Paralyzed By Polio, This British Tea Broker Changed the Course Of Medical History Forever
In December 1958, on a vacation with his wife in Kenya, a 28-year-old British tea broker named Robin Cavendish became suddenly ill. Neither he nor his wife Diana knew it at the time, but Robin's illness would change the course of medical history forever.
Robin was rushed to a nearby hospital in Kenya where the medical staff delivered the crushing news: Robin had contracted polio, and the paralysis creeping up his body was almost certainly permanent. The doctors placed Robin on a ventilator through a tracheotomy in his neck, as the paralysis from his polio infection had rendered him unable to breathe on his own – and going off the average life expectancy at the time, they gave him only three months to live. Robin and Diana (who was pregnant at the time with their first child, Jonathan) flew back to England so he could be admitted to a hospital. They mentally prepared to wait out Robin's final days.
But Robin did something unexpected when he returned to the UK – just one of many things that would astonish doctors over the next several years: He survived. Diana gave birth to Jonathan in February 1959 and continued to visit Robin regularly in the hospital with the baby. Despite doctors warning that he would soon succumb to his illness, Robin kept living.
After a year in the hospital, Diana suggested something radical: She wanted Robin to leave the hospital and live at home in South Oxfordshire for as long as he possibly could, with her as his nurse. At the time, this suggestion was unheard of. People like Robin who depended on machinery to keep them breathing had only ever lived inside hospital walls, as the prevailing belief was that the machinery needed to keep them alive was too complicated for laypeople to operate. But Diana and Robin were up for the challenges – and the risks. Because his ventilator ran on electricity, if the house were to unexpectedly lose power, Diana would either need to restore power quickly or hand-pump air into his lungs to keep him alive.
Robin's wheelchair was not only the first of its kind; it became the model for the respiratory wheelchairs that people still use today.
In an interview as an adult, Jonathan Cavendish reflected on his parents' decision to live outside the hospital on a ventilator: "My father's mantra was quality of life," he explained. "He could have stayed in the hospital, but he didn't think that was as good of a life as he could manage. He would rather be two minutes away from death and living a full life."
After a few years of living at home, however, Robin became tired of being confined to his bed. He longed to sit outside, to visit friends, to travel – but had no way of doing so without his ventilator. So together with his friend Teddy Hall, a professor and engineer at Oxford University, the two collaborated in 1962 to create an entirely new invention: a battery-operated wheelchair prototype with a ventilator built in. With this, Robin could now venture outside the house – and soon the Cavendish family became famous for taking vacations. It was something that, by all accounts, had never been done before by someone who was ventilator-dependent. Robin and Hall also designed a van so that the wheelchair could be plugged in and powered during travel. Jonathan Cavendish later recalled a particular family vacation that nearly ended in disaster when the van broke down outside of Barcelona, Spain:
"My poor old uncle [plugged] my father's chair into the wrong socket," Cavendish later recalled, causing the electricity to short. "There was fire and smoke, and both the van and the chair ground to a halt." Johnathan, who was eight or nine at the time, his mother, and his uncle took turns hand-pumping Robin's ventilator by the roadside for the next thirty-six hours, waiting for Professor Hall to arrive in town and repair the van. Rather than being panicked, the Cavendishes managed to turn the vigil into a party. Townspeople came to greet them, bringing food and music, and a local priest even stopped by to give his blessing.
Robin had become a pioneer, showing the world that a person with severe disabilities could still have mobility, access, and a fuller quality of life than anyone had imagined. His mission, along with Hall's, then became gifting this independence to others like himself. Robin and Hall raised money – first from the Ernest Kleinwort Charitable Trust, and then from the British Department of Health – to fund more ventilator chairs, which were then manufactured by Hall's company, Littlemore Scientific Engineering, and given to fellow patients who wanted to live full lives at home. Robin and Hall used themselves as guinea pigs, testing out different models of the chairs and collaborating with scientists to create other devices for those with disabilities. One invention, called the Possum, allowed paraplegics to control things like the telephone and television set with just a nod of the head. Robin's wheelchair was not only the first of its kind; it became the model for the respiratory wheelchairs that people still use today.
Robin went on to enjoy a long and happy life with his family at their house in South Oxfordshire, surrounded by friends who would later attest to his "down-to-earth" personality, his sense of humor, and his "irresistible" charm. When he died peacefully at his home in 1994 at age 64, he was considered the world's oldest-living person who used a ventilator outside the hospital – breaking yet another barrier for what medical science thought was possible.
