6 month Checkup with Dr. Standard

It's been almost 7 months since the external fixator was removed in October and we had our follow up appointment with Dr. Standard on Monday.  It was a whirlwind of a trip because we had to drive up to Baltimore and back all in the same day for her appointment.  So, we left at 5am for the 4+ hour drive up, waited for about 2 hours before seeing the doc and then drove 5+ hours back, which turned into 6 hours because of traffic.  :(

I was really worried about this appointment.  I don't know why I started to get nervous a few days before...maybe because it's been three months since we were last there and I am used to more frequent xrays and updates since we had been going every 6 weeks or so.  But I just had this fear that we were not going to get good news.

So, the xrays were somewhat difficult to read, but Dr. Standard seemed pleased with the bone regrowth in her femoral head and how it was sitting inside the acetabulum (cup).  He said that part of the head is still not completely regrown, and there is one spot, in particular, that is a bit concerning because it looks like there is a bump or divot or something in the bone.  The best way to overcome that is to maintain good range of motion and it should work itself out.  At this point, the ex fix treatment seems to have done exactly what we were hoping for - it kept the bone in the correct position while it fell apart and regrew initially and sped up the process substantially.  He said she can continue to play softball and has no restrictions on movement at all.  Hallelujah!

Here she is at softball last week:

BUT - he is concerned about her lack of range of motion since it really hasn't improved *that* much in three months.  He thinks that she may have even lost a bit of ground with regard to her abduction - which is how far apart she can spread her legs - and so he told her that she needs to start wearing her brace again at night.  She is devastated....and frankly, I'm sad for her too.  She has just gotten back to being able to sleep all night comfortably and it really isn't easy to sleep in that contraption that keeps her legs spread.  :(

He also said that we need to really double down on the stretches and make sure we are doing them TWICE a day.  We have gotten a bit complacent with them, honestly, because it's been such a struggle with Ava's attitude and our family's schedule.  She still fights us tooth-and-nail on these things and some nights, when everyone is tired, it's just easier to say, "Nevermind - just go to bed!".  But we can't do that anymore.  We have to make her do them no matter what - through tears and tantrums, they have to get done.

Here are the stretches we MUST do: http://www.lifebridgehealth.org/Uploads/Public/Documents/RIAO/ICLL/PatientHandouts/PerthesExercisesHandout.pdf

And the physical therapists at CHKD have given us a whole other group of stretches and exercises to do for strengthening and range of motion, too.  It's exhausting, truly.  But we are hopeful that with renewed resolve to follow protocol, her range of motion will improve by the time we come back in another 3 months for her next checkup.  Last week, they had a little party for Ava at PT to celebrate her 100th visit.  Not sure that's really an achievement....haha.....especially since they are billing insurance about $400 per visit!  OMG!  But they were very sweet to give her a certificate and balloon and she thought it was fun.  lol

So, all in all, a decent appointment.

Dr. Standard said that if by the middle of summer (Early to mid July), we haven't seen improvement in her range of motion numbers, we need to call him and schedule some more time in the OR - under sedation, they will do another round of Botox injections and manipulations.  Which is lovely since our insurance still hasn't approved the bills for the other two Botox injections because it's used for cosmetic reasons usually and is considered 'experimental' when used for this.  *roll eyes*  Don't get me started on insurance woes.....

Ava's gait (or walk) is fairly decent when she pays attention.  But when she's tired or has to go very far, it's pretty much like a teeter-totter.  We are hoping that once she gets full range of motion back, it will even out more.  I'll have to record her soon and put it on here so that my fellow Perthes moms can tell me if it's 'normal'.  What we are learning is that there really is no NORMAL for Perthes Disease.  It affects each kid differently and the treatments don't work across the board exactly the same for every child.  So we will just keep on keepin' on....thanks for all the prayers and support. It means so much to know that you are out there reading and following our journey!

Winter Forever.....

It feels like Winter is going to last forever this year!  We have had so much SNOW this year - which means SNOW DAYS off school and the kids are so bored with not being able to play outside much.  It's just too darn cold!

Ava is still working hard at maintaining her range of motion.  She still fights us on our daily stretches (that may never change) but seems to do better with the physical therapists, so we continue to go to PT three days a week.  It is a drain - both physically and financially, but until she can really get full range of motion and start working on strengthening and gait training, we have to stick it out.

