Hypochondroplasia - limb lengthening - surgery

The plan for Fi was to have surgery and lengthening on all 4 segments of her lower limbs at the same time, this halves the time spent in wheelchair, hospitalized, out of school, doing daily physio, etc. along with improving the overall proportionality.

Based on the size of her femurs and the lack of angular deformity, we knew she could get the internal fixator, the Precice Nail in her femurs. This means no bulky external fixator, no pin site infections, minimal scarring.

These can't be inserted into tibias of children still growing though, so she had to get externals on her lower legs, Taylor Spatial Frames (TSFs) which also helped with some minimal angular corrections that she needed.

A couple months before the surgery we traveled to meet with the surgeon and his team who discussed the plan with us in detail, we got to ask all our questions. Fi had a number of them as well and they all got addressed.

I think the thought of your child in pain is the most daunting for people. It certainly was for me. I will write about this in detail, but I can say that other than a few bad minutes during therapy she has only taken Tylenol following the surgery, once a day. Slept through every night and has been in a great mood - so all our expectations have been surpassed.

The surgery took nearly 10 hours and Fi's surgeon was able to do everything he planned to do. She had a wonderful anesthesiologist who listened to her tonsillectomy GA story and decided to change his approach and went with the TEVA approach for GA. They also did 4 peripheral nerve blocks at the very end of the surgery to manage pain. She woke up smiling in no pain.

She spent 3 nights in the hospital's pediatric ward, where they have a fantastic group of nurses, who know all about fixators and post op care for ortho patients. Every 12 hours we would get a new nurse and I always felt that nurse was the best nurse I have ever met in my whole life. Wonderful experience throughout.

Once the nerve blocks wore off, she was briefly put on a PCA on day 2, but it was making her super drowsy, so that was promptly stopped, she was given 2 stronger painpills (opioides) but we realised we didn't really need them, so we stopped and we went to our accommodation, the local Ronald McDonald House (more on them later) on Tylenol.

During the hospital stay the biggest things were for us to get used to the fixator, learn about pincare, learn the PT exercises we had to do several times a day from day 1 on and learn to transfer to the wheelchair from the bed and back and to the commode. The inpatient PT and OT came to see us every day and they were full of great advice.

Next chapter.: our experience and helpful hints fr the distraction/lengthening phase.

Hypochndroplasia limb lengthening 0

We tarted researching limb lengthening quite early on, it was something that we spent years talking and reading about, along with meeting with doctors and connecting with families whose children had it done.

I realize it's not something everyone might choose to discuss or consider with their child. I am very respectful abut everyone's beliefs and convictions and I have never engaged or will engage in a discussion abut whether limb lengthening is right or wrong. I respect that it's a personal decision.

The next couple posts will be quite factual about our experience.

I realised quite early on that if we wanted to consider limb lengthening, it would have to be done at a centre of excellence. A place where they do this surgery and treatment all day long, every day. There are 2 centres in the US who have vast expertise for limb lengthening for achondroplasia and hypochndroplasia.

It's a big undertaking, following the surgery when the bone is cut, the fixators are secured, you start to lengthen and can go about 1 mm per day, so to lengthen 5 cm in the femora and tibiae which was our goal would take about 2.5 - 3.5 months. There is no weight bearing during this time, so your child is in a reclining wheelchair with their legs extended in front of them. During this time you have to stay near the hospital to attend the daily physical therapy and regular check ups with the doctor.

This is followed by the consolidation phase which also takes a couple months when you can go home, kids can attend school, things become easier, but you still do therapy every day. After this, the fixator is removed, I am sure you continue PT as well to regain strength and correct gait.

We knew that we wanted to do the first leg surgery as young as possible which was 7.5 for a mature girl which Fi is.

We spent years figuring out the insurance, financials, basically the logistics, taking time off work, what we would do with the other 2 children, etc. I had lists of the lists of things we had to do, have ready etc.

Meeting Dr Paley

Let me preface this by saying that I have watched a ton of videos, read a lot of publications and presentations and have spoken to a number of patients and parents of Dr Paley. So I pretty much thought I had an idea about who we would meet. He actually surprised me and was a lot nicer and lot better communicator than I had expected.

This is a picture of Fi during the consultation before she actually fell asleep (it was a long wait).

