Virginia’s Biography Born Unable to Swallow Ea-Tef

I am going to use Virginia’s nickname – Ginni – because that is what I always called her.  My first college roommate was in the Summer of 1968 and her name was Ginni Reese.  During the first week, she walked in on me sitting on the floor with my head between my knees, crying uncontrollably.  I can’t remember anyone comforting me like this, but she sat down next to me on the floor and actually held me and let me cry.  After a long time, we began talking.  That was the last homesickness I ever had.  I loved her like the sister I always wanted.  We did most things together.  The lunchroom was about half a mile (maybe a little less) from us.  She helped me go and eat even if I didn’t want to.  We began making friends, thanks to her.  I often remark that when I went to Northwestern I felt like I had just been born.

I knew I had to name my baby after the Hadleys of Virginia, my ancestors.  So I named my baby Virginia Hadley, and immediately called her “Ginni.”  Of course, I lost touch with this angel through the course of events.

Ginni’s isolette was in the back in the left corner at first.  At that time I was told that babies feel no pain.  So her nurse would come and slap Ginni’s feet to “stimulate” her (Ginni would cry so hard and so would I).  I was so very mad but felt helpless.  Next, the nurse would have Wayne slap Ginni’s feet, AND the nurse began openly flirting with Wayne.  I thought about how little girls spend their lives looking forward to their wedding day.

Then the wording is “happily ever after.”  I was so scared and so young.  I felt that if I left Ginni, even just to eat in the cafeteria, they would kill her.  Oh, what I would have given to have my grandmother Mom there, and to have had a McDonald’s house to sleep and clean up in.

Ginni would occasionally stop breathing.  A nurse would put a tube down her throat that sucked all the mucous out.  Then she would be pink and pretty.  They had used the top of her head for the IV (I also remember them using the navel for it) so Ginni’s head was shaved, what little hair she had.  I don’t think the whole head was shaved because I remember it looking funny.  Later on, I kept a bonnet on her almost all the time.  One of Wayne’s uncles said something funny about her bonnet.  I was so emotional that I secretly hated this man throughout his life.  It turned out finally that I had the wrong uncle!

One day in the NICU, I noticed blood spurt up the wall at the other end of the room.  Doctors and nurses were working on a baby.  I turned my back.  Then there was a baby in my vision that some mother had tried to abort herself – but he wouldn’t die.  She finally called 911 and this baby was frequently next to Ginni.  When we came back when Ginni was six months, he had pneumonia.  I saw him resuscitated over and over.  When the alarm would go off, he would have a sweet smile on his bluish face.  When he came back to life, he was mad.  Finally, he went to Jesus.  The entire NBICU closed up in sorrow.  I couldn’t understand why I had to leave at first.  I’m sure my mind was not working clearly.  It was a sad day.

As we were going home with Virginia, a nurse rudely asked, “Are you taking her home with you?  Why?”  Then a doctor who was studying to be a doctor asked us if we had our appointment for the spinal doctor.  I looked at Wayne who looked blank.  Then I asked why we would need it.  He said Ginni had a horrible birth defect and if we didn’t bring her in for appointments she would be horribly deformed.  I took a deep breath and then nearly fainted.  No one had told me about this – a hemivertebra.

We went home on December 26, 1972.

For several weeks we dealt with a “gastrostomy tube,” as I wrote in her baby book.  It was one of the few times Ginni was not in constant pain.  We rather regretted its removal since we could feed her in the tube and then  “burp” her by moving her around, all without arousing her from sleep.  Within a week of the tube being removed, Ginni began again encountering strider and resuscitation on an hourly basis.

Ginni at her Parents Graduation.

Finally, Dr. Johnson scheduled another surgery in March 1973 called Anterior Bracheo Cephalic Arterial Suspension.

We were told an artery that went to half of Ginni’s brain was directly over the area of the original TE Fistula surgery.  In an attempt to stop the now-familiar chain of events, the artery was “stapled” to her sternum.  I wrote in her baby book that it was the “anterior subclavian artery.”  Years later Dr. Johnson said I made that name up.  Now, where would I get the name and why would I have immediately written it in her baby book I took everywhere?  I let the insult pass, as usual.  He then said it was the “innominate” artery.  I was corrected every time I tried to say it right, so I gave up and, acting stupid, would say “some” artery.

