I had a question asked yesterday about why we chose to put Erin in a helmet.  When I read the email I had to do a double-take because it was then did I realize that we had to make several decisions within the first few weeks of her diagnosis.   In my mind I was barely functioning at that time and Alan and I had to make decisions fairly quickly.  The person who emailed me provided a different perspective on the helmet issue.  Ever since reading and responding to the email, I haven’t been able to get the conversation out of my head.  I guess that’s a good topic for me to write about.

The person who contacted me is an adult living with FOP (aka “FOPer”) who saw Erin and Miranda’s pictures from this past weekend.  She wanted to tell me a FOPer’s perspective.  She said that it’s bad enough that a FOP body changes dramatically and causes enough stares and looks.  Why would we put her in a helmet that could possibly cause more grief for Erin?  I agonized over the helmet issue.  As Alan and I have done since Erin was born, we go off of doctors’ recommendations.  Before the diagnosis, some decisions we made weren’t the best for her.  Post diagnosis and with Dr. Kaplan’s treatment guidelines, we knew that she would need the helmet.  He specifically states that a helmet is highly recommended for little kids.  FOPers who were diagnosed 20 or 30 years ago, didn’t have the IFOPA or the expertise of Dr. Kaplan.  Fortunately for those who are diagnosed within the last decade have the knowledge and experience of not only Dr. Kaplan but those who have been living with FOP for a long time.  We (meaning the entire FOP community) are lucky to have a network like we do.  The babies/children who are diagnosed today are handed years of research and stories of other FOPers.  So far Alan and I have met Sarah Steele and Sharon Kantanie who are both restricted to their wheelchairs.  Both families have told us that when they were diagnosed, there was no IFOPA.  Even trying to find a doctor to monitor the FOP was difficult.  Thanks to those adult FOPers, we are lucky to know that if we protect Erin’s head now, it may lessen some future damage.

When we finally picked out the Halo helmet, I said we might as well write on it, “Look at me!  I have FOP!”    I even left a space on her helmet just in case we wanted to put a saying in addition to the stickers for decoration.  Over time the helmet has grown on me.   Erin wears it when she leaves the house.  She doesn’t wear it in the house and maybe she should.  Most falls happen at home.  Some of the Erin’s worst head flare-ups were from falls on soft carpet. 

I cannot imagine there NOT being a parent of a FOP child that wouldn’t agonize over the decision to draw more attention to their handicapped child.  I know that I (for one) was trying to find ANYTHING different than these medical style helmets that would protect Erin but conceal the purpose.  My friend Tina’s father was researching different options.  He concluded that protective gear for hockey players would be a good choice.  For now we are sticking to the Halo helmet.  Karen and I both agree that we hope our little girls will have better balance coordination by the time they start kindergarten.  Hopefully by then we can eliminate the helmet at least until she can make that decision for herself. 

As I wrap this up, I have to share my favorite helmet story.  When we were at Dr. Kaplan’s office at U Penn, we were on line to sign in at the registration desk in the Orthopaedic Surgery office.  There was a woman ahead of us with big hair, big make-up, big perfume and tight animal print clothes.  At that moment I knew I was no longer in the pacific northwest.  She turned around and smiled at Erin.  With everything “big” about her I was preparing myself for a “big” attitude.  Surprisingly the lady recognized Erin’s helmet as a Halo brand.  She bent down to Erin’s level, gave a big smile, and said, “You know, only angels wear halos.”  That woman became my best friend for that moment in time.

Saturday was a beautiful day.  Not only was the weather perfect but we had a great visit with the Friz family from Burnaby B.C.  We are only a three hour drive from each other but we have to cross the border to see each other.  The Friz have their paperwork in place to cross the border so they came down this weekend.

It was just awesome to see Miranda and Erin get together.  There was a little awkwardness at first but they warmed up to each other fairly quickly.  What broke the ice was Erin invited Miranda up to her room to play.  Miranda headed up the stairs first.  Surprise #1….Erin saw Miranda crawl upstairs.  Without thinking, she did the same thing!  Alan and I have to carry her up and down stairs.  She will walk up and down the three little stairs to our front door but every where else she wants to be carried.  They played for a little bit and then the boys wanted to go play in the playroom.  Surprise #2…All the kids headed downstairs with Miranda and Erin sliding down on their stomachs.  Erin did both flights of stairs!

