The Decibel - To save this baby, doctors had to kill part of her brain

Episode Date: June 6, 2025

Maryam Fatima was born with a condition called hemimegalencephaly, a rare birth defect where one side of the brain is abnormally large. She suffered from severe seizures from the moment she was born. They became so bad that they stopped Maryam from feeding, sleeping and breathing. Her life was at risk.That’s when a team at the Hospital for Sick Children in Toronto decided to attempt a first-in-Canada procedure to save Maryam’s life.Health Science reporter Jennifer Yang interviewed some of the medical professionals who performed the procedure, as well as Maryam’s mother, Muzna Nafees. She tells the story of how Maryam’s life was saved.Questions? Comments? Ideas? Email us at [e-mail]

So I'm here with Health Sciences reporter for the Globe, Jennifer Yang. Hey Jenny, welcome to the show. Thanks for having me. So we've got a video here today. Can you just describe what we're watching? Sure. We're looking at a really cute little baby. Her name is Miriam Fatima and in this video she's just about a month old.

She's wearing a green and white polka dot onesie, she's got a little baby toque on and she's lying on her back on a bed. Yeah she's really really little in this video and can you just tell us like what are we hearing here? The sounds you hear are just kind of the everyday sounds of Miriam's household. You can hear her mom talking in the background and some kitchen noises. She's at home here in her basement apartment in downtown Toronto. And there's something very specific though about the movement that Miriam is actually making in this video. Can you tell us about that? Yes. I mean what's striking about this video is that Miriam is twitching. Her legs are curled up at the knees and they're twitching rhythmically.

Her hands are balled up in fists and they're twitching rhythmically. Her hands are balled up in fists and they're twitching along with her legs. She has a vacant look in her eye and her mouth is kind of gaping open and she's staring at the ceiling. And it's because she's having a seizure. Yeah, it's kind of unnerving to watch because you can tell that there's something going on here, right? Is this common for Miriam? Like, does she get a lot of seizures like this? This used to be her life. When she was born, she was born with a condition where she was suffering

up to hundreds of seizures every day. Sometimes they would be happening every minute. This used to be her life, you said. So what changed? What changed was that Miriam underwent an incredible novel procedure that involved catheters and wires and tiny plastic microbeads and it changed everything. Wow. Okay, so today Jenny, you're going to take us through Miriam's story. Yeah. Well, I'm Annika Ramon-Wilms. I'm Jennifer Yang. And this is The Decibel from The Globe and Mail.

The first time I met Miriam and her parents, Musna Nafiz and Shabon Beg, was in the middle of March. They live in the basement apartment of a house that they share with three other women who are also newcomers from India. And we met for the first time to speak for the story. Okay, so my name is Musna Nafiz and I'm Mariam's mother and this is Mariam Fatima. She is my little brother. Yeah. And he is Shaban Beg. Okay.

And he is Shaban Beg, Mariam's father. Muzna and Shaban are from Lucknow in India. And they actually met at work. Funny enough, it was her boss who introduced her to her future husband. He was her boss's little brother. And she tells me that for whatever reason, her boss was just really insistent that they get together. And when they met, she was really struck

by Shabon's kindness and patience. And obviously they hit it off. Soon after they got married, and then they immigrated to Canada where they started a new life together here. Actually, we wanted children like when we had, you know, spent our time together. And then we decided, yes, we should have one now. We have got bored of seeing each other's faces.

How long? We should have a third one also. When Musna and Shaban got pregnant, they eventually found out that they were having a girl. I asked if they were happy about this, if they had any kind of feelings about this. At first Shaban was kind of like, oh, I didn't care. But then his wife cut in and she was like, oh, he really wanted a girl. He loves little girls. And I was like, oh, he really wanted a girl. He loves little girls. And I was like, oh, really?

Why is that? Turns out he's one of six brothers. So I think he just kind of really wanted a bit more girl energy in his life. I asked Musna how her pregnancy went and she said that it was just completely uneventful. There was some run of the mill morning sickness, maybe early on fatigue. But throughout her pregnancy, everything was really normal. Nothing went wrong, no issues, no hiccups. And I was doing my laundry.

