Latest news with #pediatriccare
CBS News
3 days ago
- General
- CBS News
Florida boy thrives after groundbreaking brain procedure at Nicklaus Children's Hospital
A young Florida boy who once struggled to move or even hug his mother due to a debilitating neurological disorder is now swimming, biking, and running, thanks to a treatment offered at one of the few pediatric hospitals in the nation providing the procedure. Nicklaus Children's Hospital in Miami is among the only pediatric facilities in the country performing deep brain stimulation (DBS) for children. The treatment has dramatically improved the life of 10-year-old Tamryn Hendon, whose family travels nearly 500 miles from Tallahassee to receive care. A dramatic turnaround "It was miraculous," said Tamryn's mother, Tamara Hendon. "Now he swims, he rides his bike, he runs. He could do whatever he did before the onset." Just over a year ago, Tamryn's life looked very different. He suffered from dystonia, a movement disorder that caused painful, involuntary muscle contractions. Tamryn said he could barely move and was unable to hug his mom. "He started falling backwards. He couldn't hold himself upright and he would just, his back would bend and he would fall over and it would be worse later in the day," Tamara recalled. "It was extremely scary because we didn't know, is this a life-threatening condition? Is it life-limiting?" From bedridden to biking "He quit laughing, he quit smiling and he talked about death," she continued. "He was just in a really dark place for a 10-year-old boy." After consulting doctors across the state, the Hendons met Dr. Migvis Monduy at Nicklaus Children's Hospital. "He was in a wheelchair and then ultimately continued to progress to the point that he was arching back constantly and twisting, and even sitting in a wheelchair became very, very uncomfortable for him, so he was mostly bedridden at that point," Dr. Monduy said. Testing revealed that Tamryn's dystonia was caused by a genetic mutation known to respond to deep brain stimulation. Dr. Monduy explained the procedure: "This (device) gets implanted typically under the skin in the chest area or perhaps in the abdomen. And then there are cables that are going to be connected to this. Those are the leads and those are going to be tunneled under the skin, through the neck area and then through the skull into the brain," she said. "They're going to go deep in the center of the brain, so sort of like one on each side for each half of the brain." The device delivers electrical impulses to regulate abnormal brain signals, often used to treat dystonia, Parkinson's disease, and epilepsy. A new normal "It was a really hard time, but now his cheerful self is back," Tamara said. It can take six months to a year to calibrate the device properly, and settings can be adjusted through an app. Tamryn and his family continue to make the long drive for checkups, but he now enjoys a typical childhood—riding his bike, drawing cartoons, and playing video games.
Medscape
7 days ago
- General
- Medscape
Pediatricians: Stop Atopic Dermatitis With ‘Proactive' Rx
Pediatricians should consider treating children with atopic dermatitis (AD) 'proactively,' according to a new clinical report from the American Academy of Pediatrics (AAP). The AAP defines proactive care as an 'evidence-based' approach based on 'intermittent use of anti-inflammatory agents (eg, topical corticosteroids or tacrolimus twice weekly) on previously affected skin to prevent flares' of AD. The group also recommends newer agents like the Janus kinase (JAK) inhibitors, which may have fewer side effects than long-term steroid use and are more effective than older drugs. The report comes as patients experience long waits for referrals to pediatric dermatologists, said Jennifer Schoch, MD, a professor of dermatology at the University of Florida in Gainesville, Florida, who helped write the document. The recommendations may help primary care clinicians more confidently identify and treat mild to moderate cases of AD, she said. 'Pediatricians play a critical role in initiating topical treatments, educating families, and supporting quality of life,' Schoch said. 'With a proactive approach to topical therapies and an understanding that atopic dermatitis is a chronic condition requiring an ongoing management plan, most cases can be effectively handled in primary care.' JAK inhibitors may be particularly beneficial for treating sensitive areas of the body, supporting patients who have not responded to traditional therapies, and aiding in long-term treatment. Topical JAK 'treatments offer targeted anti-inflammatory effects, which adds to our toolbox, particularly to mitigate concerns about risks of long-term steroid use,' Schoch said. The AAP also now recommends applying topical corticosteroids or topical calcineurin inhibitors proactively to prevent flares. In recent research, patients who were preventively treated with topical steroids experienced a reduction in disease severity and an improved quality of life. The proactive use of nonsteroid topical agents produced similar results. Soft Skills to Build Moisturization and avoidance of triggers like harsh detergents and low humidity continue to be mainstays of maintenance care. Patients should be moisturized using a fragrance-free, thick cream after bathing and handwashing. The AAP advises daily, short baths in lukewarm water and the use of wet wraps and bleach baths. The report emphasized the psychosocial burden of AD in children. AD is associated with mental health issues like stress, depression, and insomnia in children. The recurring, ongoing nature of the disease can place a heavy emotional and economic burden on the entire family, Schoch said. 