Hearing Aids and Wheelchairs!

A few months ago Joanna and I noticed that Natalie wasn't responding very well when we interacted with her. We'd call her name or ask her a question and it would be hit or miss if we'd get a response. We weren't sure why she wasn't responding very well. Our hypothesis was that her hearing must be struggling significantly. She has a history of ear tubes and infections so we took her to an ENT to have it checked out. In the past it seemed like she couldn't hear well and we'd have her checked out. So we figured they would do the same testing and maybe say it was an infection or a need for tubes again. This time, at a new triad ENT they did a pretty intense round of tests and noticed that her middle ear wasn't responding. They can test both the inner and outer ear separately to determine how much sound is getting into the ear and how. She was hardly hearing anything through the middle ear. So we were sent over to a specialist today at Baptist in Winston and they did a CT scan to check out the condition of her ears. They concluded that she has extreme Conductive Hearing Loss in the middle ear. The middle ear couldn't function any worse. The middle ear is made up of 3 small bones called the Stapes, Incus, and Malleus. These bones move back and forth as the ear drum lets in sounds. If they can't move then very little sound gets in. They are theorizing that Natalie's Stapes bones (the smallest ones connected to the cochlea) have fused to another bone and are no longer moving and as they should be. This means very little transfer of sound from her outer ear to her cochlea (inner ear). They don't know what caused this but think it MIGHT be connected to her arthrogryposis (bone irregularities). But as usual, since it's Natalie, they can't say for sure.   

So what can be done? Hearing Aids are the solution. Sometimes surgery can fix Conductive Hearing Loss but not in her case. They aren't damaged they are fused. Apparently surgery can fix a damage but not a fusion. The aids will essentially serve as an artificial middle ear. It should greatly improve her hearing. Possibly even be the best she's ever had. She was fitted today and as soon as insurance approves the order it will be about 3 weeks until they arrive. As expected, she picked out purple sparkles as the color for the aids. They are very cool technologically and can even be connected to bluetooth and used as earbuds. So she's gonna be fancy fancy.

There were some interesting things the doctor noted. One, he couldn't tell how long she has had this hearing loss. He said that this type of hearing loss does not happen quickly. Meaning she has most likely been struggling with her hearing for a very long time. There is the possibility that she has NEVER heard completely. She has never fully passed an ear test due to what we assumed was inability to respond (facial and verbally). Sometimes the tests would be inconclusive because couldn't respond during the test. The specialist doctor told us that many regular docs are very bad at testing kids with disabilities. He said that the testing she's had before probably weren't given thoroughly. But that is speculative and there is no real way to know. The Second interesting thing he noted was, the part of the ear that is malfunctioning in Natalie is located very anatomically close to her main facial nerve. She has no function of her facial nerves so her lack of use in her ears and face could be related. This idea is purely speculative but gives us something to think about. 

So the bad news is; she has to have hearing aids and will always need them. The good news; the aids should help significantly and her middle ears won't get worse (because they're already as bad as they can get). If her hearing were to decline further it would be due to a completely different issue. So that's the silver lining I suppose.

ALSO...Natalie was recommended to receive a power wheelchair last year. She still has mobility but struggles to keep up with her peers and others. She can crawl faster than she can walk and some days refuses to walk because it's become hard for her. Her back has gone through so much and she does a lot of therapy to keep up. The chair will help her get around when she needs it and will help her move as fast (or faster) as anyone else. She won't need it all the time but she'll use it quite a bit. It will help her gain some independance and prepare her for the potential of needing a wheelchair as she grows into adulthood. Initially she was denied approval for the chair but after a second review it was approved. So some time within the next 2 weeks she'll be getting her Natalie-sized purple power wheelchair. The company came out last year and sized her. We'll be constructing a ramp on the side of our house to accomodate the weight of the chair and let her get in and out of the house. We have been researching a lift/carrier for the car but haven't exactly figured that out yet. She's excited about her chair. I can almost guarantee she will figure out how to use it inappropriately and jump it off of things, etc. She's a mad woman haha. We'll post plenty of pictures when it arrives.

