Aidan's Trips to Shriner's Hospital In St. Louis

It was in 2006 that we made our first trip out to St. Louis to meet Dr. Whyte and his research team and to undergo testing. Aidan's week was filled with exams, blood tests, urine collection studies, dietary restrictions, doctor's conferences, Physical therapy/ Occupational therapy evaluations, x-rays, photographs, video clips taken, and a dentist visit. We had two big opportunities to meet with the research team in a "conference like" setting" in which questions were asked of us and information presented to us from them researching him and from a compilation of all of our test results, as we had to undergo tests as well. We learned that Aidan's Alkaline Phosphatase level continued to remain abnormally low, though indicative for the disease that Aidan has. They were somewhat surprised that Aidan physically was doing as well as he was given his low alkaline phosphatase levels, as usually there is a direct correlation of physical deficits to alkaline phosphatase level. Aidan's body was found to be compensating somehow for this disease. The doctors remained puzzled as to why and assured us they would look to find more solid answers through more research at our return visit in two years. Through x-rays and a skeletal survey the doctors were able to see subtle changes in Aidan's wrist bones- an outward curvature, skull, and leg joints, though nothing at that point debilitating. His skeletal survey scores resulted with a "z" score to be within normal range for someone of his age. We were told that his "soft spot" appeared to remain unclosed due to the facts that in the skull of children with Hypophosphatasia, individual membranous bones may calcify only at their centers, making it appear that areas of incompletely ossified bone have cranial sutures that are widely separated, when in fact they are functionally closed. His urine studies showed that he was spilling more calcium than necessary, but at this time we were told to not alter his diet, as he begins to get slightly older he will have a higher calcium requirement anyway and fall into his slightly higher than normal calcium intake. Aidan's dentist visit was with a phenomenal dentist that was very knowledgeable about kids with Aidan's condition and offered possibilities to help him be as "normal" as possible as he continues to battle the dental problems of this illness in the upcoming years. Aidan's dental future at this point was thought to have to consist of possibly anchoring his two sets of molars together in the back of this mouth to preserve their "life" as long as possible or to get him a specially fitted "baby denture" to allow him to have "front teeth" to help him more cosmetically with speech and "fitting in" with other children his age. We were given a name of a dentist to follow up with in our area that is familiar with Hypophosphatasia, the needs of the children with this illness, and works closely with this dentist we saw via phone and email communication. Aidan passed physical therapy and occupational therapy with flying colors. The photographs were tough because he had to stand in his underwear for the photographer, and what two-year-old thinks it's acceptable to be photographed in your underoos by a stranger? At least we knew we were teaching him right! During our closing conference with the "research team" and the infamous Dr. Whyte we learned that Aidan would have to be re-x-rayed in 2007 around the same time and if everything came back without changes, we would not have to return to St. Louis until 2008, but if they did notice changes it was deemed we would have to go back in 2007. It was also during this conference that we learned that they were hopeful to have a treatment developed and available for this illness on our return visit.

In 2007, Aidan underwent a repeated series of x-rays. The films were then forwarded to St. Louis to Dr. Whyte and his research team for review. They showed minor changes that most of the time would not even be picked up by a radiologist without an experienced eye in this field. The films were taken of Aidan's skull, wrist/hand, and knee. Some Hypomineralization was evident as well as rachitic changes, but nothing terribly pressing. With this news, it was a blessing. We were not required to go to St. Louis until the following summer in 2008.

The summer of 2008 we traveled back to St. Louis for another week of exams, bloodwork, urine tests, x-rays, and meetings with the research team and Dr. Whyte. Aidan was found to have increased arthritic changes in his fingers, wrists, and other joints, but other than that not much had changed. We were told on this visit that we should expect the worst to come. It was thought that once Aidan began going through puberty and putting on additional weight, his bones could become stressed and that is when we would begin to notice and experience the varying bony detriments of this disease and his impaired bone mineralization, i.e. bone weakness, stress fractures, hypomineralization, rachitic changes, incomplete vertebrate ossification, sometimes bony spurs, amongst other changes and symptoms like impaired physical endurance. Disappointingly we were not able to follow up with the dentist on this visit as it wasn't scheduled into the weekly plans. During this visit the family was notified of the progress of the research team of working on clinical trials for enzyme replacement therapy.

