Comeback! Training week 3

As many of you know, Sienna is completing a full marathon between now and the Boston Marathon on April 21st. The Boston Marathon is being run in her honor by Kuber, as part of Genzyme’s Running For Rare Diseases program. Each week I’m sharing our training progress and sharing the story of another FOP friend. If you are new to this series on our training, you can catch up here:
https://raremom.com/2014/02/25/siennas-training-for-a-marathon-and-bringing-her-fop-friends/

March 8th:
The weather was great and our potty planning was well timed so this was one of our more successful walks. The snow continues to melt, so we started out by walking on the local pier. At the very end, we came upon a fisherman napping in the sun. I think Sienna was surprised to see him there! He heard us coming and extended a jolly greeting. We all chatted briefly about what a great day it was for a nap (hint, hint, Sienna). We continued on our walk to the nature center (where we did not illegally feed any birds this week) and made it home for naptime.
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Our total: 2.87 miles
Kuber’s total today: 14 miles

March 9th:
Another beautiful day – I really hope it means that spring is coming! Sienna had been begging for weeks to do two things: 1) ride her “bike” and 2) take pepper to the dog beach. I decided to let her do both.

Sienna’s bike had been left outside all winter and until this weekend it remained trapped in a frozen pile of snow. Today it melted free, so here is the happy biker in action:
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* Note, miles not counted towards our goal 🙂

On to request #2! The dog beach isn’t very far away, for us and I wanted to get in at least a mile towards our goal, so I took one “wrong turn” on the way there. “I think they changed the entrance to the beach to waaay down here… Ooops, silly me, I was wrong. It was up the hill after all. Let’s turn back.”

I think Sienna may have known she was being swindled, because the entire uphill part, she yelled “faster! faster, mommy! Faster!!” No pictures will be allowed of that part…

Once on the dog beach, Sienna was thrilled. She plopped right into the sand and to my surprise, decided to make “sand angels”…
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Then she tested her rain boots in the water…
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Pretty much doing just about anything to get as dirty as possible. A little dirt is healthy, right?
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Today’s total: 1.27 miles
Running total: 9.08
Miles to go by 4/21: 17.12
Kuber’s total today: 7 miles, with a weekly today of over 34 miles. I’m glad he’s the one running the race!

Meet Zip:
And below is our fourth FOP friend, Zip! Zip and his family were the first FOP friends we met in person. We went for a delicious brunch in Texas and it was so nice to both get out the same medicine and not have to explain what it is or why Sienna was taking it. They were diagnosed about a year before Sienna, but I remember feeling like they were light years ahead of us in their journey – happy and centered. Such a wonderful family!!! Zip’s story is below, thanks to his mom Amy for sharing!

Also, they have an annual FOP fundraiser that is a BBQ competition and they give away a car! A car! Read more about Zip and the annual zipperq event here: http://zipperq.org

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Thursday, June 2nd 2011, my 36th birthday. Zip came into my room in the morning. His shoulders were swollen up like the incredible hulk. It was crazy, but he’s always been a bumpy boy. I figured he had just wrestled with some kids at school. We went about our day and the kids stayed with my parents that night so Jacob and I could go to dinner. On Friday my mom told me the swelling was still there, so I called my pediatrician. They suggested we go to an urgent care to have him checked out just to be sure nothing serious was going on. When we got to urgent care there wasn’t a doctor on duty, so a nurse looked at him. After the usual routine of questions she asked Zip to turn his head, he turned his whole body. He couldn’t just move his neck, the mobility was already gone. She sent us to the ER stating that if he couldn’t move his neck it might be something more serious. We loaded up and went to the ER and were told it was an infection in his lymph nodes. They gave us a prescription and we were on our way.

By Monday the swelling wasn’t’ any better. We went to Dr. Gordon (our pediatrician). He was stumped. Blood work and urine work showed no problems, but obviously something was going on. He had all of the other Drs. on staff come in to look at him, but they all gave the same look. Obviously they had never seen anything like it.

He suggested we come back in the morning when a few more doctors would be there to look at it. We did and still no one knew anything. They suggested we be admitted to the hospital to get some more tests run. We were in St. Francis Childrens hospital from Tuesday thru Friday. All kinds of tests and all kinds of drs. and no one had a clue. The swelling seemed to move in muscle groups over a period of several days. They were first in the neck, then to his back, then lower back, groin (during this period he gained 5 lbs and stomach was very swollen), then it moved back up into his laterals. His “touchdown” position moved to where he could barely reach his eyebrows with his hands. The whole process took about 3 weeks.

When we were in the hospital they did an MRI to check his back muscles. It showed swelling, but nothing else. We saw an infectious disease doctor, oncologists, and the hospitalists. All with their supporting doctors. We were told it may be viral myocitis, a rare muscle cancer, and a list of other things. No one was sure and that was the only thing that was obvious.

Then, miraculously, we had 3 weeks of calm. No swelling. Mobility was limited in his neck, back, arms, but seemed to be getting better day by day.

And it started again. First, with one lump on his back, then several lumps on his back. The whole time I was in touch with our pediatrician. He was baffled and we started seeing specialist. You name it we saw them. Allergist, dermatologist, etc. 34 doctors in all. No one had a clue and all of the test came back normal.

