Monday, April 28, 2014

Thyroglossal Duct Cyst - Sistrunk Procedure

As promised, this is a detailed post about my experience with a thyroglossal duct cyst and the Sistrunk procedure.  If you do not like medical stuff or gory pics, stop reading.  This is for people who are going through the same thing and want to hear about someone else's real life experience.

My thyroglossal duct cyst (TGDC) first reared it's ugly (and, yes, I mean ugly) head in August.  It came on after a upper respiratory infection as a sore, firm lump around the midline of my neck, above my thyroid.  I went to my PCP who said she suspected TGDC, but referred me to an ENT.  The ENT also said he suspected TGDC and sent me for an ultrasound.  He also put me on 10 days of antibiotics, in the hopes that it would clear things up.  He made it clear that if it was a TGDC that surgery would not be indicated unless I wanted it, or the cyst got repeatedly infected.

When we got back the results of the ultrasound we were shocked and scared.  The ultrasound showed that it was in fact a solid, vascular mass and NOT a cyst.  Obviously, this is NOT what we wanted to hear.  Since it showed up solid, the ENT sent me for an MRI for more information.  When I asked him what it could be, he said he didn't want to guess and he told me, "anything I can say will only make it worse."  The wait for that MRI felt like an eternity, and the wait for the results was even worse.  It ended up that the results of the MRI did show that it was a remnant of a TGDC.  We were thrilled and since the cyst had disappeared, we decided to wait and see if it got infected again before doing anything.

Sure enough, a couple of months later, it came back with a vengeance.  I made an appointment with the surgeon who is also the chief of ORL and the minute he saw it he said it had to go.  It had been infected several times, it looked bad, and my lack of spleen had him concerned.  He also noted that the tract went up very far to the base of my tongue.  He put me on more antibiotics and said it needed to come out once the infection was a bit under control, but ASAP.  Not what I wanted to hear a couple of weeks before Christmas, but at least it didn't give me too much time to worry about the surgery.

Because of my complicated medical history with thalassemia, I had a detailed pre-op visit, despite what the surgeon's admin wanted--she insisted because I'm "young" and "healthy" that I could just do it over the phone.  I knew this didn't sound right.  I contacted my hematologist who was really irritated by this and concurred that I really needed the full workup.  I had a transfusion a few days prior to the surgery to--as my nurse put it--get me all "shined up" and in tip-top shape for the surgery.  I also had a CT scan as the surgeon felt this would give a better picture of the cyst and duct than the MRI could.  It was really no big deal, and I didn't feel any weird sensations or have any metallic taste in my mouth.

The night before the surgery I had to stop eating at midnight, not that this was difficult as I was a total nervous wreck.  Fortunately my surgery was scheduled for the morning so I didn't have too much time to work myself into a frenzy.  I went up to the OR floor by myself and got changed, and then my husband was able to come up and sit with me.  I talked to a zillion people and answered many of the same questions over and over, which was fine with me.  The anesthesiologist was very nice.  He placed my line, using lidocaine first, although I told him that I was really a pro at getting IVs.  He also told me that they were going to intubate me through my nose to keep my oral cavity open because the surgeon wanted to do some exploration through my mouth, and might possibly have to do some of the procedure that way as well.  I had to squirt something akin to Afrin in my nose several times to help with this and to also help prevent trauma to my nose.  The surgeon came in to chat with me.  He was a lot more chipper than I was.  That's when I got really scared and started to cry.  Pretty soon after that I said goodbye to my husband and was wheeled into the OR.  The anesthesiologist put a mask on me and said it was oxygen to really fill my lungs, and after that I remember nothing else.

The next thing I knew I was being wheeled down the hall and the surgeon was joking about it being like a spa day and I got to relax.  I tried to talk and weakly asked him if he painted my toes and we laughed.  I was in and out of sleep in post-op.  At first they asked me to rate my pain and I was like, "what pain!?" but not long after that my neck and throat felt really uncomfortable so I was given some pain medicine in my IV.  I slept on and off.  I kept asking if I could go to the bathroom but they didn't want me to get up yet and I refused a bedpan so I waited maybe an hour and they let me go.  In the bathroom I saw a pretty huge bandage and the drain coming out of one end.  I was a little surprised at how big it was.

I stayed overnight in the hospital's 24-hour observation unit.  The bedspaces are just separated by curtains but it ended up being a nice quiet unit, at least on the night I was there.  My husband and sister came to visit so that was a nice distraction, but the pain was building so I was keeping on a 4 hour schedule of liquid oxycodone.  I ordered just about every soft food on the menu and was sure I'd at least be able to eat chicken soup, but even the soft noodles wouldn't go down.  Really, nothing would.  I generally felt OK while my visitors were there, but as soon as they left I started to feel absolutely awful.  The pain in my throat was terrible and I kept complaining to the nurse and was literally counting the minutes for my oxycodone.  She said that they would not give pain medicine on a schedule, that the patient needed to be awake and asking for it, but I knew there would be no problem because there was no way I'd be able to sleep.  I kept asking her if there was anything at all they had that would make my throat feel better and it took her 3 hours for it to dawn on her to offer me popsicles!  They ended up being a lifesaver that entire first night.

