Born unable to Eat

There seems to be a lack of research for Adults born with these conditions, does this mean a time bomb, is waiting to go off at some point, Research means The systematic investigation into and study of materials and sources in order to establish facts and reach new conclusions.

 

Merseyside Women of the Year Awards 2018

Carley, now 32, was born in England on 28th November 1986. She renounced at birth by her mother and placed into the care of Alder Hey Hospital. She had her first operation when she was just 5 hours old.

She spent the first 18 months of her life in hospital.

Alder Hey named her as “their own”, she had no guardian or parent until her amazing foster parents Mari and John.

Born with Vacterl Syndrome, an acronym for numerous issues, to date Carley has had 98 operations and counting and is taking 100+ tablets a day. In November 2014, Carley had a life-saving operation ‘an esophagectomy’, stretching her bowel up to her shortened oesophagus.

This operation failed, Carley’s right laryngeal nerve was damaged leaving complications such as a feeding peg and losing half her body weight, weighing only 5 stone. While hardship causes some to grow bitter, Carley lets adversity make her better.

Mum, Kim Fullington Story Date: Mon, Jun 11, 2018

It is my goal to support and assure you that there is light at the end of the tunnel.  Without a doubt, it is a long, hard road but things do get better!  Here’s my story:

In 1994 one of my twins was born with esophageal atresia and I truly had no idea how much it would change my life.  The father of my babies was nowhere to be found when I gave birth, and I didn’t even know I was having twins until my pregnancy was more than halfway through, so I went through this alone…

My son was born with a long gap, isolated esophageal atresia.  He spent the first four months in the NICU of a hospital that is located over an hour away from my home.  I had never heard of EA before I had him, so I could not have possibly anticipated what he would need to go through!  I really believed that he was born broken, the surgeon would fix him and we would be on our way…  I was clearly clueless!

After four months of trying to lengthen his esophagus 3-4 times per day, they were finally ready to attempt the surgery to repair it.  The surgery was not successful.  It simply would not reach.  In an effort to allow me to take my baby home, the surgeon pulled his esophagus through his neck to allow the saliva to drain and sent him home with his g-tube.

I was a single mom, so I needed to work to support my babies…  let’s say that finding (and keeping) a babysitter when you have twins and one of them needs special care was a pretty big challenge of its own!

When my son was sent home, I was told that when he was at least one year old and weighed at least 20 lbs, they could attempt a second surgery.  At four months old, my baby weighed only 4 1/2 lbs and was only getting 2 oz of formula every couple of hours!  He was between 18 months and 2 years old before he was finally big enough to tolerate a second repair attempt…!

Another thing I should mention about his repair was that it was a “gastric pull-up”.  They literally rearranged his intestines to pull his stomach into his neck and attach it to his esophagus.  They had limited options because his esophagus ended near the back of his throat, and there was only a “nub” coming up from his stomach to work with.

While my son was in the hospital having nearly died from dehydration from his surgery, I began noticing that when he ate, the area where they had attached the esophagus to the stomach would bulge.  When it would bulge too much, he would cough up whatever he ate!  I wondered what would happen if I put just a little pressure on it, so I worked up the courage to touch it when it was bulging…  just a gentle touch was enough to send the food through the esophagus!  Putting a little pressure on that area became a normal part of our feeding routine, and it was the only way to keep him from coughing back up what he ate.  At four years old I began teaching him to manipulate this area of his neck on his own, and today at the age of 24, he still needs to do this!

Between the ages of 2 and 5, my son began having issues and needed dilatations weekly!  Additionally, we were in the ER regularly having food that was lodged in his esophagus surgically removed!  The list of foods he couldn’t eat kept growing and growing, to the point that it was difficult for him to get pudding down.

His doctors and surgeons agreed that he needed another surgery, but nobody in my area wanted to touch him, because they said that there was only one shot left to fix him.  They said that if the surgery didn’t go well, he would never be able to eat through his mouth again!  My son’s pediatrician finally referred us to a surgeon that was 2 1/2 hours away that also agreed that he needed surgery, didn’t want to touch him himself but told me about a surgeon who at the time was said to be the best in the world for EA repairs!  This surgeon literally traveled around the world doing nothing but EA repairs.

I had to drive all the way to Michigan (over 10 hours away) to meet this surgeon and he agreed to do my son’s surgery.  My son was five at the time.  The surgery was successful, but this time we had issues with scar tissue that kept trying to close off the esophagus.  When we got home, I ended up having to take him every-other-week for dilations (keep in mind that the nearest hospital that could deal with his condition was over an hour away, but we were now working with a surgeon that was 2 1/2 hours away!).   From the ages of 5 to 10 years old, this was our life! I believed it would always be this difficult!  My son could eat whatever he wanted, but he needed regular dilations to keep the scar tissue from restricting his intake!

