Lip Reduction Surgery – June 18, 2014

Atticus had his first lip reduction surgery on June 18, 2014.  His plastic surgeon removed tissue from the inside of his bottom lip, tightened up some stretched out muscles, and stitched up the incision with three layers of stitches.  Over time, the scar tissue may help to curl the lip upward.  The focus of this lip reduction was on the inside of the lip.  Atticus will need other procedures done to the outside of the bottom lip and possibly more surgeries for the inside of his lip.

Atticus spent one night in the hospital.  He ate and drank with no problems after surgery.  It is really amazing how well he is able to eat post-surgery.  Nothing gets between Atticus and his food!  Because he ate and drank so well, and was responding to the pain medication, we were able to go back to Charlotte the next day.

Of course, as with any surgery, the bottom lip is currently swollen.  It usually takes a few weeks for all of the swelling to go away.  Once all of the swelling is gone, we will be able to see the true results of the surgery.  It may take a month or so for all of the stitches to fall out too.

Atticus had quite a bit of pain in the days following surgery, but now he is almost off pain medicine.  He is pretty much back to his normal self.  Every once in a while he points to his lip and says, “It hurts.”  But that is usually when we are cleaning it or applying Normlgel to it.

So far the healing process is going very smoothly.  Atticus is not bothering the stitches.  The only things we really need to do are clean the lip with sterile medical sponges after meals, apply Bacitracin twice daily for about one week, and apply Normlgel as needed to keep the lip moist.

We are eager to see the true results in about a month!

 

Interstitial Laser Treatment – Round One 1/29/14

Atticus had his first round of interstitial laser treatment on his tongue on January 29, 2014.  We arrived in Winston-Salem the night before and got settled in at the Ronald McDonald House across the street from Wake Forest Baptist Medical Center.  Our drive from Charlotte to Winston-Salem was not typical for North Carolina – we actually had snow and now I understand why everyone in NC freaks out at even the mention of snow – because they don’t really treat the roads.  They just don’t have the capacity to treat all the roads because snow is such a rare occurrence.  The highways were pretty bad, but we were careful, and made it safe and sound.

The following day we arrived at the hospital at 10 am.  The surgery was supposed to take place at 11 am, but they didn’t end up taking Atticus back to surgery until around 1 pm.  Atticus was a trooper though.  He hadn’t had anything to eat or drink for well over 12 hours, but we kept him busy enough in the waiting room and I don’t think he even noticed the hunger or thirst.  Because Atticus is an only child, and doesn’t go to day care, we don’t get to see him interact with other kids a lot.  There was a little girl (maybe 3 or 4) in the waiting room playing with these cool little hamster things that go when you press a button on their back.  Conveniently, she had two and when Atticus expressed interest, she let him play with one.  At one point, she looked at Atticus’ mouth confusedly and asked, “what happened to that baby’s mouth?”.  I answered, “he was born that way.”  She said, “Oh”, and went on playing. Every time Atticus would walk away, the little girl would say, “come back, baby.”  It was really cute.

After a couple hours in the waiting room, Atticus was finally called and things moved quickly after that.  Usually we wait for a while in the surgical holding area, but this time it was like bing, bang, boom and he was whisked away.  This time, they gave him a little something to make him relaxed before taking him back so that he wouldn’t have separation anxiety.

As he gets older and we get more used to these treatments, it’s not so much the worry about him having surgery – it’s seeing him sad, scared, or in pain that really gets to me.  I was glad to be spared having to see him scared and screaming for me and Brian as they took him back to surgery.

This procedure seemed to take a bit longer than previous laser treatments, mostly because of the addition of the interstitial lasering of his tongue.  After an hour or so, Atticus’ ENT came out to the waiting room and told us that the interstitial treatment had gone well – nothing unexpected.  He explained it to us as inserting a large and very wide catheter deep into Atticus’ tongue and then feeding the fiber through the catheter.  Then as he pulls the fiber out, he pulses it every 5mm or so.  So, even though there are only two incisions into the tongue where the catheter was placed, there are multiple spots inside the tongue that were lasered.  As the doctor described the procedure, I said how fascinating the whole thing is from a medical standpoint.  The doctor answered “Yes, it is fascinating…when it’s not your kid.”  He is right about that.  As I learn more about LM, it is fascinating what doctors can do and how far the treatment has come, but no one would wish any of this on their own kid.  And, I am always reminded of how far we still have to go and how some kids and adults are still really struggling, despite advances in treatment.