Backing up to going home from Ginni’s initial TE Fistula surgery, we were told suddenly that Ginni had a “Dorsal Vertebral Anomaly” and she would become terribly malformed in her back.  That’s when I nearly fainted, both Wayne and I hearing it for the first time.  His answer to my frightened query of what was wrong with her, was, “We told you this the night she was brought in – where the hell were you?”  My reply, of course, was that the doctor wouldn’t let me travel from my hospital bed where I had given birth six hours before.  No apologies from him – just a disgusted look as I tried to avoid fainting.

Appointments were kept.  In the baby book, we wrote “Hemi-Vertebrae.”  This horrible thing that was supposed to result from the extra OR missing piece of vertebrae [I was always told both ways, seemingly just to confuse me] just never grew.  God blessed us once again.  We dodged a bullet there.

1994 April 21:

Wayne graduated from Brigham Young University with a BS degree in Youth Leadership and Recreational Management.  I graduated with a BA degree in what was then called Concert Piano, and also English.  We had never left Ginni’s side so we decided to take her with us.  We used our scant money of $25 to have an exact gown like ours made.  The Music Department donated a cap and pink tassel.  That day was the happiest of my life.  My parents and Aunt Ora were there.  Ginni had a good day.  We took her right into the new Marriot Center and down many steps to our seats.  I was eight months in the family way with Melanie.  So every step out we took, the entire rows on either side gave us a standing ovation.  Many people kept taking pictures of us until we had to leave.

Time passed with a sense of normality – this odd life accepted as Status Quo.  Ginni turned out to have learning difficulties.  Her initial assessment when we took her home was that she would stay at the mental age of five and would have serious learning difficulties, besides being sterile from the x-rays performed to save her life.  I decided not to tell her of her birth defects nor of the diagnoses.  Instead, I encouraged her to study harder.  She had strider and serious breathing episodes, which she handled herself.  She also had a distinctive “bark” when she coughed to get the phlegm up.

One odd incident came about when we moved to Idaho Falls for a year for my husband’s Professional Scouting job.  Ginni had gone with us always and never out of our sight.  That included Scout camps that my husband managed [including Camp Kiesel, Camp Bartlett, and Treasure Mountain].

And yet, one day I was called to her grade school to meet with the principal.  It turned out to be several members of the school board accusing me of child abuse and probably keeping her in a closet.  I had no idea the meeting was with anyone but the principal.  I thought he wanted to know why she sounded like a “duck” as she coughed and fought for breath, so I didn’t warn my husband.  My mind is a blank for most of this, but my husband remembers taking her to Primary Children’s Hospital, back to Dr. Johnson.

Then we went to see an audio/ear specialist at the Salt Lake Clinic – that I remember.  She had an inordinate amount of ear wax in her ears for her young age.  Dr. Perkins cleaned it out without a cry from Ginni.  I never heard anything else from school.  End of that story.  And another escape from a bullet.

When Ginni was about eight years old, we decided to visit Timpanogos Cave in Provo, UT.

Ginni was fine until we started down the mountain.  Suddenly Ginni was coughing and breathing hard and was very mixed up in what she said.  In the car she became normal but it was off to Primary Children’s Hospital once again.  She seemed fine to everyone so we thought the cave ascent was too strenuous on her.

Ginni came home from Bear River Middle School in Garland – we had moved back – and that day had her first experience with PE.  The showering was awful, but even worse were the comments about what the scars were on her body.  I had to sit down and tell her about the TE Fistula and subsequent surgeries.  I even told her about her “bark.”  She was amazed that I had never told her or that she had never noticed the scars.  I answered that there was no need for her to know until this particular day.

