by Claudia Z., Spring 1999
In 1953 Claudia was one of the earliest survivors of EA/TEF. In 1988, she was repaired for a second time at the age of 35. Ten years later she made her first connections to the EA/TEF community. Now Claudia, and her surgeon Dr. Juda Jona, share her story with us.
It was July 9, 1953, when I, Claudia Beth, was born at 3 lbs., 12 oz. to Helen and Don, at St. Mary's Hospital in Milwaukee, Wisconsin. My parents (and a sister, Kim, born in 1952), could not bring me home from the hospital for nine months. I was born premature at 7-1/2 months with a rare birth defect called TEF.
Very little was known about the birth defect EA/TEF since babies born with it before 1950 died. I remember being told that a baby boy was born with this defect sometime prior to my birth and had died. However, later in the 1950's, my parents found out that there was another baby with this birth defect who did survive but, because of the strict confidentiality, names were not allowed to be released.
After the doctors diagnosed my EA/TEF as Type C, I was baptized on July 10th and the surgery was performed on July 11, 1953. It was performed by Dr. Paul Hausmann, Dr. G.C. Kreuter, and Dr. Rex Ruppa at the Milwaukee Children's Hospital. Back in those days, my hospitalization lasted nine months. Even with a successful surgery, I still experienced collapsed lungs, pneumonia, chronic infections, bronchitis, and other illnesses. In the hospital, all the sick babies shared the same rooms.
I was finally released to go home from the hospital on March 25, 1954, weighing 7 lbs., and continuing to grow. My condition was considered very sick so my parents had a private telephone line placed in their home. (In the 1950's all the phones were on party lines.) It was very urgent that my parents could get through to the hospital and the rescue squad. This rescue squad unit was trained to come to my aid if emergency care was needed.
At the age of one, I weighed nine pounds. My food consumptions were soft and pureed for a long time. I had my first hamburger when I was six years old. If I choked, my parents didn't rush me to the hospital, they'd just pat me on the back numerous times routine until I coughed it up, or they would make me swallow it down by drinking lots of water. Never ever did I have any dilatations. I don't think it was even an option.
I had another problem that wasn't noticed until nursery school. Teachers felt I was not responsive to them or my classmates. It was then that my hearing loss was detected. I have severe nerve damage in both ears resulting in the loss of high frequency tones. Either my hearing loss was a result of Tetracycline, Streptomycin, or drugs used during infancy in the 1950's, or I was born with it. Nobody really knows for sure. The doctors all thought the drugs were the reason for the hearing loss. Or is this a coincidence with some EA/TEF babies?
My parents fought to keep me in the public school systems and fought the school board about not placing me with the mentally challenged children. The conclusion of this fight was to keep me in the public schools and to work with the teachers on how to teach me, so that I could see their lips. My parents always believed that I was smart, and a "born lip reader."
School was not easy for me since I never had hearing aids until 1970. After getting the aids, my speech started to improve and I learned new sounds. School was a struggle for me, but having the teachers and my classmates aware of my hearing problem made my school years easier. So, I continued through school, graduated from high school and finished two years of college.
As far as physical activities, when I was really young, I couldn't go out in very cold weather. I remember my sister, Kim, and younger sister, Ann, (born in 1956) could build those neat snow tunnels and igloos while I remained indoors because I was sick. I remember being in my room a lot because I had to be near Vicks and vaporizers. My parents were probably more protective of me than my sisters because, after all, I was their "Miracle Baby." However, as I grew older, I was more rebellious and active and I tried more activities. I was a typical teenager.
The scar on my back was an issue as I grew up, however, my parents taught me to be "proud" of my scar and accomplishments. I would learn to ignore the back talk. If questioned, I would just simply state that I had a birth defect which was corrected after I was born. Simple explanation when young, and as I got older, the explanation was more in detail.
I do remember the girls really staring in the 7th and 8th grade during the showers after gym class. I was self-conscious enough about my physical development, while the girls would look in awe at my scar, and make funny faces, and then make mean comments. I would just quickly dress and be on my way for the next class. I had to learn to let comments go through my ears! Frankly, I didn't care because I knew that I was proud of my scar!
I dated and carried on a normal life all through school and my careers even though I would continue to get sick a lot with colds, bronchitis, and sinus problems. I met my husband Chuck in 1976 and we married on October 15, 1977. We had two daughters: Cynthia, born in 1983 and Cathryn, born in 1984. Both pregnancies, delivery, and labor were easy for me. Cyndi was 30 minutes, and Cathryn was 40 minutes and both babies were born healthy. (My husband would brag how he parked in a 30 minutes parking zone, and didn't get ticketed). Life has been wonderful for me as Claudia Z.
During the years of 1987 and 1988, I started having problems getting really sick quite often. I was treated with antibiotics, which didn't help, and taking allergy shots. I was at a Sunday morning emergency clinic and the doctor who was on call that day asked about the scar on my back. After explaining my history, the symptoms of choking, coughing food up, turning blue, trying to catch my breath, and losing weight, the doctor asked me when I had my last esophagram. I didn't recall having one. However, I later found in my baby book that it was in 1955!