When my water broke, then I called him because he's just like two streets away. So I called him, I said, my water broke, let's go to the triage. Her water broke on December 2nd, 2023, and Mariam was born the very next day. I asked Musna how she felt when she finally met her little girl for the first time,

and she kinda just smiled and she said, oh, you know, I just wanted to put her back inside. And now she was outside of her and kind of exposed to all the things that life can throw at you. She was in my hand and then they took her for cleaning her and putting clothes on and at that time they noticed this unusual twitching on the right side. That was the first sign that something might be amiss. The first few days of Mariam's life were spent in the Neonatal Intensive Care Unit,

also known as the NICU, at Mount Sinai Hospital where she was born. And then a few days after that, she was transferred to the Hospital for Sick Children in Toronto. We were worried that what had happened, like what's next, what are they doing? Doctors used to come and they used to come and update me that we have done this test, we have sent this sample for culture and we are working on it and this is fine, that is fine. She is going for MRI now. All other cultures and tests were normal, everything was normal. It was the MRI which revealed the actual causes of her seizures.

Marion was born with a rare neurological condition called hemimygalencephaly. When you look at an MRI scan of a normal brain, you can see very clearly the separations between the gray matter and the white matter. The folds of the brain look very distinct. There's clear borders and edges. When you look at the left side of Miriam's brain, it looks almost hazy. The way I thought of it when I looked at an image of her brain was it looked as though someone had painted it with watercolor.

And the paintbrush had too much water, so the paint had started to bleed across the canvas. You can't really see the distinction between the gray and white matter. The edges are very sort of blurred together and it's also too big. It's significantly bigger than the other side of her brain. So when the neurons and the cells were forming, they were disorganized. They didn't settle in the right places or too many of them formed. That's Dr. Prakash Muthusamy, one of the doctors who treated Mariam. The brain itself is that half of the brain is larger.

And what that does is it sets up that half of the brain to electrically discharge abnormally. And so these children are born with what we call untractable or uncontrollable seizures. I also spoke with Ivana Yao. She's a nurse practitioner with SickKids Epilepsy Unit. And she also played a critical role on Miriam's medical team. So it's something that happens congenitally, meaning as the baby is developing. And because it affects such a large majority,

and it's very, I mean, it's literally an overgrowth of one half of that brain. It presents so aggressively right at the get go. On December 8th, 2023, which was just days after Miriam was born, Musna and Chabon were called in for a family meeting with her doctors. Muzna and Shaban were called in for a family meeting with her doctors. And that's when they found out that she had this condition called hemimygelencephaly. But that wasn't the only bad news they got that day. They also found out from Miriam's doctors that she also had a genetic disorder called Tuberous Sclerosis Complex.

This is a genetic disease which actually causes cells to overgrow in different parts of the body including the heart and the kidneys and the brain also and often affects the brain and so by itself is a cause of seizure disease and so the association of these two although rare it's been reported but sets up that condition to be really with a lot of seizures and requiring a lot of medicines to control and sometimes not even so. The first few weeks of her life were scary, stressful. It was just a really deeply difficult time.

Here's how nurse practitioner Ivana Yao described Miriam's seizures. For her, she had a lot of seizures that were motor seizures, meaning it affects all the movements of one half of her body. And then eventually it actually spread to both sides of her body to the point where she had difficulty. Like, she had so many seizures, she couldn't keep any food down, she couldn't drink, she couldn't breathe even. I'm sure she was terrified and scared when that happened.

It's just a reflex of anyone to be like, I'm in danger. It's exhausting. I think that's another thing, especially for a little baby that's, you know, she was like two kilos at the time. It's imagining it's like your muscles are contracting constantly, like you're going to the gym, but it doesn't stop and you can't get off this roller coaster. And so it uses up so much energy like physically in her body but also her brain capacity like all of that.

Because the seizures hijack the brain, it takes away all normal function and uses all the energy towards seizures. So all the things that a normal baby's brain is supposed to be doing, it can't do because the energy is diverted to the seizures. The seizures dominated Miriam's whole existence. She was either seizing or sleeping or knocked out from the medications given to her to stop

the seizures. And at points that even became life threatening, it was hijacking her brain development. The challenge in this condition is to control seizures and allow the child to develop normally because the child's development is delayed and so they're not able to reach their milestones or they have seizure disease for life sometimes. They're never able to catch up with their peers or even worse, if seizures are so continuous like in our baby, they might not be able to breathe

on their own and then they need respiratory support. And then that's something that cannot sometimes be sustained for a very long period of time and then at that stage it even becomes life threatening. And so the earlier we can treat these children, the earlier the other half of the brain is able to take over a lot of the function and support the child. So there's this concept called brain plasticity and it means that your brain has this remarkable capacity for rewiring or relearning or adapting when other parts of the brain are injured or even missing. But the younger you are, the

more likely it is that other parts of the brain will take over for damaged or missing parts. And I was told that after about age five or six, when the brain is more fully formed, this kind of plasticity starts to drop off quite a lot. Ivana told me about a common saying in neurology which is that time is brain. Time equals brain. You might have heard this in the context of stroke. It kind of underscores the critical need for rapid treatment when someone has a stroke. But in the context of a baby like Miriam with intractable epilepsy, it speaks to the importance of getting their seizures under control as quickly as possible.