At the age of 10, his regular surgeon was on vacation when a dilatation became necessary.  I was very uncomfortable with the surgeon who met us in his place, who barely spoke English!  This surgeon turned out to be truly God sent!!  While my son was in the recovery room, he met with me and said that he decided to try something a little different.  He went on to say that after the dilatation, he gave my son a shot of cortisone at the surgery site to see if it would help…  my son is now 24 and since this dilation at the age of 10, he has not needed to see a surgeon and has had absolutely no issue with scar tissue or any issue getting food down!

After graduating from high school, My son had to jump through lots of hoops to get in, but he served as a Chemical, Biological, Radiological and Nuclear Specialist in the Army.  He is currently a security guard and a licensed private investigator!  To look at him today, you would never know that anything was ever wrong with him (except for all of his scars)!

There really is light at the end of the tunnel!  Keep moving forward and you will eventually find it!

Christy Cook, who run’s the Charity this website supports

During this time, I was in and out of the Sinai Hospital numerous times for various medical issues relating to EA/TEF. At one time, all the veins available to be used to inject fluids were depleted. The doctor told y parents that only a miracle could save my life.

A Miracle did Happen!!

The doctor performed an extraordinary surgery to find a deep vein in my groin through which I could receive lifesaving fluids. Also, at times fluids were simply injected under the skin to help sustain my life. When I was around two years of age, Dr. Handelsman performed a colonic interposition in my chest cavity to try to make a connection to my stomach. Unfortunately, the transplant did not succeed. The transplant scarred and “died” in my chest.

For the next few years, my life consisted of gastrostomy feedings and hospital visits.  When I was around three years of age, my family decided to move back to North Carolina, our home state.  After having my medical records sent to the Chapel Hill Hospital at the University of North Carolina, we made contact with a renowned surgeon Dr. Colin Thomas.

Dr. Thomas decided to transplant a second colon section outside the chest cavity just under the skin (subcutaneous)… Thinking that this colon transplant could be surgically put into the chest cavity at a later date.  This type of corrective surgery has been an unusual restorative procedure that has served me well… So, I chose not to risk having it placed into my chest since everything was working!!  If I should get a “sticky”, I simply help the peristalsis motion by massaging my esophagus!  Voila!! no more “sticky”!

Eureka! a successful connection was made!

This triumphant procedure has served me well for more than 50 years.  My corrective surgeries have been written up in medical journals and lots of medical students at Memorial Hospital have studied and learned from my medical history. I have been able to enjoy eating by mouth since I was four years old.  I have no other serious birth abnormalities. Thankfully, there are medical professionals who continue to do research and are finding better ways to treat, repair, and surgically help these precious babies who are born with this rare and unfamiliar birth defect.

No one Understands

Hello, my name is Lene Cecilie and I am 40 years old.  I was born with a fistula between the esophagus and right lung. When I was born everything seemed to be normal and I was a happy and satisfied girl. But when my mother should breastfeed me, I began to cry. She did not understand why this happened, a child does not cry when it gets food. She tried everything when it comes to feeding me. 

Mom went to different doctors and no one understood what was wrong. After seven months she came to a doctor who took her seriously and who discovered this congenital defect. I had a fistula between the esophagus and lung. I also have malformations of the trachea. 
As 9 months I was operated.  The congenital defect was so little known that even doctors had never seen this, nor operated this before. They told her that i could be a sports athlete.

But how could they say that when they did know that little about this at that time. They still don’t know how this effects me now 39 years after.  In the years after surgery, there were many trips to the hospital with numerous infections and complications. As the years went, I’ve learned to live with this. I have 34% lung capacity and is still struggling with a lot of infections, wheezing and short of breath. As little I was often teased because I had so much mucus that I gurgling and breathing heavily.

They always got the answer that “I’m glad I breathe at all.”
As an adult, I have often been with lung doctors because of my lung problems. I have, as I said 34% lung capacity and every time I’m at the doctor they say that I have asthma. Every time I ask if it is correct because I have always felt that it is not correct.
For two years since I became hospitalized because of breathlessness.

I could not speak and could not do anything. So when I was admitted in three weeks did they take all possible tests without finding any answers. Since that day I have not been able to work and I’ve been to different doctors and hospitals without getting some good answers. No one believes that this has something to do with what I was born with. It seems to me that we who were born with a birth defect has been forgotten.

 After i was operated is my disease symptoms often not visible to most people. I have what I call invisible symptoms such as shortness of breath, headache, tired and dizzy. This allows others to not always understand how sick I am. It is not easy for others to understand what it is to have only 34% lung capacity. When not doctors know and can tell me how it affects the body, it is not easy for the others.
I hope with this to focus on us who now are adults and what it does to us. Yes to more research in this area.