'Atopic dermatitis can significantly disrupt sleep due to relentless itching, leading to daytime fatigue, irritability, and behavioral challenges in children,' Schoch said. 'The disease may affect school performance, peer relationships, and self-esteem, while caregivers report high levels of stress, anxiety, and even depression.' The new report recommends cognitive-behavioral therapy for patients with mental health concerns and progressive muscle relaxation, sleep hygiene management, and melatonin to reduce insomnia. The use of written action plans may help families follow through with treatment strategies and are 'excellent tool for primary care providers to help explain to patients the stepwise approach of the disease,' said Daniela Russi, MD, a pediatric dermatologist at Phoenix Children's in Phoenix, who was not involved with the report. These plans help 'pediatricians evaluate all aspects of the treatment…in addition to providing proactive ways to prevent flare-ups.' 'This guideline is helpful in showing pediatricians that they are capable of treating mild to moderate atopic dermatitis patients,' Russi said. 'Most of the patients we see in the clinic come without any knowledge of basic skin care, frequency of baths, and use of the correct moisturizers, especially bleach baths and wet wraps.' Schoch reported financial relationships with Janssen Biotech and Exactech. Russi reported having no disclosures. No external funding for the clinical report was used.
Globe and Mail
7 days ago
- Health
- Globe and Mail
The drastic brain procedure that gave a baby a fighting chance
On a February morning last year, Prakash Muthusami peered at a panel of display screens as he carefully snaked a tiny catheter into the delicate blood vessels of a baby's brain. About two hours had passed since Maryam Fatima's mother kissed her 11-week-old daughter goodbye, tucking a Quran into her pink hospital blankets. Those blankets were now replaced with a blue surgical drape and Maryam was intubated and unconscious, paralyzed under deep anesthesia. Not even an eyelid flickered as electrical storms in her brain caused seizure after seizure. Those seizures were what brought her into Dr. Muthusami's procedure room at Toronto's Hospital for Sick Children in the first place. Maryam had her first seizure within minutes of being born and they never stopped coming – sometimes every minute. On her worst days, Maryam suffered hundreds of seizures, so many she couldn't eat or gasp in a breath. Four weeks after her birth, it looked as though Maryam was unlikely to survive. But on this winter morning, Dr. Muthusami and his team were determined to give her a chance. In many ways, it was a typical workday for Dr. Muthusami, a neurointerventionist who has performed intricate brain procedures on some 2,000 patients in the last 13 years. Some were even smaller than Maryam, whose body was so tiny it could fit onto just the headrest of the procedure table. At under six pounds, she was no bigger than a pet rabbit. But Dr. Muthusami was about to try something that no Canadian doctor had ever done before; in fact, it had only been attempted a few times worldwide. With his catheters finally in position, Dr. Muthusami was ready; he filled a syringe and slowly pushed the plunger, injecting its contents into Maryam's brain. When Maryam was born, her mother Muzna Nafees had a kind of maternal urge to put her baby back inside her body. There, she felt her daughter had been safe; now she was exposed to the dangers and uncertainties of life. Maryam was a firstborn for Ms. Nafees and her husband, Shahban Beg. Ms. Nafees was working for an e-rickshaw manufacturing factory in Lucknow, India when her boss introduced her to his younger brother, a gentle and patient man of few words. 'He was very adamant that we should get together,' she laughs. The couple married in January, 2019, and later moved to Toronto, where Ms. Nafees quickly landed an accounting job and Mr. Beg found work at a jewelry auction company. In 2023, they decided to try for a baby; Mr. Beg, one of six brothers, was delighted to have a girl. Ms. Nafees was doing laundry when her water broke. Maryam was born the next day on Dec. 3, 2023, delivered by Caesarean section at Mount Sinai Hospital, just a ten-minute walk from her family's basement apartment in downtown Toronto, which they share with three other newcomers from India. After meeting mom and dad, Maryam was whisked away to be checked and cleaned while Ms. Nafees rested in the recovery room. For the first hour of their new life as parents, everything was normal. But then, one of the nurses came in with unsettling news. 'She told me that the doctors are seeing her, she is okay,' Ms. Nafees recalls. 