I have been thinking on how I can involve you as parents on this website, this is what i have come up with. Allowing Parents to write their own story of their journey, posting that to me, and allowing me to post it here, one after the other, in date order for all to see and read.
If you wish to write something for here, send via this email ( www.birth-defect.outlook.com) The idea is for me to save a record of these on here for you all to read.contact me via my FaceBook page link on my Story page, to tell me you have emailed me.
www.steve-tofs.com (Link to my story Page)
If at any time you thought to your self for a split second, WHY ME ? Read ……. Three Angels……. this might help explain, WHY YOU x
“Once upon a time, three angels were busily working in the miracle factory. They were responsible for wrapping up all the little miracles and sending them on their way. Normally they wrapped each one in bright, sturdy paper with big, shiny ribbons. They stamped them with a delivery date and away they would go to the parents who eagerly awaited their arrival. Things usually ran pretty smoothly.
One day, however, down the conveyor belt came a little miracle that made the angels pause. “Oh my,” said the first angel “this one’s uhm…well…different.” “Yes, he is unique,” said the second angel. “Well I think he is quite special,” said the first angel “but I don’t think he will quite fit our standard wrapping procedures.” And the second angel added, “And we know he’s special, but will everyone else?” “Not a problem,” said the third angel “obviously a special miracle deserves extra special wrapping, and of course we’ll send him off with our most heartfelt blessings. Then everyone will see how special he is.” “What a wonderful idea!” replied the others. So they searched the shelves high and low for their finest paper, and their most delicate ribbons.
When they were done, they stood back and admired their work. “Beautiful!” they all agreed. “Now for our blessings,” said the third angel “for it is time for him to go.” “I will bless him with innocence and happiness,” said the first angel. “And I will bless him with the strength to face the many challenges that lie ahead,” said the second angel. “And I will bless him with an inner beauty that will shine on all who look upon him,” said the third angel. Before sending him off the third angel, who was very wise, gently tucked a note inside.
And it said,
“Dear Parents: Today you have received a very special gift. It may not be what you were expecting and you may be disappointed, angry and hurt. But please know that he comes with many blessings. And, while there may be pain, he will bring you much joy. he will take you on a very difficult journey but you will meet many wonderful people. He will teach you patience and understanding and make you reach deep inside yourselves to find a source of strength and faith you never knew you had. He will enrich your lives and will touch the hearts of all who meet him. He may be fragile but he has great inner strength.
So please handle him with care. Give him lots of attention and shower him with hugs and kisses. Love him with all your heart and he will blossom before your eyes. His spirit will shine like the brightest star for all to see and you will know that you are truly blessed.”
surgery-to-save-baby-born-with-esophageal-defect (Link to a Video in USA)
I hope this link will work as it was very interesting to watch, and yes I had a few tears because even though I was born in 1962 this was the first time I’d watched anything about it.
2018 Filip Fraszczyk 5 years old
This is a story of my son Filip Fraszczyk 5 years old, who was BORN UNABLE TO SWALLOW.
Our son was born in November 2012. My pregnancy was going well. When my water broke in the early morning me and my husband Piotr rushed to the National Maternity Hospital, Holes Street in Dublin, Ireland. After 19 hours at 00:03 am my precious little boy was born. Everything looked really well.
We have phoned all our family in Poland waking them up to tell them this wonderful news. We have been so happy. I have breastfed Filip with my midwife’s help, Annamma. Then we all went to the ward.
While I was already on my bed Filip wasn’t coming. I didn’t know that in the corridor my midwife is fighting for his life. I didn’t see this horror, my husband told me about that. On the corridor, Filip had difficulties with breathing his body went all blue like a plum. Annamma had taken a bottle with Oxygen and without the mask (because there was no time to look for one) had spray it on his face so he catches up. She was the first person who saved his life before he could die in the corridor.
We will be grateful until the end of our lives for what she has done. They rushed Filip into NICU in Holles Street Hospital. At about 4:00 am my Midwife and my husband came to tell me this horrible news that Filip will require surgery on his esophagus as he was born with Tracheo-Oesophageal Fistula / Oesophageal Atresia and VACTERL Association (this has been diagnosed later on his hospital appointments) without A and R letters. It was such a huge shock for me as we didn’t know anything while I was pregnant.
I have never cried in my whole life this much as I was on that time. In the morning about 11 o’clock, Filip has been transferred to Children’s University Hospital, Temple Street, Dublin for surgery. He had many tests done before in CUH Temple Street and in Our Lady’s Children’s Hospital, Crumlin, Dublin. Before surgery one of the Drs told my husband (I was in Holes Street Hospital) that because Filip is very tiny, he was only 2.640 kg, there is a risk he can not make it, he can die during surgery. But thank God surgery went well. We are so grateful and will be until the end of our lives to our Surgeons Mr. Sami Awadalla and Mr. Michael and the whole team who took part in the surgery and saved Filip’s life. On the second morning, I could see him for the first time after surgery.
It was so painful seeing my baby with all the wires, tubes lying in the incubator with his swollen little face. All the memories are like it was today… I cry writing this it is so painful…
Our son has spent 9 days in Intensive Care Unit and then has been transferred to Michaels B Ward. After 23 days he was ready to go home. It was just before Christmas. It was so joyful and also so stressful for us. I have been so happy having my precious son home.
Filip had and still has difficulties with weight gain. He is very fussy eater. He also suffers from GERD he is on daily medication.
Filip has a tendency to get food stuck and chokes. I left my work to look after my son because he is the most important person in my life (my daughter 3y old also :)). I’m saying it is like “live on a bomb” because I never know WHEN Filip will choke and IF this time I will be able to help him by myself or will I need to call the Ambulance.
When my son choke I get him to lean forward I put him on my knees and I give 5 back blows and then rotate him so he is facing up but with his head lower than my knees and I give him 5 chest thrusts and over and over again until the food goes down to his stomach or he vomits it back.
It is very difficult for a child to have problems with swallowing food and it is very frightening and stressful while he chokes for him itself and for me as a mother. After this episodes he usually cries, me too and after he doesn’t want to touch food anymore.
He had 2 inguinal hernia surgeries one in September 2016 and 2nd in January 2017
He also awaits surgery on his thumb which probably gonna be in 2019.
Filip gets infections very often and many times has to be treated with antibiotics. He has a very low immune system. My son also has TOF Cough so when he cries or get cold his cough sounds very heavy it sounds like he is very sick but he actually is not. Many times people were like “Oh he is very sick” but my reply only was “No he is not”
Other than that he is very happy child full of energy and always smiles. He has a little sister Olivia 3 years old, she was born healthy. He loves his Grandparents Helena and Kazimierz and Great Grandparent Maria in Poland and miss them a lot. He also loves his St. Louis Infant School. He has SNA (Special Needs Assistant) during school hours so I know he is safe. Filip is on Fortini Smoothies high calorie drinks 2 per day so even at school on his lunchtime he has 1 bottle because even he has SNA I am terrified what about if he has a sandwich and choke while I’m not around. It’s too scary for me.
Thank you for reading.
2018 born unable to swallow written in Polish
W 28tyg ciąży wylądowałam w szpitalu ze skurczami i podejrzeniem wielowodzia. 10 dni przed porodem zrobiłam badania prenatalne, które ukazały mało wypełniony żołądek Filipka więc było podejrzenie zarośnięcia przełyku. Ze względu na wielowodzie miałam wykonany zabieg amnioredukcji abyzmniejszyć ilość wód płodowych. Zabieg ten przyspieszył poród i w 32tc poprzez cesarskie cięcie przyszedł na świat Filip.
Kilka godzin po porodzie Filip został przewieziony do innego szpitala na OIOM gdzie już następnego dnia miał wykonane 2 operacje. Okazało się, że przełyk jest zarośnięty i jest przetoka tchawiczo-przełykowa, zarośnięty odbyt, agenezja nerki prawej, wady kręgów, dodatkowe pary żeber, przykurcz nadgarstka i niewładny kciuk oraz przeszedł Filip wylew III stopnia. Ze względu na długą przerwę między kikutami przełyku zdecydowano wciągnąć żołądek do klatki piersiowej. Po kilku dniach potrzebna była kolejna operacja przełyku.
W czasie 5,5 miesiąca pobytu Filipa na OIOM’ie jego stan był bardzo ciężki, doszło do zatrzymania akcji serca, długi czas nie oddychał samodzielnie, żywiony był pozajelitowo oraz przez sondę. Gdy stan Filipa poprawił się na tyle aby móc go przenieść, został przewieziony do szpitala do Krakowa na Oddział Żywienia Pozajelitowego, ponieważ próby nauki karmienia się nie udały, a po podaniu posiłku przez sonde zdarzały się problemy z oddychaniem. W Krakowie spędziliśmy 2 miesiące, tam stwierdzono u Filipa Asocjację VACTERL. Z powodu niewystarczających ilości jedzenia podawanych przez sonde (nadal zdarzały się duszności) Filip został zakwalifikowany do programu Żywienia pozajelitowego w warunkach domowych.
Przez ten czas uczyłam się obsługi cewnika, pompy, przygotowywania mieszanek odżywczych. Tak dożywiany był Filip przez 2,5 roku. Poprzez słabą odporność oraz zakażenie cewnika do żywienia pozajelitowego pierwsze 3 lata życia Filip spędził w większości w szpitalach. Często chorował na zapalenie oskrzeli. Gdy udało nam się zejść z żywienia pozajelitowego cieszyliśmy się, że w końcu Filip będzie mógł normalnie jeść. Niestety zdarzyły się w tym czasie 3 epizody, że po posiłku zaczynał charczeć, kaszleć i przestawał oddychać co kończyło się pobytem na OIOM’ie. Bywały miesiące gdzie Filip jadał już kanapki, obiady, ciasteczka.. Jednak po ostatnim pobycie na intensywnej terapii bardzo boimy się karmić go.. Aktualnie zjada po 180ml płynnego posiłku co 2 godziny.
Nie są to wystarczające porcje. Filipek jest głodny, nie przybiera na wadze. Prawdopodobnie będziemy musieli założyć sondę nosową i dokarmiać dojelitowo Filipka w nocy. Filip jest bardzo wesołym i pogodnym dzieckiem. Chociaż jeszcze wiele przed nami, cieszymy się z każdego dnia razem. Filip jest codziennie rehabilitowany, pomimo swojego wieku (4,5) nadal nie chodzi i nie mówi. Jest bardzo mądrym chłopcem i potrafi postawić na swoim J
Family of baby born unable to swallow support charities
News provided by The Sick Children’s Trust on Thursday 21st Dec 2017
Freddie Capes was diagnosed with trachea-oesophageal fistula and oesophageal atresia after a sonographer at a special scan advised mum to arrange an appointment at her local hospital. After birth, Freddie was transferred to Sheffield Children’s Hospital and underwent the first corrective operation at just 24 hours old. The Sick Children’s Trust supported his family at Magnolia House, whilst Freddie received treatment. Fortunately, Freddie has made a remarkable recovery and his family are excitedly preparing to celebrate his first Christmas. As a thank, you to the charity, Freddie and his family have joined in The Sick Children’s Trust’s first ever Christmas appeal! Mum, Gemma Capes, tells their story:
Baby Zac Born Without Esophagus Leaves NICU After 104 Days
MAYODAN, N.C — The first 104 days of his life were spent in the NICU. That is how Zachary Helm entered into this world. He was born with Long Gap Esophageal Atresia, meaning he was born without an esophagus. It’s also something the doctors said couldn’t be easily repaired.
“Our trachea that we breathe through are like straws, but his is like the straw paper. So when he breathes it actually collapses on itself and it vibrates. So that’s why he sounds so raspy all the time.” His mother, Victoria Helm, told us.
Victoria said the pregnancy was normal at first, but then they noticed something wasn’t right at a follow-up appointment. The doctors noticed the unprotected membranes of his umbilical cord was covering her cervix and that in the latest ultra sound there was no stomach.
Baby Zac Ultra Sound (Photo: Team Zac Facebook)
At first they assumed it was Placenta Previa. Doctors wanted to send her to a specialist to learn more about it. After going to see the specialist, they came up with a game plan. Victoria knew she would need to be hospitalized by 32 weeks and deliver between 35 and 36 weeks, to prevent anything from going wrong.