So Dr Paley spent an hour with us, he first explained the protocol for lengthening and then he answered every single one of my 19 questions and more, in great detail, never making us feel that he had done this a million times. He actually made me feel like he liked parents who ask questions which is a huge bonus in my eyes.

There are a few differences between his approach and the one discussed in Baltimore:
- He prefers doing the arms after the second leg lengthening
- He would do surgery for girls as young as 7 not at 8 as they can be emotionally more mature
- I think he would do internals in the tibiae at the age of 12ish even if the growth plates haven't completely closed

He was very helpful when it came to discussing non-US treatment options mentioning several different options.

He examined Fi very well, by now I can always easily tell how much any doctor knows about HCH just based on what they examine and how and he was clearly very aware of her condition and what needed to be assessed, unlike all orthos we have seen in Ireland.

All in all it was a great visit, we will see him again in 2 years and I think we will then make a more precise plan.

If anyone is interested in my 19 questions, please e-mail me and I am happy to share more detail on a more private forum.

Study comparing internal and external fixator use

Small but interesting study here

Paley video

I just realized that I forgot to share this. Very informative, although from before the internal nail times.

Cochlear implants versus limb lenghtening

Fascinating article in The Economist about cochlear implants, I can so relate the debate to the whole topic of limb lengthening discussions I have seen lately.

Also interesting to see the wider debate and critique of the article on Irish Deaf Kids.

In 10 years

My posts seem to come in floods, like everything in life. I haven't posted for a while and it's not because I haven't been thinking much, I have, tons, about the final post about long term decision making in the ELL series, about schooling, about my education plans. Life has just been too busy with birthdays, school end, medical appointments, holidays. 

I got my new passport a few weeks ago, it is valid for 10 years. I stopped to pause as it is my first Irish passport, which is kind of a huge thing. What will we do, where will be at 10 years from now? The very first thing that came to my mind is that Fi will have had all her limb lengthening procedures by then or will just be finishing the last one. That was the very first thing. Maniac, scary, hopeful, call it whatever you want, it is my reality. There is an end to this tunnel where she will be able to do all that her peers can and that's what matters to me. 

What I think of limb lengthening - part 2

Please don't read this post without reading the preface. These are my thoughts, and I have these thoughts for my child, Fiona. I don't believe for a second that my way, our way is the direction everyone else should follow.

My child is not her medical condition. Fi is a toddler, a young girl; she is a daughter, a sister, a cousin, she is daddy's girl. She is a person, she is a human being, with intelligence and emotions, both of which she has so much of, she can hardly manage them at this stage of her development as she has entered the terrible twos. She may be shorter because of her medical condition, but every millimeter of her screams of personality and originality. If she didn't have the physical limitations, she would certainly go into gymnastics, she has a remarkable ability to copy her older sister's performances.

She is NOT a dwarf, a little person or an LP. She is not a label. She happens to have a medical condition which impacts the way her bones develop in her body. She is a person with a medical condition that impacts her growth. I may use the term dwarfism sometimes, but there won't be any other nouns used by me to describe her condition. I don't call the man who had a heart attack "the heart-attacker" (or heart-attacked). I don't call a boy with autism, "the autist". I don't call our friend with diabetes, "the diabetic".

Do I like her medical condition - no, quite the contrary, why should I? Do I like the impact it has on her body, not at all. I don't understand why this question is even a question, would a mum whose child has CP like the impact the condition has on their child, or Perthes disease, or any other physically limiting condition.
I haven't made up my mind yet if dwarfism is a disability, it certainly constitutes to it in Ireland, for now, I will use the term physically limiting. Does this mean that I don't love or accept my child for who she is? Not at all. This argument against ELL always baffles me, I can still love my child and accept HER, but want to change the impact of her medical condition. There is no contraindication here. I am not my body, my body is part of me, but I have my mind, spirit, soul. And there are lots of things I don't like about my body, it doesn't mean that I don't like myself.

Fi also happens to have a very prominent squint, so we went to the ophthalmologist and she got glasses, she may also need surgery to correct it. I would be considered a negligent mother not to pay attention to her developing eyesight. I think her squint is quite cute actually, if it wasn't causing her heath issues in the long run, I might not be concerned about it.

 We also discovered that she can't hear very well because she has middle ear dysfunction, so she has had 2 surgeries for that so far and will have many more. Of course I love her for who she is, but I want her to have the ability to see and hear, so she needs to undergo treatment. But didn't God create her with the squint and the middle ear dysfunction, don't these belong to her identity and therefore shouldn't be changed? The list could go on.

