Translated into English by Julio, with the help of his friend Matt
I have just turned 65 years old and I still can’t believe it, that I have reached this age. It is a miracle come true, because I was born with a progressive neuromuscular condition called spinal muscular atrophy, not in a developed country where people with severe disabilities have a system to help them deal with the increasing needs and secondary conditions caused by this devastating disease, but in Peru where severely disabled people are on their own.
In spite of these real differences, I was blessed by God because HE gave me an extraordinary mother who taught me to fight against adversity since I was a child, and helped me strengthen my spirit through her own living circumstances, which ended up being very important in this fight with SMA over the long run. Even, in her old age, my mother Julia, who had been fighting with Parkinson’s disease for more than a decade, broke her tibia, fibula and hip. At that time she was 82 years old but never surrendered. Instead she did all what she had to do to get up again and walk around the place we both lived by pushing a wooden chair until she got a walker. When she could not do this anymore, she used to wait for me, seated in her wooden chair, until I returned from work. Then she rose to her feet and with her walker she went to the kitchen to fetch me a cup of tea. She felt tremendously happy to help her youngest son in this way. What memories she left me! What lessons to apply to my own life!
She reunited with our Lord on 5 September 1998, but she continues living on in my mind and my heart, and I even see her in the eyes of my older brothers Miguel and Cesar, who still find time in spite of their own chronic health problems to visit me at the Ann Sullivan Center where I live, and in the eyes of my sister Rosa, who is dealing with a serious health problem. I still remember vividly when I was hospitalized in 1996, fighting for my life for 53 days in the ICU until I recovered miraculously. The love and faith of a mother, the support of brothers and the prayers of so many made it possible that God would grant me additional years in this life. All of who were my primary caregivers are no longer young and cannot take care of me anymore but I know that they will be with me to the end.
Here retelling these stories and occasions for thanksgiving, I recall Jeanie Schielfelbush, a young American who appeared at my door with parish priest Giuseppe Mizzoti of the Visitacion Church in 1987 with a project to provide free lunches for the poor children and elderly through the sponsorship system of the Christian Foundation for Children and Aging People. In that moment, when destiny was on my side, I wrote more than 100 introduction letters for children who were looking for sponsors in the United States. As a result of this providential visit, not only daily food has been provided to poor children of the Visitacion Church, but I was given the chance to meet Dr. Liliana Mayo, Executive Director of the Ann Sullivan Center; and in a short time, she gave me a job that changed my whole life, a job translating articles used for training parents and professionals who work with people with different abilities. I was also put in charge of statistical data of the Schools of Families and the Notes of Parents.
The Centro Ann Sullivan de Perú-CASP, the flagship of special education in Peru, as everyone knows, is an international center of training, education and research that educates people with different abilities, their families, and professionals working with them. This great institution that gives prestige to our country, and which I am greatly proud to be part of, has helped me through the years to get donated equipment that made my life a little easier as my neuromuscular condition progressed. They also kept the accessibility of the bathroom I used in mind in the old building and in the new one. Most important, when my mother was operated on emergency because her broken hip, or when she died, or when I was on the brink of death or when I almost lost my right vision in 2012 because of retinal detachment or when no taxi driver wanted to help me get into and out of the car and could not go to work or return home, CASP was behind me. Today, when I cannot return home daily after work but still need this work to continue to support myself, CASP helps relieve this stress by allowing me to stay in an apartment at the center during the week, giving me the ability to face other necessities.
So if I have lived more than expected with this kind of progressive condition in a country where welfare laws only exist on paper, it has been because the Lord gave me a supportive family and a place where the impossible is made possible to work and fight for my life.
Thanks to my mother, my brothers, the Visitation church, Liliana and Judith, CASP, and all those angels who came to my aid from different parts of the world and during different phases of my SMA in order to make me feel like I wasn’t alone in this fight. Thank you, my God, because without you nothing would have been possible.
Not long ago, in March of this year, I turned 64 years old, and what should have been a celebration of the miracle of reaching this age with a progressive neuromuscular condition called spinal muscular atrophy became instead a dichotomy between sadness and happiness. On the one hand, I felt the physical absence of my friend Gabriel, who died of cancer in February of this year. On the other hand, my friend Charly Castillo told some stories that made me laugh to death. The pleasant sentiment passed to me by my group of friends was evident, as well as the kindness of Daniel, who sacrificed his hours of sleep to be able to help me when the visit was over. And this whole feeling of happiness and camaraderie embodied in its own way my compadre Gabriel.
It was April 2, 1962. I had attended my first day of morning period class at the Hipólito Unanue High School, and I had to return to classes from 3 to 6 pm. That evening, my mother Julia accompanied me to the tram stop to take the tramcar that would take me to school about two kilometers away when we passed by the residential complex “16 de Julio.” Gabriel was talking with a group of kids our age (12 or 13) when I made out from the insignia he wore that we were from the same school. That single walk with my mother would transform over the years into one big story. According to Gabriel, who was smoking with his friends at the time (an adult activity not done by kids in those days), I was holding my mother’s hand when I passed by him (something done by sheltered kids in those days). It was not true, but year after year, Gabriel enriched this story when he talked about the beginning of our great friendship with friends of different circles. If Gabriel was still alive, he would say my mother carried me by the hand until my fifth year of secondary school. Such was his imagination, and he was a believer in his own version of events.
I was studying my first year of secondary school while he was in his second year. Gabriel had some friends who were two or three years older than us who studied with me in the first year and who knew Gabriel because he was always being punished with them after class for bad behavior. One day, they had told him to hit me on the way out of school. I had not been helping them during the exams, so I needed a lesson. I was short in stature, but I had experience on the street and many fights from primary school on my belt. By this time I was already falling because of SMA, but I accepted the fight on the way out of school, because this was the proper thing to do back then. Gabriel looked at Flores Velasco and Ramírez face to face, as well as another kid whose name I don’t recall, and said, “I will not fight. He is from my neighborhood.” Over the years, this is how my story went, and half joking half serious, I would say Gabriel was the first hired “thug” of the time. In 1963, when I was playing at recess at 10 am, these “three friends from 2F” fought with me. I defended myself as best I could, but their strength in numbers prevailed. I promised to beat the daylights out of them when I was older, but their bad marks caused them to be sent to another school. And I began to lose strength rapidly.