However, after even more visits doctors still couldn’t see the baby’s stomach from the ultrasounds. This is what led them to believe it was Esophageal Atresia. The doctors said it wasn’t much to worry about, normally something that could be fixed within the second day of life.
Baby Zac In Hospital (Photo: Team Zac Facebook)
“We just go in and fix it,” is what they told Victoria. She accepted that she would be spending the first few days in the NICU, but was happy to hear that it wasn’t Placenta Previa.
After being admitted on January 7th, they gave her two options. The first was to move his stomach up to close the gap and the second to take a piece of his small intestine to bridge the gap. This process would take 6-9 months and he would need to stay in the NICU for that entire time.
“I don’t really like those options,” Victoria told the doctors, “Let me do some research”.
Her research led to her a few Facebook groups of mothers who were going through the same thing. The group led her to Boston’s Children Hospital. They had planned to travel up north but then they found doctor Tracy who already trained at Boston’s Children Hospital and specialized in Esophageal Atresia.
“We were lucky to find Doctor Tracy,” the family says. She had just recently moved to North Carolina and started to work at Duke Hospital.
Doctor Tracy performed the Foker Procedure and everything was a success.
So when WFMY News 2 talked to the Helm’s it was seven months down the road from when Baby Zac entered the world.
“Seven months and going so strong,” Victoria told us.
“Seven months and going so strong,” Victoria told us.
They spend their days catering to their three children, Mikaela, EJ, and Zac. They’re trying to adjust to schedules, learn his triggers, attend doctor appointments and of course, try to teach Zac to eat. The Esophageal Atresia causes Zac to mostly be fed through a device called “G-tube” or better known as gastrostomy tube. The tube is inserted into his abdomen, allowing the formula to go right into his stomach.
When asked if it hurts or irritates him, “He acts like it doesn’t bother him, he’ll pull on it and all kind of stuff.”
Currently, they are hoping that Zac just grows out of it, there is a 50/50 chance that he will.
“We just want everyone to know what’s going on with him,” She says, “it’s not really a common thing but it’s not the rarest thing either, so it’s good to have people who know and understand that some babies eat differently. They’re not bottle fed, they’re not breastfed, they’re G-tube fed”.
Watch the video for yourself on this link.
NOTE from the Parents who run this site. Please never give up hope we are here all of us in our GROUP to Support you like we have for Victoria and her Family, and we hope this shows that the stress and the strain will one day be a distant memory.
A child's journey
2014 – 2015
(POSTED AS IT WAS SENT)
Roman is a true fighter and I’m so lucky to have him
Hello. My name is Harley
I’m a proud mummy to both of my amazing boys Ashton aged 5 & Roman aged 10 months.
This is how my story starts, in February 2014 I found out I was pregnant with my second child Roman it was one of the happiest days of my life.
April 2014 I was rushed to my local A&E I was 8 weeks pregnant and had suffered a huge bleed. So they did the usual blood tests etc… Kept me in overnight for a scan in the morning. They did say on several occasions that I looks like I had suffered a miscarriage. Myself and Roman’s dad prepared ourselves for the worst. The morning came and I was taken to be scanned I honestly thought he’d gone but there he was this tiny little bean like bubba on the screen his heart beating away. I was so relieved. I was put on bed rest for a couple of weeks.
As I got further along in my pregnancy I all of a sudden had this huge bump out of nowhere I was huge. After a few more weeks I was really suffering it was painful to move, to sleep, to eat.
I was booked in for regular scans due to suffering with Obstetric Cholastasis in my first pregnancy which I suffered with again during this pregnancy. OC is horrible it makes you feel like your whole body feel like it itching uncontrollably. I also suffered with quite bad anaemia in both pregnancies too.
I was at the hospital on a weekly basis to monitor Roman and make sure he was ok.
At one of my scans the sonographer noticed that I had an awful lot of amniotic fluid more than your suppose to have. This was causing the big bump and pain. I was then diganosed with Polyhydramnios which is basically a condition that few women suffer from. Its caused by excessive fluid around the baby.
Finally after all of the problems in my pregnancy they decided to induce me on the 30th of October 2014.
Scared and excited I arrived at the hospital got a good nights sleep and was induced. It was now the next day Halloween 31/10/2014. My labour didn’t go to well, Roman’s heart rate kept dropping I was so tired and drained I had no energy left. They decided to rush me in for an emergency C-Section. Within a matter of minutes my beautiful baby Roman was born.
They quickly noticed Roman turning blue and realised that he kept choking he was transferred straight down to the Neonatal unit. After a few doctors saw him they diagnosed him with a rare condition call Oesophageal Atresia. OA is such a rare condition but also has so many different types, OA is where the top end of the food pipe by the throat and the bottom end which is attached to the stomach are not connected which results in the babies not being able to swallow, these are the medical terms and I’m probably not saying it right but this is the easiest way to explain it.
Roman was transferred to St George’s Hospital Tooting in a matter of a couple of hours as they didn’t have to specialists at St Helier Hospital to be able to treat his condition. At this point I still hadn’t seen him nor held him, they quickly let me have a quick cuddle before they took him away. I was heart broken.
He arrived with his dad at St George’s in the early hours of the 1st of November. He was put straight into an incubator put on drips and all different monitors. He was also fitted with a replogal tube into his mouth. The tube is connected to a constant suction and sits in the top end of the Oesophagus (food pipe) to drain all of the secretions (saliva/mucus) out so he doesn’t choke and it pour into his lungs which could cause infections and make him even more sick. The replogal works really well but also blocks quite a lot so they tend to flush them usually every 15mins with water to help. As the babies get bigger they usually have them in the nose which Roman did.
He was only a few hours old and had gone through so much but as I had a C-Section I couldn’t move and had to be transferred the next day.
Finally the next day arrived I was straight up with the help of my mum and friend Nina. That afternoon I was transferred to St George’s, i went straight to the neonatal unit to see my boy. I saw his tiny little self in his incubator covered in wires, drips everywhere sucking away on this ridiculously big dummy.
That afternoon I had to sign my first ever consent form for them to go in a try to repair the Oesophagus. Off he went to theatre, a couple of hours later the doctors came to find us to let us know that Roman had Long Gap OA and that they couldn’t do the repair until he had grown a little more. Down in theatre they fitted him with a gastrostomy tube which is a tube and goes directly into the stomach allowing him to be able to have his milk safely.
So now it was the waiting game we was told 2 months before they would try again. The only good thing was that Roman could finally have some milk 5 days after being born.
We waited patiently for December to come along, the day of the op was here we did the usual signing of the consent etc… Down he went again bless him. 45mins later I received a phone call to say that the gap had got even bigger and they was not able to do the repair. I was devastated, it’s every parents worst nightmare having a child stuck in hospital and now it was going to be another 2 months again until they tried for the third time. The daily trips up the hospital were heart breaking I just wanted him home as every parent would. Having to leave him there every night was horrible. He spent his 1st Christmas in the High Dependancy unit in St George’s, I couldn’t enjoy it at all I had to pretend it wasn’t really happening otherwise I would have been a wreck the whole day.
Roman wasn’t really gaining much weight so they decided to switch him to a high calorie milk called Infatrini hoping that it would make him grow quicker which it did he gained weight rapidly. Before we knew it February had arrived it was time for the 3rd attempt, all his doctors and surgeons were all very confident this time around. I did the usual signed my consent forms as always and within an hour he was taken to theatre. I was told that the op should take around 4 hours or so. So I waited patiently in the family room, 4 hours had passed and still no sign of Roman, then 6 hours then at 8 hours I was going out of my mind. One of the ward sisters went down to theatre to find out what was going on. She came back to let me know that they was just finishing up. Around 20mins later Roman’s surgeon finally appeared. He explained that the surgery was a lot tougher than expected but he had managed to do it. He also explained that as it was a tight repair Roman would have to stay in an induced coma for a week to give it time to heal to avoid any damage to it. I shook his hand and thanked him for finally fixing my baby.
I couldn’t wait to see Roman I ran over to the intensive care unit to find my baby not looking like himself at all I didn’t know what I was expecting but it wasn’t what I saw. Roman was swollen, on a ventilator he didn’t look real. He was on a ventilator due to being in the induced coma he needed it to breathe for him. I was traumatised by what I saw.
I couldn’t return to the hospital for a couple of days. In that time they had decided to send him to the Paediatric Intensive Care Unit for one to one care as they needed the room in NICU which i wasn’t too pleased about as everyone knew him there.
Now that he was on PICU his brother my eldest was finally allowed to meet him properly for the first time as he wasn’t allowed to see him before hand.
Ashton was so excited to meet his brother but Roman was still very ill. A week later he was still laying there on a ventilator that was breathing for him I mean he was taking a couple of breaths of his own but not enough to breathe for himself. Still paralysed and on an awful lot of sedation so was sound asleep. I thought was he would be off these machines by now.
They decided to wean him off the heavy medication he was on to allow him to try to breathe on his own, he finally did it after a couple of days and they took his ventilator tube out.
He was put on a small amount of oxygen just through his nose for a couple of days.
2 or 3 days later after feeding him he struggled badly with his breathing he went blue. So it was back to the ventilator but he was leaking a lot of oxygen so was only getting a very small amount into his lungs.
He begun to get really sick he wasn’t himself his little eyes were so sad I didn’t know what to do. I knew he wasn’t right but nobody would listen to me. After having a crazy meltdown I was finally heard and a meeting was called with the senior doctors and the surgeons involved with Roman. Everyone knew that he was getting worse and worse but nobody could understand why. They decided to do an emergency B&B you’ll see me talk a lot about these throughout this. It’s a Bronchoscopy & Bronchogram which takes a look down into the airway and pours in dye to see if there are any holes and where they are causing the leak problem that Roman had. They come back saying they couldn’t see anything wrong.
After having his surgeons stand around looking very upset and adamant that they didn’t know what was wrong they finally made a referral to the most amazing place in this world Great Ormond Street Children’s Hospital aka GOSH.
Even tho it should have been done as soon as the leak appeared but I was finally done.
They transferred him over by emergency ambulance on the evening of the 16th of February.
Now myself and Roman’s dad stayed at home with my other son as we didn’t not know the extent of what was happening with Roman.
The next morning we made our way to GOSH, it was all new to us we was extreme anxious but excited to see our baby. We was directed to their PICU department and shown where Roman was. We went over only to find him on this funny looking and very loud machine called a breathing oscillator which was breathing fully for him as he couldn’t. We was quite alarmed at this point. He was laying there looking so pale he had no life in him at all. Within a couple of minutes we was surrounded my doctors, anaesthetises, explaining how ill our baby really was and they had to act fast as he was deteriorating by the minute. They needed to their own B&B but Roman was so high risk it was we either do it or he wasn’t going to make it. The main problem was getting him down to theatre on the oscillator down there as they couldn’t transport it with him down there. So they had to manually pump breaths into him until he was down. After about an hour they come back to tell us that Roman has a big tear in the main part of his airway and two small ones on the sides that go off into the lungs also. This I why the leak was as bad as it was.
They decided to call in an expert, I call him my angel the most amazing man I’ve ever met Mr Professor Martin Elliot.
Me and Fletcher Roman’s dad were taken to a room away from everything and sat down with Professor Elliot, we looked at each other we knew by now that things were serious.
Professor Elliot told us what he needed to do and that was to repair the airway as soon as possible but it was going to be hard as so much damage had been caused, and it was a very hard place to operate on. He had done this type of surgery a lot and some had been successful others not. He explained how he would have to open Roman’s chest and put him onto a heart bypass to be able to perform the surgery but this was the problem, Roman was so ill and weak he was honest with us and gave us a 50% chance of him making it. It was more likely that they was going to lose him on the operating table. I felt like someone had ripped my heart out and repeatedly stamped on it. I couldn’t breathe I couldn’t see due to the amount of tears falling from my eyes. It was now 8pm on the 17th of Feb 2015, it was either sign the consent and let Professor Elliot do what he does best or we make the decision to let Roman pass away. I was so scared but I signed and put all my trust into Professor Elliot.
We went back to Roman and kissed him goodbye we didn’t know if it would be the last time we’d see him. Professor Elliot took my number and told us to go and get some rest and that he would call as soon as he had some news.