'But we saw some kind of twitching in her right arm and right leg.' It was a seizure. Maryam was transferred to the neonatal intensive care unit (NICU), where she continued having seizure after seizure. After a battery of tests, a brain MRI revealed the problem. If the MRI scan were a piece of art rather than a medical image, the two halves of Maryam's brain could have been rendered by entirely different artists. Her right brain hemisphere was like a pencil sketch, a work of realism; the brain folds looked clear and sharply defined, with the grey and white matter separated by distinct borders. Her left brain hemisphere, however, was an abstract watercolour. The brain ridges looked puffy and haphazard, with the grey and white matter blurring together, as though too much water in the brush had caused the paint to bleed. The left hemisphere of Maryam's brain was also noticeably bigger. She was transferred to SickKids, where doctors diagnosed Maryam with hemimegalencephaly (HME), a rare birth defect where one side of the brain is abnormally large. The brain hemisphere's function is so disturbed, and the neurons so disordered, that it is effectively capable of only one thing: causing seizures. Further scans and genetic testing revealed that Maryam also had tuberous sclerosis complex (TSC), a disorder that can cause benign tumours – or 'tubers' – to grow in various organs, including the heart and brain. The next two weeks were a fog of worry and exhaustion. Ms. Nafees was herself hospitalized during this time for symptoms of pre-eclampsia and she and her husband would shuffle between her hospital and Maryam's, watching in helpless despair as their baby was trapped inside a never-ending circuit of electrical misfire. 'The seizures came in a row, in clusters, every minute, every two minutes,' Ms. Nafees says. 'It's a very helpless feeling. She is in pain and you can do nothing. Just watch.' On Christmas Eve, 2023, doctors called a family meeting. Maryam's seizures could not be controlled with medication, they explained, and surgical options weren't possible for a baby of her age and size. Her chances at survival were bleak. 'They thought that it was stressing her body, her health, too much. She was suffering a lot,' Ms. Nafees recalls, tears dripping down her cheek. 'It would be better if we allow nature to take its course.' Three weeks after she was born, Maryam was moved into a private hospital room as a palliative patient. Her doctors couldn't say how long she might survive, or even whether her remaining time could be measured in hours, days or weeks. They laid out one possible scenario: When Maryam's nasogastric feeding tube was removed, she would struggle to feed. Her oxygen levels would drop, her heart rate would slow. She would get sleepy and sluggish and eventually die. Maryam was disconnected from her medical supports. All they could do now was wait. If Maryam had been bigger, older and less fragile, there was, in fact, an option for bringing her seizures under control. In 1928, American physician Walter Dandy, one of the pioneers of neurology, published a report detailing his radical idea for treating incurable brain tumours: removing the diseased half of the brain. The surgery, now known as a hemispherectomy, was ultimately abandoned as a viable treatment for brain tumours. But a decade later, it was attempted on a 16-year-old girl at Toronto General Hospital. The girl had suffered a brain injury as a newborn, which left her with epilepsy as well as paralysis on the left side of her body. Kenneth George McKenzie, Canada's first neurosurgeon, decided to remove her right brain hemisphere; while her paralysis didn't improve, her 'fits ceased completely,' according to a 1939 article. Doctors who examined her afterwards were impressed by her 'striking' physical well-being and mental alertness. And while she lost some forms of sensation on one side, she had good movement in her major joints. Throughout the 1950s, 'anatomic hemispherectomies' were increasingly used to treat drug-resistant epilepsy, but over time it became clear that a significant proportion of patients were suffering delayed surgical complications, sometimes resulting in death. The procedure has since been refined to be safer and less invasive. Today, minimal brain tissue is removed and rather than removing the dysfunctional hemisphere, it's simply disconnected from the healthy side of the brain – a 'functional' hemispherectomy. 'It still survives,' says SickKids neurosurgeon James Rutka. 'But it's offline.' Since 1987, Dr. Rutka and his colleagues at SickKids have done hemispherectomies on at least 28 children with HME. But what kind of future can a child have when half of their brain goes dark? To answer the question, Dr. Rutka pulls up photos of a former patient, who had a functional hemispherectomy when he was six. In one picture, the boy is downhill skiing; in another, he's giving a thumbs up while scuba diving. Later photographs show a tanned and muscular young man, gripping a tow rope while wakeboarding. The boy is now a university graduate. 'You can have an amazing outcome,' Dr. Rutka says. 'It's called the plasticity of the human brain. The younger you are, the more likely the other parts of the brain are able to take over the damaged areas – or parts of the brain that might be missing.' Dr. Rutka has now done hemispherectomies on patients as young as two months old but the risks are often unacceptably high for babies under one. The biggest danger is blood loss, he explains. A baby's reserve is so small, that even a small bleed can prove fatal. Ideally, a baby should weigh at least seven to 10 kilograms before undergoing a hemispherectomy, Dr. Rutka says. In the NICU, Maryam was barely a month old and weighed less than three kilograms. Before Maryam was transitioned into palliative care, her parents asked about functional hemispherectomy, which Mr. Beg had come across in his own research. But they were told that Maryam was too small to undergo the surgery, and doctors weren't sure she'd survive to the point where she would qualify, according to Ms. Nafees. When Maryam was disconnected from her medical supports, her seizures continued, as everyone expected. But then something remarkable happened. She started drinking from a bottle. Her temperature stayed normal, as did her vital signs. She was breathing, moving, looking alert. Maryam was, in a word, alive. Ms. Nafees could tell that some hospital staff were surprised, but she was not. In the weeks since giving birth to Maryam, she had come to understand something fundamental about her daughter: She was strong. 