Tuesday, December 17, 2013

Three weeks

It has been a wonderful week at home since my last post. Briella is a sweet little newborn who mostly sleeps very well - although she can be a lazy eater. She's very easy going, and we've been out and about quite a bit. She snuggles into the Boba Wrap carrier (which I LOVE!) and sleeps almost the entire time we are out of the house. Now that she is three weeks old, she is more alert and enjoys observing whatever is going on around her.

Last Friday, December 13, we went back up to the hospital and Briella was formally discharged. We were given not one or two follow up appointments, but TEN of them. Such is life with a newborn with special needs! Because of the holidays, they don't start until after the new year - but January is going to be a very busy month for us!

On Friday, we saw our orthopedic doctor as well. The most immediate concern is Briella's feet. We plan to do some taping to see if that will help, and in the new year we will probably start casting. Both of these involve manipulating the feet into a more "neutral" position, and then using tape or casts to hold the feet in that position. This will be repeated weekly or so, and hopefully over the long term, the position of the feet will improve. 

When Maddy was discharged from NICU, it was also early December. This is the peak season for colds and flus, and as such, the doctors recommended that she have the vaccine for RSV. RSV is the main cause of pneumonia and bronchiolitis in young children, and children who are at risk (such as those who like Maddy and Briella have airway malformations and restricted lung capacities) are advised to have the vaccine. It's not a true vaccine which causes the body to create antibodies, but rather an infusion of the antibodies themselves. Every season, a child needs five shots and when Maddy was born, these shots would have been paid out of pocket - for a total cost of HKD$40,000!! (about $5,000USD/AUD). At the time, we couldn't justify the expense. Fortunately now, that cost is covered by the public health system here in Hong Kong (only for at-risk children) and so Briella will be able to get those injections for free and we can sleep a bit easier this flu-season! (particularly since this time last year, Maddy was hospitalised with bronchiolitis). 

Friday was a busy day - in addition to our time in the hospital, and also attending Lana's school Christmas concert, my parents and sister arrived in Hong Kong to spend the holidays with us! It has been wonderful seeing them again, and for them to meet little Briella. Every day I am amazed at how blessed we are that she is healthy and strong and HOME! 

Last week, we took Briella to have some professional photos done. I'll finish up this post with some of my favourites :)

Tuesday, December 10, 2013

Two weeks old

Today, Briella is two weeks old. It is crazy how fast the time has gone - but in other ways it seems as though she's been with us for far longer than that! We are loving having her at home, but it is exhausting! She is still learning to eat and so feeding her takes a lot of time and energy. On top of that, I'm pumping breastmilk for her as well, which also is demanding. I'd forgotten how much work a newborn is - I haven't had a newborn at home for over five years, since Maddy came home much later. It is all worth it though! 

Yesterday, we went up to the hospital again - I thought it was to be discharged, and for some reason I was expecting to get up there, talk to the doctors straight away and then leave within a couple of hours. It wasn't anything like that though! I arrived at 10am and was promptly told to wait outside of the ward since it was not visiting hours. The nurse told me that she would call me by 3pm to let me know "if Briella had to stay, if she was given more home leave, or if she would be discharged". Not what I was expecting! I posted on Facebook and had a few comments about how the nurse sounded nasty, and in some ways it seems that way - but it is just the difference in culture here. Sometimes it sucks - but she wasn't being cruel or rude, just following protocol and letting me know all possible outcomes. Regardless, it was very impersonal and lacked any kind of warmth that you might get in a Western hospital. 

To cut a long story short, after a few hours, Briella was given more home leave and so we have to go back up to the hospital on Friday to repeat the above experience. At least this time I know what to expect! I think if all is well, she will be discharged then. She is doing very well at home, but she is still not yet back up to her birth weight and I think that is the main thing they want to check again. The feeding issues can make it harder for little ones to put on weight - so we need to keep making sure she's drinking enough every day!