The unprecedented scale and impact of the COVID-19 pandemic has caused scientists and engineers around the world to stop whatever they were working on and shift their research toward understanding a novel virus instead.
"We have confidence that we can use our system in the next pandemic."
For Guangyu Qiu, normally an environmental engineer at the Swiss Federal Laboratories for Materials Science and Technology, that means finding a clever way to take his work on detecting pollution in the air and apply it to living pathogens instead. He's developing a new type of biosensor to make disease diagnostics and detection faster and more accurate than what's currently available.
But even though this pandemic was the impetus for designing a new biosensor, Qiu actually has his eye on future disease outbreaks. He admits that it's unlikely his device will play a role in quelling this virus, but says researchers already need to be thinking about how to make better tools to fight the next one — because there will be a next one.
"In the last 20 years, there [have been] three different coronavirus [outbreaks] ... so we have to prepare for the coming one," Qiu says. "We have confidence that we can use our system in the next pandemic."
"A Really, Really Neat Idea"
His main concern is the diagnostic tool that's currently front and center for testing patients for SARS-Cov-2, the virus causing the novel coronavirus disease. The tool, called PCR (short for reverse transcription polymerase chain reaction), is the gold standard because it excels at detecting viruses in even very small samples of mucus. PCR can amplify genetic material in the limited sample and look for a genetic code matching the virus in question. But in many parts of the world, mucus samples have to be sent out to laboratories for that work, and results can take days to return. PCR is also notoriously prone to false positives and negatives.
"I read a lot of newspapers that report[ed] ... a lot of false negative or false positive results at the very beginning of the outbreak," Qiu says. "It's not good for protecting people to prevent further transmission of the disease."
So he set out to build a more sensitive device—one that's less likely to give you a false result. Qiu's biosensor relies on an idea similar to the dual-factor authentication required of anyone trying to access a secure webpage. Instead of verifying that a virus is really present by using one way of detecting genetic code, as with PCR, this biosensor asks for two forms of ID.
SARS-CoV-2 is what's called an RNA virus, which means it has a single strand of genetic code, unlike double-stranded DNA. Inside Qiu's biosensor are receptors with the complementary code for this particular virus' RNA; if the virus is present, its RNA will bind with the receptors, locking together like velcro. The biosensor also contains a prism and a laser that work together to verify that this RNA really belongs to SARS-CoV-2 by looking for a specific wavelength of light and temperature.
If the biosensor doesn't detect either, or only registers a match for one and not the other, then it can't produce a positive result. This multi-step authentication process helps make sure that the RNA binding with the receptors isn't a genetically similar coronavirus like SARS-CoV, known for its 2003 outbreak, or MERS-CoV, which caused an epidemic in 2012.
It could also be fitted to detect future novel viruses once their genomes are sequenced.
The dual-feature design of this biosensor "is a really, really neat idea that I have not seen before with other sensor technology," says Erin Bromage, a professor of infection and immunology at the University of Massachusetts Dartmouth; he was not involved in designing or testing Qiu's biosensor. "It makes you feel more secure that when you have a positive, you've really got a positive."
The light and temperature sensors are not in themselves new inventions, but the combination is a first. The part of the device that uses light to detect particles is actually central to Qiu's normal stream of environmental research, and is a versatile tool he's been working with for a long time to detect aerosols in the atmosphere and heavy metals in drinking water.
Bromage says this is a plus. "It's not high-risk in the sense that how they do this is unique, or not validated. They've taken aspects of really proven technology and sort of combined it together."
This new biosensor is still a prototype that will take at least another 12 months to validate in real world scenarios, though. The device is sound from a biological perspective and is sensitive enough to reliably detect SARS-CoV-2 — and to not be tricked by genetically similar viruses like SARS-CoV — but there is still a lot of engineering work that needs to be done in order for it to work outside the lab. Qiu says it's unlikely that the sensor will help minimize the impact of this pandemic, but the RNA receptors, prism, and laser inside the device can be customized to detect other viruses that may crop up in the future.
"If we choose another sequence—like SARS, like MERS, or like normal seasonal flu—we can detect other viruses, or even bacteria," Qiu says. "This device is very flexible."
It could also be fitted to detect future novel viruses once their genomes are sequenced.