The next morning the resident came in before 5am to take a look at me and he said he'd be back to remove the drain.  The drain was grossing me out, but the thought of getting it pulled out was even grosser.  The drain filled up with a quick 30cc right after the surgery, but after that it slowed down quite a bit and probably only did another 30cc through the entire night and morning.  Again I ordered lots of soft food and liquids but the only thing I could get down was the Dunkin' Donuts coffee my sister brought me.  The resident came back to remove the drain.  He clipped one stitch and then just pulled this long tube out of my neck.  It went across the entire front of my neck and I could feel it being pulled out.  It was gross, but it did not hurt.  I was released to go home before noon.  The hole where the drain had been was simply covered with a piece of gauze, and the incision was covered with steri-strips that I was told would fall off in a few days (they did not).

I came home with the usual, "I'll be able to go back to normal!  I don't need any pain meds!" feeling that people get when they are sprung free from the hospital.  By the time I got home, I was totally exhausted and my neck and throat were killing me.  Since I wasn't really able to swallow, we got all of my meds in liquid form:  Oxycodone, Tylenol, and antibiotics.  One tasted grosser than the next, so my stomach felt quite queasy.  Swallowing really hurt, and I often had the sensation that I was choking on my saliva.  My mom made chicken soup and I was able to drink the broth.  I know that some people say they are eating sandwiches the day of their surgery, but there is no way in the world I would have been able to do that.  That night I had to sleep sitting up both because of the pain in my neck, and because of the choking sensation I kept feeling.  If I had not taken the Oxycodone, there would have been no way I could sleep.  I actually slept upright for over a week after the procedure.

The next few days were much of the same.  Laying on the couch, taking medicine, and only eating soft foods and liquids.  I was able to wean myself off the Oxycodone after just 2 days, but continued to use the Tylenol.  It continued to hurt to swallow for about a week.  For the first few days I was not able to eat anything very solid or dry.  The first time I tried to eat chicken I choked.  By the end of the week I was able to eat small pieces of solid food if they were moist.  I would say in two weeks time I was eating normally.  I did not drive or do any heavy lifting (ie, pick up my kids) for a week.

At one week I had a post-op appointment.  The surgeon took off the steri-strips.  There were no stitches to remove as they were all internal and dissolvable.  He said that everything looked good, and as expected.  He discussed the pathology report with me which proved that the cyst was totally benign.  He also gave me the operative report.  He noted that since a fair amount of underlying tissue needed to be removed from my neck in order to remove the entire cyst, that he had to implant some AlloDerm to make it look right again.  This explained the rectangular firm thing I could feel in the front of my neck.  (It got softer over time.)  I saw him again in another month and he thought that things looked good.  He noted that my neck was still swollen, but that it was to be expected.  He said that if the scar did not look better at our 6-month visit that he could do something in the office to "correct" it.  I'm not sure what that means.

As far as my neck is concerned, it started off very swollen.  The swelling was from my chin to under the scar.  For a long time I thought I looked like a linebacker and I was worried I would always have a fat neck.  It actually took months, but it went completely back to normal.  My tongue was almost completely numb from something that happened during the surgery (maybe I bit my own tongue, maybe from a tool they put in my mouth, maybe from a temporary stitch they put in my tongue to pull it out to look at the back of it-I'm not going to ever know).  At first, it even effected the way I ate and spoke.  I was terrified that it would not go back to normal, but it gradually did over about 2 months.  A large portion of the front of my neck from my chin to below the incision was numb, also.  The surgeon said it was from all of the tiny nerves being torn when my skin was pulled back to do the surgery.  He said that it would take several months to go back to normal.  At almost five months, the sensation in the front of my neck is improving, but far from normal.  The scar is a straight-ish line, longer than I expected it would be at about 7cm.  It is on one of the folds in my neck but I personally think that it is still quite visible.  It has flattened out and gotten less red over time, but at almost five months still doesn't look great.  I was told it can take a year for a scar of this nature to look good and be barely noticeable.