In other "news", Briella's ear started "bubbling" yesterday. Most kids with diastrophic dysplasia develop cysts on their ears within the first few weeks after birth. They stay swollen for a few weeks and then harden, forming "cauliflower ears". The cysts can be treated by a variety of different methods, including compression or draining the fluid. When Maddy was in NICU, hers were treated primarily through compression - you can see some of her head bands in this post here. For Maddy, it kind of worked - her cysts are mostly flat, but they do have a bit of scar tissue in both ears (you can see the end "result" in the header of this blog). For Maddy, however, the compression bandages ended up forming a pressure sore behind one of her ears, and also started misshaping her head, so the doctors decided to stop. We'll see what happens with Briella's ears. She was born with perfect ears so hopefully they don't scar too badly!

Here's a few phone-pics for you to enjoy:

Maddy's NICU file is kept on Briella's bed - the doctors and nurses had to review her case in preparation for Briella's birth. Briella owes Maddy big time - I'm positive this is the reason why Briella had a far "easier" time in NICU than Maddy did.


This is what happens when you have older sisters ;)

Sunday, December 8, 2013


They say that a picture says a thousand words. So here is how our afternoon went yesterday:

Briella was given home-leave on December 7th, my due date, at only 11 days old. We will go back to the hospital tomorrow morning to be formally discharged. Life with her at home is exhausting, but wonderful - just as I knew it would be :)

Friday, December 6, 2013

December 6

On December 6, 2010, we brought Maddy home from hospital.

On December 6, 2013, I went in to the hospital to find Briella without a feeding tube, and taking all of her feeds orally!

Since we got up quite late, I didn't have a chance to talk with the doctors today so I don't know when discharge will be, but I hope and expect it to be very soon. I'm confident that she is ready to come home and that we are ready for her to be at home :) I fed her an entire bottle tonight and she is a LOT easier to feed than Maddy was when Maddy was first discharged! 

It was really hard to leave Briella today - I think partly knowing that we are so close to the end - and also (as you can see from the pictures above), she was SO alert. She's generally such a sleepy baby - as many newborns are - and I don't know if I've seen her this alert and awake before. I just wanted to stay and chat and cuddle with her... 

I'm very eager to talk with the doctors tomorrow to hear what their plans for Briella are - hopefully we'll be home together very very soon!! 

Nine days

When Maddy was born, she spent her first 124 days in NICU. Four months and two days. She was discharged straight from NICU and never spent any time in the special care baby unit (SCBU - an area for babies with less serious needs who are closer to being discharged).

Yesterday, I got a call from the hospital - they "graduated" Briella from the NICU to SCBU. So in total, she only spent nine days in the NICU! 

The day Briella was born, the doctor said to Bernard "Don't expect things to be the same as they were for Maddy. You have to realise that Maddy was our first case of diastrophic dysplasia so we were still learning. But now, Briella is our SECOND case!" - cracked me up... with one child, we doubled their experience ;) 

Briella is still in the hospital and continually doing better and better with her oral feedings. Two days ago, she could take 30mL, yesterday she took 45mL at one feed, and then when I was there, I fed her 55mL before she spat it up all over both of us ;) The goal is that she can take up to 60mL for all 8 of her feeds - however they may let her go home if she is doing well enough. Maddy was only taking about 70% of her daily goal when she was discharged three years ago. With the improvements that I see, it is entirely possible that she will be home next week. 

I keep pinching myself to remind myself that it is real. After our experience with Maddy, I hadn't even imagined that things could go this smoothly. Apart from one frustration with one of the nurses, I haven't had any issues with anything at all this time around - compared with last time where it felt like a fight from about day three... 

My parents and sister will be coming over to Hong Kong for Christmas - they get here on December 13. I think the odds aren't bad that Briella will be home before they get here :) That will be nice...

Wednesday, December 4, 2013

8 days old - oral feedings

Today, December 4, is the day that I was meant to deliver Briella if we hadn't rescheduled the cesarean to last week. Instead, we have a healthy 8 day old who is doing better and better every day. 

Briella is improving with oral feedings. Two days ago, she had her first bottle from the Haberman feeder. She didn't take very much but it was a start. Yesterday, I had some frustrations with the hospital/nurses because despite the afternoon shift nurses telling me that they would be consistently giving Briella oral feeds throughout the day, the nurse who was on duty yesterday at lunch told me that they hadn't been doing that. 

