most common type of hemophilia, hemophilia A — an inherited bleeding disorder that prevents the blood from clotting properly.
Today’s case is from Dr. Robert Klaassen, a pediatric hematologist. He leads the Comprehensive Hemophilia Care Clinic at Children’s Hospital of Eastern Ontario in Ottawa, Canada.
Dr. Klaassen has been compensated by Sanofi for his participation in this episode.
CHAPTER 1 — CASE STUDY
Raj Bhardwaj: In the summer of 2022, a 5-year-old boy and his parents boarded a plane heading for a new life. They were Syrian refugees who fled conflict in their country, and then lived a difficult life in a refugee camp. Now they were heading to Canada.
But almost as soon as they arrived, the little boy’s health began to deteriorate.
Dr. Klaassen: There was no obvious signs of issues on the flight. But the next day, was noted to have significant pain and swelling of his elbow. And it was quite painful. He wasn’t able to move his elbow and he became very quiet.
Dr. Bhardwaj: The boy was in a lot of pain. One of his arms was bruised and swollen. He had just arrived in a new country and he needed medical care right away.
Dr. Klaassen: When he arrived, he couldn’t do anything with that arm. He could not extend it, he couldn’t move it, he couldn’t put it over his head. So he could not, basically that arm was useless as far as being functional.
Dr. Bhardwaj: Accessing healthcare in a different country is challenging and even harder when language is a barrier.
Dr. Klaassen: They were using a cousin as a translator, so that’s one of the biggest problems, issues that comes up is communication and trying to get an understanding of what’s happening.
Dr. Bhardwaj: Eventually, through the cousin, the parents were able to explain to the emergency doctors that their son had been diagnosed with hemophilia when he was a toddler.
This could explain the pain and bruising in the boy’s arm. Something as benign as a bump or strain on an elbow can cause the joint to bleed.
That joint can fill up with blood. That creates pressure. That pressure is what causes pain. The picture of the boy’s condition was starting to become clear. But there were still a lot of unknowns.
Dr. Klaassen: I immediately start getting texts and people are trying to reach out, trying to get my advice because it is fairly, an unusual case and with not a lot of experiences.
Most of the patients I follow have a very clear treatment plan, so it’s everything spelled out ahead of time. When you have a new patient who shows up, it’s much more challenging because there’s really not necessarily a clear path.
Dr. Bhardwaj: Blood tests were ordered. Some results take days. Others, like the partial thromboplastin time, or PTT — which is a measure of how long it takes blood to clot — are back within an hour. Dr. Klaassen’s patient’s PTT was prolonged. That suggested that this young boy was missing a crucial blood clotting factor.
Dr. Klaassen: We really should treat it as quickly as possible and ideally within an hour. If it’s left for longer and especially days, and I’ve certainly had patients show up with bleeds that happened two or three days prior, it’s much more difficult to treat.
Dr. Bhardwaj: If left longer, it can take weeks and sometimes months to get the inflammation and pain under control.
Dr. Klaassen: Bottom line, if you get repeated bleeds into that joint, you’re going to end up with long-term joint damage, and you can end up requiring a joint replacement long term. You know, and these are young kids. We’re talking about a 5-year-old. We don’t want him to have to need a joint replacement.
Dr. Bhardwaj: When doctors tested for levels of the clotting factor affected by hemophilia A — factor VIII (8) — this patient’s levels were so low that they were essentially undetectable.
Dr. Klaassen knew the boy needed to be given factor VIII or the bleeding would continue, and his elbow would suffer more damage and cause more pain.
Dr. Klaassen: Then I gave the factor the following day and then two days after that I gave a third dose. And then after that, to keep things under control, I gave him an infusion two times a week.
Dr. Bhardwaj: Before coming to Canada, the family had very limited access to medical treatment.
Dr. Klaassen: So here you have a refugee family who’s dealing with all the amazing, difficult things they have to deal with.
And on top of that, they’re having to worry about their child potentially having recurrent bleeds in joints, they have to go get the treatment needed, go to see a healthcare professional to give them the appropriate treatment.
To me the whole situation is heartbreaking, knowing that your child has a severe life threatening disorder, which in North America or developed countries is very treatable and you can have a normal lifespan and a normal life, yet when you’re in a situation with limited resources like a refugee camp, you’re not being able to treat your child adequately.
