40 Years of Solitude
In a small town in northern Colombia, an alarming number of families are plagued with early onset Alzheimer’s. We meet them to find out what we can learn about the condition and its consequences from this extraordinary rural microcosm.
In the Colombian town of Yarumal, the twin towers of the basilica overlook a central plaza that slopes steeply down the mountainside. Next to it stands a school, featuring the iconic black-and-white stripes of famed local architect Augustín Goovaerts. On a sunny February afternoon the square is filled with school children in green-and-white uniforms, the colours of the department of Antioquia, some of whom sit on benches and throw bread to flocks of pigeons. The rest of the town sprawls and cascades down the side of the green hill.
In a small coffee shop at the foot of the plaza Mauricio Restrepo, a local historian, describes the town’s establishment. “In the late eighteenth century the Spanish were expanding north from Medellin into the abrupt hills of Antioquia. They searched for a flat area to establish a town but there was none to be found. So they settled on this spot, where the Morro Azul (blue hill) offered shelter from the winds, and where there was an abundance of natural water. They planted a cross and eventually they etched the city out of the mountainside.”
As a young man in the 1970s Ricardo, a construction worker for the municipal government, used to swim in the cold rivers and pools that surround the town. “He used to wake up at 3am to bathe in the Yaramual waters,” says Ofelia, his wife. “He loved to swim and he loved to party. He had many friends and his passion was to dance the tango.”
Some time in his early forties Ricardo’s behaviour began to change. “First he forgot how to wash himself, then he became scared of water and forgot how to shave. Next he began to forget where his clothes were, and started to walk out into the street bare-footed.” Ricardo was suffering the first symptoms of early-onset Alzheimer’s, a disease that corrodes the brain, robs victims of their memories and leads to dementia. But his decline did not come as a total shock to Ricardo’s friends and family. His father had succumbed in a similar way at the same age and, with time, four of his five siblings developed the disease. In the small towns and villages that dot the mountainous terrain of northern Antioquia, La Bobera (the craziness) is a well-known and accepted phenomenon, and an estimated 5,000 paisas (natives of Antioquia) currently have the disease. Since the 1970s a local neuroscientist, Francisco Lopera, has worked to unravel the mystery of the region’s unravelling minds. His findings have helped develop a trial treatment that offers one of the best hopes of finding a cure.
On the fourth floor of the Neuroscience building in the University of Antioquia, where Lopera teaches, a lab assistant peels back the lid of a white plastic container, the size of a paint pot. The brain inside lacks the expected definition of the cerebral cortex; it is withered, flaccid and surprisingly small. It looks like it belonged to a child, but this is an adult brain. “When a brain is affected by Alzheimer’s it loses weight and volume. Typically brain mass falls from 1.4 kilograms to around 700 to 800 grams. Amino acids called amyloids build up in the brain. They destroy neurons in the brain and bring on the symptoms of the disease.”
Lopera’s own relationship with Alzheimer’s goes back 40 years. During his first year at medical school in Medellín, the capital of Antioquia, he returned to his hometown of Aragón, where his paternal grandmother was in the late stages of the disease. He remembers for the first time seeing his father, a strong and hardy paisa, crying when it became apparent his mother no longer recognised him. A few years later he attended a woman from another local town, Belmira, who had also lost her memory. “Two things struck me: her youth and the fact that her father, grandfather and various members of her family had suffered the same thing.”
In 1976 Lopera set off into the Antioquian countryside to interview victims and their families and to draw up family trees. He had his suspicions that this was a case of hereditary early onset Alzheimer’s, suspicions only confirmed in 1995 when he was given permission to send the brain of a recently deceased patient to be examined at Harvard University. In the same year, a series of DNA tests on the victims families identified a genetic mutation known as E208A, which soon became dubbed ‘the Paisa mutation’ Individuals with a parent carrying the mutation have a 50% chance of developing early onset Alzheimer’s by the time they are 45.
I meet Ofelia on crowded corner of a working class neighbourhood of Medellín. At 52 years old, under five feet tall with short brown hair and sporting a red Colombia football shirt, she is stoic and reserved. But after we climb the tight metal spiral staircase to her modest second floor apartment she begins to reminisce. She met Ricardo when she was just 16, in the small town of Salamina in Caldas department. He was 24, from Yarumal and working on a construction project in town. A year later they were married and soon moved to live close to his family in Yarumal. It was there his symptoms became apparent.
