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.