'Maryam proved to everyone, against all odds, that she was going to fight for her life,' says Ivanna Yau, a nurse practitioner with SickKids' epilepsy team. 'This was not the end for her.' Clearly, not intervening was no longer an option, Ms. Yau says; a new plan was formed. Maryam's parents would manage her seizures at home until she was big and strong enough for brain surgery. Ms. Yau – who Ms. Nafees calls a 'sister, life changer, saviour' – joined Maryam's medical team and stayed in constant communication with her parents, helping them manage her seizures and medications. But about a month after going home, Maryam woke up one day and started immediately seizing. The seizures continued nearly every minute for eight hours, at which point she began turning blue. Ms. Nafees rushed Maryam to the emergency room at SickKids. There, in the epilepsy monitoring unit, she was having near-continuous seizures, between 30 and 40 an hour. A patient with drug-resistant epilepsy is defined as someone who fails on two good medications, Ms. Yau says. Maryam was on six medications, in addition to regular boluses of 'rescue medications' reserved for emergency situations, and still she kept seizing. 'It was really heartbreaking,' Ms. Yau says. 'You're in a profession that you want to help, but you feel like nothing is helping ... You're trying to give them hope, and yet you're also suffering alongside with them.' Ms. Yau says a turning point was when Maryam's seizures started affecting the other side of her body, suggested her healthy brain hemisphere was now being affected and therefore at risk. Her breathing was also under threat, prompting increasingly urgent discussions about whether Maryam needed intensive care. Time was running out. Ms. Yau started asking around, searching for options. If Maryam was still too small for a functional hemispherectomy, what else could they do? That's when someone pointed her towards some papers published out of Children's National Hospital in Washington, D.C. One paper, published in the Journal of NeuroInterventional Surgery, described three babies with drug-resistant seizures caused by HME, Maryam's exact condition. The youngest baby was only 13 days old. Their doctors had taken a radical approach for stopping their seizures, with thrilling results – all three were now seizure free. One patient hadn't had a seizure in four years. It was a glimmer of hope. It was also a hard sell. 'We're talking about something drastic,' Ms. Yau says. 'We're causing a stroke.' When open surgery isn't an option, doctors do have another trick for reaching inside the brain. As a medical student in Pondicherry, India, Prakash Muthusami was enthralled by doctors who practiced interventional radiology – a specialty that treats diseases using imaging technologies, like X-ray and MRI, to guide catheters inserted into the patient's blood vessels. It was a kind of medical magic that felt out of reach, almost mystical. But when Dr. Muthusami's friend suffered an aortic dissection, he watched as interventional radiologists fixed this life-threatening condition using just a pinhole opening at the top of his leg. 'That, to me, was so fascinating.' Dr. Muthusami wound up specializing in pediatric neurointervention, treating strokes, aneurysms and blood-vessel abnormalities in the brain without ever cutting open a child's skull. Imagine, for a moment, that the brain is an underground pool inside a rocky cavern. One way of reaching the pool is by drilling through the rock – traditional brain surgery. Another is neurointervention; by mapping the underground tributary system that feeds into the pool, you find one of the connected waterways and travel along until you reach the cavern. 'We use blood vessels as conduits,' Dr. Muthusami says. 'Much like ships and boats sail up rivers and streams, we use catheters and wires.' Recent years have seen neurointerventional approaches increasingly used for childhood diseases, sometimes in game-changing ways. In the past, treating retinoblastoma – the most common form of eye cancer in babies – often meant surgically removing the eye. Nowadays, neurointerventionists can thread a microcatheter into the artery of the baby's eye, delivering a tiny but precise dose of chemotherapy. 'This is an area where technology moves very, very fast,' Dr. Muthusami says. 'There are diseases that yesterday could not be treated. But today, there's the technology and ability.' A few years ago at SickKids, which Dr. Muthusami joined in 2013, he was in a meeting with neurology colleagues when someone flagged some papers published by a medical team in Washington, D.C. They described an audacious new approach for treating babies with drug-resistant seizures caused by HME: an 'endovascular embolic hemispherectomy.' These newborns were too small to undergo open brain surgery, so their doctors devised a workaround. Using catheters and neurointerventional techniques, they intentionally triggered a series of strokes. In other words, they couldn't disconnect the faulty brain hemisphere, so they killed it. Dr. Muthusami was intrigued but cautious. 'We discussed it, nodded around the table, and then moved on,' he says. 'It's so novel that you don't want to introduce something like that unless ... we are backed up against the wall.' That moment arrived in February, 2024, when he learned about Maryam. Dr. Rutka and Ms. Yau both approached him about Maryam's case, explaining how she had run out of options. Would he consider attempting an endovascular hemispherectomy, like the one described in the study out of Washington? 