Because those seizures are hijacking her brain development, the more energy the brain is using to cause seizures, the less energy it's directing to those normal developmental processes that will help Miriam, you know, learn to look around, to turn her head, to just grow and develop as a normal baby should. Basically, every day, week or month that passes where a baby like Miriam's seizures go uncontrolled, it's changing the trajectory of her whole future. So that's why they say time is brain. Because there are bridges from one hamster to the other, seizures, electrical activity,

can go to the other side. And so eventually what can happen is we call kindling is that the other side can learn to have seizures on its own eventually. And if that happens, it's too late for us to intervene because then they're two independent sources of having seizures. So that's kind of again, this time is brain we have to chase. Babies who have seizures like this are typically treated with medications. But Miriam was taking six medications at one point,

in addition to what they call rescue medications, which are basically reserved for emergency situations. When you talk about drug resistant epilepsy, that refers to a patient who has seizures that can't be stopped with two good medications and Miriam was taking at least six. It was a kind of torture I think for Muzna to watch her daughter have these continuous seizures. She was like trapped in this awful cycle because there was just nothing she could do but sit there and watch helplessly. So all she really did was be there for her baby and pray. It was like I used to think what should I do?

What should which part of her body should I rub or ease her from where? But she doesn't get these seizures. What should I read? What should I do to cure her? Just three weeks after Mariam was born, her parents were called into a meeting with her medical team in the NICU. And they basically had to have the worst imaginable conversation that a parent could ever have to have. The doctors felt that there was nothing that could be done to help Mariam. She was too small to undergo brain surgery that might stop her seizures. The medications weren't working.

She was suffering. She was in pain. It didn't look as though she could survive. So they called us, the nurse in charge of the NICU. Then they talked to us, like they said that it's, we are not able to control her seizures through medicines. And now her whole body has started jerking. so it's the right hemisphere also that's going to be affected that has started getting affected due to the left null functionings and even if we are able to hold her on support she won't like she will need every time anyone to look after her and to do things for her

And she is in intense pain like how like she she is also in pain So it would be better if nature takes we allow the nature to take its course So like when they told us this we also thought like when the doctors have given up they told us that once we remove her from all supports she won't maybe she could survive only couple of hours couple of days sometimes couple of weeks we don't know they said like if we remove all her systems support systems like if we remove her NG tube, her feeding would get down. Her oxygen levels due to seizures come down drastically.

So her body would slowly, slowly, her oxygen levels in the body would go down. Her heart rate would get slow and she would eventually pass away. But something very different happened instead. She started feeding from her mouth, from the bottle. She survived without any artificial oxygen. She was there. Yeah. And her heart beats everything that the nurse used to come, the temperatures, the vitals.

They were all so normal. She was seizing, but she was surviving. Like, truly speaking, I was in a hope that she would get better. I don't know why. I always had this belief that my daughter would get better. We'll be right back. So after a few days at the hospital with Mariam removed from medical supports, they decided that she was okay to go home with her parents,

but as a palliative patient, which basically meant she'll just be supported and kept comfortable. Miriam was being managed at home by her parents for about a month when one day she woke up and she started having a seizure right away, which is maybe not that unusual, but what was different this time was the seizures just didn't stop. Like one of, I remember one of the day was 9th February and she was seizing from morning

and she was continuously seizing and seizing and seizing and seizing. She was not able to drink any of her milk. She was not able to feed the whole day. At last she started turning blue when I rushed to the hospital to get her again in emergency. And then she was readmitted. Typically babies like Miriam are treated with a type of brain surgery called a functional hemisperectomy. Functional hemisperectomy is open brain surgery though. You have to cut open the skull, you have to cut into the brain.

What the brain surgeons will do is basically disconnect the faulty hemisphere of the brain from the healthy side. And this will both stop or mitigate the seizures and also prevent it from spreading those seizures to the other side of the brain. There's a potential for blood loss. And that's why it can be so dangerous for very small babies. Even a small amount of blood loss can be really catastrophic or fatal for a baby because the volume of blood they have is already so small to begin with. You have to be kind of a certain weight to be able to safely undergo a brain surgery. And for Miriam, she was far from meeting that weight threshold.

