Monday, Monday, Can't trust that day....

I added all of our updates for the week so far to the previous post, with lots of great photos from the past few days, but a friend told me she hadn't realized I was doing it that way, so I'm going to try doing updates on little posts-- I'm still not a pro blogger, so be patient with me!  I think when I started that post initially, I was hoping things were going to continue to settle down and it would be easy to keep it to a single post.  In theory, if I start making each update a new post, it will have the opposite effect and mean we have less to report, right?  We could be home in a flash!  Sounds good to me.

 One of our friends here at the hospital from last summer brought us some cheerful photos she'd printed off Audrey's blog for us to hang on her crib.  I put the one of Daddy where she could see it and she likes to smile at it.  Two other signs she's been feeling better:  trying to crawl out of her crib, and jealous of my food!

The surgical team came in a few minutes ago and got an onslaught of questions from me.  They are so great.  But my questions are hard.  It is apparently not a simple thing to know where that 60 ccs of air in her anterior chest cavity came from, for example.  They didn't even want to talk about the stent yet, probably because they had lots of other patients to round on and they knew that wasn't going to be a short discussion.  They did tell me that she will have her next esophagram on Monday, so a TON hinges on that day.  But yesterday evening Dr Rollins did come by and discuss the stent procedure with me.  They are thinking of placing the stent (at this point the plan is only if her diverticulum doesn't heal), which would be somewhat similar to a dilation except that they would put this flexible poly-coated titanium ring in there, then theoretically the scar tissue will form in a wider ring, and they will monitor it weekly and remove it at the end of four weeks.  I was so glad that he came to talk with me, because apparently Dr Downey had forwarded my email from Tuesday night to him.  Here is the email I wrote:

Hi Dr Downey,

Thank you so much for your call today!  Audrey is showing some improvement tonight and we were thrilled to see her smiling and reaching for toys.   She did test positive for rhinovirus which may explain the continued fever.  I am writing you because I was surprised to receive an email tonight that seemed very relevant to Audrey's current situation, and wanted to pass it along to you.  A friend of mine is the head of the EA/TEF parent group for Utah, and during a recent conversation I had casually asked her if she knew any more information about little Brayton, a three year old in northern California who recently passed away from complications of his EA/TEF treatment.  I had been following the family's story near the end of his life and was heartbroken when he passed.  Apparently my friend has contact with a good friend of the family, and received this information from them with more of the medical details.  I was surprised to learn that the specific circumstances that lead up to his death were complications from a stent.  We understand that this information is neither complete nor directly from the source and thought you had probably heard of the case and knew better what had actually happened.  We also understand that this is kind of a worst case scenario, but the implications were enough to spook us regarding the stent-- I think I understand now why you had formerly expressed reservations about this approach for Audrey.  Perhaps we could talk again about this in a few days, after Audrey is through her infection and we see where things are headed with this diverticulum.  I will also of course pose our questions to the team here should this become imminent.   Also got the ball rolling with Boston Children's earlier today, and they should be in touch within the week-- we would like to put their specialist directly in touch with you if that is all right, to facilitate the conversation; I will give their Doctor your office number unless you'd prefer something else.  I hope together we can find the best possible solution to this challenging situation.  Your compassionate care, as ever, means worlds to our family.  Thank you, and have a safe trip! 
ps: (See the text copied below)  No need to respond to this email, we can talk in a few days.  Thanks again.
Here is the pertinent section of the email. (I have edited it slightly from the original):
"Brayton was successfully connected, however he kept getting recurring strictures and had to go for weekly dilations 4 1/2 hours away from home. Mom could not keep doing this, so opted to have them put a stent in his esophagus to try to keep the esophagus open and strictures at bay. They sent him home, with weekly monitoring of the stent, but unfortunately the stent still ended up eroding his esophagus and part of his trachea. They ended up removing all of his esophagus and doing a spit fistula as his esophagus was completely destroyed, they then tried to repair the holes in the trachea, but with no success as it was now damaging the lungs as well, and he kept getting septic from the stent eroding in his body. They did ECMO which oxygenates the blood but is always very risky and could cause you to bleed out. Unfortunately Brayton did ultimately bleed out causing him to pass away."