So that's all for now :-)                 

Natalie

 

Hey Family and Friends,

I wanted to share an update on Natalie. Last Thursday 8/6/20 we met with Dr. Stone (her ortho surgeon) to check her weight and to see how her spine looked. Basically, after several month in the halo nothing has improved. Her weight dropped a bit and her spinal curves haven't gotten any better. 

To back up a bit i'll explain the significance of her weight drop. Natalie has always been on the negative percentile on traditional size charts. She's always been small and because of her muscle tone issues it keeps her small. She'll never be a big kid. However, in order for any further surgery to happen and any fusion hardware to be placed in her back she needs to gain enough weight so that the instrumentation can be covered. In other words, there isn't enough real estate on her back for the surgeons to cover the hardware. That's why the last fusion had to come out. It wasn't being covered and kept coming through her skin. 

So, since her last surgery we've been doing feeding therapy, night feeds via mic-key feeding tube, and giving her food that might help her gain some bulk so she could have surgery. But since she has had the halo on the second time...she's lost weight. Most of this is due to the "robbing Peter to pay Paul" effect of her night feeds keeping her from eating normally during the day. She isn't eating enough to GAIN by daily oral calorie feeds and the night feeds are simply giving her "extra" calories and keeping her from eating during the day...because she's full. She loves to eat and can eat plenty to keep herself healthy but she just can't eat enough to gain excess weight. Those of you with children, try to imagine trying to get your child to gain 5-10 pounds. It's almost impossible because of their metabolisms. Now imagine trying to get a kid like Nat to gain 5-10 pounds. 

When we met with Dr. Stone last Thursday we were faced with the conclusion that there wouldn't be another surgery for Natalie. If he were to perform surgery again he wouldn't be able to close the incisions because of her size. Essentially putting her in the "inoperable" category. The upcoming surgery (Vertebral Column Resection) that was going to happen if she gained weight already had significant dangers to it. They say with a typical scoliosis/kyphosis curvature there is an average complication rate of 69.2% with the procedure. Meaning even in the best circumstances there would be a large chance that she would have come out of the surgery paralyzed or potentially not at all. With Natalie's complications, the end result would not be good for sure. What this means is that we're officially out of surgical options. We're officially off the patient list at UNC Ortho. No more visits, xray's, etc. The last thing they'll do with her is take off her halo in the coming weeks. We're still working with the nutritionists and other doctors to work on a plan to give her some bulk but we're all on the same page knowing it probably won't happen. 

So what does this means for Natalie's long term health? It means what you imagine it means. It means she (and we) will now be living in the reality that her spinal curvature will cause her lungs and heart to struggle. How long will it take to get bad? There is literally no way to tell. She was already in the danger category before her first surgery last year. It could be years or decades or less before it gets life threatening. But we can't see into the future. We have hope that it will be a long time before any complications come but all we can do is hope and wait. 

This obviously is not the outcome we'd hoped for but "it is what it is". We're in a bit of a daze trying to process it all thus the lack of updates and communication with folks asking about her the past few weeks. She is happy through it all. Stubborn as ever and glad to be getting her halo off one last time. This update is heavy and seems hopeless but it doesn't mean we're giving up. It just means that at this point, the door to surgery has been closed. One day as medicine and surgery progresses we hope to have another option. But for now, we don't. We'll search out other future options for sure while still living in the present. So be on the lookout for updates as/if they come. 

Natalie will be starting the 3rd grade on Monday. She can't wait to get back to her school family with Mrs. Garris and Mrs. White and the friends she loves so much. We considered not sending her back due to COVID-19 and her underlying conditions but we've chosen to let her find joy in the midst of the chaos without regret. We've taught her over the years to love God and to love people and we know that as she goes back to school she'll be able to accomplish that.

Please continue to pray for her. Pray that God might be glorified through her and that people might be loved well through her experiences.   

We love you all- Lyle, Joanna, Isaiah, and Natalie.

Another Step...

As you know (or maybe don't know), Natalie has had a hard time healing from her spinal fusion surgery. Her skin and the hardware just isn't cooperating. The hardware has come through her skin twice and each time it's been exposed her doctors have removed screws and parts of the rods. Her plastic surgeon has a hard time finding enough good fat on her back to cover the incisions during each surgery. She also still hasn't regained the ability to walk independently farther than a few steps. She'll be turning 9 on May 21st and she is having to crawl like a toddler to get around the house. She does have walkers to use but they are so cumbersome for her to use that she skips the step of using them most days. Additionally, her cervical spine (or neck) strength has not improved and it is causing her to hunch over more than ever. This lack of neck strength has been causing a lot of the hardware issues as it puts unneeded pressure on the skin and hardware. So to say she is struggling is an understatement. She keeps chugging along but, as you can imagine, it's taking a toll on her physically and mentally.