Pain and swelling so far have been the biggest obstacles.  Atticus was able to eat a few hours after surgery, so they felt comfortable sending him home the next day.  We were told that swelling would peak at 72 hours and then decrease from there.  We are currently almost at 48 hours out and the poor little guy’s tongue and lip are very swollen.  It is amazing that he can eat and drink through all the swelling and the pain.

Everyone is curious to see what the results of this treatment are going to be.  Right now, it’s a waiting game.  It could take a month or so before we really see what effect this treatment might have on the size of his tongue.  Luckily, I learned early on, even while pregnant to be OK with not having immediate answers.  I’ve grown patient and hopeful.  I take things one step at a time and am grateful for the positive things that are happening today, right now.  Like, the fact that Atticus is eating and drinking and that his pain can be controlled through medicine.  I’m grateful that there are treatment options out there for him.  I’m grateful that he is healthy and strong and able to withstand all of the surgeries that he is going to have to have in his life.  But, as I’ve said before, this is part of his journey in life.  A journey that may come with its obstacles, but one that will teach him the strength, pride, and resilience that will take him far in life.

Interstitial Laser Treatment

Atticus had an appointment with ENT today to discuss his upcoming interstitial laser treatment.  We had a lot of questions going into the appointment, and some were answered, but because of the nature of lymphatic malformations, no doctor can ever answer every question.  There will always be unknowns, and that is just something that me and Brian have learned to accept and be OK with.

I had done some research on interstitial lasering of venous malformations, so I went into the appointment knowing a little bit.  First, I have to explain that I’ve been calling Atticus’ malformation a lymphatic malformation, which is sort of the general term, but his actually has a large venous component and probably only a small lymphatic component.  I guess technically, it is a mixed venous lymphatic malformation.  There’s a whole classification system that was developed by Mulliken and Glowacki in 1982, if you’re interested.

Table 1.: ISSVA Classification of Vascular Anomalies

Vascular Tumors	Vascular Malformations
	Simple	Complex	Flow
Hemangioma Pyogenic granuloma Hemangiopericytoma	Capillary (CM) Venous (VM) Lymphatic (LM)	Lymphatico-Venous (LVM) Capillary-Lymphatico-Venous …	S L O W
Hemangioendothelioma Tufted angioma	Arterial Arteriovenous (AVM)	Capillary-Arterio-Venous …	F A S T

I’ll try to simplify the classification.  Vascular anomalies are a heterogeneous group of congenital blood vessel disorders.  These can be broken down into vascular tumors (the most common vascular tumor being hemangioma) and vascular malformations.  Vascular malformations can be lymphatic, capillary, venous, arteriovenous, or some combination of these.

Because Atticus’ malformation is venous lymphatic and goes deep into the tissue of his tongue, he is a candidate for interstitial laser surgery, which penetrates deeper into the tissue than a surface laser does.  Atticus is scheduled for his first treatment of this type on January 29th.  They will use an Nd: YAG laser and will enter the tongue with a catheter, which the laser will pass through.  They will probably laser two spots on each side of the tongue for a total of four spots.  Atticus’ team of doctors has not performed this specific type of laser treatment before, so they will be conservative on the first treatment and be more aggressive in future treatments, after they see how he responds.

No doctor has done a ton of these treatments because it is just not very common.  However, Dr. Gresham Richter in Arkansas has probably done more than any other doctor.  We are lucky that Atticus’ doctors have a direct connection to Dr. Richter and are consulting with him on the specifics of the procedure.  Atticus’ team of doctors feels comfortable and confident in their abilities to perform the procedure.

We asked about risks of the treatment.  The major ones seem to be internal bleeding in the tongue after the procedure, or the possibility of hitting a nerve in the tongue.  There are really no new risks to this procedure over any other procedure Atticus has already had.  We are expecting swelling, which will affect eating, so we will see how to deal with that after the procedure.  Because Atticus has a trach, swelling affecting the airway will not be an issue.

The most we can hope for is some shrinkage to the tongue.  We are not sure how much shrinkage to expect.  They are going to be conservative in their first treatment, so really, only time will tell.  We have to be patient and positive – two qualities that we quickly learned to embrace after Atticus was born.  There are no quick fixes or easy answers when dealing with vascular malformations.  At this point we are all too familiar with the answer of “I don’t know” from doctors.  While this can be unsettling, we appreciate the honesty of Atticus’ doctors.  For now, we just take it one day at a time, stay positive, and hope that this treatment will make a difference.

