4 week Post Removal Checkup

Today was Ava's 4 week appointment with Dr. Standard.  It is hard to believe that we are already four weeks past removal surgery....it's been quite a whirlwind!

For those wondering, the pin sites have healed up quite nicely.  I don't have a current picture, but this is what they looked like just a week after removal and they look even better now!

She has had absolutely no pain since the ex fix was removed - which is just incredible to me.  The sites don't hurt at all, and the only time she complains at all is when we are doing stretches/exercises and during physical therapy when they are really working her joints.

Ava was so excited to get back to school after a week off - most kids her age probably wouldn't feel that way, but she absolutely loves school and her teacher, classmates, and even just learning!  I love that about her personality!!  The school continues to be wonderful with her and very accommodating to us.  She has been wearing her abduction brace 23 out of 24 hours a day - she only gets to take it off for physical therapy and when she goes to the bathroom/showers.  She truly hasn't complained very much about it at all - I guess we are lucky in that she is distracted by school and once it's on, she just doesn't think about it much.  I think she's only said a few times to me "Do I *have* to wear it all night?"

The biggest struggle right now (aside from her Range of Motion exercises) has just been maintaining the schedule.  Here is her schedule during the week:

6:45-7am - Wake up, get dressed.
7am-7:30am - Eat breakfast and do 15-20 minutes of dr. prescribed stretching exercises

7:30am - Leave for school

8am-3pm - SCHOOL (including 30 minutes of swimming during PE class)

3pm - I pick her up from school and we drive to CHKD
3:30pm-4pm - Homework in the waiting room

4pm-5pm - Physical Therapy appointment

5-5:30 - Drive home

5:30-6pm - 'free time' and/or finish homework/reading

6pm - Dinner

6:30-7pm - 20-30 minutes of dr. prescribed stretching exercises

7-7:30 - Shower/bedtime routine

7:30 or 8pm - Bed

Phew.  That is a packed-full day for her.  Usually by the end of PT, she is exhausted and whiney and the rest of our night (including stretches) is a tearful mess.  We are all stressed to the max. PT 5x/week is a nightmare.  The other kids who had removal surgery the same day as Ava are already on a 3x/week PT schedule, but because Ava has such issues with tightness and her ROM (range of motion) is so much less than them, we have been trying to maintain the 5x/wk schedule.

While we are seeing *some* improvement in her ROM, I was very concerned that when we came back to see Dr. Standard today, he was going to recommend that we put her under anesthesia to see what he can do with the joint and possibly do some further treatments.

Her knee did not bend more than 20-25 degrees the whole time that the ex fix was in place.  There was simply nothing we could do - or the PT department could do - to help with that.  The pins closest to her knee were just SO close and right through her quad muscle.  But most of the other parents I talked to said that their child's knee bend came back 3-4 weeks after removal surgery.   Ava's knee is not coming back like that.  It is moving, but very, very slowly.  She is resting at about 35 degrees now....and during PT, they can get it to 45 by pushing and working it a lot.  I have taken some pictures, and when I look at those, I definitely see improvement.  But day-to-day and week-to-week, it seems like we are not really getting anywhere.

First picture is the week we got home and the second is last night.  

Her hip flexion is also not coming back as quickly as we had hoped.  That's the one where you bend at the waist to sit up straight.  She is only bending at about 45 degrees right now - PT can get her to about 50-55 degrees when they really work it.  So, that makes sitting in any chair very difficult.  Not being able to bend at the waist AND not being able to bend the knee is like a double whammy.  Makes sitting anywhere (school, in a car, at a restaurant) very difficult.  She ends up 'perching' on the edge of a chair with her leg out to the side most of the time.  So, she is still sitting in her wheelchair at school because trying to sit perched on a chair all day would just not be comfortable at all.  At least in her wheelchair, she can be somewhat comfortable.  

SO - today, we met with Dr. Standard.  They took xrays again of her hip and he said that the femoral head regrowth looks really great.  She is contained and round and healing nicely.  He released her to nighttime-wearing of the brace only!  WOO HOO!  That means she doesn't have to wear the brace at all during the day anymore and can work on learning to walk more properly again!  He said that if there was any kind of malformation, divit, knuckle, etc. in the head AND she had these types of ROM issues, he would be worried.  But because her bone healing looks so good, he is not as worried about the motion.  Because she has the underlying skeletal dysplasia (yet-unnamed-tightness-in-all-joints), he anticipated that her ROM would be more slow.  His exact quote was, "This is going to be a slow journey for her...not a race".  I guess I wasn't anticipating it to be THIS slow....and my concern was that it would impact her eventual outcome.  He said that while range of motion is incredibly important to long-term success of the ex fix surgical procedure, it would take years of constant range of motion issues to impact the bone regrowth at this point.  So, I don't need to worry that these months where she's really tight in the hip will affect it.  Unless she starts not making progress or LOSING range of motion.  So, we just need to continue to work at it and remember that slow and steady progress 'wins the race'.  His reassurance that this is nothing to be seriously concerned about made me feel SO much better.  He said that one other little boy had ROM issues and didn't get his knee/hip back to normal for a YEAR AND A HALF.  Which simultaneously made me cringe AND feel better about Ava.  LOL!  The nice thing about going to a doctor who sees so many patients with this condition is that he's literally seen it all.  He's treated the worst of the worst cases - so he has perspective big time.  It keeps you remembering that 'it could always be worse'.  But the bad thing about that is you sort of feel like nothing is a big deal to him - he's always seen worse.  What a catch-22!

His next goal for her is to get to 65 degrees in both the hip and the knee by the time he sees her again in 6 weeks.  If she hasn't progressed (or goes backward), he will want to schedule her for OR time to put her under anesthesia, work on her joint and possibly use Botox to loosen her muscles around the knee/quad.

