This article originally appeared in Undark.
Brad Fuller He was just a baby when he was diagnosed with polio as a result of a 1952 outbreak. In 1951, he was taken to an emergency room miles away from his home in northeast Pennsylvania. For nine months, Fuller remained in the hospital, where he was kept for long periods of time with an iron lung. This ventilator, which is large and metallic, helped him to breathe. Fuller was not allowed to visit his parents. Fuller’s earliest memories are of a nurse holding Fuller in a pool with a mineral water and instructing him how to kick his legs.
This year was the peak of the epidemic, with approximately 58,000 Americans becoming poliomyelitis and 3000 dying. Fuller had it all. Fuller was spared by the disease, which left him with a weak right leg and left arm. His recovery allowed him to continue playing tennis, football and as a psychologist. While he was part-time at St. Joseph’s University, Philadelphia, he built a successful career in non-profit leadership. Fuller stated that despite his past, he still felt strong.
In his 40s, Fuller was offered a second doctor to help him with his post-polio. Fuller was shocked. Fuller was stunned. The symptoms of muscle weakness, poor balance and increased muscle weakness had started to show. He had once fallen on his knee and inflicted serious injury to it. Because he is so prone to falling, he now has a complete leg brace. He said that if he were a normal person — someone without any background in mental illness — “I would immediately go into denial.”
Although scientists believe the poliovirus is a virus that has existed for thousands of centuries, it didn’t cause widespread epidemics until late 1800s when the United States, Britain, and other countries began experiencing severe outbreaks. In the early 1950s, polio terror had already paralyzed or killed more than half a million people worldwide. Hospitals created intensive care units to treat the large number of people who were sick. Many ICU patients in those hospitals were young people and children.
A pair of very effective vaccines followed, with the first licensed in 1955. Within a few years, the incidence of this virus dropped dramatically and was quickly eradicated from all countries. The widespread acceptance of vaccines is a sign of good public health. Fuller recalls that her mother “would have given anything” to get a vaccine for her kids. There was one downside for people who were already infected but survived. Polio “supposedly disappeared,” Fuller said. This means that research has ceased.
For patients with post-polio syndrome (or PPS), which now affects between 25 and 40 percent of survivors, this history can be bittersweet. These patients now face a variety of symptoms as adults that stem from the initial infection. They can experience pain, fatigue and muscle weakness again. According to estimates, PPS can affect up to 300,000. It could also be affecting as many as 20 million people worldwide. Marny Eulberg is a doctor with PPS and the founder of one the United States’ first dedicated clinics for polio. She said that most polio survivors are over 65. Many survivors in the developing countries are younger.
Fuller had another advantage. Fuller’s doctor recognized signs and symptoms of PPS. Patients often struggle for many years to understand their symptoms. However, they are finally diagnosed with PPS and find that no treatment is available. Researchers are still trying to find a way to cure PPS, but the cause is unknown. The few scientists who are still researching the disease believe that further research will help to understand the long-term effects of polio and slow down patients’ physical decline. However, funding is limited.
The sentiment within all funding agencies is that polio has vanished. Frans Nollet is a prominent figure in European postpolio research and heads an Amsterdam University Medical Centers specialty clinic. He said, “It is difficult.” He said, “People believe the disease is gone. But the patients are still there.”
Like polio itself, Post-polio syndrome has been known since antiquity. It didn’t catch the attention of the medical community until the 1950s, when the first generation of polio victims grew up.
Marilyn Fletcher was a polio survivor and physician who approached the National Institutes of Health to help. Fletcher was able to arrange a meeting with Marinos Dalakas, neurologist, in 1982 after numerous phone calls and visits. She told Fletcher that at least 20 people living with polio in Washington D.C. had developed new symptoms. To describe the condition, these individuals invented a term called “post-polio syndrome” Fletcher stated that similar symptoms could be seen in thousands of Americans. Fletcher stated that D.C. residents were upset at the way doctors ignored them.
Dalakas initially was skeptical, and he didn’t take any action. A month later, Dalakas received an email inviting him to visit Fletcher in the office of the Surgeon General. Dalakas wrote in 1995 that Everett C. Coop was newly appointed and expressed concern over the “emerging phenomenon of PPS” as described by Dr. Fletcher. Dalakas led a survey of the first kind to find out more.
2.800 people with medical records that indicated a polio-related disability were sent the survey. Nearly 2,500 individuals responded to the survey, many of whom also sent letters or personal photos along with questionnaires. Dalakas, in an email to Undark, stated that many of the answers contained information which was highly stimulating and emotional. Dalakas recalled that a man aged 50 who had contracted polio when he was 19, struggled to learn how to drive and even dress. Dalakas quickly saw the devastating effects of PPS.
