When I was born in 1968, my parents assumed everything was fine. They took me home and being her 1st child, my mom thought everything was normal. But, I cried all the time; especially when being held. My grandma told my mom that she needed to take me to the doctor. He told my mom that I had pneumonia & that I had water on my head or brain, which was causing it to swell.
They sent us to Little Rock, Arkansas. I was diagnosed at around the age of 3 months at either Arkansas Children’s Hospital or the University Hospital. I’m not sure which. I was seen throughout my childhood at both places. Mom can’t remember everything they did to me, but she says I spent several months there. I had fractured ribs, which probably happened during birth. My mom and dad were not allowed to hold me, because the nurses and doctors were afraid they would break me. So, we missed out on a lot of our mommy/baby bonding time. They did Craniosynostosis surgery on me to open up my soft spot; because it had closed early. That led to me having to have surgery again when I was 10 years old, where they put a plastic plate in my head to protect it. Since the soft spot was opened surgically, it wouldn’t reclose on it’s own. What prompted them that this needed to be done was an incident on the school bus. A high school kid dropped a stack of books on the top of my head causing me to throw up. They said it could have been more serious, since there was nothing there to protect my head.
My mom says I was a pretty happy child. I loved to sing and talk to people everywhere we went. I never crawled, only scooted on my bottom. I was very small for my age. I’m still only 4′ 10″. When I was a toddler, I would often step down too hard or my legs would buckle underneath me and I would cry, but then I would get back up and go on. I think I probably had a lot of fractures in my childhood, but we didn’t know it. I lost my baby teeth when I was 2 years-old, when my cousin who was also 2 kicked me in the mouth. My teeth were always bad. They were long and my gums were really bad. I was told I had Pyorrhea. When I was 18, I had all my teeth pulled and got dentures. As a child, I was slower than the other kids as far as running and playing the rougher games and stuff. But, I didn’t really miss it. I wasn’t an athletic kid anyway. I did like to go walking in the woods by our house picking wild flowers, playing in the creek, and I still played outside a lot in the yard. I jumped rope and ran, but not fast. I never learned how to swim or ride a bike.
My mom was very overprotective of me. I was allowed to skip P.E., in high school, and I was allowed to leave my classes 5 minutes early, so I would have time to get to my locker and make it to my next class before the bell would ring. This would also allow me to avoid the crowded hallways with all the pushing and shoving. When I was 6 years-old, I had a surgery to remove a benign cyst from one of my eyes. I do not know if that was related to HPP or not. Other than the rib fractures, we really never knew that I had any fractures. Mom always just said I was probably having growing pains. Nothing was ever painful enough, I guess to have it checked out. At 14, though, I had a seizure, which also was most likely related to HPP and high B6. We didn’t know to look for that then, but I’m guessing if we had, it would have been high. When I woke up in the hospital, they said I had old partially healed fractures in both tibias. We had no idea how they had happened.
I had our daughter when I was 22 and our son when I was 26. Probably around the age of 24, I started getting repeat fractures in my tibias, fibulas, hands, fingers, feet, toes and ribs. Because they didn’t really know what else to do with me, they would just cast me or tell me to wrap the less severe fractures. They were afraid if they ever tried to straighten out and rod any fractures, that they would never heal. I think the lack of the rods for stability, sped up the bowing and made my legs, especially, even more deformed. Also, in my early 20s, I had a period of time that lasted about 3 months or so where I had extreme pain all over my body to the point of tears. I saw a doctor in Little Rock, who told me I had Rheumatoid Arthritis and that due to the HPP, I would eventually be on kidney dialysis. I still do not have any trouble with my kidneys, except that they sometimes work too well. Road trips used to be a nightmare, with bathroom stops. That has actually gotten a bit better since Strensiq™. I do know that kidneys are an issue for some with HPP. As far as the Rheumatoid Arthritis, I’ve had several doctors tell me since then, that I do not have it. So, I’m not sure why that doctor though that I did.
