Waves of sadness, waves of gratitude, waves of fear, waves of hope. My emotions are all over the map right now.
I am a busy lady. I am a teacher, I am on the board of trustees for the local library, I teach sunday school to a rambunctious group of 3 year olds, I am working on my master's degree, I am a wife, a mother, and now apparently I am a researcher of a crazy genetic disorder called FOP.
The logical Shannon would tell herself that time heals all wounds, fears, and heart ache. I think the strong me would give the weak me a good slap across the face right now and tell her to snap out of it because I can't change this and I really just need to deal with it.
My question is how?
I know that many in my shoes right now would be cursing God for letting this happen to such a sweet little girl. I can't. I need him more than ever right now. I need my fears calmed and my worries washed away. I think this is an opportunity that the Lord is giving me to see if I really believe everything I think I do. Do I trust him? Do I really believe in all that faith, hope, and charity stuff? Do I really believe that he answers prayers?
Contrasted with how I'm feeling right now it would be very easy to say no. But yes, it has always been yes. And that isn't something that can come in waves.
Saturday, October 29, 2011
Addilynn had the test done three months ago so we were starting to hope that we were at the end of "to-do list" because our negative result was less important than finding a cure for this horrible disease.
Unfortunately for our family this was not the case.
Addilynn's FOP story begins with her adoption. Daren and I flew to Alaska in January 2010 with the excited expectation of adopting our first child. Our birthmother was due any day and we had been longing to have kids for several years. When she was born she was perfect and beautiful and exactly everything we could have ever wished for.
A few days later I was admiring God's handiwork-- her perfectly shaped ears, long angelic fingers, and soft dark hair. I looked at her feet. I noticed that they were a little bit wider and that she had large toe knuckles on her big toes. This didn't strike me as anything wrong, but something in me knew that those feet were just a little bit different. That thought quickly left me and my heart continued to dwell in the gratitude of being a new mother.
Addi's fop feet at about six months
At six months old I took Addilynn in for her scheduled immunizations. They were out of one of them so I had to return a couple weeks later. She had just learned to sit up so as we were waiting for the nurse to come I had her sitting on the table. The nurse came in, said hello, jabbed Addi hard with out restraining her or warning me, put on a band aid, said see ya, and left. It all took probably less than a minute and I was honestly a little flabbergasted. Addi quit crying and we left.
Addi @ 6 months old
I didn't think too much of it until about a month later when she started scooting around. She had always been a really active baby and wiggled a ton. Daren was playing with her and tried to get her to do bicycle kicks but she started crying and her thigh muscle was really stiff. At first we thought that was all it was and tried massaging it. The pain seemed to increase and we got really worried. I took her into the doctor and the PA said that she needed an x-ray.
The x-ray showed calcifications typically associated with traumatic injury. We got pummeled with a series of horrible questions about whether we or a babysitter was beating our infant. The PA then said that we would need more information and would need to schedule and MRI. The PA also made a comment about the possibility of not having an anesthesiologist willing to work on an infant close by and that we might end up going down to Primary Children's in Salt Lake. I decided to by-pass the extra doctors at EIRMC and go straight down to PCMC.
We were down in Utah for a family event anyway when Addi's pain continued to worsen. We decided to go to the ER at PCMC and sat there all day. She had some CT scans and the doctors were baffeled. We probably saw 8 different doctors that day. It just so happened that one of the leading docotors in sarcoma passed through at the right moment and the ER doctors had him take a look. He said he definitely wanted to see us. We met with him the next day and he looked at Addi and said that the chances of infantile sarcoma were very rare and that the more likely diagnosis would be a condition called Myositis Ossificans. MO is usually associated with sports injuries and car accidents and not babies that have had a run in with a bad nurse and a poorly given immunization, however, this is what we expected it would have been caused by. We were to watch it for the following month and see if it continued to grow.
It did and we were concerned with the possibility of it being cancerous. We scheduled an MRI and then a surgery to remove the mass and preform a biopsy of it just before Thanksgiving. The biopsy turned out to be benign and we were given the official diagnosis of myositis ossificans. This was good news but also brought the possibility that it could return, and it did.
At this point we were concerned but not too worried. It wasn't life threatening. She was walking with a bit of a gimp but other than her leg she was meeting every mile post and loving life. She had no idea she was any different. She had developed an aversion to doctor's offices though. She HATES x-ray machines and the crunchy paper on examination tables. She did get more scheduled immunizations and every time I was really nervous but made sure that the "blonde nurse" didn't come near us again. Because both Daren and I were busy with work and having a hard time connecting with the Orthopedist down in Salt Lake, we didn't end up getting in to see him again until the end of May.
Addi @ 17 monthsWe were first seen by the specialist's resident and I could see he was really concerned. He said they needed to take it out as soon as possible because the mass was the size of her foot. When the specialist came in he concurred that they needed to do surgery again but they needed a plan to keep the MO from returning.