In 1964, when I passed to my third year and found myself with Gabriel, who was repeating the year, in Third A, we were already friends. He sat on the last row, while I sat on the first. That morning, we made the first negotiation of the many we would make in our lives. We compromised and chose the middle row.
One of those days in 1965, when the students decided not to go to classes in order to go see a James Bond premiere or just to wander around, Gabriel told me, “Shorty, when I get married and have a son whom I will call Gabriel Alonso, you will be my compadre” (that is to say, the godfather of his son). “Yeah, whatever,” I responded. Many years later, the son he imagined as a kid was not born. He instead had Rosángela, and even after many of his friends requested the honor of godfather, Gabriel kept his word. In this way, he became my compadre! A man of his word.
He was at the point of failing the year in 1965, because he took three courses during the break (called vacationals), among them biology, a course taken by fourth-year students. Flores Pezo, the extraordinary teacher of the course, always said, “Whoever does not pass the course in nine months will not pass it in the vacational.” Gabriel was the exception. His future compadre, the best student in the course, prepared him day and night, and he passed not only this course, but also the other two and graduated to fifth year with the rest of our friends. An achievement that made him proud and that he remembered his whole life.
Another story he always told over the years was about an oral exam Professor Flores Pezo gave the students in Fourth A. Then, everyone without exception was being failed by the professor, who had no better idea other than to call on me for an oral exam. I knew the most complex subjects of the course, but the simple parts were my Waterloo, and it was these parts he asked me about. My answer was not complete, and to show that he had no favorites, he gave me the worst grade I received as a secondary school student. Through the years, I nodded every time Gabriel told the story of how he passed and to top it off, how I failed that oral exam. Of course, he didn’t mention that I ultimately received the best grade in the course and was second on the honor roll. But it was his story, not mine.
I could tell many more stories of our time in secondary school, in which my compadre Gabriel was the main protagonist, but I will only mention that we would always play practical jokes on one another and sometimes end up in the middle of the road head-to-head with the wheel of a bus or injured in some bushes. These child’s games, or palomilladas as Gabriel called them, were no cause for laughter at the time, but through the years, they were the source of conversation and much happiness. Young people do a lot of crazy things, and my compadre was no exception. He lived up to the nickname Loco Muro we used for him.
Gabriel studied chemical engineering, and at university, we would get together only to attend gatherings with friends we had in common and to study strength of materials or differential calculus. All the while, my spinal muscular atrophy continued its relentless course, and soon I lost the ability to go anywhere independently or to get up off the ground when I fell. When it was no longer possible for me to attend university due to the progress of SMA, Gabriel, who lived at that time in the district of Rimac, would visit me on weekends. And he would visit more when he began working professionally for first-class chemical companies.
I attended parties with Gabriel. It did not matter if these were on the second or third floor. He would help me climb the stairs, and if I fell while dancing, he would immediately pick me up. He would accompany me home and lay me down, and then he would go back to his house. Until a year and a half before his death, if we both went out somewhere, he would go there and come back with me.
When I was carried to the Hospital 2 de Mayo, in 1972, due to a perforation from an ulcer, I decided then that I wanted to live and that SMA was not going to take me so easily. As always, my compadre Gabriel was the first to arrive at the hospital and extend his arm for the blood transfusion I really needed.
Gabriel finished his degree and worked for a prestigious brewing company in Peru, and he never forgot about his friend Julio. He regularly came to my house, where he was considered another member of the family.
A man as generous as Gabriel saw his luck change at the end of the 80s when middle-aged people were hardly ever being hired, while I was given the opportunity of my life by Dr. Liliana Mayo, Director of the Ann Sullivan Center, in 1987. Thanks to this spin of fate, I was able to see after my mother to the end of her days as I had promised her when I was a child, and I confronted SMA with better strategies and greater resolution.
My mother suffered a fall in 1994 and fractured her tibia, fibula and hip. Dr. Maceda performed an emergency operation on her, and her life was saved by an international campaign begun by Dr. Mayo and Dr. Leblanc, as well as friends of mine from England, Switzerland, Spain and Venezuela. Ten days after the surgical intervention, my mother Julia had a severe case of urinary retention and cardiac problems. Alone at home, I managed to call Gabriel and the doctor who had operated on her, who suggested I look for a doctor to treat the urinary retention while he looked for a cardiologist to give her emergency treatment. It was already morning when the danger disappeared completely. My mother Julia overcame the problems from her surgical intervention, and with the appropriate therapy, she stood and walked again with assistance across the ground full of holes in our neighborhood, which was not appropriate for her age (82) or her acquired disability. She lived four more years, and my friend Gabriel was at our side until her last days in 1998.
I also fractured my tibia and fibula in 1995 and spent three months with my left leg in a cast, which because of its weight and my atrophy made it impossible to stand. Even so, I did not stop working. In 1996, I had a bladder perforation and an enormous stone that should have gone down the intestines, but it went strangely into the pylorus, where it stuck. The doctors thought I had cancer.
I remember that night. I was at home feeling sick, and since I had been refused several times at the Hospital Almenara emergency room, I asked Gabriel to take me to Hospital Rebagliati, where we began to arrange my hospitalization, even though as a patient I did not belong to this general hospital. They did not accept me, but they sent me back to Almenara in an ambulance with documentation requesting my admission. Gabriel ran back and forth until dawn to do the analyses for me to be able to be hospitalized. Sixty days later, my life was saved miraculously, because the odds of me leaving alive had been against me.