Then in October of 2015, my husband had a business trip to New Jersey, which we decided to combine with a day of sight seeing in New York, and then after he finished working, we planned to go to Jacksonville, FL for a karate seminar that my husband likes to attend every year. Our very 1st morning, we were about to cross on the ferry to see the Statue of Liberty. I stepped into the aisle of the tour bus, not realizing there was a step down. I grabbed the pole, twisting completely around falling and breaking my leg completely. You could tell through my jeans that it was all the way broken. It was the worst pain I had ever felt in my life and I was really scared. I didn’t know what they would do. They had no choice but to straighten my leg and rod it. They actually didn’t officially “know” what to do with me. They ended up having to use a pediatric rod, since they couldn’t fit the adult rod down into the marrow. I think that’s how they explained it. Usually an HPP patient keeps their rods for added stability to help prevent more broken bones. But, in this case, they said I needed to have it removed after a year. It’s been a year and 6 months and I can’t find anyone to remove it. They have since contacted me several times and gotten information and new x-rays for a report they are writing for some medical journal to discuss the procedure they used. I was very scared that I would never heal and would be even more stuck in my scooter than before. For the 1st couple of months, healing was happening very slowly. Then on December 2, 2015, I started Strensiq™. Everything started changing very quickly. My leg started healing quickly. I had old fractures that were healing. I had broken my arm many years earlier and it still burned from time to time and especially in the early part of healing from this leg rodding surgery. I was putting a lot of weight on my forearms getting up and down. After a couple of months on Strensiq™, my arm stopped burning and hasn’t burned since then. It has healed. I feel better both mentally and physically.
Some of the things that have changed with the medicine are, I can now stand on my tip toes. I can look up into the cabinet when I get something out, instead of bowing my head. I can dance a little. You don’t want to see a demonstration of that. The real biggie is that before Strensiq™, I couldn’t even take a single step without holding on to something. It had gotten to where even using a cane or a walker was too much work and very painful. Now I can walk without a cane or walker either one for short distances. For example, I will use my cane when we go out to eat or hold onto my husband’s arm. I still use my scooter a lot, but I can get around in my house without the scooter, if I choose. I still use it a lot around the house, though, if I’m going to be up for a while, which is most of the time. But, during the night, if I go to the bathroom, I don’t always use the scooter. Or, if I’m on the couch watching TV and need something from the kitchen, I’ll just go get it without the scooter. That is, if I don’t say “Honey, will you go get me this or make me a glass of tea” or whatever.
Another big change since starting Strensiq™ is my pain level. I had gotten to a point of pretty much constant pain. I was taking Advil, Tylenol, Aleve or whatever over the counter meds I could alternate pretty much every day; sometimes several times a day or taking larger than recommended doses. Sometimes it wouldn’t touch the pain and it never took it away completely. Being in pain all the time, changes who you are. I’m a much happier person now. My pain level is nonexistent compared to what I was going through before Strensiq™. I have been having some problems with my right leg, when I walk on it for not very long at all. It hurts where my old fractures were in that leg. It is still badly deformed just like the other one was before I broke it and had it fixed.
Noone where I live wants to do my rodding surgery or remove the rod from the previous surgery. So, I have an appointment in June for a consultation with a surgeon in Arizona to discuss him doing a rodding on the right leg. This surgeon has done this type of surgery many times. He has a long-time HPP patient, who is a friend of mine. He has performed many different “hardware” surgeries on her, and several other HPP patients have been operated on by him, as well. I am very excited. I know that with this surgery and Strensiq™, I am only going to get stronger and be able to walk better & better with time.
My husband & I are expecting our 1st grandchild in October and we are so excited. I used to be resigned to the fact that when I had a grandbaby, the only way I would get to hold them is if I sat down and someone placed the baby in my arms laying down with a pillow to help hold them up. I recently picked up my nephew’s 3 month-old and lifted him up in front of me with no trouble. My arms are stronger now. Because of Strensiq™, I will be able to enjoy being a Nana more. Of course, we are praying that our grandbabies do not have HPP, but it is comforting to know that if it happens, there is treatment now. We have hope. I believe if Strensiq™ had been available to me as a child, I wouldn’t have ever gotten to the point of needing a scooter. This gives me hope for our grandbabies and all other children born with HPP in the future. Strensiq™ has completely changed my life, my hopes and plans for the future.