So, here's what I learned from this yucky experience:
1.  If you have an ultrasound and it says you have some awful horrible solid mass in your body, it might not be.  Get more imaging done.
2.  If you don't have a spleen or you are super anemic or if you have some other issue that puts you at higher risk than a "typical" person, go ahead and get the full-on pre-op done.  The extra visit is inconvenient, but probably worth it.
3.  If you are having the Sistrunk procedure, make sure to get all your meds in liquid form.  I don't care what anyone says, swallowing probably isn't going to be very easy or pleasant at first.
4.  If the nurse is too dumb to offer you popsicles, ask for them.  They are SO soothing, and if you are like me, probably the only thing you'll be able to eat that first day.
5.  Hopefully you have a great family like mine and they'll stock you up with lots of soft, yummy foods for when you feel like eating.
6.  Stay on the pain meds for the first day or two and don't fall behind.
7.  If you have that gagging feeling after the surgery, sleep sitting up or at least partially upright.
8.  Be patient.  (I'm still in this stage.)  I'm sure it depends, but if you have a large incision like mine, it's probably going to look crappy to you for awhile.  It does start to get better.  And, if you lost sensation anywhere (tongue, neck) be patient about that, too.  Zillions of nerves regenerating take a long time.
9.  If you hate the way your neck looks before or after the surgery, buy yourself some nice scarves.  They are very fashionable, anyways.
10.  You might be terrified.  I was terrified.  And, in my opinion, that is OK.  They are cutting your neck open and rooting around near some pretty important stuff.  So, it's OK to be scared.  Before you know it, it will be behind you and you'll wonder how you walked around with that yucky looking thing on your neck.

So, since a picture says a thousand are some pictures.  Again, if you are not here to read about the Sistrunk procedure or thyroglossal duct cyst, stop looking now.  I'm being very brave sharing these as they aren't exactly, ahem, flattering...

This is the end of October 2013.  The bump had gone away and then came back
with a vengeance.  The next week I saw the surgeon and he said it had to come right out.

This is when it was at it's grossest.  This is two days before my surgery and I was
very worried because it looked so angry.

One day before surgery.  It looked like it was going to pop out of my neck.  For this reason,
the surgeon had to cut away part of the skin that the cyst was attached to on the outside.

This is a few hours post-op.  You can see at this time, it wasn't really even
swollen yet.  If you look carefully, you can see the drain on the left side
of my neck.  I'm smiling because I was drugged and laughing with visitors...the worst
was yet to come.  The pain that first night was really bad.

Day after the surgery.  You can see the swelling.  It got worse before
it got better.

Just over one week post-op after seeing the surgeon and having the
steri-strips removed.  I thought it looked horrible.

Just over one month post-op.  My neck was still definitely swollen.

Almost 5 months post-op.  Much less swollen and flatter, but I think the scar still
looks pretty noticeable and crummy.

Monday, April 21, 2014


Well, this is where the rubber meets the road.  Emmie's first Ferriscan was scary because, well, I find general anesthesia to be scary and having your child "go under" is a million times scary.  But we sort of knew that the results would be less than stellar because she hadn't started chelation yet.  And, as promised, the results were in fact less than stellar.  (For you Ferriscan experts out there, her cardiac T2* was 21 and her Ferriscan liver iron was 25.  For you non-experts, that's pretty low for cardiac T2*, and way too high for liver iron.)

This year was scary times 10.  I was still terrified about the general anesthesia even though I knew better what to expect, but we were also really anxious about the results.  At the time of her 2nd Ferriscan, Emmie had been on Exjade for just about 12 months.  As you might have read here, we fought about it, cried about it, gagged over it, and struggled a LOT to find a way that was palatable for her to take the medicine.  In the end, Emmie took every dose of Exjade in yogurt or drinkable yogurt.  As I stated in my post about Exjade, I felt pretty good about this as it was recommended by our hematologist (one of the leaders in the field) and because there is one study and lots of anecdotal evidence to suggest that Exjade works in food despite the fact that it is not on the label.  However, now and again I have heard other parents saying that their doc doesn't recommend using Exjade in food, or that Exjade doesn't work well in dairy products, and so on.  Still, my stance has been that having Emmie take the Exjade in food seems preferable to her not taking it at all.  And, if it's not working, there's always Desferal....

So, the Ferriscan.  Overall, our experience was better.  The hospital allowed us to do pre-op for both girls on the same day, which was helpful.  Our experience in pre-op was NOTHING like our experience last year.  Last year there were literally patients everywhere, even flowing out into the hallways waiting to be seen. This year we were the only patients there, and we were in and out in an hour.  It would have been less if I didn't make a habit of asking the same questions over and over.