There are two "visiting times" at the hospital - the first is at 12-1pm, the second is at 3-8pm. Parents are not allowed in the hospital outside of those times. Because of the way Briella's feedings are staggered, if I want to be there for a feed, I need to be there either at 4-5pm, or at 7-8pm. Neither of those times are ideal for me when I consider the schedules of Lana and Maddy (I either miss dinner time or bed time), but I think it is important so I'm trying to be there for at least one bottle every day. 

After yesterday's frustrations, I went back up to the hospital in the evening ready for a bit of a fight - however the nurses who were on duty at that time were very supportive of our efforts in oral feeding so it wasn't much of a fight after all! I managed to feed her a bit, however she was very sleepy and didn't take more than about 10-15mL. 

Today, the nurses have been more consistently giving Briella oral feeds regularly throughout the day and when I went to give her the bottle this afternoon, I noticed a huge improvement! She now is actively sucking and swallowing, and it was much easier to get her to take 30mL even though she was very sleepy. 

I spoke with the doctors today (which hasn't happened very much - I seem to always miss the doctors rounds!) and they were very positive. I asked if we could try 100% oral feedings for 24 hours the next time that the feeding tube comes out and they said they would try that. We did that with Maddy and the first attempt was not successful (she was not drinking enough and after 24 hours they needed to replace the tube) but the second attempt was successful. Also, the doctors said that we should expect Briella to be home within one to two weeks!! 

If all continues to go well, they will move her out of NICU soon and into the special care ward - and from there it is only a short step from being home with us. I'm so happy that she has done so well and is so healthy - here's hoping that she continues to get better at oral feeding and she will be home in no time at all!!

(attempting to get a photo of my sleepy girl with her eyes open!)

Monday, December 2, 2013

Briella - 6 days old

The past couple of days, I've been really missing Briella at home and wishing that the girls could meet her. Today, I planned to ask the doctors bluntly what their plans for her would be rather than guessing and hoping and trying-not-to-get-my-hopes-up-too-much. 

I was happy to see today that Briella not only was off the oxygen tube (that happened yesterday), but they have also removed the IV! So currently she is not hooked up to anything apart from the monitors! Good job, Briella! 

I asked the nurse what the plan for her is, and basically, as I'd only dared to hope, they are going to start oral feedings and once she can take her entire feed by mouth, she will be able to come home! This morning, the occupational therapist started training and fed her 1mL by syringe. I asked if the nurses can continue to feed her orally at every feed, and if we could use the Haberman teat to feed her. The nurses were fine with that, so this afternoon, I brought it in and we tried it out - she took about 15-20mL by mouth! She needs to be able to finish around 60mL. 

I'm hoping that if she practices at every feed, she will be able to achieve this goal quickly so she can come home. The end is already in sight :) It could be a week, it could be more, but I'm pretty sure that she should be home for Christmas :) 

Saturday, November 30, 2013

Briella - 4 days old

Today, Briella is four days old. She is such a sweety and it is already getting hard leaving her in the hospital! The CPAP was turned off yesterday and she is still getting some extra oxygen via a little cannula under her nose. I've been able to hold her (yay!) and she loves snuggling. Yesterday she cried when I put her down at the end of visiting hours :( 

Medically speaking, things are moving along slowly. Briella has been seen by most of the specialists who need to evaluate her and the prognosis seems good. The two things that we are waiting for before we can bring her home (in my understanding) is 1) her breathing needs to improve a bit - she's still having some "insucking" around the rib cage when she breaths, meaning that she's straining a bit. She also has some stridor but chances are she'll have that for a while. I still think that her breathing is stronger than Maddy's was at birth, and the doctor who was looking after Maddy agrees. 2) she is still being fed by the NG tube. She needs to start feeding orally. I'm HOPING that this will start taking place next week. 

