Found this it was called (The First Survivors )
Being Born unable to Swallow
A Doctor Remembers…Robert Linsig by George Humphreys, M.D.
(Ed. Note: Dr. Humphreys is now in his upper nineties and still as sharp as a whip. We were very excited to have his account of this story, as I am sure you will be, too.) He writes, “I am delighted that you were able to get in touch with Mr. Linsig and wonder if you could get him to write his memory of his childhood difficulties, as well as confirming the fact that for the past 45 years he has had no trouble eating. That alone should encourage parents whose children may not yet have reached that stage. I thought his story might be interesting reading for other parents of such children to learn that the solutions to these children’s’ problems took time and patience on the part of both the parents and the doctor. Robert Linsig is the first person born with atresia of the esophagus to survive into adult life. He is now sixty-one years old. I first saw him in 1942 when he was brought to me at the age of seven. Bobby Linsig’s story, as I remember it, is as follows…”
He was born in 1935 in Warwick, New York. He was a vigorous baby, born at full term. Nothing abnormal was noted at birth, but it soon became evident that he promptly regurgitated everything he swallowed. Within a week of his birth, a temporary gastrostomy was done, and feedings were begun with no difficulties. As he continued to tolerate gastrostomy feedings and regurgitate everything swallowed, his doctors decided to convert the temporary gastrostomy to a permanent one. A flap of the anterior gastric wall was used to form a tube placed through the abdominal wall at an angle so that feedings could be given by inserting a rubber tube through the opening, then withdrawing it until the next feeding without having gastric contents escape during the interval. This method of feeding was successful. As he grew, however, his mother became aware that when hungry, he often took food, put it in his mouth and chewed it up and swallowed it, only to regurgitate it a few minutes later.
This is very long you can read it here
Millicent Lesley Collins
I was born Millicent Lesley Collins to an Italian-Irish-Polish couple in Providence, Rhode Island. Even at best this young couple did not get along very well. Soon after my birth, it was discovered that I had a birth defect. I could not swallow. The top part of the esophagus was missing. The esophagus is the tube that carries the food from the throat to the stomach. And so I was flown to Boston Children’s Hospital where I became a resident of the hospital for the first four years of my life. During those four years, an artificial esophagus was created and perfected. This esophagus was made from my own skin through skin grafting. The skin-tube was placed on the outside of my chest. At the end of the four years, it was decided that I should be exposed to a home-like atmosphere, but I could not return to Rhode Island.
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I was both stunned and exhilarated to get your phone call the other evening and to hear you had been trying to locate me for so long. I realized I had been trying to stay obscure but did not know I had done such a good job at it! You mentioned other health problems I may have, and they are numerous. I have a hiatal hernia, a heart murmur, scoliosis, things out of place inside of me, and an uneven number of ribs, plus carpal tunnel syndrome, duodenal ulcers, depression, neurological problems, and arthritis. I am so glad there is a newsletter now for people who have children with these birth defects. It will be such a help and comfort for them to be able to read about others who are having the same problems as they are. I am also extremely interested in the cause of these birth defects and of all the other illnesses as well. If there is any way that I may be of help to you regarding my own birth defect, and research going on now, please let me know.
You asked me to write briefly about what my life has been like up until now living with TEF. I will try to start back as far as I can remember. It’s in bits and pieces. First, I want to say how very fortunate I was to have had Dr. Cameron Haight, along with Dr. Towsley, thoracic surgeons at Ann Arbor University Hospital, as my doctors.
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Keith Edward Dochterman, born at Rocky Mountain Hospital, Colorado, on December 27, 1956, at 8:16 p.m., was transferred to Children’s Hospital on January 2, 1957, is nothing more than a diaper and a t-shirt. Mom and Dad used a blanket to wrap me up in, and Mom said I slept all the way there. That was a good thing because I had a hard time breathing. That day was the first time my dad held me.
January 3, at 10 a.m., Dr. Marianna Gardner called my mom and told her that she had figured out what was the matter with me. She also said I was going to be operated on at 1 p.m. My mom found my dad on his milk route (he worked for Borden’s Milk), and they went to meet Dr. Ackers at the hospital. He told my parents that we didn’t have a 50\50 chance, but that he would do the best he could. When he came out of surgery, the anesthesiologist was with him. I had made it, and my parents kept thanking him over and over. Dr. Ackers told my parents that this was the man, the anesthesiologist, they needed to thank because he had kept me alive by breathing for me with (bubble).
Dr. Ackers told my parents that he had put a plastic tube down through my esophagus. He said that one side was about 1\8 of an inch thick, and the other side you could see through, so it was like sewing wet tissue paper together and trying to make it hold. When they took that tube out of me, that was going to be the end result.
My mom and dad always told me that I was Dr. Acker’s pride and joy while I was in the hospital. My mom said that every time they would go to the hospital to see me, Dr. Ackers would be carrying me down the hallway.
I came home from the hospital for the first time when I was one month old. I was put under the care of the woman who diagnosed me. She was regarded as an excellent Pediatrician. Her name was Dr. Marianna Gardner. She was a very tall woman with a very raspy voice. When I would hear her coming down the hall, she would scare me! But apparently, she took very good care of me.
When I was a young child, Dr. Gardner passed away, and from that point, I was under the care of Dr. Edwin Williams.