Dr. S gave us a lot of good suggestions on how to stretch her hip in fun/different ways so that she doesn't fight us so much.  One is by getting a yoga ball/bounce ball with a handle and making her sit on that instead of in a chair to watch TV.  :)  He agreed to give us a prescription for a muscle relaxer, too, to try to help with PT.  Then, he called down to the PT department at RIAO so that we could go right down and have a knee flexion brace made to help hopefully stretch her knee and get some extra bend in.  So, down we went.  They luckily fit us in and were wonderful getting it custom made for her.

They made it out of casting material, so Ava got to have her first cast put on and sawed off all in the same day!!  

It has a dial to turn on one side to bend it and she is supposed to wear it for 30 minutes 2-3x/day. (Not sure when we're going to fit THAT in, but....)  Dr. S. did release us to 3x/week PT, though.  He said that exhausting her - and the family - is probably not helping anything and that perhaps by going down to every other day with PT will help her to feel more in control.  He said that some kids work better with manipulative therapy and other kids work better when they are in control of the stretching/exercises.  We are pretty darn sure that she falls in the latter group.  ;)  So we are hoping that taking a bit of time off of structured PT, the times she is there will be more effective.

We are four weeks away from our BIG family vacation - celebrating that this year is almost over and that we are (hopefully) through the worst of it with Perthes Disease.  We are going on the Allure of the Seas - a Royal Caribbean cruise ship and can.not.wait to have some down time as a family!!!  Until then, we will continue to keep working on range of motion....range of motion....range of motion.  :)

Post-Op appointment with Dr. Standard

Tomorrow, we are 1 week post removal surgery and headed home after Ava's PT appointment!  She is so ready to be back home, see Carter, and go back to school on Monday.  She honestly cannot wait!  My schedule will be insane for the next week as I have to fit in 8 sessions and a night of 6 family minisessions in 6 days.  Ack!


We had our post-op appointment with Dr. S today and he addressed our concerns about ROM (range of motion) in Ava's hip flexion and her knee bend. Basically, he said to chill out.  He is a very calming person and he does NOT overreact to things.  One of the reasons that we chose him to take Ava's case is because he sees so many Perthes patients and often takes the hardest cases.  So we know he has literally seen just about every possible complication and outcome.  Ava was originally not supposed to be a 'hard' case, but her underlying tightness was a concern.  Dr. S. said that when he took the fixator off in the OR, he assessed her ROM and was able to flex her hip 80-90 degrees (when she was under anesthesia).  Therefore, whatever is going on that is preventing her from flexing her hip more than 30 degrees is not actually in the hip itself.  It may be that Ava is perceiving pain and tightness and 'locking up' the joint and she just needs to stretch it little by little and get used to that motion again.  He said that her knee did not bend at all under anesthesia and said that it may be a long time before her knee bends to 90 degrees.  :(  But he does not anticipate that either her hip flexion or knee bend are going to be long term problems....depending on your definition of 'long term'.  He did say that is a slow journey and not a race for her.  We are not to expect her to go from 30 degrees tight to 90 degrees in a few weeks.  It may be a month...or two.

While I am very relieved that he isn't concerned that there is something really 'wrong' with her hip, I am not excited about the prospect of dealing with this inability to bend at the hip and at the knee for months longer.  It is very difficult when she can't sit in a regular chair (or her desk at school).  And she is fighting stretches both morning and night because 'it hurts'.

The really good news was that her Xrays look very good!  He said that her hip looks "perfect" and her abduction is really good (which is why the brace isn't hurting or bothering her).  The head of her femur is round with no malformations, 'knuckles' or 'divits'.  They couldn't get the second angle on xray because you have to be in a froggy position with both knees bent in order to get that one and Ava can't bend her knee.  So there is a chance that the ball isn't as perfect as it looks on the one xray image, but if that is the case, it is only slight, so that's no concern.  The head is completely inside the socket where it's supposed to be, which is a big plus!  So if she can maintain her range of motion, this is a very, very good outcome from the fixator surgery.  It would mean that she wouldn't need any further surgeries unless something changes in the future.

This is the frustrating and difficult thing about Perthes Disease.  There is no way to tell how it will end up.  First, no two cases are the same and no two outcomes are the same.  So, you can't compare your child to any other child - or your child's progress to any other child's progress.  There are so many factors that go into Perthes Disease that even just deciding whether or not surgery is the right option is overwhelming. You can't look to other kids and say, "Well, it worked for Rylan and Reese, so it will work for Ava.  Their cases are all different.  Age, gender, range of motion, containment, rate of collapse, amount of involvement, etc. are all factors.  Then once you decide on a treatment plan, there is no guarantee that the treatment/surgery will work perfectly or that the outcome will be positive even if that surgery goes well.  I have heard stories of children who had fixator surgery, it looked wonderful and then the child relapsed - even years later.  They started to lose range of motion or have unexplained pain or need another fixator or osteotomy surgery or total hip replacement surgery, even.  It is a frustrating disease with many unknowns.  So, the only thing we can do is take the positive milestones as they come and work through any challenges - hoping and praying that we will continue to have good days.

Our mantra is going to be stretch, stretch, stretch.  Range of Motion, Range of Motion, Range of Motion....until we just can't take any more.  The more she moves, the better - we are going to figure out how to get her in the pool as much as possible and maybe look into equine therapy.  Apparently being on a horse is great therapy for Perthes, so I need to figure out if that's an option for us.

Ava quickly posed with Dr. S as he rushed out the door to see another patient.  He was very busy today - probably the least amount of time we have seen him yet at an appointment - but he made sure to stress that we were to stay the course and he will see us back in 4 weeks where we will decide what to do if her ROM hasn't improved.  So, it's back to Virginia Beach tomorrow and starting up with PT at CHKD again on Monday.