The Long-Term Vision: Hospitals and Transit Hubs
The device has been designed to connect with two other systems: an air sampler and a microprocessor because the goal is to make it portable, and able to pick up samples from the air in hospitals or public areas like train stations or airports. A virus could hopefully be detected before it silently spreads and erupts into another global pandemic. In the case of SARS-CoV-2, there has been conflicting research about whether or not the virus is truly airborne (though it can be spread by droplets that briefly move through the air after a cough or sneeze), whereas the highly contagious RNA virus that causes measles can remain in the air for up to two hours.
"They've got a lot on the front end to work out," Bromage says. "They've got to work out how to capture and concentrate a virus, extract the RNA from the virus, and then get it onto the sensor. That's some pretty big hurdles, and may take some engineering that doesn't exist right now. But, if they can do that, then that works out really quite well."
One of the major obstacles in containing the COVID-19 pandemic has been in deploying accurate, quick tools that can be used for early detection of a virus outbreak and for later tracing its spread. That will still be true the next time a novel virus rears its head, and it's why Qiu feels that even if his biosensor can't help just yet, the research is still worth the effort.
It could also be fitted to detect future novel viruses once their genomes are sequenced.
The dual-feature design of this biosensor "is a really, really neat idea that I have not seen before with other sensor technology," says Erin Bromage, a professor of infection and immunology at the University of Massachusetts Dartmouth; he was not involved in designing or testing Qiu's biosensor. "It makes you feel more secure that when you have a positive, you've really got a positive."
The light and temperature sensors are not in themselves new inventions, but the combination is a first. The part of the device that uses light to detect particles is actually central to Qiu's normal stream of environmental research, and is a versatile tool he's been working with for a long time to detect aerosols in the atmosphere and heavy metals in drinking water.
Bromage says this is a plus. "It's not high-risk in the sense that how they do this is unique, or not validated. They've taken aspects of really proven technology and sort of combined it together."
This new biosensor is still a prototype that will take at least another 12 months to validate in real world scenarios, though. The device is sound from a biological perspective and is sensitive enough to reliably detect SARS-CoV-2 — and to not be tricked by genetically similar viruses like SARS-CoV — but there is still a lot of engineering work that needs to be done in order for it to work outside the lab. Qiu says it's unlikely that the sensor will help minimize the impact of this pandemic, but the RNA receptors, prism, and laser inside the device can be customized to detect other viruses that may crop up in the future.
"If we choose another sequence—like SARS, like MERS, or like normal seasonal flu—we can detect other viruses, or even bacteria," Qiu says. "This device is very flexible."
It could also be fitted to detect future novel viruses once their genomes are sequenced.
The Long-Term Vision: Hospitals and Transit Hubs
The device has been designed to connect with two other systems: an air sampler and a microprocessor because the goal is to make it portable, and able to pick up samples from the air in hospitals or public areas like train stations or airports. A virus could hopefully be detected before it silently spreads and erupts into another global pandemic. In the case of SARS-CoV-2, there has been conflicting research about whether or not the virus is truly airborne (though it can be spread by droplets that briefly move through the air after a cough or sneeze), whereas the highly contagious RNA virus that causes measles can remain in the air for up to two hours.
"They've got a lot on the front end to work out," Bromage says. "They've got to work out how to capture and concentrate a virus, extract the RNA from the virus, and then get it onto the sensor. That's some pretty big hurdles, and may take some engineering that doesn't exist right now. But, if they can do that, then that works out really quite well."
One of the major obstacles in containing the COVID-19 pandemic has been in deploying accurate, quick tools that can be used for early detection of a virus outbreak and for later tracing its spread. That will still be true the next time a novel virus rears its head, and it's why Qiu feels that even if his biosensor can't help just yet, the research is still worth the effort.
Spina Bifida Claimed My Son's Mobility. Incredible Breakthroughs May Let Future Kids Run Free.
When our son Henry, now six, was diagnosed with spina bifida at his 20-week ultrasound, my husband and I were in shock. It took us more than a few minutes to understand what the doctor was telling us.
When Henry was diagnosed in 2012, postnatal surgery was still the standard of care – but that was about to change.
Neither of us had any family history of birth defects. Our fifteen-month-old daughter, June, was in perfect health.
But more than that, spina bifida – a malformation of the neural tube that eventually becomes the baby's spine – is woefully complex. The defect, the doctor explained, was essentially a hole in Henry's lower spine from which his spinal nerves were protruding – and because they were exposed to my amniotic fluid, those nerves were already permanently damaged. After birth, doctors could push the nerves back into his body and sew up the hole, but he would likely experience some level of paralysis, bladder and bowel dysfunction, and a buildup of cerebrospinal fluid that would require a surgical implant called a shunt to correct. The damage was devastating – and irreversible.