Eventually we all ended up on the main floor of the house.  The girls were in the living room playing a game of “turtle” as it got dubbed.  The girls would fall on the couch on their backs and get stuck.  They both have new FOP bone along their backs which prevents them from twisting and turning.  We would hear, “Come help please!  I am stuck!”  A parent would go free them and then they would do it all over again.  Check out the picture.  This is surprise #3…I am amazed on how each girl got up from the floor.  They both do it exactly the same way! Meanwhile Brennan, Larkin, and Owen were becoming fast friends.  They spent a lot of time outside.  Brennan and Larkin took Owen all along the walking trail that is behind the house.  There were times when the boys were quiet that we had to go check on them (sometimes silence is not a good thing).  

After lunch we gathered the troops and headed to the skate park.  We are so lucky to have a beautiful trail that connects the subdivision to the skate park.  The playground equipment is geared toward older kids.  Erin and Miranda could only go on a few things.  On our way there, Marilyn Hair, Sarah Steele’s mother, stopped in to meet the Friz family.  She walked with us to the park where we swapped stories and watched the kids have a great time together.

Throughout the day I picked up on similiarities of the girls.  Both love their dairy products (i.e. milk andcheese to be specific)…Both love their stuffed animals (Miranda had her puppy “Pops” and Erin with her baby “elfanant”)…Both carry around toys all day.  Okay, Miranda beats Erin on this one by a landslide.  Miranda would disappear and then return with an arm full of toys.  Slowly throughout the day she became attached to different toys so her collection grew exponentially. 

Pete and Karen are definitely our type of people.  I, for one, didn’t feel any awkwardness at first.  I guess it’s because we’re connected by FOP.  However if we were neighbors and we didn’t have FOP in common, I would put money on it that we would all be really good friends.  Miranda and Owen have birthdays in January and we’ll definitely be there for them.  Brennan was so sad when Owen had to leave.  With tears in his eyes he said that watching them go home was just as hard when we left our cousin’s house in New Jersey.

Thanks Pete and Karen for coming down from Canada.  Alan and I truly enjoyed the visit and I am set on getting our passports together.  We’ll be seeing you soon!!!!

Additional photos of the day are posted in the “Photo Album” section.

Our beloved Aunt Fran did some research and found out another way you can help donate money towards FOP research through the Combined Federal Campaign (CFC).  If you work for the federal government, you can make a contribution to the National Organization of Rare Disorders code #11992.  Here is a part of the email that Aunt Fran sent to me….

“I checked out the website for the National Organization for Rare Disorders.  They have published information booklets for the medical community for nine of the disorders they deal with, but not for FOP. (http://www.rarediseases.org/programs/freebooklets) Here is a quote regarding the publication of such a booklet.

“Individuals or organizations wishing to discuss the possibility of creating a future booklet for this series should send an e-mail to jcampbell@rarediseases.org or call NORD and ask for Jean Campbell.”

With your knowledge and interest, maybe you could get the ball rolling.  If I can help, I’ll be glad to also.

They only had one report on FOP on their website. They are very restrictive in its use, though.  It is at:
http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Fibrodysplasia%20Ossificans%20Progressiva%20%28FOP%29“

I have contacted the IFOPA and asked if they would eventually be listed on the CFC.  I have not heard back.  In the meantime, any donations to the National Organization of Rare Disorders code #11992 will help those living with FOP.

Update:  I received an email from the IFOPA.  They are not eligible to be listed on the CFC.  However they are actively looking other ways to get on the list.  Just a reminder, if you would like to contribute, see if your company has a program to match your donation.  You can even donate in the honor of Erin McCloskey.