Good Days...but Not So Good News

We continue to have good days here in Baltimore.  Ava is active, not in ANY pain (Hallelujah!) and in good spirits.  Our stay at the Ronald McDonald House has been good and we have spent the last few days touring the attractions of the city before going to Physical Therapy appointments each afternoon.

The bad news is that after today's PT session, I was brought in to talk with the therapist and Dr. Standard's PA about her hip ROM.  There are many aspects to range of motion and one important one is called hip flexion.  It's the bend that your hip does in order to get into the sitting position in a chair. You bend 90 degrees (at least) at the waist, right?  Well, Ava is only bending about 30 degrees in her Perthes hip.  When she had the fixator on, for a while, she was doing better.  We were able to stretch her to 90 degrees or so..but in the past couple months, she started to lose some of the flexion in her hip.  We thought it was just tightness, or her unwillingness to 'push past the pain'.  But now it's concerning.  The pins are gone and the tightness and inability to flex that hip is very, very bad for her recovery. The therapist said that they sometimes see this type of tightness, but generally it's extension, not flexion.  And more than that, the range of motion begins to improve the first week - even if it's ever so slightly.  Ava's is not moving - neither is her knee bend.  They are both stuck at 30 degrees.  She is not fighting the therapist (even though she fights with me to do the same stretches morning and night) so the therapist doesn't feel like it's a conscious refusal to do the motion.  The PA called Dr. Standard (who was out of town for the day) and we waited to hear back from him.  He said that they may choose to put Ava under general anesthesia again (as soon as this Friday before we are set to leave to go home) so that they can see what her range of motion is without her brain controlling it.  Sometimes it is a muscle memory issue, and for some kids, it's that they anticipate pain or discomfort so much that they basically create additional tightness. The only way to tell is to test the joint under anesthesia.  We heard back that Dr. Standard would like to see what we can do with physical therapy for about 2 weeks and then come back up and do the procedure under anesthesia if she hasn't made significant progress.  :(   Not only do I not want to drive back up here in 2 weeks (taking more time out of school and my work schedule), but I also don't really want to put her under again and go through all that.  We do have an appointment with the doctor tomorrow, so I am going to talk with him extensively and figure out what we think is best.

I really need her to relax - which she doesn't seem to know how to do.  I don't know how to motivate this child.  I don't know how to get past her fear.  She is so incredibly stubborn.  And most of it is in her own head.  It is truly like she can not 'get over herself' and control her emotions to get through it. She's currently having a complete meltdown every time we have to take off the band aids on her leg where they are covering the pin sites.  And when I say meltdown, I mean she pulls a tiny bit on the corner, says that it 'hurts TOO MUCH' and refuses to take the rest of the band aid off.  I got so frustrated tonight.  I asked if it hurt as much as the pin care and she said YES.  It's like everything is the same type of pain to her.  Taking off a band-aid is the same as a steel rod in your bone???  Are you KIDDING ME???  It's so irrational that it would be funny if it weren't so incredibly frustrating.  I fear that the hip flexion and knee bend are the same way.  It's like she's gotten it in her head that it's going to hurt, so it is going to hurt and she will tighten up and refuse to do it.  I don't know what to do with that.  Nothing I've tried (or the child psychologist we saw in VB for a while suggested) works with her!  She is a special case, this stubborn child.  Argh!

We did get passes to go to the Aquarium this morning before PT.  Both of the other boys have been before, so they didn't go with us.  Just Ava & I had a really nice time, too.  Here are some pictures of what we saw:

After PT, all the kids went to the movies to see "Alexander and the Terrible, Horrible, Very Bad Day"  It was a REALLY cute movie and we all enjoyed it.

Finally, here is a video of Ava walking her her Scottish Rite brace.  Without crutches, she has to waddle - or walk 'like a cowboy' in order to have balance.  I think that once her right knee bends again, she will maybe not have to waddle quite so much, but for now, this is how it's going to be.  :)

If you could send up a few extra prayers for us to get past this fear and tightness issue, I would really appreciate it. We were really hoping that the next leg of this journey was going to be complication-free.  :(

The Brace arrived....fun in Baltimore!

Yesterday, the brace maker finally came to the Ronald McDonald House to deliver the braces to all three kids staying here.  We were told that we had to make an appointment to have measurements taken at the brace maker's office here in BALTIMORE at least 24 hours before surgery so that they could get the brace made and delivered to the hospital and fitted onto the child in recovery.  So, we drove up Wednesday night so that we could make it to the brace maker's office for the 8:30am appointment time.  (Yikes!)  Well, the day of surgery comes and....surprise... no brace.  Apparently UPS 'lost' the braces (I'm not saying that is a lie, is just smells funny to me having shipped literally hundreds and hundreds of packages in the past few years and seeing how UPS tracks packages) so they wouldn't be here until Monday.  None of us moms were happy - including the kids, because they had to deal with the big pink pillows strapped to their legs whenever they were laying down, but didn't have anything on while getting around, and since that is all they wanted to do (as you saw from my pictures earlier!!), they actually liked that. But the brace is an adjustment - especially after days of having nothing on.  It's called a Scottish-Rite brace and abducts the hips 20 degrees. So the kids end up walking like a cowboy, ha ha!   The brace is supposed to be worn for 23 out of 24 hours a day for at least 4 weeks.  So, it can really only be removed for physical therapy and shower/bathroom time. They have to sleep with it on and everything (or use the dreaded pink pillow at night)!  But they had a full 72 hours with NO BRACE from surgery until Monday, so they really got used to the freedom. No ex fix and no brace?  It was like heaven!  Until it was time for the brace fitting.

Luckily for us, Ava's brace seems to fit really well.  We have found a few advantages of her being so small - one was that the Dr. released her to full weight bearing right away.  She isn't heavy enough that her hip takes much force as she walks. Some of the boys who weigh more have to continue with the crutches for the full 4-8 weeks because of the pressure.  The other nice thing about being light is that the brace doesn't seem to rub her skin in any spots or irritate her.  And, being a girl, she can wear leggings underneath the brace and they are tight enough to the skin that fabric doesn't get bunched under it or anything like that.  She said her legs do sweat underneath, and really likes when it comes off, but it doesn't seem to bother her once it's on.