We returned home with June and spent the next few days cycling between disbelief and total despair. But within a week, the maternal-fetal medicine specialist who diagnosed Henry called us up and gave us the first real optimism we had felt in days: There was a new, experimental surgery for spina bifida that was available in just a handful of hospitals around the country. Rather than waiting until birth to repair the baby's defect, some doctors were now trying out a prenatal repair, operating on the baby via c-section, closing the defect, and then keeping the mother on strict bedrest until it was time for the baby to be delivered, just before term.
This new surgery carried risks, he told us – but if it went well, there was a chance Henry wouldn't need a shunt. And because repairing the defect during my pregnancy meant the spinal nerves were exposed for a shorter amount of time, that meant we'd be preventing nerve damage – and less nerve damage meant that there was a chance he'd be able to walk.
Did we want in? the doctor asked.
Had I known more about spina bifida and the history of its treatment, this surgery would have seemed even more miraculous. Not too long ago, the standard of care for babies born with spina bifida was to simply let them die without medical treatment. In fact, it wasn't until the early 1950s that doctors even attempted to surgically repair the baby's defect at all, instead of opting to let the more severe cases die of meningitis from their open wound. (Babies who had closed spina bifida – a spinal defect covered by skin – sometimes survived past infancy, but rarely into adulthood).
But in the 1960s and 1970s, as more doctors started repairing defects and the shunting technology improved, patients with spina bifida began to survive past infancy. When catheterization was introduced, spina bifida patients who had urinary dysfunction, as is common, were able to preserve their renal function into adulthood, and they began living even longer. Within a few decades, spina bifida was no longer considered a death sentence; people were living fuller, happier lives.
When Henry was diagnosed in 2012, postnatal surgery was still the standard of care – but that was about to change. The first major clinical trial for prenatal surgery and spina bifida, called Management of Myelomeningocele (MOMS) had just concluded, and its objective was to see whether repairing the baby's defect in utero would be beneficial. In the trial, doctors assigned eligible women to undergo prenatal surgery in the second trimester of their pregnancies and then followed up with their children throughout the first 30 months of the child's life.
The results were groundbreaking: Not only did the children in the surgery group perform better on motor skills and cognitive tests than did patients in the control group, only 40 percent of patients ended up needing shunts compared to 80 percent of patients who had postnatal surgery. The results were so overwhelmingly positive that the trial was discontinued early (and is now, happily, the medical standard of care). Our doctor relayed this information to us over the phone, breathless, and left my husband and me to make our decision.
After a few days of consideration, and despite the benefits, my husband and I actually ended up opting for the postnatal surgery instead. Prenatal surgery, although miraculous, would have required extensive travel for us, as well as giving birth in a city thousands of miles from home with no one to watch our toddler while my husband worked and I recovered. But other parents I met online throughout our pregnancy did end up choosing prenatal surgery for their children – and the majority of them now walk with little assistance and only a few require shunting.
Sarah Watts with her husband, daughter June, and son Henry, at a recent family wedding.
Even more amazing to me is that now – seven years after Henry's diagnosis, and not quite a decade since the landmark MOMS trial – the standard of care could be about to change yet again.
Regardless of whether they have postnatal or prenatal surgery, most kids with spina bifida still experience some level of paralysis and rely on wheelchairs and walkers to move around. Now, researchers at UC Davis want to augment the fetal surgery with a stem cell treatment, using human placenta-derived mesenchymal stromal cells (PMSCs) and affixing them to a cellular scaffold on the baby's defect, which not only protects the spinal cord from further damage but actually encourages cellular regeneration as well.
The hope is that this treatment will restore gross motor function after the baby is born – and so far, in animal trials, that's exactly what's happening. Fetal sheep, who were induced with spinal cord injuries in utero, were born with complete motor function after receiving prenatal surgery and PMSCs. In 2017, a pair of bulldogs born with spina bifida received the stem cell treatment a few weeks after birth – and two months after surgery, both dogs could run and play freely, whereas before they had dragged their hind legs on the ground behind them. UC Davis researchers hope to bring this treatment into human clinical trials within the next year.
A century ago, a diagnosis of spina bifida meant almost certain death. Today, most children with spina bifida live into adulthood, albeit with significant disabilities. But thanks to research and innovation, it's entirely possible that within my lifetime – and certainly within Henry's – for the first time in human history, the disabilities associated with spina bifida could be a thing of the past.