This is where Dr. Prakash Muthusamy comes in. You heard from him earlier. He's a neuro-interventionist. And what that is, is I treat diseases of the blood vessels in the brain, but I do that without surgically opening the skull, so I do that from inside the blood vessels. When I was thinking about the differences between a functional hemisperectomy and the way a neuro interventionist might operate or access the brain, I kind of thought of this like underground subterranean pool. One way of getting there is to drill through

the rock, to go from the ground and drill down and get into the pool that way. But the other way of accessing that pool might be to find one of the underground, you know, tributary systems that feed water into the pool, and then to follow along that waterway until you reach the pool at the end. And that's kind of what a neuro interventionist does. They go into blood vessels and they follow those blood vessels using catheters until they can eventually reach the brain where they can do their interventions. You know, when it was clear that Marion was running out of time, Ivana was telling me that she just became really, really fixated on looking for other options like thinking outside of the box.

She said another neurologist had actually shared with her a paper that described this novel procedure called an endovascular embolic hemisphericity. And basically instead of going through open brain surgery, you used a neuro-interventional approach, achieving the same result. She brought it up with folks around the team. It was discussed. She talked about it with Dr. Rutka and they decided to approach Dr. Muthusamy

and ask, can we do this? Is that something that as a neuro-interventional team, can we offer that? Is my room, my team, is my team ready to do all of this? Is that something that as a neuro-interventional team can we offer that? Is my room, is my team ready to do all of this? So I got back to them the next day and I said, I think this is something we can offer. I think this is something we should do if everybody thinks this is the next best thing for a child that we've run out of options for but obviously we wanted to

meet the family first and explain this to them in terms of a procedure that we think we can technically and safely do and achieve the same result as what we wanted in the surgery but at the same time something that we've never done in Canada before. As Dr. Muthusamy said, this had never been done before at SickKids. It had never even been done before in Canada. It had only been attempted a few times in the world. To date, there's one center in Washington, D.C., where only 13 patients have undergone this procedure.

The way this procedure works is that first, the doctors have to enter the patient's blood vessels through the femoral artery, which is in the upper thigh. And what doctors will do is feed a catheter into the artery and start threading it up the patient's body through their torso, up into their neck, and into their brain. Once there, they feed even smaller micro catheters inside the first catheter because once

you get into the brain, especially in a baby, those blood vessels can be so tiny like little threads. So these tiny tiny micro catheters are threaded through the first catheter and then they start navigating the twists and turns of the blood vessels in the brain. And they continue doing this very carefully until they reach their intended target destination in the brain. Once those catheters reach their target, Dr. Muthusami will load up a syringe with these plastic microbeads and then he will inject those microbeads into the catheter which now serve as a pipeline into Miriam's brain and those beads will eventually block the blood flow in her brain and cause a series of mini strokes that will effectively kill off those parts of the brain, which are the parts that are causing her seizures.

And we're talking about something drastic. We're causing a stroke. That's Miriam's nurse practitioner, Ivana Yao, again. It sounds crazy, is that you're giving her a neurological insult to cure another problem. As you can imagine, this type of procedure can come with some pretty serious risks and complications. A whole host of things can go wrong. You could accidentally cause strokes in areas where you didn't mean to cause them. And after the procedure there are new things to worry about. The patient could have a bleed, there could be brain swelling, which would require emergency

surgery to cut open their skull. Death is always on the table with something like this. Musna told me that her and Shabon took just over a day to reach their decision. It was like for me, I don't have any option. I cannot see her like this. So I don't have any option to say no to it. I wasn't having a very good feeling or very comfortable. I wasn't very comfortable and confident about that functional hemispheric to me, that open surgery. And I don't know that God listened to my prayers and he got something apart from that. The first one was scheduled for February 20th, about a couple of weeks after she was readmitted to SickKids, and there would be four procedures in total,

with about a week or two in between each procedure. Miriam went in for her first procedure at 8 a.m and she was in that room for about I think four to five hours altogether. For the procedure Miriam was under deep anesthesia. This meant that she was unconscious and paralyzed. She couldn't move a muscle. She couldn't even twitch her eyelid. But her seizure activity was still happening in the brain and they knew this because of the EEG leads that they had attached to her scalp, which was monitoring the electrical activity in her brain all throughout the procedure. Throughout the procedure, Dr. Muthusami has his eyes fixed on these display screens over Miriam and what they're showing are her blood vessels and what's happening inside of them. He could track