Dr Downey wrote me back briefly expressing his sadness at this tale and that it reminds them to always be very thoughtful in any approach, which I'm sure Brayton's team was.  While welcoming of additional input from the surgeons at Boston, Dr D expressed his caution about both sides of a second opinion, both in the sense that "if you ask 5 surgeons, you'll get ten plans for success" and being wary of blanket statements or guarantees of success.  Each child is so different.  Nothing's to say that what worked for one, or did not, will happen the same for Audrey.  But even knowing this, my mind is reeling with questions about sweet little Brayton's treatment.  How long was the stent in place?  Were there other health issues contributing to his complications?  His mother also sought a second opinion from the Esophageal Treatment Center at Boston Children's, what did they say?  Even if they had gone to Boston, it may not have been the miracle solution.  But perhaps by learning from their experience, Brayton can help, or even save, Audrey.  That beautiful family was irreparably damaged by this experience, and my heart still goes to them in thought and prayer so often. The best way I can think to help them now, and to honor Brayton's memory, is to be strong and do all we can to help Audrey in her similar fight.  At the moment, Dr Rollins has me convinced that the stent has good potential to help Audrey-- they've had success in four previous patients over the past three years, one of which was a long gap situation similar to Audrey.  But that email made my blood run cold, and a little voice in my brain keeps saying that learning more from both Brayton and Boston could change everything.  I love what my friend Candi said, "I suppose the worst thing that could happen is they could say that our Utah Doctors know what they're doing, right?"  haha! That totally IS going to happen.  I just hope we get some fresh ideas going that could help her.

(Update: I just learned that Brayton's severe esophageal erosion happened within just a couple of weeks after the stent was placed, in spite of weekly monitoring by his local (less specialized) hospital.  Ugh.  Looks like we have some really tough decisions ahead.)

The other thing I've been reading nonstop about is jejunal interposition.  Apparently it is another one of the main surgeries used to replace an esophagus, but I hadn't really heard of it until I saw this chart showing the pros and cons of each approach.  Dr Downey prefers the gastric pullup as a fallback surgery if we are unable to save her esophagus--which he hasn't given up on yet--and I know he must have excellent reasons.  (Although the G.P. is a commonly used surgery in these babies, the thought of pulling her stomach up into her throat still makes me nervous and sad.  I need to learn more about it to combat those feelings.)  When I ran across that chart I was surprised to see that the list of  "cons" for each of the traditional surgeries was fairly lengthy while the list for the J.I. was tiny.  I was suspicious that it was simply an incomplete list and wanted to see for myself, but it gave me a glimmer of hope to see something new so I've been reading like a maniac.  If we were to lose Audrey's esophagus through all of this, I wonder if that would be our best next recourse.  Jejunal interposition sounds interesting because it
A - maintains peristalsis (progressive muscle action toward the stomach),
B - doesn't have the fast-growing problems of colon interposition, and
C - follows the natural path of the esophagus because the diameter of small intestine is small enough to fit in the natural spot behind the trachea, but unfortunately it is a very difficult surgery to perform and it appears to be only in the last few years that doctors have been successful with it.

I looked down at Audrey snuggling in my lap.  She's been fussier this afternoon, maybe hungry or sore from her increased movement yesterday, and she had a really hard time sleeping for her nap.  I sighed and gazed out the window for a moment, watching an airplane fly above the city skyline, and for a few seconds the sun flashed off the body of the plane, making it look for all the world like a silver bullet flying through the sky.  Could this surgery, with the likely accompanying plane ride to Boston, be our silver bullet?  Is there something else we can do to save her esophagus endoscopically?

Here is the study I'm currently reading.  http://www.biliaryatresia.org/reference/EA-Jejunaltranspostion.pdf  And here is the second email I sent Dr Downey:
Much agreed.  Thanks for your thoughts.  I am wary of that as well, although I also respect that one second voice on the matter could yield valuable ideas.  Ran across one website in particular last night that intrigued me regarding the potential of jejunal interposition vs. the other major approaches we have talked about for a fallback plan should it become necessary in the future, wanted to send that your way for your thoughts. Shows an interesting comparison and contrast of the different traditional approaches; I am curious whether you agree with the website's assessments.   There were some key advantages to using the jejunum, notably a maintained peristalsis.  Look forward to talking later.  Here is that site if you get a minute and want to check it out, it's very straightforward.  http://www.childrenshospital.org/clinicalservices/Site2807/mainpageS2807P23.html

Dr Downey responded by sending me the following (heavy medical language) info and a recommendation that I visit the PCMC library to read more.  I am hungry to learn, so this was great.  But definitely sobering.

1. J Pediatr Surg. 2009 Oct;44(10):1928-32.

Surgical outcomes of esophageal atresia without fistula for 24 years at a single 
institution.

Holland AJ, Ron O, Pierro A, Drake D, Curry JI, Kiely EM, Spitz L.