Dr. Bhardwaj: Think about that for a second. This is a child who spent the bulk of his young life in a refugee camp, living with a rare and painful disorder with only sporadic access to treatment. The possibility of a fatal bleed looming over every moment.
Dr. Klaassen: If you get a head injury or, um, significant abdominal injury, you can bleed to death without access to those kinds of treatments. So you have a child who’s at risk of having long-term joint problems and also this, hanging over their head, this risk of their child potentially bleeding to death.
I mean, this is the reality of many patients with hemophilia in the world, because it’s such an expensive disease to treat, there’s so many hemophiliacs out there who are not getting the appropriate treatments.
Dr. Bhardwaj: According to the National Center for Biotechnology Information, about 75% of people with hemophilia live in the developing world and don’t have access to routine care.
Dr. Klaassen: And it basically can have a significant impact on their quality of life because they stop doing the important thing that 5-year-olds do, running around and playing games and, you know, throwing balls, kicking balls, and they may stop doing that.
They can change to be someone who’s more sedentary, you know, watching TV, reading books, that kind of thing. That sets them up for a lifetime of inadequate physical activity and potential for all the complications of being sedentary.
Dr. Bhardwaj: Fortunately Dr. Klaassen’s patient is doing well now. He has had no more significant episodes of bleeding.
Dr. Klaassen: When he came here he had significant limitations in his elbow and his shoulder. So, we very quickly got our physiotherapist involved and she’s so crucial to this. She assessed him, and at the time he did not have normal range of motion when she first assessed him and because we aggressively treated his bleed, he was gradually able to improve so that now he’s at full range of motion in his elbows.
Dr. Bhardwaj: Full range of motion for this 5-year-old means he was better able to do activities most of us take for granted — like dress himself. Feed himself. Tie his shoes. And he can have fun with other kids.
Dr. Klaassen: And so he is able to play basketball, he’s able to play volleyball. He’s playing soccer, so it’s really nice to see that he’s fully recovered from the problems he had and is now leading a normal life.
Dr. Bhardwaj: To make treatments easier for the patient and his family, Dr. Klaassen switched him from IV infusions twice a week in a clinic to an under the skin injection.
Dr. Klaassen: The big advantage is it’s much easier for families to learn how to do. We gave it initially once a week. Just trying to stack things up to get things to stable levels.
And then we’re giving him a subcutaneous injection every two weeks. With that, he’s done remarkably well. He’s done really no breakthrough bleeds. He’s fully active, participating in all kinds of sports. There doesn’t seem to be any residual joint damage.
CHAPTER TWO — LESSONS
Dr. Bhardwaj: We live in a world that’s more closely tied than ever before. Dr. Klaassen has thought about what he learned from this young Syrian boy who suddenly needed healthcare in a foreign country.
Dr. Klaassen: There’s the language issue. It’s always a challenge dealing with language and working through translators. We’re very fortunate at our institution, we have excellent translators that help us. So I think that’s one key thing, is access to reliable, good translation services. And then, the second thing is culturally understanding where they’re coming from.
So, for instance, with this family, they were used to treating hemophilia as a catch up, where they have a bleed, and then they treat the bleed, and then they stop. And then, we’re constantly playing catch up, dealing with the last bleed that happened.
Dr. Bhardwaj: In much of the world, healthcare is necessarily focused on solving the problem at hand. Preventing and mitigating problems is a luxury many simply do not have.
Dr. Klaassen: So getting them to understand our mentality of using preventative treatment — prophylaxis — was a challenge. So you need to have them understand. So that is definitely something I learned from this family, is trying to get them to understand the importance of preventative treatment. Not waiting for the bleeds to happen, but to try to prevent the bleeds from happening.
Dr. Bhardwaj: Thanks for speaking with us Dr. Klaassen.
This is DDx, a podcast by Figure 1.
Figure 1 is an app that lets doctors share clinical images and knowledge about difficult to diagnose cases.
I’m Dr. Raj Bhardwaj, host and story editor of DDx.
Head over to figure1.com/ddx, where you can find full show notes, photos and speaker bios.
This season of DDx is sponsored by Sanofi.
Sanofi is a global healthcare company that develops and delivers medicines and vaccines for millions of people around the world. Thanks for listening!