One benefit of not having the brace in the PACU after surgery also is that it would have been much harder to get it on with the gauze, pads, and bandages that were all wrapped on her leg after the pins came out.  Plus, I would imagine that it was tender and could have been painful to put the brace right on.  So, waiting a few days, we could see that it really did fit well (so no big adjustments to be made) and it doesn't hurt her to come on and off!

This morning we all headed off to the Science Center for a little field trip.  The kids really had a blast together.  We were just talking about how wonderful it is for them to have each other.  Not only just to pass the time, but also to have comic relief and others who are going through the same thing.  They motivate each other and they challenge each other and they watch out for each other.  <3

Here are some pictures I took at the science center:

DJ and Cooper laid on a bed of nails - Ava was too chicken!!!

Last night, for "Activity Hour" here at the Ronald McDonald House, they had Karaoke.  The boys were so shy, but after a little convincing, Ava jumped in and sang a few songs with one of the bigger girls!  I even got some video!!!

Go Ava!!!

The Zoo (& Post Surgery Shower)

We started the morning on a positive note - headed to the Baltimore Zoo for some fresh air and animal watching!  The new penguin exhibit was supposed to be great (and it was!) and we had a great time seeing the animals and feeing the giraffe!  So cool!!

We got back to the Ronald McDonald House and relaxed a bit before it was time for the first post-removal surgery shower.  I have been dreading this for 48 hours.  I asked them to go easy on the tape, but apparently nobody listens worth a crap because this poor girl had surgical tape across her belly and down her leg on the front and back.  I guess the boys had a different OR staff, because they didn't have hardly any tape and Ava had a ton.  She is so anxious about everything that just pulling the tape off was horrible.  We hadn't even tackled getting the gauze pads off the actual pin sites or anything - it took 45 minutes just to get the tape off!!!  We worked slowly - tried using baby oil to loosen the tape, but that didn't work. I also asked them three times for adhesive remover at the hospital and she told me they didn't have any.  What a crock!  I should have pushed more.  :(

After nearly an hour of getting the tape and pads off, it was time to get the gauze off the actual skin.  I knew there was no way we would be able to pull them off without getting them wet, but I also knew that getting them wet might not be good because of the amount of blood that the gauze pads had soaked up.  The minute the water soaked them, the blood just started pouring out.  Which freaked Ava out entirely.  I tried explaining to her that she was not bleeding AT ALL - it was just the gauze that had, like a sponge, soaked up everything 2 days ago and now that it was getting wet, it was just running out.  But it didn't really help.  She had fibers of the gauze pads literally stuck to the holes where the pins were!  It was every bit as bad as the first few pin cares.  :(  She said to me again, "I never wanted to do ANY of this!" as she sobbed and sobbed.  It just breaks my heart that she blames me for doing this to her.  I know that it will be a distant memory one day....she won't really remember all the details of this whole process.  I can only pray that all of this heals her as much as possible and she never has to do anything like this again for Perthes.  

Once the gauze pads were OFF, it was fine.  The water didn't hurt the sites, we put antibacterial soap on and made sure she was clean, but didn't really touch them at all.  She has a ton of adhesive residue across her belly, but we will have to work to get that off later.  I have lots of pictures, but I'll just post one of the sites in the shower and one of the Barbie band-aids we put on afterward!  Ryan said he thought they looked really great - and I guess I agree.  I mean, it could be worse.  None of them are big gaping holes - actually, the top back pin site looks nearly closed already!  But it is hard to look at for sure.  

After dinner tonight, the three Musketeers (Ava, DJ, and Cooper) got to have some of the cake that Cooper's mom had made for them.  We were missing the fourth, Buddy, but he wasn't able to come to the RMH.  So we saved him a big piece and went ahead and gave the kids their little sweet reward! They really are doing AMAZING.  They are all up and walking around - we are having to yell at them to slow down and stop pushing it already!  The little hops, the fast walking with crutches/walker, the climbing up onto things (yes, really!) to get something just out of reach.....they are just incredible kids!!  And they have gotten along so well.  Three is sometimes a hard number, and although the boys have definitely paired up and gotten a bit 'boyish' at times, they've all three played together and the boys have been great about including Ava.  Probably helps that she's more of a tomboy than a girly-girl.  :)  She loves playing air-hockey with them and even beat each of them once or twice!!!

Tomorrow is our first PT appointment at RIAO - so we are all hoping that they do as well 'working' as they have playing!! 

Incredible Removal Surgery Day!!

One of the moms who has been through this process told me that 'removal surgery was a breeze!' a few weeks ago.  Honestly, I was so scared to believe her.  I was terrified that it would NOT be a breeze for us and that if I got my hopes up, I would only be disappointed.  You see, this entire process has just been so incredibly hard that I had lost the faith.  So, I figured we would prepare as best we could have just have no expectations about how this part would go so that we weren't disappointed in the outcome no matter what happened.

We woke up this morning super early in order to get to Sinai Hospital for our 6:30am check-in & intake paperwork.  Cooper was also to report at 6:30, so we knew that these two kids would be the first surgeries of the morning.  DJ and Buddy were set to arrive at 8am, so they would be next in line. All four kids were having their ex fix removal surgeries, so we knew we were in good company.  DJ's mom and Cooper's mom and I have been in pretty close contact the past few months.  We have a Facebook message thread between the three of us that has 1800 messages back and forth.  :)  We have sent pictures of the kids' pin sites, asked each other for advice, and shared information about what was coming up or what we heard from the Dr's office.  We sympathized with each other's struggles and celebrated the milestones.  It has truly been amazing having other moms going through the same thing at the same stage together - and counting down to this day together!!