Department of Pediatric Surgery, Institute of Child Health and Great Ormond
Street Hospital for Children, University College London, London, UK.
andrewh3@chw.edu.au

PURPOSE: The aim of the study was to evaluate the surgical outcome of esophageal 
atresia (EA) without fistula for 24 years at a single tertiary center for
pediatric surgery.
METHODS: The study used a retrospective chart review of infants diagnosed with EA
without fistula between 1981 and 2005.
RESULTS: Of 33 patients with EA without fistula, 31 charts were available. Mean
birth weight was 2327 g (range, 905-3390 g), and 71% were male. Most common
associated anomalies were cardiac (n = 6; 19%) and renal (n = 5; 16%), followed
by vertebral (n = 4; 13%) and anorectal (n = 2; 7%). The median initial
esophageal gap was 5 vertebral bodies. Six had a primary repair, and 25 patients 
had esophageal replacement at a median age of 7 months. This involved gastric
transposition in 20 (1 followed failed jejunal interposition), colonic
interposition in 5, jejunal interposition in 1 (after a failed colonic), and
repair at another center in 1. With a median review of 9 years, 21 patients had
long-term sequelae with the need for multiple further surgical procedures
including an antireflux procedure in 5. One patient died.
CONCLUSIONS: Management of EA without fistula remains challenging. Most patients 
required staged treatment that included esophageal replacement. The frequency of 
late complications indicates the need for programmed long-term review.

PMID: 19853749  [PubMed - indexed for MEDLINE]


2. Semin Pediatr Surg. 2009 Feb;18(1):44-9.

The surgical approach to esophageal atresia repair and the management of long-gap
atresia: results of a survey.

Ron O, De Coppi P, Pierro A.

Department of Paediatric Surgery, Institute of Child Health & Great Ormond Street
Hospital, London, United Kingdom.

The optimal approach for esophageal atresia (OA) repair and technique used for
long-gap OA repair are controversial. There are few data comparing the outcomes
of the different approaches and techniques. We performed a survey of current
practice of 88 pediatric surgeons and asked experts to provide us with
definitions and rationales behind their management strategies. There were no
differences between UK and non-UK surgeons. Although the majority of pediatric
surgeons perform minimally invasive surgery (68%), only 16% have performed
thoracoscopic OA repair; however, 46% are planning to carry out thoracoscopic OA 
repair. Gastric interposition is the most preferred technique for long-gap OA
when primary anastomosis is not possible, with 94% of those surgeons who use the 
technique satisfied with it. Growth of the esophageal ends by traction is the
other major technique used, but only 76% of surgeons who use it are satisfied
with it. Most surgeons repair < or =2 patients with long-gap OAs per year.
Long-gap OA should be managed by a limited number of surgeons at each center.
Even among experts, there is little consensus on the definition of or the optimum
technique for repair of long-gap OA.

PMID: 19103422  [PubMed - indexed for MEDLINE]


3. Pediatr Surg Int. 2005 May;21(5):369-72. Epub 2005 Apr 13.

Ileal pedicle grafting for esophageal replacement in children.

Bax NM, Van Renterghem KM.

Department of Pediatric Surgery, Wilhelmina Children's Hospital, University
Medical Center, P.O. Box 85090, 3508AB Utrecht, The Netherlands. N.Bax@wkz.azu.nl

Reconstruction of the upper esophagus in small children remains a challenge. Free
jejunal interposition as frequently used in adults is much less appropriate in
children because of the limited vessel size. The use of a jejunal pedicle graft
in children has been described, but gaining enough length may be a problem. A
pedicle graft of terminal ileum may be a better option, but this technique has
never been described. We report a child with esophageal atresia and distal
fistula who had a very short upper esophageal pouch. Primary repair was
impossible. The fistula was ligated and a gastrostomy created. A second attempt
at anastomosis was not successful either, and a cervical esophagostomy was
created. The child was fed by gastrostomy and received sham feeding orally. When 
the child was 10 months old, the upper esophagus was successfully reconstructed
with a pedicle graft of terminal ileum. Postoperatively there was a limited leak 
of the proximal anastomosis, which healed spontaneously. The distal anastomosis
had to be dilated on a few occasions. With a follow-up of 1 year, the child is
eating well without gastrostomy supplementation. On imaging, the ileal pedicle
graft looks somewhat tortuous but contracts nicely. We feel that ileal pedicle
graft reconstruction of the esophagus should be part of the instrumentarium of
pediatric surgeons dealing with esophageal reconstruction.

PMID: 15827751  [PubMed - indexed for MEDLINE]


4. J Pediatr Surg. 1999 Nov;34(11):1630-2.

Reoperation after esophageal replacement in childhood.

Dunn JC, Fonkalsrud EW, Applebaum H, Shaw WW, Atkinson JB.

Division of Pediatric Surgery, UCLA School of Medicine and Department of Surgery,
Kaiser Permanante, Los Angeles, CA 90095, USA.