We went back to the accommodation that GOSH had provided for us, we sat and watched the Chelsea match as the boys are big fans. We then had to contact all our family to tell them after plenty of tears everyone decided to pray and keep strong for Roman as there was no doubt in their minds he was pulling through.
Myself and Fletcher spent a couple of hours in the chapel at GOSH which I must say is one of the most beautiful places I’ve ever seen, praying and pleading with God and loved ones that had passed to keep him safe.
After a few hours I finally got the phone call to say that he had made it through surgery and had behaved the whole way.
Professor Elliot asked us to meet him in the waiting area of Flamingo ward which is the Cardiac Intensive Care Unit. We literally ran there as fast as we could.
Professor Elliot explained what he had done in surgery. So he had repaired the big hole on the airway with a pericardial patch, there’s a sac around the heart that doesn’t really serve a purpose so he used a small piece of it to create the patch. He also had to undo the stitches on the 2 other holes that had been created at St George’s as they were too tight and of course he then had to undo the repair that was made to his oesophagus.
I was shocked according to St George’s they didn’t know what was wrong with him but they clearly did.
My poor baby nearly lost his life due to there actions.
After thanking the great man that saved my baby’s life I finally got to see him in recovery.
There he was on a ventilator, cannula’s in his feet and hands, chest drains bubbling away, all wired up to his meds and sats monitor.
He was finally safe but the next 48hrs were critical.
The 48hrs passed so quickly before we knew it he was having his drains taken out and slowly being weaned off his medicines.
He was still on a ventilator but he had his colour back he was opening his eyes and looking at me and fletcher.
He started progressing really well they decided to put a replogal back in his nose due to the amount of secretions he was producing but unfortunately it went straight through his the top end of his oesophagus which was a cause for concern as the secretions were leaking into his body. They managed to get his secretions under control and he was ok for around 2weeks after. During a feed milk started coming up from his stomach into his airway which caused stomach acid to undo part of the pericardial patch they had placed on the hole.
He went down to theatre so they could find out what was happening, they found a fistula between the stomach and the airway a connection between the two that was causing this to happen.
Roman deteriorated yet again he ended up with another major oxygen leak which cause a pneumothorax and his lungs kept collapsing, the air leak was causing everything to push against his heart. His heart rate kept dropping badly to the point we were losing him nearly every couple of hours. They placed a drain on his side to release the air so it would relieve the pressure on the heart.
The doctors and surgeon Nagarajan Muthialu another amazing man pulled us to one side to explain what was happening. They needed to urgently operate on Roman again. Mr Muthialu needed to perform the same life saving surgery that Roman had only had 3 weeks previous to now but this time the survival rate was even less he had a 25% chance of making it. It was the same thing again if we didn’t do anything he definitely wasn’t going to make it so we had no choice but to sign the consent and let him go again. We was asked if we would like to have him christened as they didn’t think he would make it through the night.
He went to surgery early the next morning, he was put back on a heart bypass again and Professor Elliot and Mr Muthialu worked their magic again.
He made it of course my boy is a fighter he wasn’t going to give up on us like that.
He came back into recovery the cheat drains were there again bubbling away, the ventilator doing its job he was highly sedated and pain free.
Over the next couple of weeks he got stronger and stronger, he made an amazing recovery his surgeons are still pretty amazed with him.
He was discharged from CICU and transferred to PICU. It felt amazing to have him out of there and on the road to recovery.
The next few weeks were spent in PICU recovering and trying to wean him off the ventilator. He failed twice but on the 3rd time he did it.
I could finally cuddle my baby after months of just looking at him. It was the best thing ever.
Roman over the next few weeks had to undergo regular B&Bs and other things which may I add involved him going under anaesthetic each time.
Finally it was time to meet with Professor De Coppi Roman’s now general surgeon, he had wanted to give Roman an Oesophagostomy which is a small 2p sized hole at the side of his neck and bring the top part of his Oesophagus out through it to allow his secretions to flow freely into a stoma bag. We could have potentially had another replogal but that would have meant Roman staying in hospital for up to a year as it required specialist care that I could not provide at home.
We went ahead with the surgery and Roman now has a stoma at the side of his neck which I have to attach bags to several times a day so his secretions can come out freely without him choking or any spilling into his lungs.
Roman handled the surgery like a soldier but had to be put back on a ventilator temporarily to allow him to recover nicely which he did.
Another week passed and Roman was discharged onto Squirrel Ward which is a surgical ward that usually results in taking your babies home.
Roman was doing so so well until we was a week away from discharge and he started struggling to breath again my heart sunk. They decided to transfer him straight to PICU and put him into a ventilator as he was exhausted and was really fighting to breathe. The next day he was scheduled in for a B&B and an MLB which is a Microlaryngoscopy and a Bronchoscopy its a look down into the airway.
So here we was again back down to theatre he went. They discovered what they expected and that was narrowing in the top of his airway where the repairs were made. The scar tissues were causing it to tighten its the same we get a cut it starts to feel tight as it heals. This is what was happening to Roman’s airway. They also noticed he has severe tracheomalacia the bottom part of his airway was floppy.
They had to balloon the airway to open it back up and cut some parts to allow it to stay open.
The tracheomalacia would have to heal it’s self.
Roman ended up having this procedure done a few times over the next couple of weeks.
He was finally taken back off the ventilator the following day and transferred back to Squirrel Ward.
I think it was around 2 weeks later it happened again, they tried there hardest to avoid it they tried adrenaline nebulisers, salbutamol ones nothing was working it was a trip back to PICU again.
He had all the same procedures again, and yet again was taken off the ventilator a couple of days later and back to Squirrel ward.
Roman finally started behaving and two weeks later was allowed to come home for two days as a trial.
I haven’t really mentioned what’s involved in Roman’s everyday care he has to obviously be tube fed through his stomach so 4 times a day he has gravity feeds which involves giving him milk through a tube that locks into his gastrostomy peg in his stomach. The tube gets attached to a syringe which the milk goes in and you basically hold it until the milk goes down.
Then at night he has a milk pump that does it for me I just have to fill a bottle with his milk every 4-6 hours and the milk pump does the rest.
Roman’s is on several medicines some of which I have to make up myself and I have to give these through the tube in his belly 3 times a day.
He obviously has his neck stoma too which I have to change the bag on several times a day. I have to cut out the sticky disc that goes directly onto his neck attach the plastic bag to it and put it in place.
I have to bath Roman several times a day due to his bags coming off or leaking and obviously he gets through a lot of clothes that need constantly washing.
I also occasionally have to give him a nebuliser to loosen any secretions to make it easier for him to push them out of his stoma. That’s done by filling saline water into the machine and holding it in front of his face for around 20mins.
When he really struggles I have to use his suction machine which has an anchor shaped tube on the end and I put it in his mouth to get any saliva he may be pouching or any mucus that may be stuck at the back of his throat . Also when you are at home you don’t have disposable syringes you have to wash each one and sterilise the buggers.
Anyway back to the 2 day release home from GOSH I had to be trained to be able to do all of the above for him.
I stayed overnight on the ward to get a taster of what it involved which was amazing it was nice to feel like I was caring for him as any mother wants to do but I had been deprived of for so long.
I loved every minute and the next morning Roman came home for the first time EVER!!!
It was so overwhelming at first my house was inundated with people wanting to see him.
When I finally got him to myself I started doing all his bits for him. Made sure all his stuff was sterile and all surfaces where I prepared his stuff was too.
I took him out in his pram went shopping with him. I could finally be a normal mum.
After the two days he had to stay a night in GOSH just to see how he was doing but was discharged fully the next day. I had my baby back.
Unfortunately it only lasted a week his breathing got bad again, he had caught a chest infection, it was straight to my local A&E then straight to GOSH.
He was rushed straight to Squirrel ward again, where they stabilised him but the next day he deteriorated again. He ended up back on a ventilator in PICU. I’d only just got him back and he was taken away from me again I was in bits.
He was put on IV antibiotics for a week had another MLB and they ballooned the airway again and after 2 weeks of being at GOSH again he was back home.
He’s been back home ever since I think it’s been about 3months now. Roman goes back to GOSH regularly for his MLBs to keep his airway open.
My baby is now 10 months and doing so well. He’s behind developmentally but he’s getting there slowly and I couldn’t be more proud.
We are surrounded by an amazing community team of nurses, health visitors, physiotherapists, speech and language therapists and dieticians.
The past year has been hell for me and my family and my boys have been through so much.
I don’t know what the future holds but I want to set up this fund for my beautiful baby boy to ensure anything needs or requirements he has we don’t have to worry financially.
I would also like to donate half of whatever is raised to Great Ormond St which I will upload to Roman’s Just Giving Page.
Roman is a true fighter and I’m so lucky to have him. He deserves everything and more.
Thank you for taking the time to read this.
Lots of love, hugs & kisses Harley, Ashton and Roman x
Parents Story USA 16-06-2015
And when they get sick, all bets are off.
Luckily for most parents, the flu will come and go. One or two nights of staying up with a coughing baby are quickly forgotten as they get older.
For Ryan and Nathalie Hadlock, that is not the case. Blessed with three children, their youngest son Hayden has been in and out of hospital repeatedly since birth with a number of critical issues stemming from a malformed esophagus, which affects his ability to eat and to breath properly.
Now three years into the battle for Hayden’s health, the Hadlocks are beginning to feel the stress of caring for a son who is medically fragile. “He died in my arms,” Nathalie said, describing Hayden’s first moments after birth.
As she breastfed, the milk was not going to his stomach because of a gap in his throat.Hayden’s first weeks were spent in an incubator, and he underwent a six-hour operation to attempt to correct the problem.
“I couldn’t hold him for three weeks,” she said.In medical terms, Hayden’s condition is known as esophageal atresia and tracheoesophageal fistula, along with severe tracheomalacia, sleep apnea and asthma.In simple terms, eating doesn’t work.
Also, Hayden’s respiratory system is directly affected, meaning he requires constant monitoring.If he eats or drinks too much, he will vomit. If the food he eats hurts his throat or is followed by acid reflux, he, like a typical three-year-old, will not touch that food again. The list of things he will eat is growing smaller and smaller, dad Ryan said.
”Ryan and Nathalie are patiently waiting for the big phone call from the hospital to schedule surgery which could start to improve Hayden’s condition.The surgery was scheduled once before, but Hayden caught a cold, so everything was delayed. He is waiting for his turn on the list again. Once the procedure is done, the recovery time will take around six weeks, Nathalie said.Ryan and Nathalie have been relatively quiet about Hayden’s condition, not wanting to draw attention to themselves.“My pride got in the way,” Ryan said, explaining that the family is beginning to feel the emotional and financial toll of dealing with Hayden’s needs. “Everyone has fought this battle.” Ryan works as a truck driver, but needs to stay in the area, because he may need to rush home at a moment’s notice.
Nathalie stays with Hayden 24/7. “I know his breathing,” she said, able to distinguish which sounds are okay and which sounds mean trouble.Hayden takes five different medications at nine different intervals throughout the day. There is no margin for error, Nathalie said, explaining that some of the medications could affect Hayden’s heart if administered in the wrong dose.After between 50 and 70 trips per year from West Brome to Sherbrooke, monitoring Hayden’s medication, and checking his breathing three times per night, Nathalie reached her breaking point. She tried to lay down one evening and get some rest. “Come check on me,” she said to Ryan, “I think I’m dying. ”Familiar with the cost of calling an ambulance and unable to afford it, Nathalie drove herself to the hospital.
“Every time we get ahead, something else happens,” Ryan said, explaining the family’s decision to seek financial aid through a campaign on go-fundme.com. Maintaining a household with two healthy and active pre-teens, with the added costs of hospital trips and specialized equipment for Hayden, is too much for a single income. Ryan and Nathalie’s extended family has also helped as much as possible, but with Hayden’s upcoming operation and long recovery, the family is hoping to focus on the needs of their three children without the added stress of making ends meet at the same time.
To make a contribution, visit www.gofundme.com/swjr5ns. (Cut and Paste)
I was sent this today, and thought i would post the hole thing, because I feel you all would understand better.