Something similar happened in 1999, when my mother Julia had already died. I again overcame another problem with my bile duct thanks to the extraordinary expertise of Dr. Rae and his medical team. At that time, as before, thanks to God and the support of my siblings Miguel, César and Rosa and my friend Gabriel, life prevailed over death.
In this new century, I had to fight for a plot of land that belonged to me by law in the neighborhood where I was born. When my mother Julia was living, we had the right to a plot of 50 square meters. At her death, the right diminished, and I had to fight against this injustice. Thanks to God, reason prevailed, and I built the house I had promised my mother. As always, my unconditional friend came through to bring it to completion since my weakened body would not permit me to oversee the work myself. It took time, but for me who knew no impossibilities but rather persistence and relentless battling, the dream of having my own house became a reality.
A glance at my small room or the rest of the house is enough to recognize not only the strength of a man who did not surrender in the face of adversity, but also the incomparable help and the contribution of a great friend in the process of its completion.
I never remotely thought I would outlive my compadre Gabriel, who except for his controlled diabetes in the last few years, had always enjoyed good health. For this reason, when Gabriel visited me on January 9, 2012, and said he had stage four colon cancer and that he did not know if he could survive the illness, the news hit me hard. I knew in my heart that my compadre was going to go before me, since the colon cancer had spread to the liver and there was not much left to do.
Days later, I confronted another big problem. From one day to the next, I was losing the vision in my right eye, and it was necessary for me to have an emergency operation due to a retina detachment I had. CASP, where I work, the help of American friends and the expertise of Dr. Wong were God’s instruments in the recuperation of my lost vision.
As soon as I felt better, I called my compadre Gabriel, because it was my mission to help him on his way toward his final departure. I called him every weekend, and we would talk for a long time about all our school experiences. And as he became weaker, he consulted me on how to avoid bedsores that could develop by being seated a long time. So I recommended him water and gel cushions, urinal bottles and other such things that my experience with spinal muscular atrophy had taught me. He followed all the instructions I gave him, and he experienced in the flesh the muscular destruction that SMA had caused in me through the years. He more understood the compadre who could not go at his speed when I was 30 or 40 years old.
In December 2012, my compadre Gabriel came to tell me goodbye. He knew he did not have much time and asked me if I would make the effort to visit him, because he would not be able to come to me. I called him regularly on the phone, and I was able to visit him on January 30, 2013, when the holiday break was over. My compadre, faithful to his custom, said to me, “A beer, compadre, because you did not come here for nothing.” I saw him very weakened. He could barely move in his chair, and his swollen abdomen looked like that of a pregnant woman.
He was very swollen, but even so, he drank three beers with me and my assistant Domingo. He asked for two more for us, because that was the way my compadre was. He could no longer drink, but he did gladly eat the ham sandwich prepared by Domingo that he liked so much. Before I left promising to return, he said to me seriously, “Compadre Julio, do you think I will be alive for my birthday, you know, March 26?” I could not lie to him, because I had never done so, but neither could I tell him what I thought. I only managed to say to him, “Compadre Gabriel, the days you have left, whether few or many, spend them with your loved ones, my goddaughter Rosángela, your father Gabriel and your sister Leonor.” The next day, Leonor called me and asked me how my visit went. I told her Gabriel asked me if he would be alive by his birthday. She asked me the same thing, and although really his birthday was almost two months away and would be impossible for him to reach, I could not imagine and refused to accept that his end was a matter of days. The truth was my compadre had been waiting for his best friend to visit him to begin his final journey.
On February 3, Jorge Mandujano, a friend from the neighborhood and a former student at the Hipólito Unanue High School, where we studied, came to my house around 7 pm to give me the news: “Julio, Gabriel has left us. I am going to begin the burial process right away.” Gabriel was so organized that he had asked our friend Jorge to be in charge of the process at his passing and paid all the expenses beforehand. Such was my compadre: foresighted, sincere, loyal, honest, good at dancing (the best within 200 miles, he always said), and staunch defender of the weak in the face of injustices.
Several months have passed since his death, and even as my life changes from the progress of spinal muscular atrophy, I am adapting and continuing the tenacious fight. And this is possible because I had the luck of finding along my way people with a big and supportive heart like my compadre, friend and brother, Gabriel Muro Rodríguez.
I am not telling you goodbye, compadre, because I know one day we will see each other again. Until then, Gabriel, see you later.
Translation by Matt Watson, Julio’s friend also with SMA
*The great majority of people with severe disabilities are not employed and do not receive any financial assistance from the government.
The presentation of staff awards at the Ann Sullivan Center of Peru (CASP) for 2012 had come to an end, and while the staff left in a bus contracted by CASP, I stayed at Dr. Mayo’s house in La Punta, waiting for a taxi to come pick me up.
The summer vacation had begun that twenty-second of December for most of the staff, with the exception of some members of Administration and Maintenance. For me, a true challenge was underway. On returning home and contemplating the Administrative Efficiency Prize I had been awarded that evening, I asked myself who was going to take care of me during the vacation period from December 24 to January 31 of the following year. I have had spinal muscular atrophy since I was a boy, I live at home alone, and so my anxiety was valid.
Hiring caretakers from a specialized agency was not within my means financially, and as I saw it, I had no other option other than ask for additional help from Daniel and Domingo, the people who care for me for hours at a time and who also deserve recuperating rest from the exhausting work of assisting me.
My hands were very tired and weak due to the progress of my neuromuscular condition and the work I have done on the computer throughout the years. It was necessary to rest them with the hope of recuperating some of the lost strength, but as usual, I had two translations to do for staff training, due by my return to work on February 1. No doubt, other urgent jobs from our director, Dr. Mayo, would be arriving by email to be done immediately. The whole job entrusted to me was a challenge, because my right index finger, the only finger I write with, did not have any more strength left to push the keys. The moment also constituted an opportunity to test work strategies for when I can no longer type. My mind knew what to do, but the physical help from others was indispensable to be able to carry out my daily routine.
