Living with HPP: A St. Louis Shriner’s Trip

Hi, my name is Danielle and my children Grayson (8yrs) and Briley (4yrs) have odonto/mild childhood Hypophosphatasia. We live in Austin, TX. My family and I just finished our third trip to St. Louis Shriner’s Research Center in July 2018. Our first trip was in 2011 to the old hospital where Grayson was diagnosed when he was 19 months old. We went back to the brand new hospital in July of 2015 with Grayson and Briley.

As I think back to that first trip all the emotions and questions flood back into my mind. Grayson, at 16 months, had just lost his first teeth and after a few months of searching for answers we came across what we suspected was his diagnosis. Hypophosphatasia. More researching led me to contact Dr. Whyte at Shriner’s. A phone call over my lunch break confirmed what I didn’t want to hear. Dr. Whyte, too, suspected Grayson had HPP and suggested we visit to help determine the severity. Luckily, they were able to get us a date to visit just a month or two later. So that was that. St. Louis here we come.

But, we didn’t know much of what to expect, pack, how the week would go, or what knowledge we would leave knowing or not knowing. This is what brings me to this blog. As I have continued along this journey I have encountered many parents facing these same questions, thoughts, and emotions. I hope that I can help those of you traveling to Shriner’s or those in the process of deciding to go or not go.

For us the decision was easy, but for others it may not be. We had the financial power to do this trip. If you do not, speak with Shriner’s or NORD (National Organization for Rare Diseases) to see if you can receive a grant for travel. For my husband and I, we knew we wanted to gain as much knowledge about our children’s care from the best doctors out there. At the time, and maybe still so today, Dr. Michael Whyte, and his team were the best to give us this. I also learned that all tests ran would be covered by Shriner’s without using our insurance or personal money. I also love the fact that when we visit Shriner’s we are playing an important role in research. There is nothing more I want in this life than for someone to find a cure for this disease! If I can’t be the one to find it I will do anything I can to help someone else discover. And the quicker the better!

Now, onto what to expect. Let’s begin with travel arrangements. Flying into St. Louis should be fairly easy to figure out. We have driven from Austin, Tx and flown. We have rented a car and Ubered it. An Uber from the airport to Shriner’s should be $20-$30. The drive is about 25 minutes. We arrive Sunday or earlier because you will need to check into the hospital at 8am Monday morning. This last trip we stayed Sunday night at Parkway Hotel. It was located a mile or so from Shriner’s and was lovely. Each room has a refrigerator which is a must if you are traveling with Strensiq. The kids loved the warm chocolate chip cookies in the evening. They have free warm breakfast, however, your child will need to arrive at the hospital fasting from the night before so they can’t eat. The hotel also provides a shuttle which dropped us off and picked us back up at Shriner’s.

As far as packing, overall comfortable clothes depending on the weather. Bring a pair of slippers or flip flops for everyone to wear about the hospital. Bring your own toiletries. They have a small box, but pretty picked over last time we were there. Bring some snacks for the parents and a reusable water bottle for all. If you have room bring a pillow and blanket. Maybe even a towel…the hospital towels are barely bigger than a hand towel, thin, and rough. Don’t get me wrong, we got by this last trip without those things, but if I were driving I would bring them!

The Shriner’s Research Center is a top notch, state of the art facility. It was built in 2015. The Research Center is located on the 3rd floor. Each family gets one room to share with your own bathroom. They have pack and plays, high chairs, baby bath tubs, and I am sure anything else you might need. The beds are hospital style beds so don’t expect to be super comfy. As stated above, if I was driving I would bring my own pillows and blankets for sure! The rooms have a TV with cable and Playstation. You can check out movies from the Recreation Department. They have tons for all ages! The bathrooms have a shower, but bring your own toiletries.