I mentioned earlier that Erin’s preschool class is participating in a fundraising event for their school.  Each student made a snowman picture and Artwork For Education turned them into greeting cards.  They are available to order online.  Go to www.artworkforeducation.com, choose “Washington” for the state, then CLC AM/PM Preschool, and finally click on Killen.  Erin’s snowman is the bottom right image. 

Last night I saw Erin’s back is becoming more bumpy.  She came home yesterday from school and was very upset.  There was nothing I could do to console her.  I had to put her down for an early nap.  She did much better in the evening time but then she loves to watch Alan play Guitar Hero/Rockband on the Xbox360.  After every song she says, “Daddy rock out whoo hoo!”  Eventually she curled up on the couch and Daddy’s groupie fell fast asleep.  This morning she woke me up E-A-R-L-Y because her neck was hurting.  So today has been touch and go for her.

Tomorrow is an exciting day for us.  The Friz family from Burnaby B.C. will be here.  Karen and I have been in contact since we received Erin’s diagnosis.  Karen’s daughter Miranda was diagnosed about a year before Erin.  Since the two girls are close in age it has been our goal to get them together.  Karen, Pete, Miranda, and Owen will spend the day with us in Woodinville.  Alan and I have really enjoyed meeting other FOP families.  It’s helped us see that each case of FOP is different.  Yeah, it’s been tough at times but instead of dwelling on the negative aspect of FOP, we’ve seen so many positives.  Karen posted a newslink this morning on FOPonline and I have listed the link below.  It’s about Shane Terry, a little boy with FOP, from Watertown, NY.  A police department in California heard of his story and “adopted” him.  Very heartwarming….

Okay, so many urban legends surrounds the whole soda tab myth.  However, I can attest that charities do collect the soda can tabs for donations.  When we were at Philadelphia’s Ronald McDonald’s House, they had so many soda tabs, it was crazy!  So here we are, complete Diet Pepsi soda junkies (Fitness Together would not be happy with my admission to that)…and I started pulling our soda tabs.  Originally my plan was to donate them to the Seattle’s Ronald McDonald’s House.  Now I am researching how the IFOPA can benefit from them.  The downfall is the Ronald McDonald’s Houses have such a large fan base that they can actually benefit from soda tabs.  Here we are…a small, fairly unknown genetic disorder that gets little “airtime.”  With the economy completely tanking, this may be a way to help the IFOPA without pulling cash out of our wallets.  If you care to, please save your soda can tabs (or if you are from the Pacific Northwest they are called “pop can tabs”).  Yeah, just a little terminology I had to get used to when we first moved to Seattle.  Feel free to save them for awhile and mail them on to me.  I am assuming I’ll have to collect quite a bit before I can cash them in.  I am still researching on how they can benefit the IFOPA.  If the participation is not enough to make an impact, I will GLADLY drop them off at one of the two Ronald McDonald Houses here in the Seattle area.  I would totally break down in tears if I opened up an envelop full of soda tabs.�

Are you on Facebook?  If so, please become a fan of “International FOP Association *IFOPA*.”  Ashley Kurpiel has started the fan page.  You do NOT need to be a member of the IFOPA to join the fan page.  Several pictures have been added and the FOP community has joined in.  Ashley is the woman whose story will be shared on Discovery Health Channel’s Mystery Diagnosis this winter.  Yep, she’s the one who turned me on to Facebook.  We got in contact with each other after the producers of Mystery Diagnosis introduced us.  During my first conversation with Ashley, she had me look at her photos on Facebook.  Of course I had to sign up and become her friend to see the photos.  There was my downfall.  Now I am addicted to this online site that I use to waste time.  So by all means, if you’re on FB, look me up and we’ll become friends too! 

P.S. Thanks Ash!

I, for one, have truly enjoyed a “normal” week again.  We all went through our own “re-entry” phase when we got back from our trip.  With all the illness gone, Alan returning to work, the kids back to school, and I have the daycare up and running again….we all feel renewed.  Larkin has had a little trouble reacclimating to school but that’s because he was gone for two weeks.  He’s feeling the pressure of missing so much school work.  Erin’s preschool is doing a fundraiser where we can purchase greeting cards with the student’s artwork.  Erin made a snowman for her card.  It’s so cute!  She sort of got the snowman assembled correctly.  Family and friends can expect a holiday card from us that displays her artwork.  Such a good idea!!!

