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.

Team Meeting Update

9/12/19 Natalie Update:

Today Joanna and I met with Natalie’s doctors and hospital administration reps, etc. Each person at the meeting represented a team or department that is monitoring Natalie’s progress. These teams are developing a path for Natalie to go home. Some of the groups are; Orthopedics, Gastrointestinal, Pulmonary, Nutrition, Ortho Engineering, Nurse Management, Floor Coordinator, nursing students, and a few other people that just happened to wander in the conference room.

As you can imagine, getting Natalie home is a complicated course to navigate. All departments need to monitor her and give her the green light. So far, all of the departments have given her the go ahead with a few caveats before discharge. A few departments are going to monitor her over the next few days to make sure she’s ready.

For example, pulmonary wants to check her oxygen saturation levels while she sleeps since she is up to her 20 pound traction weight. They’ll bring her back for a full-on sleep study in October but don’t feel it’s necessary yet. The oxygen tests will ensure that her lungs aren’t being stressed due to the extra weights. No major tests but just a simple fingertip monitor at night.              

The GI team wants to calorie track and watch her weight specifically to see how much poundage she can pack on between now and the end of her traction. The more weight she gains the better chances of successful placement of growth rods IF Dr. Stone thinks rods are a good option after traction. If she isn’t gaining enough there is a chance of a temporary feeding tube or a type of medicine that will increase her appetite. This would be used to fatten her up. We’ll just see how much food she will eat. It’s hard to keep weight on her because she exerts so much energy throughout the day just doing everyday activities. She burns it all off. So now she gets to eat anything and everything as much as her little heart desires.

The Ortho Engineering component has a bed lined up for her at home BUT this bed does not currently have an attachment for her traction assembly. The only bed currently set up for this type of traction assembly is at the hospital, so they say. The engineering team has developed an add-on for the at-home bed but the parts won’t come in for the add-on for several weeks or possibly months due to insurance stipulations, etc. Instead of waiting for the add-on they have approved for Joanna and I to design and build an add-on for her bed at home. It’ll be a simple solution. Whenever insurance and bureaucracy get involved with innovation it never ends well. So we’re just going to get some simple parts and do it ourselves. They also made some modifications to her wheelchair and walker and those should be done by the end of the week.

What all this means is that we’re still in a bit of a “limbo” waiting to see how she performs in the testing from each individual team. Hypothetically, as soon as her bed is delivered, we can equip it with the traction add-on and we can start bringing her home (assuming the teams are happy with the testing).

So please keep praying for her teams and the desired outcomes, and for her to keep a good attitude while she’s here. She is busy with Hospital School, great activities, and videos but she is also ready to get home to be with her big brother and her dog. Joanna and I have been rotating every 3 or 4 days and it’s been going well so far. We’ll continue that strategy for as long as it makes sense.

As we have updates we’ll keep shooting them your way. The picture on this post is of Natalie and the “heads” of her teams here at UNC. They’re all very good to her and are working hard to give her everything she needs.

Thanks for everything, we love you all!

The Good News and the Bad News (That's not actually so bad)

Hey Everyone!

I wanted to give an update on Natalie so far this week and give a few specific areas where your thoughts and prayers can be focused.

So far Natalie has been doing an amazing job here at the Hospital. Her Doctors, Nurses, Specialists, and other kids on the floor are having a good time getting to know her. Her pain levels are low and she's feeling comfortable for the first time in years. We're very thankful for how her back in particular is feeling and how low of an impact the pins (screws) from the halo are having on her. She is up to 15 pounds of weight and will be at the full 20 pounds by Monday morning. We've joked this week about how well she is literally "hanging in there". She is eating like the mini-queen she is and being pampered with all kinds of treats. I think if it were up to Natalie she'd just move in permanently. Which brings us to the specific areas of concern. I'll start with the "Bad (but not so bad) News".

The Bad: So far, even though her back is feeling great the traction therapy has done very little actual correction to her spine. Her neck is very straight now, which is wonderful, but her spine is still very contorted. What this means is that Dr. Stone has already said that she will more than likely be in the halo on the longer end of the spectrum. He said yesterday that the 3 month period will realistically be where we'll be able to tell how much it's working. So she'll wear the halo, be in her wheelchair, and use her walker for a while longer while she is stretched out. So that is the not so good news. There is a small correction so far...but it very insignificant.