'I think I was silent for a few seconds,' Dr. Muthusami says. Those seconds contained a jumble of questions. Was he ready for something like this? Was his team? What if something went wrong and Maryam wound up in a worse state? He called an old friend and mentor for advice: Was he crazy to consider this? 'It was very counterintuitive to our entire lifelong training,' he says. 'We protect the brain. But here, we are actually going in and causing many strokes.' On the other hand, what was the cost of not trying? Maryam's seizures were becoming life-threatening and he had the team and resources required to take a chance on a risky and novel procedure. And the longer they waited to stop her seizures, the longer her brain was prevented from developing normally, putting Maryam at ever-greater risk of profound disabilities. By morning, he knew his decision. 'This was the only way we could give her a fighting chance.' But the ultimate decision lay with Maryam's parents, and the medical team could see how daunting this idea might be to them, Ms. Yau says. This was a procedure that had never been done before at SickKids, or anywhere in Canada. They would be intentionally damaging parts of her brain. And the risks were stark; a small 'misadventure' with the guide wire could tear an artery, or the strokes could trigger dangerous brain swelling. Death was a possibility. Furthermore, the procedure wasn't even expected to cure Maryam's seizures – just quiet them down to buy her time until she's ready for open brain surgery. Her parents deliberated for a day and a half, but there wasn't much debate. 'We did not have any options other than saying yes,' Ms. Nafees says. 'Because we can't see her like this.' Dr. Muthusami and his team started by using MRI scans to create a 'map' of Maryam's left brain hemisphere. Then, over several days, they devised a strategy for taking it down. They needed to avoid triggering one big stroke, which would increase the risk of brain swelling. So instead, they planned a sequence of mini strokes; think of the careful, stepwise demolition of a building, with dynamite planted in different wings across the building to avoid triggering one massive collapse. Maryam's endovascular hemispherectomy ultimately took place over four procedures, each separated by about a week. Each time, Dr. Muthusami would stroke out a different brain territory, until eventually they all came together to span the entire brain hemisphere. The night before Maryam's first procedure, Ms. Nafees stayed at the hospital overnight at her daughter's bedside, as she regularly did. It was a sleepless night, with Maryam's oxygen levels fluctuating constantly and her seizures firing non-stop. Perhaps the busyness held Ms. Nafees together; she reflects now that a still, quiet moment with her daughter in the dark that night could have invited in the fears that threatened to break her. At 8 a.m. on Feb. 20, Ms. Nafees sent Maryam off with a kiss and the Quran that had been given to the family by SickKids' religious care team. Then she and Mr. Beg settled into the waiting room, where they sat and prayed. Throughout the entire procedure, Maryam's seizures continued. She wasn't moving because of the anesthesia but the electrical activity in her brain was picked up by the EEG electrodes on her scalp. Not too long after Dr. Muthusami deployed his first salvo of microbeads, one of his colleagues from the epilepsy team announced what they were seeing on Maryam's EEG readouts. 'We were watching those seizures die away,' Dr. Muthusami says. 'When we were targeting an area, the seizures from that area would go away.' It was working. About four hours after Maryam was wheeled into the angiography suite, the room filled with the lapping of ocean waves, the sound effect layered into the opening bars of Champagne Supernova, the 90s Oasis hit. How many special people change? How many lives are living strange? It's the song Dr. Muthusami likes to play when wrapping up a major procedure, though he can't exactly explain why. And before parting ways, he and his team – which on this day included a second neurointerventionist, radiology fellows, technologists, nurses, epileptologists and anesthesiologists – often turn to each other to shake hands or high five. From there, Dr. Muthusami's first stop was the family waiting room. 'He told me that everything went very well,' Ms. Nafees says, her voice quavering as she recalls the moment. 'And she was very strong, like a rock.' Maryam wasn't out of the woods yet, though. After a procedure like this, there are worries of a bleed or brain swelling, which could require emergency surgery to remove a piece of the skull. One of the first 13 babies to undergo this procedure in Washington, D.C. died afterwards from complications, according to a recent paper. Maryam was taken to the ICU for close monitoring. But everything was fine. And when Ms. Nafees saw her daughter's first seizure after the procedure, she noticed an immediate difference; her limbs were no longer curling and the twitching was now mainly restricted to her face. 'Her EEG pattern really changed,' says Ms. Yau, who was on vacation at the time but received play-by-play updates from her team. 