Neavey’s Spine Saga: Part 3

And so, Kelly started the series of countless calls and referral requests and calendar consultations to get an appointment scheduled for an evaluation for Neavey at Scottish Rite Hospital in Dallas. Our appointment would be a few months later. On June 11 we met with the Scottish Rite Chief of Staff and his team. These would be the people working with Neavey if we decide to do the surgery. Neavey did a great job on the three hour plus drive to Dallas. We spent the first night we’ve had with her in a hotel, and the next day went to Scottish Rite.

The hospital was smaller and more manageable than we expected. We were welcomed and then escorted around by a series of helpful volunteers, some of whom seemed to have been former patients at the hospital. The surgeon and his team were very nice. They wanted to hear from us about what our life with Neavey is like, what she is like, how her days go, what we want for her, etc. After looking at her x-rays and doing a fairly brief hands-on evaluation, the surgeon said that he felt spine surgery would help her and that he thought we were likely to want to do it. Like the first surgeon we saw in Houston, it was Neavey's kyphosis that he was most worried about, though he obviously acknowledged the severity of her scoliosis too. The surgeon in Dallas said that with a curve as bad as hers, the curve just wants to get worse. It sounds like this is partly a characteristic of neuro-muscular spinal curves, the type of scoliosis and kyphosis that Neavey has. It sounds like the progression of a bad curve is also due in large part to gravity pulling down continuously on the top of her body.

The surgeon said that they had worked on many kids "like Neavey," by which we think he meant kids with multiple impairments, severe developmental delays, neurological issues, etc. Based on Neavey’s overall condition and the condition of her spine, the surgeon was very surprised and glad to see how much flexibility Neavey had in her spine. We think this is probably due to our devotion over the years to maintaining her physical therapy and to working with her body so frequently, and to doing a lot of different positioning.

Largely because Neavey has relatively good flexibility, the surgeon wasn't in a big hurry for us to do the surgery. He said they wanted to see Neavey and us again in 8 months to reevaluate and see where things stand. I gather that the flexibility is so important because it allows them to straighten the spine more prior to surgery (more on that in a minute), which I also gather allows for more effective correction and less traumatic surgery, and maybe better results for the surgery. The other reason he wanted to see us again is that they don't like to go into the discussion of surgery the first time they meet with a family and evaluate a patient. They prefer for us all to get to know each other a bit, and for us to have some time to consider things. We appreciated this about them. Scottish Rite is a Masonic hospital, as are the Shriners hospitals, and only recently has Scottish Rite had patients pay at all (to the extent that their insurance will cover costs, but it sounds like patients don't pay out of pocket), and lots of their staff volunteers. I mention this because it speaks partly to how caring and selfless the environment and spirit of the place was overall. Also, it just demonstrates staggering generosity. In some ways, it was a low frills kind of facility, but it was small and friendly, and most importantly for us, it specialized on bone conditions, spines being one of their particular specialties, and we had a good feeling about them. Certainly the surgeon and his fellow and nurses seemed expert. This is one of probably 6 to 12 hospitals in the country where we could have this surgery done for Neavey.

During the visit the team described the surgery a bit, and the benefits and risks of surgery. The first step in doing the surgery would be for us to drive Neavey to Dallas 4 to 6 weeks before the surgery for a short procedure where they attach a halo to Neavey's skull. This would allow her to undergo "halo traction," using hardware attached to her wheelchair, and probably to her bed. Halo traction can dramatically straighten the spine. The straightening is temporary, and once traction ends, the spine quickly would return to its prior curved state. But, by correcting much of the curve prior to the surgery, the surgery then basically fixes the spine in the straighter position achieved with traction. The spine is permanently straightened in two ways. First, an apparatus of rods, pins, screws, hooks, etc. is attached to the spine to hold it in position and perhaps straighten it a bit more. Next, bone grafts are used from cadaver tissue in order to fuse the spine. In Neavey's case, the surgeon said they would fuse the spine from her pelvis all the way up into her neck. Over the course of 6 to 12 months, the grafts cause the spine to fuse, in effect making the spine into one long bone. At this point, the metal apparatus becomes largely unnecessary, but it is usually left in place because taking it out would involve another surgery, and leaving it causes no harm. It is typically only removed if there is infection.