The Good News: Since Natalie is doing so well with her pain management and is adjusting so well with her equipment then Dr. Stone is going to try to send her home as soon as is reasonably possible. He's even shooting for sometime next week! Rather than have her wait for the traction to work he has asked us if we'd be willing to take her home and see how she responds over the next several weeks and months. He'd have us come back every Friday for x-rays and other testing but other than that we'd be home in Elkin where we all ultimately want to be. Dr. Stone has never sent a Halo traction patient home before but since Joanna and I are the first ones at Chapel Hill to learn the system then he thinks we'll be able to take care of her at home just as well as she would be cared for in the hospital. So we'd once again be the guinea pigs (which we are used to). So be on the look out for coming home updates. I'll send word as soon as she's been sprung!

So that's the news, good and bad. With this comes a great deal of uncertainty and a significant amount of logistical maneuvering. Here are the areas that you can pray for in the upcoming days:

1. That Natalie will remain feeling great and will continue her therapy so Dr. Stone will remain confident that she is fit to come home.

2. That all of her pulmonary markers will be consistent with her progress so far. By that I mean that she is actually feeling and breathing as well as she seems from an objective standpoint. She'll have labs drawn to study oxygenation, etc. Pray that she hits her goals there.

3. That all of her equipment will be cleared to come home. Her walker and her wheelchair have already been assigned to come with us upon checkout but her bed has yet to be approved. The docs are fighting with the admin team on this one. I'm sure a resolution will be met but it will significantly effect our time-frame if her bed is not approved.

4. That we'll figure out how to transport all of her equipment. We have a plan to get everything home and set up BUT we never thought about what it looks like to actual take her places. Her chair and walker have a large crane-like system attached and it will be challenging to figure out how to get her to school, church, shopping etc. I'm certain we can figure it out but it may take a few of us "redneck engineers" to set up a system haha.

5. That once home, she'll be able to maintain her physical therapy regimen. It's easy at the hospital to go for long walks but it will be another story once we're home. So pray that we'll be able to figure out how all the pieces will fit together.

6. That Joanna and I will find peace about bringing her home. On one hand we really want to be home as a complete family. On the other hand there is quite a peace about being in the hospital and having someone watch over her with us. We think it's the best to bring her home but we would love to feel better about it. Consistently being the guinea pig sometimes takes a toll.

So that's all for now! Thank you once again for your love and care for Natalie and for us. We can't wait to see how well she does over the next few weeks and can't wait to see everyone in Elkin ASAP!

We love you all.

P.S. I put one of her old x-rays before the rod was taken out and one of after. You can start to see a difference if you squirt real hard :-)

Thursday 8/29 Natalie Update

Hey Folks,

Just wanted to give a update on Natalie! She has been a super busy little gal so far this week. She came through surgery like a champ and has hit the ground running. Dr. Stone did an amazing job with her procedure. He and his team were able to get it all done in 3 and a half hours versus the 6 hours that was anticipated. He got the growth rod and all of the hardware out safely and without a hitch. He was also able to place her halo securely. As expected, Natalie woke up angry but she was able to calm down when Joanna and I were allowed to go back to post-op. As soon as her procedure was done the doctors hooked her up to 5 lb. weights and gravity started to do it's job. Once we got up to the room the anesthesia started to wear off and she slept comfortably in her bed.

Tuesday morning she woke up and got to work. Her team came by one by one and started setting goals. She has an Ortho Team, a Physical Therapist, an Occupational Therapist, A Hospital School Teacher, 2 nurses per shift, and many other great folks and departments that check in on her throughout the day. Since she is the first kid at UNC to have this procedure Joanna and I are the "guinea pig parents". We get to teach the nursing staff how all of her equipment practically operates and we also get the opportunity to give input on how we can all make the process and equipment better for the next round of kids. The nursing staff and doctors have been given instructions on the concept of everything but none of them had ever seen the equipment or know how it works in person. It's kind of fun telling doctors and nurses how to do things haha. It's also a lot to take in and we are learning new things every day.

As far as Natalie goes, she is up and moving around all day every day. Her first day out of bed she stayed in her wheelchair (which his it's own weight system attached) for 5 hours. She also walked the short end of the hallway and back. The second day she walked around the entire floor several times with little to no hesitation and she stayed in her chair for several hours. Dr. Stone even came by her room and told her that she is able to go anywhere on the hospital campus while she is staying here whenever she wants to in 1 hour increments. She can go outside, we can wheel her around the hospital or she can walk as much as she is able. This is a huge deal for her because it helps give her a sense of normalcy and will allow her to not get in the "hospital funk" as quickly. Today she even went to a science class with a few of the other kids where she made a jellyfish puppet and got to learn to identify a lot of sea animals. Our biggest problem now is getting her to slow down and take breaks. She's a fireball of a patient. She has no quit and we love that about her.