In Bear River High School, Ginni wanted to play the oboe.  I knew it took more air than any instrument I was familiar with.  When Wayne said he did not want a “dying duck” sound around, my mind was made up.  I went that week and rented an oboe.  The sound was atrocious, of course, but she got better and better.  I even sent her to private lessons for the double-reed difficulties.  She would get very winded and then would try all the harder, with my encouragement.  Building muscles is what this life is all about.

1990 Autumn:

At 18 years of age, Ginni described what happened to her at school.  “Suddenly I couldn’t breathe, and my face turned red with a burning sensation throughout my face that continued nearly down to my throat, but more in my head.”  She tried to grab water and drink.  She also said sometimes it felt like it moved something like maybe something was lodged.  Effects would last for up to half an hour.

Extreme exhaustion followed.  Back to Primary Children’s, we went, to see Dr. Dale Johnson again.  We had given him gray hair.  He took her into surgery thinking he would have to build a “cage-type” affair, either inside or outside the trachea to stop the “malacia.”  He had consulted a specialist in Canada and also one in Australia for advice.  Yet, when he operated he found that her heart had changed position and that the Aorta coming out of the heart was lying right on top of the original surgery from 1973.  He then sutured the heart to the sternum and said any future open-heart surgery through the sternum would kill her instantly.  [Remember this when you read of her death at 45 years of age.]  She was in the hospital for three weeks, and Dr. Johnson didn’t believe us when we said she played the oboe – but she reassured him she did.

Ginni married the year after that surgery [Anterior Aortic Suspension] at 19 years of age.

She continued experiencing worsening symptoms and would have to drink a lot of water to get any food down.  Finally, in November 1991, a “Flexible Fiberoptic Bronchoscopy” was performed with the patient awake for evaluation of symptoms of “dyspnea.”  The major findings were that the right “anterio-lateral compression of the trachea at the thoracic inlet” was collapsed to approximately 25% of normal diameter with “expiration,” and left anterio-lateral compression of the distal trachea,” which was felt to be less severe than above.  No surgery was involved.

In 1992:

The very next year, Ginni was nearly 20 years of age and began itching on her feet constantly.  This spread to Ginni’s lower legs over a year and the itching there was intermittent.

1992 June:

I have to turn to the doctor’s notes.  “Ms. Hunsaker had worsening of her chronic dyspnea.  At that time an exploratory thoracotomy was performed and the aortic arch was suspended to the sternum. There was no mention of the malacia at the thoracic inlet.”

1992 October:

“The patient was seen in follow-up and felt to have some symptomatic improvement.  Spirometry at that time revealed FEV1 2.5/FVC 3.0 (83%).  These results were apparently ‘modestly improved’ from pre-operative values.”

1994 October:

At 22 years of age, the itching began spreading to her entire body.  It was fairly consistent but seemed much worse at night, attributed to increased awareness.  She could not visit us without taking off her shoes and socks and scratching her feet hard!

1995:

Ginni called Dr. Johnson and told him she was having constant burning in her esophagus and life had deteriorated to an alarming degree, notably both with eating and with the constant itching.  She asked him what could be done.  He referred her to a Dr. Hillam in Brigham City, a gastroenterologist, and surgeon in typical small-town private practice.  He prescribed Prilosec twice a day.

Then Ginni’s symptoms became critical.

She could feel “material” coming up into her throat, choking her and cutting off her breathing.  She would burn in her chest now, and it created pressure in her chest and exquisite pain.  Her husband, Jeff, reported cessation of breathing for long periods during the night, keeping HIM in a state of fear.  Each morning there was evidence of aspiration.

Dr. Hillam did a scope of her esophagus which showed tissue changes and made him worry about Barrett’s Syndrome.  Once inside, he found the stomach had 1/10^th the acidity of a normal stomach, so he insisted on doing a partial wrap of the esophagus.  He also saw sclerosing in the liver and wrote he did not understand the significance of it.  The surgery was done and Jeff did a wonderful job taking care of her.

