Treatment Plan

Our visit to Baltimore and the Ruben Institute of Advanced Orthapedics (RIAO) was great.
 First of all the drives were pretty uneventful and Elsie did so great in the car! Plus I thoroughly enjoyed driving through the Appalachians. Gorgeous!

When we got to RIAO it was a beautiful sunny day.... I took this as a sign of what would follow :)

We arrived at 1010 for our 1100 appointment. By 1050 we had been checked in, gotten X-rays and talked to a fellow. Not bad considering we were told we may have a few hour wait!! By 1130 Dr. Standard came in with Lee (a nurse who I've been emailing and does the chats) and with the fellow, Fredrico. We talked a lot... Great discussion. We're really comfortable with the RIAO staff and Dr. Standard.

Summary of Dr. standard's diagnosis:
Current leg difference: 7.5 cm
Projected Leg difference in adulthood: 24cm give or take
Description of her severity "mild to moderate"
Evaluation of other parts of her leg (ankle, knee, foot): Appears to have good joints. Many cases are not as fortunate, though no promises that knee surgery won't ever be needed (or any other for that matter)

Here is a summary of our treatment plan:
15-18 months: Superhip Surgery This is like a salad bar surgery... Meaning there are about 15 possible things he could have to correct, Elsie may need 1 or up to 15. One thing we know she'll need is the femoral head to be straightened. This procedure will likely add 2cm to her left leg.

3-4 years: First lengthening. This will use an external fixator during the recovery. We will have 14 weeks of potentially staying in Baltimore for physical therapy and follow-up visits with Dr. Standard. If we decide to try and do physical therapy at home, we would only stay between 2-6 weeks in Baltimore and then come home. We'd then have to visit every two weeks through week 14. After week 14 we'd most likely return home and send periodic X-rays to Dr. standard until he calls the lengthening complete. At that time we'd return to RIAO once again and have the fixation removed. This procedure could add about 7 cm. This will be the most trying and challenging portion of Elsie's treatment. After hip and first lengthening, Els' legs will be pretty close to even, however as she grows, the difference will grow as well. Nice to think though that for a little while the difference won't be too noticeable - a glimpse into the overall success.

8-10 years: Second lengthening. This lengthening would use an internal lengthening device. No external fixation this time. This technology is something Dr. Standard developed (with others). We'd have physical therapy, time in Baltimore and of course recovery. however, not as intense as the first time. Again this could add 7 cm 10 years or so: remove a growth plate in her right keg. This is an option if we wanted to reduce the amount of lengthening needed in her left leg. Not sure what we'll decide, but this could eventually make up for 5 cm of the difference. Overall, this move would reduce Elsie's full-grown height by 2" which isn't a big deal coming from parents who are 5'10" and 6'2".

13-15 years: Final lengthening!!! Same as the second. This is where we'd make up the final difference. Girls' legs are generally done growing about 14, so we will do Elsie's surgery when she is done. This lengthening will depend on if we shorten right leg and the success of others.
So that's our going forward plan!!! We go back to RIAO to have a check-up and to plan/schedule her hip surgery in about 6-8 months. I'll update on other parts of our visit soon. :)

Recent Visit with Dr. Nowicki

Aghh!  Where has the time gone?  Over a month without an update and I have so much to share!

On Wednesday, April 25 we visited with Dr. Nowicki here in Kalamazoo.  We had some x-rays done of her knee and hip.  Good news…. Dr. Nowicki was very pleased with the results.  Based on what he saw, he definitely thinks that our best option will be limb lengthening.  What a relief, I just hope that Dr. Standard and Dr. Herzenberg can confirm.  I know limb lengthening will be a trying process, but I feel so blessed that we have a clear front-runner, that it isn’t a ‘six to one, half dozen to another’ scenario…. where Matt and I are forced to choose what’s best. 

So, you’re asking what was it that made Dr. Nowicki pleased?  First of all, he was pleased with her movement of the leg, and to hear that she is bending and rotating it well without signs of pain… (reminder, early on any movement in her leg was obviously painful – leading to her wearing a harness)  Also, the x-rays showed what appeared to be the femur being held into place, even though we couldn’t see a femoral head.  A certain gap in the hips, along with her movement, lead him to believe that there is a femoral head present, but we just can’t see it on the x-rays (whether it hasn’t ossified or it may be turned).  This is great news because often PFFD children are missing their femoral head.  On top of that, Dr. Nowicki thought her hips looked great and said that hip reconstruction may not be needed!  Though not definite, I take any good news we can get J 

Soon I’ll add a few shots of her x-rays from 4/25/2012…. 6.5 months old.