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The site this came from
May 11, 1990, our daughter Jaclyn was born. Though the delivery was without complications for Terri, when her water broke, there was an awful lot of amniotic fluid. Our friend and nurse midwife, Joyce commented our daughter came out with a limp body and very blue, cyanotic. There was no movement. When Joyce suctioned our daughter’s mouth, she took a deep breath, cried, and went limp again. Joyce became very concerned. She commented how small our daughter was. Joyce said, “Terri, do you think you could have twins”? She did another manual exam and said, “Terri, I thought you might have had twins, but I don’t feel another baby.” Again, our daughter was very limp, and Joyce was extremely concerned. She said, “I don’t know what is wrong. The baby doesn’t move, and she is just so small.” Joyce put a catheter down her throat and suctioned. Our daughter became agitated, cried, and started to move. She went limp shortly after and Joyce was now extremely nervous. Her words were, “Terri, I just don’t know what’s the matter.” She has all her fingers, toes, arms, legs, eyes, mouth. Everything physical was fine. Joyce said, “Terri, do you want me to baptize your baby”? Terri was still trying to recover from the delivery. Terri said, “What do you mean?” Joyce said, “Terri, I don’t know if your baby is going to live. I just don’t know what is the matter. She is so small and blue and won’t move.” My wife turned to me, and I said to my wife, “Terri, Joyce wants to baptize our baby because she may die.” My wife said, “Yes.” Joyce took the water in her hand and placed her fingers on our baby’s forehead; she said, “In the name of the Father, the Son, and the Holy Spirit…” I said to Joyce, “Don’t worry, If our baby dies, that’s another part of life. We live and we die.” Joyce now had asked her assistant to call the ambulance. I was transported with my daughter to a hospital in Washington D.C. I spent the night suctioning my daughter in the Neonatal Unit. All night it was my daughter and myself. It was quite a night, talking with her. A doctor came in at 5:30 in the morning. She said, “Hello, can I do anything?” I said, “No.” At 7 am a nurse from the morning shift came in and said, “What are you doing here?” I said, “Taking care of my baby.” She said, “You’re not allowed in here.” I said, “If I wasn’t here, who would take care of my daughter?” She said, “You have to leave or I will call the police.” I said, “That’s up to you.” I was removed, but that’s another story. I can’t explain the pain of how cruel humans can be, but this nurse fits the name.
That afternoon my wife and I came back, and the nurse was mean and nasty. “Don’t touch your baby, she doesn’t need that, don’t move her.” That same day we were called and were told to please come to the hospital immediately. Terri had just asked Greg to call Children’s Hospital to see if the baby could be transferred there. We were trying to figure out how to get around the red tape when we got the call. Your baby needs immediate surgery, and we have to transport her to the hospital what they can do that. My wife and I went to the hospital, and our baby was in an enclosed glass incubator with tubes and lines hooked to her. There were 10 people waiting to transport her. My friend, the mean nurse, was there. I asked who was in charge. A girl in overalls stated that she was from Children’s Hospital National Medical Center in Washington D.C., and she was in charge. I asked her if I could talk to her. The mean nurse was not happy. When I explained to the nurse from Children’s Hospital that my wife had never gotten to touch or hold her baby, would she let her do that? I told her how I was treated and all that had happened. The nurse from Children’s went over to the incubator and said, “Mrs. Burke, would you like to hold you, baby?” The other nurse was livid. She started to say something. I said, “You’re out of you jurisdiction.. and smiled.” The nurse from Children’s said, “Once I come into this hospital, I am in charge of this infant.” The only thing I could think was “Paybacks”!
We followed the ambulance to Children’s’ Hospital National Medical Center, and so began our life with Esophageal Atresia.
There is so much more…the surgeries, the palsy, and reflux to this day. Our daughter is alive, many times a royal pain, but, as I promised her in the hospital I would take care of her and other children or parents who would go through this.
Many Story’s Birth 2 Adults, EA-TEF and TOF-OA below, all children at some point will reach Adult Hood, much more research is needed in this field of medical understanding. I have not changed the spelling of the story’s below they have been posted as written.
Hi, am Amy Needham I am 30 years old
Hi, am Amy Needham am 30 years old I have tof and a heart condition called SVT I had an operation to make my heart a little bit better when I was in my early 20s but I still get problems.
But to help me tho this I enjoy to paint and I often paint animals n fantasy art, it helps me a lot. another thing that really helps is listening to music, music therapy is brilliant… For all those adult tofs out there keep positive and find out the thing your most good at and enjoy doing and you do it!
Taking one’s mind off illness tho pleasure is the best way! Plus… don’t forget to have a Milky before bed it will help with reflux or a banana… another hobby I do is a bit of horse riding when am well enough… It’s so good and it exercises
My dogs are my life I see to them all the time keeping my mind off things to look after my babies work so well… And at the moment am happy my mum n dad look after me all the time bless them! I hope this piece of information helps tofs out there. Xxx
photo included is one of a dog I painted x
This is the story of me, Law Turner Adult Tef
I am 55 years old and have lived a relatively healthy adulthood but as a newborn I had TEF. I come from Detroit, Michigan born in 1962, I have 3 older siblings, back then both of my parents drank and smoked cigarettes ( I do not know if this had any cause and effect on my TEF) my mothers parents had just passed away while pregnant with me, I was told growing up that she had a mini-stroke during the pregnancy.
Above I have included my papers from the hospital as I was not expected to live, so I was not given a name for a few weeks other than Baby boy Turner. I do not remember too much for the first few years maybe about the time I was 3 years old, as I was not allowed to go outside and play with other kids my age. During my operation, I was placed face down with my right arm cocked back (supine position) so my right shoulder has always been pushed forward to this day.