1995 Summer:

Ginni began waking up during the night feeling like she had breathed in something, and would be choking and gasping for air.  She would run to the sink for water and would have vomited, but she could not.  She never vomited normally anyway, just had saliva come up with no food.  She would wretch, choke, and fight to breathe to remain conscious.  The acceptance of these critical symptoms as “normal” brought back the feeling of us against the world, with us MAKING her live.  Jeff had become more and more aware of the seriousness of Ginni’s health.

Next came allergy testing, sent to Washington, D.C.  She was allergic to all known food groups.  Within the next two months, she experienced numbness in her entire body.  Then the numbness came with severe stinging.  Serious breathing episodes occurred where she used an inhaler to avoid the emergency room upon the suggestion of our family doctor, Dr. David Wilding.  I wrote, “We simply had to accept crises as normal and deal with them ourselves.  Why??”

1995:

Surgery was scheduled with Dr. Hillam for December 1 to undo the partial wrap, cut away 2/3rds of the stomach to create a funnel situation into the intestines that would be brought up and looped.  This would create a new opening for the bile far below the stomach area.  I was very wary of permanently changing her stomach.

I reached out on the internet for help.  Gregg Burke was in charge of the TEF Database.  He gave us names of doctors including their personal physician in L.A., Dr. Kathryn Anderson.  We also received the names of Dr. Keith Ashcraft of Children’s Mercy Hospital in Kansas, Dr. Arnold Coran in Michigan, and Dr. Harry Hendron of Boston.  Next, Dr. Keith Ashcraft referred us to Dr. Paul Hyman in California.  He recommended the following two doctors:  Dr. Edward Feldman and Dr. John Vierling, a liver specialist in Beverly Hills, CA.  Ginni called Dr. Feldman and he was rude to her but gave her a reference to Dr. Andrew Ippoliti in Beverly Hills, CA.  It seemed like keeping Ginni alive was like the first day we brought her home – entirely in our hands.

Ginni came to my home crying.

She wanted to go to Dr. Vierling’s office [at Cedars-Sinai Hospital in California].  He had said, yes, the wrap needed to come down but she needed immediate evaluation of her liver.  He said that her bile ducts may be blocked or narrowed which would block up bile and cause itching [Colostasis].  The first thing to be done would be a “cholangiogram,” a specialized x-ray of the area, looking closely at the bile ducts.

1997 December 7:

After two more phone calls to doctors, we called Gregg Burke in Maryland.  Some of his ideas to try were to cut out milk, fruits, and bread and to start eating rice.  Concerning Ginni’s breath, he said she could have a “pouch” with food hanging up.  It could be in her throat or even her esophagus and perhaps in the stomach.  He suggested that good resources to talk to were Dr. Dave Merkin of Maryland for nutrition and Dr. Hardy Hendrin in Boston [who would soon be forced into retirement].

In one set of notes I have found from the University of Utah Pulmonary Clinic, I am amazed at the wording.  I will type them in here word for word.

Outpatient MRI Report Findings:

An MR Chollangiogram was performed, which shows a patent and normal-appearing common bile duct.  The intrahepatic common bile duct also appears normal, as does the region of the bifurcation.  The intrahepatic ducts appear slightly attenuated for age, an appearance which is likely secondary to the patient’s multiple liver abnormalities.  There are no areas of cystic dilatation of the biliary ducts noted.  There are what appears to be dilated bile ducts within the inferior aspect of the posterior segment of the right lobe of the liver, where the patient has a mass noted on her MRI examination.  It is uncertain whether this is a scar or dilated bile ducts within the mass, however, this represents dilated bile ducts.

The gallbladder appears normal, with the exception of some component of sludge.  A fluid collection is identified anterior to the liver, likely in the inferior anterior aspect of the right chest.  The etiology of this fluid is uncertain, but this may be secondary to the patient’s multiple previous thoracic surgeries.

IMPRESSION:

• Normal extrahepatic and intrahepatic common bile ducts, with attenuation of the intrahepatic duct. This may be secondary to the patient’s multiple hepatic masses.
• Probably dilated bile duct, inferior aspect of the patient’s right liver mass.