When our primary assistants are no longer in a condition to support us because of their advanced age or chronic illness, our life depends on human beings who help us do the things we cannot do on our own. That is, getting up, bathing, combing hair, getting dressed, going from the bed to the wheelchair, leaving the house, going to bed, etc. And if I have arrived at the age of 63 with SMA, it is because people with great sensitivity came into my life to accompany me on the difficult stretches of my long journey through life. And this last vacation was no exception.
Daniel: A 20-year old young man, who has supported me since May 2011, without even thinking it over said to me that I could count on him. These comments show the kind of person he is. Since the first day of vacation, Daniel typed what I dictated to him in Spanish from the English texts I had to translate. Before that, he would stand me up against the wall by propping a chair against my chest to keep me from falling — a miracle, because in theory, it is impossible for me to stand. In this way, my hands rested, pressure sores were avoided, and life was prolonged. When I could no longer support the weight of my body, Daniel would sit me on the bed, and I would correct the text I had dictated. Neither disability nor age differences are obstacles to forming a good friendship if people focus more on the human being than on limitation. Thanks, Daniel!
When we live in a world of four walls for most of our existence, we only observe the real world through a window, through television, like observers of a movie. However, there are human beings who bring a little bit of that world to us, and their visit comforts us. And just as if no difference existed, we interact with each other freely.
Jessica: Training area assistant at CASP. She had announced her visit on December 28, the equivalent to April Fool’s Day in Peru, but it was no joke. And I was not surprised either, because the year before, right at Christmas, as the world was celebrating the birth of the Child Jesus, she had the generosity to remember her friend who was confronting hard conditions in life by calling him on the telephone to convey her great optimism and vitality. Without forgetting she had put drops in my right eye after my surgical operations and complied with my other requests for help, that day at home, she listened attentively as I told her about my fight against adversity through the years, about my mother Julia, about my projects, and about the help I needed from her for 2013. She had lunch with me, and the hours she spent with me were pleasant and greatly appreciated. A week later, her first contribution for the year arrived in the mail. Thanks, Jessica, for being like you are. May God always guide your steps wherever life takes you.
Domingo: At the end of the year, this friend let me know that he would give me his support from January 1 through the rest of the month. He would come in the morning and Daniel at night. However, a health problem prevented him from helping me, and Daniel had to help me more in the day. Nonetheless, he was the one who carried me in my wheelchair to downtown Lima or to shop in the supermarkets of the capital city. His contribution was greatly appreciated.
Matt: A teacher at Mississippi State University who is also affected by spinal muscular atrophy, as well as his brother Blake. He has become a friend through cyberspace as well as tech adviser along with Blake. I consult them a lot on keyboards, on how to type with one hand either through an iPhone or an iPad. Together with their mom Clarissa, they supported me during my retina operation, and not losing my vision is something I will thank them for, as well as the other special people who intervened, for the rest of my life. The beginning of the New Year 2013 found me speaking with Matt through Skype about my projects, tasks to do and the way he could help me with them. He happily agreed! The friendship of all of these was a gift from God. Thanks, Matt, from the heart.
Roberto: The friend who came to put me to bed a little after 12 am on January 1, when I was speaking with Matt. The occasion gave rise to a long conversation between Roberto and I that lasted until 5 am. No one knows how God will answer our prayers until the opportunity presents itself. My friend was leaving on a trip back home in January, and so I was without his help on the weekends. However, God had another alternative for me.
Denisse: My friend from CASP, the kind of person God sends to make our lives easier, made her first visit of the New Year. We have known each other since 1987. And since then, she has visited me many times in my life — when my mother Julia was still alive, when she passed away and Denisse made a small economic contribution I will never forget, when I stayed at my sister Rosa’s house for ten years, when I returned to my neighborhood for the final fight. And after her last visit, she had the generosity to comment to other friends on the way to imitate her actions: “Our friend Julio — it is always an inspiration to see him and talk with him, and above all, the good it does him to receive visits you wouldn’t know. You will leave super recharged.” In short, a friend like none other.
My friend Roberto had already left on his trip with his family, and I had not found anyone to help me go to bed on Fridays and Saturdays or to get me up on Sundays and go to bed on Sunday nights. I ran the risk of having to spend all night in a seated position. But not so.
Javier: Roberto’s nephew came to my house on January 10, and he immediately made himself available to help me on weekends. Daniel, who has become a teacher by experience, taught him how to sit me on the bed, lay my body flat, take off my clothes and use his arm to situate me on the pillow where I lay on my side to prevent sores. This intensive course made him see how my body falls over in the bed and how severe my condition is. Far from being scared off, he put more attention and care into managing me. Through his sensitivity, and initiative, he gets me up early in the morning before going to work and sits me in my chair, and I wait for Daniel in a comfortable position. Thank God!
Emails between Lima and Kansas intensified, and new tasks arrived from CASP. It seemed that I was at work like normal, but no, I was at home. Thank God Matt was only one click away. After some coordination, some jobs were sent to him, while I did the urgent and short-term translations. Thanks to this, tasks from Autism Speak, International Ponseti Association, USAID, Panama and other translations on leadership and problem-based learning were completed.
Sunday, January 27: From Kansas, Dr. Liliana Mayo responded to me, “Julio, you are an angel. The completed tasks have arrived just in time because we (American consultants and I) are going to meet together in a half hour…thanks a lot.”
The objective was complete! I had not had the rest I needed, but working for people with different abilities and the professionals who work with them comforts the soul and makes us feel like our goal in life is being fulfilled. Thanks, Matt, for helping me make it possible.
Personally, when the benefits of people with disabilities only exist on paper, you have to appeal to the world when necessary to keep working and living. And the world responds to just causes:
Brenna C.Randal, one of the members of the Kansas group who visited me last year, wrote to me “I have talked with Enriquez (from Dragon Naturally Speaking) about the Dragon Speak program–weighing/balancing the pros and cons of the beginning and more sophisticated software. I hope that what we chose will support you in your translations. Have a desk top microphone to accompany”
Jeff Seitz, who donated me an electric chair in 2004, told me, “Julio, I am going to get to work on sending the chair to you. I will fill out the forms and stay in communication with you. My first step will be to get the shipping arrangements made and then send it on its voyage.”
