St. Louis Follow-up

What a whirlwind these trips are!  Doctors, doctors and more doctors. It is a bittersweet experience.  We love our one-on-one (I guess it’s actually two-on-one?) time with Havyn but hate that it has to be under the circumstances. We really got to see her personality blossom this trip. What a sweet, social, smart, funny, and amazing young lady Havyn is. If you have ever watched any of our Facebook live videos with her you have seen her charm. She is such a lovely little girl.

The next few paragraphs will detail what we spoke about with each of the doctors. If you want to get straight to the results, be sure to jump down to TLDR (too long don’t read) summary at the end. Oh and some pics from our trip, too!

We again stayed at the HavenHouse which is a great place in St. Louis for out-of-towners in for medical treatment. It’s very affordable and welcoming. This is where Havyn really shined. Watching her interact with the other families was a real treat. One afternoon I wasn’t feeling so well so I laid down for a nap. Steve took Havyn to the store to buy pickles and cheese that she was craving. They brought it with them to dinner and Havyn shared with the other kids in the room! Later that night she found another sweet girl to share her chocolate cake with!

Our day on Thursday started out with Dr. Kim the endocrinologist.  This was Havyn’s first time seeing Dr. Kim and she was great.  We hadn’t shared before this trip, but in the last month or so Havyn began showing signs of early puberty… don’t even get me started on this. She is 4!!! I noticed some changes in her moods and inability to control them as well as some unnamed other things. 🙁  Precocious puberty is somewhat common in NF1 kiddos with the optic pathway glioma tumor.

Ahead of this trip, I called St. Louis to let them know we were suspecting early puberty. They ordered blood work which we had done in Louisville before we left. Total side story, but wanted to brag on Steve a second… So a few weeks ago I was out of town for a personal retreat. While I was gone Steve bravely took all 4 kids by himself to Norton hospital for the girls to get labs drawn and for Amelia to have a stomach x-ray.  (Amelia is showing signs of a food allergy.)

Dr. Kim reviewed the results with us and everything looked normal for Havyn’s age. She told us, however, that the labs should have been drawn in the morning because one of the hormones tapers off through the day. (DUH, I should have remembered that from pregnancy tests!) Since the original labs were drawn in the evening, more blood work was ordered for Havyn. In addition, they ordered a bone age test (hand x-ray).  The bone age test helps doctors estimate the maturity of the child’s skeletal system. The hormone levels can be tricky to read but sometimes early puberty will show itself by advanced bone growth. Steve and I found this test to be fascinating! While Dr. Kim explained how the hormone levels worked and how it causes precocious puberty I was in awe of our amazing Creator!!! She spoke of how one hormone triggers another and that one triggers a certain reaction, etc. The complexity of our bodies is just an awesome testament of a very detailed God!!!

The results came in from the tests this week. The blood work confirmed that she is in fact experiencing precocious puberty.  Something called the Luteinizing Hormone is elevated. She’s at .2 and a normal reading should be less than .1.  The nurse we spoke with said the numbers may not sound like much of a difference, but they are. Her bone age x ray came back normal, so that is a plus!  We are still figuring out the treatment plan. She needs a specific type of hormone blocker. This will regulate her hormones and hold off puberty as long as she is taking it. It’s a very safe and successful treatment. Without it Havyn may enter full-blown puberty by age 6 and would end up with severely stunted growth. (She needs all the help she can get with her shorty parents!) The main options are a quarterly injection or a small implant replaced every 16 months. We’re leaning toward the implant.

The next stop in St. Louis was with Dr. King the oncologist.  She talked to us about how Havyn tolerated chemo and said she looked great.  She met with her whole team that afternoon to review the last 2 MRI’s and later called us to confirm what we already knew that the tumors looked stable.  She did mention that if her tumors began to grow again that they would use a different combination of chemo to treat those and not what Havyn had before. Several years ago they would have continued to use vincristine and carbo but now they have more options to use that have great results. There’s about a 50/50 shot that her tumors will grow again.

Then we saw Dr. Gutmann the NF-1 neurologist. We’ve said it before but this guy is gold. He’s so smart and has dedicated so much time and energy to this stupid disorder. He also has incredible bedside manner and is so sweet with Havyn. He and his team spent quite a bit of time looking Havyn over and talking with us. He has been watching her for learning disorders. A year ago the testing they did placed Havyn with a potential ADHD diagnosis. This year they let us know that she’s possibly on the autism spectrum. This is something we had suspected but it’s still not exactly welcome news. We will have her meet with a neuro-psychologist here in Louisville for further evaluation before she starts Kindergarten in the Fall.

Dr. Gutmann also confirmed that three bumps we’ve found on Havyn’s skin are small neurofibromas. These tumors are kind of the calling card for NF-1. We had been confused because usually people don’t start seeing them until puberty. But it’s all making sense now.

Dr. Gutmann’s team also invited us back to a NF symposium later in the year. We hope to be able to attend and hear some of the latest news and learn more about what is being done around the world to combat NF.

Our final visit was supposed to be with her ophthalmologist, Dr. Hoekel. He was sick so we saw one of his partners Dr. Timmerman. She did a great job with Havyn. She confirmed that Havyn is seeing great and she doesn’t see any worsening of the tumor from the back of the eye. And she reminded us a ton of Aunt Jamie!

Here’s how you can pray:

Selfish prayer – That we all are healthy for our Disney Make-a-Wish trip.  (6 days away) That we have a relaxing and magical time experiencing Disney through the Make-a-Wish perspective.  

And for our 2 year-old, Arlie. He is seriously strong willed and testing us in ways we haven’t been tested as parents of 3 older kids.  

Pray that Havyn’s tumors remain stable! She’ll have her next MRI on April 13.

For NO more tumors!!!!!

Pray for upcoming testing to reveal correctly if she has any learning challenges and that the right school programs would be set in place for her.

Prayers that God would continue to open our eyes to other hurting families and help them to see Jesus in their own journey!!!

Prayers for these doctors that are researching Havyns genetic disorder!!!

Friends Havyns made while staying at the Haven House. (Both families dealing with kids born with club feet and other medical stuff. One is in leg casts still and the other one is on the other side and doing well. Havyn has an instant bond with these kids in casts!)

Thanks for reading and praying. Love you all!!!

TLDR: Trip was good. Havyn has started puberty and needs to receive a hormone blocker. Brain and spinal tumors are stable. She is being watched for ADHD and autism spectrum. Her eyesight is great.

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