So I had an esophagram done and later had a bronchoscopy. The tests revealed a 1/2 inch hole in the esophagus, and a 1/4 inch hole in the trachea with a fistula of 1 cm. in width. Wow!
Here I was, 34 years old and was about to have a second repair surgery. Now the question was, who was going to do it? I consulted with several heart surgeons, each having different ideas as to how they were going to correct this problem. My daughters' pediatrician heard about my case (at a routine check up for one of the girls) and referred me to a pediatric surgeon since this was known as a pediatric problem. So, that is how I met my pediatric surgeon Dr. Juda Jona, of Milwaukee Children's Hospital. Dr. Jona is currently teaching and practicing in Evanston, Illinois.
Prior to my TEF-type C repair surgery to be performed by Dr. Jona, I would go to the Wisconsin Blood Center to bank my own blood for six weeks. Even though I weighed 98 pounds and could have passed for a teenager at Children's Hospital, Dr. Jona was kind enough to do my surgery at St. Joseph's Hospital, (at our request). Surgery was done after six pints of blood was saved, and I had dosed myself with vitamins (crushed in water), eaten lots of ice cream, and other soft foods to try and gain weight.
My second repair surgery was performed on July 27, 1988, at the age of 35! The surgery lasted 4-5 hours, and 4 pints of my blood were used. Dr. Jona took out the fistula, sewed up the holes and also patched the area using tissue from outside the lung area. I was hospitalized for seven days and remember being hooked up to a lot of machines. My most vivid memory was close to the date of being released when those chest drainage tubes had to be pulled out. I was given self-release morphine by machine, but when the doctor or specialist came to yank out the tube, I didn't get a chance to push the button! The second lung tube was pulled out later, after I was told when to push the button ahead of time. I also remember that I didn't want my two daughters to see me all hooked up to machines since the girls were only five and four years old. I had my husband bring the girls in after the machines were gone.
I think about how my parents couldn't take me home for nine months back in 1953, compared to my husband who took me home after just seven days in 1988. Recovery for me was a lengthy process at home. I couldn't eat solids for a while. I ate lots of soft foods until I could start some solids. I consumed very tiny bites, food cut up very small, and small quantities at a time. Swallowing was very difficult and for months it still didn't feel right. The points came when I couldn't swallow a corn kernel from corn on the cob. It had been about six weeks since my repair surgery, so I ended up back at the hospital emergency room having the corn kernel sucked out. After this episode came the beginning for me having dilatations of the esophagus. Remember, I never had this done to me while I was growing up.
My first dilatation was done at Children's Hospital, by Dr. Jona. I was now the big baby at Milwaukee Children's Hospital! Anyway, between 1988 and 1998 I had five dilatations. Now I have a gastroenterologist perform my dilatations (since moving to Green Bay in 1992.) The other thing that's new for me as of this year, 1999 is being on drugs called Prilosec and Populsid. One keeps the acid down and the other keeps the food down. We use these to help with my esophageal reflux problem.
Another interesting fact that I must mention is that the surgery, care and stay at the hospital in 1953 cost my parents $15,000 while in 1988 it cost my husband and me, $23,000. The possibility of the cost difference may be due to the nine month stay compared to seven day stay at the hospital and the different charges of hospital care, surgery costs, and doctor fees. My parents were not well off and it took them three years, beginning in 1953 to pay for the costs of my surgery and care. Back in 1953, there was an organization called the Red Feather, that helped raise money for people in need of help. My parents did receive some help through this organization.
Now the question will be how aging will affect me with a EA/TEF condition. It is a fact that this defect could recur and therefore, the holes and fistula would need repairs, but hopefully not for another 35 years! Most of the babies that have repairs have them very soon. So why after all these years did I have repair surgery at the age of 35? Dr. Jona speculates that as I was growing up, the holes grew. Another possibility is that the medical equipment and implements used in 1953 are not used today. Therefore, the surgery back in 1953 may not have been as precise. If my second repair surgery was done in the later 1950's, would I still be alive? Am I a pioneer of the EA/TEF birth defect?
You parents out there are so lucky to have this EA/TEF support group. I think about my parents who had nobody to relate to for all their stresses. My mother had a nervous breakdown a couple years after my birth. My Dad, who is 80 years old now, wants you to know that he and my mother, who is deceased, struggled through this without any help. Again, names of parents of EA/TEF patients were confidential and no contacts were allowed. That's just the way it was back in the 1950's.