From Matt: “We have purchased the iPad Mini for you and will get it to you ASAP. This should offer you a variety of ways to type that will make your job easier and faster. As someone who has similar loss of hand strength, I cannot imagine how you use a traditional keyboard. I depend on alternative ways of typing that have greatly improved my productivity, and I hope this iPad will help you in this regard.”
There are still many challenges to overcome day to day, but the vacation ended up being better than imagined.
Animal studies suggest that spinal muscular atrophy may result primarily from motor circuit dysfunction, not motor neuron or muscle cell dysfunction, as is commonly thought
Results from a study in fruit flies conducted by scientists in the Motor Neuron Center at Columbia University Medical Center in New York suggest that spinal muscular atrophy (SMA) — commonly thought to be a disease of muscle-controlling nerve cells calledmotor neurons — instead results from the dysfunction of motor circuits (networks made up of different types of specialized neurons that coordinate muscle movement).
A phase 2-3 clinical trial based on the findings is testing whether an existing drug called dalfampridine can improve walking ability and endurance in adults with type 3 SMA. (Dalfampridine is marketed under the name Ampyra for treatment of multiple sclerosis.)
In a second study, researchers identified the molecular pathway in SMA that leads to problems with motor function.
The findings could point the way to new therapeutic strategies for SMA.
From Lima sin barreras
Press release # 11
Lima, August 27, 2012
Original in Spanish
The vicious cycle of poverty plus having a disability and even more poverty is the common denominator of the stories of people with disabilities who the state and the society do not include. Julio Chojeda Torres, 63, has worked as a translator at the Ann Sullivan Center in San Miguel for 25 years. Like all adults, he has a work schedule of eight hours a day. He lives in Cárcamo, in the Lima District, close to the malls of Las Malvinas and among marginalization and delinquency. The only family he has is his two elderly brothers of 76 and 79 years of age. He was close to losing his house when Cárcamo was a slum and the housing structures had no title deeds.
Chojeda was diagnosed with spinal muscular atrophy at age 17. Spinal muscular atrophy is a hereditary degenerative illness that causes muscle damage and weakness. Approximately four in 100,000 people have this condition.
Because of his condition, Julio became an English translator. To learn more about his condition, he began writing letters to various U.S. institutions and specialized medical magazines. He knocked on embassy doors and wrote letters requesting donations. This way, he learned English.
What does it mean to live with a progressive neuromuscular illness like spinal muscular atrophy (SMA), muscular dystrophy (MD) and amyotrophic lateral sclerosis (ALS)? Monday through Friday, a vehicle from the Ann Sullivan center picks up Julio at his house and takes him to work. If it weren’t for this, he would have to pay between 15 and 20 sols for a taxi to his job. He would spend 40 sols daily. “Sometimes, taxi drivers do not want to carry you, or they charge you more because they say they lose time,” Julio said. “You find many looks and attitudes of rejection in the street.”
“What happens when you live in a poor neighborhood, when you have a large family and the children do not get an adequate diet with enough protein? That child will live in a cycle of poverty. But if you add to that the variable of disability — disability impoverishes families,” Julio said.
For example, the electric wheelchair he uses at work was donated, as well as his walker. The price for this type of wheelchair, which dates from ’97, is 8,000 U.S. dollars. The appropriate creams he uses to avoid skin wounds costs another 70 to 80 sols. “Could a man with a minimum wage of 750 sols afford this?” Julio asked.
“We people with disabilities do not have physical barriers only. We do not have access to education, nor do we have the same rights. If we do not have the same rights, how can we compete as equals? In these conditions, we are not going to be able to have a job, and without a job, we are not going to be able to earn money and cover our basic needs, which are many due to our disabilities. We want to have the same rights and the same opportunities,” he added.
“I do not have medical insurance for my condition. The insurance from the state, as everyone knows, leaves us aside. Our system has not gotten better. If someone with this type of disability were to be born right now, living conditions will not be any better for them. Each individual can only depend on themselves to face it,” he said.
Dr. Carlos Wong, who saved me from blindness in January, examined my operated right eye on May 4th and said, “Julio, you are ready for the intraocular lens implant. Your retina is completely reattached. Go to the information desk and find out on the cost of the surgical operation, and date for it.”
Taking into account the scheduled tasks I had do for CASP in May and June, and my personal needs, we scheduled the operation for June 4th. Once again Dr. Mayo gave her approval and CASP paid for it while funds were gathered to cover this expense.
Paul and Ginger Marto, my sponsors via the project between the Kansas-based CFC and the Visitacion Church in Lima, who had helped me in many ways through the years, let us know that their contribution was on its way. This nice gesture made me feel blessed and more grateful.
Domingo, one of the young men who cared for me during the post-operation recovery period of the retina surgery, came for me early in the morning of June 4th to take me to the Sacro Coeur Eye Institute, where I was waited at 8:00 am. One hour later, I was taken to the room next to the operating room, where my blood pressure was measured, and they inserted a thin tube in my vein to administer a saline solution plus anesthetic. When the surgery of the first patient ended, I was taken to the operating room seated on a mobile operating chair.