The kids came together like old friends yesterday.  DJ and Ava hung out for a little bit before Cooper arrived and then the three kids played together and the moms finally got to chat in person a bit.  We all turned in somewhat early for the big day ahead.

We woke up this morning and headed to the hospital.  Pre-op takes FOREVER - just hanging out in the room waiting for the doctors to arrive.  The anesthesiologist came in and gave us the standard schpiel about how it would work.  They would give Ava 'happy juice' to keep her calm and also to help block her memory in case she has to have surgery again, she won't remember the bad stuff.  In the OR, they'd give her a bit of nitrous oxide and then the gas to put her to sleep.  Once she was out, they'd do her IV and give her a shot in the tush that would act as a pain block from the waist down for 4-6 hours.  She didn't want to take the 'happy juice' (she's got a huge issue with taking liquid medication after all the yucky tasting crap after the first surgery) but we finally got her to drink it and pretty soon she was feeling loopy and giggling up a storm.  We took the last pictures of her with the fixator before saying BYE BYE forever!!!

The anesthesiologist asked her if she had any loose teeth, so she showed him the one that has been sticking up for months now.  He said he really doesn't like the kids to have loose teeth when he puts in (and takes out) the breathing tube in the OR because there is a chance that it could dislodge and the child could get it stuck in their windpipe.  So, he quietly said that he would use a piece of gauze and try to pull it (once she was asleep).  If it came out easily, it would be fine.  If not, he wouldn't worry about it.  We told him that he had our permission to give it a really good tug because she is such a wimp about pulling teeth anyway and that one really needed to come out!!!  Well, when we got to the PACU after surgery, lo and behold, her tooth was in a jar and he had left $5 with it from the Tooth Fairy!!!  Hooray!!

Before they wheeled her back to the OR, we suited up and took some pictures - we were all so excited for this surgery!!

When she woke up, she was in surprisingly good spirits.  I had warned her that she might feel groggy and grumpy, but that we would be there and she would be okay and we needed to focus on the happy part - no more fixator!  She was sleepy, but happy and giggled about her tooth, even!

The bad news was that the brace was not ready like they said it would be.  We drove all the way up here a day early to get brace measurements so that it would be ready for surgery day.  Well, apparently, the brace maker called Dr. Standard's office (or they called asking about the braces for all four kids) and they blamed it on UPS.  So, we were left with the pink abduction pillow strapped to her legs, which is less than ideal, but works to keep the hip in the right position while they sleep.  They are supposed to wear the brace 23 out of 24 hours a day, but they can't do that with the pillow unless they sit in the wheelchair all day long - which these kids were NOT going to do once they got the ex fix off today!!  He said it was okay for them to be free of it for a bit.  The brace should arrive tomorrow - Monday at the latest.

Ava asked Dr. Standard if she could keep the fixator, so we have all the pieces except the pins that went into her leg (they said they are generally not given back).  The packet with all the hardware was on the foot of her bed and I picked it up and was shocked at how heavy it felt!  I knew it was a lot, but GOSH it's just amazing that she carried that thing around on her little frame for so long!

While we were waiting to be discharged, Dr. Standard came in to talk with us about what he saw during the procedure.  Not only did they take off the ex fix, he also cleaned up the pin sites - no more granuloma!!! - as well as releasing some tissue around where the pins went in so that the skin doesn't pucker so much as it heals.  They also did an Arthrogram (where they inject dye into the bone to better see how much regrowth there is) and Botox injection to allow her to have the most range of motion possible.  He said that Ava's hip and healing looked 'beautiful' and he is very pleased with the amount of regrowth he saw on the arthrogram.  He will have pictures of it for us to see on Thursday when we have the follow up appointment with him.  But all in all, he is VERY pleased.  He said that if she maintains the Range of Motion and works hard at PT, this should be the only surgery she needs!  MUSIC TO MY EARS!!!  He also said that one of the benefits of being so small is that she can be pretty much fully weight bearing right away.  I was really surprised to hear that.  He said that she can use crutches to stabilize herself with the brace as she walks (when we get the brace), but that she is more than welcome to stand on her leg and walk on it too.  Once the brace comes and she gets the hang of walking in it steadily, she doesn't need to use crutches or a walker. I had no idea!  I was super excited at that news, but Ava was not.  She's been looking forward to using two crutches for the longest time....she begged me to use both with the fixator and I said no...so she would love to use crutches for a few weeks.  We'll see what the future holds with PT.  He said that her knee bend is a bit concerning - even under anesthesia, her knee joint is very stiff.  He said it's not the worst he's ever seen, but she is going to have to work hard to get it back.  It may take 3-4 weeks, but he expects that with proper PT, it shouldn't be a problem.  We were so happy to hear everything he had to report.

I asked Ava if she had any questions for him before he went on to the next patient and her only question was, "When can I go??"  :D  She was so ready to get outta there!!!  It was also a HUGE day because she got to put on PANTS for the first time in 4 months.  She said, "You don't know how good this feels!"  Ha!  The pin sites are covered with big bandages and tape and then they have a compression band (like an ace bandage) wrapped all the way around her leg.  We get to change the dressings and shower her either tomorrow or Sunday.  (My preference is Sun, but we'll see)

She felt so good that she wanted to go get something to eat for lunch - so we headed downtown and ate at PF Chang's.  I swear it was the most that girl has eaten in 4 months!  She had four helpings of brown rice, 2 chicken lettuce wraps, and some beef and chicken!  I was stunned.  Hope that keeps up - I hate how skinny she's become!  When she got in and out of the car, she said, "Wow - my leg feels so LIGHT!"  And I bet it does!

We headed back to the Ronald McDonald House and she did a little homework (told you she was feeling good!) and then we hung out with the boys for the rest of the afternoon and evening.  They were all up and walking around with crutches/walkers and happy as clams!  They played board games, hung out in the game room and played a ton of ping pong and air hockey and just had a blast! It was so awesome to see her standing without that damn thing on her leg!!!