The team said that the halo procedure is about 20 minutes, and that we would be able to take Neavey back home the next day. The spine surgery itself would take about 6 hours. If the surgery goes smoothly and she comes off the ventilator ok, she would go straight to their recovery floor, where we could be with her 24/7. If she has trouble coming off the ventilator or there are other serious complications and she needs to be in ICU, then she would be taken to Dallas Children's Hospital just down the road (because Scottish Rite doesn't have an ICU). The surgeon said this happens less than 5% of the time. If Neavey stays at Scottish Rite, it is just a 52 bed hospital, where she would probably stay for about a week recovering. They would get her back into her chair a day or two after the surgery, and the main thing we would be waiting for to take her home is for her wound to heal enough. It would take about a month, apparently, for the initial recovery period and for Neavey to return to pretty much her full level of functioning. It would be 6 to 12 months for full recovery and fusing of the spine.

Benefits of the surgery would be that the curve in her spine would likely be significantly corrected, and if the surgery and recovery were successful, her spine would be permanently straighter, and a further curve would not be possible. This would give her greater trunk strength and control, she would probably be able to keep her head up more, and be much more upright than she has been in years.

The team was clear about the risks of the surgery too. This is a major and life-threatening surgery. Mortality rates are low, though. The surgeon thought at their hospital probably about 1 in a thousand. The biggest danger during the surgery is blood loss. Neavey would need a transfusion for sure. The surgeon said that if bleeding was a severe problem, they would stop the surgery. Infection is the other major concern, both just after the surgery, and in the first year afterwards. Response to infection varies based on what happens, and rates of infection are hard to say. It would need to be monitored closely. They would also do a full physical health workup on Neavey prior to the surgery. Her nutrition and her epilepsy could both conceivably make her ineligible for the surgery. They suspect she would pass the tests to proceed, but they are conservative about doing surgery because the surgery and recovery are very taxing on the system, and they wouldn't want to risk working with a patient who was compromised even before the surgery.

It has been harder to get straight answers to questions concerning the dangers of not doing the surgery. It seems, though, that the risks of not doing the surgery may not be quite as horrible as we understood from our orthopod. At Scottish Rite, they seemed to be more concerned about Neavey’s lifestyle. As her curves progressed, she would likely be impossible to feed orally, it would probably become impossible for her to go into her wheelchair and she might be bound to a bed. There is a chance it could cause discomfort and other complications as well. Both her orthopod and the team at Scottish Rite seemed to feel it would be likely that, without the surgery, we would be unable to continue to care for her at home.

As things stand, we'll see where we are in 8 months. Kelly and I and Neavey’s dad are feeling more and more like we'll need to do the surgery. If we are going to do the surgery, it is probably better to do it sooner than later, while Neavey is healthy and before the curves get even worse. Electing to do this surgery is a lot for Kelly and me to wrap our heads around. Like all parents, often we subject our child to unpleasantness because it is for her own good. But, this would be raising the level of invasive unpleasantness to an entirely different level. And, we would be subjecting her to real life-threatening danger and pain, and without any certainty that we will be helping her.

Parts of the process are especially horrible to think about because we don't think Neavey would be able to understand what is happening to her, or why we are doing it. Other parts, like the halo traction for example, might be terrible for us, but for her, not be as big a deal. The team in Dallas said that aside from initial headaches, the halo and traction really shouldn't hurt her at all. While we dread the prospect of watching her go through halo traction, and being unable to hold and cuddle our kid the way we normally would, she won't be looking in a mirror or reaching up to feel the strange apparatus on her head. If we move forward with the surgery, and if we can keep her comfortable during the traction and as she recovers from surgery, then Neavey might not be bothered that much or for that long. And certainly we don't think she would remember any of this. So, while the spine surgery and all it entails could make for the worst and hardest few months of our lives as Neavey’s parents, for Neavey it might be something different. We would hope it might amount to just some miserable days, and probably a bad month or two, but with benefits that make the rest of the time she is alive far more exciting, stimulating, pleasant and comfortable.