VACTERL CHILD. (8-06-2015)
I am planning the second Celebration of Life for families with special needs kids in NH – by invitation right now to see how it goes and I came upon my letter to a young woman whose non-profit helped me put on Maddie’s Celebration last year. I have copied it and hope you can use it some way. Maddie has VACTERL. Keeping in mind the letter was from last year, to date, Maddie still struggles with eating, and GI issues, but many other health related issues have been kept at bay. I often tell people, when they with good will say how well she looks, that we have to force health on her. Every single minute of every day we have to watch our Maddie because infection could strike anywhere, or question has she eaten, had enough fluid, does she need a rest and so on. She is our little happy beauty and I can’t imagine life without our sweet girl.
“Dear ***, Thank you for you phone call. It made my day, my Christmas. I wanted to tell you that when I saw on the MSN news feed about how a young woman was helping people by fulfilling dreams, how inspired and filled with hope I was for my daughter Maddie. I was drawn in immediately because you have VATER or VACTERL association as does Maddie. I suddenly saw my little girl grown up and having a full life helping others and I thought, I would love to have you in Maddie’s life as a role model and hear your journey through life with this difficult, at times painful and often misunderstood condition.
While I am writing this, my little girl is lying on the couch, we spent the morning at Dartmouth to fix her tube since her flush wasn’t working last night, and the doctor put silver nitrate on irritated tissue under her Ce-colostomy tube. She is having a sick spell right now that has already lasted a week and a half. You know exactly what I am talking about.
Madeline Lucy was Dan’s and my four child and first girl. We kept the gender a surprise and I was overjoyed when, on February 1rst, 2009, Dan looked at me and said, “It’s a girl!” I still remember her on my chest and I was crying saying “I can’t believe it!” I knew God had a wondrous plan for the baby, because a week before she was born I had a vision – I was resting in my room and I suddenly woke up and saw the bedroom window filled with a beautiful soft light and a breeze was blowing the sheer curtains over the bassinet that I had set up for the baby. I suddenly knew, the baby was going to be Madeline Lucy. Her name means Tower of Light.
We noticed very quickly that something was wrong, but didn’t understand the severity as it was to all come in the days, weeks and now years to follow. Twenty-four hours after Maddie was born she was taken to Dartmouth Hitchcock in Lebanon, New Hampshire, because of finding a hole in her heart, then two days after that to Boston Children’s Hospital, because another hole was found. There she had open heart surgery to repair three holes in her heart. While I knew it was serious I did not understand the full extend of what was happening as my mind and my body had shut down. I was heart broken and physically ill for fear that my baby was going to die and I did not know how to handle this, death is not new to my family. My parents drove from Ontario, Canada to be with Dan and me, and Dan’s brother was with us in Boston. They knew that Maddie might not make it and didn’t tell me that the doctors had uncovered the third hole in her heart. After her surgery they all breathed a sigh of relief then explained the extent of the situation to me. My parents had to go back home after a few days and thanks to some tough love words from my mother, I had pulled myself together. The night before they left for Canada, my Mom and Dad were crying, because I was lying in the hotel bed sobbing, but my mom said, “Liza, please, you have to pull yourself together, that baby needs you, Dan needs you. I can’t leave you like this.” Sometimes you need a hug and other times a good shake and they were right, when I wasn’t near Maddie, her little heart would slow down, her oxygen would drop, but when I was close by kissing and holding her little hands, her heart would beat faster and her oxygen levels would improve. It was truly amazing.
My sister-in-law, was back in our home in New Hampshire, 2 1/2 hours away looking after my three little boys, the oldest only five and half at the time. She was having them stay with different family and friends, meals were coming in, then Dan’s parents came from Kentucky and stayed for three weeks. It is an ordeal to orchestrate someone else’s family when they are away for two months with a sick child.
One night when we came home to visit the boys, we got a call that Maddie had flat lined from choking on some milk, but was quickly revived. We rushed back to Boston and that day they discovered the Tracheal-Esophageal fistula. Dan and I had continuously questioned why she kept choking and this was the initial reason why I sent her to the nursery that first night of her birth. Follow your instincts and question everything, no matter how trivial it may seem at the time.
After her throat surgery Maddie was on a feeding tube, then a few weeks later was brought back to Dartmouth, so that I could be mother to my boys and my baby in the hospital. I could not stand being away from my boys, but I needed to be with Maddie. It was pure agony, but after eight weeks of being in the hospital Maddie came home. She had a special feeding bottle but after not nursing for nearly two and half months Maddie was full time nursing at three months and that was pure delight for me and her.
Three months after her birth she had her third surgery, which was to repair her anal fistula. This was very traumatizing, because of the long painful process after the surgery to expand the new anal opening. Maddie was on antibiotics to prevent kidney infections so her bottom skin would break down and bleed, she could not pass stool without medications, enema’s and suppositories. These were painful for her and I often felt like I was torturing my own child and felt helpless. We landed in the ER several times for kidney infections and blockage. She had a Ce-colostomy surgery this past summer and still has issues and will be going to Boston for them to look at her and give their opinion of how to help her. The surgery for the c-tube was difficult for Maddie to recover from, she spent the night vomiting and having diarrhea every twenty minutes and she was in horrible pain. I was wondering what kind of mistake I had made, but fortunately it got better. She does not have an appetite and nibbles like a little mouse on food and we often supplement with Instant Carnation breakfast, Ensure or yogurt drinks just to make sure she has nourishment. These are Maddie’s biggest struggles.
Winter 2009, Maddie had two hand surgeries in Boston; her left hand has four fingers as the thumb didn’t work, so they moved her pointer finger and made that her thumb. With the right hand they warned me that they might not be able to save the thumb, but I burst into tears and I was quickly consoled that they would do everything to save her other hand, which they did. I love her little hands and Nickle Creek has a beautiful song called “The Hand Song”, by Nickle Creek, that I sing to Maddie sometimes.
At this point my dates get fuzzy, because our life became a whirlwind of trials; Dan lost his job, I began work as an LNA, and became pregnant with our fifth child. Dan is now teaching at a small Catholic school and am I home again. Maddie in the meantime was to have Kidney Reflux, Tethered Cord Spinal surgery, and again her latest surgery was for the Ce-costomy. While at times it is difficult to have two other babies in tow, my two youngest, both girls have been a comfort to Maddie. She loves her Sisters.
There was a time when Maddie was about two and a half, and she choked on a stone from a pill-box ring I had just got. It was a little bigger than an almond. I knew she had swallowed it but she appeared to be fine, but I knew something was not right. Dan took her to the local ER and at first they didn’t see anything then they lowered the placement of the X-ray and there lodged above her trachea was the stone. They decided it was best to have Dan come home and get me and us take her so she didn’t panic and get it completely stuck. We had to drive forty-five minutes to get to Dartmouth as the local hospital was not equipped to take it out safely. It took all night and in the morning the doctor came out, a little serious, and that stops your heart for a second, but they had to push it down into her tummy, because it kept falling out of the grip of the tool. While we had been waiting, I told God, this is not how He was going to take her. After all she had been through for her to choke on a pill box stone, NO WAY! Sometimes, prayer just comes out like that and that is okay. God understands and He loves us more for our honesty. She passed it out and I kept it as a token of God’s love and just for the humor of it.
This is just a part of Maddie’s life. She is a beauty inside and out. She is my little hero and she has taught me how to suffer with love. Although she gets anxious going for her appointments, like today, hiding under the Christmas tree so I couldn’t get her, as soon as it is over, she is laughing and waving at people making them smile. I love to see her give people who love her the biggest hugs. Maddie has fleeting moments where she is trying to understand why she is sick…it is often in the bathroom, and she doesn’t feel good. I usually sit on the edge of the tub, to keep her company. One night not too long ago, we were both tired but she was feeling sick and need to be on the potty. She was teary and said, “Why do I have to be sick and not anyone else?” I told Maddie that she was very special and that God had made her this way so she could be like Jesus and help people get to heaven. She beat her little fist on her knees and said,”I DON”T WANT THAT JOB!” It was so honest and real. She got very silly and goofy a few minutes later, then suddenly says to me,”Mommy, thank you for my tube.” I thought my heart would burst.
She is loved by her wonderful Daddy. Every night they have a tradition, after she gets changed on our bed, she says, “Princesses feet must never touch the ground” and Dan always carries her into bed, now with her Sister who is two and a half on his back. The baby who is ten months will soon be requesting the same treatment. Maddie’s older brothers are incredible and I am not just saying this. I am so proud of my boys, one is 9, the other is 8 and the youngest is 6. They really love their little sisters and have been so concerned for Maddie. They have teared up at seeing pictures of her when she was in Boston,and have willingly sacrificed their birthday parties because of Maddie being sick a lot or having a surgery. They might have a fleeting look of disappointment, but they quickly recover and understand. I have been so proud of them, but take their disappointment to heart, and promise that they’ll get their reward someday, because love is always rewarded. I told the boys about the party that would be for them too, their faces said it all, “Wow, a party for us and our friends!”
Dan and I have had an amazing support group, we have been surrounded by our wonderful, caring families, and good friends who stood by and share our sorrows, struggles and joys. They are often daily a phone call away and I have family and friends who would drop everything in a heartbeat to help us. We have been very blessed. Maddie had an OT who was faithful about coming to our house from the time that Maddie was three months until she was three and did incredible work with Maddie and was emotionally supportive to me. Maddie now has wonderful pre-school teachers in Jame’s House Pre-School- she gets multiple therapies, but mostly lots of love and cuddles. I am so grateful to these wonderful women for there support and understanding of Maddie’s conditions. I admire and respect these caregivers, because Maddie is not a job to them, but a beautiful human being…”
I have often taken a ride on the hope and prayers of family and friends, because there are some days that I have difficulty accepting that my little girl has to suffer so, but I know we are held in the arms of our Creator, our Comforter. To quote the Good Book, “Are not five sparrows sold for two cents? Yet not one of them is forgotten before God…Indeed, the very hairs of your head are all numbered. Do not fear; you are more valuable than many sparrows.”
Thank you for letting me share the Maddie story! (FROM STEVE…… Thank you also x)
Marta TOF POLAND 20-05-15
Marta was born in 26.11.1994. Weight: 2100, height: 53.
In 29 week we found out that she has hydramnios. The doctor said that in this illness there can be some defects but I don’t have to be scared because it couldn’t happen. When she was born she was put to incubator, because of her weight. After an hour the doctor came and said that she has a defect ( coadunation esophagus with fistula trachea bronchial tubes, lower). She said that :it is difficult defect and she has small chances to survive, that’s why she has to be taken to another hospital. I didn’t know what she was talking to me. I decided to go to see her and I saw that she was intubated. The doctor said that: there is a possibility that she could die during travelling there. She said also that my husband can come back home. I was waiting long hours and when the doctor came to me and said that she has to have an operation and she doesn’t have one kidney.
I was exhausted and I decided to come back home. My husband was waiting in the hospital and after 5 hours, the doctor said that she is in critical condition and they did her conduit and fistula dribble. They said that there are no chances for here to make it. She was taken to ICU. That’s when my daughter’s ordeal started. Every day I tried to go to Poznań. Marta’s doctor never said when she was going to operate her. He said me that I can’t be with her and I should forget about her because she will die. But after 3 months (13.02.1995) I called to ask about my daughters’s condition.
There wasn’t any answer, when suddenly the doctor answered and told me that my daughter was operated 8 times and during these operations she was reanimated 3 times. He said that I should come here as fast as I can and see her for the last time. He said that money which we spent on Marta would be enough to buy a new car. He always suggested that money are important. I was heartbroken. We went to see her, she was on ICU. The nurse let us to see her. I saw in her eyes that she is fighting and she is exhausted. Head of hospital ward said that we should her go and we should pray for her. We went to home because our older children were waiting. After 4 months we got a call that she was reanimated again ad she has pneumonia. Next days were happy they called me that it is better and I can come because they organized a room for me on ICU and I can be with my daughter there. I had my daughter in my arms and after 2 hours something bad started to happen with her. One reanimation more and then I understood that this illness is really unpredictable. Then I decided to be with her all the time. She was getting better and the doctor explained me that he is surprised with her condition. He also said to me that in Poznan they don’t make operations of gullet. I said that I will do everything to save her. I was staying on ICU ward and then was ok but when we went on a walk she came back with pneumonia. In April we were placed on pediatric ward. We got the best doctor Szwarc, in the meantime she said that we can go home for few days on condition that I will have a nurse and a doctor there, and then we will go to Kraków.