Since I was not allowed to play outside I learned how to read and write at 3 years old, by reading the encyclopedia, both my parents never finished school, my father dropped out at 11 years old and my mother at 14, so by the age of 4 I was already out reading them. My father worked building trains and was very creative. My mother was very conservative and stayed home. Somehow I knew I was different than the other kids in the neighborhood, just not sure why by the age of 5 I developed a double hernia (great another operation) I probably weighed in about 30 lbs. I’m not very big and never have been. So I was now at the age for school and was put in a class that the children there still seemed to be eating crayons and glue, my teacher recognized my reading and writing abilities and had me bumped up 2 grades, so now as well as being small for my age I am now in a class with children 2 years older..(ugh)
I was immediately drawn to the quietest kid in class, his name was Mark and come to find out he had a brain tumor and would not last the year. A hacking cough in class got me in trouble a few times as the teacher thought I was making fun of him. We had physical education classes but I had no upper body strength because when I had the TEF operation the muscles cut across the lats which in turn severed the nerve endings for my right side pectoral muscles as well as you can see in some of the pics I post. So yes I was picked on (bullied) for most of my school years, But most of my bullies became friends even through high school.
My brother was short and stocky but was very athletic, he got me into running, when I started I was like a turtle in a molasses factory, but it was a non-invasive sport (my mother didn’t want me to get hurt) and the only real competition I had was myself. I focused on being the best, my brother was the fastest cross country runner in the school and broke a few records at the school, he was focused, something I was lacking. So I ran every day, before school, after school until my junior year of high school we (the team) qualified for the state meet. I also started Martial arts training to protect myself and the exercise and discipline.
At this time Soccer/Football was becoming popular in the states in the late 70’s early 80’s, and of course my brother focused his attention on that and started playing for The Detroit Express, we would kick the ball back and forth but I had never played before and found it fun, (little did I know it’s a very rough sport) I also found out I have a High Tolerance for pain. I started a team at the school with me and one other guy…that’s it..no other players, no coach…we traveled around until we found others like us, looking for a team and started our own team.
First time breaking a few ribs hurt, but I managed to keep playing, I found out I was still the fastest player on the team, just needed to work on my skills.
It was now the mid 80’s and work bored me, I was truck driver delivery (think UPS) and I kept up with the running because it hurt a lot less than being pummeled by men who outweigh you by 100lbs or more on the soccer field, I was weighing in at 145 lbs the same weight I am today. So I looked for a new adventure…
The U.S. Army…I went for my physical where I had to strip down to my underoos, I looked like Frankenstein (according to their doctor) but I was told if I could do 13 push-ups they would let me in..THOSE WERE THE HARDEST PUSH-UPS I HAD EVER DONE..but I passed..and went to boot camp, being shy helped as I did not open my mouth…I shut up and listened..also I was a bit older than the other recruits…I spent nearly 13 years in the Army and never regretted it. I received an Honorable discharge around the year 2000.
I have hardly been sick, maybe a small cold now and then, I still work out just not as much as I did when someone like a drill instructor was yelling at me. There is probably more to this story that I did not cover, but the main thing was that I was born with TEF and I did not let it slow me down
..Sorry for the grammatical errors, it’s been a while since I’ve written anything
Claudia Born with EA-TEF 1953
Claudia: Her Surgeon’s View
Juda Z. Jona, MD, Head, Division of Pediatric Surgery, General and Thoracic, Evanston Hospital, Illinois
I first met Claudia in 1988 when a “full grown” woman came to my clinic in Milwaukee carrying a large x-ray envelope with her. I was certainly baffled when I found out that she was not a mother of a patient but rather the patient herself. When I informed her that my practice was limited to the surgical care of infants and children, she countered by stating that although she was an adult, her problem is that of the very young. And sure enough, when I reviewed her films it was readily obvious that Claudia had recurrent TEF. I was especially amazed not only to find out that she was the first successfully repaired EA/TEF in the state of Wisconsin, but also that she, in essence, had symptoms of TEF all of her life. None of the family members nor the various medical doctors (from pediatricians to OB/GYN to internists) even thought to relate her chronic coughing (especially when drinking) and repeated chest infections to the possibility of recurrent TEF. From her story, I concluded that her recurrence happened early in the postoperative period (back in 1953) and that is one of the reasons she had to spend 9 months in the hospital and resort to the “best healer of all,” God, to see her through this dangerous time. And here she was, a mature woman, mother of two, who played tennis (and even smoked cigarettes!) who endured a “stormy” infancy and lifelong lung symptoms; who despite it all remained kind and mannered and to whom one had to speak straight to her face so that she could understand one’s speech. She, in other words, was irresistible and I promised her that I would correct her problem surgically.
At the time of the operation, the TEF was located in the conventional site, where the old one was situated. There was very little inflammation around it – suggesting that the tracheoesophageal fistula — was, indeed, of longstanding. When we disconnected the trachea from the esophagus, we repaired the two side holes in them. In order to secure the repair and prevent re-recurrence of TEF, I “borrowed” adjacent tissue and imposed it between the two suture lines. She healed promptly. However, as you heard, her esophagus developed some scarring in the area for which occasional dilations were required. It was particularly interesting for me to talk to Claudia about her lifelong esophageal problem. Since all babies with EA/TEF have impaired motility of the esophagus, Claudia is (was) not an exception. She can describe a “bolus” (chunk) of food slowly descending down the esophagus with the “descending” discomfort it produces. Her post problem with “food getting stuck” in the esophagus is a most typical occurrence in our babies and most parents can agree to that. She soon learned to chew her food well, eat small pieces and drink frequently with her meals. No question these points are driven home frequently to all parents with EA/TEF babies. The message from Claudia for all of us to remember is not only the triumph of the spirit. Also, one need not to ignore symptoms but to pursue them with persistence and vigor. Claudia is lucky not to suffer the consequences of her chronic TEF, namely chronic, debilitating lung
condition (I do hope she stopped smoking by now – a hint Claudia!)