We know that whatever we decide to do will be scary and hard. But for now, at least, we don’t expect to feel especially conflicted, no matter how we decide to proceed. With Neavey, there has rarely been a clear path forward, and there’s never been much about Neavey’s future that we take for granted. So far we feel like we’ve been able to give Neavey a life that is very much worth living, and in which she seems mostly happy and satisfied. Our idea is that Neavey’s happiness is as dependent on feeling comfortable as it is on anything else. Though the surgery could make for some terribly uncomfortable times, it also might give her many more years of greater comfort that are very much worth living. The surgery could go badly, of course, but we are fairly resolved anyway that one day we will lose the fight with Neavey’s disease. If trying to treat Neavey’s spine turns out to be how things go bad, then it is. All we can do for Neavey now is all that we have been able to do for her before, or will be able to do for her ever – to try to gather the best information we can, and then use it to create for her the best chance we think we can give her at a life that is most like what we think she wants.

Neavey’s Spine Saga: Part 2

Now fast forward another seven months or so to March, 2012. It is time for Neavey’s next check-up with the orthopod. I (Keith) opt not to miss work for the appointment since we’re anticipating a routine visit, and Neavey’s dad will be there with Kelly to help lift and transfer Neavey for xrays, etc. When the doc comes in, he asks if we ever met with the spine surgeon he recommended. (He never received information about the appointment, even though he should have since he had referred us and even though we'd confirmed with the surgeon's office when we were there that they would send information to the orthopod.) We (Neavey’s dad and Kelly) reported what we heard from the surgeon, that Neavey wasn’t a candidate for surgery and that the surgeon suspected her scoliosis and kyphosis probably wouldn’t get too much worse anyway.  



The orthopod responded with clear frustration and a little anger about the fact that he hadn’t heard about this appointment and that we hadn’t followed up sooner. He felt we really needed to get a second opinion. He was quite concerned about how much the curves in Neavey’s spine were continuing to progress, and he worried that, without surgery, the curves could get so bad that they would threaten the functioning of Neavey’s vital organs (heart and lungs in particular), and would almost certainly be debilitating enough for her that we would no longer be able to care for her at home. The timing of the surgery, the orthopod indicated, would be tricky. The surgery might or might not be urgent, but if the curves progressed too far surgery might no longer be an option. Harder still was the likely scenario he described that by the time Neavey’s curves had progressed so far that we’d be certain surgery was necessary, it might already be too late for the surgery to be undertaken.

The orthopod referred us to what he felt was our only alternative for a second opinion: Scottish Rite hospital in Dallas. He explained that Scottish Rite specialized in more unusual pediatric bone cases, and told us that the surgeons there would have much more experience than anyone in Houston doing spinal surgery with a kid like Neavey.  (This was consistent with what the Houston spinal surgeon had told us as well.)  Because the wait for an appointment at Scottish Rite might be months, the orthopod wanted us to set-up an appointment immediately.

And so, the prospect of spinal surgery loomed again. Kelly and I both dreaded our next appointment, and expected to face at least a pair of difficult and unclear choices. What we anticipated hearing went something like this. One choice would be to subject Neavey to a major and life threatening surgery that would attempt to straighten her spine. Even if she survived it, the surgery could be unsuccessful (a likely outcome, according to the surgeon in Houston), leading to further surgeries, possible infection, additional complications, and the prospect at best of a long and difficult recovery and rehabilitation. Or, on the other hand, we could choose not to do the surgery, and risk watching Neavey’s body, over the course of her teenage years, gradually contort until it almost literally squeezed the life out of her. She might suffer months or years of even more limited mobility, and even more intense and difficult care, and her decline could be long, slow and painful.

Of course, each choice could turn out ok too. The surgery could go smoothly and might help Neavey. Or, the curves in Neavey’s spine might not progress so radically. Kelly and I figured we'd want not to subject Neavey to surgery, but mostly we tried, simply, to just focus on the next appointment, and attempted not to think too much about Neavey’s spine until we had more information about the choices we faced.

Fortunately, Neavey helped us by being mostly happy and strong, and by serving, as she does, as a compelling reminder for us that at most moments all that matters is the present, and that the future will work itself out without us trying, in vain, to foretell or control it.

Neavey’s Spine Saga: Part 1

A new Neavey saga, and the topic of a series of three (for now) new blog posts, started about a year and a half ago when we had our regularly scheduled 6-month check-in with Neavey’s orthopod (or bone doctor, as we sometimes refer to him).  The appointment was uneventful, and the doctor said we could wait a year to see him again. 