In July of 2011 Aidan returned to Shriner's Hospital in St. Louis again. Aidan's first day at the hospital begins a specific diet (only certain food selections are given and everything is weighed). No cheating by having something outside of the diet and everything given must be eaten or accounted for. Water is a free item, you may have as much as you would like. Mom has already submitted paperwork and met with the dietician so that the amount of food Aidan is given compares to what he would normally eat at home. Adjustments to quantity can be made if needed. There are limited food selections, but a fair choice; you may find your child repeating a selection during their stay. You are also allowed four snacks a day, at any time, but you must ask for them. Rice Krispy treats and popsicles were Aidan's choice of snacks. A 3 day urine collection also begins. Temperature and blood pressure monitoring starts. Tests for today consist of x-rays and a bone density scan. The doctors will be looking for any changes in his bone structure. Aidan also met with one of the doctors to have his measurements taken, height, weight, how far he can stretch and bend, reflexes, dexterity, and strength. The doctor will also review Aidan's medical history to see if there are any changes. The doctor also noticed that Aidan has more teeth that are beginning to peek through. Numbing cream to prepare for blood tests and monitor at night (one night only) to see how he sleeps. The rest of the day is filled with RT (recreational therapy) doing crafts, playing games, and meeting new friends. Today's special event at RT was a scavenger hunt. Secret, it didn't matter where you finished, all the kids got to pick a prize once they were done. RT is a huge game room complete with a playhouse, games, wii, play station, pin ball machines, basketball, air hockey, pool table and a reading area. You may also ask permission to make an unscheduled craft.
Day 2: Blood drawn this morning. Should your child be afraid of having their blood drawn the hospital will send someone trained in distracting them while the blood is being drawn. Initial meeting with the research team to discuss any concerns Aidan's mom or the doctors may have and any questions that need to be addressed so far. The number of members on the research team has increased and can be a little overwhelming when you first walk into the room. Don't let the amount of specialists in the room intimidate you, they each specialize in a particular area and are there to help your child. The doctors are very patient and do not rush you. Take advantage of their expertise and ask any questions you may have, do not hold back. If you do not understand the information or answer presented to you, ask for clarification, the doctor will reword it until you do understand. Even though the setting is overwhelming, it is a relaxed atmosphere and the staff will do everything they can to help you feel comfortable. You will also get to view your child's x-rays. Meeting with Occupational Therapy. OC also takes measurements more in line with dexterity, for instance how far you can spread your fingers. They will also do different strength tests. Aidan then met with Physical Therapy for a 6 minute duration walk, relay test and jump test. We were advised most kids Aidan's age with HPP will do an 11. something on their relay test, Aidan did a 9.6. Aidan had a concern in this area since he feels he doesn't keep up with the kids at school on the mile run. Hypophosphatasia children will tire more easily than a normal child. The craft at RT today was a windsock. Tonight was also pet therapy, something all the kids look forward to. Nina, was a wonderful dog, said hello to everyone, gave paw, jumped, retrieved things and played catch with the children. It is amazing to watch these dogs around children with wheel chairs; the dogs know just where to approach the child. It was also Bingo night at recreational therapy and Aidan was one of the Bingo callers. Everyone gets a prize in the end.
Day 3: Normally, Aidan's hospital stay would be a week (5 days) but since we went on a holiday weekend it was only 4. Still on 3 day urine collection which at the end of the day, will turn into three concentrated specimens. After dinner Aidan will not have anything to drink or eat until urine is collected 3 times.
Day 4: Aidan finished his concentrated urine test this morning. Diet returns to normal, he can have want he wants for breakfast and lunch. Exit consultation with the research team. Dr. Whyte advises us that Aidan is doing well. Something in his body is still combating the effects of Hypophosphatasia. His calcium was a little high, but his hospital diet was more calcium than he eats at home. Prognosis is good, but not definite since we need to wait and see what happens as kids in Aidan's age group age. The doctor's are hopeful that as Aidan's grown up teeth begin to come in they will take root and remain in place, for Aidan this will be huge since he has never had a lot of teeth. He will finally be able to experience foods that he has not been able to eat before. Aidan does not have to return to Shriner's until the year 2014.

We did the 2011 entry a little different than previous years. This is our third time at St. Louis and normally it is a pick up from the airport, to the Haven House or hotel, to the hospital and back to the airport. Mom stays at the hospital and dad or mom mom take a shuttle or taxi back and forth. This year we were a little more knowledgeable about the trip and we wanted to share some things with you for those who may be going to Shriner's Hospital in St. Louis in the future. We added some information about Recreational Therapy and some of the activities your child can participate in. You can also sign out movies to watch. We know it is tough taking your child to a hospital for a week; this gives them some play time and relief. Parents can also access the internet and there is a computer set up in the parents lounge if you do not have a laptop. You will need to get the password from RT. The Shriner's are wonderful about shuttling you back and forth from the Haven House, you do have to pre-schedule and you will need to return at a certain time. They will also hold your dinner in the kitchen for you, if you pre-arrange it. There is a cafeteria in the hospital with a variety of food and the price is really economic. We seemed to always miss the times that it was open. This year we learned that there is a hotel within walking distance, keep in mind it is not next door, but it is not a bad walk. Hilton St. Louis Frontenac is located right down the road on the same side as the hospital. They have two restaurants and of course, room service. They will also pick you up at the airport and return you there as well. They will shuttle you anywhere within an 8 mile radius. They will advise you that they leave on top of the hour. It really depends on how busy they are. They also shuttle you to the Metro Station located at Richmond Heights. This was our first time using the metro. If you stay a minimum of two nights and let the hotel know you are with Shriner's you will receive a special rate. There is a grocery store with a food court section directly across the street. Close by are a few malls, one you will pass going to the hospital. You will also pass a few places to eat along the way. The Science Center in St. Louis is huge. The main building is free, although there is a fee for some things inside should you choose to do them. You can also ride a trolley through this area, it is a park and there are numerous things to do. We stopped at a restaurant on a lake called the Boathouse. The St. Louis Zoo is beautiful, lots of intriguing animals. This is free also; once again there are shows or exhibits they do charge a small fee for. We also didn't know you could get a pass out from the hospital, with your doctor's approval you may take your child out for a limited time. They will equip you with everything you need in regards to urine collection, and allowed snacks. You will have to make sure you have a clear schedule as far as testing and meeting with doctors during the time you want to pass out. We took a pass out along with Aidan's roommate and parents and took the boys to the Arch. The cost of this was $10.00 per adult and $5.00 per child. It is an experience you will never forget, whether you just visit or ride up in it.

Aidan will be returning to Shriner's Hospital in St. Louis in the summer of 2014. We will keep you updated while we are there and we will post his experience when we return.