We were then referred to the Huntsman Cancer institute to talk about the possibility of receiving radiation treatment before and after the surgery to keep the MO at bay. I was very impressed with this doctor because he told us very point blankly that this was uncharted territory and he needed to do his research. He wanted to deal conservatively with this because he felt that in our trying to avoid cancer and dealing with this MO problem, we might inadvertently give her a secondary cancer from the radiation. Looking back and knowing what little I know about FOP I am so grateful we didn't take this risk.
We were then referred to a rheumatologist. I had never heard of this type of doctor before and was constantly adding new vocabulary to my vernacular. Our rheumatologist dealt with inflammatory diseases and the hope was that he could prescribe some type of medicinal therapy to limit inflammation before and after surgery because that is what was believed to be causing the bone to form.
By this visit, it was July and Addi was 18 months old. At this visit the first thing this doctor asked was to see Addi's feet. This sent me flashing back to examining my newborn's wide little feet and chunky toes, part of me knew that he knew what he was talking about and that we were finally on the right path to figuring out what to do with Addi.
After seeing the bunion like knobs on the sides of her toes he explained the possibility of Addilynn having the condition Fibrodysplasia Ossificans Progressiva. He told us that it was rare and that there was a very good chance that she had MO, but that we needed to be sure that she did not have FOP before we went into surgery again because it would only make it worse.
Addi had blood work and x-rays taken of her feet and our doctor began to communicate with Dr. Eileen Shore and Dr. Frederick Kaplan. Based on our doctor's preliminary findings, they suspected a possibility that Addi could have this genetic disorder and offered to do the genetic testing in their lab in Philadelphia.
We made arrangements to have the testing done and came back for extensive x-rays (remember that this is one of Addi's least favorite activities) and the drawing of 5 vials of blood (we avoided the crunch paper and she got to sit on Daddy's lap.) Then we said good bye to the box that held all the answers and waited.
We waited, and waited, and waited. There were days where we couldn't stop thinking about it and days where we didn't care that we hadn't heard because we didn't want to know. During this time I really was made aware that we aren't alone in this. Our family belongs to the Church of Jesus Christ of Latter-day Saints. You might know us as Mormons. Point being, we believe in a Heavenly Father that loves us and in His Son, Jesus Christ who loves us just as much. We believe that because of this love, we receive blessings that may not answer our current heart's desire, but shape us into the people we were always meant to become. Our family is a family of faith. Because we live in this world it is easy to loose sight of optimism and attend the "why me" pity party. I am human and felt my self sliding into this on a fairly regular basis over the last few months. However, each time I did someone would step into our lives and lift the burden just enough to let us know that God was truly watching out for us. The last 3 months have been about preparing us to be able to be strong through this and overcome this challenge in our lives.
The last 3 months have also been about educating ourselves. I am increasingly grateful for technology and social networking. I watched a video of a mother who's daughter was diagnosed in the 80's. The only thing she could do to learn more about FOP was to run down to the local library and read a paragraph about it. I don't know how I would live not knowing what this is with out IFOPA or youtube. I have found some blogs of other mothers who have children that fight with FOP. I don't know quite yet how this disease is going to change our lives, but the thing that impresses me most about the families that are trying to beat this thing is there strength and optimism. They are so full of hope even though there is not yet a cure, let alone a reasonable and consistently effective treatment to speak of. I don't know anyone with FOP yet personally, but I am looking forward to getting to know them and their stories. Addi is such a happy and determined kid, I really feel like she is going to fit in with this bunch.
When we finally got to speak with Addi's doctor in Salt Lake last night we were apprehensive to say the least. We were really hoping that he was going to tell us that the test was negative and that we were cleared to keep working on keeping down the MO with naproxen and get back in touch with the surgeon to take it out again.
After we hung up with the doctor, Daren and I just looked at each other and started crying. Daren said to me, "You know though, we already knew it." And we did. We knew it back in July when Addi's Doctor mentioned it for the first time.
Addi @ 20 months
So where are we now? Addi's SLC Doctor is working on a packet of information to pass along to her pediatrician and daycare provider, and also letting Dr. Kaplan know that we are interested in meeting him. I love how he has his interviews in his office and has all of the pictures of his patients. It shows me that he is truly motivated to help these people. I think there is a special place in heaven for this man. Angel of mercy. We will hopefully go and return before January. Addi turns 2 on 1/11/12 and it will be one less plane ticket to buy.
Emotionally we are a little numb. Like Daren said, we knew it already. That knowledge has just been confirmed by science. As a teacher, I have always believed in the philosophy that knowledge is power. The more we know, the better we will be able to fight this thing. I am inspired by those who have been living with this and taking this bull by the horns. We either run away with fear or we deal with it and we are going to be doing the latter. I think we have made up our minds to be part of the solution rather than victimized by the situation. God help us.
I am writing about this for a few reasons. I believe that there is power in stories and truth. There will never be a time where Addi will be able to remember life before FOP. There is so much information to process here that I really need to write it all down. Hopefully in my writings and experiences I can answer the future questions of future FOP families until one day we can finally end this disease.
I'll be keeping you posted.