We organized everything but it was really difficult because she was crying all the time and there were some problems with conduit, but we’ve managed. After few days we got the date of our travel to Kraków (19.09) by plane. Unfortunately the weather was not good and we were transported by an ambulance. New place and meeting with docent Bysiek, he promised me that we will stay here. He decided to operate but Marta must have 5 kilo. 20.11 docent said that he will operate on 25.11. He explained me everything. In operation will take part professor Grocholewski and docent Bysiek, but he doesn’t know what there will be when he will open her. At 7 a.m. Marta is in the operating room. I was really stressed and waiting for long hours. The doctor was giving me some information from time to time. At 4 p.m. I was sitting in the chapel when the nurse said that the operation is finished and the doctors went home.
Marta was on the ICU. Docent told me that coaptation was done but there were complications and they had to change position of stomach and she will be sleepy for 1 week. After a week they decided to disconnect Marta from respirator but it didn’t work out. When I came to hospital Marta wasn’t in her room I was thinking that she died. But she had bronchoscopy to see if there are any coccuses that were causing closing larynx and trachea but result was negative and doctors didn’t know what to do they decided to do an operation after one month and make tracheotomy. 13.12.1995 Kasia our friend from room died we were depressed ( she was 15 years old). My husband came and we went on ICU, Marta wasn’t there we were really scared. The doctor said to us that she is breathing and miracles happen, so we have to believe. After a week we came back on surgeon ward, Marta had solitary and docent came to us every day, he was really happy and said that it is getting better but Marta had lost her voice. It is improving.
She was fed by spoon but the gullet was narrowed. The doctor started to widen that with beads. He decided to let us go home but I have to learn how to do it. I came back home to my older children. At home 3 times a week I have to widen her gullet and Marta is crying and I think that I am bad mother because I have to do it. After a month we came back and we found out that they closed this conduit to early. Kraków sent us to Berlin on laser widen her gullet. Doctors in Poznań done nothing to help us. We went to Kraków every time. Marta was eating well and widen at home are done for 5 years. It found out that pharmacologic removal of reflux was right and Marta finished hospital treating at the age of 5 by operating her urinary reflux.
Alissa EA-TEF U.S.A 25th April 2015
Before you sit down and read this story, may i just add something. What you are about to read here, is from a young 25 year old Lady, who in my view is outstanding.
For me to be able to post this is an honour.
Someone tonight Quoted Winston Churchill, to me, I would like to quote it again, because this lady has done what Winston Churchill said, ‘ when going though Hell, keep going, Never give up!
This young lady has my respect 100%
Ok, so I guess I just want to share a little.
Most of you may not read this, but it’s good for me to explain it, in pieces and bits to people it’s difficult to understand, so here it is…..
I was born without my esophagus, which in medical terms is called Esophageal atresia. Along with the atresia, my heart is on the right side of my chest, rather than the left.
When I was 2 years old I had a surgery called Colonic Interposition, which is when part of the intestine and part of the colon are brought up and used to make a “bridge” if you will to connect my neck to my stomach.
Along with that until I was two, as well as after the surgery done at 2 years old I had what’s called a J/G tube, a feeding tube and a vent tube.
Now, from 2 years until I was 21, the colonic interposition seemingly worked quite well.
When I was 21, in 2011 however I started presenting horrible and uncontrolled pain and nausea. Seeing as I don’t have a real esophagus with the muscles to throw up, having nausea with the inability to throw up makes things a million times more difficult to deal with. Along with the lacking muscles to throw up my esophagus makes a right hand turn in front of my trachea, which we’ve found out makes a simple Endoscopy a nightmare. For example, the time I ended up intubated in the ICU just from and Endoscopy.
Anyways, going in and out of the ER countless, literally hundreds of times Yale finally hooked me up with the head of Liver Transplant, now I don’t need a transplant, but along the journey they found that I had never formed the portal vein in my liver, which caused varicose veins to grown in and on my stomach (gastric), as well as on my liver (hepatic) and all over my gallbladder, squeezing it into a malformation.
In December 2011, having started getting sick in August of 2011, the head of liver transplant did and open Coly (removal of the gallbladder)
This is usually a very simple, non-invasive procedure, well not for me.
With the varacies it was dangerous as you can bleed out from nicking one incorrectly, as well as with my previous surgeries, the surgeons said there really was no way to tell what was going to be inside me when they opened me up. To make matters way worse, I have a super high tolerance to pain meds, and I withdraw way quicker and pretty violently than most people. I was on fentanyl patches every 48 hours, and the insurance kept screwing it up, because it’s supposed to be every 72 hours for normal people, but it was going through my system at a rate way faster. So the insurance, as makes sense, needed confirmation and request from my surgeons. As well as the fentanyl patches, I was on dilaudid push (IV) 4 MGS every 4 hours with 50 MGs Benadryl push (Iv)
So all that said, at that point in time, liver transplant thought that was the only problem, well it wasn’t until hospital ER visits started happening at least every 48-72 hours with dehydration, heightened pain, and massive weight loss, I went from 130 pounds to 91 pounds; not until all this as well as my mum begging for what’s called a Hyda Scan, or Gastric Emptying Test to get done, which most drs put their nose up to did they FINALLY get GI surgery involved saying oh god, we think it’s anatomical Gastroparesis!
Trying everything from Domperidone (not even legal in the US) to Reglan, to Zofran nothing helped. I just kept dropping weight.
So GI surgery inserted what’s called a PICC line, which gives continuous IV access as well as IV nutrients. However, this was not easy either because my hearts on the opposite side, so it had to be done differently than normal, and more delicately.
Something wonderful happened along the way though, I woke up in ICU again for the drs to tell me, they had no medical explanation for the fact that my portal vein causing the varicose veins which posed danger to bleed to death, miraculously formed itself, which meant that I didn’t need a portal shunt or a liver bypass. There have been many miracles along the way, regardless of the hardships.
So after getting the PICC, still in and out of the hospital for months, July 18th, 2013 comes along, when heads of GI Surgery, head of Thoracic Oncology, and Liver Transplant decided to go in and open me up and fix when they thought was wrong, not knowing what to find inside.
It was an 11-12 hour surgery which included re-doing my original colonic interposition, cutting away 6-7 inches of the mock esophagus I had, and re-attaching it in various places along it’s tube, as well as moving the entrance to my stomach, which was placed at the bottom, so by moving it closer to the top they hoped to make it a more gravitational pull to digest.
Something they did not know before going in was that I had what took four hours to remove, massive scar tissue round my heart.
They again, placed feeding tubes and a G tube for venting, which I got out last May, 2014. Just under a year after surgery. Although it’s not April 2015, and the holes from those tubes aren’t closed, they are closing very slowly.
After that surgery it was hell with the medications they had me on again.
Fentanyl 75 MCGS every 48 hours, along with 50-75 MGS of Benadryl push, along with Dilaudid push 4MGs every 4 hours or more, as well as 8 MGS of liquid Valium every 8 hours….I put my mind to it and finally found in house (hospital) Drs who safely titrated me off those. It took from November 2013 to March 2014 to find a Dr understanding and willing enough to do so. My body just doesn’t handle narcotics well at all. Proper usage, I was still withdrawing so they’re just not good for any sort of long term use for me.
Now, it’s April 2015, having ups and downs but man I look at some of the pictures from even just a little over a year ago, and things have come a long long way. I still meet Drs who just seriously don’t get it, but my surgeons are always there. They’ve been the ones who’ve stuck by and not faltered for me in the medical community.
Malin TEF SWEDEN
Hi! Thank you for letting me join the group, here’s a short presentation of me: ( Please Note This is a hidden Group attached to all this, If interested Talk to BEV STARK )
I was born with EA/TEF 25 years ago and 6w ago my son too arrived with EA/TEF at the same hospital (same nurses at the ICU and same due date!). We both have completely isolated EA/TEF, no other syndromes or conditions.
Since it is the first time this happens in modern time (with the recent huge leap forward in DNA-research) they have started a DNA-mapping on me, my son and fiancé.
And since both me and my son definitely have the necessary mutations that causes EA/TEF they are confident that in 2 years (the time it takes for the DNA-mapping to finish) they will have found the answer to what causes EA/TEF from the mapping of our samples. So it looks like some answers finally are around the corner waiting for us EA/TEF people! smile emoticon
That’s a little about me, thanks again for letting me into your group And oh, a heads up, while I do speak english it’s not my first language (Swedish is)..
So I might have some weird translations going on especially when it comes to medical terms .
Tristan Barry’s Story so far USA
Trying to write our story is a difficult one. My husband and I worked for a long time and lost another baby just trying for the son we have today. Sometimes we feel guilty that we had a baby on purpose but we had no way of knowing what the future held.
Less than 1 year after we got married, we learned we were expecting our son. Pregnancy was more amazing than anything I had ever experienced once we learned to ignore the “you shouldn’t be walking” and the “wow you got huge” comments from rude people. Don’t even get me started on the grabbers.
At the 20 week scan his doctors noticed a single umbilical artery and ordered more scans. We are understandably concerned but after researching SUA we told ourselves there was a high likelyhood that our baby was fine and not to worry. Over the next 10 weeks there were multiple scans, EEGs, etc. With no signs of problems we thought we were in the clear. We couldn’t have been more wrong.
At 30 weeks (10 weeks early or 7 months along) my water broke and I was taken to the nearest hospital. After 12 hours of all natural zena style labor, our son was born. We thought it odd that his cry seemed medlodic. It was unlike, the cry of any baby we had ever heard. After a minute the doctors brought him to me long enough for me to kiss his forehead before wisking him off to NICU. I was releived that he was okay. We knew nothing about prematurity or the risks involved. We were totally oblivious to the many things that can go wrong.
We learned a lot. In fact, we got a crash course that Friday morning. After they left, I had severe complications and well, let us just say that I should not be here.
My husband came down to ICU so scared to tell me about our son because his mommy was so close to death. I was. I felt it too. When he told me what was wrong with our baby it was greek to me. We didn’t have a camera so a friend gave us a little cheapo cam corder. The first time i saw him since his birth 2 days before and he was on respirators and he was covered in wires and iv lines. He looked like a human pin cussion. I hurt for him and wanted to be there but I was unable to move due to a hemoglobin level of 2.
We didn’t even have time to research his illness before the surgery was done. His poor daddy went from one bedside to another for 2 weeks not knowing which of us would survive. I remember asking, “so they fixed him the right?!” Yes was the answer the doctors gave us.
When Tristan was 1 week old, I finally got to see him from a wheelchair. He was in an open crib with a chest tube and ventilator in his mouth. The nurses said he was just coming off the meds that kept him sedated. As soon as he heard my voice his eyes opened and he reached for me. I had never been no so scared as in that moment.
Then as time went on we learned of his other problems, heart defects, solitary kidney, lung colapses, GERD. They had to dilate the surgery site just to put in an ng tube under florscopy to be safe. Most days his blood gases were just awful. Slowly he got better and better. Tristan beat the odds when he should have been a victim of them.
After 2 months, he came home with a host of equipment. 3 days before his first trip to the er for aspiration. After 2 weeks in picu and a diagnosis of severe GERD via 24 hr probe test. He went home on more medications. 7 days later, we were back at hospital.
This time we came to Joe Dimaggio’s Children’s Hpspital. The meds worked, sort of, but he had a reaction and had to come off them. This time he stayed for a month. Test after test was done and they thickened his feeds with cereal. They tried to send us home but we were concerned about thicker liquid in his airway and refused to leave. Sure enough, he vomited into his airway the next day and we had to watch 4 nurses working tirelessly to get him breathing again. I can’t explain what you feel when you watch your baby turn blue and go limp. It isn’t fear. It isn’t pain. It’s utter helplessness. You feel your self shrink back and pray as you watch people do thier best to bring your child back to life.
After this, we decided to go ahead with a nissen fundopication with a gtube. Not an easy decision but when faced with a blue or worse… dead baby, there was no other decision to make. Many know the woes of tube feeding. Much is hard to remember except that parents of normal babies who complain of sleep deprivation in our presence receive a verbal slap automatically.
