Saturday, May 31, 2014
Last week, Maddy had a CT scan of her spine. The purpose was to get a very clear picture of the structure of her scoliosis in order to plan towards possible surgery. As I mentioned way back in October in this post, our doctors here in Hong Kong thought that she may have a hemivertebra and if so, it could potentially be removed buying some time before we need to put in growing rods. Dr Mackenzie disagreed but ultimately, our doctors here "needed" to see for themselves.
Today, I picked up the CD with the CT scan and here are the images of Maddy's spine:
The verdict is, there is no hemivertebra. Several of the vertebrae are "wedge shaped" but there is nothing that we can do surgically to prevent or delay the need for growing rods. Apparently this kind of scoliosis is one of the hardest to correct, and it's further complicated since Maddy is so tiny. The doctors here need to take some time to discuss an approach to the surgery, but we may be looking at needing growing rods put in within 6-12 months. That is one surgery I am NOT looking forward to! Once the rods are in, it means surgery every few months to lengthen the rods until Maddy stops growing. We have our next appointment in August (since we will be in Australia for a month, leaving in 3 weeks time) so we will find out more then.
Maddy has been complaining of back pain more frequently and seeing these images worries me! Please keep her in your thoughts and prayers, as well as the rest of our family. Going into this is quite stressful and worrying. I know ultimately she is a tough cookie and we have to do what we have to do - but I wish we didn't have to deal with this with our three year old :(
Posted by Nicole at 10:55 PM
Tuesday, May 27, 2014
Shortly after the TVB Pearl documentary aired last September (the documentary is available on youtube through the linked post above), my friend Serene (the mother of Nathan in the documentary) decided to start the non profit organisation "Little People of Hong Kong". She has been working tirelessly together with some of the medical professionals who were involved in the documentary to get it off the ground and up and running. So far, there have been multiple events for parents, and she has also been involved in educational seminars for medical workers as well.
I'm so glad that Serene thought to start this organisation which brings together many families affected by dwarfism in Hong Kong. Being bilingual, she also has access to the local community which I am largely unable to communicate effectively with. I would have liked to have been involved more with LPHK but with a new baby, and now two kids with medical needs, time is scarce these days!
The website is at http://lphk.org/ and there are many pictures of Maddy there. Go and check it out. They are working on becoming a recognised charitable organisation and so will be able to give receipts for tax-deductable donations soon.
Posted by Nicole at 2:36 PM
Monday, May 19, 2014
Last month, when we were in Australia, we had Briella's dedication service at our home church. I did a little speech and thought I might as well share it here as well.
I am writing you this letter on the eve of your dedication. You are loved by so many people, and most of all, by your family – your daddy, your two big sisters and me. I always said that I wanted three girls, and God saw fit to give me that desire of my heart.
It took me a while to decide whether or not to have that third baby, afraid of repeating what was a traumatic pregnancy, afraid of having to relive some of the hardest days of my life. When we finally decided to have you, God gave us you quickly – maybe he knew I might change my mind.
At first, I was selfishly hoping and praying that you wouldn’t have the dwarfism that affects your sister, but I knew deep down that God knew the perfect child for our family, and that child was you. I didn’t know it at the time, but God has already used you so much to heal me from the negative experiences that I had previously. I don’t think that would have been possible if you didn’t have dwarfism.
It took us a long time to decide what to call you. Your daddy and I often have very different tastes! We both had lots of ideas but we couldn’t find a name we both loved. In the end, your daddy said he wanted a name with “El” in it so he could maybe call you “Ellie”. I wasn’t even sure if “Briella” was a real name until I looked it up and found that it is a short form of “Gabriella”, meaning “God is my strength” – perfect for you.
We knew your middle name, “Faith”, before you were born. You see, Lana’s middle name is “Joy” – she was given my middle name, as our first born daughter. We planned to use family names for all of our children, but when we were expecting Maddy, “Hope” was something we had to cling to through some very dark days so it was only natural to give her that name. Having started a new tradition, there were only limited names to choose from and your daddy and I (miraculously) agreed that we liked the name “Faith”.
Its interesting though, the bible says that “Faith is the substance of things hoped for” – “hope” is something that is often tentative and flickering, like a candle in a dark night, but “faith” has substance and a basis for strong belief. With Lana, we had the joy of having an easy and perfectly average baby. With Maddy, we had so many worries and fears that at first, we had to choose to hope. That hope grew and grew as we saw her doing things the doctors said she would never do, and as we grew in confidence in parenting her. With you, we never had many of the same worries – we knew that you would figure out how to use those fingers that do not bend. We knew you’d walk and talk – maybe a bit slower than other babies, but in your own time you’d get there. With you, we have faith.