1998 January 15:

Surgical Pathology Report

• Liver Biopsy showing complex features.
• One fragment shows generally intact architecture, but with a slightly enlarged and fibrotic portal tract with periductal fibrosis and a focus of ductular proliferation (this pattern is sometimes erroneously called “bile duct adenoma”).
• Other fragments are highly abnormal with fibrosis, thrombosed vessels, sclerotic bile ducts, and multiple foci of ductular proliferation (this pattern is sometimes called “bile duct adenoma”), one of the foci is somewhat atypical.

This biopsy is not diagnostic.  The fibrotic area could represent the central portion of focal nodular hyperplasia, partial nodular transformation, or a form of congenital hepatic fibrosis, but the correlation with clinical and imaging observations is necessary.

1998 January 19:

CT Guided Core Biopsy Mass Right Lobe of Liver and CT Guided Core Biopsy Mass Lateral Segment Left Lobe of the Liver.

DIAGNOSIS:

• Liver, right lobe, biopsy: Suggestive of liver cell adenoma.
• Liver, left lobe, lateral segment, biopsy: Suggestive of focal nodular hyperplasia (FNH).

1998 February 4:

Department of Defense / Armed Forces Institute of Pathology / Washington, D.C. – Consultation Report on Contributor Material:

• Liver Biopsy, Left Lobe (N): Focal Nodular Hyperplasia.

Liver Biopsy, Right Lobe (N):  Benign hepatocellular neoplasm suggestive of Focal Nodular Hyperplasia.

We agree with you that the sections of the biopsy from the left lobe (Block 13) are from a focal nodular hyperplasia (FNH).  The biopsy from the right lobe (Block A) could be from a hepatocellular adenoma, as you suggest.  However, several histologic features favor an FNH.  These include part of one septum, patchy sinusoidal capillarization (as is seen in the extensions from the septa of FNH), the well-developed reticulin framework, and the presence of occasional pseudoxanthomatous cells (cholate stasis).  We would expect some focal positivity with rhodanine and/or Victoria blue stains.  Cytokeratin stains in FNH often show a change of phenotype of liver cells (adjacent to the septa) to a bile duct-cell phenotype.

1998 March:

We had an appointment with Dr. Tietze (Gastroenterologist) at the University of Utah Medical Center.  Virginia was taught how to give herself injections of a medication to deal with the pruritus.

My notes stop here so I will put down what I remember.

Ginni called me and was having severe Braxton-Hicks contractions.  My mind finally clicked on and I knew she was in labor.  I took her and Jeff to St. Ben’s hospital after calling them and Ally Phipps.  Ginni was INDEED in labor and about to deliver.  The time had come and I did not need decisions any longer, since it was out of my hands.  Dr. Silver listened to my recent history and the fact that Ginni did not feel regular pain.  I answered every one of his questions.  My husband, Wayne, showed up some time through this.

Another thing – they had wanted to send her by LifeFlight to the U of U Med Center, but her labor was too advanced.  Jeff had hysterically said he could not afford it.  I turned to the doctor and said the corny-sounding phrase, “Money is no object.  Do what you must.”  But Life Flight turned back.

The machines showed a low heart rate for Noah, although the doctor had planned on a regular delivery.  Then he took me outside and said they were going to have to do an emergency C-Section.  I quietly told everyone what was happening as the medical team prepared.  We did an extremely wild and fast “bed drive” down the hall with everyone talking over each other.  We arrived and Jeff wanted to go in.  The doctor said okay.  And the doors closed.

Soon the doctor opened the doors and grabbed me, saying I had been a part of everything all along and I should be in there.  I saw the nurses glare at me but he said it would be okay.  The doctor said there was no time to scrub and gowned me himself.  Jeff stood at the right side of her head and me at the left.  I could stand on tiptoe and see everything.  It was all very fast and professional.  Then there was Noah, held up for all to see, breathing and everything!  He was snatched away by another medical team and we were left with Ginni.

Ginni had been given anesthesia of some sort and was quite asleep.  I watched her as they stitched her up, placing her uterus on her upper abdomen.  The area inside her was absolutely empty, not the way I had imagined it.  It was so routine and Dr. Silver and the nurse talked about everyday things.  I had started in nursing way back and was doing the “book” learning before clinical, enough that I had a major in English later on.  But I had never realized the boredom that comes after the excitement.