Sadly Erin is STILL struggling with this flare-up.  It started at the end of our trip back east and it hasn’t let up.  I have said this before and I’ll say it again, thank God for Naproxen.  Thirty minutes after we give her the medication, she’s back to her old self.  The meds are keeping the swelling under control somewhat, but she still feels absolutely miserable when they wear off.

Washington state just passed a proposition for legal use of marijuana but only if the patient has less than six months to live.  I am not an advocate of using illegal drugs by all means.  Although the legalization of marijuana had sparked a heated conversation with the FOPonline folks.  I can only imagine the pain FOPers deal with.  Everyone has their breaking point.  I do think it’s essential that patients that live with chronic pain should be allowed accessibility to have options for pain management.  Erin has been able to communicate fairly well to Alan and I when she is in pain.  Only the other day she was extremely whiny and she eventually told us her elbow hurt.  We jumped on it so quick.  I dread the day her elbows lock up.  There was no swelling in the elbow so I continued with the Naproxen.   I know she’s been in pain these last few weeks.  She’s been super cranky!  I was gone for several hours this morning before she woke up.  When she realized I was gone, she cried for 30 minutes screaming for me.  Alan had to take her in the shower to calm her down.  One day, I know, she’ll be better in communicating her pain to us.  Until then all we can go on is her actions.

Oh how I love Halloween!  I always have the best intentions to really go all out on the house decorations.  Unfortunately like the past Halloweens, we carved some pumpkins and then put out a self-serve candy bucket.  Our children became Indiana Jones, Princess Liana from Barbie’s Diamond Castle (just in case someone didn’t know), and some zombie-like creature.  Erin REALLY got into Halloween this time around.  She did really good.  All the homes here have agrigate steps leading to the front door.  Some houses like us have three steps but then there were other homes with 10 steps.  I was supposed to take video of the festivities but I had to hand over that duty to Uncle Sean.  I had to carry Erin up and down steps at every house.  She would yell “Up please!!!” as she approached stairs.  If we came to a house that was a bit scary, she wouldn’t let go of my hand.  Then as her bucket started to get heavy, that got passed to me to carry.

The good news today is that Kate stopped by with a rep from Olympic Pharmacy.  The rep brought two different models of strollers/push chairs for special needs.  After about a 45-minute discussion of Erin’s needs, we decided that the EaSys models would work better than the Serval.  Serval strollers are used quite a bit with children with Cerebral Palsy.  They look like they are one step below of a wheelchair.  The EaSys models look more like strollers.  Luckily we were allowed to keep the EaSys Swifty model to test drive and we used it tonight.  We REALLY liked it.  Erin totally enjoyed riding in it (which we were surprised) and we really liked not having to carry her.  We get to keep it for a week.  Then I will talk with the rep about the pros and cons of the stroller.  Both Kate and the rep highly recommended an EaSys model that is an upgrade to the Swifty.  The EaSys Jazz can fully recline in addition to the seat base tilts which is a great feature for Erin.  We’re hoping to see what the Jazz looks like in person next week.  It’s on www.exomotion.com too.  Then Kate will write up the requirements and the paperwork will be submitted to the insurance company for approval.

I’ve asked FOPonline folks if any children under 10 years is using a special stroller.  I’ve already received some good feedback.  Basically every one’s FOP progression varies.  For whatever reason, Erin’s lower back fused now and not later.  Oh well, we’ll just make the best of it.

When we were back east, I was told to post something everyday.  Even if it was just to say, “All is well!”  Okay, so I let a few days go by, but I THINK we are all at the end of this round of colds.  As one takes a turn for the better, another falls prey. 