Last night, as we were doing her usual wound care routine, we noticed that another part of her hardware had become exposed. We immediately texted her doctors and told them that we really need to discuss and reevaluate the long term plan. We can't keep "putting a band aid" on the situation. They responded back quickly, set up a time for another surgery on Friday 5/9, and also scheduled a telecall to discuss what the options were. After talking with her doctors they have come up with the following plan. Since the current configuration isn't working; Take all the hardware out, Reattach the infamous halo for a few months, and then evaluate and plan the next steps. Currently the next step plan consists of another procedure called a Vertebral Column Resection or "VCR". This procedure would actually remove entire vertebrae from her spinal column. They'd also move up into the cervical spine and put temporary posture-type rods to help hold her head up as it strengthens. This type of surgery is extremely complicated and carries with it many risk factors. It would involve a neurosurgical team along with her other teams to monitor and complete the procedures.

So tomorrow starts the next step of her journey. She is beyond thrilled at the idea of having her halo back. She always says that she misses it and when we told her about the surgery she actually yelled and pumped her fists and said "Yay, I get my wheelchair back!". So I guess that's one silver lining. This time there won't be much expectation of the halo straightening her spine but it'll moreso be a means of keeping her upright and off of her lungs and heart. It will alleviate much of her pain as well.

As can be expected, we're pretty hearted about the whole situation. Before we talked with Dr. Stone today we called our second opinion Doc down at Shriner's to see what he would do. He said that he would take the hardware out, put the halo back on, and reevaluate. So it was good to hear that his opinion would be exactly what Stone said. But still, it a heavy thing.

Please keep us in your prayers. Pray for rest, for healing, and for clarity.

Also, this stay will be different than the others because of COVID-19. They are only allowing 1 parent to come in to the hospital for the ENTIRE stay*. Which means the parent that comes in with her has to stay...there can't be any trading off or visits from the other parent. So pray for the time that we'll be apart. The plan is for Joanna to go to UNC with Nat since all of her work is done remotely and she can do that in the hospital room as needed. Also, there's nothing like having Momma with you when you're in the hospital. I'll stay with Isaiah to do school, to tend to everyday things, and to just be here with him.

So keep us in your prayers.

*Feel free to Facetime Nat or call her once she feels up to it. She likes to see people. Also, Joanna would never ask for it but since she won't be able to leave the room feel free to send her some Grubhub or Uber Eats (if they'll even let them deliver) because hospital food gets old 😀             

Second Opinions

We wanted to give a quick Natalie update. Last time I posted I wrote about the need for second opinions. We hoped Dr. Stone's plan was wrong and we really needed some folks to dream with us a little bit before we took any next steps for Natalie. During this time of "dreaming" we've gathered opinions and have presented her case to many other specialists. We've taken trips to Florida and to the Chief-of-Staff (Dr. Wattenbarger) at Shriner's in Columbia, South Carolina. Dr. Wattenbarger also presented her case to several other kypho-scoliosis specialists at their weekly meeting. She's been reviewed by doctors at the Texas Scottish Rite Hospital, folks at Duke University, and is currently under review with a specialist in Philadelphia that focuses on high-kyphotic curvature correction. We're also hopefully meeting with some local folks this week that are going to take a look at her from a few different holistic angles. We want to cover all the bases.

The consensus the doctors have come to so far is: Dr. Stone was right. Some of the doctors had technical differences on how they might perform the surgery, a few others had opinions that seemed a bit far off, but ultimately the opinion so far is that fusion is the most appropriate option. We're still not at peace with this outcome but we feel much better knowing that many other people that specialize in this area agree with Dr. Stone.

We've also met with Dr. Stone again at UNC for a more in-depth discussion on the specifics of the procedure. He walked us through what would specifically happen and why he thinks it's the best option. He understood our initial apprehension and was confident in his decision even though we wanted second opinions. Seeing his confidence in the procedure helps us begin to find some peace. He said no parent will ever be 100% on board with surgery but finds that most folks land around 80%. We're getting there. We are now beginning to accept that Natalie needs this fusion.