Her scars are healing pretty nicely.  They are turning a more purpleish color rather than red/pink and the top ones (that you can't see in this picture) seem to be healing more quickly.  Doesn't surprise me since the bottom three were the ones that were such gaping holes and gave her so much trouble with the ex fix.

I recently went through the 'tub of medicine' and organized and threw away a lot of stuff from the ex fix days.  I lined it all up on the kitchen counter and couldn't help myself as the tears started flowing.  I never imagined I would have to give my child Oxycodone....for months at a time.  I never imagined having this much medication and medical supplies in my house.  We have been so spoiled for so long with good health and no physical issues that this came out of nowhere and really shocked us.

On March 7th, something truly AMAZING happened.  Something that was not at all amazing 8 months ago.  Ava rode her bike.  I know, you're thinking, "So what?  She's almost 9yo." but the range of motion and strength required to ride a bike is something that has eluded her since she got the ex fix off.  But she LOVES riding her bike and even when she got the all clear from Dr. Standard, she still wasn't able to.  But that's all over now, baby!  She's off!!!

We met with some friends to play and Ava even was ROCK WALL CLIMBING!!  Truly incredible!

ROM Improvements!

We are continuing on the path to better range of motion!!  FINALLY - and I know it sounds like I'm being melodramatic here, but it truly felt for a while like we were never going to get back to 'normal'.  I know that when your child has a disability or disease, they say that your definition of normal changes.  I never accepted that for Ava.  I read all about Perthes Disease and the stories other parents told and I thought to myself, "There HAS to be a better way. There is no way that she will never be able to play sports or run or be a kid.  That just cannot be."  I did SO MUCH RESEARCH and read and read and read.  We chose Dr. Standard in Baltimore because he offered us what nobody else that we saw could - a chance for our child to be a KID.  A normal kid.  Not a kid with wheelchairs and walkers and crutches and restrictions.  A kid who, after some really rough months of surgeries and treatments, would have a normal body and normal range of motion and be allowed to do normal kid activities.  We were fortunate that he was only 5 hours away from our home, but I am pretty sure that we would have traveled however far we needed to for him to have treated our daughter in order to maintain that normal childhood for her.

January 14th, 2015 - 3 months after the external fixator was removed, Ava made it to TRIPLE DIGITS in her hip range of motion!!  104 degree hip flexion (and 80 degree knee flexion!).  The physical therapists who have been so wonderful and patient with her since June were thrilled, too.  They literally were doing cartwheels for her in the back of the gym!  5 days a week of PT since....ummm...forever is finally paying off!

January 17th, 2015 - Ava made it to 112 degrees in the hip and NINETY DEGREE KNEE BEND!!! We were whooping and hollering!  And she got to pick where we went to dinner that night (her choice? Olive Garden).  It is amazing to see her sitting up straight at the table in a regular chair.  Truly a blessing!

January 19th, 2015 - We traveled back to Baltimore for our 3 month follow up appointment with Dr. Standard.  When he came in and saw her flexion and read her range of motion numbers, he said it 'made his day' to see how far she had progressed!  He said her xrays looked really good - the hip is contained and round and looking like it is healing in a beautiful shape.  There is always a chance that it will develop into an odd shape or have 'divits' or something in it, so we have to keep watching it.  But for now, she looks like a textbook case of success!

Then, he said the magic words that she's been waiting to hear:  You can run AND jump and be a kid!  That's right:  Released to Full Activity!  She can participate in PE at school, she can jump around and run to her heart's content!!  She needs to continue with PT and keep her range of motion progressing.  Ava asked if she can play softball in the spring and he said "Absolutely!".  Then she asked if she can go to a Bounce House and he said, "If it's okay with your mother..."  (which is is totally NOT okay with me, but we'll talk about it in the coming months!!!)

Ava wrote him a lovely little card and he was so happy to pose for a picture with her before we left.  We don't have to go back to Baltimore for THREE MONTHS!  Woo Hoo!

Range of Motion: Part 2. GOOD NEWS!

So, when we left off it was the week before Christmas and we had the surgery to manipulate Ava's hip and knee joints to see if there was anything that could be loosened up or that would show on the arthrogram as hindering her range of motion.

Unfortunately, after the surgery nothing changed.  Ava's ROM continued to hover around 60 degrees no matter what we did.  We had a nice quiet Christmas and she actually had a week off from PT appointments because her therapists were both on vacation.