Erin has been cranky all week.  With no other symptom I have been suspicious she was on the verge of a new swelling.  She’s been crawling into bed with us in the middle of the night.  When she comes home from school she quickly becomes agitated and only wants me to cuddle on the couch with her.  Today she asked for me to take off her shirt and there I saw a new swelling on her back.    I have no idea when she’ll be able to get her flu vaccine.  She can’t have any vaccines during an active flare-up.  I just hope we’re not in some vicious cycle of illness and flare-ups.

The kids are all excited that tomorrow is Halloween.  Even Erin knows what trick-or-treating means.  Today in preschool the kids dressed in their costumes and had a little Halloween party.  She came home all excited because she wore her princess dress.  I’ll be sure to share pictures of the kids in their costumes.

Other FOP news - In Sweden, Marie Hallbert works hard to advocate on behalf of her son, Hugo, and other FOP patients.  She is Sweden’s point of contact for their FOP community.  Marie has started lecturing about FOP.  Her first round of lectures started this month.  She recently posted a picture and blogged about her experience.  The website name is FOP Sverige.  I used Google’s free translator to convert the language to English.  She also has updated photos of Hugo who is now 10 years old.  There are pictures of his back that clearly show where the new FOP bone has grown in his back.

I have been meaning to provide an update but since we got back everyone (except Larkin) has either been sick or has had a tooth problem (me).  Alan finally went to the doctor today.  His cold set into his chest and now he knows first hand what I go through twice a year with bronchitis.  Lovely stuff!  On Monday I started feeling a dull ache in the upper right side of my mouth.  By yesterday I was popping Motrin as often as I could.  The dentist squeezed me in yesterday and surprise…I need a root canal.  Yea!  I think this is all payback b/c we enjoyed ourselves the two weeks we were gone.  Our karma has never been the greatest.

In the meantime, I started several different blogs but never published them.  Then today I received a summary of our visit from Dr. Kaplan.  Thank God for these summaries b/c I forgot some important tidbits.  First, her pulmonary stenosis (heart murmur) is minor and not relevant to FOP.  Second, when Erin had her MRI in late May, it was noted that she has myelination deficits in her cerebellum.  Basically the nerves in her cerebellum are not fully covered in myelin sheaths.  Dr. Kaplan also told us that at this time that doesn’t have any clinical significance to FOP.  I have learned through the FOPonline group, that there are other with FOP that have additional complications such as central nervous system issues.  Specifically, I don’t know the details.  However, I do remember the neuro-surgeon who we saw in May told us that the cerebellum attributes to balance.  Erin has always struggled with her balance.  Kate (Erin’s OT/PT) worked with Erin for two years to help her learn how to redistribute her weight and how to catch herself.  Although the myelin sheaths issue is not linked to FOP, I am hoping that doesn’t attribute to additional issues down the road.

Dr. Kaplan goes on to confirm that Erin has classic manifestations of FOP (clinically and molecularly).  He again stated that Naproxen and Prednisone were perfectly suited medications to take for suppressing FOP flare-ups and pain.   He made note that she does have “limited range of motion of her cervical spine in all modalities, normal range of motion of the jaw, very limited range of motion of both shoulders, and limited range of motion of the left elbow.”  Gosh he’s good.  He was doing part of his examination without her (and I) knowing.  He did mention that some FOP patients develop a bony area around the knee.  He spend some time feeling around her knees but did not feel these bones in Erin.  In his summary he adds, “I could not palpate any medial tibial osteochondromas, but cannot be sure that she does not have them.”  Tonight I will be googling “medial tibial osteochondromas.” 

Dr. Kaplan ended his summary thanking us for coming out and noted how adorable Erin is.  Well of course she is…she’s my mini-me!!  He does want to see Erin annually.  That’s good news for my NJ relatives.  Now we get to visit them more than once every ten years.

I’ll end this blog with a couple links that was shared on FOPonline.  One is of a show on Discovery Channel called “Mysteries of Mutants.”  They did an episode on FOP and I cannot find this DVD for sale nor can I find in on Discovery Channels’ line-up.  The other two are small shorts or a newspaper article about two other little ones with FOP.  Enjoy!!

Brooke’s Story

Zach’s Story

Mysteries of Mutants