Trip to Baltimore!!!!

Here it is Monday night and we leave Wednesday morning for Baltimore!  I CANNOT believe it!  We are so close to the appointment.  What am I feeling?

Overwhelmed – so much to do, in so little time! 

Nervous – Baby and 10+ hour car ride (and that’s if we didn’t stop or hit any construction).  Oh yea, I get car sick too.  Plus I know I’ll be a ball of nerves leaving my oldest behind… which brings us to the next emotion.

Sad – I do not, I repeat DO NOT want to leave my 2 year old Mimi.  I don’t think she’ll understand us leaving her but taking Elsie.  Plus, I’ll just plain miss her. 

But, most of all I am EXCITED! – I am excited to meet Dr. S, Dr. H, Lee a nurse and the rest of the RIAO team.  I know we’ll soon spend lots of time here, so I’m looking forward to essentially acquainting myself to a large part of our future.  Wednesday is the first step into a major life event, so how could this not be exciting. I’m very optimistic – I know God will take care of Elsie and I truly believe that RIAO is where we’re supposed to go! 

Expectant – Let it be known, I have high expectations of this trip.  Up through now, we have very little answers on plans for the future, just a list of possibilities.  However, I am confident that with Dr. H and Dr. S’s experience, they will be able to give us a fairly accurate picture of what Elsie will be facing in the next 14-16 years. 

Finally, I’ve never been to Maryland, so it’ll be fun to visit a new place even if expedited!  

Multiplier Q&A

I participated in the most recent chat with Dr. Standard and Dr. Herzenberg on April 26.  Last time I participated they suggested we download the multiplier app for our iPad.  As I discussed earlier, we did this and it predicted a 12.4” discrepancy in adulthood. 

I know my measurements aren’t official since it doesn’t take into account bowing of the bone, nor am I an official measurer.  However, I still wanted to see what the Drs thought of this.

So, I shared these results with Dr. S and Dr. H on 4/26 and they said, even if that were accurate, they could probably perform surgery and correct that severe of a discrepancy!  YAY J

Multiplier Prediction

There is an application available for my iPad called "Multiplier".  It's icon is below:

During the monthly LLD chat that Dr. Herzenberg and Dr. Standard host, they mentioned this app as a tool that can predict the limb discrepency in adult hood, based on a child's current stats.  So, I figured if they're supportive of it, I would give it a try.

I had to select Femur, Female, input Elsie's DOB and the current discrepency (8cm).  This spit out a calculation of 12.4 inch discrepency at full growth.  Matt and I obviously aren't expert measurers, and we aren't trusting Elsie's future to an iPad app.  However, it's the first prediction we've got.  Unfortunately, it is a steep difference.  Within the next few months Dr. Nowicki and Dr. Standard should be able to give us more percise predictions, as well as what the implications of such predictions are.  It is a neat tool though.

Change of Doctors

Originally, we wanted to see Dr. Paley and shared our brief communication with him. However, since then we did finally make the decision to switch to Dr. Stanard and Dr. Herzenberg of RIAO in Baltimore. We're seeizng Dr. Standard on May 10th and I'm very excited!

Tough Choices

25 Months ago today I became a Mommy.  Ever since I've been making choices.... how long to breastfeed, when to start feeding food, what type of childcare we wanted, when to have another baby.... the list goes on.  Some of these choices were easy, even fun to make.  Others were given greater consideration prior to actually having my answer.  It's part of my role as a parent.  I've been given my children's future as the greatest responsibility.  Of course I knew I'd be making choices on their behalf.  But nothing could really prepare me for the choice I'll soon be forced to make - how to treat Elsie's PFFD. 


Lately I've been obsessing about what decision we will make.  Limb lengthening, rotationplasty, amputation...something else?  I don't know the risks and benefits of each to truly make an informed decision, but within the last week I can't think of anything else.  I am thinking about it 24/7. 