'There were a lot of shouts of joy, screaming even. We were all ecstatic.' Maryam's second procedure unfolded just as smoothly, as did her third and fourth. By the time she was discharged from SickKids on April 17th – a date that Ms. Nafees considers to be her daughter's true homecoming – she was down to just one or two seizures per day. The plan was to bring Maryam back for a functional hemispherectomy in about six months, but then something surprising occurred. Over the summer, while visiting family in India, Maryam was playing at her grandmother's house when her eye started twitching, just for a few seconds. To date, this remains Maryam's last seizure. Her doctors now believe she may never need brain surgery. 'That exceeded our expectations,' Dr. Muthusami says. It's impossible to say what lies ahead. Maryam is still taking seizure medications and her tuberous sclerosis could always present new problems down the road. At 18 months, Maryam has yet to crawl or walk and is noticeably weaker on one side, giving her a slightly lopsided grin. But the living half of her brain is already taking over. Today, Maryam is a happy toddler who loves spicy food, clapping along to The Wheels on the Bus, and smiling at her grandmother during one of their daily video chats. When her dad absentmindedly puts down his phone, his daughter's tiny hand will dart out to gleefully snatch it. Ms. Nafees is a woman of steadfast beliefs – in God, in fate. She believes it was Maryam's destiny to be born in Toronto, where she had access to not just a specialist like Dr. Muthusami, but the constellation of medical expertise required to pull off such an extraordinary procedure. But her greatest belief, from the moment she laid eyes on her little babu, has always been in Maryam. 'Whatever she wants to do, I think she will do,' she says. 'It was she who fought for herself.'
The Guardian
28-05-2025
- General
- The Guardian
Pippa White's death in NSW hospital was preventable and ‘system failed her', medical experts tell inquest
The septic shock death of a two-year-old girl in a regional New South Wales hospital was preventable, senior medical experts told an inquest. Pippa Mae White died on 13 June 2022, two months before her third birthday, after doctors at the hospitals in Cowra and Orange wrongly assumed she had an acute viral illness rather than the serious bacterial infection that killed her. Giving evidence on Wednesday at the coronial inquest into her death, a 'conclave of experts' who weren't involved in Pippa's treatment said she should have been checked and treated for potential sepsis much earlier than she was. The NSW deputy state coroner Joan Baptie is examining whether Pippa's death was preventable and whether she received appropriate care in Cowra and Orange, and from the Newborn and Paediatric Emergency Transport Service team. The inquest previously heard that Pippa presented to Cowra hospital about 2pm on 12 June 2022 with a fever, vomiting, and a heart rate of 171 beats per minute, considered in the 'red zone' for sepsis. She did not have a blood test until nearly 4am on 13 June, after she was transferred to Orange, which revealed she had a serious infection. She was administered antibiotics and given a chest X-ray, which showed she had pneumonia that had caused a 'complete whiteout' of her left lung. She suffered two cardiac arrests and died just after 1pm. Five of the seven medical experts who submitted a joint report to the inquest gave oral evidence on Wednesday: Prof Simon Craig, Prof John Raftos, Prof Warwick Butt, Prof Adam Irwin and Associate Prof Phillip Braslins, along with GP Robert Morton. While there was some disagreement on when the paediatric sepsis pathway should have been activated for Pippa, all the doctors agreed investigations into possible sepsis – including blood tests – should have been started much sooner than they were. The experts agreed medical staff became 'anchored' on the wrong diagnosis of a viral illness. They stressed the importance making 'serial observations' of vital signs heart rate and oxygen saturation more frequently than was done in Pippa's case. The inquest was previously shown a video of Pippa in the Cowra emergency department making a 'grunting' noise while breathing, which the doctors on Wednesday said would have been identifiable with a stethoscope. Raftos said Pippa was already a 'very sick' and 'lethargic' child when she was taken to Cowra and she needed a rapid response. He said if medical staff in Cowra didn't have the capacity to treat Pippa right away, as the inquest previously heard, they should have arranged for an ambulance to take her to Orange, or for her mother, Annah, to drive her there. Raftos said the alternative was 'doing nothing, which is what they did'. Craig said the 'make or break' point when 'things went absolutely wrong' was at 1am on 13 June when there were 'delays in escalation' at Orange hospital despite Pippa having become 'critically ill'. The junior doctor in charge of looking after Pippa in Orange, Dr Christopher Morris, last year cried in the witness box as he gave evidence to the inquest and said he wished he had called a rapid response earlier in the night. The experts on Wednesday said the senior paediatrician on-call the night Pippa was admitted, Prof Adam Buckmaster, should have assessed her that evening. The inquest previously heard he didn't attend the hospital until the morning of 13 June. Most of the experts agreed Pippa should have had her blood tested and been administered antibiotics around 9pm on 12 June after arriving at the hospital. 'She should have been on the [sepsis pathway], she should have had an IV [intravenous drip] inserted, she should have had bloods done, and she should have had antibiotics given. I think it's very clear,' Butt said. 'I believe the death was preventable, and that means the system failed her,' Butt said. The inquest continues, with a final block of hearings expected later this year.