As for myself, I was very happy to learn about this group in October 1998. I had never met another person with the same birth defect as myself, age 45, until just recently. I attended my first meeting in Illinois, on January 19, 1999! There were tears in my eyes as I met other EA/TEF families and realized that I'm no longer alone. I want to thank a dear friend, Betty B. (whose baby, Isabella, has the same TEF- type C) for communicating with me through E-mail. She contacted me through Dr. Jona, who did Isabella's surgeries this past year. The newsletters I got through this organization were a "wow" for me to read. Reading and rereading all the stories that you parents write concerning your babies, and the problems everybody goes through, along with the support out there, has been overwhelming for me. The stories I read make me wonder if any of those procedures were done to me, but perhaps there was a different medical term for it.
Having repair surgery at the age of 35 gave me the new, sound understanding as to what my problem was truly about. Experiencing the pain and fears and understanding what my parents had to go through, as an adult, I could feel what a baby must be going through. I don't have any memory to my first year of life other than what my parents have taught me.
For all you EA/TEF babies out there, the most important thing about this birth defect is YOU. Maintain your hopes and dreams and achieve your goals. Live your life to the fullest and continue to live and maintain a healthy, normal lifestyle. Keep your scars as a reminder of who you are. I have two scars on my back and I call it my "Cross!" We are the Miracle Babies!
For you parents out there, communicate, teach one another, have hope and faith, pray to God for your strengths and guidance, thank your doctors, love your family, support this wonderful group, finally and most important, LOVE your EA/TEF BABY.
As I conclude my story, Dad wanted me to add another memory to this story that is very important. We all know that you parents and survivors of EA/TEF, and the doctors, have some faith in God. This incidence happened when I was seven months old, when I was still on oxygen 24 hours a day. My condition took a turn for the worse and became very grave. Mom and Dad were called to the hospital immediately, along with the priest. All the nurses and doctors were around my bedside. I was blue and lifeless. Father Marshall Day laid his hands on me and said a prayer. They all said prayers. Then, all of a sudden, my eyes opened, my color turned back to normal, and I was alive again... This was witnessed as another MIRACLE.
Betty B. mentioned at the end of the meeting in January a quote from Dr. Jona that I never realized: "Claudia is the first EA/TEF Survivor in the state of Wisconsin. Also, the first oldest known adult to have a second repair surgery at the age of 35!" I am a pioneer EA/TEF baby!
Sincerely, T(terrific) E(excellent) F(friend),
Claudia, Green Bay, WI
Claudia: Her Surgeon's View
Juda Z. Jona, MD, Head, Division of Pediatric Surgery, General and Thoracic, Evanston Hospital, Illinois
I first met Claudia in 1988 when a "full grown" woman came to my clinic in Milwaukee carrying a large x-ray envelope with her. I was certainly baffled when I found out that she was not a mother of a patient but rather the patient herself. When I informed her that my practice was limited to the surgical care of infants and children, she countered by stating that although she was an adult, her problem is that of the very young. And sure enough, when I reviewed her films it was readily obvious that Claudia had recurrent TEF. I was especially amazed not only to find out that she was the first successfully repaired EA/TEF in the state of Wisconsin, but also that she, in essence, had symptoms of TEF all of her life. None of the family members nor the various medical doctors (from pediatricians to OB/GYN to internists) even thought to relate her chronic coughing (especially when drinking) and repeated chest infections to the possibility of recurrent TEF. From her story, I concluded that her recurrence happened early in the post operative period (back in 1953) and that is one of the reasons she had to spend 9 months in the hospital and resort to the "best healer of all," God, to see her through this dangerous time. And here she was, a mature woman, mother of two, who played tennis (and even smoked cigarettes!) who endured a "stormy" infancy and lifelong lung symptoms; who despite it all remained kind and mannered and to whom one had to speak straight to her face so that she could understand ones speech. She, in other words, was irresistible and I promised her that I would correct her problem surgically.
At the time of the operation, the TEF was located in the conventional site, where the old one was situated. There was very little inflammation around it - suggesting that the tracheoesophageal fistula -- was, indeed, of longstanding. When we disconnected the trachea from the esophagus, we repaired the two side holes in them. In order to secure the repair and prevent re-recurrence of TEF, I "borrowed" adjacent tissue and imposed it between the two suture lines. She healed promptly. However, as you heard, her esophagus developed some scarring in the area for which occasional dilations were required. It was particularly interesting for me to talk to Claudia about her lifelong esophageal problem. Since all babies with EA/TEF have impaired motility of the esophagus, Claudia is (was) not an exception. She can describe a "bolus" (chunk) of food slowly descending down the esophagus with the "descending" discomfort it produces. Her post problem with "food getting stuck" in the esophagus is a most typical occurrence in our babies and most parents can agree to that. She soon learned to chew her food well, eat small pieces and drink frequently with her meals. No question these points are driven home frequently to all parents with EA/TEF babies. The message from Claudia for all of us to remember is not only the triumph of the spirit. Also one need not to ignore symptoms but to pursue them with persistence and vigor. Claudia is lucky not to suffer the consequences of her chronic TEF, namely chronic, debilitated lung condition. (I do hope she stopped smoking by now - a hint Claudia!)