One Year Since Tongue Reduction

Today is special for a lot of reasons.  Not only is it Atticus’ year and a half birthday, but it is also the year anniversary of his major tongue reduction surgery.  I’ve really been thinking about how far he has come this past year.  Back on November 7, 2012, we didn’t know what to expect after the surgery.  The doctors performing the surgery had only done one other tongue reduction, so we had very little information about recovery, how much blood he would lose, how long he would take to heal, and what things would be like afterwards.  Would this affect his ability to eat, speak, or taste food?  There were so many unknowns.  Atticus stayed sedated for a full four days after surgery and was even on paralytics so that he could not move.  I try to imagine that happening now.  At the time, I was OK with it because I’d rather he not be in any pain.  Now that he is so active, expressive, and talkative, I can’t even imagine seeing him lay there for four days straight without moving.  Me and Brian really had to be strong during that time, for ourselves, but also for Atticus.  Babies pick up on the emotions of their parents, so no matter how much I had to worry about or how upset I could have let myself become, I stayed strong and happy and positive for Atticus.  I learned to fight for the needs of my child.  I realized more than ever that me and Brian know Atticus better than anyone and, despite not being medical professionals, we know best what he needs.

I am so grateful that I had my parents with us on the day of surgery.  The surgeons didn’t end up taking Atticus back to the OR until around 4pm and surgery went until 6:30pm.  My parents helped to pass the time and keep our minds on other things.  Soon after my parents left, Brian’s mom came and she was a huge help as Atticus started to wake up and begin healing.  I am so thankful that despite living over 600 miles away, our parents stepped up to the plate and helped us through a very trying time.

And now here we are – 12 months later, and Atticus is doing wonderfully.  Some days, I think that his tongue looks so good that you can hardly even tell that it is big.  You know that his tongue has gotten a lot smaller when little kids will only notice that he has a big bottom lip.  His tongue used to be so large, that you couldn’t even see his bottom lip.  But, that is all cosmetic.  The really important things – like his eating, speaking, and breathing – are another source of joy and thankfulness that we experience every day.  Although he still has his trach, his O2 saturations are always perfect (unless he is sick).  He does not let his tongue get in the way of his eating!  I hardly even have to buy the pureed baby food anymore.  He has primarily moved on to table foods – I just have to mash them or stick them in the food processor to chop them up.  His favorite food is still sweet potatoes.

His language development is amazing.  He says ‘baby’,’car’,’da da’, ‘Ollie’, ’airplane’,’apple’,’what’s this?’, among others.  He loves to point to things and have you say what it is.  Every time he hears an airplane overhead (even in the house – his hearing is amazing!), he points up and says ‘airplane’.  When we are out, he points to babies and kids, even 10 year olds, and says ‘baby’.  I laugh and tell him, “No, you’re the baby!”

Atticus is also exceptionally detail-oriented.  He points to a car and I say ‘car’.  Then, he points to every part of the car, even the headlights and taillights and he wants me to tell him what they are.  He is used to his nurses taking his pulse, so when the nurse puts the stethoscope up to his heard, he flips his little wrist over as if he’s looking at a watch to take his pulse.  He doesn’t miss a trick – and he loves mimicking what he sees.

He has a smile that lights up the world.  I keep myself surrounded by pictures of his smiling face at work to remind myself that as long as I have him and his smiling face, that I have nothing to be unhappy about.

I love you, Atticus, and I am so proud of your strength.

Laser Treatment

Atticus had his second laser treatment on September 4, 2013.  His first was on May 1, 2013.  In May, his plastic surgeon and ENT lasered both his tongue and lower lip.  The tongue and lip both swelled pretty bad (because of his tracheostomy, his airway was not a problem).  This time, they only lasered his lower lip, to give his tongue a little break.  His lip swelled pretty badly at first, but has gone back down.  Each time they laser, his lip should get a little bit smaller.  They will just keep chipping away at his lip and tongue.

When Atticus first got his tracheostomy back on October 31, 2012, we thought that he would only have it for about 6 months.  The thought was that by that time, the swelling from his major tongue surgery would be gone.  However, after his first laser treatment, Atticus’ ENT sat down with me and Brian down and explained an updated timeline.  He said that it would make more sense to leave Atticus’ trach in until all of these laser treatments are over, which would probably be another 3 or 4 years.  The new goal is to get Atticus’ tongue to a more management size by the time he starts school.  To do that, he will require laser treatments on his tongue and lip every 4 months for probably the next 4 years or so.