where the beads were going because he had mixed them with a contrast dye that were showing up as black. So that's how he would know where they were going and he could see that they were starting to reach the target areas in her brain and as they got there, he could also start to see that the arteries were now starting to vanish from the screen, which meant that the blood flow had been successfully blocked. The beads were doing their job. And as the blood flow stopped, those parts of the brain started to die off. And as that happened, the doctors who are

monitoring Miriam's seizure activity started to report back that her seizure activity was also falling off. It was a direct vindication of what we are doing in that we are creating a stroke in this brain that's abnormally producing electrical activity but in front of us that abnormal electrical activity was going away. It was working. The seizures were falling away. At the end of the procedure, the people in the room would have started to hear the sound of lapping waves because Dr. Muthusamy always likes to play music in his procedure room and he always likes to wrap up with one song,

Champagne Supernova by the British band Oasis. After the procedure, Dr. Muthusamy spoke with Muzna and Shaban. on. He told me that everything went very well, smooth, and she was very strong like a rock. She stayed all strong like a rock over there. Everything was fine in the aftermath of her first procedure and the same for the second, third and fourth. It all went off without a hitch. I can't explain like what I felt and how I felt. It was just amazing.

Like my daughter got, you know, she cured. It was like God is just great and miracles happen. Mariam was discharged from SickKids for the second time on April 17, 2024. And even though Miriam had already come home once before four months earlier, her mom told me that she really considered this to be Miriam's true homecoming. Miriam was now down to just like a handful of seizures a day, maybe one or two. And the plan was to bring her back in about six months time to have a functional hemisperectomy,

the more traditional open brain surgery to sort of finish off the job. But her family decided to go to India for the summer for Miriam to finally meet her family, her grandmother, who she loves so much. And something really remarkable and surprising happened while they were in India. One day, Maryam was just playing at her grandmother's house and she sort of started to twitch. Very familiar sight for her mother, Muzna. She

was having a seizure. I remember that seizure. It was last days of July. She was playing and all of a sudden it came and after that I haven't seen any of it. I stay with it every hour and I haven't seen any. It's now been more than a year since Miriam had her fourth and final procedure but of course Miriam has challenges. You know, the healthy surviving side of her brain is now starting to take over some of the function of the left hemisphere that was effectively killed off. But she still has a really weak side, her right side, that she's now learning to use through the help of physiotherapy and occupational therapy.

She hasn't hit a lot of the developmental milestones that she should be hitting by this age. For example, she's not crawling or walking yet. But every day, Miriam's parents are helping her at home to overcome these challenges. Miriam, are you on to the set? The wheels on the bus go round and round, round and round. When I spent some time with them at their home, her mom was, you know, singing wheels on the bus with her

and, you know, bringing up these plastic vegetable toys and encouraging Maryam to try and cut them with a little plastic knife and to use her right side, which is weaker. One, two, three! Now you do, you do. Hold it, hold it. Yes, yes. Now we will do it together. Miriam is just a happy, delighted little child. She has a really great temperament. She's so sweet. She has this little kind of crooked smile. She's just a little kid developing this whole personality. We have no idea what will happen with her seizures in the future. Right now, she hasn't had a seizure since July 2024, but there's always a chance they could come back.

There's always a chance something could come up with her other diagnosis, the tuberous sclerosis complex. She's going to be closely monitored by a neurologist for many, many years. But incredibly, right now, her doctors think she may never need to have a functional hemispheric me to undergo brain surgery. She's still currently on some medications, but they're always talking about whether she can be safely weaned off of one more medication.

Today, Musna is just full of hope and optimism for her daughter. When she looks at her all she sees as possibility, she thinks that there's nothing Miriam can't do that she wants to do. Seeing her today, I wish that she will lead a normal life. She will study, she will play, she would, like, whatever she wants to do, I think she will do. She won't need anyone anymore for her support.

She will take care of herself. All by herself. She will do all this stuff. It was she who fought for herself. Dada! Dada! That was Jennifer Yang, the Globe's Health Sciences Reporter. That's it for today. I'm Maynica Ramon-Wilms. Madeleine White produced this episode. She produces The Decibel along with Michal Stein and Allie Graham.

David Crosby edits the show. Adrian Chung is our senior producer and Matt Frainer is our managing editor. Thanks so much for listening and I'll talk to you soon.