We have many dreams for you. We hope that you will grow up to be sweet, kind and generous. We hope you will be loyal towards your family and friends, innovative, strong and positive. Most importantly, we hope that you learn to love Jesus. Ultimately, this is your life to live. We will try to give you the tools to equip you for whatever life throws at you, but you will need to make these decisions for yourself.
Whatever comes your way in this life, we will always love you and be there for you to support you however we can. We are family, and in the words of Lilo and Stitch, “Ohana means family, and family means no one gets left behind or forgotten”.
Posted by Nicole at 12:52 PM
Monday, May 5, 2014
So we're all still kicking, life is busy, I haven't forgotten about this blog. We have just returned from two weeks in Australia, which was amazing. We had perfect weather and had a great time with friends and family. While we were there, we had Briella's dedication service which was really nice but also highly unorganised.
Briella ended up being in the hip spica for a total of three weeks. That was an experience! Even being cast up to the waist wasn't enough to prevent Briella's stubborn feet from slipping back in the cast. She needed to be recast once. Her foot began slipping a second time but it wasn't bad enough to recast her again. After the spica was removed, she was meant to be either in leg casts, or little boots (like the ones with a bar that she had overnight after the surgery) for another couple of months, but our doctors realised that it was futile since her feet were slipping out almost immediately. Instead, we are back to taping and stretching. We have very frequent physio and orthopedic appointments which are focused on her stubborn little feet, but we have had ok results from the tenotomy, gaining around 20 degrees of movement in each foot. Her right foot is now quite acceptable, however her left foot (the more stubborn one) will likely need further work done on it.
While Briella's feet are taking up most of my time at the moment, my current main concern is Maddy's spine. We have been waiting since October for a CT scan to give us more information about exactly what is going on with her spine, and in that time it seems as though her scoliosis has gotten worse. In addition, in the past few months, Maddy has been complaining from time to time of pain in her back. Being only three, it is hard to know exactly how bad the pain is and whether it is related to her scoliosis or not. After contacting our spinal ortho, he advised us to come in for an extra appointment. The doctor there told us "given the degree of Maddy's deformity, I'm sure she is in pain on a daily basis, however she probably has a pretty high pain tolerance as well". That's not a nice thought :( He said though that he thought Maddy's pain was coming from her hips rather than from her scoliosis, however the orthopedic doctors who look after Maddy's hips disagree - they think it is spine related.
We (ironically) received the appointment for the CT scan for while we were in Australia (the only two weeks out of the last year or so that we were out of Hong Kong)! Fortunately they were able to reschedule it without waiting for another six(!!) months and the new CT appointment will be on May 20. After this appointment, the doctors will be able to have a clearer picture of the exact condition of Maddy's spine, whether or not there is the much-debated hemivertebra, and whether we need to start thinking about surgery.Our follow up appointment is on May 26, so we will get the report from the CT scan then.
Please keep us in your thoughts throughout this month as we learn more about Maddy's spine and what the plan is for managing it. Ideally, it's best to delay any major spinal surgery for as long as possible, as surgery restricts the growth of the spine. We will see what happens...
Posted by Nicole at 2:09 PM
Wednesday, March 12, 2014
(the above picture is Briella's feet with the physio tape that we were using to stretch her feet prior to her tenotomy)
This time last week, we were in hospital doing all the pre-op consultations for Briella's surgery last Thursday. It was a long day with lots of back and forth-ing. We saw a few orthos, as well as the anesthetist, the occupational therapist and the physio. We also had to get pre-op photos taken of Briella's feet. The plan was made to do the tenotomy with a local anesthetic as well as sedation, which would require an IV and also for Briella to fast from 3am until the procedure, which was scheduled at 10am.
With babies and surgery, I think that often the two hardest things are the fasting and the IVs, rather than the surgery itself. Babies can't go very long without milk, since their tummies are so small - and they don't understand why they are not being fed. That night, I attempted to wake Briella at 2:30am to feed her once more before she would have to fast, but she barely stirred.
As expected, Briella woke up starving hungry on Thursday morning and screamed for an hour before she fell asleep, exhausted. It was the first of at least 5 or 6 times for the next two days that she would scream herself to sleep :( I managed to take her to the hospital without waking her and keep her asleep until it was nearly time for the procedure.
The above picture is Briella in the smallest sized operating theatre gown that they had! I guess it would have fit an average 5 year old - it was double Briella's length!