About 5 months later (in July of 2011) we noticed that Neavey was hunching over more and her body looked more crooked.  Neavey has always had very minor scoliosis, which the orthopod warned us might get worse when she hit puberty (Neavey turned 13 in April, 2012). We scheduled an appointment with the orthopod to have Neavey checked out.  At this appointment we got the news that Neavey's scoliosis had significantly worsened.  The doctor was alarmed by the fast progression and said that surgery could be necessary in the future.  He wanted us to meet with a spine surgeon soon to have Neavey evaluated and to hear options. He referred us to a surgeon that he described as one of the best in Houston and, “the one I would take my child to.”

Neavey's scoliosis xray from about
a year ago at her appointment with
the surgeon (two images merged).
Her spine has worsened considerably
since then.

We made an appointment immediately. The soonest they could see us was in  September. We dreaded the appointment for the 6 weeks or so until it occurred.  Once we met with the surgeon we actually felt relieved when he told us he thought he couldn’t help Neavey, that she wasn’t a good candidate for surgery and there was no effective treatment for her scoliosis. In fact, he was less concerned with her scoliosis than her kyphosis (basically a front to back curve, creating a hump effect or hunchback).

Neavey's kyphosis xray from
the same day

Normally, of course, hearing from your child’s doctor that “there is nothing we can do” isn’t exactly good news, but we mostly felt relieved she wouldn't have to undergo surgery. The doctor also said he suspected Neavey was nearly done growing, and he thought there was a good chance that the curves wouldn’t get that much worse anyway, which of course left us feeling still better.

We walked away from that appointment prepared, yet again, to let Neavey’s disease do to her what it would while we did for her all that we could, helping her stay as strong, happy, healthy and engaged as possible, for as long as possible.

Time Flies

Where has the past year gone? It has been almost a year since our last post. Many of you follow us on Face Book, so you may already feel caught up. But just in case, here goes a quick account of last 11 months.

Weight Gain!
After another illness in April 2010, Neavey bounced back as usual but concerns about her weight became an issue in May during a Neurological check up. After scrutinizing her calorie intake, her dad, Keith and I went on a calorie push for a few months. In late May 2010 she weighed 49 lbs. As of last week, (January 2011) she is up to 60 lbs. Most of that weight gain occurred before November.

Seizures on the rise.
By mid July Neavey had gained 5 lbs during the big calorie push. Yay! BUT, with that came a jump in seizures that caused her doctor to increase her Keppra. And as luck would have it, she couldn't tolerate the increase. WIthin 3 days she was screaming uncontrollably from the "anxiety" side effect caused by overly high levels of Keppra. We decreased the dose and waited a few says for her to normal out again. As any one with a screaming baby knows, this can drive a sane person over the edge. By early August my nerves were shot.

Back at School
By late August Neavey's seizure activity was on the decline and school started. Luckily Neavey was granted an extra year at Memorial Elementary where the teacher and aides have perfected their "Neavey care" skills. Things were going great until the second week of school when she came home with an ear infection. She missed a couple of days of school and then was right back home again with a throat infection in September. Seizure spikes in October kept her home for a day or two. By November she had yet another ear infection. Then a really bad cold got the best of her in December, this one she shared with me.

The New Year - 2011
Concerns over blood chemistry had us a little concerned in January, but luckily her weight gain has caused her Zonegran levels to fall enough so that we are not as worried about her Bicarbonate level. We are in the middle of figuring out how and where she will transition into a middle school setting next fall. These kinds of things must be dealt with now and in the early spring.


On the horizon
Next up, more dentist visits. Oops forgot to mention the visits to the dentist to extract "shell" teeth. Neavey doesn't like to give up her baby teeth easily. Thy have to be extracted, which she is not usually too happy about. Luckily she has the best dentist EVER (Dr. Bruce Friedman)! She only cries for a few seconds. He and his staff are top notch!


A check-up with the bone doc is due as well. We're a little afraid of how all this growth will have affected her hip dysplacia and scoliosis. And it is time for new wrist orthotics. The cool pink Beniks must be replaced. I think we'll stick pink!

Viruses, Allergies, & Recovery

The end of February and the first half of March were plagued by illness and allergies. There were lots of long nights, but only one very scary one where Neavey's airway became seriously compromised.

