Interview with a Family

This is an interview with a mother of a child who is living with O.I. I have known them my entire life, so this was the reason I got into this subject for my project. Jonathan just graduated from 6th grade, and is recovering from a fracture which put him in a wheelchair.

When did you first find out Jon had OI? What was yours and Mike's reaction?

Jonathan was first diagnosed with O.I. about 10 hours after he was born. When he was born one of the nurses noticed that his cry was a painful sounding cry. Because he was breech and sitting on his legs/criss-cross and because I had very little amniotic fluid (which is what lead them to deliver Jonathan 15 days early) they decided to X-ray his lower legs to see if something was hurt/broken? The X-ray caught a part of his upper leg which they noticed had been broken and was healing. That lead to them x-raying his whole body where they found he had 7 fractures. 6 were in various states of healing and his arm was newly broken. We of course were so sad when we first found out. All the information that you first find out about O.I. is very devastating. It's a very long list of horrible things that are typically found in someone who has O.I. On one hand we were sad, because we had just been giving such a life-altering diagnosis for Jonathan, but on the other hand, we were completely in love with this new little spirit that Heavenly Father had entrusted to us. We loved him from the moment we saw him and knew we would do anything for him to help him live a happy and productive life.  The first few months were very hard. Jonathan broke 7 more bones by the time he was 4 months old. I felt so helpless. I'm a person who fixes things when there is a problem. But there was no way to fix this, or undo this. It was something we had to come to terms with. And of course there were so many unknowns at that time. No one knew if he'd be able to sit or crawl or stand or walk. He seemed to be breaking his bones from absolutely nothing...so the situation looked very grim at the beginning. Here is one of my favorite pictures of Jonathan the day he was born.

  

What type of O.I. does Jonathan have?

When he was born we were told he has type III, which is the most severe form (other than type II which is lethal at birth). This was because of the number of in-utero fractures he had when he was born. When he was 2 months old and we had our first appointment at Shriners in LA the doctor there, who had quite a bit of O.I. experience, told us that his full body X-ray looked "too good be a type III, but not good enough to be type I"...so that makes him type IV, which basically is a catch-all for everything in between type III and I. The expert doctors that we see now don't like to use the types because there is such a variance between them. I've seen type III's who rarely break and type I's who break all the time. They, and we, prefer to use "mild," "moderate," or "severe". I call Jonathan "moderate".

What kind of research did you do when you discovered that Jonathan had O.I.?

The internet was not as vast 12 years ago as it is today! But we were still able to find a good deal of info on O.I. on the internet. Much of it was depressing, but that is where we first learned about Pamidronate treatments. And because of that initial research we did we were able to start Jonathan on this medicine, which was still experimental, when he was 8 weeks old! We lived in Provo, UT at the time and the drug was only administered in Los Angeles, CA and Montreal, Canada. So we flew to LA for his first treatment. My mom, who was an absolute angel to us, contacted anyone she could find online who had a child with O.I. She would write to them and they would share letters of encouragement and support. My mom would print these off and bring them to me. They helped me feel like I wasn't alone. Our continued research led us to Shriners Hospital in Montreal where the worlds top O.I. doctors were located. We flew there when Jonathan was 3 to be evaluated. One of the doctors there eventually started an O.I. clinic in Omaha, Nebraska which is where Jonathan started going when he was 5. The care and experience we get there is amazing!

How many times have you had to bring Jon to the hospital with breaks?

Wow...I don't even know. He's had over 40 fractures. So he's had A LOT of trips to the hospital, doctors office, Omaha, etc! When Jonathan was 13 months old he had his first rodding surgery, which is were metal rods are placed inside the length of his femur bones. This made his femurs straighter, so they would break less. It also acted as an internal splint so that when he broke his femur he didn't need to have a cast. This was a huge blessing for us! Jonathan had a handful of femur fractures before he had these rods placed and a broken femur always meant a spica cast. A spica cast goes from your ribs to your toes. It's basically a half body cast and it is HORRID! However, the rods have a high complication rate and sometimes bend when his leg breaks. Jonathan has had 9 rodding surgeries in all to place or replace the rods in his legs.  Jonathan also goes to the hospital often for his Pamidronate infusions. These were every 8 weeks as a baby...and now every 4 months for a 2-3 day IV infusion. He's grown up in hospitals and has lots of wonderful nurses and doctors who have made the hospital a fun place for him...for the most part.

Has Jon needed services at school? What kinds of services?

Jonathan has had an IEP (Individualized Education Plan) since he was in preschool. Some of the services we've had are a one-on-one aide to help keep him safe at school. He has also had 2 one-hour water therapy sessions a week...which have been wonderful for him. Some things that he has in place now is an extra set of books to keep at home, permission to leave class early or late if needed to avoid the mad rush in the hallways. He has adaptive PE, meaning he can do different things if he needs to for PE...though he prefers to just do what the class is doing whenever possible.

What has your experience been with the professionals you've come in contact with?

We learned pretty early on that most doctors think they now about O.I., but really only a handful REALLY know what they are talking about. That is the main reason we fly to Omaha for Jonathan's clinic visit (a once a year major check-up) and for any surgeries he needs. The peace of mind that comes from having a skilled surgeon who works on probably more than a hundred O.I. kids versus the local doctor who has maybe seen a few cases in their entire practice of medicine. I seen far too many families who have been given misinformation by well meaning doctors that have had profoundly negative affects on their child's well being. We feel so blessed to have gotten in touch with excellent doctors early on. 

How has O.I. affected your other children?

I'm not sure...because its' such a normal part of our life. Jonathan is the oldest of 4 boys, so his brothers have grown up knowing they have to be careful around him.It's just second nature. For example we are extremely vigilant about wiping up any spills on the floor because Jonathan slipped once and broke his leg and needed surgery. So our kids just see us wiping up ANYTHING that has spilled. All of our boys, by about 18 months old, just start wiping up their own spills. It's pretty funny to see it, but they somehow just realize that's what we do in our house. I am also amazed to watch Joanthan's brothers play with him. They know how to rough house with Jonathan but also have this innate understanding of not letting things go too far. I think my boys are pretty nice kids, maybe because pounding on your brother, hitting, kicking, fighting, etc...was just never an option in our house. The boys have always treated each other pretty kindly. They are very willing to be helpful to Jonathan when he is hurt. I remember one moment that was particularly touching, Jonathan (who was 11 at the time) had broken his leg while Andrew (8 yrs) was not home. When Andrew got home and I told him that Jonathan had broken his leg he just burst into tears. I think he knew the pain and the frustration that Jonathan feels when this happens and he was feeling the same frustration for him too. My boys are great friends...whenever it's the weekend or summer they all sleep together in the same room. And of course they are all in Jonathan's room, the smallest room in the house! Because Jonathan's room is downstairs (the other boys are upstairs) as it's safer for him not to be going up and down stairs. All in all, I think it's made my boys much more aware that sometimes people have disabilities and it's just a part of life...not that big of a deal.