In there, Dr. Manuel Perez Martinot said to me, “Julio, if you feel some discomfort during the intervention, please tell me.” A nurse on my left side checked my blood pressure, and another one put an oxygen tube into my nostrils. While the lens was being implanted, I thought positive by evoking the words of encouragement I had received from friends. Clear into my mind came words such as “You are in the prayers of many more folks than you know”, “You are in my prayers this day”, “We are praying for you and will be sending positive, healing energy to you.” “Our prayers are with you always, and we will be praying especially for a successful surgery on Monday. God bless you, your surgeons and the whole Ann Sullivan staff and community”, “You are in the best of hands. I am sure your surgery will be a success”, “You are in my prayers, Julio”, “Ya sabes van mis oraciones y energía para ti todo saldrá espectacular (You know, my prayers and energy go to you. Everything will turn out well)”, “We will be praying up here in Canada for you”, “Julio your will and determination are strong and along with the really good surgeon who will be performing surgery, you will do great”, “You are in my prayers. I hope, God willing, you will gain as much of your sight back as possible”, “Todo va salir bien don julio, estaremos orando (Everything will turn out well, Mr. Julio, we will be praying for you)”, “I wish you a successful procedure, improved sight and a quick recovery tomorrow”….
I was still immersed in my thoughts when I heard Dr. Martinot saying, “It is over.” Then they put a bandage on my right eye, and I was taken back to the waiting room where I was given a pill for pain and rested for a while. There I realized that I could look with the operated eye and that shapes were more clearly defined, and I felt really great!
At one o’clock I was at home encouraging my 79-year-old brother Miguel to stand and walk around the room. The next day, Mother’s Day, he had been operated on the left knee and could still not move his left leg when he was discharged from the hospital two weeks later because his left leg was very swollen and he experienced great pain. The previous week of my operation I called Martin Avilez, the physical therapist of CASP, who keeps me functional in spite of the severity of my spinal muscular atrophy, and asked him to help my brother Miguel fight back his depression so he can beat his fear and walk again.
One week later, Dr. Mariella Navarro said that my right eye had no infection or inflammation, that the IOL was firmly in place, and that I could get back to work on June 19th.
Miguel was taken to his home (on a fourth-floor apartment) to celebrate Father’s Day with his children. By then he was more independent and able to go around my neighborhood by pushing his walker. One day before, knowing that I had no caregiver service during the night on weekends, he woke at 3:00 pm and entered my room to ask me if I needed help. His nice gesture moved me to tears because not only did I need help, but because I remembered how this generous man went across the city of Lima at night in 2006 to help his younger brother get into the bed and get me ready at six o’clock the next morning to go to work. My sister Rosa then could not help me physically any more, and he and Cesar, my second older brother, took turns to help me get into and out of the bed until a caregiver was found. Now I live on my own but all of them including my deceased mother Julia made up a formidable team that helped me live beyond expected. Now my will power, and CASP, is the driving force to get ahead.
On June 19th, I was back at my job. Staff of the training area where I work, who had followed all the process of my two operations, welcomed me and asked me if I could see them. Yes, I could. Seated at my Mac I could see them on the right side; and from a standing position, when Martin helped me up to release the pressure from my buttocks, I could look out in all directions.
God had made it possible that I regained much of the vision I had lost, when the retina was detached, and that light prevailed over dark, thanks to the request of many people who prayed for my health. Thanks from the heart.
My gratitude is extensive to Dr. Liliana Mayo and the Centro Ann Sullivan; Dr. Manuel Pérez Martinot and Dr. Carlos Wong who made the miracle, the staff at the Sacro Cuore Clinic that was very nice to me; Dr. Linda Lawrence whose help was crucial, Ana Mayo who helped his Peruvian friend from Spain, the Watson family who knows what it is like dealing with SMA, Paul and Ginger Marto, and the unknown donor who helped to save my retina.
Thanks also go to my brothers for being with me on this long struggle, Daniel and Domingo who took care of me, and friends who sent me their best wishes.
A new year always brings new challenges to people with spinal muscular atrophy, and 2012 has been no exception.
On January 9th, I went with my partial caregiver Daniel to the Centro Ann Sullivan del Perú (CASP), a school for people with developmental disorders where I work, to charge the 7-year-old gel batteries of my 1997-made Invacare wheelchair because I was afraid the old chair would stop working if the battery terminals crossed. He plugged the charger cord to the chair first, then into the wall outlet, and I asked the administrative staff working in the training area office to disconnect it from the socket wall when the orange light stopped blinking. On February 1st, I had to be back at work because my holidays ended by then, but destiny had another thing in store for me.
At 11 o’clock in the morning, on January 12th, I was in the Solidarity hospital being diagnosed with retina detachment and was told that I needed a complex, expensive and urgent operation. For a man with spinal muscular atrophy whose only income to survive comes from his wage, the surgery cost was out of reach. However, I was willing to fight for my vision.
Back at home, two hours later, I made a call to Denisse Ramos, a social worker at CASP, and asked her to inform Dr. Liliana Mayo, our General Director, then in Kansas, about my health problem. Later when the effect of dilation drops had gone away, I sent a letter in English to Liliana telling the symptoms I had experienced in the previous days and what I was told in Solidarity, which she forwarded to Dr. Linda Lawrence, ophthalmologist in Salina, Ks, to get her opinion.
At 6 o’clock in the afternoon, after watching the sunset from the main avenue of the neighborhood where I live, I returned home and found 16 missing calls on my cell phone screen. Right away I called Dr. Rosa Oyama, project and research director at CASP, who informed me that I had a retina exam at the Sacro Cuore Clinic the next day, Friday 13th, and that then I would be given a date for my surgery.
At night, reading my copy of mails addressed to other people I learned how my help request had gathered speed since I made my first call. Dr. Mayo had written to Dr. Manuel Pérez Martinot (Director of the Sacro Cuore Eye Institute) as follows: “I am writing you because I would like you to give an appointment for Julio in your eye institute. He is a 62 year-old coworker who fights courageously against the Spinal Muscular Atrophy and we want to know your professional opinion about his retina detachment and see how we can help him,” while Dr. Lawrence wrote to Liliana, “I send to Manolo’s new email and texted him. Someone should call him first thing tomorrow. Dr Wong, the retinal surgeon I believe is there on Friday. Hopefully either he or Manolo could see Julio. We pray that all will be okay.”