BACKGROUND: Esophageal replacement is associated with significant morbidity that 
may lead to operative interventions. This study reviews the management and
outcome of children who underwent reoperation after esophageal replacement.
METHODS: Eighteen patients who underwent esophageal replacement from 1985 to 1997
were reviewed retrospectively. Ten patients underwent reoperation. Patient
management, perioperative morbidity, and the dietary intake at follow-up were
recorded for each patient.
RESULTS: Of the reoperated patients, 7 had esophageal atresia, 2 had caustic
ingestion, and 1 had achalasia. Nine patients received a colon interposition, and
1 received a reverse gastric tube as the initial esophageal replacement. Seven
patients required revision of the anastomoses. Three patients required complex
esophageal reconstruction: 1 underwent gastric transposition, 1 underwent free
jejunal graft, and 1 underwent gastric transposition combined with free jejunal
graft. Seven patients were eating well at follow-up. Two patients still required 
partial gastrostomy tube feeding. One patient died 6 months postoperatively from 
aspiration pneumonia.
CONCLUSIONS: Esophageal replacement continues to be a challenging operation
associated with significant complications. Most reoperative procedures were
directed toward strictures and persistent fistulae. Complete graft failure can be
managed by gastric transposition or free jejunal graft. Despite the perioperative
morbidity, most patients have good functional outcome.

PMID: 10591557  [PubMed - indexed for MEDLINE]


5. J Pediatr Surg. 1993 Aug;28(8):990-4.

Development of a technique for jejunal interposition in long-gap esophageal
atresia.

Cusick EL, Batchelor AA, Spicer RD.

Department of Paediatric Surgery, St James's Hospital, Leeds, England.

Primary anastomosis is the treatment of choice in esophageal atresia. There
remains a small number of infants in whom establishment of primary esophageal
continuity fails or is unrealistic due to a long gap. Esophageal reconstruction
then necessitates an interposition/transposition procedure. The most widely used 
tissues are colon and stomach but each has significant limitations. Jejunum is
theoretically the ideal esophageal substitute being of appropriate diameter and
having good peristaltic activity. Its use in the past has been limited by the
precarious blood supply and restricted length which result from the short
mesenteric pedicle. We have attempted to resolve these limitations by using a
microvascular anastomosis to augment the blood supply to the proximal jejunum in 
a series of 5 cases (2 pure atresias, 1 esophageal atresia with proximal fistula,
and 2 atresias with distal fistula). In a sixth case (atresia with distal
fistula) a free jejunal graft was used. In one case initial surgery was confined 
to cervical esophagostomy and feeding gastrostomy, in the remaining 5
interposition was necessitated by failure of a primary repair. The age at surgery
ranged from 8 to 16 months. The development of the technique and outcome in each 
patient is described. We conclude that a free jejunal graft is preferable to
augmenting the native blood supply and intend to continue with this latter
technique.

PMID: 8229605  [PubMed - indexed for MEDLINE]


6. J Pediatr Surg. 1988 May;23(5):483-9.

Long-term results of jejunal replacement of the esophagus.

Saeki M, Tsuchida Y, Ogata T, Nakano M, Akiyama H.

Department of Surgery, National Children's Hospital, Tokyo, Japan.

Since 1969, jejunal interpositions have been carried out in 19 patients for
esophageal replacement. A segment of the upper jejunum was used to bridge the gap
in the right thoracic cavity. The distal esophagus and its sphincteric mechanism 
was preserved in all but four patients, who had peptic strictures. Early
postoperative complications such as total necrosis of the graft, perforation of
the graft, and anastomotic leak developed in three patients (16%). There were no 
operative deaths but there were two later deaths (11%). We were able to follow 12
patients over a long term. Among these 12 patients there were two anastomotic
strictures, one of which was dilated successfully by bouginage, and one marked
redundancy of the jejunum which necessitated surgical correction. Both height and
weight were lower than -2 SD on a Japanese standard growth curve in two patients 
who had anastomotic strictures. Transient stagnation of swallowed barium at the
lower esophagus was the common finding; it was observed in seven cases (58%).
Only three patients (25%) complained of occasional feelings of delay in
swallowing. Stagnation with a mildly redundant jejunum was the common radiologic 
finding in these three patients. Reflux of the gastric content into the esophagus
did not occur. All the patients, except one who still has dysphagia due to
anastomotic stricture, can eat anything they wish at almost normal speed. These
long-term results indicate that jejunal interposition with preservation of the
lower esophagus is a recommendable procedure for esophageal replacement.

PMID: 3379557  [PubMed - indexed for MEDLINE]