To me it is as simple as that. I consider the fact that she may never be able to clean herself after going to the toilet, because her arms are too short, part of her medical condition. The fact that she wears 6-9 months trousers at the age of nearly 2 and is nowhere near being able to walk any significant distance is also the result of her medical condition. She is developing tibial bowing, the same goes for that. Yes, I think her legs are adorable and absolutely kissable, but chances are they will cause her pain and discomfort. It is not a cosmetic issue to have lordosis and back pain, or to struggle with elbow extension and reach for life.

These medical symptoms to me don't constitute an identity which should be assumed by her, which should become her, because she was randomly born with HCH. These are all medical issues, and rather significant ones at that. So as Fi's mother, it is my job to do exactly what I do with the squint, the hearing, and every other ailment, any of my children have: to do my research and become knowledgeable about the different treatment options so that I can make a well-informed decision for her, with her. Thankfully, we are entering a new era of ELL, the internal devices will make a huge difference in the safety, pain levels, complications, etc.

In the next part I will write about my dilemma of conditioning, brain washing and children's ability to make long-term decisions for themselves.

What I think of limb lengthening? - Preface

This is part one of a series, I have been thinking about writing for a very long time.

I am a diplomat; I like getting along with people, I appreciate the freedom of choice, beliefs and speech and I am acutely aware that my truth is just as subjective as anyone else's.

This changes rapidly if someone becomes my friend and I care about them, in this case, I tend to have a misguided moral duty to tell them my version of the truth, to question and challenge them at times. I don't call many people my friends and they don't get a sugarcoated version, they get brutal honesty, Eastern European style. My friends love me for this and I am lucky to have a few such friends myself.

So, I have been struggling with this whole controversy around limb lengthening, reading the different parent forums, so many speak up so strongly against it, and I just don't feel it's my place to start a heated argument with any other parent. In fact, I don't want to have an argument at all as this is such a personal, belief system shattering/challenging topic. But this is my blog, and this is my place of honesty, this is my heart-wrenching, soul-searching evolution. So I hope none of my readers will start an argument as I am just not up for it, this topic is a lot more personal than it would allow an argument.

So where do I start?
Firstly, I know my limitations, I know that I don't understand many of the other parents. We are so different culturally, our understanding about the relationships of society and the individual is so different based on where we were born and raised. Our inbuilt moral values, our understanding of right and wrong is different, I suppose this is what makes life interesting.

Secondly, I don't do the judging thing, when I say 'each to their own', I absolutely mean it, my not-understanding does not result in me believing that I am right, it just results in me acknowledging that we are all different and what is righter than right for me, can be completely wrong for someone else.

Thirdly, I believe that whether or not we are seeking out treatments for our children, (whether it's GH treatment, Biomarin's new drug or lengthening), or if we choose not to do anything, we all do this with the utter conviction in our hearts that we are doing the best for our children.

Fourthly, I am a research geek, through and through, I need to read a lot, analyse and think through things a lot more than most people I know. For me, having an overwhelming pool of data offers comfort, I am very brain driven and I believe that knowledge is power, so reading arguments which are not research based is hard for me, but my intellectual arrogance is kept in line by knowing that as parents we all want the best for our children.

And last, because I treat everyone with respect, I expect the same, whether anyone agrees with my choices or not, I hope all can believe that I have done my homework and I am doing my very best for my child, I can't do more, but I certainly won't do less. This can be very hard if your approach is the opposite, but it only takes remembering that our view is all subjective. Unless we get into the realm of religion and believing in the absolute truth, in which case, I still think that even if you believe in God and the absolute righteousness, your own version of this will be tainted and subjective by who you are.

OK, so that was the longest preface ever written, in the next part I will explain why I am FOR limb lengthening and in the third part I will discuss the topic of the child's choice.