It was not easy to determine what this condition meant. The study participants collectively reported many different symptoms, and new signs often appeared years or decades later than the original infection. Often, these new symptoms occurred when the survivors were still in their 20s, 30s, 40s, 50s. Experts disagree on what is PPS because of the heterogeneity. A formal diagnosis is made after a period of stability for 15 or 20 years without any new weakness or impairment.
Frances Quinn was just 1 years old when she contracted polio. She said that her symptoms were never stable. She felt weakness in her right upper arm and thumb at 14 years old. According to her, the changes were caused by her growing weakness in her upper right arm and thumb, as well as the loss of baby weight. Quinn said that there is a lot of information about PPS which doesn’t really come from good science. Quinn was a U.K. government physicist for the majority of her adult life. PPS is when a patient recognizes something is wrong, and finds someone to listen.
Katharina Sunnerhagen from the University of Gothenburg, Sweden raised similar concerns. My problem is, “For instance, if your child had polio at the age of 1, when will you be able to maintain your health?” Adolescence and childhood are periods of high physical and mental growth. To be able to enjoy stability for 15-20 years, a person who has survived polio must wait to become a man.
The World Health Organization officially defined PPS in February 2022 when it revised its International Classification of Diseases. According to the WHO, PPS is now called “post-polio progression muscular atrophy.” Some critics have suggested that this definition does not accurately reflect patients’ symptoms and increases the risk that they will be given the wrong type of care. It is worth changing it. Quinn asked Quinn. Quinn replied, “It took forty years to get PPS recognised and creating more names only increases confusion.” Disability payments may also be affected by some sufferers, although for the time being, doctors in the U.S.A and U.K still use the old classification.
Because symptoms can often present in middle-life making it hard for patients to maintain their jobs, government recognition of PPS has been crucial. Carol Ferguson was a mother and polio survivor. She worked at a Pennsylvania bed and breakfast but had to leave because she couldn’t stand for the entire day. She said, “My doctors made it clear that this part of my life was finished.”
Some patients with postpolio had only mild symptoms at first. Some needed braces for their legs, calipers or iron lung. The reemergence can be devastating for those in the last group. Eulberg was just four years old when she contracted polio. Eulberg recalls that her parents promised her that she could walk with no leg brace if she worked hard enough. That promise was kept in junior high. However, decades later her muscles began to weaken so she had to get a brace. She told a Medscape reporter that it “felt like failure” in 2020.
Unexpected risks can also be present. Anesthesia may prove fatal in rare cases for patients with PPS. They might need half of the usual dose due to increased sensitivity and dependence on sedatives. Patients with PPS may experience difficulty swallowing or breathing, and they might be slower to wake up after treatment. This could indicate that they are at risk for the same neuromuscular conditions as others. John MacFarlane was a former President of the European Polio Union and a campaigner. He had presented a talk on PPS to British Medical Association. His wheelchair fell into a hole and he was unable to get out of the conference building. His arm was pinched and had to be plated. Undark told him that he woke up 14 hours after the surgery.
FOr many years. Ferguson, a former B&B worker didn’t know she had contracted the poliovirus. She recalls that her family assumed she had the “summer flu” when she was two years old. At age 11 Ferguson suffered from a severe case of drop feet and was diagnosed with the poliovirus. Ferguson’s mom was skeptical and it was never mentioned again. Ferguson said that self-blame and feelings of guilt may have played a role in the current situation. Many parents thought that their kids would stay healthy if they kept the house clean.
Ferguson was a very clumsy child who often stumbled. She fell on ice skates and required minor surgery. Three days later, she was in intensive care. She recalls that the anesthesiologist probed her for explanations as to why she hadn’t woken up in three days. He warned her about the summer flu and said, “Don’t ever forget that.” Ferguson had been suffering from the symptoms of polio. He did not give an official diagnosis for PPS.
Patients must have a series of scans and blood tests to confirm that they are healthy. Ferguson recalls that she started this journey and was confronted by several doctors. One believed she had multiple-sclerosis, while another thought she might have a brain tumour. After these conditions had been ruled out, a University of Pennsylvania neurologist performed an EMG test, which measures the motor neurons’ function. Ferguson was lying on her back with needles in her legs, and her husband held her hand as the EMG recorded her muscle activity. Undark told Ferguson that her husband called her students halfway through to demonstrate to them how “old polio” looks.
It was an emotional moment for Ferguson. It was the end of her journey towards diagnosis. She said, “We had it.” She said, “All other options had been eliminated.”
Ferguson sought help from a polio clinic, Englewood in New Jersey, to manage her symptoms. This clinic, like other ones, provided access to a variety of specialists including occupational therapists and nutritionists as well as rehabilitative doctors and physical therapists. Ferguson was walking 10,000 steps per day to stay healthy. She was using frayed muscles and overdoing it at the clinic. Ferguson stated that this insight and the care provided by the clinic were what saved her.