Dr. Gordon suggested we go see Dr. Connelly in St. Louis at the Childrens Hospital. She is a Neuro Muscular Pediatrician. On July 28th we made the trip. We met with Dr. Connelly. She took Zip’s shoes off and looked at his feet. Then she did several muscle strength measurements. She determined that we would have a muscle biopsy the next morning. But right before we left she suggested we do an ultrasound first of Zip’s left bicept. In the second set of flare ups his left bicept had become hard and he lost mobility in his arm a little. We met with her partner who preformed the ultrasound.
As he did the ultrasound he was talking about what he was seeing. He didn’t understand it. There was bone inside of Zip’s muscle. Once again, they were stumped. Still on for the biopsy the next morning they ordered more blood work and sent us on our way for the day. Zip was hungry so we walked across the street to the Panera Bread for lunch before we had the blood work done. We walked in the door, hadn’t even ordered, and my phone rang.
It was the doctor who had done the ultrasound. He asked us to come back after we finished our lunch. They had figured it out. I heard it in his voice.

Zip had the best macaroni and cheese of his life. Jacob and I just sat there. Not really sure what to think but focusing on keeping composed.

We headed back to the dr’s office and they took us straight back to the room. All four dr’s came in. The one that had called asked us to remove Zip’s shoes. They all just sat there staring at his feet. We were baffled. What did this mean?
From there they stumbled around. They didn’t know what they were telling us. They had never seen it before. This was very serious. He told us that we were not going to do the biopsy until after the blood work for this “condition” came back. I was confused? Just 15 minutes earlier the biopsy was a safe procedure, now, they would have no part of it. I don’t think he mention FOP the entire conversation. He knew we would google it. He said he would if he was given the information for his child, but to be warned that it wasn’t confirmed until the blood work confirmed the diagnosis. They had the genetic blood work taken and we left. So confused.

Zip was ready for a nap, so we headed back to the hotel. He went straight to sleep so I read the sheet they had printed off the internet about FOP. What was this? Crazy thoughts. I went for a walk and sat down on a bench in front of the hotel. I googled FOP. I didn’t need a blood work test to confirm. They had definitely figured it out. He had all of the tell tale signs. The toes, the way he crawled, the bumps all signatures of FOP. That’s also when I read the story of the stone man. His body turned to stone and he died from malnutrition all because of this FOP thing. I texted my family and told them the diagnosis. Told them I was exhausted and going to sleep. Knowing that they would have time to do a little research I would have less to try to explain. How could I explain? What was this?? I sat on the bench in front of the hotel. Looking like a crazy woman I’m sure. Just reading my phone and crying.

When I got back up to the room Jacob and I started talking. Why were we given this? Because we could handle it. Zip could handle it. Arlie could handle it. We agreed that we would see a family counselor to help us sort things out and have someone to talk to. We both sat silent for a long time. Just watching him sleep.

That night is kind of a blur. I talked to Dr. Gordon. He just told me how sorry he was for the diagnosis. No other helpful information. I talked to my parents. Jacob talked with his parents. It’s a strange conversation when you are trying to explain something you know nothing about.

Then I got a text from some girlfriends. We were scheduled to have dinner that night and they had all met up. Being sweet they texted that they missed me. I texted back something to the sort of, “thank you for being such great friends”. That’s when I got the phone call. The first one I ever had having to say out loud that my baby had FOP. Ginger was so sweet. I couldn’t have been very coherent. I sat in the bathroom and told her what I knew of FOP. We both cried.

The next day we flew home. Arlie was staying with some friends and they invited us to stay for dinner when we picked her up. We couldn’t. I couldn’t. Not ready yet to talk about anything we picked Arlie up and came home. I was scared to death. What would home be like? What would a life with FOP be like in our house. The questions were terrifying.

We walked in the house and I felt a huge calm. We were home. It wasn’t nearly as bad as I had expected. We were still a family. Then I realized the most important thing. We still had Zip. And no matter what we still had time.

After being on the oncology floor at St. Francis and at the hotel in St. Louis where it was all families with sick kids I was grateful my baby was at home tonight. He wasn’t diagnosed with cancer, leukemia, anything like that. Maybe we could handle this. Just maybe.

Our family had so much support in the 2 months that we had no answers. Tons of people contacting us to let us know they were thinking of us. We had an answer now, but couldn’t physically talk about it without falling apart. The night we got home I sat and composed an email. Just the facts. Zip has a rare genetic condition. One in 2 million. We didn’t know much more and would keep every body updated as we could. I couldn’t bring myself to actually put FOP in the email. What if they looked it up? It would scare them to death. It had me.

That night I also sent an email to Dr. Kaplan. He is THE FOP dr. in the US. I told him about Zip’s diagnosis. I didn’t need blood work, I wanted to meet him. Learn more!
He replied back with in 15 minutes. The conversations that ensued led us to an appointment with him in Philadelphia. August 25th, 2011.

By Tuesday of the week after our St. Louis appt. Dr. Gordon had sent Dr. Kaplan a letter. We started Zip on Gastrochrom and Singulair. All in hopes of reducing the FOP. At least we could do something. We have since added Celebrex to the routine.

The whole family made the trip to Philadelphia. Jacob’s cousin Emily lives there and Arlie could stay with her while we had our appointment. It was great to get away just the four of us and have some family time with the Gordon’s.

Dr. Kaplan was fantastic. I cannot say enough positive things about him, his team, and every other doctor we have come into contact with on this journey. He did a fantastic job of explaining FOP and what we could expect to happen. They are so hopeful that a cure is coming. Close. Close. Please be close enough to help my boy.

Over time the conversations have gotten easier. The more we learn about it the easier it is to talk about it. I would much rather people ask questions and learn with us than assume something that isn’t true.

We have resumed a life with FOP as a part of us. It is not ALL of our family, but an integral part of our family. Sure we can’t do some things, but there are a lot of things we CAN do. I am grateful everyday that we figured out what it was. Can’t solve a problem if you don’t know what the problem is. We can control it to some extent and will do our best to do that. Some things are out of our control and we will live with that too. We have such great support. Family, friends, Doctors are all willing to help and try to learn.

Amazing people doing amazing things!!

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