Tuesday, April 25, 2017

Houston, we have Zinging!

It is with a happy heart that I'm sharing this post...Josie felt her first real "zing" yesterday in school.  For about 2-3 weeks we have been trying to decipher whether Josie's self described "tickles" were actually "zings". Her pain tolerance is so high we questioned what her definition of a "zing" actually is. We've been emailing back and forth with her doctors trying to decide. I had even emailed her doctors this video of myself tapping along her facial nerve to try to help us dissect what was happening.

Well yesterday, cool little Josephine gave us our definitive answer...
She came home from school; she played in the yard, helped me plant garden and then, nonchalantly while she was helping me with dinner told me about her day....

"Mom, today in school I was reading at my desk and I reached up to scratch my ear (points to her left ear) and it hurt (points to her right cheek). Like I kinda wanted to yell "ouch", but I didn't. It was a weird feeling. Do you think that's normal?"

attempting to keep cool but stopping what I'm working on says, "Well, wait, what do you mean? What hurt? Where did it hurt?"

continuing to prep the cauliflower I'd given her to work on for dinner, casually repeats her story

pausing to soak in her words and then gradually smiling as I respond...."Yes, I'm pretty sure that's normal. I think that's your zing."

big smile, flatly responds "Well good, I can finally have my surgery." Then we went to tell dad.

And just like that, we're off. I confirmed her "zing" with her doctor this morning and we are officially in their books for surgery on August 16th (which is later than we expected but were assured is an acceptable time-frame). I was told to expect a 9-12 hour surgery, 3-5 days in the hospital, and then another 3-4 weeks recovery time (part of that being at home). I'm feeling so much relief, joy, gratitude and at the same time kind of completely sick to my stomach. The joys of parenthood!
This graphic pretty much sums up her next surgery.

Monday, March 13, 2017

The Countdown...

Our Little Lady Liberty this past Halloween
I am never quite sure where to begin when updating Josie's blog after we've been on a hiatus. It sort of feels like trying to catch up with an old friend that you haven't chatted with in a long time; its just hard to know where to begin.

Where We Left Off...
My last update was post-op last August. Josie had just finished the first step in the facial reanimation procedure, which was grafting a nerve from her ankle and placing it strategically into her cheek. When we were sent home it was with the instruction to wait for her nerve to start "zinging". This "zinging" would indicate that it was time to begin the next phase of reanimation. We were told the window for this happening was anywhere from 4-6 months all the way up to a year after surgery (so anywhere between mid-December all the way up to August 2017).

Since last August's surgery, Josie has bounced back without missing a beat. She has been enjoying the 3rd grade, she has continued to be active on her swim team and has been a reluctant cheerleader for her brother's basketball team. As you can tell by her Halloween costume this past fall, New York is never far from her heart. She has a love for Lady Liberty (and all things New York).

Since August Dave and I have done a pretty good job of continuing on with our every day life and not thinking too much about the if's and the when that the "zinging" will happen. Lately though, I can feel the dark shadow of nerves and the uncertainty creeping up on us. It happens almost unconsciously. At any time within the next six months Josie's nerve will begin firing signals and we will have a small window of time to return to New York for surgery (about 4-6 weeks). It all feels very daunting and at the same time so very exciting.

Facial Reanimation Part 2...
The second phase will consist of her doctors grafting an 8-inch length of her inner-thigh muscle and placing it into her affected cheek. This grafted muscle will be connected to her newly functioning grafted nerve and will help her to gain back her facial movements; such as smiling, puckering, and expressions as well as giving her more facial symmetry. The way I understand it, there will also be a nerve in the grafted muscle that will be "plugged in" to her existing facial nerve (which will be near her left ear). This will serve as an insurance plan of sorts, helping her gain even more facial mobility. This next surgery will not be as easy of a recovery for Josie as was the first. She will have a sizable incision in her leg. Her doctors also hope to shave/shape her cheek bone as well as remove excess tissue inside her cheek and on her tongue. I am so thankful that she has come to this point, but in the same breath I'm also dreading this for her.

Moving forward...
Call it mother's intuition or God whispering in my ear, but I feel like things are probably going to happen sooner than later for Josie. She has told me that her face feels funny sometimes, sort of like a hand going to sleep. She tells me her face feels numb and tingly at times. It isn't all of the time, but I'm guessing this is the start. Also, so many people have been inquiring about her, praying for her, and offering to help in anyway they can. It's like all of you know that it's near, too.