Alzheimer’s eats up memories in the reverse order to that in which they were created. Often the first thing a person forgets is his profession, then with time they are left with only childhood memories. In Ricardo’s case, after forgetting how to swim and shave, he entered a period when he would collect and then hide small children’s combs around the house. He had aggressive spells, when he would bully his grandchildren by pinching their arms or tripping them up, like a child in a playground. At the age of 55 he no longer recognised Ofelia, but his face would light up upon seeing his granddaughter — the offspring of his only daughter, to whom she bore a strong resemblance. As the disease develops it infects the frontal cortex, causing hallucinations — commonly those of spousal infidelity—and the sufferer can fall victim to fits of jealousy and rage. Ofelia has experienced all of this.
Until quite recently, Ricardo could walk around the town and the family would take trips back to Yarumal, even though he no longer recognised his old friends. Then, a year ago, he wandered away from Ofelia and was knocked over by a car. “He got away from me for one minute. It was only a moment.”
Ricardo spent several months in hospital, lost use of his left side and has been bedridden ever since. Now 60, Ricardo lies in a bed in a room he shares with Ofelia, dressed in green tracksuit bottoms and a black vest. His teeth are gone but he chews incessantly.
“Ricardo’s decline didn’t come as a total shock to his friends and family. His father had succumbed in a similar way at the same age and, with time, four of his five siblings developed the disease.”
One thing that isn’t lost to Alzheimer’s is tactile perception. Ofelia frequently rubs Ricardo’s shoulders to calm him, but if she tries to change him or move his limbs, he acts aggressively, grabbing at her neck with his right arm. Doctors prescribed medicine to control the aggression, but Ricardo is now too weak to pose a threat and Ofelia stopped dosing him. Even without the medicine the economic cost to the family has been huge. Ofelia never worked, and on her husband’s state pension and the meagre takings from occasional shop work she must pay for 120 diapers a month, as well as a number of creams. But her children are supportive and chip in from their own salaries when the family pot runs low.
“Alzheimer’s can cause great suffering in a family, but especially so when the victim is young” says Lopera. “If a father falls sick at 45 it’s an economic disaster for the family. If it’s the mother, the nucleus of the family home, it can be just as bad. It’s always a tragedy. The effects can be just as severe on the carer. They have to give up their life project and this can lead to mental health issues, including depression.”
The University of Antioquia’s neuroscience department runs a “Care for the Carers” program, employing a number of psychologists to treat those individuals who may have been caring for a relative for several decades. But Ofelia cuts a determined figure. “I’ve never had any problems,” she says “this man worked his whole life for me and my family, I won’t abandon him now.”
Francisco Lopera grew up in Aragón, a small dairy farm village near Angostura, another Antioquian town with a high prevalence of early onset Alzheimer’s. Dressed in a colourful cardigan in a small office, the silver-haired Lopera recounts one of his earliest memories:
“I lived in Aragón until I was ten. It was a two street town of 500 people, and because it was the last stop on a small country road, I always imagined that I was living on the end of the tail of the world. Once a day a vehicle would come to the town, deliver the daily goods and take the milk to market. It was a religious town, and some time in the 1960s the villagers became convinced that the world was going to end with the coming of the new year. We stocked up on supplies and waited by the town’s radio for news of the end of the world. I was very afraid, but the one thing that calmed me was the thought that, living out here on the tail of the world, we would be the last place affected.”
Through DNA studies, researchers at the University of California have traced the origin of the E280A mutation back to a common ancestor, thought to be a Basque conquistador present in the region around 375 years previously, before Yarumal was founded. The rough terrain of the surrounding Andes, combined with the preference for large families, meant that the gene spread rapidly in the isolated towns of Northern Antioquia. Yarumal, however, was a focal point for the region.
“The agreeable climate, at a lower altitude than the surrounding dairy towns, allowed Yarumal to develop an agricultural industry,” says Restrepo. “The town produced maize, beans and vegetables, the staple of the paisa diet. It was also a dry port for traders passing north towards the coast and a regional market town. It was the first town to get motor vehicles, the gramophone and cinema. In the 1950s and 1960s if you lived in the region and wanted to go to the movies or dance tango, Yarumal was the place to go.”
Yarumal became the place where young couples met. “The families, particularly the rich ones, began to marry amongst themselves,” Restrepo continues. “My own great-grandparents were first cousins and my grandparents and parents too. Many families would have a dozen children and this helped spread the Paisa mutation amongst the community.”
But despite the growing numbers of locals falling victim to dementia in their early 40s, the problem was never correctly diagnosed, and in many cases, the victims became a source of shame for families. “No one from the local government did anything,” says Restrepo, “the families were left to care for the patients at home.”
The towns around Antioquia are renowned for their folklore centring on witchcraft and black magic. “People attributed the disease to a curse brought on by black magic for infidelities, or by a priest in a local village who had cursed his parish following a series of thefts from his church.”