ABC News
24-05-2025
- Health
- ABC News
The high-tech ECMO machine saving lives and giving families a chance to make memories
Warning: This story contains content that might upset some readers. Five-week-old Sidney Mahony lies in his mother Ashleigh's arms on a secluded balcony at the Queensland Children's Hospital on a summer's day in February. It's 3pm and time to say goodbye — way too soon after their first hello. Dad Lonni is here and Sidney's older sister Tully, then still a toddler. Other family and close friends have also arrived. Some of the hospital team who have cared for Sidney since his birth with a rare and severe heart defect are among the 20 or so people who have made their way to the hospital's seventh floor balcony, to support his family as he takes his final breaths. "It was a beautiful day," Lonni recalls, three years later. "Brisbane couldn't have turned it on any more." For most of his life, Sidney was attached to a machine known as ECMO — an acronym for extra-corporeal membrane oxygenation, a high-tech heart and lung machine. At the end, it's what allowed his parents some precious extra time to make memories with their baby boy. In other cases, such as 15-year-old Sunshine Coast teenager Byron Blackmore, it saved his life. Sidney and Byron are among more than 270 children who have been treated with ECMO since the Queensland Children's Hospital opened in 2014. ECMO patients are so critically ill, only about two-thirds survive. The hospital's three heart surgeons are the only medical specialists in the state qualified to place children on the machine, which has become a treatment of last resort. Queensland Children's Hospital director of cardiac surgery Nelson Alphonso says intensive care specialists will typically call on heart surgeons to place a child on ECMO in cases of severe heart or lung failure, or a combination of both, when all else has failed. "They only call us when the patient is really at death's door, to come and intervene," Dr Alphonso says. Occasionally, they'll move the baby to the operating theatre to connect them to the ECMO machine. But in most cases, there's no time. "The children are too sick to be moved and so we will place them on ECMO where they are," Dr Alphonso explains. "In some instances, we have to open the chest in the paediatric intensive care unit (PICU) and connect the baby to the ECMO machine. "Sometimes we put patients on when they're in a cardiac arrest situation, when they're undergoing chest compressions and being resuscitated." Sidney was about a week old in early January 2022, when he was first put on ECMO after being born with hypoplastic left heart syndrome, also known as "half a heart" because the left side of the heart is severely underdeveloped. He was also born with only one kidney. Towards the end of open-heart surgery to re-plumb his heart's right chamber — allowing it to take over completely from the underdeveloped left side — he suffered an unexplained leak in a key heart valve. Dr Alphonso had to repair the valve on the operating table. When Ashleigh and Lonni finally saw their baby after the operation, he was connected to ECMO — a machine several times his size. "I didn't really know what to expect," Ashleigh says. "There was a lot of blood … just seeing blood going through tubes, an open chest. "I know that they explain it to you, but when you see it in person it's really confronting — like this is your body working for you outside your body, keeping you alive. In simple terms, ECMO takes over the work of the heart and the lungs. It siphons blood out of the child, removes carbon dioxide and oxygenates it before sending it back to the body, giving the patient's heart and lungs time to rest and recover. Having their newborn attached to so many tubes and machines meant bonding was far from instinctual. Picking him up and holding him in their arms was impossible. But with help from hospital PICU staff, they were able to give him a bed bath, using wipes to wash tiny body, and change his nappy. "We wanted to treat it — the situation — the same as we would if we had Sid at home," Lonni says. "We weren't going to just be hands-off parents. That's not our approach. "We wanted to feel useful in some way." Ashleigh gets emotional as she speaks about the simple task of wiping Sidney's eyes when he could open them. "I found that was the best," she says. Tully, then aged 15 months, also spent time at the hospital most days to be with her brother. Sidney was eventually taken off ECMO and doctors returned him to the operating theatre to close his chest. "We thought the worst was over," Dr Alphonso says. But the next day Sidney's heart stopped. "He went into cardiac arrest in front of me," Ashleigh recalls, her voice trembling with emotion. Sidney was reconnected to the ECMO machine to give him every chance of recovering. But when his other organs started failing and his hands and feet went black, doctors met with Ashleigh and Lonni, who were already preparing themselves to farewell Sid. "At this