Testimonial Of Aiden Matthews
My name is Aiden Matthews. I was born on 26 November 1990. Shortly after birth Doctors found I had a condition called Tracheo-Oesophageal Fistula (TOF), Oesophageal Atresia (OA) with Tracheobronchomalacia (TBM). I am now a 25-year-old man who is able to live a mostly normal life. My past 25 years have been a journey, to say the least, of both tough and good times. As I grew up and got older, living with my condition became easier as my body adapted and I adjusted through trial and error with regard to eating patterns and habits, which foods worked or didn’t, day to day living, severity of colds, flu’s and pneumonia, signs when something is amiss and the severity of such things.
When I was just 3 days old I underwent my first TOF repair, which was not a success as the gap between the two ends of my Oesophagus were too big. The ends were closed off and a Gastrostomy tube (feeding tube inserted directly into the stomach) was fitted. At 1 month old, I was then transferred to the Johannesburg General Hospital where Professor Peter Beale took on my case. He attempted a TOF/OA repair again which this time round was a success. Due to the join being very tight I was still unable to tolerate feeds and was fed through a Gastrostomy tube which was then only removed at 7 months old at which point after my first oesophageal resection, I then started eating smooth watered down foods with no lumps in it at all.
I also at 7 months old underwent an Aortopexy (A surgical procedure where the Aortic Arch is fixated to the sternum so that the Tracheal lumen is pulled open) to treat Tracheobronchomalacia. I battled with acid reflux from the beginning; I would aspirate and develop pneumonia. It was then decided by Prof. Beale and my mother at 8 months old to go ahead and perform what would be the first of my 3 Nissen’s Fundoplications whereby the Fundus of the stomach is wrapped around the lower part of the Oesophagus and sewn into place. This prevents food and gastric juices (acid) re-entering the Oesophagus. (In simple wording it prevents Gastric Reflux) I also had my Dilatations starting from 1 month old. These used to be done weekly, then bi-weekly at first for 7 months but they started becoming more prolonged in time until there was a 7-year gap between, till I had my last one.
At the age of 16 years old I developed Barrett’s Oesophagus after it was found that my last (3rd) Nissan Fundoplication had failed. This is a condition whereby the Oesophageal lining is damaged due to Gastro-Oesophageal Reflux of a long period of time. It was then decided due to the severity and Oesophagectomy would be performed.
This took place on the 7th of January 2008. There was firstly a decision of a Colonic Interposition ( Where the colon is used to replace the Oesophagus) to be performed but as a result of too much scar tissue surrounding the colon from all previous surgeries, it was decided to perform the Pull Through Oesophagectomy(When the stomach is used to replace the Oesophagus). After the 11 hour procedure, I developed Adult Respiratory Distress Syndrome and was ventilated for 6 weeks and spent a total of 7 weeks in ICU. As a result of the Induced Coma, I then developed Muscle Atrophy (Loss of muscle tissue and nerve supply) in which I had to learn to use my limbs(arms and legs) and to walk again. This is when I got my nickname The Living Legend by My Stepdad, Mother and many others Trigger Thumb- Repair was done at 4 years old.
One of many Gastroscopes, but in particular one was to retrieve money in which I had swallowed a 10 cent coin (1994) and it got stuck in my Oesophagus. (Was nicknamed Mr Money Bags.
Perthes Disease( Blood supply to the femoral head of the hip bone is disrupted and bone cells die resulting in softening of the bone, bone loss and distortion) in which at 6 years old I had an Osteotomy (Cutting off the hip bone and alteration of weight bearing) then at 20 years old underwent a Total Left Hip Replacement.
Anal Fissure- had a fistulectomy to repair it at 22 years old
Haemorrhoids- had a Haemorrhoidectomy (Removal of Haemorrhoids) at 23 years old.
An inguinal hernia due to weakening of abdominal wall in which I had a repair done at 24 years old
Stenosis of Pylorus (Narrowing of the Pylorus situated at the opening of the stomach into the Intestines). Underwent a Pyloroplasty at 25 years old. Today I am qualified as an Enrolled Nurse and wanting to pursue a further career in Emergency Medicine as a Paramedic, which I hope to start my studies within the next year to achieve my goal.
My name is Tudor Morgan-Huggett. I am an identical twin that was born prematurely at 28 weeks in 1995. I was diagnosed with Tracheo-Oesophageal Fistula (TOF) and Oesophageal Atresia (OA). The rare condition affects one in every 3,500 babies in the world. I then spent months in the hospital and to date, have undergone 95 operations. I spent until the age of eleven on oxygen, due to chronic lung disease, and until twelve months ago was fed via a gastrostomy. I have various other conditions, one being hearing impaired in both ears.
I have faced many challenges at such a young age up until now. Due to the severity of my conditions, consultants said I wouldn’t survive beyond the age of three. The strength that I had, to overcome this shows you that you should never give up, believe in yourself and that you can go through the pain to prove the doctors wrong.