Neavey was at her dad's house, and luckily her step mom saw the trouble Neavey was having breathing and had dad call Keith and me. It was definitely an episode that was going to take a team response. Within 10 minutes of the call Keith and I were on our way to his house, driving through an almost surreal 2 AM fog, armed with suction machine, pulse-oximeter and other various bits. The best bit was a simple tongue blade. You know, the "say ahhh" thing. Neavey's pediatrician has coached me on how to help Neavey recover her airway when it is "clogged" with excessive mucus. Inducing a gag reflex to encourage vomiting is the best way to clear the upper and even lower airway in some cases. It is much less invasive than sending a catheter down her throat to "suck it out". The gagging and vomiting worked like a charm. She spewed up at least 100 mls of thick yellow mucus that was clogging her upper airway. Her pulse-ox went from 80% to 94% within minutes. If you don't know, at 80% pulse-ox you are pretty much ashen or blue in color. Thank goodness for my mom's foresight to get Neavey a Pulse oximeter for Christmas. It might sound like an odd gift, but so helpful! YAY for Grandma RN.

That was how the second virus went down. The third (plus the onset of allergies) is about two weeks behind us now... We are hoping for an uneventful summer! The slide show above is from pictures taken of her between virus 2 and 3. Keith and I got her this the adjustable bench like the one she uses with Jacy (PT) for Christmas. She has been doing a great job of bearing weight through her arms as she works at the bench.

Groovy Hand & Wrist Splints

New custom Benik hand & wrist splint

Neavey's new custom hand & wrist splints are not only fashionably pink like her wheelchair, they also make her look like a rad rocker. Don't you think? Although she does come from a line of drummers, (me, my baby brother and my dad) she is not wearing them due to injury sustained while playing the skins. Since Neavey uses her hands less and less these days, her wrists have started deviating toward her ulna (the outside bone in the forearm). In order to reduce the chance of muscular contractures, she wears her left (less functional hand) Benik for 8 hours during the day and the right one for 8 hours at night. They have metal stays on the top and bottom and a hard plastic stay along the ulna to keep her hand and wrist in neutral. She doesn't seem bothered by them at all. I think she looks rather tough in them!

She was fitted for these along with new ankle foot orthotics and a new nite splint in January when I realized she had outgrown her old ones. Our awesome orthotist, Jason, at Dynamic Orthotics confirmed that she had at least an inch of growth since May of last year. I have only a few inches left on her. YIKES!

Havin a ball!

In January Neavey had another visit from Jacy's supervisor, Brian. No, not as in the uncle variety. This Brian subbed for Jacy back in November and tried some different things out with Neavey that I wanted him to do again and show both Jacy and me how to pull it off. Becasue, if you didn't notice those ginormous guns on Brian, then my photography is lame. However, he was hoisting Neavey up onto this giant therapy ball and rolling her and that ball around like they were wiffely. I would never have guessed Neavey would like the upside down business etc.. But she does! YAY Brian, for showing something to add to Neavey's therapy arsenal. Jacy has incorporated the "ball" work into Neavey's physical therapy regiment and it's going great.
GO TEAM NEAVEY!

Uncle Brian Does it Again!

Alli, Brian and Laura

Uncle Brian (my brother) and his wife Laura
have become quite the advocates for Neavey's
visual stimulation. Over the last year and
a half they have been contributing
to her "Sensory Theatre"with some
great sensory solutions to enhance
her visual attention.

Many of you have seen or read about her Laser Star
projector which is still a favorite not just for Neavey,
but for us as well. For Neavey's 10th birthday,
Brian sent a Space Projector that casts a lava
lamp-like effect on to any flat surface. Just pop
in an oil effect wheel and watch the bubbly goo go!

The latest installment intensifies the effect of the
Space Projector. The pod Neavey is lying in
brings the bubbles within inches.
Visually stimulating indeed! The pod pops
open and folds down like one of those dashboard
sun visors. Easy, breezy fun time.

Thanks little brother! We love you guys!

November

Bundled up and waiting for the zoo to open.

The highlight of November was Neavey's Class Field Trip to the Houston Zoo. The goats in the petting zoo were the only animals Neavey paid much attention to...