There is a family dining/kitchen area with a fridge/freezer, microwave, sink, and even some community snacks stocked in the cabinets. You can purchase food from the downstairs cafe or bring in your own. For breakfast, we walked to Vincent Van Doughnut. They are known for their creative donuts, but their coffee and quiche were fantastic too. It is just a short mile away and a safe walk. Trust me, it feels good to get some fresh air and sunshine. For lunch/dinner we ordered from Sauce on the Side (which is also right next to the donut shop so you could walk there). They delivered to the hospital. Their salads and calzones are delicious and big enough to share or have left-overs.

The Research Center also has a living room/play area with a TV, toys, books, board games, and my son’s favorite…air hockey. The Recreation Room is every child’s favorite part of the stay. They have amazing staff and volunteers who entertain the kids. They will post a schedule and this is where the kids hang out every minute they are not sleeping or doing tests. They have craft time, game time, Bingo, Pet Therapy, free play, and much more.

Monday: Check-in at 8am. This was fast and we were up in the research center starting the kids on their research diet with breakfast. They will stay on the research diet the entire stay. This will try to replicate their calcium intake at home. The kitchen staff will be in contact with you prior to your stay. They have lots of kid friendly options and your kid(s) will be well fed!
While are kids were busy eating we spoke with nurse and answered questions about family history updates and how kids were doing overall. After the kids got some food in their bellies they begin to do their initial check-up. Just think of this part like you are going in for their yearly physical. They will also start their 24 hr urine collection. They will explain this, but just tell your kids the toilet is off limits. All urine goes in the jug and you get a new jug every 24 hours.
You will go into an exam room and meet with Dr. Gottesman. We love his friendly bedside manner. He is great with kids and truly has a passion for treating kids with HPP. I tend to discuss a lot of my questions with him during this visit.

The rest of the tests for your stay depend on the hospitals scheduling for that week. Briley did her PT and OT Monday, but Grayson’s were Tuesday and Wednesday. Over the course of the next few days they will schedule Physical Therapy Evaluations, Occupational Therapy, Bone Dexa scans, x-rays, pictures, and blood work.

Tuesday: Most likely your child will do their blood work first thing in the morning before they eat. You can ask for someone in the Rec room or a extra nurse to come in and do distractions if you think that will help. We didn’t have much planned Tuesday morning so we were told we could leave and be back after lunch. We took this opportunity and went to the St. Louis Zoo. This is one of the best zoos in the nation! A must see! The zoo is free, but you do pay to park. It is about a 10 minute drive. We took an Uber there and back. We spent 3 hours. It was hot so we wouldn’t have lasted any longer, but we didn’t hit it all. The kitchen packed up our kid’s lunches for us. When we came back we did bone scans, x-rays, and PT for Grayson.

Wednesday: You will meet with Dr. Whyte and his team (10-15 people) on. I believe most of these happen between 10 AM and 12 noon. We were in there for less than 30 minutes. They will go over results and findings, answer questions, ask questions, and give their opinion for care. Come loaded with a pad of paper and write down your questions ahead of time. I usually write them as I come up with them leading up to the trip and then add to it during the visit if I think of any more. If this is your first trip there they will want as much family history as you can give them in regards anyone displaying possible signs and symptoms of HPP.

When we finished our meeting with the docs we did our extreme CAT scans on our wrists and ankles. This is one of 12 machines in the nation and is cutting edge technology. It gives a more detailed bone density report, but just in a specific area. We went to photography later for pictures. Bring shorter length shorts for this. For my shy guy, this was his least favorite part of the trip because he had to take off shirt and roll up his shorts. They will do pictures from different angles and want to take pictures of site reactions and teeth as well.

Wednesday night the Shriner’s sponsor pizza and salad for the families and staff. This was very nice gesture.

You are done each day no later than 4:30 pm so you could venture out for an evening excursion. Each night the Recreation Center has a volunteer or staff member who does an hour long activity. These were fun so I am not sure my kids would have wanted to miss any of these. Bingo with awesome prizes, a scavenger hunt in wheelchairs, and board game of choice night.