So for now, Natalie's procedure is scheduled for January 7, 2020. She'll remain in her halo until then so she'll not collapse. Unless there is a last minute hail mary from somewhere out of the blue this will be the plan. In the meantime we'll be trying to get back to living our version of normal until the surgery. Although Joanna and I are burned out we're hoping to snap out of it soon and get rolling again. We appreciate all of your kind words over this last little bit and we also appreciated everyone's comments and opinions on the upcoming procedure. Thanks for getting riled up with us :-)     

I'll give any updates as they come. We love you all.

Back to the Drawing Board

Monday 10/28/19 Natalie Update:

Two weeks ago Natalie and I went to UNC for x-rays to check the progress of her halo traction therapy. There were minor corrections in her curvature but nothing that made her doctors super excited. Since there was such little progress they decided to give it two weeks before checking again to see if more stretching would occur during that time. So we waited the two weeks and we were able to have a week at home without having to ride back to the hospital or go to football practice (which was nice). It was pretty clear that Natalie’s lengthening progress was slowing down a bit so we decided that both Joanna and I should be at this appointment this past week in case her doctors wanted to discuss further plans or adjustments. So we checked the kids out of school, hit the road to Chapel Hill, and made an afternoon out of it.

As always, we rolled into the Ambulatory building parking lot looking like the Beverly Hillbillies with our trailer full of medical equipment, did the usual rigmarole with the x-ray teachs, and waited around for a report. Quickly, Dr. Stone came in and explained that in his opinion the halo was no longer working and that we needed to proceed with another surgery on November 5^th (yes, next Tuesday). He had already decided and had it scheduled. He proposed that Natalie needs a permanent posterior thoracic/lumbar osteotomy and fusion. There is a longer official name for the procedure but essentially it is; going into the back of her spine and manipulating some of her bones around (via osteotomy), straightening things up as much as possible and permanently attaching her thoracic spine and part of her lumbar spine in place (fusion). The way her back and body functions after this surgery is the way it will be forever. After this procedure is done there would be no more operations for Natalie. Literally the last one for her back at least. As we supposed, Dr. Stone couldn’t give us a clear idea of how much correction could be made during this procedure but rather he gave us an honest but disheartening answer of “it won’t be worse”. He went over why he thought this was the best next step and he told us that there were “no other options” that would be appropriate. He rightly communicated that without a solution she would eventually “expire” from cardio-pulmonary dysfunction over time. He said growth rods or any other distraction-based options wouldn’t be a good idea because she was too small and that they wouldn’t ultimately work due to her Arthrogrypotic posturing (meaning her bone/joint stiffness, curvature, and positioning...we can discuss this another day). He said that there were other techniques out there like tethering (VBT) but the colleagues he has advising him on Natalie would not recommend any of these techniques at this time.

So, needless to say, we were devastated. Once again, we have another situation where her team is saying, “we’re out of options”. This procedure would be significantly impactful to Natalie’s overall growth and well-being for the rest of her life. Because of the thoracic fusion a portion of her torso would inevitably be stunted. The overall impact of this stunting is yet to be seen. She could possibly keep her current trunk rotation since most torso rotation comes from the cervical and lumbar region of the spine but that rotation could also be stunted. Also, most likely she would still have a significant curvature of scoliosis and kyphosis and her mobility would be limited similar to what it already was pre-halo. I asked hypothetically if technology advances would this surgery have the potential of reversal if a newer procedure were invented? The answer was “no”. So it’s truly permanent. Dr. Stone was 100% behind us on looking for as many second opinions as we needed which gives us confidence in him all the more. I think that’s what all doctors should recommend. At first we agreed with him that this next procedure was inevitable and since we weren’t medical experts we would take his opinion and get the surgery on the books. So we signed the waivers, got the surgery soap, and we got it on the schedule. But we didn’t feel right about it. We talked all the way home and then just sat in shock, silence, and exhaustion for a few days. We just felt that we weren’t ready to give up hope just yet.