The team with Noah reported back.

They had drained some of the water in the abdomen – AND I remembered that was definitely NOT supposed to be done.  Then Noah was in critical condition and they called for an ambulance.  I said goodbye to a sleeping Ginni and to Jeff who would stay with her, and we rushed to our car.

I prayed unashamedly for Noah to live – I couldn’t stop myself.  We drove quite fast but the time getting there seemed much longer than ever before.  We arrived and hurried to the NICU.  The nurse said visiting hours were over the period and where was the mother.  I said snidely that the mother was in another hospital recovering from a C-Section, and all I wanted was to know that Noah had arrived alive. She was unremitting, saying she could talk only to the mother.  I tried to tell her I was then the legal guardian in place of her mother, but she was totally obstinate.  Tears erupted from me.

Then a young doctor grabbed us and said come on in.  We went in through the maze of incubated babies and were able to see Noah breathing fine in an incubator, with wires everywhere.  I thanked him profusely because that set my mind at ease.

We were in the waiting room when two doctors who looked like kids told us what was to be done.  They asked why the fluid had been partially drained at St. Ben’s Hospital.  I said it was out of my hands at that point.  Then, whatever was said next, I got mad.  I told those two punks that why should the students operate and not the creator of the original surgery.  I demanded that Dr. Dale Johnson be brought in.  They said, “You don’t want that old man!”.  Wayne restrained me, I think.  The conversation had become shouting and all the other parents were watching us.  Finally, they called Dr. Johnson in.  I gave him the recent history and he gladly took over Noah’s case.

He was a bit grayer but otherwise, he looked like a miracle.

After Noah was stabilized, the surgery was set.  We stayed in the new-to-us Ronald McDonald House for the night.  Then the surgery was performed with expertise the next day.  Noah was indeed alive.  Part of his intestines, including his lowest valve into the intestines, were necrosed and had to be removed.  The bowels and the lower part of his esophagus were resected.

To follow was the routine scrubbing up and gowning to see him during visiting hours.  If the nurse remembered me, she didn’t show it.  When Ginni was better, she and Jeff took our room at the Ronald McDonald House and we went home to rest.  Noah seemed to weather his surgery and ensuing care like a pro.  Wayne thinks Noah was in the hospital for a month.  It was hard to say goodbye to Dr. Dale Johnson.  How do you thank a man who saved both your daughter and her son?

Many doctors’ appointments later, Noah was fine.  One day, Ginni went to the restroom and the doctor said to me to look at Noah, but not as a grandparent.  I looked at him on the floor and his face was similar to a Downs’ Syndrome patient.  I said so and then said he seemed so perfect.  She agreed.  It seemed as though we had missed another bullet.  I wondered how many more we could miss.  Years then passed.

2008 May 2:

I was taking Ginni to Salt Lake City to shop.  She said she thought she was having Braxton-Hicks.  I felt her stomach and then sped to the U of U Med Center.  They bypassed normal procedures and we were in a bed in the “impending delivery” room.  The anesthesiologist came in to give her an epidural but Ginni said firmly that she felt like she had to push.  The nurse backed the doctor off and we each took Ginni on each side and laid her down.  Little “Virginia Natalie Hunsaker” came right out on the bed.  I got to cut the cord, and Wayne and Jeff came about 20 minutes later.  She had “pooped” while in the uterus causing meconium aspiration syndrome.  She also had immature lungs.  Natalie stayed in the hospital for 15 days.

2012 December 2:

Ginni gave birth to another daughter at St. Ben’s Hospital in Ogden, UT, named “Ava Grace Hunsaker.”  The baby was a bit premature.  However, Ginni had edema and blood clots, after which she got pneumonia.  She had to go to the U of U Med Center for approximately 5 days, from the day after Christmas to December 31.  Ginni had been really sick for quite a while before and afterwards with nodules on her liver.

I have no notes from that time.