Despite their benefits, polio clinics have been shutting down, said Carol Vandenakker-Albanese, a professor of health sciences who runs a clinic at UC Davis. She said that many of the doctors who used to specialize in the treatment of the disease are now retired.
Undark was told by four specialists that PPS is not well-known among medical professionals. Although there have been a lot of studies published since 1980s, doctors often don’t keep up with science. Richard Bruno, a psychophysiology graduate, said that many doctors simply do not believe in PPS. He also led Englewood Clinic where Ferguson was treated. Sunnerhagen in Sweden was told by a patient that ankle pain caused by childhood polio could be due to her asking her doctor.
Ferguson volunteers for a Pennsylvania-based support organization she founded. It publishes newsletters, personal stories and a listing of specialists. There are support groups in other states as well. They allow patients to share information about their symptoms, possible treatments, and offer tips and tricks for helping doctors educate them and advocate for themselves. A German doctor working with PPS argued for medical cannabis and developed a protocol that can help with patients’ symptoms. He said that insurance companies and physicians were reluctant to approve the treatment.
Brad Fuller is a survivor, whose first memories are of swimming in a pool. He would love to see more assistance from the U.S. for PPS patients. Many of these people need wheelchairs or braces to live productive lives. However, such aids are expensive.
Eulberg said that she advises patients at her clinic in Wheat Ridge, Colorado. “Basically,” Eulberg explained, “we are telling polio survivors: “Yes, it would have been wonderful if all health care providers you met knew more about polio. But, that is not going to happen. “You’ll have to advocate for yourself.
You2001 Antonio Toniolo was a physician and microbiologist who suffered a severe car accident. He spent the next two years in rehabilitation, visiting patients who had suffered from polio and seeking treatment for muscle weakness. Toniolo, at that time, was working as a medical student at the University of Insubria in Italy. He recalls that survivors began to refer to Toniolo as “Doctor”, and asked him, “What are your thoughts on polio?”
As it is now, PPS’s cause was unknown. Scientists know that some motor neurons in the body are destroyed by the first infection. These cells allow brain and muscles to communicate. Some of these neuronal cells grow rapidly, to 7 to 8 times their normal size. However, they become unable to sustain themselves, and eventually die. It is still unknown why patients become more severely affected by this phenomenon.
In the 80s and 90s researchers were focused on understanding PPS’s causes and defining it. Toniolo started to question if the poliovirus could still be in survivors’ blood, as earlier research suggested. This would explain their symptoms. He collected blood from 100 patients who had suffered from post-polio syndrome in 2013, and then compared them with samples from 50 other survivors. He discovered traces of poliovirus within the blood and spinal fluid of approximately two-thirds (or less) of PPS patients compared to very few samples from the control group. He suggested that the virus could be still circulating and cause cell death or inflammation in these patients. The condition could be treated with antiviral medications.
Toniolo presented preliminary data to a few PPS-related events as well as in a 2015 mid-study report. The research was not published in any scientific journals and it never got finalized. Undark was notified by Undark via email that “this was costly research and required funds as well as competent people.” The work was not completed due to lack funds and retirement at the close of 2018.
No one has yet tested whether antivirals might work for PPS. Dalakas leads a multi-center, large study that examines whether proteins found in blood from healthy donors can neutralize cytokines detected in blood and spinal fluids in some patients with PPS. The therapy called IVIG is expected to calm the immune system and improve patients’ quality-of-life.
Although the treatment was not proven effective in Sweden, it has been tried in other countries. Nollet said that regardless of the results, it would be useful for patients to see if it works. His clinic is one of those participating in this study, which was sponsored by Grifols, a Spanish company.
Swedish officials are reorganizing care for rare diseases to ensure that survivors of polio will receive expert treatment. This decision was driven by recent developments: In July, New York health officials announced that the virus had paralysed a man who lived in an area with low vaccination rates. This was the first case of poliovirus in America for many years. The virus was still spreading throughout the country, even though it had been stopped in some areas. For the first time ever in many decades, the virus was detected in the United Kingdom, Israel, Malawi and Mozambique in 2022. This alarming discovery prompted calls to vaccination campaigns and raised public awareness.
Sunnerhagen said that the Swedish government learned from New York’s cases that there was a possibility of polio epidemics again. This is because the university hospital has applied for special status. The clinic houses about 1000 post-polio patients. This includes a large number of people from Sweden and a smaller group who came to Sweden as children. She stated that “we need to spread knowledge” to bring attention to the fact that there are still polio victims in the society. Sunnerhagen does not expect every physician to have a complete understanding of the condition, but she is hopeful that at the very least they will be able to refer patients.
Patients are driving a lot of their medical care for the moment.
Ferguson stated that she can understand why doctors might not be able to diagnose a multisymptom disease caused by an old infection. She said, “But it is difficult to deny the existence of this disease.” I am a living proof that even a mild case of the virus could cause permanent damage.
This article originally appeared on Undark. You can read the original article.