From here on out, every other day Dr. Stone will be adding 2 pounds of weight to her halo system until they reach 20 pounds. Then they'll stop at 20 and let the weights start stretching her back out. As you can imagine adding 20 pounds to a girl that is only 33 pounds will be pretty extreme. Her bed, her wheelchair, and her walker have a weight system that essentially pulls her head upward whenever she is attached (which is all the time). We've entered the phase where Natalie will become frustrated. As the weights get heavier it will be harder for her to maneuver freely and the more constricted she'll become. So pray that she'll have the patience and stamina to stay positive and to fully understand that she has to keep these weights on indefinitely.

So that's been our week so far. We'll be here chasing Natalie around the hospital until the next step begins. We still don't know how long the process will take but at least it has started. We're so thankful for everyone here at the hospital and for everyone back in Elkin, Raleigh, Wilmington, Florida, and all over the country and world. Your thoughts and your prayers have truly been felt. All I can continually say is that your love for her has been overwhelming in the best sort of way. I'll send out plenty of pictures and updates as they come via Facebook and i'll update the blog too.

For those that have asked, Our Address here is:

101 Manning Drive
Room 7CH04
Chapel Hill, NC 27514

Big Day for Natalie 8/26/19

Photo by our dear friend Amaris (Amarisphoto.com)

Tomorrow is the “big day” for Natalie. I’ll be posting updates as she hits specific milestones or has clear needs coming up in recovery. This way everyone can know precisely what is going on and will know how to focus prayer, thoughts, and encouragement. So I’ll start tonight with a lengthier post about what she has coming up tomorrow and we’ll go from there!

Her procedure is scheduled for 2:00PM. We’ll arrive at UNC around 12:30 and wait as her operating team preps for all of the intricacies of the surgery. There is a chance they’ll move the time but we’ll update if needed. The procedure will happen in 3 stages. The stages are:

1) Removing the existing mobile part of the growth rod- This is the biggest part of the rod that runs the length of her back. It’s the part on her back that most people notice protruding from the top. That large “hump” at the top is actually a metal rod and not her back at all. There will be an entry point at the top of her back for its removal.

Pray that Dr. Stone and his team will work swiftly but precisely to get all of this hardware out without introducing any bacteria, virus, etc. This area in the past has been prone to infection because of the positioning of the incisions. Pray that infections, etc. can be avoided.

2) Removing the stationary hardware- This is the tricky part. The stationary hardware is what is actually connected to her spine and surrounding areas. As you can imagine there are plenty of areas in which the surgeon will need to have unimaginable steadiness to make sure he doesn’t damage anything while removing these parts.

Pray that the team can remove all of the hardware (screws, wire, etc.) safely without damaging any nerves or discs or bones. There will be an access point at the top and bottom of her back to remove these components.

3) Positioning and connecting her Halo- While still under the general anesthesia the team will affix 8 screws around her head into her skull. They are tight and will obviously be quite an adjustment for Natalie. She knows that it is screwed in but does not fully know what all this entails.

Pray that Natalie will understand that the pain associated with the halo will subside as she recovers and pray that she will understand and accept that she has to wear this device for the duration of her time at the hospital and potentially after she is released.

Natalie is a funny but ornery patient. During Pre-Op she is given a medication that makes her very “happy”. Typically she spends this time laughing at us, pointing at things that aren’t actually there, and talking to anyone that happens to walk by her room. She becomes “Social Natalie” and has a great time. However, in Post-Op, she ALWAYS wakes up angry and tries to get out of bed. Every. Single. Time. Although it’s pretty comical to see her get riled up it’s very important that she stays as calm as possible after the surgery. So if you will, please pray that her stubbornness will hold off until she at least gets to her room. Her strong will serves her well most of the time but tomorrow she’ll need to remain peaceful and still.

I think that about sums up tomorrow’s activities. It will take anywhere from 6 to 8 hours to get everything done in surgery but we will give an update as soon as possible after she wakes up. So be on the lookout! 

We've gone through surgeries with Natalie but nothing of this magnitude. This is new for us so we appreciate you all coming along with us. We are so thankful for everyone on this journey. Joanna and I truly can’t articulate how much it means. We love you all.  

Natalie's Update

It's been several years since this blog has been updated. I'm not sure why we ever slowed down with updates but I suppose it's like most everything else, life gets busy. We've mainly given "Natalie updates" via Facebook over the past few years but I feel that what she is facing in this next season of her life requires a more detailed post.