Tristan had multiple proceedures due to gtube complications, a dual inguinal hernia repair, stomach flu, colds, and finally cdiff by age 1. I know it is alot but we were just glad he was still here.
Tristan has had a very difficult time with gaining weight thus far. When he hit 18 months and weighed only 13 lbs, we tried something new. A blended diet. It was a godsend. The nissen left Tristan with constant retching and within 2 weeks,
it had all but stopped. He began making milestomes. Even walking.
Now he is 2 and half. He just made 23 lbs and that is awesome. He still gets all of his calories by tube but he is also up to 2 cups of cheerios and 2 slices of bacon a day. That is huge for him!
Tristan still ends up in picu every time he gets a cold. He has Asthma, Lung disease of prematurity, heart murmur, solitary kidney, scoliosis, pectus carinatum, sensory processing disorder, global developmental delays, VATER Syndrome, and is a suspected autistic. He is our pride and joy.
I have come to call EA/TEF parents the special ops of the parenting marine corps. We became far tougher than our parents had been. Far tougher that we ever thought we could be. We know the feeling you get each time you hand your child off to a surgeon and the fear of the unknown. We know levels of sleep deprivation known only to torture victims. We know how empty we feel the day we no longer cry while holdingour child down for an iv or blood draw.
We smiled when people said they just didn’t know how we did it. Whatever! Neither do we!
I joke often that it isn’t like there is an exchange program.
We are used to telling people that his cough is fine and that it normal for a pack a day smoker….. they never laugh but we do. If there is one piece of advice I can offer for anyone just starting out in this type of journey…..
Learn to laugh at anything you can. You will go crazy if you don’t.
Besides, our little TEF/TOF warrors are watching us and learning how to be strong.
We look forward to Tristan doing better as he gets stonger and education others that fixed does not mean cured. Not by a long shot.
Life is great and every day is a blessing. No one knows that like those who have been where we have.
Marshall Shill is the archetype of a successful scholar athlete who has achieved his successes despite substantial and overwhelming adversity.He began his inspirational journey with a rare birth defect called a tracheoesophageal fistula; and, although surgically repaired, he has experienced problems with swallowing throughout his life. He now conscientiously chews each bite, and must chase his food with water.
Marshall is described by one teacher as, “… an exemplary student, a hard-worker, amazing athlete, great artist, and on his way to being a phenomenal young adult”. He is a prolific and diverse school and community volunteer, and has worked diligently to maintain a very respectable 3.69 GPA at MUHS.
He is a National Honor Society member, a MUHS Representative at Boy’s State, the MUHS Site Commission Student Representative, a Vice President of the Cottonwood Youth Commission – logging 150 volunteer hours, a proactive and vocal member of the Anti-Defamation League, a member of the Captain’s Club, a Verde Valley Youth Baseball Camps and Youth Football Clinics volunteer, and was a recipient of the Governor’s Volunteerism Award.
Marshall’s stellar athletic career has been wrought with adversity and set-backs. He played football his entire freshman year with a fractured ribs, and a high ankle sprain limited him to half the season as a sophomore. He was a QB. Prior to his junior year he had right A-C joint surgery, and then later a right shoulder separation. Despite not being able to play, he never missed a practice, helping out wherever needed.
Determined to play, Marshall taught himself to throw and punt using his left arm and foot. As a senior he played DE, and now as a left-footed punter made AIA D-III All State as Punter. Marshall was also a 1st Team All Region in Baseball.
Marshall Shill’s successes despite overwhelming adversity epitomize the characteristic of Courage, as espoused by this Shaw Award. In addition to receiving this special award, he is being recognized as a Top-33 Scholar Athlete by the Chapter.
17-02-15 Danielle Ea-TEF Mum
I posted this a couple of years ago and it still applies to our family and many friends we know going through difficult times and dx’s for thier children. I love this little poem.
Welcome to Holland
I am often asked to describe the experience of raising a child with a disability – to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It’s like this…
When you’re going to have a baby, it’s like planning a fabulous vacation trip – to Italy. You buy a bunch of guidebooks and make your wonderful plans. The Coliseum, the Michelangelo David, the gondolas in Venice. You may learn some handy phrases in Italian. It’s all very exciting.
After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, “Welcome to Holland.”
“Holland?!” you say. “What do you mean, Holland?” I signed up for Italy! I’m supposed to be in Italy. All my life I’ve dreamed of going to Italy.
But there’s been a change in the flight plan. They’ve landed in Holland and there you must stay.
The important thing is that they haven’t taken you to some horrible, disgusting, filthy place, full of pestilence, famine and disease. It’s just a different place.
So you must go out and buy a new guidebook. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.
It’s just a different place. It’s slower paced than Italy, less flashy than Italy. But after you’ve been there for a while and you catch your breath, you look around, and you begin to notice that Holland has windmills, Holland has tulips, Holland even has Rembrandts.
But everyone you know is busy coming and going from Italy, and they’re all bragging about what a wonderful time they had there. And for the rest of your life you will say, “Yes, that’s where I was supposed to go. That’s what I had planned.”
The pain of that will never, ever, go away, because the loss of that dream is a very significant loss.
But if you spend your life mourning the fact that you didn’t get to Italy, you may never be free to enjoy the very special, the very lovely things about Holland.
Written by Emily Perl Kingsley
Maureen Adult Ea-Tef
Steve, You asked me to tell you my story. Here it is.
I can tell you I am 64 yrs old. I was born w/o and esophagus. I had the top and bottom but nothing in between. By the time I was 3 yrs old I was “connected”. The doctors took part of my colon and made an esophagus.
I had this done at the University of Minnesota. I was the second patient ever to have this done here. I was fed through a tube in my stomach for the first 3 yrs before I was connected. I have had relatively few problems with this.
11 yrs ago I developed a hernia on my diaphragm which I had 2 very major surgeries to correct but was never actually fixed. This was caused when they originally made the esophagus, they made a hole in my diaphragm to connect the ends of the esophagus and over the years the hole expanded and now my stomach sits on top of my diaphragm.
This causes me to have a hard time breathing. The last two surgeries didn’t fix it.
I still manage to live my life normally. I manage to grow up normally, marry and I have two grown daughters and a granddaughter. I live in USA.
6-1-15 Craig TOF DAD UK
Not the greatest at this sort of thing but I’ll give it a go . . . After laying our first TOF/TEF child to rest back in 2004, Paula, my partner of 21 years & myself decided to take a break from everyone and everything and took ourselves off to Spain. A few weeks after our return, we discovered that we had bought more home than we thought? ……
Paula was carrying another little mini-me inside her. Everything was going great up until around the 34 week mark, when a Mothers 6th sense told her that all was not well. In hospital we was told she had everything from Braxton hicks (or however you spell it?)to a water infection. After the loss of our 1st child Paula was insistent that she was going nowhere, so they found a small room for us to stay and wait until she was feeling a little better.
After a few hours of me rubbing her back and putting pressure on various areas of her body to help with the pain, things just got worse. On examination, it was discovered Paula was now in labour. Before pain meds were able to even kick in, Cayden Riley Eagleton was born. He was not breathing and no heart beat could be found. After what seemed like forever, they got some signs from him and he was rushed out of the room before we could even say hello to him.
I still to this day have flashbacks of Paula begging them to not let her baby die and the memories are still so fresh in my head that I cry as I write this. They later gave us news that he was stable and in ICU. I was soon after asked to leave as it was very late and everybody needed to get some rest. I didn’t even make it home when I received a phone call asking for me to return as all was not well and that baby was being prepped for a move to G.O.S.H. This was to be my introduction to parenthood, TOF/OA and situs-inversus (mirror image dextacardia) All his internal organs were back to front, his ear was only partially developed, and the suction tube that was placed in his mouth just stopped and was unable to be fully inserted.
We were now going to be residents in one of the world most famous children’s hospitals for the next 7 and a half months. After many ops including TOF/OA repair, 4 fundoplication attempts, a diaphragmatic hernia op and dozens of other procedures to relocate body parts so as to not cause obstructions to other body parts, we were now able to go home.
And that’s where the real work begins…….. sleepless nights with night feeds, tubes being pulled out. Trying to pacify baby with severe reflux symptoms. And the list goes on and on and on. Cayden will soon be coming up to his 10th Birthday this January the 21st and I thank everyone whoever had a part to play in giving me the chance to be a Father to my beautiful baby boy. He is to be our only one, as both Mum & I know we haven’t got it in us both physically and emotionally to go through it again. It tore my sole out to see a baby/small child in the pain my boy was in at times. And I would never want to run the risk of ever having to witness all that again.
I thank you Steve, Bev, and the rest of my extended TOF family for the love & support their group has given me since it was founded. – God bless you all xxx (Craig Steve here your very welcome I look at it as we are one big Family ….. and its good to have you here, even if this made me cry a wee bit, God Bless you Craig)
4-1-15 Lyza EA-TEF MUM
Niko was born April 9, 2014 after a difficult pregnancy. I was induced at exactly 38 weeks due to a kidney stone I refused to take pain meds for and my blood pressure spiked.
Niko was born before the doctor could get to me, only 5 pushes. He was 6lbs 4oz and had a full head of red hair. Right away, he started turning all sorts of shades of blue and grey.
I wasn’t allowed to even hold him. After 3 hours of nobody knowing what was wrong, I called my step son’s paediatrician to help. She diagnosed a heart defect and sent him an hour away
to a trauma hospital. With in 10 minutes of arriving there, They diagnosed EA/TEF and sent him to a Children’s Hospital.
By the time I fought my way out of the birth hospital, my precious boy was already being prepped for surgery. They did a new style of surgery on him and were successful.
On his 2 week birthday, he was released to go home and I was told he was fixed and good to go.
It was a lie. By the time my son was 6 months old, He had endured 7 surgeries for subsequent issues like reflux, blue spells, strictures and tracheomalasia .
He is also now fed by a tube in his tummy. He is still at 9 months old, unable to swallow. His oesophagus just doesn’t work and formula just sits there. We are still working on that.
Niko also has a few heart defects that we are in the process of learning the severity.
TEF babies are fixed but never cured. My son is half fixed. He almost lost his life due to lack of awareness, and is having complications due to lack of awareness.
Please start talking about this birth defect So that future generations don’t have to suffer from this!
3-1-15 BEV a TOF MUM in UK
Bailey is now a teen TOF
He was born in Oct 2000 . 42 weeks + 5 days ,over due ! Our 9lb 59cm beautiful boy with his full head of dark hair was just like his Daddy . It was fri the 13th!
Bailey wasn’t breathing when born but soon was ok !!! He went into a room with me I couldn’t move still frozen from the emerg c section .
He wasn’t good at 36 hours and a lot of me shouting he was diagnosed with TOF/OA and enforced phnumonia the silly STUDENT midwife had fed him ? His lungs were full he was suffocating .
9 hours later he was transferred to the RVI at Newcastle .
At 72 hours bailey had his Tof repair and from there he thrived fed at 10 days home at 2 weeks . 12lb at 4 weeks fantastic nature amazing .
At 4 months he was diagnosed with silent reflux . The reflux was bad and wrotted all his teeth they were removed aged 2yrs when he was in theatre having a nissen fundoplication full 360 wrap
tight too . Bailey always ate orally and ate most things too . But after his fundo he had terrible dysmotility and still does . He cannot tollerate meat and is still very fussy with what he does eat but he
grows well and gains weight always . He’s had several stretches and been to theatre 38 times mainly endoscopies but the rest are fir bronchoscopy fundo and his repair .
Bailey had a heart murmer but he’s apparently grown out of that ?
He is also presently bring investigated fir POTs or vasovagal episodes but that’s another fight . He collapses goes grey his heart rate either goes mad or drops suddenly ? Also stops breathing and
seizures ( reflex anoxic seizure) but always these are different and no warning !
Bailey is a well adjusted young man who we are very proud of . He doesn’t like to dwell on any of his conditions . When he was 10 we found out how bad his TM is chronic the worst 3 specialists have seen !
His trachea is permanently collapsed over half way across ! This slows him down in sport but he tries paintballing basket ball etc but he’s tired after and that often causes his collapses ?