I have to share this final story with you before I sign off, it's just one more sign that Josie's journey has not been in vain. Last week I connected with a mother of a child who has a diagnosis similar to Josie. She had sent an email through the Children's Craniofacial Association's list serv. This list serv is a way for parents to connect with other parents who have children with like diagnosis'. I have been a part of this group since Josie was 4 years old. I usually ALWAYS delete the emails before I even open them because they are typically from parents of kids with diagnosis' very different from ours, but this time the subject line caught my eye, "Infiltrating Lipomatosis". Now believe me, you do not see that term everyday. In fact, Infiltrating Lipomatosis is so uncommon that spell check thinks I'm spelling it wrong and once upon a time if you googled it, Josie's blog was one of the few things that popped up. Trust me, it's just not common.

Anyway, I immediately emailed the mom back. I learned that she is a mother to a sweet faced 9-month old baby girl in Norway (who in so many ways looks a lot like Josie at that age). She is just beginning her journey and I vividly remember everything about those early days. I remember feeling so completely alone in our diagnosis and being so frustrated with doctors and all the unanswered questions. Now here we are 9 years later, able to share our experiences and maybe even be a comfort to someone else.

After emailing with this mom she started a Facebook group and our small group quickly grew to 6 mother's across the globe with children that have this common diagnosis. We have been sharing and learning so much from each other these past 5 days. A mother of a sweet girl from Denmark shared with me that she has been reading Josie's blog for some time now, which felt like another full circle moment. Over the years this blog has evolved from a way to keep family updated after surgeries, to friends of friends that started following Josie's journey. Never did I grasp that it could reach so many. I have struggled at times with the spotlight that the blog puts Josie and our family in. By nature, I'm a private person and much prefer to be a wallflower so this has never fully been a comfortable place for me. But, in retrospect I'm so thankful that Dave started this blog all those years ago. It has been an outlet, helped us to connect to others that we may have never had the opportunity to otherwise, and in a way given our struggles purpose. I've always felt that there is a grander plan beyond that we were dealt a rough hand. The grand plan is still unraveling, but I find comfort in knowing there is one.

Thursday, August 11, 2016

Post-Op Update

Waiting is Hard (about 3 hours into surgery)

While everything that happened yesterday was extremely complicated and over my head, I'm going to do my best to try to explain the details of it. Ask me questions if this is clear as mud...

The surgery itself consisted of the nerve graft and bone shaping on the inside corner of Josie's eye. 
Small incision below her eye for bone shaping

The nerve was harvested by using a scope and going in through a small incision made behind her right ankle bone. The harvested nerve ran the length of her lower leg and extended to the outside of her foot (this nerve is a sensory nerve, so Josie will loose some feeling on the outside of her foot but will not loose any motor function). Once harvested, the nerve was connected or "plugged in" to her facial nerve on the right side of her face or her "good side". To do this Dr. O made an incision that ran parallel to Josie's right ear.  After connecting the grafted nerve, Dr. O tunneled it across Josie's "good side" and over her upper lip using the incision by her ear as well as two small incisions made inside her mouth and a final one made next to her left ear.
So to put this more simply, imagine the grafted nerve as an extension cord that has been plugged into a working outlet (the outlet being her facial nerve on her good side) and then extended over to the area it is needed (the left side of her face). Over the span of the next 4-12 months the grafted nerve will gradually gain power and ultimately come back to life. When her nerve starts to come back to life she will experience a zinging sensation on her left cheek when the right cheek is touched. Once that zinging sensation starts the second step in the reanimation process will need to happen within 1-2 mos. 
Her leg is wrapped to help with swelling

The bone shaping was the simple part of yesterday's surgery. Because of the tumor, Josie has excess bone growth on her cheek bone. Her doctors shaved the bone down on the inner corner below her eye through a small incision. 

There were several other issues that her doctors had hoped to address during yesterday's surgery but because of the length of time spent under anesthesia and the many incisions that she had already they decided against further complicating her recovery. This may result in another small surgery yet this year, we'll know more after we follow-up with her doctors next week.