Apart from that, everything else, I believe, will mostly be done as an outpatient. The ENT is not insisting on an immediate bronchoscopy (unlike our situation with Maddy) and is happy to follow up with that later. Orthopedically, her x-rays of her spine don't completely rule out every issue, however they don't raise any major red flags either so we will be cautious and follow up with that later too. Her hips, like Maddy's, are not fantastic - however unlike Maddy, our current ortho sees no benefit in using a pavlik harness

Her feet are going to be a difficulty though. Maddy has fantastic feet for her diagnosis and I don't know why, I just assumed that Briella would too. Briella however has the more "typical" diastrophic club feet that often are very difficult to correct. At this stage, our ortho is planning to start serial casting for them when she is around a month old.

Briella is drinking more and more these days and so in the next day or two should be able to have her IV removed - yay for one less cord! We still haven't been given any particular time frame for how long she will be in, but we are praying and keeping our fingers crossed... it is exhausting pumping, looking after two older kids and running back and forth from the hospital sometimes multiple times a day. I can't wait until our family of five is together!

Thursday, November 28, 2013

Briella Faith

As the title of this post suggests, we have decided on a name for our little one: Briella Faith. "Briella" is a short form of the name "Gabriella" meaning "God is my strength".

Today, I will be discharged from hospital to recover from home. I'm doing very well - both physically and emotionally. The cesarean was not as painful as I'd imagined and certain aspects of the recovery are also significantly easier. When I was admitted to the hospital, I requested that I could recover in the prenatal ward rather than the postnatal ward. The prenatal ward has some postnatal patients as well, and I knew that it would be easier on me emotionally than being in the postnatal ward with 50 newborns. Thankfully they were able to accommodate my request as my hospital stay here has been so much easier than my previous two experiences!

Briella is still in the NICU. Like Maddy, her main issue is her breathing and airways. She had other medical needs such as cleft palate and clubbed feet (which are new to us, Maddy's feet are great!) But these are not what is keeping her in NICU.

It will take at least a few days or so for all the necessary specialists to see Briella and they need to consult with each other about the management plan. Although the doctor told Bernard perhaps only a few weeks, this is just a guess until we know what the plan involves...

In the meantime, Briella has started feeding through a tube, so I am pumping again. Thankfully my milk has started to come in so I'm able to meet her needs. Every time that I've seen her, she's been asleep. Since she is still on the CPAP, we are not yet able to hold her. Hopefully we can hold her soon! Also, until she is discharged, Bernard and I are the only ones able to visit her. Lana is quite upset about that, and for me, that has been the hardest thing so far :(

Please continue to pray for us, especially little Briella...

Tuesday, November 26, 2013

Welcome, baby girl!

After a lot of waiting, our third daughter (still unnamed) was born today at 1:17pm via cesarean section. She weighs 3.4kg and had apgar scores of 7 (1 minute) and 9 (5 minutes). She needed a bit of help starting to breathe due to wet lung syndrome (possibly due more to cesarean delivery than her dwarfism) and was taken quickly after birth the NICU where she was put on oxygen. Other than that, she seems healthy and strong. The doctors estimate that she may be home within 2-3 weeks which would mean she's likely to be home for Christmas! I haven't spent much time with her yet since I am still recovering from the cesarean. She looks so much like Maddy did at birth!

Thanks so much for your prayers, messages and other support. I will continue to update as we learn more.

Monday, November 25, 2013

The day before D-Day

Having a scheduled cesarean is a strange thing, knowing the birthdate of your child sometimes even weeks in advance. It's so crazy for me to think that by this time tomorrow, I'll have a newborn! 

Today, I had my last appointment with my private doctor. He confirmed that yes, the baby is still breech. She seems as though she's a big one, maybe 8lb or so already. The estimates aren't always accurate, but based on the scans, it seems as though if I'd gone another week or two, she would have been my biggest! That might be the reason why she just didn't want to turn around. He said "Seems like she wanted to be born by cesarean!"

After a great lunch with three of my closest friends here, I headed up to the hospital for a few hours for the last pre-op things. I met with the anesthetist again who confirmed that they will try the spinal block first. There is a chance that it may not take due to my spinal surgery and if that's the case, I'll need a general anesthetic :( I'm praying that is not the case! I also saw the obgyn and got some blood taken in case I need a transfusion (it's rare but better safe than sorry!) 