Both my boys and I have a genetic immune dificiency MBL mannose binding lectin this doesn’t help with the many chest infections bailey has it stops the innate immune system kicking in so we all get
poorly quicker and for longer ! Bailey worst of all with his other issues .
TOF is here for life bailey is fixed but he’ll never be cured . Our next adventure is into adult care !! That fills me with dread local care is not good . We are proud of bailey and all he had overcome always trust
your intuition as parents you are right in all your decisions because YOU make them for your child your most treasured possession .
Proud to be the mum of a TOF x
INTERESTING……… A Gentleman of 81 under goes a massive op in 1933
I have meet Someone called Mike Evens who lives close to me, he said he was born this What I had, in 1933. Mike Evens is 81
I asked him to write something for here for me, this has just turned up, ( after looking it up, its not the same but I feel that you might like to read what he wrote to me, giving his age. )
( I can’t tell you very much concerning my own experience as I was barely two weeks old at the time, I think I was operated on at Putney Hospital London around the 28th April 1933,
I was then transferred to a nursing home, where the Ward
Sister fed me on Milk and Brandy. From then until approximately 40 years old, I suffered from very severer indigestion, but since then I have been fine. (Sorry I haven’t got any better details than these)
This is what Mike had, I have posted this because of Mike’s age and to allow you to think that this Gentleman under went a massive op in those days, and has made it to the ripe old age of 81
This is a wee bit of what his op was.
The outlet of the stomach into the small intestine is called the pylorus. Stenosis means a narrowing. Pyloric stenosis means a narrowed outlet of the stomach. It occurs in some newborn babies.
Food and drink pass down the gullet (oesophagus) into the stomach. Here they mix with acid and are partially digested. The stomach then normally passes the food and drink into the small intestine to be fully digested
and absorbed into the body.
A narrowed or blocked outlet from the stomach (pyloric stenosis) can lead to a serious illness unless it is treated.
28-11-14 Seren’s story Debbie UK Parent
Seren was born, one day overdue, at about 5 pm one day at the end of February 2014 after a tricky pregnancy with pretty much weekly ultrasound scans for the last 12 weeks. However these didn’t pick up her TOF/OA despite a few warning signs as I look back now. She was just over 7lbs, with lots of dark hair and was grey and bubbling at the mouth and only able to whimper, not really cry. Seren was whisked away to SCBU after about ten minutes.
By the time I got there an hour later, she had been x rayed and TOF/OA diagnosed by probably the kindest pediatrician in the world who was already on the phone arranging her transfer to Birmingham Children’s Hospital. We sat in SCBU with her until 12 midnight when the Neonatal Transfer team took her to Birmingham in a transport incubator that looked capable of space travel.
Seren had her repair surgery at 44 hours old. The 8 hours we waited for her to come out of theatre were the longest of my life. We have been incredibly lucky that she recovered well. Subsequent MRI scans have shown a spinal problem which may or may not need to be treated at some point but she is newly crawling at now 9 months old and is full of life. I have no adequate words to thank the medical teams who looked after her, nor the whole of the TOFS community who I have leaned on for support and advice this last 9 months.
28-11-14 Natalie From Canada.
21-11-14 Mara Lee Weskamp Ebert A Proud USA MUM Here is Logan’s Story From U.S.A
Look who accompanied high-school senior Logan Ebert to his first day of school last week.
The highly regarded and ingenious Dr. John Foker didn’t fly from Minnesota to make sure the 17-year-old came through his big day OK. No need for that. Though Logan started life with a couple of critical birth defects,
he’s now as strong and healthy as any kid at Sonoma Academy.
Foker attended the school assembly that launched the new school year because Logan was picked to deliver the student convocation address. He and his folks, Sonoma’s Fred and Mara Lee Ebert, invited the Harvard-trained heart and
thoracic surgeon to come hear it.
After all, Logan’s speech thanked Foker for the gift of a normal life. And it held him up as an example of what can be done by someone who chooses to act rather than let an opportunity pass by to challenge oneself.
Logan told his nearly 270 fellow students he was born in Colorado without most of his esophagus, the tube through which food passes from the mouth to the stomach.
Logan was a newborn receiving nutrition through a tube when his parents hit the library at the University of Colorado’s medical school to research the available surgical remedies to “esophageal atresia,
” among them moving the stomach up into the chest, or fashioning a length of esophagus from an extracted piece of colon.
The Eberts were unhappy with all of them and their potential long-term negative effects. Those “weren’t the options my parents were looking for,” Logan told his schoolmates.
“Without the help of the Internet, my parents spent weeks poring over the university’s medical library books searching for a better answer,” he said.
Mara Lee Ebert discovered an article written by Dr. Foker, a University of Minnesota surgeon and professor who mere months earlier had had pioneered a difficult but apparently successful surgery performed on a
baby boy with a very similar defect as Logan’s.
The baby, only about two months old, was missing most of his esophagus. As Foker commenced surgery on him, he thought he would connect the two separated ends of esophageal tissue.
But when he peered into the baby’s chest and saw what he had to work with, he quickly realized the gap was too wide. In his speech, Logan described that moment in the surgery.
Dr. Foker, he said, “had to think of something fast. Like a life-saving new medical procedure in two minutes fast.
“So he stopped, ate a Snickers bar and did just that. In two minutes, ignoring the naysayers in the room with him, he took his groundbreaking medical procedure to a new level and devised an entirely new method … “
After the speech, Foker, a tall and understated man of 76, said the kid had it essentially right.
What he did with that baby was to place sutures in both ends of the incomplete esophagus and apply tension — not to stretch the tubal tissue but to stimulate it to grow.
“If it’s not connected, it doesn’t get the signal,” Foker said. The tension provided the biochemical signal for the newborn’s incomplete esophagus to grow.
After only about two weeks of tension, that first child’s esophagus grew enough for Foker to surgically connect the ends. “Nature does it all,” the surgeon said.
As soon as they learned about the new surgical remedy to severe esophageal atresia, Mara Lee and Fred Ebert, then residents of Denver, asked several doctors in the area for their opinion of it.
“Four out of five doctors told us it was ludicrous and would never work,” Mara Lee said. But she and Fred agreed they were impressed by Foker, the depth of his experience and his clear desire to forge advancements in his craft.
Logan wasn’t yet nine weeks old when they took him to the University of Minnesota to become the second patient to undergo the Foker Method. The doctor gives them much credit for the decision to allow a stranger to try to
mend their baby with a new and not yet accepted procedure.
“In my mind, it takes a lot of courage,” he said.
The surgery on Logan went well. In just 10 days, the tension caused Logan’s esophagus to grow enough to be connected, Foker said.
Foker also repaired holes in Logan’s heart. The baby recovered fully from the surgeries.
The grateful young man who stood before his fellow students, their parents and the entire faculty on the first day of school stands 5-foot-11 and competes in basketball and track.
“I do feel really lucky that everything worked out,” he said.
His friend the surgeon has now performed or assisted with more than 200 Foker Method surgeries. He has taught the procedure at several hospitals around the world and he co-created the world’s first center specializing
in the technique at Boston Children’s Hospital.
He responded humbly in the Sonoma Academy gym when Logan’s announcement of his presence brought a prolonged standing ovation.
After his patient’s speech, the doctor said, “If at my age you can be inspired, it was truly inspirational.”
19-11-14 Jacqueline U.S.A Mum
We are in Pittsburgh, PA. Luckily, we live about 20 minutes from the Children’s Hospital. We had no idea that anything was wrong until Hayden was born. He had respiratory distress when he was born and they took him directly to the NICU. We found out a few hours later that he was a TEF and is dextracardia (his heart is mirror image) he would be transferred to Children’s Hospital as soon as he could be transferred. I was told to get to the NICU and hold him until he was picked up by their transfer team. The next day, he was operated on. I am not sure what happened in the operating room, but they had to break sterile field and he came back on several drips and heavy narcotics. They extubated him too soon and he also stopped breathing and had to be resuscitated. Things got really bad and his lungs continued to collapse. He had fluid around his lungs that was causing the collapsing. To get rid of the fluid they had to insert three chest tubes into his body, he was dumping fluid. It was unbelievable how much fluid came out of his tiny body. I thought it would never end. The tubes would get clogged and his heart rate would sky rocket and his lungs would collapse again. This went on for about a week. Then he went in for emergency surgery to see what was going on inside. They ended up cleaning out the fluid, putting in another chest tube and fixing a tear in the initial repair. After almost a month, they had to increase the size of his breathing tube. Hayden was not happy with his new breathing tube. He ended up arresting twice in one day because of little things, such as the nurse took his temp so he decided to bronchospasm where he clamped down his lungs and they could not get air into them. After that day the nurses tried to bother with him as little as possible. Only my husband and I could touch him, but very little. After a few quiet days, he began to turn around and started to get better. He was off of the ventilator about a week later, he was on the ventilator for 38 days. I was unable to hold him for 33 days in a row. I held his hand through every poke and prod. Hayden was on fentanyl for quite some time. They had to wean him off of this by giving him methadone to help with withdrawal. As far as eating, he was on TPN for a total of 60 days. There was always a reason for feeds to stop. He never had a fair chance of learning how to drink from a bottle. He had an NG tube placed during his first surgery, so that is how he began feeds. We would start the bottle at about 5 cc’s get up to about 20 and then have to stop for one reason or another. Hayden ended up getting a staff infection and ring worm while in the hospital. After 50 days in the NICU, they moved us to the surgical floor. We were very reluctant to move down because of all we had been through. I was trying so hard to get him to eat full feeds with the help of OT
and Speech. The surgeons wanted to put in the g-tube and perform a Nissen and I was totally against it. While we were working on feeds, Hayden had another episode and the doctors thought he had aspirated. Feeds were stopped immediately. After a week of NPO, we found out it was another infection, he did not aspirate. The doctors said if I went through with the g-tube surgery, I could be home in a week. My husband was desperate to get out of the hospital and I reluctantly agreed. He had the Nissen/g-tube a week later. He was white as a ghost after surgery and the nurses and doctors on the surgical floor thought it was normal. They never checked his blood levels. After two days, he was rushed back to the NICU. He was severely anemic and needed a few blood transfusions. There was also internal bleeding in his abdomen. The doctors in the NICU couldn’t believe his condition and promised not to send us back to the surgical floor. It took awhile to get him back to “normal”. Then we were sent to the NICU step down unit. We were there for two days and ended up back in the NICU. This time he freaked out every time he ate. No one could figure out why. I would hold him as he screamed and thrashed. It was horrible. One doctor actually told me that he just wanted to be held. I knew my baby and could tell there was something seriously wrong. Finally I asked the doctors to do an ultra sound on his abdomen. This is when they found that he is 1 of 3 babies to ever have gall stones.
They think it was the TPN that caused the stones. This was treated with heavy antibiotics. During this time he also was diagnosed with hypertension. A few weeks later during rounds, the doctors told my husband that we would be leaving the hospital the following Monday. We never thought we would hear that news. He had to turn away because he just started crying. We moved to the Children’s Home which is a place that teaches you how to use your home equipment. We passed all the tests and were discharged in less than 48 hours. We were determined to get home before Thanks giving. We went home the day before. We came home with a feeding pump and an A&B monitor. I worked really hard to get off of both. After a month, we no longer needed the monitor nor the feeding pump. I had him drinking about 4 oz. five times a day and began to introduce pureed solids. He was very finicky and everything had to be just so to get him to eat. I would stand on my head and cried often. He was barely on the growth chart but was showing growth. He was taken off of Zantac at six months even though he was diagnosed with GERD. He did well. We saw a physical therapist for about one and a half years because of his delay in gross motor skills. He did exceptional. Walked at age 14 mos. We also saw an OT and a nutritionist to help with feeding. We followed up with several specialists for awhile including nephrology, surgery, cardiology, feeding experts, pulmonology and his pediatrician. At this time, Hayden is 3 years old. He is on the growth chart. He is beautiful and intelligent. He goes to pre-school. He drinks and eats anything that he likes that is. He has never had to be dilated. He has never had food stuck. He is only seen by pulmonology because he was hospitalized once with RSV when he was one and a half. He does not take any medications. If you didn’t see the amount of scaring on his little body, you would never know how hard he had it. It seems like a lifetime ago. I hope my story helps give you hope.