At the end of the day Joanna and I are both realists when it comes to life and Natalie. But, we both have a pretty large dose of idealism in our DNA. We like to dream. We know this permanent surgery will more than likely be the only option for Natalie and we can live with that BUT, what we can’t live with is not seeking out people to dream with us for a while. We need to make sure there isn’t something else out there. So we’re starting over for a season. We’re seeking out doctors to look at Natalie holistically and make recommendations based on several aspects of her livelihood and not just survival. We want people to take into account the impact of a preadolescent fusion and how a permanent solution on an 8 year old might be too hasty. We know that the option given by UNC will Lord willingly keep her alive BUT we hope for more than mere survival. We are hoping to find a doctor that can help allow her to grow as much as she can and to physically become the young woman that she was always meant to be. We want her to look back on her childhood and know that we fought well for her. If her mobility is limited then so be it. If she can’t run like other kids then we accept that reality. We want Our Natalie to be exactly who she is meant to be. As we decide on her next step I look back on all the times doctors have told us that there were “no other options”. Most of those times, they were wrong. Before birth we were told that the only options for Natalie after birth were pretty dire…they were wrong. While in the NICU they said she’d only be able to eat from a feeding tube…they were wrong. They told us that she might not walk…they were wrong. They told us a growth rod was the only option for her back…they were wrong. They told us she’d only thrive in a separated classroom for intellectually disabled children…they were wrong. Just last year we were told that Natalie would never get a High School Diploma and I can’t wait for her to prove them wrong. Dr. Stone is probably right about this final surgery but I have to hold onto a glimmer of hope that he might be wrong. Once we explore as much as we can then we’ll be reasonable and do what needs to be done. But until then, we’re gonna keep dreaming.

So we’re currently scheduling visits this week. We talked with the Paley Institute in West Palm Beach, Florida today and they specialize in cases like Natalie. The Paley Institute is not covered by her insurance but they will allow us to self pay for the initial visit to see if other options are out there. We have no idea what timeframe as to when they’ll be able to see her but we hope it will be sooner than later. We’ve also scheduled an appointment with the Shriners' Hospitals who work with experienced ortho specialists that can weigh in. We met an amazingly kind man here in town while eating at “Harry’s Place” that got the ball rolling for us with the Elkin Shriners. We’re meeting with him tomorrow afternoon to talk about visit options. After that, we’ll regroup and see what else is available. We know this next little bit will be exhausting and to be quite honest we’re already exhausted but, I think in the end it’ll be worth it. So please keep us in your thoughts and prayers over the next little bit. We’re heading into uncharted territory so any reasonable tips or guidance in these areas will be so greatly appreciated. We’ll get there eventually.

Thanks again for all your kindness through this process.

Home for now! (Phase 2)

Welp, we made it home after a month at UNC. It's good to be back in Elkin. Like I mentioned on Facebook it feels like "Phase 1" is complete. Her old rod is gone, her Halo is attached, she is starting to straighten up, and we're home. Now we get to figure out "Phase 2".

Natalie was sent home with her Halo along with a wheelchair and a walker. She was also given an adjustable tilt hospital bed with a custom traction attachment made by our new friend Chris Bryant. Most kids that have the Halo get straightened up before they leave the hospital and then have a follow up surgery at the end of their hospital stay. Natalie's spinal curves are so extreme her doctor's thought it would be best if we go home while her spine continues to straighten. They initially thought that she would straighten in about 6 weeks and then they'd develop a plan from there. However, the doctor thinks she will have several months of straightening before a new surgical plan can be made. There really is no way to forecast how long her back will take to straighten but for now we're going with the generic "months". It may be sooner but it is unlikely. We also don't have many indicators of what type of surgery she will have at the end of her traction. There could be growth rods, fusions, or something that we've never heard of but we have zero inclination as to which procedure will best suit Natalie. Once we get to that point we'll be sure to share the plan. We're used to waiting with Natalie and this won't be any different. Until then we'll keep on doing "Phase 2".

So what is "Phase 2"? It's simply waiting and trying to give both of our kiddos as much normalcy as possible.

We've figured out that Natalie's wheelchair can be transported in the back of our Subaru. It's not pretty but it gets the job done. We'll be able to take her to school, to Isaiah's football games, and pretty much anywhere else in town. For now, it works and that helps a lot.

