St. Louis, Day 1

Let me preface by saying that this post will be long – lots of details about what we learned today. It’s therapy for us and provides a journal for us to look back on. I really don’t expect people to actually read it. The synopsis is:

Havyn had a great day and we got to meet a bunch of great doctors. We learned a few things, we ate some lunch, and we saw a big cockroach. We are officially in love with St. Louis Children’s Hospital and the doctors here. Well, except for the cockroach. Oh yeah, we went to the zoo. The end.

If you’re looking for a good sleep aid, feel free to read on.

The morning started off with a nice breakfast at HavenHouse and then we were on the road to the hospital. Note to self: when you think you’ve left enough time to account for morning rush-hour traffic, add some more.

First thing to note is the setup of the hospital. We are used to driving around town and moving from building to building when we need to see different doctors. This is a one-stop-shop. You park once (which is free) and then all of the doctors are in the same building. This is very convenient but also leads to great teamwork which you’ll see below. We are used to Kosair (a fabulous children’s hospital in Louisville) but it seems to be more of an inpatient facility or a place to go when you need a procedure. I’m sure they have all that here, too, but it sure is nice having all of the office visits in the same location.

Our first visit was with the Neuro-Oncology team. They specialize in kids with brain tumors. How amazing is it to have a team like that!? We first met with a social worker who got some info from us. She made sure to ask us how we’re doing with all of this and whether we have a good support system. It was so nice to say we have an AMAZING support system. We are so loved. She asked us a bit how our hope was. We assured her that we have great hope and faith and how we’re able to really lean on God through all of this.

Shortly after that (no long waits at all today… which says a lot) Dr. Gauvain came to talk with us. She had a lot of questions about Havyn’s tumor (optic pathway glioma) and our current treatment. She agreed we seem to be doing the right thing with the chemo plan we’re on. We did learn a bit more about side-effects that we must have missed before. She said they typically prescribe pain killers of kids on this particular chemo. The vincristine can cause pretty bad jaw pain in the early weeks. We think Havyn may have had some pain but we weren’t really watching for it. While they give vincristine to their patients, she mentioned that the jury is out on whether that drug is all that beneficial. Some hospitals don’t use it and stick only to carboplatin. Interesting to know.

We talked with her more about the spinal lesion and the potential tethered cord. She was very curious about the lesion and said that in her experience a hamartoma (not a tumor) seems less likely. She asked us if we were seeing a neurosurgeon. (This story is about to get really cool.) We informed her that we were not lined up to see one. She said something like, “We have a neurosurgeon in our practice but he’s not usually in clinic on Wednesday but he was here earlier. In fact, I think he may still be here. Let me see if I can catch him.” She came back and told us that Dr. Limbrick was indeed there. He was supposed to be in surgery but it was canceled. She asked him if he could see us, he agreed, and was in the process of reviewing Havyn’s MRIs. A few minutes later, she walked out and in walked Dr. Limbrick. How amazing is that! I’m pretty sure neurosurgeons don’t typically sit around waiting for people to pop in and want an impromptu appointment. As we told him, that’s what we like to call a “God thing.”

Anyway, Dr. Limbrick shared several things with us. First, he is not convinced that the spinal lesion is a hamartoma. He thinks it is more likely a low-grade astrocytoma. Feel free to spend some time googling those, but the basic difference is that an astrocytoma is a tumor and a hamartoma is not. If it is an astrocytoma, the protocol would be to treat it with the same chemo she is already getting. He agreed that it is unnecessary and too risky to get a biopsy at this time. If it grows during the chemo, that would be a different story. He also talked quite a bit about tethered cord. While it’s possible her spinal cord is tethered, he said it is unlikely and hard to know for sure. It does extend a bit lower than typical, but not crazy far. And while tethered cord can lead to scoliosis, it would appear more like a long, gradual curve over the length of her spine. Her scoliosis is sharp and in a confined location. Because that location is also right where her lesion is, he thinks the scoliosis and lesion are connected somehow. He said he’s going to ask their radiologist to re-evaluate the MRIs to get his opinion on what the spinal lesion is.

We talked with him about several other things, but he left us with an overall assessment. While Havyn is the only NF patient we know well, they see all kinds of them. We don’t have much to compare to, but he said comparatively, Havyn is doing well. On the range of things, she is somewhere in the middle. Her symptoms and complications could be much, much worse – and they see plenty of those – but they also see plenty who have far fewer complications. We have a very high opinion of Dr. Limbrick but hope that he never has to cut on our girl’s back! Oh, and he seemed somewhat disappointed to find out that we weren’t scheduled to meet with Dr. Luhmann who he describes as the best bone-deformity surgeon in the world. We’ll meet with someone else in his practice tomorrow, but Dr. Limbrick insisted that Dr. Luhmann be brough up to speed. He promised us that he’d be in contact with him within five minutes after he walked out of the room and that they would together review Havyn’s images and records on Thursday.

