Epiphysiodesis Procedure

On Thursday morning Mary had a "distal femoral epiphysiodesis using the phemister technique" aka a procedure to stop the growth in her growth plate in her longer leg in hopes to allow her shorter leg to grow and catch up to minimize her leg length discrepancy. The current discrepancy is approximately 3 cm.

This was procedure was number 7 for her fibular hemimelia. The first 5 were all associated with the external fixator in 2012 at age 9, then at age 11, she had an 8 plate put in her left knee and work on her left foot to help straighten it out. Mary is now almost 13.

• We had the first surgery time at 7:30, 
• Mary was more nervous for this procedure than I anticipated (older and wiser I guess),
• Dr. Scott's portion was only 37 minutes and we were called to recovery right before 9:00. 
• Other than shaking from the anesthesia initially (reminded me of me after by my deliveries of the girls), 
• recovery was fast and we headed home at 10. 

• We bought a machine that allows cold water to constantly "ice" the area for the first 48 hours (you do check the skin periodically and there shouldn't be direct contact with the skin). Insurance didn't cover it but we assume she will have more procedures, plus her parents aren't getting any younger and anyone can use it. We think it helped recovery a lot.

Mary has a strong pain tolerance. Although they prescribed a stronger pain med because the procure involves bone pain she only took over the counter ibuprofen at home. It is always nerve wracking going into the first night after a procedure because of concerns of pain management but Mary slept all night. Her first real discomfort after the procedure wasn't until 5:45 the following morning. Even then she said it was sore not painful. 

She really wanted to go to school the next day but the hardest part of the procedure has been using crutches. Her shorter, weaker left leg has to support her body since the procedure was on her stronger, longer right leg. She was unsteady as a result. 

She is very mature about the medical stuff and she realized it's better not to push too hard too fast so she can have a shorter recovery in the long run. If she went to school she also couldn't use the ice machine that seemed to be helping so much. 

It is day 3 and Mary hasn't had any medicine, she has iced a little bit and even practiced walking without crutches. 

So this procedure has proven to be "easy" as the medical world says it is. Medical easy isn't always easy in reality :-)

The procedure is a gamble because there isn't anything to precisely determine the best timing. You don't want to do it too early so that the short leg turns out to be longer than the longer leg that had the growth stopped, and doing it too late means its kind of wasted because the shorter leg doesn't get to catch up very much. Dr. Scott says it appears we have gotten pretty lucky, the growth plate looked like it still has some growth in it but not a ton. We have to wait and see the true success in 18-24 months when she should reach her adult height. She is almost 5'1".

Pre-Op for Epiphysiodesis

We had Mary's pre-op for the Epiphysiodesis on Wed., Dec 12. 

• On the longer leg Dr. Scott will make a small incision on each side of the knee,
• He will cut less than a cm. square piece of the bone (distal femur), twist it 90 degrees, and reinsert it. 
• He will also scrape the growth plate really well because he wants to make sure that growth plate stops growing.
• They explained by just doing the two sides it confuses the bone enough to stop growing in that area.

• He did take another standing x-ray with a 3 cm block and confirmed he wants to keep the 8 plate in her left knee for longer, he says sometime it rebounds a little when you take it out

Other surgery info:

• No liquids after midnight the night before
• Surgery would be cancelled if she had a fever of 100.5 and/or had a very productive cold or hacking cough
• We currently have the first time slot (because they know we will be on time :-) ), unless a small baby needs the slot.
• The actual procedure is only 45 minutes so she should only be away for 2 hours.
• They will give her local pain medicine (Novocain) before they close her up, and bandage the knee.
• She should have loose sweats to wear home, no yoga pants
• She will be weight bearing as much as tolerated, use crutches if need to originally
• We said we would pass on the wheelchair offer
• As she knows from before the more you move the better
• PT is optional, sometimes the knee loses range of motion
• We have a pain medicine for at home, advises to stay ahead of the pain initially (as usual)
• He is going to order "the ice man" for us which wraps the knee and surrounds it with cool water to help reduce pain and swelling.
• Her dad was good to think about the orthodontic forsus (a metal version of rubber bands) Mary has right now for her braces. We were given the chief anesthesiologists contact information and was advised to have it removed for surgery because it limits the mouth opening. SO she is off to the orthodontist to have it removed.

Step 2: Epiphysiodesis: shorten the long leg

We saw Dr. Scott on Sept. 21. Mary had a standing x-ray to:

• monitor how the 8 plate is doing straightening her left leg, 
• help determine the current discrepancy, and 
• a hand x-ray to calculate how much more growth she has left.

standing x-ray using a 3 cm. block

bone x-ray to determine amount of growth left

close up of 8 plate

The conversation was very similar to the conversation in May. Mary was given 2 choices:

1. epiphysiodesis to shorten her long leg:

• he goes into the distal femur and scrapes the growth plate so it won't grow as long as it would have if it was left alone. 
• The procedure is done on an outpatient basis, it takes 90 minutes of his time, and the recovery is she will be soar for a couple of weeks.
• the femur grows .9 mm a year
• If she has 2 years of growth left she will gain 1.8 cm.
• He believes she is 3.5 cm difference in length so this should get us close to the goal of less than a 2 cm. difference.
• If she wants to go this route he wants to do it sooner rather than later. He does NOT advise waiting until summer.
• Pediatrician believes she will be around 5'4" and I believe she is 5'1" now.
• Bone age shows over 13 but not 14. She will be 13 in February.
• The other growth plates continue to grow it is just the femur that is stopped from growing

2. PRECICE internal leg lengthening:

• rod is put into femur,
• after one week, use magnets 2 times a day to lengthen the bone
• based on her difference she would lengthen for 25 days so it would be over in 32 days
• the rod stays in for one year for the bone to consolidate
• it requires an inpatient surgery with 3-4 days in the hospital
• there are no external pins so infections are not a concern
• it's easier than an external fixator but still more to go thru 
• she will be on crutches and have a knee brace to protect the knee from "sliding" 

Additional info: 

• ankle looks like it is holding up because of her afo's when she was little.
• He no longer wants to remove the 8 plate in December as he planned in May. The growth center is still open and he may over correct because it tends to slide.

So when we left Mary had information to help her decide what she wanted to do and we were to call and let him know. 

In May Dr. Scott focused on the epiphysiodesis that he has been mentioning since she was 5 months old but at the Sept. appt. he did talk about the PRECICE internal lengthening as an option because he just finished a successful one on a girl similar to Mary. 

At first I was leaning toward the internal lengthening because the shortening of the right leg is not an exact science and has always made me nervous. But then I did some research and remembered a lengthening is still a lengthening with pain, spasms, etc. etc. There is a risk that we do the epiphysiodesis which is a much simpler procedure but the guess about the amount of growth she has left is off and we need to do the internal lengthening after all.

The beauty of Mary getting older is she makes the decisions. She has decided to have the epiphysiodesis done on December 10 at our favorite hospital. Once I heard that I felt like it was the right decision. 

We are continually reminded that the treatment for FH isn't an exact science which can be difficult at times but so far we have been lucky with the treatment so far. Wish us luck with this one.

Surgery Date

Dr. Scott's surgery scheduler called on Friday. We are scheduled for surgery on December 10 at our favorite hospital (yeah!) for:

• at least the removal of the 8 plate in her left leg. 
• If we decide to do the Epiphysiodesis (which restricts the growth of her longer leg) it's not a problem to add it since we are already on the schedule.  

We will know the definite plans at our next appointment in September.

Unlike past surgeries where I pro-actively called to schedule I was bummed to get the call.

The surgery date is a week before she is out of school for the holidays which I was bummed about but when I told Mary she wasn't bothered by it. It's nice that she is older. I can tell her what's going on and if she's not bothered I can let my own concerns go. 

This is the LAST surgery that we have been EXPECTING but I have learned to not expect it to be her last. 

It will be nice to have both surgeries: the leg lengthening and the potential Epiphysiodesis that we have known about for 12-13 years behind us.

8th plate 12 months later and next steps

Today we saw Dr. Scott. Mary had a standing x-ray done while using a 2 cm. block. 

Unfortunately, her leg wasn't as straight as he would like during the x-ray for a more accurate measurement.

8 plate - is straightening her short leg/ inserted 13 months ago

We discussed:

• the 8 plate is straightening her leg and he would like to keep it in until December 2015,
• He is guessing she has a 3.5cm difference and he would like to get her closer to a 2cm or less difference to minimize discomfort as an adult
• There are 3 choices:

1. do a 2nd lengthening using an external fixator- no one is really leaning towards that option
2. stop the growth of the "normal leg" which has been mentioned since she was diagnosed at 5 months old
3. use a new internal lengthening device called a Precice. He has used it about 4 times so far and it has been successful. He is about to use it on another patient and he will let us know how it goes.

Now that Mary is 12 she has a big say in what we choose. 

I asked about doing nothing and leaving the difference as is but he does not recommend that- bummer!!!

We will see him in Sept. to check in and do another standing x-ray using a 3 cm block, and a hand x-ray to check on how much growth she has left. She is projected to be around 5ft 4in. She is currently 5 ft tall. On average girls stop getting taller around 14-15 years old.

At the moment the plan is to have an outpatient procedure in December 2015 to remove the 8 plate from her left knee. She will be sore for a couple days afterwards. At the same time he would go into her "normal" right leg and stop the growth. She will be sore for a couple weeks from this procedure.

The only things that ever bugs Mary at times are:

• her ankle can be stiff in the morning,  
• her foot can get sore if she has been really active, 
• Interestingly, sometimes she can feel the nerve tingling in her foot like she experienced in her leg during the ex-fix lengthening. He asked her to tap above the area next time it happens and let him know what happens if anything.

PS: I just reviewed the choices with Mary and she is very mature. She sees the downside to all 3 options:

• another lengthening doesn't sound great,
• she is nervous about the Precice being so new, 
• she isn't sure about being shorter than she would have been and she may not want to do anything on her right leg, only the left. 

The nice thing is there are options and we have time to decide...