On Friday, prayers were being answered. Dr. Carlos Wong, one of the best experts at retina in Peru, examined my right eye exhaustively and confirmed the Solidarity’s diagnosis. Then Dr. Pérez Martinot asked him to schedule me for surgery the next Thursday, January 19th, and instructed his administration to charge me as a social case. As a result, the cost of retinal surgery was cut by 50%. At night, Dr. Mayo gave us (Denisse and me) the green light for the operation, because she had gotten a donor to fund it. The following morning, Saturday, January 14th, health workers from the Centenary Peruvian –Japanese Clinic (such as had happened in Solidarity Hospital) were helping to have ready the pre-operative tests in record time. I only told them that I had SMA, that I was likely the only one with this condition in Peru working full time and didn’t want to lose my sight because I needed to continue earning my life. God did the rest.
The night before the operation, reading mails from friends who sent their thoughts and healing energy to me, who prayed for a perfect outcome, who sent me hugs and their hands to hold my hand as I went through the surgery process; friends of organizations who had called me our voice to Spanish-speaking people and said that I had touched people well beyond my immediate surroundings and a lot of supportive energy would be focused on me as I embraced this new challenge or that they would pray for God’s guidance of the surgeon’s hands and also for my speedy recovery; minutes before I turned the computer off, I received the last one coming from Mississippi. It was from my SMA friends Blake and Matt, who putting themselves in my shoes and knowing how I fought courageously against this deteriorating disorder in a country where severely disabled people are on our own, said to me that help from their family was on the way. At 11 pm I went to bed hopefully optimistic, because the fear of how I was going to pay for the around-the-clock caregivers I needed, medicines and other operation-related expenses and whether the post-operative period would take more than I had supposed began vanishing.
On operation day, at 2 o’clock in the afternoon, the skilled hands of Dr. Wong started repairing my detached retina by using a combination of the Retinopexy and Vitrectomy procedures. So he removed the entire crystalline lens and all the gel, inserted a band to flatten out the retina and used gas to make the retina stick. Three hours later, the surgery ended and the performance was described as brilliant according to a medical report.
Living on my own in a 50 square meter house and needing a more spacious place to be cared for by caregivers and visited by my brothers Miguel and César, both of whom are nearing eighty years of age, Dr. Mayo’s invitation to go to the center to spend the post-operative phase was like a gift sent from heaven, which I accepted immediately. So there I went with Domingo, Martin and Daniel who care for me the whole day, especially the first three weeks because I had to sleep on my stomach and/or keep my head down for the gas to make the retina stick. It was not easy or comfortable, but they helped me make it through.
On March 5, I celebrated my 63rd birthday, and this special day found me as usual working in the training area of the Ann Sullivan Center. During my checkup on March 9th, Dr. Wong examined my attached retina and said to me, “Julio, congratulations (and congratulations also to him), the retina will last more than you can live (2025 in my mind) and your vision will improve when the intraocular len will be placed. Perhaps, on April 20th, we will fix the date.” God through good-hearted people made this miracle possible! God bless them always!
My gratitude is extensive to Dr. Liliana Mayo and the Centro Ann Sullivan; Dr. Manuel Pérez Martinot, Dr. Carlos Wong who made the miracle, and the staff at the Sacro Cuore Clinic that was very nice to me; Dr. Linda Lawrence whose help was crucial, Ana Mayo from Spain who did her bit and the Watson family who tipped the scales in my favor in this fight.
Thanks also go to my brothers for being with me on this long struggle, friends who sent me their best wishes and Mrs. Chantal Fort de Peschiera who kept friends updated about my operation and recovery process.
California Stem Cell, Inc. and Families of Spinal Muscular Atrophy Announce Completion of Pre-IND Meeting with FDA for Stem Cell-Derived Therapy for the Treatment of Spinal Muscular Atrophy Type I.
October 14, 2009.
Significant Progress made Towards Moving Novel Therapy into Human Clinical Trials.
California Stem Cell, Inc. (CSC) and Families of Spinal Muscular Atrophy (FSMA) announced today that they have completed a formal pre-Investigational New Drug (Pre-IND) meeting with the Food and Drug Administration (FDA) for guidance on the clinical and regulatory pathway and requirements for submission of an IND to initiate human trials for a stem cell-derived motor neuron replacement therapy for Spinal Muscular Atrophy (SMA) Type I.
SMA is the leading genetic cause of death of infants. It is a disorder that results from a chronic deficiency in the production of the SMN protein, which is essential to the proper functioning of the motor neurons in the spinal cord. SMA is typically marked by the deterioration of the muscles that control crawling, walking, swallowing and breathing. There are no approved therapies for the treatment of SMA. Approximately 1 in every 6000 babies born is affected. 1 in 40 people are genetic carriers, indicating approximately 7.5 million carriers in the United States.
CSC, a leading stem cell therapeutics company, has developed a stem cell-derived motor neuron replacement product, for the treatment of SMA Type I. Pre-clinical studies, completed in collaboration with professor Hans Keirstead of the University of California, Irvine, have shown clinical proof of concept through the demonstration of functional benefit in animal models treated with CSC’s motor neuron replacement product, MotorGraftTM.
MotorGraftTM has the potential to provide benefit for SMA by two mechanisms: 1) direct replacement of the motor neurons lost during the disease course, resulting in new muscle innervation, and 2) providing a nursing support function to remaining motor neurons.
“We are greatly encouraged by the feedback we received from the FDA” said CSC COO Chris Airriess, “It will greatly assist us in preparation of our final IND application. Completion of this key milestone is an important step towards moving our SMA program to the clinic.
“We are very pleased with the outcome of the Pre-IND interaction with the FDA” said Kenneth Hobby, President FSMA. “FSMA has invested almost $2 Million over the last 8 years to develop this motor neuron replacement therapy for SMA. This meeting was a significant accomplishment towards the filing of the IND and ultimately bringing this therapy to patients
Project Cure SMA Group Publishes Open Label Valproic Acid Trial Results. The paper entitled “Phase II Open Label Study of Valproic Acid (VPA) in Spinal Muscular Atrophy” was published today in the online Journal PLoS ONE. This clinical trial was fully funded by Families of SMA.