By this point we were WAY late for our ophthalmology appointment, but the oncology team called over to let them know. Despite arriving so late, Dr. Hoekel got us back pretty quickly. He did great with Havyn. Getting a three-year old to cooperate for an eye exam has got to be one of the most difficult jobs on the planet. He used all of his bells and whistles – and even a magic duck! (You’ll have to ask Havyn how she made the duck quack!)

He did quite a bit of testing and started to notice some vision difficulties in Havyn’s right eye (the one with the tumor). To get a better look at things, he put dilating drops in and sent us away for 30 minutes. We went downstairs and had a delicious lunch in the cafe. Seriously – great hospital food. It was during this lunch that we were visited by a “little” insect. I’m pretty sure it was a service animal that had lost its master. Is there such a thing as a seeing-eye-cockroach? Everyone was pretty freaked out, needless to say. It was actually comical to watch the staff react to this little visitor.

When we arrived back to Dr. Hoekel’s office, he took time to examine her some more. Her vision tested as 20/25 in her left eye, but 20/40 in her right eye. This is not the kind of vision problem that glasses can fix – it’s vision loss. And here’s the crazy thing: it’s not necessarily caused by the big ol’ tumor wrapped around her optic nerve. In NF patients, the tumors often wrap around the nerve but don’t always do a lot of damage. He said there’s not a lot of evidence that the size of the tumor has much say on the degree of vision loss. He’s seen patients with massive tumors who have incredible vision. Every case is just so different. He said he had seen five optic glioma patients today… and each one different.

Quick thought break… this variance of symptoms seems to be a hallmark of NF-1. There is just not much predicting which complications you’ll deal with and how each will progress or respond to treatment. From tumors to cancer to learning disabilities to bone deformities to skin problems and on down the list. There is just so much to watch for. It is really amazing (and not in a good way) how one little gene mutation can cause so many issues… and how vast the differences are between patients. I don’t think we mentioned here, but a few weeks ago at church we met a woman in her forties who just recently found out she has NF-1… and has been relatively asymptomatic her entire life!

Okay back to Dr. Hoekel. So the vision loss is more likely caused by the brain which doesn’t want to work too hard. (I think Havyn’s brain takes after her daddy!) If it’s having a harder time processing images from one eye, it will just start to shut that one down and rely on the other unaffected eye. That’s probably what’s causing Havyn’s wandering eye and vision loss. The best treatment is more patching. When you patch the good eye, it forces the brain to use the other one, thereby strengthening its ability. We’d already been patching 1-2 hours per day and he told us to start patching “the daylights” out of it. Four hours or more if she’ll allow it. Hopefully this will lead to improvements in her vision.

He also said the swelling behind her optic nerve has gone down. Previously there was a very pronounced halo around the nerve from where the tumor was pressing. The reduction in swelling leads us to believe the tumor may be shrinking. There is concern, though, that there has been some atrophy. He’s not sure whether it’s a good thing or not that the swelling has gone down. He said it’s a wait-and-see game. He wants us to have her vision screened every three months to check her acuity. The hope is that it would improve each visit with the patching.

He asked at one point if we’d seen Dr. Gutmann yet. We haven’t. We’ll see him tomorrow. Here’s what he had to say about Dr. Gutmann: “I’m 48. I’ve been practicing here 24 years. Before I even got here I think Dr. Gutmann had written 50 papers on optic pathway gliomas. There is nobody better. A few of us travel once a year to a conference with leading doctors from around the world to discuss optic-pathway gliomas. Dr. Gutmann is constantly being stopped by all of the other doctors. They bring their most difficult cases to him and ask his opinion.” Okay… so I think we have this guy on too high a pedestal, but we feel extremely honored that he’ll be helping manage the care for our girl.

That’s about it. Is that enough? We had a great rest of our day. We went to the zoo despite Havyn’s protesting. She was much more interested in going back to HavenHouse to color and play games. But with the zoo a few blocks away and free, we made the trip anyway. We saw about a third of the animals and it was a lot of fun. Tammy even got her Dippin’ Dots. Then we retired to our home-away-from-home and made some art. We capped off our night with some St. Louis style pizza with some friends.

We’re looking forward to another long day tomorrow. And if you’re still reading this, you get rewarded with some photos!

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