If you're familiar with this blog then you'll already be fairly acclimated with Natalie. Since before birth Natalie has kept us on our toes and she isn't slowing down now. If you're not familiar with this blog then I encourage you to read some of the earlier posts. The first couple posts will be most helpful to understand Natalie as we know her. It's wild how her early diagnoses or lack there of are still the same 8 years later. Her abilities and disabilities have mostly remained the same while others have gotten worse over time. This post will deal mostly with a few of her issues that have gotten worse and ways that we (and her medical team) are hoping to relieve the symptoms of her "conditions".

Basic overview of Natalie up to this point:

Natalie is disabled. I realize the word disabled is loaded and may not be the correct way of speaking in some situations and applications. However, Natalie in my opinion, is the physical embodiment of the word. Being disabled does not mean that a person cannot do something but rather it means that a person with a disability goes about their day doing things differently. Natalie was born with very low muscle tone all over and inside her body. What this basically means is that ALL of her muscles are much weaker than an average person. Her leg muscles, her arm muscles, her feet, her hands, her tongue, her eyelids...everything is weak. It's amazing she can eat or talk or walk or function semi-normally in general. But she does. Even though she is keenly aware that she has these disabilities it's almost like she doesn't even acknowledge them. She has developed completely different ways of doing everything. For example, when she realized her lips were unable to move taking away her ability to make labial sounds (d, b, etc.) she developed a way to maneuver her tongue and use it as a type of pseudo-lip instead. Or when she realized she couldn't use her lips to give kisses she somehow came up with a way to make a kiss sound and gives kisses the way any other little girl gives her Daddy and family kisses. When Natalie kisses your cheek, it's special. The best way to understand how Natalie's mouth works is to buy the game "Watch Ya Mouth" https://www.target.com/p/watch-ya-mouth-game/-/A-51812560. It's a mouthpiece game that makes it impossible for a person to close their mouth or lips. That's how Natalie is 24/7 but she finds a way to do normal tasks.

Another way that her low tone disability affects her is in the most obvious of ways. Because her muscles are weak it's hard for her to remain upright. Because of this she has developed scoliosis (major curvature of the low and mid spine) and Kyphosis (major curvature of the upper spine and neck, or a "hunch back"). So her body is practically collapsing on itself. The spine curves are bad but are mostly cosmetic however, because of the severity of the curve it causes her weight to compress her lungs and heart. Without correction, to put it bluntly, she would stop breathing and her heart would stop. That's why we decided, with the direction of her medical team, to put in an experimental magnetic growth rod in hopes of slowing this progression of scoliosis and kyphosis. Over the years we now know that the growth rod placed in her back is not working. Her curvature (especially in the upper back and neck) is becoming worse and is so severe that it's almost pointing downward instead of up like it's supposed to. The rod is now pushing it down instead of correcting it.

Since the rod is no longer working and has been failing over the last few years we are now able/forced to try something new. Her former Orthopaedic Surgeon retired this year and he never quite had a solution. He was fantastic to her and cared for her well but just didn't have the resources at UNC to figure out a comprehensive plan to fix her back and neck. After his retirement he was replaced with a relatively young Ortho specialist named Dr. Stone. Dr. Stone came in "guns blazin'" with a plan and a technique that he routinely performed at his former hospital in Atlanta and that is used by several hospitals across the US. The first time he met her he said, "We have to do something about this" and that something is what we're gonna do. On August 26th Natalie at the University of Chapel Hill will have surgery to remove her old growth rod and will begin a new process with a whole new method and technology in hopes of keeping her healthy. The hope is to also correct the scoliosis and kyphosis. The new technology and method is called "Halo Traction Therapy". It's an epic procedure but it's a fairly simple process to understand. She will be the first patient at UNC to have the procedure.

Here's how it works:

- While under anesthesia, the old magnetic rod will be removed and her team will place 8 screws into her skull starting above her eye brows and around her entire head. Then a metal "halo" will be attached to the screws.

- Once out of surgery, slowly over weeks, weights of up to 1/3 her body weight will be added/attached to the halo contraption above her and behind her. She'll wear the halo and weights 24 hours a day. When she's walking, when she's sitting, and when she's sleeping. It does not come off until the end. Over time, with the help of the halo, weights, and gravity it will hypothetically lift and straighten her spine. As mentioned above, this will take the weight off of her heart and lungs allowing her to continue breathing.