Leaving Baltimore

Saturday was the save a limb ride, a perfectly organised event. We did the one mile walk with our friends. One of the girls who did the walk has ACH and had one leg lengthening. It was amazing to see how well she could walk,run, jump, dance.
My friend's daughter is such a wonderful, beautiful,intelligent kid. I surely wasn't so pulled together at the age of 15. I would never think that she has HCH. It was amazing to see all these children and their families. There wasn't too much small talk, I learnt soooooo much, will take weeks to distill it all.
Fi obviously has a great sense for drama as she decided to take 40 steps on her own at the fundraising dinner. I will post a video and write more about this next week.
We left B.more believing in a better future. We saw the reality and it was an awful lot yo take in and digest,but our trip has been extremely worthwhile and I don't say that lightly,it's not a walk in the park to travel with such a young child.
We have seen the doctor,met some new friends and also some old ones,who gave us a tour of Baltimore and DC.
The part I struggle with most is the consumerism that I have seen here, I forgot... It's pretty disturbing, will write more about this no doubt

Meeting Dr S.

Was exactly the way I imagined. I love doctors who don't shy away from questions, and he did not. He gave detailed knowledgeable answers, measured correctly and was pleasant and I could see that he genuinely cares about his patients.
He did not have any ortho concerns and said that the bowing of her right tibia is very mild, borderline infantile bowing.
He was very specific about the ortho complications, differences between HCH and ACH. I really can't fault him.
Hospital facilities are amazing, very easy to go to PT, pool, accommodation.
Support staff seem to be rather inefficient and I met a few weird characters. I did not realize how hard it must be for the mums to be here for months, being so close to so many others who are in a similar situation. It seems very involved,maybe I couldn't do it. At the same time I can really see the benefits as well.
The biggest take away for me is not to try and look after Fi on my own, should we take this route. since the grandmas aren't working, they could help. Yes that could get a bit tricky too, but I think she would really benefit from someone else being around as well. Both for emotional and physical support.

Baltimore

Arrived in Baltimore  fine. Fi did amazingly well, hasn't even whimpered. Thank God for inventing nursing. Bmore is a lot nicer than I thought. Trees and nice people everywhere. The hospital accommodation is fine, I can see how it could get pretty lonely and overwhelming when staying here for months. Just met my hero friend, E. whose daughter will have her final lengthening next week. I am all emotional with the jetlag and seeing kids with fixators. Will see the doctor in an hour.

My heroes

My heroes are 2 mothers that I have met online, will call them E. and C. for the purpose of this blog. They have 3 kids each (I think) and one of their daughters has HCH and ACH respectively. The girls are in their teens and have had their first leg lengthening and arm lengthening already. These women and their daughters will probably never know how much their e-mails, blog posts, FB messages have helped me over the last year. They give me hope and guidance for so many of my questions.
These mothers and their kids are real fighters, they haven't given up, they haven't let this diagnosis knock them out, they and their kids couldn't follow the "accept and live with it" route - please understand I am not dismissing this route, I fully appreciate that it is what most Western people are comfortable with. I admire people who can take something that's seemingly negative and make a positive thing out of it. (That's just not me, I am the worst pessimist in the world, my husband has said to me that I should become a worst case scenario adviser for the Pentagon... and I think I might do quite well at the job :-))
But it's hardly the road we will choose to follow. A very wise man who I think actually opposes ELL has said to me so well that in his opinion the choice of ELL is an extremely personal one, it is so dependent on the cultures and traditions you were raised in. It's almost like the question of religion, people exhibit such strong views on it.
I think Western cultures are a lot more individualistic, so I can see how being your own person and everyone being different are values that people admire. Yet to me the choice to allow my child to live life like anyone else seems so natural. So I am keeping an open mind, I am continuing with my research...

New internal limb lengthening device

I have meant to write about this before, but weren't sure if it would be used for HCH/ACH patients. I just found out yesterday that in fact this new lengthening device can be used for second and third lengthening. This is extremely exciting news: much less pain, only one small incision so no more pin site infections. It is a new device so I am certain that over the next few years there will be lots of best practice gathered around the use of it.

http://www.paleyinstitute.org/?q=node/47

http://www.lifebridgehealth.org/Main/PressReleases/Sinai-Hospital-Surgeons-Codevelop-First-RemoteCont-354.aspx

Body proportions

I am now fascinated by body proportions, I guess it has never occurred to me before how these change throughout childhood, I would have just said, "yea babies are all head when they are born and then that changes".... To be perfectly honest, I don't even know what the HC was of my other 2 kids, I am sure it was measured at the hospital, but it was never measured again and I certainly didn't take note of it. 

The length of my kids has never been measured either except at birth, I always just know what size clothes to get them when they are about to outgrow the previous set...