When Lopera first set out to visit the families in the 1970s he found that many simply refused a consultation. “That surprised me. The families already knew that the dementia was incurable and they could see no point in meeting a doctor. I gave a presentation in a local community and a young girl, one of 12 siblings stood up and told me that, in her view, God had made things just so. Six of her brothers and sisters would fall sick, and the other six would care for them. I was struck by the fatalism of the situation.”
Surprisingly, the offspring of patients with the Mutation paisa do not appear to despair for their fate. According to Lopera there have been no instances of abortions for fear of passing on the gene and there have been no requests for euthanasia. “People understand now that it’s a question of chance. They know that 45 years is a life worth living, and who knows, perhaps by then there will be a cure.”
In a ground floor room at the University of Antioquia, the patients’ good spirits are in evidence. A dozen or so men and women in their 40s and 50s with early symptoms of memory loss are engrossed in a project to make small pouches containing scented herbs. They are joking and laughing and greet me warmly when I am introduced. The scheme, which involves making handicrafts and elements of music therapy, is designed to keep brains active and well trained. One of the women running the workshop remembers Ricardo as a man full of laughter and, of course, a passion for tango.
Such treatments can only slow down the onset of the disease. The hopes for finding a cure rest on diagnosing and treating Alzheimer’s before symptoms appear and damage has begun. In this respect, the carriers of the Paisa mutation gene represent a rare opportunity for doctors to experiment with new treatments.
“Traditionally we treat people when they are sick, but its too late,” says Lopera. “So far most studies into a treatment have begun when a patient is already sick. For early onset patients, Amyloids start to infect the brain at age 26, but they cannot be detected. By the time symptoms appear at around 34, the damage has already been done. What we’re doing is focusing on second stage prevention, treating patients who are outwardly healthy, but with early stage damage.”
Lopera’s plan is to provide patients with a high probability of contracting Alzheimer’s with an anti-amyloid injection which he believes may have the potential to prevent the build up of the amino acid on the brain. So far the challenge has been to gather the necessary sample size to conduct the trial. When news of the trial treatment was announced, thousands of individuals with Alzheimer’s in their family tree came forward. But the strict requirements for patients — they must have the Paisa mutation and be in their 20s — has prevented the group from being fully assembled, although Lopera says he expects to have completed it by the end of the year. The patients will then undergo five years of treatment with regular brain scans to identify the build-up of amyloids.
When The New York Times wrote a story on Yarumal in 2010, local and international media descended on the town. The story became exaggerated. Some reports claimed that half of all inhabitants over 45 suffered from the disease and parallels were drawn from a section of Colombia’s greatest literary work, Gabriel Garcia Marquez’s One Hundred Years of Solitude. In the book, the fictional town of Macondo is affected by a plague of insomnia. At first the residents are benefited by an extended waking life, but soon they begin to suffer universal amnesia. The parallels with La Bobera in Antioquia were too profound to ignore. Sitting in the coffeeshop in Yarumal, Restrepo offers warm greetings to dozens of people who pass by, some of them do indeed look disorientated, but it’s not Alzheimer’s affecting them, it’s the bottle.
“Alzheimer’s eats up memories in the reverse order to that in which they were created. Often the first thing a person forgets is their profession, then with time they are left with only childhood memories.”
“The biggest problems this town faces are alcoholism, unemployment and prostitution. If you were to talk to people on the street, they would probably be aware that the town has a number of Alzheimer’s sufferers, but only because they have seen the TV stations showing up to give interviews.” The citizens face more imminent threats. Yarumal was home to one of the most deadly paramilitary groups during the country’s civil war. A group of individuals known as the Twelve Apostles, made up of clergymen, police and businessmen, undertook a process of “social cleansing” that claimed the lives of dozens of individuals suspected of having links or sympathies with guerrilla groups.
The real lesson from One Hundred Years of Solitude may come from something as prosaic as an editorial decision. In the early 1980s new editions came with a family tree of the Buendia family conveniently printed in the first pages, after earlier readers found themselves overwhelmed with the novel’s frequent flashbacks and the repetition of names down the generations. For new readers, being able to trace the family tree made everything that much clearer.
“I doubt the Yarumal story is unique,” says Lopera “It’s quite possible that similar cases exist in other remote areas across the world. The first lesson we have learned is the importance of constructing genealogies of the extended families of all those affected with the Paisa mutation. This should be the first step taken, not just for Alzheimer’s but all diseases. As the world grows older Alzheimer’s could present a serious public health problem not just in the developed world but in the developing world too. If our trial is successful we might open a window: the possibility of a cure.”
To explore the subject of ageing we teamed up with The Powerful Now, an IDEO + SYPartners initiative poised to creatively redefine ageing as a path of continual growth instead of decline. Together we wanted to explore the ways in which health, money, work and communities will exist in our future, and initiate discussions to find radical new solutions.