- While the pressure is off of her lungs, heart, and spine the docs will take the time to try to fatten her up as much as possible to give her the energy to heal and to grow in ways that she needs to. She'll more than likely be given some form of feeding tube to supplement her usual eating. We don't know if will be a mic-key button or the usual "through the nose" type tube yet.

- Once she is stretched and fattened, the medical team (ortho, cardio, pulmonary, etc.) will decide the next steps. Everything after the surgery is unknown. No one knows how her body will react to the halo or how the docs will proceed from there. There could be a new plan for rods, or a new plan of bracing, or fusing, or any other thing that's out there to fix backs. But, we don't know what strategy yet.

- She'll be at UNC for a minimum of 6 weeks. There is no speculation as to how long after the 6 weeks she'll be there. The docs have told us to plan for "months" of stay.

With this in mind, Joanna and I felt that we should let those who are interested know what we'll be up to over the next few weeks and months. Like everything with Natalie we'll be playing it by ear. We've begun to schedule out staying with Natalie and staying with Isaiah and traveling back and forth from home to the hospital. Between the hospital, school, working, and all that fun stuff it'll be a big adjustment for all of us and a logistical challenge but we're working it out. Our hope is to make both Natalie and Isaiah's lives as normal as possible during this process. Isaiah starts 4th grade on the 7th and Natalie will start 2nd grade then as well. They are going to be in the public school here in Elkin and Natalie's assignments will be done at the hospital.

We're both nervous and excited to be taking another step with Natalie. We always want what's best for her and we know that this surgery is necessary for her livelihood. The straightening success rate for most kids that have this procedure done is overall very positive but since Natalie is the only person of her kind (as her doctors refer to her) we are hoping for the best. We know that it won't completely straighten her out but we're hoping for enough correction that her lungs and heart are no longer in danger. She is aware of the surgery and is looking forward to something being done. She's been in a lot of pain and is excited to have the rod removed.

Once we get settled into a room i'll let everyone know what room we're in and all those details. Visitors, and letters or cards, or any support for her will make Natalie's days in the hospital a bit easier. So feel free to stop by. You don't even need to call in advance...just come on up. She also LOVES looking at facebook and would love to read comments of encouragement. Feel free to share her story and this blog with anyone that may be interested. The more people that are praying, thinking, or overall sending good vibes the better. We'll be posting blog updates while we're in hospital so please bookmark this blog if interested and stay up to date with our Natalie :-).

Thanks for your support!

Lyle, Joanna, Isaiah, and Natalie Sacco

6 months and counting

We finally have an update.

Natalie had her CT scan done today. As a reminder a few of Natalie's doctors were concerned that the front of her scull was closing faster than the back.  This would result in multiple surgeries.  Her doctor read the results and said that her scull and brain were growing normally and there was no need to look any further into surgery.  That alone was a great relief.  I'm thankful to say Lyle was able to take off of work and be there to celebrate with Natalie and me.

Secondly, we have made it 3 months without using Natalie's g-tube!  We met with a pediatric surgery nurse today to discuss having it taken out.  We came so close to having it taken out today but were not able to convince the nurse completely.  However, we have agreed to wait a few more weeks to prove Natalie truly does not need the tube any more.  Hopefully, come January 12th, 4 weeks from today, we will see her g-tube removed.  It was a great blessing having Lyle there for the conversation with the nurse today. We were able to tag team and really bring to light what life at home for Natalie looks like.

This coming Monday we have an appointment with Natalie's neurologist.  We will be getting results back from her last lab.  If it comes back positive there is a strong possibility that Natalie could take a medication that would allow her to catch up with her piers within 2 years.Assuming that comes back negative, we will work on a plan of where to go next.  Do we give up?  Do we take a brake from testing? Are there any new tests?  Do we start over? etc...

Thank you again for all of your prayers!

The results are in...

...and they're negative.  

Seven months ago I wrote a blog saying Natalie's results from the next generation DNA study should be coming in, within the next few weeks.  Five months later we did finally get her results.  This type of test covers what is referred to as mapping the human genome.  It covers approximately 80% of the genes that make up our bodies.  It helps explain why we think and move the specific ways we do as well as determines possible flaws in our DNA.  Our hope was to get a little closer to knowing what's wrong with Natalie and how we may help her as well has future babies in her position.  We went for a formal meeting where they told us that all of Natalie's results came back normal or in the medical terms, negative.  In fact the one test she had that came back with positive results when she was still in the NICU was redone and came back negative this time.  We find ourselves in the same position as always of just not knowing.  The doctor told us that they would not give up on looking for what is wrong with Natalie but they are lost as to where to go next.  He said that most likely anymore testing done will be the results from some different research project he finds and enters Natalie.