The great support from my family, friends and relatives has helped me massively. Along the way, my family have been my inspiration, without them, I wouldn’t be the person I am today. My mother, father, sister and brothers have always been there for me, through an endless amount of hospital admissions, procedures and appointments, the latest operation being January this year. I am extremely close to my family; they are always there supporting and encouraging me to be successful in life.
I missed many lessons due to hospitalisation. When not in the hospital, I attended Pembroke Primary School and Chepstow Comprehensive School. The staff members, teachers and other members of the community were very encouraging, they were made quite aware of my set of conditions. During my time at Chepstow School, I achieved awards for different sports and overcoming adversity at the schools Oscar and BAFTA’s evening, I was very proud to be honoured with this great accolade. Having achieved three A* stars at sixth form, I am currently in my third and final year at Cardiff Metropolitan University (UWIC), studying Sports Development.
I currently play for Chepstow Cricket Club, Caldicot Town AFC and I am a member of the Cardiff Metropolitan University Futsal Club where we play in the Welsh Futsal League. I play for the Wales Deaf Futsal Squad. Playing for Wales is a dream; it’s an amazing feeling to play for the country of my birth. Recently, I have been shortlisted for the Great Britain U21’s Deaf Football Squad. I will be attending along with other selected players, the monthly training camps in preparation for the European Championships to be held in Poland, in August this year, (2016). This personally, is a great achievement for me.
I am a very outgoing person who enjoys meeting new people and gaining new experiences. I have travelled abroad and explored some amazing countries; such as Italy, Spain, Portugal, and France. I love the different cultures. I am so happy to have been able to achieve all that I have done over the past few years. I have challenged myself to work hard and strive to be successful in the future. I hope you find this short story of my experiences of growing up and beating the odds, a warm and an encouraging read.
I really hope that this will give solace and comfort to other TOF’s and their parents.
Tudor Morgan-Huggett said he was wanted to help others by posting his story on this website
Adult EA-TEF makes the Paper, click on Paper Cutting to enlarge it, to read.
Thank you for allowing us to include this on this Website
My Story by Carlos Defazio
Hi everyone, as I have just joined the group that Steve and Bev created, which I found after seeking further information about our condition, on the internet 2016. I would like to share my story with all the other members, of the facebook group, and you reading this on this website.
I am a 42 year adult from Uruguay who was operated of EA and TEF when just born, in 1973, being the first case to survive in Latin America and the third in the whole America.
Since then until four years ago I had no trouble in feeding or living regarding my original condition.I grew up happily, and unaware of all the suffering that I had undergone as a baby, but very aware of all the looks that I get in the beaches, swimming pools and any place where my body should be exposed.
The only reminder of this condition was a plastic surgery at the age of 11 years, performed on my back on the external original scar of the operation, since the doctors had not even bothered to remove the stitches as they thought that I would not survive the operation, like the others babies before.
Yet, in 2012 after an endoscopic control, doctors discovered strong stomach reflux and the formation of a diverticulum between the oesophagus and the trachea, just where the original surgical stitches were made.Since then I have been treated with medication for the reflux, but doctors have no intention to perform any further surgical attempt, due to the risks of such an operation, and to their lack of experience in the specific field.
From then until this moment they advised me to live on until this diverticulum may break completely, making it impossible to eat or breath properly. Then and only then, they would operate on me.
So I have been trying to gather more information about my condition, and about the medical experience and teams that would be able to treat properly patients with such a condition like ours. I am really happy to share my story with all of you and to see that I am not alone in this world that often does not recognise our condition….
Feb 2016 update
Hi everybody, Updating news from my state of (non) health…yesterday, at 42 years old I had a control check endoscopy, resulting in discovering another diverticulum, smaller than the other found previously, always in the same old area where the original internal stitches were made to repair my fistula when I was born…..Slowly all is falling apart and doctors keep on telling me incredible excuses such as: it is normal, do not worry, best not thinking in the possibility of a new fistula… So I keep on living with this pouches inside my chest, full of food, reflux and so on…
Thanks for listening to my story, greetings to all of you… Carlos, from Uruguay
Sarah has started to write a Blog of her life link below
I was born with multiple birth defects; the most serious was Esophageal Atresia and Tracheoesophageal Fistula (EA/TEF) which means I was born without a complete esophagus. The top part of my esophagus ended in a blind pouch and the bottom connected my stomach, with all of its acids to the bottom of my trachea, just where my airway branched into my one functioning lung. This was a very dangerous combination. Babies born with EA/TEF usually have multiple birth defects throughout their midline tract and problems with their breathing and eating because you should have 2 tubes – one for breathing (trachea) one for food (esophagus). I had one and a half and only one lung. There are less than 10 cases like mine (EA/TEF and missing a lung) in the medical literature. Most of the babies born like me died before they reached 6 months old. The day after I was born, surgeons put my esophagus together – the first of over 20 surgeries I’ve had in my life. The surgeons that worked that day had over 60 years combined experience. I was the first that they had seen with one lung and a large fistula.
In addition to having one lung; I had a 90% collapse of my trachea which forced me to need a tracheostomy and a ventilator until I was three years old. The ventilator and trach also caused devastating damage to my hearing, but that’s the price I paid to be alive. The tracheostomy saved my life because it allowed me to breathe, but put me at risk for pneumonia. With one lung, any pneumonia is life-threatening. I spent a week or 2 in the Intensive Care Unit almost every winter throughout preschool and elementary which meant I had to work twice as hard to learn and felt like I was constantly behind the other, hearing kids.