It all started from last week's chat when Dr. Herzenberg said Elsie's leg looks to be on the severe side of PFFD.  He mentioned a total of 7 surgeries.  Initially I was relieved he thought he could do surgeries.  That's what I've been hoping to hear.  BUT then my wheels have been going since then.  If Elsie had surgery to reconstruct her hip at 2 and then 6 surgeries for limb lengthening..... what would her childhood be like?  I'm guessing that the 6 surgeries is comprised of 3 to put on a fixator and 3 to take it off.  If that's the case, I've seen in multiple sources that a fixator is on for 8 months for each lengthening.  If Elsie has 3 lengthening surgeries, that's a total of 24 months in a fixator.  24 months of cleaning the needles everyday, 24 months of possible complications leading to chronic pain.  If this was the path we chose, would Elsie get a fun, carefree childhood?  Also, I'm starting to wonder what the increased risk is that's associated with greater discrepency.  Will there be a chance that we could go through these surgeries and her leg be shorter still?  If lengthened to meet the right leg eventually, how strong and functional will her left leg be?  We are making a list of all of the concerns we have so that come June we can ask Dr. Paley. 


Aside from the Physical concerns that Elsie may face, I'm wodnering how in the world we'd make it happen?  We live and work in Michigan.  To have so many surgeries and recovery in MD or FL we'd have to take off a significant amount of work.  Not to mention the finances associated with living in a hotel or short-term apartment each time.  The worst part to think about?  If I'm in FL or MD for 4 months at a time, where would Mila be?  I can't stand the thought of dragging her away from home, but even more so I can't stand the thought of her not being with me.  But then I think of the fact that part of those 4 months are bound to intersect with the school year...how could she stay with me.  My only logical answer to this is to MOVE.  Seems far fetched I know, but it's the only way I feel like we could stay together as a family, keep the girls in school as well as Matt and I being able to keep our jobs. 


So, needless to say, the Limb Lengthening is exhausting to think of.  Are the benefits worth the physical toll it could take on Elsie? 


On the flip side, I recently connected with a family whose little girl had her leg amputated at 9 months due to PFFD.  Her mother graciously has been open with me on their experiences and with photos.  Her daughter learned to walk with her prosthetic leg, her daughter had a single surgery and may never have another surgery again, her prosthetic is all that she knows (and as Mom says "she loves it!").  This beautiful little girl is seemingly able to enjoy her childhood just like others her age.  The prosthetic is functional and isn't noticable visually unless you're looking for it/know it's there.  This mom told me that they've never looked back.  So in April we see Dr. Nowicki again.  I want to find out more about our non limb lengthening options and the associated risks. 


Hopefully come June after seeing Dr. Nowicki and Dr. Paley, we'll have a good idea of our options and the risks/benefits of each.  Matt is trying to wait to think about it until we have all the knowledge... I wish I could. 


I pray alot about Elsie.  I want to make the right decision, I want a miracle in her leg, I want to know what God's plan for her is.  Right now my heart and head are pulling me towards amputation.  Seeing the little girl with the prosthetic calmed me.  I saw what I want to see in my daughter.... a little girl loving life.  A little girl in her home, with her family....every day.  But I can't help but wonder, am I REALLY leaning this way because I think it'll have the greatest results or because it'll be the easiest option?  It is obviously easier to not travel and go through all the surgeries.  But deep in my heart I'm feeling this is (with my current knowledge) the best.  If it comes down to it and we have an amputation.... will 18-year old Elsie understand the decision?  Will she wish we would have tried something else?  What would she have done differently?  These same questions would apply even in the event of Limb Lengthening.  Would Elsie be regretful of all the time she spent in the hospital?  The time away from home?  The pain associated with the needles?  Was it worth the years and years of physical sacrifice in the end?  And that's the worst part - no path is the easy one.  There isn't a right and a wrong.  And it's a decision I don't want to make - but at the same time, I trust no one else to make it for Elsie.  She's my precious little girl and no one cares for her like I do.... and no one would put the time and consideration into choosing like I will.

Chat with Dr. Herzenberg

Through PFFD.org I was introduced to a monthly chat that is offered to parents of children with limb lengthening needs.  It allows us to ask Drs who host questions and they give us their thoughts.  Dr. Herzenberg and Dr. Standard are both Ortho surgeons, like Dr. Paley, also among the World's finest.  They are the co-founders of the International Center for Limb Lengthening at Sinai Hospital in Baltimore, MD. 