It was around Christmas week when I started to question whether all of the decisions we had made had truly been the right ones.  What if we had made the wrong choice?  What if we never should have done the ex fix surgery to begin with?  It sure seems like she's WORSE off now than she was before! At least she could bend her hip before the ex fix.  At least she could sit comfortably!  What if what we did made things WORSE long term?  Maybe we should have just done the core decompression and not the ex fix.  Were all those months of hell for nothing?  All the pin care?  All the horrible showers, the sleepless nights, the walkers, wheelchairs, crutches....all of those months and medical bills and physical therapy and stress and it's WORSE???  I cried one day in the car on my way to take her to the last PT appointment on Christmas Eve.  Here we were, on Christmas Eve, going to physical therapy.  Physical therapy that didn't seem to be doing ANYTHING.  Maybe we had made a decision that would negatively impact my child forever.  And our family had to endure months of hell for what???  It was a low point, I'm not going to lie.

And then last Friday, January 2nd, 2015, Ava had her first PT appointment of the new year.  My mother took her because I was in Georgia visiting my sister.  I got a phone call from my mom and when I answered, it was Ava!  She said, "Mommy!  Guess what my number is!  GUESS!"  I dared to hope.  I took a deep breath and literally closed my eyes. She yelled, "SEVENTY FIVE!!!" 75 degrees!  OMG!  I couldn't believe it.  She had made progress!  FINALLY!  The next day, she had PT again and called to tell me that she made it to 80 degrees!  At this point, I was in shock.  Two days in a row?  Progress?  Seemed like a dream.  We took a few pictures during our nightly stretches and a video showing her knee bend.  It's much more fluid and her hip is staying up past 60 degrees all of a sudden!

And just yesterday, I may have cried big, fat, happy tears when she came home from PT and announced that her hip flexion was 90 degrees.  I honestly wasn't sure that it would ever happen.  Ninety degrees!!!!  Knee flexion 73 degrees.  It's happening.  She's really making progress!!!!

I do still have one major concern and that is Leg Length Discrepancy.  Since we did not do a bone-cutting surgery like an Osteotomy, I didn't think that there would be a major difference.  But Ava seems to have a BIG difference in hers.  :(

The next time we meet with Dr. S, that will be my first question.  Trying not to worry about things now that we are moving in the right direction, but it's hard after a year of worrying.  LOL!

90 degrees YAY!!!!

Range of Motion: Part 1

I can't believe that I haven't updated the blog in so long!  We got to the holiday season and everything just dropped away!

So, after our appointment in November with Dr. S where he said 'slow journey, not race' and we had the knee brace made, we continued to do PT 2-3x per week and stretches at home and try to encourage Ava to work hard at range of motion. By our cruise in December, nothing had changed.

We had a wonderful time on our vacation to the Caribbean - Ava loved it and we all had time to rest and relax and reset.  Traveling was HARD with a child who can only bend about 55 degrees at the hip, though.  She can't sit up straight in any chair, so driving, flying, etc. was rough.  If you have never tried to sit in a chair without bending in the crease between your hip and leg, I urge you to try it.  After about 10 minutes (at the most!), your lower back starts to ache and at about 30 minutes, your leg and back are on fire.  So, the day of travel to FL and the day of travel home were really hard.  I was very worried about her on the ship in a couple ways.  First, the pool on deck.  I was so worried that she was going to slip and fall, but I wanted her to have a good time and just be a kid and play.  It's so hard when you're a parent and you want to create these experiences for your child to be able to just be a CHILD after all the surgeries and physical setbacks and all the ways she's been separated from her peers this past year.  It's heartbreaking, really.  And the ship has a "kids club" where you can drop kids off and they have them put with their own age group to do activities, games, etc.  First, I was worried about dropping her off and not being able to really explain what was going on with her...why she can't sit on the floor like the other kids, why she can't bend her knee, or hip to sit, why she is not allowed to run or jump AT ALL, etc.  And I was worried that the other kids would maybe make fun of her or make her talk about why she couldn't do some of the games.  They play a version of dodge ball called 'gaga ball' and they did other running activities.  I just want so much for her to be an 8yo and play.  :(  But she had a great time and everything went smoothly for her.  She made friends, got her face painted for the first time ever and we truly had a nice vacation.