During that same meeting, the ENT also described the results of putting a scope down Atticus’ throat to check and make sure that the lymphatic malformation was still limited to the front of tongue and lower lip.  I was so happy to hear that the results of the scope showed no growth in the malformation, that I wasn’t even too upset about the updated timeline for the tracheostomy.

Sure, it may seem upsetting that my child will now have a trach possibly until he starts school. But, there are so many worse things that we could be dealing with.  I cannot change the situation, but I am in control of how I react to the situation.  I am not upset.  Instead, I am grateful that tracheostomies exist, as they have given so many people the opportunity to live and breathe, even with airway obstructions or other medical problems.

So for now, we keep going on our journey of laser treatments and trach changes, suctioning, and organizing medical supplies.  All of these experiences have already made me a better person.  Every day I am thankful for my health, the health of my child, husband, and family.  A day does not have to be extraordinary for me to be happy and grateful.  The minute I wake up, I am grateful that I am healthy and that I get to experience another day of everything the world has to offer.

Tracheostomy and Tongue Reduction Surgery

This post will cover Atticus’ progress after leaving St. Louis Children’s Hospital NICU up until December of 2012.  We returned to our then-home, Champaign, IL, on May 18, 2012.  Doctors felt that Atticus might be in NICU for months learning how to eat, but since he proved that a big tongue was not going to get in the way of eating, they discharged him after only 11 days.  Brian’s mom, Pat, was staying with us for the month following Atticus’ arrival home.  We had a good time.  I’ll always remember our ‘movie night’ tradition.  How many Alfred Hitchcock movies did we watch in that month?  I’m not sure, but it was a lot!

On August 1, 2012, we packed up Atticus and our two cats (Mr. Sprinkles and Oliver) and drove over night to our new home of Charlotte, NC.  Atticus’ Ear Nose Throat (ENT) specialist  in St. Louis gave us the names of a few ENTs in North Carolina.  I did my research and decided on an ENT at Wake Forest Baptist Medical Center in Winston-Salem, NC.  I figured that if we didn’t ‘click’ or if this was out of the doctor’s realm of expertise, we could always try one of the other doctors on the list.  Wake Forest Hospital is about an hour and a half from Charlotte.  Back in the Midwest we were used to driving three hours from Champaign, IL to St. Louis, so and hour and a half seemed like nothing.

We met with Atticus’ Wake Forest ENT on August 13, 2012.  It was always a concern that we would get to the doctor and he would look at Atticus and say, “I have never dealt with this before.  I’m sorry, but I can’t help you.”  He didn’t though.  In fact, the ENT proceeded to tell us about another patient of his – a little boy – who had just undergone a tongue reduction surgery the prior year.  This little boy also has lymphatic malformation of the tongue.  I immediately felt better knowing that this doctor had seen this before, and was involved in other tongue reduction surgeries.

That day, we also met with a Plastic Surgeon at Wake Forest and she also seemed really confident in her ability to treat Atticus.  I liked their team approach.  The two of them (ENT and Plastic Surgery) would be Atticus’ main team of specialists.

Atticus was put on Propranolol, a medication typically used to decrease blood pressure  Propranolol has had good results in shrinking hemangiomas.  Atticus did not have a hemangioma though, but his doctors still felt it was worth trying.  It was pretty clear after a couple weeks that the Propranolol was not shrinking his tongue.  I wasn’t surprised.

At an appointment with ENT in late September of 2012, Atticus’ doctor dropped the bomb that he wanted to do the tongue reduction surgery soon, and by soon, he meant the following month.  We had still been thinking in the St. Louis model of not doing anything until after Atticus was a year old.  Not only did they want to do a tongue reduction surgery so soon but they also wanted to do a tracheostomy a week before the tongue reduction to ensure that there would be a stable airway during and after surgery, in case of major swelling.

That appointment really sticks out in my mind.  It was a lot to take in, and we just were not expecting this to move so quickly.  The idea of a tongue reduction surgery was a lot to digest.  I had never heard of anything like this before and it sounded rather barbaric.  A tongue reduction basically involves cutting a pie-shaped portion out of the middle of your tongue and sewing the sides of the tongue back together through the middle.  If you are imagining that being done on your own tongue, now imagine your tongue is about five times thicker.  Atticus’ tongue is a lot thicker than a normal tongue, so that is a lot of tissue that needs to be cut through and stitched back together.

Atticus’ doctor talked to us about the high risk of blood loss.  He explained that there is a large vascular component to Atticus’ malformation.  There last case of this was mostly lymphatic and since Atticus’ malformation is so vascular, they were worried about bleeding.