I attended my first session on Feb. 16 and was pleased to see how accessible the Drs are.  I was able to talk to Dr. Herzenberg when it was my turn to ask questions.  I sent him a picture of Elsie's Xray.  Unfortunately, he was in agreement with Dr. Nowicki that Elsie's discrepency was on the severe side for PFFD.  Of course this is without a physical examination, but it was a hard blow for me to take.  I guess I've been holding out hope that her case wouldn't seem severe to someone who deals with limb lengthening on a daily basis. 

There was good parts though... Dr. Herzenberg mentioned that even though most Doctors would recommend amputation, he believes they could operate (estimated total of 7 surgeries - that's disappointing!) and also, he was asking about if Elsie keeps her knee bent or if she can straighten in.  When I said she straightens it, he said that's a good sign.  So that made me happy. 

Even though we're going to see Dr. Paley in June, I would like to keep our minds open to working with Dr. Herzberg or Standard.  I keep thinking of how personable Dr. Herzberg seemed via the chat.  Also, I like the thought that monthly I could communicate with him in an open chat Q&A without an official appointment.  Finally, others in the online community that I've been fortunate enough to learn from have had great experiences with him.  As an added benefit, MD is much closer to home than FL. 

Shots in the Short Leg

We do get the immunizations according to the schedule suggested for children.  The last two well baby check-ups for Elsie (at 2 months and 4 months), she has had to get 3 shots total.  From everything we've heard, the leg with PFFD is completely fine to accept the shots.  Elsie's pediatrician and I decided each time that we'd give her up to two shots in her right leg (normal) and if needed, the third in her left leg (short). 

The first round of shots in December went well.  Our pediatrician made a point to grab the most experienced nurse to give Elsie her shot in the left leg.  She wanted to make sure someone with experience could find the best mass of muscle (and would have the experience to know to do this!).  Of course she cried and immediately wanted to nurse.  However, that was the extent of our recovery.  After a few minutes Elsie was back to normal. 

This past Friday (Feb 10) we had the 4 month shots.  Again it was three shots, so two were going in the right and one in the left.  I didn't ask for the most experienced nurse, but will be sure to in the future.  A really young girl came in to help with the shots.  I don't even know if she was aware of Elsie's short leg.  Again, this is something I will make sure to bring attention to in the next round of immunizations.  However, I trusted the process since it went so well last time.  Little E again cried and wanted to nurse.  We did that and as I went to dress her again I noticed the shot seemed to be right ON her knee.  I was mad, but like my husband said, we can't change it now.  It's there.  Looking back, I wish I would have said something and remembered the name of who gave the shot (so I could avoid her.... although facially, I'll probably recognize her)

About 2 hours after Els' shots she became extremely upset and unconsolable.  She basically scream cried for a few hours in a row, with only a short nap to break it up.  She didn't want to nurse, she didn't want Mommy to sit.... I just had to hold her and bounce, bounce, bounce with her on my chest, her head on my shoulder.  This may sound typical to any baby who is upset, but I assure you she is NEVER upset like this.  She truly has the most happy disposition of any child/person I've ever met.  So I was nervous to say the least.  I took her tiny pants off and her entire thigh was red and swollen.  Luckily, the paperwork we were given said this is a known side effect (something like 1 in 6 children react in such a way).  Even though it is known to happen, I am convinced it was because of the placement of the shot.  Neither my older daughter Mila nor Elsie have ever reacted such a way to shots, just this one with poor placement.  I felt terrible.  Thankfully no long term harm, just a very uncomfortable baby. 

Why am I doing this?  85% is to complain and vent (I've been getting more mad each day) and 15% is just to encourage other parents to speak up if you want to.  That's what I've learned from this.  I will next time make sure I ask for a more experienced nurse and and I will for sure inform, or remind, them of Elsie's leg.  I'll ask them to make sure they take time to find the best mass of muscle and perhaps even share with them about the poke to the knee. 

Thanks for listening! 

Hard to see, this is 5 days later showing the poke.  I wish I had a picture of what it looked like swollen, but I didn't want to put her through the torture of a photo shoot then.  It's obviously much closer to knee than in the thigh muscle.

Developing

When Elsie was born the Doctors told us that she would develop as any other baby.  The PFFD doesn't link to other physical delays or disabilities.  This makes sense, plus, Elsie doesn't know any different - her leg has always been this way.  Though we heard this, it is comforting and great to see her developing just as Big Sister Mila did.  Below are some recent pictures of what I call the Happiest Baby Ever. 