So, we had an incredible day!  Thank you to everyone who was thinking of us, praying for us, and sending good vibes our way.  Clearly, whatever you did worked.  All of the kids got good reports from the doctor and we are so glad that they are all doing well and happy.  Hallelujah!!!!!

It's finally here! Removal Surgery TOMORROW!!!!

I swear there were times in the past 4 months that I thought this day would never come.  It has been a long and hard fought journey.  If that seems melodramatic, I assure you that it isn't.  If I have learned nothing else, I have learned that everyone goes through something.  And we just have to get through.

But we have learned so much.....


I have learned that you never know what someone is going through just by looking at them.

I have learned that 'handicapped accessible' doesn't always mean easily accessible.  It often means 'barely meets the code'.

I have learned that children are incredibly resilient, but that you can only push them so far.

I have learned that bureaucracy is pervasive...and the healthcare and insurance industries are full of it. (Literally and figuratively!)

I have learned that if you want something done right, you have to do it yourself.

I have learned that mothers don't get a break.

I have learned that there is nothing more heartbreaking than seeing your child in pain and being able to do nothing about it.

I have learned that nobody is perfect.

I have learned that I have a shorter fuse than I wish I had.

I have learned that my daughter shares that trait.

I have learned that the love of school can get a child through the worst days.  And good teachers & administrators are priceless.

I have learned that people are good and will help, give, and assist.  You just have to ask.

Above all, I have learned to have a greater appreciation for those who care for disabled children or their elderly parents.  Being a caregiver to someone who has physical or emotional needs is absolutely all-consuming. It is the first thing you think about each morning and the last thing you think about before going to sleep.  Putting that person's needs first changes a family, changes the dynamics, changes relationships.  It is exhausting and terrifying and worrisome 24/7.  Thankfully, our journey isn't nearly as difficult or long and drawn out as some people's.  And I will thank God daily when we get through treatments if this is the only trial we have.

Tomorrow, Dr. Standard will take the fixator off my child.  He will take the pins out of her femur bone and hip bone.  From what I understand, they will not stitch the pin sites because they won't heal as well and could possibly trap infection inside.  But the sites should heal (at least on the outside) in about 2 weeks.  Today, she was fitted for a Scottish Rite Brace, which will keep her hip in an abducted position for at least 4 weeks.  She'll have to wear it 23 out of 24 hours a day.  I'm sure that it will not be comfortable and she will complain about it, but my plan is to remind her of the fixator - ANYTHING is better than pins in her leg!!!  She can take if off for physical therapy and showering, but has to wear it overnight and everything. :(   We will have an hour of physical therapy 5 days a week for 6 weeks.  We are staying here in Baltimore next week for our first week of PT at the RIAO (Rubin Institute for Advanced Orthopedics).  They are the experts and see kids all the time with this condition, so I trust them to get Ava started on PT correctly.  Her knee bend is a huge issue since she has never been able to bend more than 50 degrees and it pretty much hovers around 30 degrees.  I am hoping they will have some good suggestions for us to get it to bend once the pins are out of her quad.  She's most excited to use both crutches to get around.  We'll see if they think that is best for her.  I just don't know about that.  The last thing I want is for her to fall.  I thought most of the worrying would be over once the fixator is off, but one of the moms told me a story about another little girl falling after removal and breaking her femur and now she has to have another surgery! Because there are 6 holes in the bone where the pins went through, the integrity of the bone is compromised somewhat.  ACK!  I swear, it never ends...the worrying!!!

But for now, we are going to take it one day at a time and try to celebrate how far we have come!  Tomorrow is a big day and we are excited to get it over with and move on to the next step.  Thanks to all who have supported us this far.  We will definitely update tomorrow and as the next week progresses!

Single Digits!

We are finally within SINGLE DIGITS of removal surgery.  9 days remain until we get this damn fixator off my child....and it cannot come soon enough for us.

While Ava is doing well in general, we are still dealing with pain and difficult pin cares.  At school, she is amazing.  Everyone I run into from parents, volunteers and teachers say that she is positive, upbeat, happy, just cruising around school either in her wheelchair or motoring along with her crutch. None of the teachers can believe how well she's getting on.  She has truly amazed me with her physical abilities through this process. She has always been 'active' - not athletic necessarily, but always moving, always going, never sitting still.  I wondered how this process would affect her and how we would keep her occupied and busy not being able to move much.  HA!  What a laugh now. This child rarely sits still - even with an external fixator!  She has not used the wheelchair in months with the exception of sitting at school during lessons.  Well, every once in a while if we go to the zoo or somewhere like that.  But daily, it stays at school - this summer, it stayed in our garage.  She is absolutely certain that she wants to walk.  Ava gave up her walker about 2 months ago and gets around with one crutch only.  She is supposed to be at 50% weight bearing on her fixator leg.  When using the walker, I felt that she was stepping on it more than that, perhaps.  With the one crutch, she leans on the crutch and away from her fixator leg, which seems to take weight off it for sure.  But she can really MOTOR with that one crutch.  Sometimes, I have to tell her to slow down - she's walking at least as fast or faster than my pace!!!  She is up and down the stairs, on and off chairs, plays on the floor with her brother, in and out of the car, etc.  Other than sleeping at night, she is never laying down and rarely sitting for any length of time.  She hasn't taken pain medication other than Tylenol for weeks now even though her activity level is really high.

Oh, I seriously caught her doing PUSH UPS last week when she was playing outside with her brother.  And not the 'girly' kind like I have to do, either.  LOL!