The same drugs that saved my life when I had pneumonia took away my hearing by the time I was fourteen months old, but I was originally born hearing. This lead to many communication barriers even though I have cochlear implants (CI). It is hard for me to talk with others in a noisy area because my CI’s do not filter out the noise to hear conversations. Being deaf is actually the least of my issues compared to my other issues, but it impacts my life deeply and every day.
Most people are born with two lungs, a heart on the correct side of their body, and a stable trachea. I was born without any of those things. This meant I had a very rough start of my life. I was given a tracheostomy to save my life when I was four months old. The trach saved my life and kept me alive and well for seventeen years. However, in exchange for the trach, I had to lose my hearing. The trach itself didn’t cause me to lose my hearing, but all of the hardships of having my trach combined with antibiotics to combat my high fevers caused my complete hearing loss. The fevers were in the 105-6 range. All of this has led me to be the woman I am today. However, the story of my summer surgery was a step in the right direction to reverse my gastric pull up and get rid of my tracheostomy.
Otherwise, I would throw up. After I ate every meal, I would run to the nurses’ office right next to the bathroom to suction out any coloured mucus from eating. Sometimes I would have to suction out chucks of food from my meal. When I tried to join in groups for classwork or fun activities I was simply told I couldn’t join my peers. My teachers would pull me aside and tell me to just work quietly alone. Although one of my classmates wanted to invite me to her house to hang out, but her mother told her she couldn’t have me over because of my issues and the trach. I really wanted to get rid of the trach so I could eat without aspirating and feeling full. I also wanted the experience of being treated like a normal person without being shunned or stared at all the time. I was never laughed at to my face but I was shunned from my peers and their parents too. In 9th grade, I took a trip to Boston to get a 2nd opinion on if the trach could be removed since the three decannulation attempts failed – after 4 hours, after 1 day, and the last failed attempt failed after 4 days. Dr.Jennings and his team spent that summer evaluating my anatomy and making a plan for the 2014 surgery, just before I entered 11th grade.
The first trip to Boston was scary, but a success. I was approved for the 2014 surgery which took over 32 hours to compete and almost a full year to recover from. Throughout the evaluation I made one huge discovery. I was born without a uterus also known as MRKH which would make me infertile. Many people with EA/TEF are infertile or struggle to support a pregnancy due the fact that they have a hard time eating to support themselves let alone another life. Also another factor as to why people with EA/TEFs tend to be infertile or unable to carry a baby to full term is due to the fact they can have defects in their reproductive system which is unfortunately common with people with EA/TEF. Surprisingly, the EA surgery team didn’t know this, but the MRKH specialists were not surprised, as they had seen other teenagers with TEF and MRKH. I went into the first of 2 surgeries scared, but with high hopes.
Beep beep beep… the ventilator droned on. I slowly woke up. Everything around me was fuzzy. “What day is it? Where am I?” I asked myself. The walls were sterile white. My parents were sitting to next me in a chair watching me. I couldn’t recognise them right away. The drugs that had kept me in a coma for a month prevented me from recognising them or anyone else in the room when I woke up. I was so exhausted that I let myself drift back to sleep. Falling asleep while on pain meds makes me so light headed and so peaceful. It was almost like floating in a swimming pool on a warm day. I was in the ICU for a month afterwards and on the floor in Boston children’s hospital for another month. The recovering period was long and unconformable. I had to have my pain managed and relearn to walk again. This required six months of physical therapy. I had a lot of support from my family and friends as well.
For the first two years after surgery, everything was fine. I was able to eat without feeling full in my chest, aspirating, or throwing up. However, six months after my nineteenth birthday; I got a chunk of a hot dog down my esophagus. I ended up in the ER to remove the hot dog. While in the ER I found out several things. First of all, adult GI doctors and ER doctors know almost nothing about EA/TEF. The adult GI doctor on call in the ER asked what an anastomosis was. Once the doctor finally scoped me, she didn’t see any pieces of hotdog instead she saw mushy chunks of food over the past two days prior to the ER visit mushed tighter with air and liquid together in a ball. The food ball was so large that it took 45 minutes to clear out the mess. After that unexpected ER visit, I went to an esophageal dysmotility specialist two hours away from where I live. He scoped me and found partial esophageal webs. He removed the webs and my eating and swallowing went back to baseline.
Another six months later I ate a rhubarb pie and I got it stuck. The next morning, I woke up with a burning feeling in my esophagus. I had a hard time swallowing my breakfast; by the next day I couldn’t swallow my spit and my pain was about 9 out of 10. My mother took me back to the ER. The ER doctors’ first question was “why did you come back? We don’t want to help you.” Then it took almost an hour to get my pain meds from the time I asked for pain meds to the point I got them. Instead of getting all of the chewed up food out of my esophagus; he took some of it out and shoved the rest of the reminding remnants down further. Then after the scope was done, he told my mother that if I hadn’t been stopped swallowing my spit he wouldn’t have helped me at all. Excuse me, but by the time I am unable to swallow my spit I am severely dehydrated, my pain is also severe, and I probably hadn’t eaten a good meal in a couple days. Why would anyone wait until their condition is out of control to seek help? It only makes the medical staff job much harder and the patients loved ones worried, stressed and scared.