point, you have to recognise in a way you're not prolonging life, but you're prolonging death," Dr Alphonso says. "Since death is inevitable, we usually place the baby in the parents' arms and then we stop the machine. You grow close to the family during all of these journeys. "It does take its toll on everyone." The hospital gave Ashleigh and Lonni time with Sidney to make memories before the ECMO machine was switched off. "We got a couple of extra days where we just got to lay with him in a big hospital bed, and not just his own, to actually cuddle our child and make it our space as well," Lonni says. Ashleigh wipes away tears as she recalls those last days with her son. "They really let us do as much as we possibly could," she says. "There were no limits. All the walls were down. It was like celebrating our son. We got really encouraged … to make memories and we definitely did." Like Sidney, about half of the children treated with ECMO at the Queensland Children's Hospital (QCH) have severe heart disease. Intensive care specialist Sarfaraz Rahiman, the hospital's ECMO service medical lead, says other common reasons children use the heart-lung machine include severe pneumonia and sepsis, a life-threatening response to an infection. "ECMO has dramatically revolutionised the way we look after children with severe heart and lung failure," Dr Rahiman says. "It's intensive care in the truest sense. It's one of the most complex and invasive treatments that we provide." He says children can be placed on ECMO for anywhere from a few days to more than a month. "We've had kids on ECMO for up to six weeks and some of them have done well after," he says. Dr Rahiman is also deputy director of the QCH's PICU, a large team of more than 300 people, including doctors, nurses and allied health workers, who work together to care for the state's sickest children. He says the unit has provided about 1.8 million hours of intensive care to more than 18,000 patients in the past 10 years. About 1.5 per cent of those were treated with ECMO. Maleny State High School student Byron Blackmore has no memory of his time on ECMO last year, but is grateful for the treatment that saved his life. "Without it, I wouldn't be here," the 15-year-old says. Byron bears a scar from neck to naval after two open-heart surgeries at the QCH last year for a genetic heart condition, known as hypertrophic obstructive cardiomyopathy, which causes the heart muscle to thicken. Although Dr Alphonso "cut out a lot of the muscle" in January last year, Byron required more surgery in June. He came out of the second operation critically ill and on ECMO. His mum Jemma Mays described the situation as "terrifying". "It was a pretty scary time for all of us," she says. "No one wants to see their child like that." When she asked Dr Alphonso whether Byron could die, he responded "he's a very, very sick boy". Byron was unconscious and unable to speak to his family for two weeks, but they played Broncos games on television and talked him through what was going on. "It was so hard not being able to have him talk to us," Jemma says. "We still spoke to him." His grandfather also sent a video of him singing Bob Marley's Three Little Birds — which includes the line "Every Little Thing's Gonna Be Alright" — that was played to the teenager in PICU. Byron's older brother Hayden, 21, drove to and from the South Brisbane hospital every second day from the Sunshine Coast to spend time with him. "He would just spend hours with Byron, holding his hand, helping the nurses reposition him, just loving him," Jemma says. Byron was weaned off ECMO and after 28 days in hospital was finally allowed home. He spent months recovering from the effects of being on the machine. "They said it would take about six months to recover from," Jemma says. "And because Byron has mild cerebral palsy as well, there was a lot of flow on effects he had with that. "He had tremors in his hands, and he's had to have a lot of physio. It's been such a big thing." But Byron is alive and asked how he's feeling, he says "amazing". Two weeks after Sidney Mahony's death, his family's house at Oxley, in Brisbane's south, flooded during the 2022 deluge. "You're dealing with being in such flight or fight and shock of what's happened that when you lose your belongings, it doesn't feel like anything," Ashleigh says. "The grieving of the house was almost non-existent because Sid died." As they dealt with their trauma, it was Tully who helped them through their heartache. "Tully really saved us," Ashleigh says. "She was always the joy in the room. "I remember at the time being like: 'I'll be able to thank you when you're older for helping me.' She's been through a lot. She's very emotionally aware and a very caring girl." And then a year ago, Harlan was born. Nothing can ever replace Sidney, but Harlan's entry into the world has brought some healing. "A lot of my emotions and a lot of my grief were resolved in that moment of having to hold Harlan in my arms," Lonni says.