And then there was the trach.  I had thought that we passed that hurdle.  We were nearly certain that Atticus would be trached right after birth or at least before leaving the NICU.  He proved that he could breathe on his own, so a trach wasn’t necessary at that time.  The reason for the trach now was because of the possibility of swelling after surgery.  I don’t think the idea of Atticus getting a trach really bothered me until the ENT said the words, “I want you to understand that Atticus won’t be able to make sounds once he has the trach, at least at first.”  That was it.  I started to cry.  I remember looking at Atticus in the doctor’s office.  He was on my lap.  He had just recently started making sounds and now that was going to be taken away.  We wouldn’t even be able to hear him cry anymore.

My parents came to Charlotte to be with us and help out during the tracheostomy and tongue reduction surgery.  We all drove up to Wake Forest Hospital very early on the morning of October 31, 2012.  On his first Halloween, Atticus was put to sleep and his doctor inserted a trach into his neck.  While he was sedated, they also did an MRI to look at the malformation and see if it had spread at all and to see if it was still isolated to the front of the tongue and lower lip.  It took a while to perform the trach and do the MRI but finally we were able to see him.  He was in the Pediatric Intensive Care Unit (PICU) on all sorts of machines and tubes.  Seeing him like that was more intense than I was expecting.

The sedation started to wear off after a while and I remember Atticus looking up and starting to cry.  Only, no sounds were coming out.  I don’t even know how to describe it besides unsettling.  To see his face crying but not hear the cries…made me cry.  The Plastic Surgeon stopped by his PICU room that evening and she could tell I was upset.  I can’t even remember what she said but I remember her putting her arm around me and saying something that made me feel better.

Atticus was moved to Intermediate Care after a couple days and me, Bri, my mom, and dad all spent the next week hanging out with Atticus in his hospital room.  He would need to stay hospitalized for the next week while his tracheostomy healed and then the following Wednesday, November 7^th would be the big surgery – the tongue resection.

A few days following his tracheostomy, he was back to his normal self (except now breathing through a trach).  He seemed to get used to the trach very quickly.  Brian and I took the next week to learn everything we possibly could about trach care, including changing a trach which involves taking the trach completely out and inserting a new one.  That seemed really overwhelming at first but after a few times, it wasn’t so bad (now it’s so routine that we don’t even think about it).  All of the nurses in Intermediate Care quickly fell in love with this truly bouncing baby boy (we brought his Jumperoo to the hospital so he’d have something to do for the week).

Then, on November 7, 2012, Atticus underwent his tongue reduction surgery.  After trying to keep his mind off of not eating all day, they finally took him back to the operating room around 4pm.  The surgery took around two hours.  Me, my mom, my dad, and Brian were all sitting in the waiting room when the ENT and plastic surgeon came out to tell us how everything had gone.  Atticus had bled…a lot.  Atticus needed a blood transfusion, but he was doing well.  They removed as much of his tongue as they possibly could without compromising his tongue’s range of motion and other things.  We were immediately relieved to hear that everything had gone well.  The only thing that worried me was the risk of blood loss, but now that my fears were put to rest on that, I knew that everything would be OK.

It felt like forever, but we were finally able to go back to see him in PICU.  He was heavily sedated and also on paralytics so that there would be no risk of him messing with or trying to pull out the stitches from his tongue.  We were told to expect a lot of swelling and that it could take months for his tongue to go down to the size that it would now be.

Atticus spent three entire days completely sedated.  On Saturday, they started easing up on sedation and paralytics.  By Sunday, he was awake, but in a lot of pain.  I can’t even imagine the pain that he must have been feeling.  He had just had more than half of his tongue cut off and sewn back together.  The hardest part post-surgery was seeing him in so much pain.  Since he was so young (6 months exactly on the day of surgery), there was no way he could verbalize his pain, but you could just tell.

It was during that time at the hospital that I realized what a strong baby I had given birth to.  He had already gone through more in his 6 months than most.  He spent a total of three weeks in the hospital and ate through a feeding tube for about a month after surgery.  We were told that the recovery would be tough and that there would be bumps along the way.  Atticus did so well though.  The stitches all came out naturally.  Everything stayed intact, and the swelling eventually subsided.

He is a true fighter and always has a smile on his face no matter what.  Even with feeding tube, trach, arm restraints, and stitches in his tongue, he still smiles through it all.

11/23/12

12/6/12