While the crazy guy was trying to climb Trump tower with suction cups last night, Josie was waking up in recovery. After 7 hours of sedation she did have more tummy issues than normal (for her) that lasted into the the early hours of the morning. This led to her being slow to eat, drink, walk and feel better today but ultimately she did and was released from the hospital shortly after 6 this evening. Her swelling is minimal and she has bruising that is just starting to show but overall I've been surprised with the ease of her recovery so far. We head back to the hospital to have a drain removed in the morning and then we end our stay next week with a follow-up appointment with her doctors. We are going to have to start facing next steps sooner than our comfort level allows, but for now we're enjoying the calm and relief of having another big surgery behind us.
Blogging and Sleeping

Wednesday, August 10, 2016


Today. Today I sit in this all too familiar waiting room of Lenox Hill hospital. I'm sitting here trying to process emotions, thoughts, and fully encompass all of the ups, downs and in-between that have brought us to this monumental moment in our lives; today. I'm flooded with memories of the early days of surgeries and carrying my sweet 4-year old in for her first big surgery with Dr. Waner. I'm remembering the battles fought with insurance companies; the disappointments; the tears; the excitement of seeing Josie's tumor slowly fading away, surgery by surgery. Today is the precious day that makes all the tears and pain over the past 9 years worth it. Today Josie's doctors have made the impossible possible; they say she is "disease free". Today her doctors have begun the process of bringing back her smile. Today is not easy, but my heart is so grateful and full. Today is a good day.

Surgery began around 9:30 this morning (eastern time) and is expected to go all day. We've heard at the very least to expect surgery to last 5 hours, but I heard another doctor say to expect 6 p.m. tonight. While the doctors have a laundry list to accomplish today we find comfort in knowing that Josie is in the best hands humanly possible; one of God's many gifts in our lives. I will do my best to post an update after surgery, in the meantime we have the best distractions with us today.

Wednesday, April 20, 2016

A lot to Smile About

We had a great visit to New York this past weekend. It was a dual purpose trip for me as I was working a good portion of it with my sister, Holly at NY's bridal week. Holly has been modeling at markets for a wholesale bridal and prom company called Allure Bridals since she was 19. When Brooks and Josie were almost a year old Holly talked me into going on my first market trip and the rest is history. We look forward to these trips as much for the sister bonding as getting to see our second family with Allure.
Dave and Josie arrived in the city on Saturday and spent much of the weekend enjoying the sights before our appointments at the start of the week. Josie was able to stop by the bridal market and see what mom does when she says she's "working a market". I was tickled to get to show her off and for her to see her Aunt Holly in all the pretty dresses. It was definitely a memorable trip.

Dr. O & Dr. Waner
We spent a good portion of Tuesday morning in the offices of Dr. O & Dr. Waner. It always feels a little like coming home for me when we walk into their offices. Several of their staff have remained the same since we first met in November of 2011, even after they switched hospitals. I always have the strong urge to greet all of them with a big hug, but force myself to keep my arms at my sides.

Both doctors agreed that Josie's tumor showed little to no growth and that her asymmetries were due "simply" to differences in bone growth. Now I know shaving & shaping bone sounds awful and complicated, but it is a small victory to us that her tumor has stayed at bay for the past 2 years. Any bone work that Josie has had done in the past has healed relatively quick and given her little to no discomfort. We were enthusiastically given the green light to begin the reanimation process starting this summer (shaping the bone beneath her eye will also happen during this surgery).

Dr. O spent a good amount of time walking us through slides and video of the reanimation procedures as well as before and after photos and videos of patients. Their results were so exciting and reassuring to us, as were Dr. Waner's words..."there is a nearly 100% success rate". The following video is of a boy who had the reanimation procedures. While he has a different reason for facial paralysis, his results make my heart happy. The video also gives a good idea of what will happen clinically during Josie's procedures.

The Super Dr. Super

Dr. O and Dr. Waner were insistent that we meet a Maxillofacial surgeon that they have worked very closely with. I have to admit I had my reservations about meeting Dr. Super. I worried about opinions not matching up with our University of Iowa doctor and being forced to decide which was right. I worry way too much. 

While the meeting very nearly didn't happen due to a last minute emergency surgery & our overlapping appointments, the super Dr. Super made it happen for us. From our first hand-shake and exchange of greetings we were drawn in to Dr. Super. The picture used from his website does not do him justice, he was so kind-hearted and had this Santa-type smile. 