We have a system now for school and it's going well.  I walk her in and she goes straight to the classroom (instead of to the gym where all the kids wait for the bell to ring).  During PE, she goes to the library, sometimes gets homework done so that she doesn't have as much on days we have physical therapy, or helps the teacher in the classroom.  Her teacher told her that she was 'her secretary', which she absolutely loved!  LOL!  Then, I have to come in to pick her up about 15 minutes before the last bell in the afternoon so that we get out the door and to the car before all the kids are dismissed.  We are obviously protective of her and want to be sure that she doesn't get knocked over or fall down!  (Especially since she doesn't ride in the wheelchair!)  But I will be SO glad to get back to regular car pool - and so will Carter.

So, during the day, she's doing fine.  But when she comes home, we have physical therapy 3x/week and shower/pin care every other day.  Plus 30-45 minutes of homework each night makes for difficult and stressful evenings.

Here's what the fixator looks like  after pin care, but before the sponges and gauze are on.  It's still hard to see this big metal thing attached to her body.  :(

Pincare continues to be horrible.  I really hoped that by this point (well, way earlier than this, but certainly by the end) things would be easier.  I hoped that Ava would realize that shower and pin care is something that is going to happen no matter what and figure out a way to deal with it.  We have tried all of the strategies suggested by other moms/kids who have gone through this, the child psychologist, school counselors, and friends and family.  Unfortunately, Ava's stubborn streak has taken root and the anxiety and stress has turned into all out refusal to participate or find a way to cope.  The open wounds where the pins go through her skin are raw and constantly cause her pain.  Touching them or messing with them in any way makes her cry out in pain.  In particular, the granuloma on one of the pins just won't heal.  It got better for a little while - but basically since July, we have struggled with it.  She cries every time we do shower and pin care.  Partly out of fear, partly when we clean the pin sites and it actually hurts.  We have started to do it every other day instead of daily just to save all of us some stress and pain.

For those of you who don't know what pin care actually is, we have to clean the pin sites daily or every other day with antibacterial soap.  The first step is to take off the sponges that cover the pin sites.  For a long while, just taking off the sponges was cause for Ava to freak out.  They often 'stick' to the skin or the hair on her leg, so she has anxiety about getting them off.  We found that just getting in the shower and letting the water soak them softened up the skin and hairs enough for them not to hurt so much coming off.  For the past month, she has requested to soak in the tub first - so she lays in the water until the sponges float off!  Sometimes that takes 15-20 minutes.  Then I have to take stock of how much actual 'pin care' needs to happen based on how much skin/puss/yuck is around the openings.  We aren't really supposed to poke around at them, but anything hanging around the edges needs to come off.  That's the part she really hates.  Usually, I take a long piece of gauze and 'see saw' around the pins to get the stuff off.  But the skin is tender and somewhat raw and she hates the process.  It would probably only take me about 5-10 minutes to do that if she would let me just do it quickly.  But the fear takes over and it ends up being a long, drawn out process.

And then there is the granuloma.  I was REALLY hoping that it would go down and stay down, but it has reared it's ugly head again!  The bottom pin finally completely cleared up, but the one above it is the problem.  :(  The doctor and PA have both told me a few times that "The best way to treat a pin site infection is to remove the pin"  But, I'm seriously sick of hearing that.  I am glad that it will soon be better, but that certainly doesn't help our day to day life and dealing with it now.  We have done the silver nitrate treatment multiple, multiple times and it just doesn't seem to ever take care of it.  Sometimes it gets smaller but never goes away.  It's bigger now again.  And tomorrow we will be starting another round of antibiotics because two of her pins are starting to look infected again and I would rather be safe than sorry with them right before surgery.

See how the bottom pin is nearly completely healed, but the top one has that huge bubbly granuloma?? UGH!  

Here's the progress from 2 weeks ago until today.  It just keeps getting BIGGER and more painful.  Poor girl!

I talked with a friend of mine who has worked in a pediatric cancer unit and I said to her that I always hear the stories of the kids who are 'so brave' throughout their treatments.  The ones who 'never complain'.  They take their pokes and IV's and treatments well and their parents say that they are 'so strong and stay positive' through everything.  I mentioned to her how I just felt so frustrated because Ava didn't seem to be like that.  She hates this and doesn't hesitate to tell us.  She cries, complains, and is upset every.single.time we have to do pin care.  She is mad that she can't do the things her friends do - participate in PE, play ball like her brother, etc.  My friend (who is so wise) said that those stories we hear about the kids who never complain are the exception....not the rule.  Of course they put those kids on TV.  They milk those stories on the news or for the telethons.  But generally speaking, kids aren't the easiest patients.  They don't understand and they don't deal well with change or stress.  They do complain.  It's NORMAL.  But kids are resilient.  Most of the moms who I talked to whose kids are a few years out from this process have said that their child doesn't really remember what it was like.  They don't have the same memories that we have - they remember not liking it, but it doesn't imprint such a horrible memory in their little brains, thank heavens!  Soon, this will be a faded memory and we will have gotten her childhood back.  Soon she will be able to run and play with her friends, go to summer camp next year, play a sport, even.  And we can't wait!

We went to Build-a-Bear and she got to pick out a new friend - she was excited to see a set that had crutches!  It comes with a cast, not a fixator, of course, but it worked!  She also got a doctor outfit and named her bear Dr. Sparkles so that she can bring her along for the next surgery!

We had a little party to celebrate 2 weeks to go - and Aunt Abby brought cake!!

So, here we are.  9 days before surgery - 7 days before we head up to Baltimore again.  Trying to remember that it could always be worse.  We are nearly at the end of this hardest part of treatment.  I know in my heart that once this fixator comes off, even though the next phase will not be EASY, it will be EASIER.  And right now, that is all that matters!  We will celebrate the milestones and pray through the hard times.  Next week, we will be that much closer to getting back to normal!  HOORAY!