Tomorrow morning, I need to be up at the hospital at 7am. I am the 2nd surgery of the day, so generally speaking, it will be possibly around 9:30-10am. Emergencies take precedence over scheduled cesareans though, so it could be later than that. I guess we won't know until tomorrow for sure... 

Usually with cesareans at this hospital, you need to stay 4 nights afterwards, so I probably will be in until Saturday. Depending on how I'm doing and how the baby is doing, I might stay shorter or longer. If she obviously needs significantly longer in NICU, I'd prefer to recover at home. If she is doing great and can come home say after 5 or 6 nights, sometimes they allow the mother to stay longer so they can go home together. We'll play it by ear.

I'm very nervous about tomorrow - both about the procedure and recovery, and also about the health of the baby. If I could have one "wish", I'd wish that she could come home with me. How likely is that? It's impossible to say. She'd need to be healthier than Maddy was in order for that to happen... It wasn't until a day or two after that I'd packed my hospital bag that I realised that I hadn't even packed any clothes for her :( I have now - in faith ;)

I was talking to Lana yesterday, trying to prepare her for the birth, my hospitalisation and the possibility of her maybe not meeting her sister for a few weeks. She told me in a wistful voice, "I hope the baby can come home quickly because I REALLY want to hug her..." Made me cry - me too Lana... 

Please be praying for us, and especially baby, tomorrow - and we hope to update soon with good news!

Thursday, November 21, 2013

26 November

Two posts in one day! I haven't done this since Maddy was little!

Yesterday, I posted about my experience with the ECV. Since it failed, we will need to schedule a cesarean. Yesterday, the hospital gave me the date of December 4 which was a week after the doctors had verbally advised me that it would be. I had some concerns and so I emailed the doctor.

My main concerns were these:
- if I went into labour naturally and the baby was still breech, I would need an emergency cesarean
- emergency cesarean is not preferable for the mother OR the baby and the risks are a bit higher than if it were planned (just depending on how "emergency" it truly was). 
- since the baby already has known health issues, it would be preferable to have the team of doctors ready and prepared in advance, rather than using whichever doctors were on call at the time. 
- (selfish reason) - if I had an emergency c, Bernard would not be allowed in the theatre and I would be alone for the birth of the baby. While it might not be as big of an issue in a routine pregnancy, we don't know how her breathing will be until those first few minutes and I would not want to go through that without my husband.

In addition to the above, my due date according to LMP is December 7 - however according to all of my early ultrasounds, the baby has consistently measured between 6-10 days ahead, putting my due date by ultrasound to somewhere around November 27 to December 1. 

So as mentioned in my previous post, I contacted the doctor last night and he replied within a few hours saying he would review my file and get back to me. This afternoon, I had a call from the hospital advising me that they have moved the date to November 26. 

So unless a) I happen to go into labour within the next 5 days, or b) the baby happens to turn within the next 5 days, we will be meeting our little girl next Tuesday. 

Please continue to keep us in your thoughts and prayers - this is the end of one journey, but the beginning of another...

Maddy update

It's been a long time since I updated about my 2nd daughter! Much of this blog in the past few months has been focused on my pregnancy and the potential medical needs of my 3rd daughter. But I did want to update a few things about this gorgeous little one:

Maddy is growing well and still is extremely healthy. It has been nearly two months since we have seen the orthopedic doctors and we are still waiting for a CT appointment for her spine. I think the stress of this pregnancy (which is no where near as bad as the stress of my last pregnancy!) has taken my attention off Maddy's needs in some ways, because I'm sure I'd be more worried about it if I weren't focused on the new little one. I've figured though that the CT scan would at least give us more information about her spine and whether or not the suggested surgery is viable. Even if the suggested surgery is not viable, more information cannot be a bad thing! 