With all of that said, I want everyone to know that Natalie is doing great!  She is learning to walk.  The 2 curves in her back have stopped progressing which means her back brace is working.  She recently had surgery to correct her clubbed feet which has done a phenomenal job.  She's starting to get a little daring and letting go to try to stand on her own.  Her voice is getting louder and her vocabulary is growing.  

She still presents us with some challenges such as loosing her energy quicker than an average 23 month old.  She still cannot move any facial muscles above her bottom lip therefore she can't make sounds such as the m, p, and b sounds, smile, or blow.  She's still using a bottle 50% of the time.  She tires using a sippy cup and cannot close her mouth around a straw.  She understands that she is older and we can tell she's starting to get irritated that she can't do more.  She wants to get down and crawl next to Isaiah when we're playing outside or walking through a store.  She wants to eat everything that we eat but she doesn't have the stamina to eat tougher meats like beef.  

At the same time Natalie continues to astonish us with how she does things differently.  The average child when they start talking say only the beginning of words.  For instance, baby would be "bay" or Isaiah would be "Isay."  However, Natalie does it the other way.  Baby, is "aby" and Isaiah is "Saiah."  She also gets very vocal at the doctor's office.  The last time she was casted she told the doctor, "Go, Go, Go!" while pointing to the door.  The last time at the dentist she pulled her head away and said, "ALL DONE!"  Isaiah just told us the other night after taking a bath with her, "Momma, Did you know that Natalie can talk!"  She also is starting to mimic the tone in people's voices buy using inflections in her speech.  

We have a lot of fun with our family and are so thankful that we all have each other and all of you out there.  Thank you for loving my family and please never forget that we love you too!

My Baby's Growing Up

Here we are at the brink of Fall with a 15 month old.  Lyle and I have heard the oh so familiar phrase, "Can you believe it's been a year?"  We shake our heads and smile, but then we laugh with each other when they walk off.  Instead of the normal response parents should be giving Lyle and I just want to take a deep breath and say, "YES, we can believe it.  It's been the longest year of our life!"  haha  It's with great pleasure to be able to laugh and joke about where we've been, where we are, and where we're headed with our sweet Natalie.  We're full of relief, exhaustion, excitement, joy, and encouragement to be celebrating a year of Natalie's life.  A year and half a go Dr. Goodnight told us our little baby girl had a 50/50 chance of survival after birth.  Isn't truly wonderful to be celebrating on the 50% chance side of life!?!
With all of that said here's where we are as a family with Natalie's diagnosis and development so far.

Diagnosis - NONE
However, Natalie's neurologists entered her into a medical trial that is referred to as, Next Generation DNA testing.  It allows a lab to test 20x the amount of DNA than what's been done before.  This a 5 year long trial so Natalie's results will be reviewed for the next 5 years as the medical field changes and becomes more knowledgeable.  The only conclusion that has been made about Natalie is that wherever her genetic informality is a normal lab does not have the resources to find it.  We're hopeful to see some results in this medical trial.  Natalie's doctor called and said we should be receiving the results in the next few weeks.

• Back
• Natalie is now wearing a back brace.  Because her core has taken so long to strengthen she has developed scoliosis, an S curve in the spine, and kyphosis, a hunched back.  

• Feet
• Still in the process of correcting her clubbed fee.  We are casting, taping, and wearing special shoes depending on the week.

Development - A lot (currently 15 months old)

Natalie is continuing to develop and surprise her therapists on a monthly basis.  No one really knows what to expect because she continues to build her strength in the opposite direction of a normal child.  :)  What is expected during the infant - toddler development is for the child to first strengthen their core and work they're way out to their limbs and eventually fingers for the fine motor skills.  Natalie does the opposite.  She gained her strength starting with fine motor skills and is gradually working her way to her core.  Although she may not be able to walk she can spot and pick a cat's hair out of the carpet even if you did just vacuum twice.  lol  The problem this presents is that a baby's core strength is what signals the body to start maturing.  As you are probably familiar with babies are very flexible creatures.  As their core strengthens it sends out signals.  It tells the muscles and bones where to settle and stop being so flexible.  It tells their insides when to mature, whether the stomach can handle table foods or cows milk versus mom's milk or even whether swallowing something lumpy is ok.  With all of that said here's where Natalie is today...