Natalie will be starting half days at school on Monday. She'll do half days until she builds up some endurance and shows that she can physically handle a full day. Principal Colbert and her teachers (Mrs. White, Mrs. Garris, Mrs. Simmons, and others) have been unbelievably gracious to Natalie through this whole process. Mrs. Garris is out on maternity leave but Natalie is so excited for her and her new sweet baby girl. Joanna and I went by Elkin Elementary yesterday to show all of her teachers how to use all of her equipment and they just jumped right in learning. We are completely confident that they will carefully transition her as needed and will make sure she is safe at school. It's refreshing to know that Elkin Elementary has amazing educators that are also loving Moms and Dads that care so much for their students. Natalie is very excited to get back to school and so are we.

Also in the next week or so we'll start visiting church again. FBC Elkin, Elkin Presbyterian, and the Methodist Church here in town have shown us so much love over the last few weeks. They have showered Natalie with encouragement, cards, notes, and treats that have made the hospital so much easier for all of us. People can learn so many lessons from these folks on how to care for their neighbors. We were practically strangers to them yet they chose to love on us and treat us as their own. We can't wait to see everyone again from all of these congregations and figure out how we can plug in and serve along side you.

We'll also (hopefully) start building the treehouse that we've been promising Isaiah. I think the weekends will be nice for getting out in the yard and working on it as a family. He already has his design ready.

Joanna will continue to work from home and i'll be starting back as a substitute teacher in Wilkes County. Thankfully this gives us flexibility during "Phase 2".

We will head back to Chapel Hill every week to two to get x-rays for Natalie and to meet with other departments within her team. We don't expect many significant changes for quite a while so I probably won't be updating the blog unless something important comes up. But we will be posting progress pictures and sending updates via Facebook/Instagram.

I've attached a picture of Natalie's most updated x-ray. On the right is her pre-op x-ray and the one on the left is Monday 9/23. Initially she had a 148 degree curve and now it is at 101. You can see some major changes in her back, chest, and neck. That's certainly progress!

Hope to see everyone soon!

We love you,

Lyle, Joanna, Isaiah, and Natalie

Friday Update: 9/20/2019

I have a few things to update but nothing major. Basically we're at a standstill with coming home. The team is ready for us to go home but it takes a long time to get details and tests and paperwork together. She is doing great and ready to come home!

Thursday night Natalie had a sleep study done to monitor her oxygenation levels. They hooked up brain monitors, oxygen, and all kinds of other wires to see how she is tolerating the 20 pound traction weights. If she gets positive results from the sleep study then pulmonary can release her to Ortho. We're waiting to hear official results but the preliminary comments so far have been positive.

Also Natalie has been given an NG (Nasogastric) feeding tube. The GI team and the dietician have suggested small night feeds through the tube to give her extra calories. She is currently eating about 90% of the calories she needs to gain weight. They need her at about 110% so the NG tube, hypothetically, will help get her to 110%. The tube is a temporary solution and should come out sometime before her next surgery (whenever, and whatever that will be...hopefully in the next several months). Joanna and I will change it out from home about every 6 weeks or so. So if you see her tube don't be scared of it, it's just a tube. It doesn't hurt her and she isn't being fed through it if you're talking with her. She only uses it while she is sleeping. At most it's just a bit uncomfortable and cumbersome for her. Once GI and Diet feels that her caloric intake is good then they can release her to Ortho.

Once all of the teams have released her then the Ortho team (who is the head department in the procedure) will officially release her to come home. After the release/discharge we'll go back to UNC every week or so for x-rays, weigh ins, neuro checks, and any other tests needed. There is a small chance that all of this communication will happen this weekend but realistically after about 4:00 pm on a Friday the hospital shuts down progress until Monday morning. But we're not in a rush, we had already planned to be at the Hospital for longer so if they discharge her early it's just icing on the cake. We want things to happen when they need to and when her team thinks it's appropriate.

On a practical note, Nat's bed should be delivered to the house today from Bridge Street Pharmacy and a preliminary set up for her traction bar is being machined this weekend here in Elkin. She also has a ramp built to get in and out of the house. So when she does get discharged we'll have her room ready at the house.

Thanks again for all the amazing love you all have shown our family. We love you all.  I'll send more updates as they come.