With regard to the DAFOs mentioned in the last orthopedic post (linked above), we have had some made - however they are not the hinged kind that I was expecting. The kind we have are thicker at the ankle and are extremely difficult to buy shoes for. Instead, the orthotist added a sole to them so she can wear them without shoes. Maddy has currently been wearing the new orthotic about half the time and continuing to wear her old orthotic insoles the rest of the time. Here is a picture of her funky new "shoes" (taken before the sole was added onto them) ;)

Since September, Maddy has been going to not one, but two new schools. It has been a hard transition - it took a few weeks for her to stop crying at drop-off time, but now she is mostly good. She prefers her regular school to her special needs school, but her special needs school is helping us so much at this critical age with her self-help skills. She has been working on things like pulling up her pants and putting on socks. Given that her fingers are inflexible and her arms barely come to her belly button, this is a bit like you or me learning to do these tasks with our elbows!! She has been experimenting with a few different methods and a few different tools. All in all, I think these schools are perfect for her current needs so it's worth the busy schedule and the (few) tears that still pop up from time to time. 

The other week, Maddy had an appointment with the cleft team at the dental hospital. She had back to back appointments with the dentist, orthodontist and oral surgeon. She was AMAZINGLY good - opening her mouth wide when asked, following instructions, allowing them to scrape the plaque off her teeth, even allowing them to stretch her lips out to take photos of her teeth. I wasn't expecting her to be so compliant - I know that when we took Lana to the dentist earlier this year, Lana wasn't anywhere near as compliant, and she's usually my easy-going child! We had all the staff at the hospital talking about how good Maddy was and they have never seen a child this young with that much ease. I think a lot of it has to do with the number of doctors appointments and therapy sessions that she has had - it's been her whole life really! I'm so proud of her :) (and her teeth are crowded, but they are healthy strong teeth. We'll start worrying about the spacing issues once she starts getting her adult teeth!)

Wednesday, November 20, 2013

ECV results

Today, I spent the day in the hospital attempting to turn the baby from breech to head-down. It was a full day with a lot of monitoring and a lot of waiting! The doctor usually does two attempts, but because we are a little more "high risk", he only tried once. The baby didn't turn so is still breech, but thankfully we didn't experience any complications and so did not need an emergency cesarean today.

Because the baby is still breech, they have scheduled a cesarean for December 4. I'm a bit concerned that it is too close to my due date (only 3 days "early", usually they do it 1-2 weeks early) - I don't want to go into labour and then need an emergency cesarean which will be more risky for me and the little one - plus Bernard will not be able to accompany me. I've contacted the doctor to ask if it is possible to move the date closer but I'm not holding my breath - I'll see what he says. 

All in all, I think I'm glad we tried. If we hadn't, I probably would have wondered "what if"... although of course since she didn't turn, it does feel in some ways like a waste of a day, and unnecessary discomfort as well - but we would have never known if we hadn't have tried.

There still is a (small) chance that the baby will turn by herself before the scheduled cesarean, in which case they will cancel the surgery and wait for her to come naturally. We'll just have to wait and see what happens. It is surreal to think that in just a couple of weeks, she will be with us!

Tuesday, November 19, 2013

NICU - what I'd do differently

The other day I posted a summary of our experience with Maddy in NICU three years ago (ironically, she was released exactly three years and one day before my due date with this little one!) It's not often that people get the chance for a "do-over" for an experience like this, but in a way we do. Having been through NICU once and getting to know the ins and outs of it all, I think I know better now what to ask for, what to insist on, and in general what I'd do differently. Also for the hospital, Maddy was their first diagnosed case of diastrophic dysplasia so they (hopefully) are now more prepared and informed for a second case. 

A few weeks ago I met with the neonatologist and had a couple of requests for him. I'll summarise them and his response below.

Firstly, and most importantly (in my opinion), I do not want to use the orthopedic doctors at this hospital. About five minutes down the road, run by the same hospital authority, is a pediatric orthopedic hospital. They also run a spinal clinic. ALL of the specialised pediatric orthopedic doctors, AND all of the specialised spinal orthopedic doctors are based out of this hospital instead of the hospital where we gave birth. When Maddy was born, I didn't realise this at the time, but she was seeing a general "adult" orthopedic doctor. I have requested that we use Maddy's current pediatric orthopedic doctor instead. This will give us a more accurate picture of the state of this little one's orthopedic issues, and in particular, her spine. The neonatologist informed me that within the last year or two, ALL of the NICU orthopedic issues are referred to this pediatric team rather than the general orthopedic team - so hopefully that makes a big difference for our little girl!