• Eating her first table foods
• She has fallen a little behind on her feeding abilities but her feeding therapist says it's the lack of her core strength.  As her core strengthens her digestive track matures and her appetite will increase.  We would say she's right around a 12 month's old abilities in eating.
• Starting to put weight on her legs when you hold her in a standing position.
• Leg strength and lower abdomen are around 6 month's old
• Has full head control
• She was able to move to  forward facing car seat this past week.  She's on target with the exception of lifting her head more consistently while in a crawling position.  This has more to do with her back muscle versus her neck.
• Is starting to crawl!
• 8 months old
• Is a ahead in her cognitive
• She has more words in her vocabulary than normal.  She notices and reacts to people's mood changes.  16-17 months old

I'll have to say it's quite entertaining to have a baby that does so little but understands so much.  What perhaps makes us laugh the most about Natalie is the fact that she laughs the hardest when someone gets hurt.  If your remember the youtube video, "Ouch, Charlie that hurts,"  then you've seen a glance into Natalie's mind.  She laughs when you fall, if you cry, if you scream, or when her brother, Isaiah, is in trouble.  What catches people of guard is Natalie still can't make facial expressions.  When you hear a belly laugh coming from a straight faced baby it's a little confusing, but hilarious.  It goes perfect with the fact that she's typically laughing at someone getting hurt.

Isaiah is thrilled to have a mobile sister.  He told me just this past week that Natalie is older now so we need a another baby.  I knew it wasn't just me that finally feels like my baby is growing up.

New house, New therapists, New medicine, New skills

A lot has changed in the past 2 months.  We have moved back to our Willow Spring house.  Lyle has started a new job.  Natalie turned 10 months old. Natalie had to switch therapists since we've moved to a new county.  Natalie is on a daily medication.  Natalie is making major steps in her abilities.

Natalie has been doing great.  Since the last time we talked she started a medication called Mestinon.  It gives her nerves more time to process the commands coming from her brain and being sent to her muscles.  We have seen great results from this medication.  She is able to eat in a normal time span and does not have to nap afterwards.  She is consistently rolling from her stomach to her back.   She can sit up on her own between 1-2 minutes.  She has also started putting pressure on her feet.  People other than her family can tell when she's smiling.  She has started cooing and babbling.  We're hearing her voice for the first time other than the sound of a cry or grunt!

She had 2 assessments a couple of weeks ago which both concluded that she is above average in her cognitive communication.  Meaning, she notices changes to her environment such as who's speaking, when her name is called, when someone come and goes, and understands simple commands more than average baby her age.  On the physical side of things she has accomplished about a months worth of improvement in a 2 months time versus a 4 months time.  She is sitting on her own, rolling to get reach for toys, and kicking.  Typically a 10 month old would be crawling and in the beginning stages of walking just to give you an idea of where we are.

We're really excited about all of her changes.  These past changes are making our lives a little more easier as far as toting the little girl around and helping her play.  The biggest problem we are running into now is trying to keep her from sitting up and falling out of her rocker.  :)

The feeding tube is out!

Natalie's feeding tube is officially out!  January 12, 2012 the UNC CH doctors, therapists, and nurses finally agreed to take it out after 6 hours of deliberation.  She had her tube for approximately 6.5 months.  Our understanding is that it is rare for a child to have a feeding tube under a year's amount of time.  Natalie has done exceptionally well with her eating progress.  She's working with a speech therapist (who essentially is a feeding therapist).  She's worked up to 1.5 jars of baby food a day which puts her right on target for what a baby her age should be eating.  She has a special feeding utensil that helps stimulate her facial muscles to encourage her to eat.  Typically she take a 30 minute nap after each meal because it's an exhausting task.

We also received the results back from Natalie's EMG.  As a reminder, an EMG tests for the reaction between the nerves and the muscles.  It tells whether there are delays, weakness, or slow reactions.  To our disappointment it was another inconclusive test.  The doctor who performed her EMG said he was not able to get enough reaction from her limbs and was not able to get any reaction from her facial muscles.  He is known as one of the most experienced and best doctors in the region for performing and reading EMGs.  He said that Natalie is the first patient he has ever had that he was not able to get some conclusive results.  Once again Natalie stuns a doctor.  :)

However, we go to visit her neurologist on Monday February 6th, who will be prescribing Natalie 3 different medications.  Originally she was not going to try Natalie on the medications without a conclusive result from the EMG, but after further research she found that they are be-nine.  Be-nine meaning there are no side affects if they do not improve her muscle functions.  We will got through a testing period to see if any of these improve her muscle memory/ functions.   We should be able to see a difference between 1-9 days per medication.  Hopefully she will start on one of these next week.