Trying to Roll - She gets soooo close.  Anyday now!

Strong Core (even better now, but hard to capture in a pic)

Happy, Strong Girl

I hesitate to share this, but I am simply because other Moms (or parents/guardians) could be thinking the same thing with their child.  Since Elsie's PFFD was such a surprise, a very small part of me use to worry we will have something else pop-up that we aren't expecting.  I hate that this crossed my mind, but it did - and on a rare occasion, still does.  Everything Elsie does is normal, just like any other baby.  And the older she gets, and the more she develops, the less it crosses my mind.  Many days, I don't even think of her leg or the condition - she's just E.  :)

Going to See Dr. Paley

Dr. Dror Paley is the world renowned limb lengthening surgeon, and the man we hope can help Elsie.


Initially, our plan was to see him once we knew if/when she'd be a candidate to begin limb lengthening surgeries.  Even Dr. Nowicki has talked about Dr. Paley and his abilities.  Recently, my husband and I have been talking about going to see him as early as possible to ensure we have the best advice right off the bat.  So, while on Dr. Paley's website I noticed his email address listed on the contact page.  I emailed him and here was our communication over a two day period.  Each time I emailed him he got back with me within hours! 


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From: Jackie
Date: Thu, 22 Dec 2011 11:38:44 -0500
To: Dror Paley
Subject: Daughter Born with PFFD


Hi Dr. Paley-
I have been browsing your website and truly hope we get the opportunity to meet with and work with you someday.  My daughter has unilateral PFFD and is 10.5 weeks old.  We're currently seeing an Ortho Pediatric Surgeon in Kalamazoo, MI.  When should we engage your office as to her condition, if working with you is our hope and goal?
If you're interested, I've added some pictures and an xray on a personal blog I started about our journey with PFFD.  It's www.elsiesleftleg.blogspot.com
Thanks for your time-
Jackie


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She is at a good age for you to come out for your first evaluation. I generally like to see them by around 12 months of age.
Dror Paley, MD, FRCSC
St. Mary’s Hospital
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Thank you Dr. Paley-
 
We will work with your office to visit prior to her 1st Birthday.  I'm guessing it would be most beneficial to visit after an MRI, so that you can review it as part of your initial evaluation?  Would you suggest this?

Thanks-

Jackie
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I prefer to get the first MRI at 18-24 months. For now just come in for an evaluation and new xrays that we will get on site.


Dror Paley, MD, FRCSC
St. Mary’s Hospital
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So we are no longer getting an MRI as early as we thought!  This thrills me because I won't have to put Elsie under anasthesia as young.  Also, if it's under his direction, I completely trust it!  So it's set; we've set up our first appointment to go to Dr. Paley's in the beginning of June!  YAY.  Plus, who wouldn't look forward to a trip to West Palm Beach, FL. 

Just to be clear - We REALLY like and trust our pediatric ortho doctor here (Dr. Nowicki).  However, we just want to make sure we do all that we can, as early as we can for our Little E. 


You can also find Dr. Paley on facebook.  <--------- click there!!

Life without the Harness

So, I'm a bit behind on updates.  Elsie has been out of her harness for 3 entire weeks as of today!  What a blessing.  I remember with my oldest daughter, Mila, baths, changing clothes and even changing diapers seemed inconvenient.  However, after 10 weeks in a harness and doing the same tasks - they are much easier without the harness and no longer seem inconvenient. 


I saw on pffd.org that another mom's child was in a harness.  She too could understand the desire to have a squishy baby 24-7.  Let me tell you, it's been a great blessing to have her back to normal! 


Since taking her out of the harness we haven't seen any signs of pain.  It is evident that whether dysplasia or fracture or  whatever, the harness did the trick! 


Here is a picture of Elsie the last time she wore the harness!  How cute :)

It was a good time to go because Little E was about to outgrow it!

The only difficulty we faced from losing the harness was Elsie was use to sleeping in it for 10 of her 11 week life.  The straps kept her legs in the air (as shown above) the entire night.  So the first week without the harness Elsie had a hard time staying asleep.  She expected her legs to levitate :)  But we've worked through that and sleep is back to normal. 

Needless to say, we haven't missed the harness!