A New School Year

Ava started school and everything went GREAT!  We have finished the first two weeks of 3rd grade and she is really loving it.   Her teacher set up a special table up on risers for her so that she can sit comfortably in her wheelchair and work like normal.  She is still using the one crutch to get around, but seems to put less weight on her leg than she does using the walker and she's able to move more fluidly - sometimes TOO fast.  I find myself telling her to 'slow down' all the time now!

School has been a welcome distraction.  It is wonderful to see her occupied and happy again - glimpses of the girl she was are coming through again.  She is certainly tired in the evenings and trying to figure out how to fit in homework, dinner, shower, & physical therapy (30 min drive there, 1 hour PT, 30 minutes home) has been a challenge.  But we are making it happen.

Pin care is still a struggle - at this point, I'm not expecting it to get any better until we get the ex fix off.  I was really hoping in the beginning that after a few weeks or after they healed or after whatever-thing-was-going-on-at-the-moment.....that she would get less anxious and we could do the shower and pincare without the tears and anxiety and yelling.  But apparently, it's just what is is and we're going to have to get through it.  5 weeks left.  We are definitely counting down.

I went to a dr. appointment (for me!) last week and the nurse who checked me in told me that she used to work in an orthopedic area and had to do pincare with adults who had similar things applied to their bodies.  She said it was terrible for them - that grown men would rail at her angrily because it hurt so much.  Makes me so sad for my little girl and the pain and anxiety she has been dealing with the past few months.  :(

On a good note, she is finally feeling strong enough to wean off of the narcotic pain medication.  So we are slowly working toward going to Tylenol only, which I hope will also improve her mood.  Those drugs can alter personality and suppress appetite, which I think we have both.

We had our next 6 week Xray on Friday, so we're waiting to hear back from Dr. S about how her bone healing looks.  Pray for lots of re-growth and good hip position!

Wound Care **Warning: Pictures may be Graphic for some**

As I explained last time, we have had a lot of issues with wound care in the past month or so.  Starting about mid-July, we had to treat the hyper-healing tissue on Ava's lowest two pins with silver nitrate sticks.  The tissue looked like it was bubbling up over the wound and it was causing a lot of pain, not to mention stress when we had to do daily shower and pincare.  It was truly horrible - an hour or more of crying and screaming to get the sponges off and then clean the pinsites.  Here's what they looked like:

OUCH!  Just looking at that now makes me cringe.  :(

So, Dr. Standard said we should treat them with silver nitrate sticks, but they are prescription only and somewhat of a pain to get.  So, we had to go to the pediatrician locally to get him to do the silver nitrate treatment first.  We actually went twice before I realized that it didn't seem to be working and re-evaluated with text messages and pictures sent to Dr. S and his PA's.  We went to the pediatrician, he applied the silver nitrate, the next day we did shower and pincare and the nitrate just fell off in the shower, but didn't seem to have taken any of the granuloma off.  So, we asked them to give us a prescription for nitrate sticks to do it ourselves at home (since it was going to be an ongoing process).

Here's what it looked like from beginning to end.  First the granulomas, then the application of silver nitrate.  (It's basically a cauterizing agent, but didn't hurt, thank goodness.)  Then, by the next day, the nitrate had turned the tissue black and was hard like a scab.  

We learned that if we left it on for 48 hours without doing shower or pincare, when it came off, it actually took some of the tissue with it.  The black 'scabbing' stuff seemed to make the wound finally start to heal around the edges, which it was unable to do before.  The last picture is after SEVEN silver nitrate treatments.  It was literally over two weeks that we worked with the silver nitrate sticks and pin care to get the scabs off every other day and then re-apply the nitrate.  Ava did not tolerate it very well. While the nitrate itself did not hurt, it was just one more thing on top of all the other stuff she has to deal with.  And taking off the scab and doing the pin care around them was hard because they were so tender and sore.  :(    The nitrate also stained her skin in places and still has not come completely off.

Finally we had progress.  Once the tissue got down to her regular skin level, it was much more manageable and hurt her so much less.  The following two pictures are from earlier this week.  Finally the wounds are less fresh and looking smaller - closer to the pins themselves.  HALLELUJAH!

Ava has decided that she really likes to soak in the tub. That's right, she gets into the bathtub and manages to get down and lay in the tub of water!!  She can 'float' a bit and really soak those pinsites.  It really helped when we were trying to get those silver nitrate scabs off - soaking for 10 or 15 minutes softened them up and then we could take soft gauze and slowly work it off.  Still not easy, and still a total stress for all of us, but I just keep thinking 'baby steps'.  

Also, remember this long pin in the back of her femur and how awful it looked back in June and early July?  

Well, look at it NOW!

Oh, happy day!  It has finally healed!  Yesterday's pin care was under 35 minutes and with very few tears.  I can't express the relief.  I literally have knots in my stomach preparing for her shower every day.  The stress and anxiety for ME is overwhelming - I just cannot imagine how hard it is for her and it makes me so sad.

I am also a little worried about Ava's weight loss.  She is so thin now....and it's so not like her.  She was always my chubby baby, stocky toddler and solid little girl.  Now I can see her ribs and she is as skinny as her 5yo brother.  :(  We just can't get her to eat much at all, even when it's her favorite food.  This may be in part from the pain medication she's taking, so we're trying to back off the meds a bit so see if that helps.  

Okay, enough gross pictures and wound care talk.  On to something more fun.  We had a little get together for Ava and some of her classmates at a frozen yogurt spot in town last weekend.  I figured it would be really good for her if some of the girls saw the fixator and got used to it a bit.  They were awesome.  They never blinked or made a big deal of it.  A few questions answered by Ava and that was it - they just chatted and ate their sundaes and giggled like girls do!  Made me feel so good about  her starting school next week and the girls who will be there with her. 

We also had a milestone recently....we have made it HALFWAY with the fixator!  We had a bit of cake to celebrate and are really counting down the weeks at this point until removal surgery.  I know that starting school is going to help pass the time for sure.  Can't wait to see this smile more.