We got the call the day before the scan that Emmie would be one of the first scans of the day, arriving at 7:15 am.  We were so relieved to hear this, as it's less time for her to be fasting, and it's less time for me to be hysterical waiting.  We were taken back pretty quickly into what I'd like to call the "Scary Anesthesia Room."  This time, I wasn't the only one who was scared.  (You might remember last year Emmie was mesmerized by the TV, not noticing anything else in the room.)  Emmie's eyes were as big as pie plates as she watched the nurse fill syringe after syringe, laying them all out on the table in full view.  It seemed everywhere she looked there were more syringes or tubes or face masks or you name it.  It was so obvious to me how being just one year older made it so much scarier for her.  She knew something "bad" was coming, and there was NO distracting her.  Well, except for the Versed.  So we very quickly had the Versed/mask discussion with the anesthesiology team and we all agreed that since everything went well last time that we'd just repeat that.  (Of course we got the disclaimer that from one time to the next children can have different reactions to the same drugs, so we weren't totally in the clear.)  This time I let the nurse give Emmie the gross-tasting Versed in the syringe and she cried and gagged a bit, but then it was almost as if the drug started to take effect immediately.  I'd say in 5 minutes she seemed six sheets to the wind, slurring her speech, cuddling me, and calmer.  We laid her on the bed and put Curious George on and she kept asking if the people were "real" or in the TV.  At 8:20 it was time for us to leave the room, and even though Emmie was pretty well drugged, she cried such a sad cry that really broke my heart because last year she did not cry when I left.  We took comfort in knowing that the Versed not only relaxes you, but it also gives you amnesia, so she has no memory of us leaving her.  I could see through a crack in the window as we left that the nurse had the mask on her face and that she seemed peaceful.

As promised, we got a call at 9:15 saying that everything was going well and that she had about 20 minutes left.  They told me that she was given "laughing gas" at the beginning, and then had an IV inserted in her right hand, and then she received the general anesthetic.  They said she was comfortable and doing great, and that they were getting great pictures.

At 9:40 we were called back to recovery.  Emmie was extubated in the procedure room and woke up enough to breathe on her own, and then went back to sleep, likely from the remaining effects of the Versed in her system.  She seemed peaceful and after about 15 minutes she woke up normally and gracefully.  She was happy to see us, and right away asked me to get in bed with her so she could lay on me.  Her voice was a bit scratchy.  She asked for a popsicle and was looking for her Leappad to watch Caillou, both signs that she was feeling pretty good.  Three popsicles later she was doing great and given permission to leave.  That was probably before 10:30.  Emmie was chatty, hungry, and very clingy on the ride home.  She wanted me to somehow navigate the curves of Storrow Drive while reaching into the back seat to hold her hand.  Which, I was happy to do.

After that it was a pretty normal day.  She had a great lunch at Nana and Grampy's, played, and took a nap.  She woke up asking for spaghetti, so I made a tuna pasta puttanesca-style that she ate two huge bowls of.

 The only thing she really gave me any flack about all day was taking her Exjade before dinner.  How yucky it tastes, how the stuff at the bottom of the glass is gross, how she needed an M&M to get it down.  And, as we fought about it, I had this sinking feeling in my stomach.  What if, after all of this Exjade stuff for a whole year, she's one of the 30% of patients who are non-responders to the drug?  What if those moms were right and Exjade doesn't work well with dairy products?  What if we haven't been crushing it enough?  What if the sprinkles and Dora yogurt and daily Exjade Happy Dance and now splitting the dose so we do it all TWICE a day...was all for nothing.

But it wasn't.

The NP called the next morning with literally some of the best news I've ever heard.  Emmie's liver Ferriscan was down to 5 and her cardiac T2* was up to 35!  I cried and cried and nearly dropped the phone as I asked her to repeat those numbers again and again while I waited for the report to show up in my inbox.  Yes, Emmie's ferritin had started to come down slowly, particularly in the last two months.  But I never, ever thought that we would see results like this--actually, some of the BEST results I have EVER heard of.  With her latest ferritin still at 2200, even KNOWING what I know about the lack of correlation between ferritin and total body iron, I never expected her to have a liver iron in the normal range.  And when the NP said to me, "Do you remember the Exjade non-responder study we worked on?  Well, I would classify Emmie as a responder."  To which I replied, "Yeah, you freakin' think?!?!?"  Very scientific of me.

So, she did it.  Well, being honest here...we all did it.  While the bulk of the burden was on Emmie to take the Exjade, I'd be lying if I didn't say that it was a team effort.  So we hugged her tighter than ever and went to The Cheesecake Factory to celebrate.  And when we asked her why we were having a family celebration, she said, "Because I did a good job with my medicine, and because my belly pictures were great!  And I don't even remember them putting the IV in!"  Spoken from a real trooper, a great role model, and our inspiration.

Relaxing while the Versed was taking effect.  Emmie, not Mama.
I was totally hysterical and could have used some myself.

This is just a few minutes after she woke up!  Lookin' great!

I was feeling SO SO SO relieved when this picture was taken.

Nothing like having my baby back in my arms.

Exactly one week later, we'll be back, doing it all over again for Rosie...