As far as growth stages: (Natalie is 8 months old)

• Natalie's upper body, neck, and head strength is around a 2 month old.  
• Her lower body strength is around a 4 month old.
• Her cognitive and feeding is around an 8 month old.

Major milestones since the last time we talked:

• Turned from her belly to her back twice!
• Eating baby food, fruits and veggies.
• Putting toys in her mouth.
• Can accurately grab and pull your earrings out.  (sadly I know this from experience, haha)
• Has attempted once to push up with her arms while on her tummy.

She's a beautiful growing girl with a wonderful big brother!  Thanks again to all of you for your continual support.  It helps Lyle and I deal with the time between each blog.

Ready or not, here we come 2012!

Natalie's blood test came back "sort-of negative."  The test was for a congenital myasthenic syndrome called RAPSN gene sequencing.  In other words, a vary narrowed down DNA test.  What the "sort-of negative" result tells us is that Natalie does not have this particular syndrome however we are headed in the right direction.  Our next move is for her to have an EMG.  They will test her for a delay in what is called the neuro-muscular junction.  This is where the nerves talk to the muscle.  Natalie's doctor believes that there is a big possibility that there is a delay in the two talking to each other.  The EMG will give us a definite yes or no.  If we get a yes we will start her on 1of 3 different medications that will help improve the communication between the 2 which will result in a much quicker development.  If the EMG does come back saying Natalie's issue is in her neuro-muscular junctions then we will know what her issue is.   However, what is causing it will still remain a mystery.

Natalie's doctor asked permission to enter her into a Federal grant medical study being called the next generation of DNA testing.  It will allow us to test Natalie to an extent humans have never been tested.  It simply requires 1 test tube of blood and a slew of questions.  While she is being tested under this grant all of her medical costs and care will be paid!  This testing will allow Natalie to be tested for more DNA abnormalities.  We're excited that she will be part of a program to help future parents and kids in our position. This program starts in January but the actual blood tests won't be taken until early spring.

Here's an update on what Natalie can do now.

• turn her head while laying on her belly
• sit up with little back support
• starting to make cooing sounds
• making some great progress in rolling from her stomach to her back
• eating rice cereal
• has enough strength in her arms to hold a normal size baby rattle

Our goal we would like to have reached by the next time I write: 

To have Natalie's tube removed on January 12, 2012.

Please feel free to ask any questions.  I know that was a lot of medical jumble.  We love you all!

Refreshed

Thank you for all of your prayers.  We're in our first week back from Lyle's fall break and feeling refreshed.  We were able to spend some much needed family time.  We were able to focus on just our family.  God is good and His timing is perfect.

Natalie is doing great with her therapies.  All of her therapists are impressed with how quickly she picks up on  each new exercise they teach her.  She is finally starting to hold her head up!  She's also back to taking full bottles each time she eats.  This past week her smile got a little bigger, she's started making those sweet baby coo's, and can sit up in my lap.

Our next set of appointments are October 31st.  We have a nice break from doctors.  I'll update you as soon as we find out anything.  We most likely won't be getting any test results back until late November to early December.  In the mean time enjoy the beautiful fall weather!

Days blur together

I just want to give everyone a real quick update.  Natalie is doing well.  She had her 4 month check up where she weighed 10lbs 1oz and was 24 inches long.  She has fallen a little behind on her growth, but the good news is she's still growing.   She had a bad reaction to her immunizations.  She had a fever and was too tired to eat by the bottle.  The last 24 hours have been tube feedings, holding, rocking, and all of the other things that come with a sick baby.  However this is the first time that Natalie has been to tired to eat but still able to cry when she was hungry or not happy.

My main reason for this post is to ask for some specific prayer.  Lyle and I are tired.  Our goal for Natalie functioning as a normal child 3-5 years from now is feeling really far off.  Patience is running thin for each other and Isaiah.  We're struggling with shifting blame to each other for what is and isn't getting done as well as who is getting the least amount of sleep.  haha  I suppose it's really comical when you think about it but in the moment it is simply miserable.

Thankfully this next week is Lyle's fall break at school.  He will only have to work his night job.  We really want to be able to take this time and give each other the breaks we need as well as spend some quality time as a family.  When I say we're tired, yes I mean physically but also emotionally.  Pray that we will feel that we are getting more sleep than we are and that we will use our time together in the best way possible for all 4 of us.  We would like to feel somewhat normal again.  lol "good luck"  i know.