Special Guest Mom Post: Ahlea's Story

I'm excited to share another mom's branchial cleft fistula story here on the Branchial Cleft Abnormalities blog.  Thanks Ahlea, and remember to check back in with us in December to report how the removal surgery went!!

I'm trying to build up the blog with more stories/pictures of real experiences by parents.  If you would like to contribute a blog post reflecting on your child's journey, please write a short summary of your child's case, how it was resolved and which doctors/hospital you used, advice you would offer other parents and before/after/AFTER surgery pictures.  Our community of parents who visit the blog (like you did!) for education and a "real world" view of their child's condition and what to expect would thank you! 

AHLEA'S STORY: When my little girl was 2 days old I noticed a pore on her neck. I mentioned it to our pediatrician and she ordered an ultrasound. At her 2 week appointment the pedi told us she didn't see anything but she wanted to have an ENT take a look at it. 2 weeks later we met with the same ENT that put tubes in my sons ears and took out his adenoids. She is the one that diagnosed my daughter with a branchial cleft fistula. She gave a very vague description of what it was then mentioned removal. Honestly, the moment she said the word surgery I went blank. I don't remember one word she said after that. 

We live in a small town and our hospital is a small county hospital that isn't very advanced. I loved the ENT there but had no idea what was going on with my girl. She gave me a referral to an ENT in Champaign, IL at a bigger hospital. We got a better idea of her condition. However, I wasn't comfortable with the guy we met. He told us he would want to remove it within 3-4 years but by 10 at the latest. This was the day I turned to Google. I still didn't completely understand her diagnosis but definitely should not have tried to get more information on my own. And that Images button! Oh don't even consider it! When she was 3 months old she got RSV and there was green goo coming out of her neck. They didn't seem concerned but it freaked me out. 

Feeling panicked? Read this.

When I started this blog, I did it mainly because I couldn't find a single picture of what my child would look like post-open neck surgery.  Would she be scarred for life?  For a few years?  Hardly at all?  What was I subjecting my girl to by proceeding with branchial cleft extraction--in REAL terms, not a medical journal article or WebMD synopsis?  I thought if I had desperately wondered about these things, others might too.  So I did a simple parent's blog on this rare congenital disorder and hoped one or two parents (let's be honest...mostly moms :) would see my baby's pictures and feel comforted in a way I hadn't been.

Two years later, we've had over 6,000 pageviews on this site by parents all around the world.  I believe it's still the only parent site dedicated to this condition (although I would love to link to anyone else out there if you know of one!).  And while I never expected it, the reader comments I get have been touching, inspiring and rewarding on their own.  These are parents that have found comfort here after researching a suspicion, or getting the diagnosis, or preparing for surgery the next day.  Sometimes, I get one like I did today that makes me want to reach out and hug the parent that has found us:

BradnKimmySeptember 5, 2013 at 12:30 AM

Hi Kelly. I have a 3.5 month old son. He was born with a small indentation on his lower neck (docs all gave the "baby acne is normal" excuse) but it never went away. I've kept my eye on it since day 1 and noticed that if I pull the skin tight around it, it looks like a small red pimple, then if I gently squeeze the area a clear, mildly sticky fluid comes out... I haven't yet brought him to a doc about it because I finally starting doing the online research tonight and ran across your blog.

I'm so scared that this is what he has. Im only 22, just got married April 2012, had my son May 2013... I just feel like the past year has been a blur already and now THIS is a potential threat. But I would like you to know that ready through all of your posts is helping me catch my breath and stop panicking quite as much. I'm crossing my fingers that he doesn't have this, but I think it's pretty clear to me that he does... I'm really happy to see how amazingly your daughter has recovered, though!

BradnKimmy, I'm giving you and other panicked parents out there a big old virtual hug, because I know how overwhelmed you must feel.  Having a baby with a medical condition is scary and big and intense, especially when it's a weird one you have never heard of and might require them slicing into his neck.  Couple that with all the other "stuff" of life and it seems a little unfair. But  I've been married 10 years, and my oldest is now a first grader, and while I'm no expert I do know for sure that it's always something.  My oldest had pilomatricoma (see HER blog here...) and between the two girls and their surgeries I spent six nights in Mott Children's Hospital in 2011.  I thought my heart would break, I thought *I* might break, but I didn't, and we made it. And you will too. 

Advocate for your child, get him or her the best care possible and take care of yourself and your spouse in the process, but above all, take a deep breath, because this is not fatal, your child will be okay, and a branchial cleft abnormality is but a small bump in your long parenting journey.  Hugs and blessings.  xoxo ~Kelly

Yep, our Branchial Cleft surgeon Dr. Green is pretty brilliant...

Scott Hollister, left, and Dr. Glenn Green of the University of Michigan
used plastic particles and a 3-D laser printer to create the first-ever airway splint
to save the life of a baby boy who used to stop breathing nearly every day. / AP

When he's not working on rare branchial cleft abnormalities, our surgeon Dr. Glenn Green of the University of Michigan is making pediatric ENT history!  Check out this MSNBC story on him and his team creating the first-ever 3-D laser printed implantable device, in order to save the life of a little guy with a constantly collapsing trachea.  Moral of the story?  Always find a world-class surgeon to open your baby's neck.  

If you loved your branchial cleft surgeon and think he or she is world-class, tell us!  Let's share our best branchial cleft surgeon recommendations from around the country in the comments to this post and make sure every parent can find a great surgeon in their region to consult with on this rare congenital disorder!

UPDATE: Two and Half years later...