Group photo, and random girl walking by.

Neavey was very perky in the Gift Shop! Must be my kid?

Mid- November: Neavey is supposed to be in her standing frame about 45 minutes two times a day. She used to mostly put her head down on a pillow. Lately, we are seeing a little more energy She is holding her trunk and head up very well. We think this is the benefit from her new vitamin D regiment.

Turkey Day!

Hi, Aunt Di!

The tickle progression

Neavey, pretending to ignore the tickling.

But wait, maybe she'll succumb to the giggles....

And...Di has achieved full on TICKLE TORTURE!

Keith's Aunt Di came to visit in October. She loves Neavey to pieces and Neavey really enjoyed all the attention. It was so nice to have Di come hang out with us. We enjoy sharing Neavey with anyone who can find the time to visit. Book your spring and summer visits now!

New Feeding Smock

One of Neavey's honorary grandmothers, Linda, made this feeding smock from some sketches, an old smock about 10 times smaller and some materials I sent plus some from her own stash. It is working out great. Now we only need about 10 more.

Excercising with Keith

Cool Capri's

Neavey's gaining strength back after the swine flu.

Huge accomplishment here for Neavey to sustain this position with only a mild assist.

Home Again - Home Again

Heading home on Saturday - Goodbyes to nurses

Saturday night set up with suction machine that my mom( the airway pro and RN supreme) helped me acquire. And Oh did the hospital bed that Mr. Jason gave Neavey years ago come in handy.

Monday - Not ready for school yet, but happy with balloons (from one of the publishers that Keith works with) that had met us at home upon our return.

H1N1 Crisis

Night 4 - still on O2

No rest for the weary! Physical therapy started for Neavey on day 3. This is day 6. She was starting to perk up a bit

TCH provided a standing frame as well as a therapist while she was in hospital.

asleep in standing frame

Day 6 - acting more like herself with Keith.

September 11th Neavey's rapid flu culture came back positive. We will never really know if she in fact had the swine flu because Harris county had stopped differentiating between the flu strands after a month of 95% of the follow up testing coming back positive for H1N1. We were told to assume it was H1N1 and to proceed with caution.

Because Neavey cannot clear her own secretions very well if at all when she is sick, we were forced to take her to TCH's ER the next morning, She was admitted and spent 7 mostly hellish nights on the Neuro. Floor. Thank goodness for awesome night shift nurses who don't freak out over seizures (you'd be surprised how many nurses do) and rock out the at upper airway suctioning. Thank you Michelle! Neavey started acting a little like herself around day 4 or 5 but continued to: run high fever every other night, need oxygen, and need upper airway suctioning. Alas, she did get to come home one week after being admitted and has hung in there since then. Keith and I survived only with the help of great nurses and friends. Thanks to all of you who made us meals, brought supplies to TCH and provided rides to and fro.

July Rocked!

June was definitely a warm up for July. Neavey kept up her perky-ful tempo!

Neavey has a knack for the camera. By knack, I mean the moment the camera goes on she immediately stops doing the cool, great thing that prompted filming. Curses!

Le Bleu water bottles make the best shaker toys! They have a perfect narrow neck that Neavey can grasp. I've only been able to find this water at Specs.

Last Christmas my mom, brother (Justin) and his partner (Amy), Keith and I filled many bottles with bric-a-brac and assorted rattley bits for Neavey. They are the perfect handheld toy for her.

Sitting with Keith

June was an energetic month.

Other May Moments

Bubble time is a fun weekend event. We have Neavey's former occupational therapist, Sue, to thank for this fabulous birthday gift.

Neavey during office hours. Neavey sits in a tumble form chair with a tumble form tray and pushes her blinking, vibrating and music playing (the trifecta of therapy toys!) therapy button toy.

Keith mastered the art of a half ponytail!

Grandpa Larry and Barbara sent this therapy tool. The blue snake vibrates and can be manipulated into different shapes and postitions for muscle stimulation.

Sometimes Neavey is just sooooo cute she can't help herself. And the big drippy drool just adds to the cute? I just brought her home from school here. She is hangin' out with me for a few minutes while I put away her lunch kit things.

Last session with Jennifer

In May Neavey's speech pathologist of the last few years moved to England. Yay for her...Booo and hiss for us. We will miss her!