The week we returned, we headed back to Baltimore for another checkup.  Technically, we were 10 weeks post removal surgery and Ava's range of motion numbers were dismal.  Her knee flexion was around 55-60 degrees and hip flexion around 60-65 degrees.  Her external rotation is barely anything - maybe 5 degrees.  These are bad. Most kids 8-10 weeks post removal are past 90 degrees in both hip and knee, if not full range of motion restored.  I called early that week and asked the PA whether or not Dr. Standard would want to do a surgery while we were in Baltimore to assess her range of motion under anesthesia like he had mentioned at our last appointment.  He really wanted her at least to 65 degrees and she was BARELY making that....and only sometimes.  The PA said she talked to him and he said that we wouldn't really need to do that yet.  So, I decided to take Ava alone since there was no need for Ryan or my mom to come if they weren't doing surgery.

Fast forward to the day of the appointment.  They took more Xrays and he came in to talk to us about Ava's progress.  He basically said the same thing.  This is a slow journey for Ava.  There have been other kids who have had a tough time with regaining range of motion and even one who spent a year and a half to get back to 'normal'.  I asked about anesthesia/surgery and he said he thought it was fine to wait and see what happened over the holidays and we could do it in January if nothing changed.  I pushed back a little saying that I really didn't see how anything was going to change and that I really wanted to know what was going on.  I knew that my January calendar with portraits was busy and didn't really know if I could come up to Baltimore again for a few days at a time.  He said, "Well, if you are really concerned, I can fit you in tomorrow morning.  I'll make Ava my first surgery of the day and we can get it out of the way."  Ava was NOT happy.  She really didn't want to miss her last day of school and holiday party and immediately burst into tears.  But I knew that we should get it out of the way....we were in Baltimore already, and if it could help, I wanted to do it sooner rather than later.  I convinced her (okay, maybe bribed her) to accept that it was for the best.  We hung out with Annabelle, another little girl who went through the ex fix process recently and was there for her 2 week post op checkup and they had a great time together.

So, bright and early the next morning, we headed back to Sinai Hospital and checked in for surgery. I honestly didn't realize how hard it is to have a child go through surgery before.  Even 'low-risk' surgery.  This basically was like exploratory surgery.  They put her under anesthesia and his plan was just to move the joints around to see what happens when she is asleep and not feeling pain or fighting the movement.  He said that sometimes scar tissue or stiff joints can loosen just with a little manipulation and that he can't really do too much of that RIGHT after taking out the ex fix pins for fear of breaking bones.  Young children's bones are still not as hard and so with the holes in them, they obviously can fracture more easily.  So, 10 weeks post removal, the holes are not as dangerous.  But leaving your child unconscious in the OR and sitting in a waiting room not knowing what is going on is so nerve wracking.  Even though I knew that she was fine, really.  Barring any crazy complication, she was fine.  But crazy complications happen and it is hard to sit in a waiting room, alone, and just wait.  Not knowing.  He said it would be about 20-30 minutes....I was in the waiting room well over an hour.  And since we weren't planning on surgery and no one came with me, I was alone with my thoughts and fears.

When I got to the PACU (post anesthesia care unit - in other words recovery), Ava was literally coming up off the bed. Crying and whimpering and her body was bucking.  I was shocked - that had never happened the past two surgeries.  She was asleep when we got to the PACU those times and just slowly came out of anesthesia.  Apparently, the medicine they give before surgery to young children....they call it 'happy juice'.....helps them to sleep more deeply and wake up more easily.  Well, before the surgery, Ava refused to take the 'happy juice'. She said it tasted bad the last time and didn't want it.  I thought it was just for her to be calm and not remember going into the OR, so I didn't push her to take it.  They didn't tell me that it would make coming out of anesthesia so awful.  :(  It was hard to see her so upset and uncomfortable.  The nurse said she wasn't really awake, but the tears escaping her eyes and the confusion I saw was not fun.

Dr. Standard came in shortly after to talk with me.  He said that he was able to manipulate her knee AND hip both to just short of 90 degrees in the OR.  He didn't really feel anything big 'let go', so he wasn't sure if anything would change in her basic range of motion quickly.  But he definitely said the joints *can* get there.  He even took a picture in the OR and sent it to my phone as proof!  He said he injected dye into the joint and watched as he manipulated to be sure that he didn't see any physical reason that things aren't bending in her hip.  So, it is just a matter of continuing to work at it.  I was not sure I liked that answer.  Not sure I knew if it would be better  or worse.  If there was a physical problem that was causing the lack of ROM, we could maybe 'fix' it.  But there isn't.  So what do we do next??

So, we packed up and drove home - it was a long drive and we had to stop every hour or so because it's hard for her to ride in the car comfortably. And we continued to work on PT over the holidays.

More in the next post!