Since the first trip to ER left us with no solutions and a lot of stress I had to go to a different ER in a different town for help later that week. I still couldn’t eat anything and it hurt to swallow anything. The second ER was kind and helpful. The GI doctor there took me down to the GI lab and scoped me and found a large ulcer down in my mid and lower esophagus from leaving food down there so long and shoving food bits lower down into my esophagus. He also did an emergency dilation. I had to be admitted overnight for observation and pain management. I was discharged with a treatment plan for my ulcer and a follow-up appointment. I was grateful for the help I got in the second ER/hospital trip but by the time I was treated for my issue I had gone a week without eating much and my body was breaking down protein for food. In other words, I was starving. If the doctors from the first ER trip had been understanding, compassionate, and had done their job thoroughly then I wouldn’t have had to go through all that.
My life has improved since of the 2014 Boston surgery in some areas but at the time it has caused issues. For example, I have had more issues with my esophagus than I have had in my entire childhood. However, my breathing is quieter and easier with the exception of recurrent respiratory infections. Right now, I am under a treatment plan under a couple of different doctors and things are going well but what happens health-wise in the next ten to twenty years? I have no way of way of knowing what my future holds for me. Right now, I am planning on going to college and pursuing a degree in family studies and exploring my future career options. Whatever I do with my life, I require really good health insurance to ensure any and expected medical emergencies EA/TEF related and day to day care. This requires careful planning of my career and life in general. I am going to have to make big decisions and ask big questions such as, where am I going to live after college, where can I work that will cover my medical bills, Which doctors will I have to switch to that can handle me and are willing to help me if I move away from my hometown, If I am unmarried and living in my own apartment how will I get to the ER in the event of an emergency, who will speak up for me if I need it once I get to the ER.
These are things that young adults with EA/TEF have to worry about as leave home and step outspread wings on their own. I am not trying to scare anyone but for a young adult with EA/TEF the world out is more complicated than for a non-EA/TEF adult and leaving home for the first is scary for everybody. Imagine negotiating the world as a young adult with a condition that most doctors have little to no understanding of and an uncertain future? That’s why doctors need more awareness and understanding of EA/TEF and how to assist adults or ask for an experienced doctor to step in. In my mind, understanding and awareness lead to compassion and compassion helps stressed and scared EA/TEF adults and their loved ones once they arrive at the ER for help and treatment which would make our lives less stressful than it already is.
Looking back thinking 'where have the years gone'
Steve Wyles (ME)
Over the past few months, I have talked to Adults born before most of you were born, about our time in Hospital’s, for me the early Children’s wards where long, girls one side, boys other. No TV or if there was it was at one end and black and white. A few tables in the middle were for doing games or just colouring in things, boy was I good at colouring without going over the lines. Parents could not stay, you were on your own, Nurses took no messing around, you were there ill, they had a job to do. back then they cleaned the ward, and by god, they cleaned it well. (Not like now, were cleaner might talk a wee bit of English but will mop around a case rather than mop and move and mop were case sat?) Talking in England here. I often look back and wonder, was I saved from eating Hospital food by being Nil by Mouth, or did I miss out? Those metal bowls for ice cream or jelly. I don’t remember much of my early days, maybe I just blanked it out I was there so much.
In early Adulthood being on a very big ward, again ladies one side men on other, the noises of being an Adult …………….. I felt lost. I used to get out of bed and pump it to the highest point I could climb on a chair to get back into bed, why you might ask, so I could see over the top of people each side of me, so I could watch what was going on. It’s funny what one remembers, like that curtain being pulled and staying closed all day, then at night in the dark bringing in a long black box, so the person behind that curtain could hide in it and get off the ward without others seeing………. No one told me it was a one-way ticket out.
Being in Hospital was for me like the feeling one must get when after school you get home and you feel safe, I still to this date get the same feeling and I relax knowing it’s warm and safe. I hate needles, and yes I have passed out in some weird places when being told, this won’t hurt……… I was rushed into Hospital once in a very bad way, followed by a bucket of my blood, Nan bless her was there too, while she sat outside the A&E room she heard the Female Nurse say, Steve we need to take some blood, and me replying saying, can’t you take it from that bucket? I don’t think I can spare much more……….. Nan said she then heard one of the younger Nurses say….. you can hold onto me if it helps ( I was only in my 20’s be rude to say no ) Then the nurse said, Steve, this won’t hurt………………………………………………………… BANG the nurse and two tables went flying, l had passed out. woke up on a ward with no Nurse around. I spent 8 weeks being moved between 3 Hospitals, wired up to blood and drips, one time my Sister came to visit, then training to be a Student Nurse.
I remember I had drips in both hands, and reaching up to adjust the wheel on the pipe to slow the flow into my hand, Helen said, what are you doing, they are timed to flow at that rate. Well, I was a pro myself by then if I’d of called over a Nurse she did what I had just done, and after all said and done, it was my hand hurting. If you read my Story on this site your of read a bit from a Lady called Pauline ……….. this stay was where we meet.
life in Hospital for me as a child and young Adult back in 60’s 70’s and 80’s etc moulded me I guess to the person you see today, there are things I hate in life that most never give a thought about, but on the flip side I picked up life skills to that have allowed me to do things others might not feel happy with. I have always thought about others first and always tried to be happy go lucky, finding the funny side to most things that might have gone belly up. As I grow old I might be getting like an old grumpy old fella, but least I can smile about it all.