Hi readers! I have got a few comments recently and figured it might be time to post an update. I'm so thrilled you have found us (look, it's a community!) and hope our story (and others in the comments) might help you navigate your child's branchial cleft abnormality path a bit better. Here's a picture of our daughter 2.5 years after her surgery. We've had NO problems and her scars are very hard to notice (there's one horizonal one about 1 inch and a smaller one at the old opening site that's about a centimeter:

Can you see them?  Didn't think so...It's pretty hard to photograph well...

How about now?  That's the larger one in the middle of the photo and the small one is at the bottom.  I think she'll be good to go by Prom dress season!

 God bless all of you and please, continue to share your stories and let us know how your little one's journey turns out!

Four months post surgery

Her scars look quite good...yay!

We're done!

Surgery is over!  It went well, although not as ideally as branchial cleft fistulas CAN go.  Dr. Green, from U-Michigan Pediatric ENT, conducted the surgery and she was in good hands.  Here's the basics:


Our daughter's branchial cleft abnormality was a fistula tract, with one opening at the base of her neck.  Dr. Green actually SHOWED us what he excised--the tract looked like a bloody red nightcrawler worm (I actually thought it would make good fishing bait!).  About the width of a pencil around and about 7 inches laid flat.  This was connected at the top of her neck to her tonsils, so they had to come out in order to get all the cyst/fistula material out.  If it's not all removed, it's possible it can regrow.  So our little 16 month old had her tonsils removed and two large incisions on her neck.  The one at the base of her neck was 4 stitches and the one further north was 9 stitches.  These are ugly, large visible scars and we hope they will fade with time.  But as a parent reading about this surgery beforehand, it's best to prepare yourself for the Frankenstein look on your child's neck.

Her recovery was okay.  The first night we spent in the hospital so she could be observed for infections or hemotomas, and hydrated intravenously.  She went home the next day with antibiotics and Tylenol 3 (with codeine).  She hated the T3 so we switched her to regular Tylenol on day 3 and she seemed unaffected.  The first night home we woke her up to get the T3 but after that we let her sleep and she did okay. 

We are going to get the stitches out in a few days and then plan to be DONE with this ordeal!  God bless anyone who has a little one with this condition and I hope you have learned from our story!

Parental Googling--AKA, a doctor's worst nightmare

Yes, I've become "that mom"...a week has passed since we heard Kate would need open-neck surgery on her branchial cleft fistula.  Now that I have had some time to think about it, I'm flabbergasted the best treatment for this is a one-inch incision to remove a 2 cm tract.  Really?  I had to do some investigating.  I found two interesting studies, both by Texas doctors who had done a cauterization on the tract.  I'm not 100% sure either of these findings exactly parallel what Kate has, since the terminology starts to swim around on me a bit, but it seems at least worth investigating further:

 
Endoscopic cauterization of fourth branchial cleft sinus tracts.

AND:

Periform Sinus Tracts in Children

So, I did what any slightly obsessive, over-protective mother would do--I contacted both these doctors and asked them more.  Dr. Green and U-M both have my fullest confidence, and if we need to do the full surgery, they're getting our business.  I just need to know I have exhausted every non-invasive, progressive option that exists and made sure it is not a fit for Kate's particular case.  Who knows, perhaps our family will pioneer the technique of cauterizing, not surgically excising, these branchial cleft abnormalities at University of Michigan hospital!!??!

A Branchial Cleft WHAT?

We finally met with the University of Michigan's Pediatric Ear Nose and Throat specialist who could tell us more about the procedure related to Kate's branchial cleft cyst. But of course, our story doesn't start with the ENT consultation yesterday; it began 10 months ago, on the day our baby girl Kate was born...

She was perfect in every way, but I am an overly observant (DH would say "nit-picky") mother so I continued to look her over. I noticed, during our first night at the hospital, she had a small open pore at the base of her neck. It seemed to be "leaking" and then crusting over. I thought it odd but newborn skin is notoriously "acne"-prone due to the birth hormones so I assumed it would quickly resolve. Over the next two weeks, it would occasionally leak small amounts of mucus-like material, which would crust on her shirts (pretty gross). On her two week birthday, she fell ill with a high fever and was hospitalized at University of Michigan's Mott Children's Hospital for several days. In my quest to discover the source of the fever, I mentioned to the doctor this tiny pore on her neck that seemed to still be leaking on and off. This bit of information drew immediate attention, to my everlasting surprise. The staff suspected the "open pore" might be the end of a thyroidal cyst or tumor. Okay, now you have my attention! They sent her for a neck ultrasound and discovered it was likely a branchial cleft cyst, a congential defect that wasn't causing the fever (which did go down) but WOULD continue to be a health concern and require surgery at a later date. HUH? I was flabbergasted.

Kate's "pore" was really the opening to a branchial cleft cyst running vertically up her neck. We were told it would never close on its own now that she had been born and could, if left, become infected someday and threaten her nearby carotid artery. This birth defect runs in families and is otherwise benign. And then, it hit me--*I* had the remnants of a branchial cleft cyst on MY neck! A small colorless mole on the base of neck that I had had as long as I could remember was likely a branchial cleft cyst that had closed off before my own birth. And my older daughter had an identical mark on her neck, similarly closed before birth! So at least we know which "nit-picky" mother is to blame for this condition!

Fast-forward 10 months, during which Kate's pin-hole opening on her neck continued to leak fluid occasionally. The leakage seemed worse in the days before she came down with a cold (we learned that upper respiratory onset can exacerbate branchial cleft cysts, so this makes sense). But typically, we only noticed it leaking once a week or so. However, I could only imagine our beautiful daughter as a teen, preparing for prom and worrying about pus leaking out of her neck. If for no other reason, removal of the branchial cleft cyst was going to be necessary, and I know she'll thank us later. Details of our ENT consult in the next post...