The paper presents the data from an open label trial of VPA in 42 subjects with SMA to assess safety and explore potential outcome measures to help guide design of future clinical trials. The results indicated that VPA was well-tolerated and without evident hepatotoxicity. Carnitine depletion was frequent, and temporarily associated with increased weakness in two subjects, indicating a need for co-administration of carnitine with VPA. Clear decline in motor function occurred in several subjects in association with weight gain. Mean fat mass increased without a corresponding increase in lean mass, suggesting that weight gain is likely to be significant confounding factor in future VPA clinical trials. A significant improvement in motor function, as measured by the Modified Hammersmith Functional Motor Scale (MHFMS), was observed in participants younger than 5 years of age.
The authors concluded that the study provides good evidence that VPA can be used safely in SMA subjects over 2 years of age in the setting of close monitoring of carnitine status. However, they also indicated that further studies of VPA in infants and young children are needed to better assess safety in this more vulnerable cohort, since children under 2 were not included in the current study.
The results presented within the PLoS ONE publication suggest that while there may be a potential treatment benefit in a subset of younger non-ambulatory type II children, conversely, older subjects may be at risk due to excessive weight gain. Given the uncontrolled nature of the study, it is unclear whether the improvement in some younger subjects reflects a therapeutic drug effect, maturation, or increased cooperation leading to improved functional measurement scores. These data underline the importance of randomized, controlled efficacy studies to assess the impact of therapies in SMA, and that the same drug could have differing results in select subsets of patients.
“These results are significant not only because they are a first step to determining the therapeutic benefit of VPA for SMA, but also because they inform us about proper clinical trial design. These results demonstrate the need for randomized controlled trials in subsets of patients, since much weaker (Type I) and much stronger (Type III) patients may respond quite differently to the same intervention. In addition, the clinical outcome measures used to examine patients over such a wide range of strength and function will differ. Using the correct measure for each population will be critical to prove efficacy”, says Dr. Kathryn Swoboda, lead author on this paper.
The data presented in the paper indicates that several additional clinical studies are warranted in order to assess the efficacy of VPA for SMA. These studies, funded by Families of SMA, include:
1) A double-blind placebo controlled trial of VPA and carnitine in a non-ambulatory group of Type II children.
Click here to see preliminary results of a trial in this group called CARNIVAL.
2) An open label safety study in Type I infants, an extremely vulnerable group of patients.
Click here for details of an ongoing trial in this group called CARNIVAL TYPE I.
3) A double-blind placebo controlled crossover study in ambulatory adults with SMA.
Click here for details of an ongoing trial in this group called VALIANT.
“Families of SMA is pleased to have the first clinical trial results published from the work of the Project Cure SMA Clinical Trial Network. Funding clinical trial initiatives allows our community to achieve multiple goals. It gives us the means to develop the required outcome measures to test drugs in all SMA populations, to conduct trials to test repurposed drugs for safety and efficacy in SMA patients, and to build the necessary infrastructure, including adequate regional clinical trial site representation across the US for future new drug trials”, says Kenneth Hobby, Executive Director of Families of SMA.
In 2001, Families of Spinal Muscular Atrophy established and single-handedly funded a clinical trials network called Project Cure SMA. This network has conducted natural history studies that increase our understanding of Spinal Muscular Atrophy disease progression, built models for designing SMA clinical trials, and now runs clinical trials with existing drugs.
Families of SMA’s investment of over $6 Million to date in five multi-center clinical trials is helping to test existing drugs that may lead to a treatment for Spinal Muscular Atrophy. In addition, as novel drugs currently being designed for SMA become available, having a fully operational clinical network with a sufficient number of sites to conduct pivotal SMA drug trials will help attract and encourage biotech and pharmaceutical companies to invest in SMA drug development.
From Access Press (Minnesota’s Disability Community Center)
May 10. 2009
Living with muscular atrophy in Peru
Interview Dr. Liliana Mayo of Peru
by Kelly Lee, Advocating Change Together
My name is Dr. Liliana Mayo, Director of the Centro Ann Sullivan del Peru (CASP), and I would like to tell you about Julio, a person with spinal muscular atrophy who has been working as part of my team since 1987 and is 60 years old.
When I met Julio, he lived in extreme poverty with his eldery mother in a 16-square-meter house made from adobe that it had not running water or toilet, and not much to eat.
In a time quite different from the present time, when his condition was diagnosed, he only was told, “Julio, I am sorry but your disease had no cure.” Even some physicians who had examined him predicted that he would not live very long. Julio understood how hard his life would be if he fought this battle on his own. That is, without means and without support from the government. So he taught himself the English language to communicate with other people like him and institutions around the world conducting research about SMA, and he learned through specialized magazines that people with severe disabilities were joining the work force in developed countries with the help of computers, and that adaptive technology was making their lives easier. For a heart that knows no bounds, he had to make connections and get additional information so he could gain access to available technology in Peru.
Julio has learned from the persistence that faith moves mountains, so he found people who helped him to get the basic things he needed to work and make a difference in his life. In time, God answered his prayers, and he could get a second- hand battery-powered wheelchair, a computer with wireless keyboard, and an Internet connection to do additional work at home to pay for his
part-time caregiver, and to help other disabled people less fortunate than him.
Julio talks about how important it is for people with disabilities to have a job and to feel needed by other people.
Julio works as our translator and is in charge of translating materials for professionals and families of people with different abilities, as well as of entering data about grades and attendance of parents at training meetings. Julio is my friend! He has turned 60 years old and is the only man with SMA in Peru who has reached this age working full time.
Julio shows us what a person with a severe disability in Peru can do while working and helping other people to fight for their rights, persist and knock on doors to get what he needs in this stage of his disease and never, never take no for an answer.