We went into our appointment with him knowing that he was out of network for us and that we'd have to cover the cost of the office visit. During our visit with him he said he wanted to take a baseline CT scan while we were there as well as photos. Knowing how much scans would be out of pocket I asked if we could wait to have the scans done at home with someone in network. Without hesitation he said "how about this...no charge." I know other doctors have done this, but for the most part this doesn't happen for us. We both thanked him profusely & his response was "see, we New Yorkers aren't all bad." He took his time, looked at her scans with us & agreed with our U of Iowa doctor in that her jaw bone is symmetrical and that he saw no problems or urgent needs other than preventative orthodontia. He is going to work with our Iowa pediatric dentist to set up a treatment plan. 

I looked at Dave after leaving his office and said all of that was well worth the $250 office fee. What peace of mind; that is just completely priceless to me. The only down side to the whole visit with Dr. Super was that we really, really liked him and want to continue under his care. He has treated other patients with Josie's diagnosis & he has a phenomenal track record (if you look him up). We're hopeful that our insurance works with us.

Speaking of looking him up, after our visit I was reading more about Dr. Super and found that he isn't just any old Maxillofacial surgeon. He is THE Chief of Oral & Maxillofacial Surgery at Lenox Hill as well as the Director for the Center of Dentofacial Deformities and Corrective Jaw Surgery. 

My take away lesson from this trip: it takes a village of specialists when it comes to treating complicated cases. I need to stop fearing what we'll find out next & just be thankful that God keeps opening all the right doors for us.   

Thursday, February 11, 2016

I am sorely overdue for sharing updates regarding Josie, but the truth is, since my last post (almost a year ago) there really hasn't been much to share. There has been a lot of boring footwork since then but I will spare you the details of all of that. The quick update is this...we did end up seeing a Maxillofacial surgeon in Iowa City last spring (at Dr. Waner's request) and his opinion was that Josie did not need to have Orthognathic surgery (reconstruction of the jaw) at this time. His recommendation was to monitor her on a yearly basis and if need be, have the surgery when she's 16. While we were very relieved to hear this, Josie's doctors in NY had warned us to expect this answer from most surgeons.

Fast forward 11 months and today you find us planning an April trip to New York. Josie's doctors would like to see her for a consultation before beginning the facial reanimation process, we are also going to be meeting with one of their recommended Maxillofacial surgeons while we are there. At this point details are pretty vague beyond that. We are very anxious to get our face time with her team of doctors and to have our numerous questions answered.

This whole process is very consuming and triggers a lot of emotions and anxiety (for our whole family). We have hesitated to talk about this trip, even with our extended family just to spare Josie's nerves. She has a lot of anxiety about surgeries and hates for us to talk about it in front of her. We're hoping this can be our quiet way to share updates without causing her added anxiety. Thank you to all of you who continue to ask about Josie and lend your support! It's hard to believe that this whole process began just 4 short years ago.

Monday, March 2, 2015

Next Steps . . . Ortho-what???

It's been nearly 3 months since I last posted. In that time Josie has quickly bounced back to her happy self, we survived celebrated the flu holiday season, and the scars of surgery #6 have already begun to fade. Watching Josie heal is truly a beautiful thing.

Next Steps . . . Ortho-what???
We were sent home from New York with the good (and scary) news that Josie gets to have her next surgery at home. We were given instructions to find a good maxillofacial surgeon that can perform an orthognathic surgery (Josie's upper and lower jaw-bone is over-grown due to her tumor so this surgery will correct that). I think it is so stressful shopping for a great surgeon and I shared my concerns with Dr. O before we left NY. She assured me that they would give the final blessing in our selection and that has given us a little more confidence in this whole doctor shopping process.

We decided to start our search by talking with our pediatric dentist and from his suggestions we now have two doctors that we will be meeting with in the coming weeks. Our first appointment will be at the University of Iowa next week and then the following week we will meet with a doctor in Des Moines. Once we have selected a surgeon, Josie will most likely have surgery this summer.

Josie will have to undergo another MRI at some point this summer, they will be looking for any sign of re-growth in the tumor. If there isn't any significant growth, and once the orthognathic surgery is behind us, Josie could technically begin the reanimation procedures at anytime. I find myself constantly trying to look ahead and guess what will be best for Josie (that's the planner in me) and all the while I know what experience has taught us . . . we just need to cross one bridge at a time. Always easier said than done. So that is where we are right now and that is where I'll leave you.
Just noticed poor Millie in the background . . . she had the flu :(