Something I just remembered, while in hospital once, I was on level 8 up in the clouds, and being an Adult was stuck on the ward, Staff wouldn’t let us out, not even my smile could get us (others from the 4 bed ward) off that ward, that night on shift change over My best Friend came up and had parked his car in full view of our ward, Staff left for home, while walking across the car park where my Friends car was parked, we set of the remote controlled car alarm, My God she jumped, the next day again kicking our heels thinking of something to do, we made paper aeroplanes, and seeing how far we could get them from 8 floors up ………….. Yep, you guessed it, we got caught by the Staff Nurse from the night before ………………………………………….. yippy that day we got off the ward on LITTER PICK UP. Was it worth it … dam right it was. And yes she knew it was me and the car, but it was banter friendly banter between us and the Nursing Team.
Click to Enlarge. You’re see my Name on this Medical Paper, this research is very much needed because your Children will be classed as an Adult one day.
Times change, I found the picture on Adults holding puzzle pieces, and as you can see used it on this page, reason is this, for every Adult now who was born in an age where I was everything was a learning curve for the Doctors and Nurses looking after us, puzzle bits slot together allowing, you to move on to find the next bit and so on. This puzzle is still growing by the day, by the people learning how it all fits together but without the box lid to follow.
Adult care and understanding on what is in front of us is a very very short puzzle, no one thought that far ahead I guess, we are walking on thin ice, and without the data or understanding seem’s the ball has rolled backwards to when I was born, a lack of understanding of what is coming. Thank you for your support and understanding to why I set this website up, and with Bev Starks help the Facebook group, and our friends in the USA working on an idea that could help start the puzzle started for my age group. Remember this, your Child is also headed this way too.
Hopefully, more story’s from Adults will follow from here.
A Quote from an Adult TEF about looking at life born unable to swallow
Lack of records is absolutely correct! In fact, some hospitals are gone that were in place when we were born and had our surgeries. This is the sad part….no info available to chart our medical difficulties as well as progress. Plus, most of the doctors that treated us as well as the surgeons have left this planet earth.
Alison Adult TOF 1-07-2015
So in Feb 1972, I guess you could say my parent’s world was turned upside down literally.I was their third child born so you can say that my mum pretty much had the mothers instinct down to a tee!
In those days l suppose woman were to just give birth accept what your doctor told you and go home which was pretty much how my life started. Mum and Dad took me home although they were a little worried that l wasn’t feeding properly and just being sick whenever l was fed.
The day after l went home mum took me to see Dr as I still wasn’t feeding properly.Incredibly the Dr’s reply to mums concerns was….you are a fussing mother take her home and tilt her cot up !! Listening to docs advice and against her gut instinct, she did as she was told but still no relief from sickness. The second day at home things took a turn for the worse so my parents rushed me to Alder Hey hospital in Liverpool. My breathing wasn’t too good by this time either so it was pretty much a get down there and don’t leave til someone listens……Thankfully! After the various test, it was found l had oesophageal atresia which had led to double pneumonia too. Back in those days the Dr’s there didn’t know about EA so literally had to search for a consultant who did.
My hero was found in Mr Roger Cudmore, from Southport UK, he drove down to Liverpool to deal with my care. That same night at 2 days old he performed my life-saving operation. I still can’t get my thoughts around myself as a tiny baby lying in an operating theatre going through this fight for life! After my operation, my parents and family were called in and told to have me baptised there and then as by all accounts l wasn’t expected to pull through. The nurse on the neonatal who was looking after me became my godmother, these days l don’t think that would happen! Anyhow against the odds l made it through what would be the beginning of a long road of hospital stays/tests and apt’s.
At 6 month old l was ill again and was found to have a kidney blockage, My parents were told back then that with EA you were likely to have extra fingers and toes or renal issues (as my party piece at primary school l used to tell kids that to see their reaction!) Warped l know lol……..So again major surgery followed that diagnosis at which l was found to have a duplex (left sided joined in the middle ) kidney. My earliest memory of knowing l had a problem with swallowing food was around 7 years old and being in the bathroom with Mum banging me on the back trying to get food out that was stuck. Between 7 and 13/14, it was a regular occurrence around the dinner table, Mum warning me to chew my food than me not being able to even swallow my saliva. This always ended in a trip to the hospital for a theatre visit for dilation……Funny how it all just seemed”normal” to me.
Even my barking cough which l had all the time was just part of me, l never questioned any of it. Age 11 saw the return of kidney issue with kidney stones so again major surgery as back then that was the only way to deal with them. I spent 3 months at that time onward M2 with nurses that became like family in the end as a large part of my life was spent there even took my school exams there at times with a teacher from my secondary school watching over me……very strange feeling doing exams attached to a drip!!
Once I turned 16 l was discharged from Children’s Hospital and pretty much forgotten about medically regards EA and l just assumed that l was fixed for good……no follow-ups nothing it was if that part of me was just forgotten. I still had times when l would get food stuck but as an adult, I’ve kind of learned to stretch my neck upwards which thankfully helps the movement of food to go down. Along the way only now do l realise health issues like reflux, a slightly leaky valve in heart/murmur and this year breathing issue are all connected to this birth defect, how can that be in this day and age there is still not enough information out there about our condition.????
So that damn duplex kidney was the bane of my life with problem after problem until age 35 l had a full nephrectomy of the left kidney, strangely felt liberating to get rid of it after all those years of pain. So there you have it, if your still reading thank you for taking time out to do so, also l would like to thanks to Steve …….without him l would not have known what l do now about this condition, as back when l was a child it was pretty much swept under the carpet and now l cannot get medical records to read through as they are gotten rid of after 25 years…….so this site is educating me now at 43 years old about my health as a child!
from Steve, Thank you I can relate to a lot of this above and reading this brought back forgotten memory’s