15-11-14 Christine’s Husband was born with EA-TEF and this is her STORY with TWO Children to cope with
Daniel was born at 39 weeks. Since my first baby had TEF I had several ultrasounds to keep an eye on him. I was told they could not tell whether he had TEF or not. I was diagnosed with IUGR and they wanted to induce but I went into labor on my own. I was expecting that he would be born healthy as I could not imagine having another baby with the same birth defect.
When Daniel was born I tried to nurse him. He coughed and turned a little blue on me. I instinctively pulled him off the breast and handed him to my friend that had come for support. She in turn handed him to the nurse to be evaluated. I had told my midwife during my pregnancy that if there were any problems I wanted some bonding time with him. I had felt stripped of that privilege with my first born.
I signed paperwork for Daniel to have a chest x-ray and permission to transport to T.C. Thompsons Children’s once again. The nurses let me hold him before they put him in the incubator for transport. Since both hospitals were now connected with each other I got to be transferred by ambulance to close to Daniel. I was immediately impressed with his surgeon who was a new mom herself and just returning back to work. She was fully supportive of me pumping and freezing my milk for him as she was still nursing her daughter. I think Daniel had a long gap TEF and atresia. The first day they fed him with formula forgetting I had been freezing milk for him. He had a bad reaction and started having a lot of secretions and trouble breathing. They put a tent over his isolette. He was being fed with an NG tube. Once he started to stabilize again they did a barium swallow to check for leaks. I don’t remember exactly when but at some point they allowed me to feed him my milk in a bottle.
On Valentine’s Day I received a call from Daniel’s surgeon saying she had a present for me; I could attempt to breastfeed Daniel. I was excited to say the least. My husband filmed the occasion. It was stressful though as I was given time constraints as far as feeding. Daniel did great and took to nursing very well. I was worried because I had tried giving him a pacifier but he would gag on it and scare me. He had problems gaining weight so I suggested that he be fed more often than every three hours. I was given the go ahead since I was going to feed him on demand at home anyway the pediatrician said. He finally gained enough weight to go home. I was relieved and nervous at the same time. He was in the hospital for nearly a month.
We had been home for less than two weeks when Daniel started to want to nurse every hour around the clock. I was exhausted. He turned blue on me during one feeding and I called the hospital. Since it was already Friday they said if he did it again to take him to the ER. In the meantime they scheduled a barium swallow for Monday. I had a bad feeling but I was too afraid to trust my instincts. Monday morning came and during his test the doctor yelled, “suction!”
Daniel was admitted to the hospital. They had a hard time getting an IV in and took him to another room. I wasn’t allowed to him since he had developed a stricture. His dilatation would not be until the next day. That was one of the worst twenty-four hours of my life. I pumped my milk for him and put it in the hospital’s freezer. I could not bare to hold him since I felt guilty that he might smell me and could not feed. My husband held and comforted him throughout the night while I pumped off and on and tried to get some sleep. Daniel’s crib looked like a prison to me.
After he recovered from anesthesia the next day I was allowed to nurse him again. Daniel’s surgeon said his stricture was pretty easy to remove. The surgeon suggested at least one more dilatation and then see if he needed any more. His last dilatation was in April of 2003. He was on reflux meds for a couple of years.
To date he is almost twelve. He has ADHD, Asperger’s Syndrome (Autism), developmental delays and learning difficulties.
He just started catching on to reading skills.
He loves computers, jumping on the trampoline, teasing his siblings and making me laugh.
15-11-14 Desiree’s Story
My pregnancy with was pretty uneventful except for borderline gestational diabetes and a short hospital stay for a bad kidney infection. When Desiree’s due date came and she hadn’t come yet I begged my midwife to do something to get my baby out as I was miserable. She told me to pack my bags and come to the Women’s Center at midnight to be induced. She told me to try and rest beforehand but I was too excited and nervous to sleep. At midnight my husband and I checked in and tried to prepare ourselves for however long it would take for her to make her entrance into the world. She was born around 1 p.m. in the afternoon. I got to hold her for just a minute and then the nurses took her to weigh and evaluate her.
When they tried to get a tube down her throat to clear out secretions it would not go down. I was unaware of this until a nurse came into my room asking me to sign paperwork for her to have an x-ray and further medical treatment if needed. They next time Desiree was brought in she was in a travel incubator on her way to be transported to T.C. Thompson’s Children’s Hospital at Erlanger Medical Center in Chattanooga, TN. The surgeon called me during the night to let me know she was going into surgery and called me later to let me know when he was done. I believe she had a short gap.
As the reality set in I shed a lot of tears that night when I heard other babies born that night. In the morning I was released so that I could go see Desiree at the NICU. Seeing her in the NICU that first day was shocking and heart wrenching. I felt helpless seeing her there. The lactation consultant asked me if I wanted to pump milk so that Desiree could have it via her G-tube and I said yes. She brought me the necessary bottles, labels, showed me the refrigerator and storage bin was with Desiree’s name on it.
Then she showed me where the lactation room was. I finally felt like I could do something to help my little girl recover from her surgery. After a few days they did a barium swallow and found no leaks. I was given permission to feed her my pumped milk via a bottle. I got to try and breastfeed the day before she was released to go home. She came home the day before Thanksgiving.
The Dr. left a mickey button in in case she had any problems before her follow up visit. She was at the hospital for less than two weeks. She was a very fussy baby. She nursed every three hours. She didn’t sleep well during the day and would arch her back after every feed and scream. As a first time mom I thought I must be doing something wrong. I didn’t discover until after her brother was born and she was three that she had G.E.R.D. By the time she was one she was diagnosed as failure-to-thrive. She had quite a few colds her first two years.
She hit a lot of her milestones late; walking, potty training, dressing etc. Except for talking. Hopefully I passed on my love of talking on to her. To date she just turned fifteen as of 11/11/14. She is behind academically but she has smarts of her own accord. She likes to draw horses, sing, make crafts and acts like a “little mommy” to her four siblings. She has a guitar she wants to learn to play.
She hasn’t needed dilatation’s or more surgeries since birth.
.14-11-14 Huma Ayub Tof MUM UK
Amna was a much awaited baby, she came in my life after I had to undergo numerous medical tests and procedures to check for infertility but everything used to come out normal. Our happiness and excitement turned into anger and disappointment when they could not find her stomach in one of the scans. To prevent preterm labor i had an amino-drainage at about 32 weeks pregnant measuring 42 weeks. They took about two litres of fluid out. My relatives thought I was carrying twins!
Amna was born at 38 weeks by an emergency c section and was incubated immediately as she had some breathing difficulties. She was born with type C When she was one day old she had her first surgery. They managed to close the fistula and wanted to put the replogle tube as her gap was too wide to join.
However the whole plan changed when she had a cardiac arrest in the middle of the surgery and the surgeons had to create a spit fistula. She had heart scans and MRI to check if something is wrong. Fortunately, everything came out fine and she doesn’t have any other issues. She came home after one month stay in hospital with gastrostomy. We are waiting for her to grow and be more stronger for her gastric pull up surgery at Great Ormand street Hospital. She is growing well and we are happy with her progress so far. I do feel depressed and sad at times but what keeps me motivated is the fact that I am a mum to such a brave little girl.
She has made me a much stronger person and I can’t imagine my life without her!
13-11-14 Ryan Tof DAD UK Macie’s story
Macie was born with type B pure oesophageal atresia and was moved straight away into a neo-natal unit at arrowe park hospital (Wirral). She was then transferred to Alder Hey Children’s hospital to have a stoma on the right hand side of her neck so the saliva could be released.
Then Macie had a gastronomy tube placed and put onto specially formed milk. Macie was released a few months later and omitted home. On the 29th October 2014 Macie went into alder hey to hear about her operation.
The next day on the 30th she went down to theater and had her operation. The operation was a success and took just 6 hours. Macie has had a jegenostomy and had her stoma closed. Macie was then on the ICU ward in alder hey and then moved onto the HDU were she pulled out a tac tube and aspirated blood into her lungs.
Macie then was taken back to ICU for more recovery.
Macie is now on a ward waiting for more results on her release from hospital and she is recovering really well and am very happy of her progress. xxx
12-11-14 Michelle TOF MUM UK
My son David was born at 36 weeks , my waters broke and I called the hospital , 17 mins later he was born . He was purple at birth and when the midwife tried to feed him he went blue and started choking. He was rushed to scbu but it took two days for them to figure out he was a Vactrel baby. Rushed to Manchester children’s hospital and taken straight to surgery for a tracheal oesophageal fistula and oesophageal atresia to save his life . 9 hours of surgery later and we were told the gap was the farthest the surgeon had seen and only time would tell if the join would heal.
3 months of intensive care on life support with several more operations for other problems was the hardest time of our life’s . David was finally allowed home on the 13th December 2013, it was only days until he was rushed back to hospital with bronchitis and needed oxygen and emergency care to live. Christmas Day he can home to us again. Many more hospital admissions followed over the next few months with surgeries and stays needed.
David is now a year old, he has just started to eat blended food and start to progress physically. He has many surgeries ahead of him and is still behind developmentally but he is such a strong boy we have faith he will catch up.
This year has been the hardest of my life but also the most rewarding , I thank god for giving me and my son the strength to deal with these issues.
5-11-14 From a Parent in the U.S.A
My daughter, Nova Leilani, was norm February 28, 2014. At nineteen weeks pregnant we went in for our ultrasound, anxious to fins out if our three year old son would have a baby brother or sister. The idea that would find out much more than we were expecting never crossed our mind. “Congratulation, it’s a girl! However, I am sending you to a high risk specialist because I suspect your daughter may have a condition where her stomach and esophagus aren’t connected.”
I couldn’t comprehend what was going on. There had to be a mistake. But it was no mistake. Nova had short gap Esophageal Atresia and we needed to prepare for surgeries and long term stay in a NICU when she was born. On top of the anxiety, sadness, anger, frustration and confusion, I had to try and keep a happy fave for my son while on bed rest due to high fluid and early labor.
Nova held out till 37 weeks, it was a miracle she wasn’t premature. She spent her first 67 days in the NICU. Those weeks were filled with g tube placement, suctioning saliva, surgery to connect her, a leak at the connection site, her esophagus closing, dilations, oxygen, learning how to use her feeding pump, learning terms I had never known before, disagreeing with doctors on her care, not seeing my son or husband…. Until finally she was discharged.
We were thankful she was home but pur gut told us this wasn’t the end. After being home ten days she was med flighted to the hospital. They discovered a laryngeal cleft and after surgery sent her home again.
We spent another two weeks covered in throw up and our parenting instincts telling us something was wrong. We researched and talked to other mothers before deciding at midnight one night to drive her to the Boston children’s emergency room.
She spent another three months in BCU .. Three months that I am utterly in debt to the doctors for the changes they made. They listened to us, they talked to us, they didn’t treat us as though we were silly parents that didn’t know what we were talking about. She switched nova to a J tube to reduce the vomiting, they did a tracheopexy to stop Nova’s trachea from collapsing, and they cut out the piece of esophagus that was closing and redid her connection surgery.
Suddenly our little girl wasn’t throwing up, wasn’t choking, and was able to be discharged. We anxiously waited for our month follow up to see if her esophagus was staying open. I burst into tears when hearing it had stayed open and they didn’t need to see her again for two months.
Nova Leilani is now eight months with her next appointment coming up. She has finally hit 14 lbs and is eating baby foods by mouth along with small amounts from the bottle.
I still worry about the future. The light at the end of the tunnel seems so close yet so far. Will she ever catch up developmentally? Will the surgeries ever end? Will she ever eat solids like a typical child or adult? Will this g/j tube ever be gone? Will she be embarrassed of her scars? Will she tell her story to friends proudly? We pray she will. We are raising her and her big brother Caiden to appreciate the small steps and be proud of the struggles our family has gone through.
Seeing Caiden play with nova, tell everyone around her to wash their hands, and tell his teachers at school that his sister is a super hero makes me realize that I do not feel any sadness or anger anymore. I am so lucky to have such an amazingly strong little fighter. I would not wish a chronically sick child on any one. I would not wish seeing your infant on a breathing tube and paralysed on anyone.
But those of us who have been through this, also realize how absolutely blessed we are to be chosen to raise such a miracle.
Thank you for taking my offer up. x