Friday, August 31, 2012

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.

Saturday, August 11, 2012

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.

Friday, August 3, 2012

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.