As expected, they had great difficulty finding a vein for the IV and after four failed attempts, they decided to abandon it and to perform the procedure under a local anesthetic only. In retrospect, I wish I would have insisted on this in the first place. It may have spared Briella from the need to fast, and it would have spared her the pain of four failed IV insertions and the resulting bruises on her tiny hands. The things I will note down in case there is ever a "next time"!
It soon became apparent that the casts would not stay on, and therefore would not provide the best results from this surgery. Instead, they decided to make AFOs with a bar to hold the feet in position.
It was getting to the end of the working day when Briella's main ortho decided that even in these boots, her left foot, which is the more stubborn, was slipping out of her AFOs. Since the orthotic department was closing, we were given one more night of home leave and asked to come back on Friday morning.
On Friday, we came back to the hospital and spent over an hour trying to modify the AFOs in such a way that they would stay on her feet without slipping. Each effort required stretching and manipulating Briella's ankles which were still tender from the surgery the previous day. After exhausting all options, her ortho asked me what I thought about the spica cast. I told him that we better do whatever will give us the best results from the surgery - and while I wanted to avoid the spica cast, I know we do what we have to do.
Putting the spica cast was pretty traumatic for poor Briella - it took about an hour of stretching and holding her little feet to get them into the best position. As the cast goes over both the knee and the hip, it is far less likely to slip and therefore will hold her feet in a better position as it heals.
The first night was pretty rough, with Briella being very uncomfortable and waking every hour or two all night long. She has slowly adapted, however, and is sleeping much better now. She still is far more fussy than usual and I think it is hard for her to get rid of her gas. Before, we would bend her at the hips and rub her tummy, and that would most effectively get it out. Now, her hips are locked in a mostly straight position so she can't bend.
The cast will stay on for about four weeks, at which time we will have to remove it as we are travelling back to Australia for easter. It's not recommended to fly in a spica cast as flying causes you to retain a bit of fluid and swell. Hopefully we will have good results and by that time, the AFOs will stay on Briella's feet better. She will need some casts/splints/AFOs for the next four months, full time, and after that she will need to continue to use them for nights and naps.
Diastrophic club feet are stubborn and often have a high recurrence rate. This is just the start of Briella's "journey". Fingers crossed for a good outcome!
Posted by Nicole at 1:13 PM
Wednesday, February 26, 2014
Unlike Maddy, Briella has been "blessed" with the typical diastrophic clubbed feet.
Currently, the most effective treatment for clubbed feet is the Ponseti method, which involves casting and re-casting the feet weekly to stretch the tendons and ligaments into their correct positions. Diastrophic clubbed feet are not typical, but this kind of casting can help. There are a few problems with casting diastrophic feet, however - the feet and legs are tiny tiny tiny, and most diastrophic clubbed feet have very tight Achilles tendons causing the feet to point downwards. These issues combined can make it very difficult for casts to stay on the feet.
For the past three months, we have been using an adapted method which involves using physio tape to pull the feet into a better position, along with stretching the feet multiple times a day. Using this method, we have seen some improvement, however it is not reasonable to expect that this method could produce the same results as serial casting. Our ortho thinks we have achieved close to the limit of success with this method alone.
Many kids with tight Achilles tendons (including kids with diastrophic dysplasia) can benefit from a tenotomy. This procedure involves snipping a bit of the Achilles tendon in order to loosen it. We've known for a while that this would be a necessity for Briella - and at our appointment with our orthopedic surgeon yesterday, he scheduled it for next Thursday, March 6th.
As far as procedures/surgeries go, this is very minor (I'm not even sure whether to call it a "surgery" as she will not need a general anesthetic even - she will have sedation and a local anesthetic). There is even a youtube video of the procedure which I will not link here. After the procedure, the casts will be better able to stay on Briella's feet as the feet will be able to be "flat" rather than pointing down.
While this will hopefully be great for Briella's feet (and therefore her future ability to walk), it will provide some logistical issues as currently, I mainly carry her in an infant carrier which probably is not suitable for use with casts. We might have to start using a stroller more, which provides other logistical issues - but such is life in Hong Kong!
Please keep us in your thoughts and prayers over the next week or two as we prepare for the procedure. Hopefully we will have great success, and the casts will now be able to stay on Briella's little legs. It shouldn't be terribly painful or uncomfortable for Briella, however she might not like the idea of having the casts!
Posted by Nicole at 11:13 AM
Saturday, February 8, 2014
It has been a long time since I updated the "Maddy's Story" page on this site - last update was in May 2012 before Maddy's cleft repair! At the time, she'd only recently begun to walk, and I was stressing over her speech issues. We still weren't even sure whether or not we would have another child. It definitely was way past time for an update!
So if you'd like to hear our new, much brighter "medical story", go and check it out here.
Posted by Nicole at 12:53 PM