Anyhow, here is a little bit of what I have found out over the last few weeks.... 


The average length of a baby at birth, however, is 20 to 21 inches (50-53 cm). Most infants reach 1 ½ times their birth lengths (adding 9 to 10 inches) during the first year. 


Babies usually double their birthweight in four or five months and triple their birthweight by one year. - This I did know I have always been compulsive about weighing all my kids, to be more precise breastfed babies typically weight anywhere between 2.5 times to 3 times their bw by the age of 1. 

Now to the proportions.... Infants do not look like small adults.Their body proportions (relative size

of body parts) differ from those of adults. For example, an infant’s head is about one-fourth his or her total

length, while an adult’s head is one tenth of his or her total height (more like one eight according to some sites...). Unlike adults, the forehead of an infant is wider than the chin. 

From birth until six months, the infant’s head is larger than the chest. In most six-month-old children, the

chest becomes larger. The difference in the distance around the chest as compared to distance around the head continues to increase with age.

Besides having a large head, an infant has a long trunk, a “pot-bellied” abdomen, and short legs. The gain in

length during the first year is because the trunk grows. The legs do not grow much longer at this time. (I am not sure how true that is for Fiona, I have been measuring her legs and they certainly grew quite a bit).


The abdomen sticks out because the internal organs are large for the baby’s small body. Because the center of gravity is high on the baby’s body, the result is poor balance. No wonder babies toddle rather than walk!

At birth, the average infant weighs about seven pounds and is about 20 inches long. He will measure 24 inches three months later. By his first birthday he will typically be 30 inches in height, and by age two, 35 inches. An average girl will attain half her adult height before her second birthday. 
Target height for leg lengthening is the (fathers height + mothers height) divided by 2 and then - 7 which is 162 cm for Fi. This is the way a girl's projected height is calculated, I think it's plus/minus 7 or 8; of course our height is not determined by our parents' height to the last cm since then all siblings of the same sex would be the same height...

The different parts ol the body do not grow at the same rate, or at the same time. Instead, the head develops first, the torso next, and the limbs last. At birth a baby's head is about 25% of of the total body length, or about 60% of its adult size An adults head is normally less than 15% of the total body length. 


I never realized either that most of the head growth occurs by the age of 2, it is mind blowing if you think about it...

The torso develops next and is about one-half of its total length by the end of the second year. 
The legs and arms grow rapidly from the second year to the fourth year, at which time they are about one-halt their adult size. - I guess this explains why hypo is noticed by this time if not before... 


This progressive growth rate (head, trunk. legs) is called cephalocaudal development.

An infant's behavior, as well as body proportion reflects a cephalocaudal, head-to-trunk, direction of development. An infant's earliest and best organized behavior is the nursing or sucking response, which is a behavioral illustration of the fact that the head has the earliest growth increase . However, the sucking response is instinctive. The child s first purposeful behavior stems from the shoulder and pelvic girdles. The infant controls his head before his neck, his neck before his shoulders, and his shoulders before his trunk it is later in infancy that effective movements appear at the elbows, wrists, knees, and ankles.

Visit with the orthopedic surgeon

We went to see the local orthopedic surgeon who does limb lengthening here. I don't think we would want to have the surgery here, as they can't possibly have as much experience as the US centres of excellence, but we had an opportunity to see him and I wanted to hear what he had to say.

 Similarly to Dr Paley he said the best age to have the first surgery would be between the age of 8-9. I won't go into the different methodology he uses and neither did he at this stage. But there was one interesting thing that he said, I guess my primary concern would be the pain involved (and of course the potential complications), whereas in his view the pain is controllable. He said that the hardest part was having to put the whole family's life on hold for months after the surgery. He also confirmed what I suspected, that everyone will have complications, which I guess reaffirms my feeling about wanting to go to  specialized centre...

Limb Lengthening thoughts

I hope this link is going to stay valid for a long time. I guess in my mind there is no real question whether or not it would make sense for Fi to have ELL done. However this article does walk one through the thought process for any parent and at one stage it almost had me convinced. I think it's always good to hear both sides of the argument.

http://home.comcast.net/~dkennedy56/lp_chap09.html

Limb Lengthening Story 1

This story is about a girl who has hypo and had ELL done.
http://www.washingtonpost.com/wp-dyn/content/article/2008/11/21/AR2008112101749.html?sid=ST2008112501915