Secondly (and this one is maybe a little selfish on my behalf), when Maddy was born, she did not need any emergency breathing assistance. For her first few hours, she was breathing independently. I've asked that if this one is similar, if we can have even 15 minutes with her in the delivery room before she is taken to NICU. The neonatologist's response surprised me - he said of course, and that I could even try breastfeeding if I wanted! Maddy on the other hand was on an IV only for her first few days and didn't start taking any milk even by NG tube until she was about 3 days old! In fact I wasn't even cleared to attempt to breastfeed until she was over three months old.

Speaking of breastfeeding, another big thing for me is the way that the hospital manages the breastmilk. They say that they support breastfeeding but many of the policies imply otherwise. You can read some of my previous frustrations here - but in short, I want to insist a) on 24 hours notice for any change in feeding schedule so I can provide them with the right amount of milk in the right number of bottles, and b) absolutely no formula ever for whatever reason. With the formula, Maddy was fed formula multiple times for different reasons. On some occasions, it was because of a change in feeding schedule (eg, going from feeds every 3 hours to every 2 hours). On other occasions, it was simply because a nurse forgot to defrost a bottle. While I am not anti-formula in general, it bothers me greatly that they were giving this milk which is dairy-based to a child with a dairy allergy. I don't know if she reacted to it back then because I wasn't there, but when she was older she had strong reactions to dairy (including formula), producing a lot of mucus, sneezing, some swelling of the face etc... These issues could have been disastrous when combined with her airway issues! The doctor was supportive of my position - however I also know that what the doctors say is not always what the nurses follow through with.

I haven't asked this yet, some things I think we are better off waiting to see what they say first - BUT if we are in the situation again where the hospital is not actively treating this child but rather waiting for her to outgrow her stridor, I want her to be discharged and followed up as an outpatient instead. I wanted this for Maddy too, but first time around they were far more cautious, and we weren't prepared to discharge her against medical advice. This time, however, I will definitely be much more strong in requesting this. 

In addition, I feel as though Maddy was fed via NG tube for far too long. This often leads to speech delays because the muscles in the mouth aren't stimulated as much as if they were feeding via breast or bottle. Speech delays have been our greatest developmental struggle with Maddy and she's had to work very hard to catch up! With this baby, I do not want her to be fed via NG tube as a precaution. If they feel she needs to be fed via NG tube for medical reasons, I think they should at least do a swallow study to "prove" the medical need for it - particularly if it is to be for more than a week or two! 

A big thing for me is the visiting hours. Visiting at this NICU is highly restricted - parents only, and ONLY between 3pm-8pm. Anyone with children aged 3 and 5 would know that this is the worst time of day. Lana gets off the bus at 3:10, then we have about 3-4 hours in which to play some games, have a snack, do a bit of homework, piano practice, violin practice, eat dinner, have a bath and get into bed. Ideally, I would like to go to the hospital either when the kids are in school, or when they are in bed at night. If this little one is in NICU for only a week or two, I think we can manage. It won't be easy but I can be flexible. If it is for four months however, it would be HUGELY detrimental for our other two kids... I would hope that the hospital could be accommodating, however knowing them, I know it's not likely. I recently learned that a friend of a friend requested to see his daughter outside of visiting hours in a one-off situation and was rejected - and she passed away only 2 or 3 days later :( I honestly don't know how to approach this issue with the hospital - again I will see what happens first... 

After I met with the neonatologist, he said to me "Don't expect that just because you had one experience with Maddy that it will be exactly the same with this little one as well" - he said it in a positive way, that maybe things will be easier this time around. But coming from the same man who, when I asked him "If we had to do it over, what would you do differently during Maddy's stay in NICU", told me "Absolutely nothing" - I'm really not sure what to expect. In addition, he will be away for the whole of December so unless I have the baby in the next week and a half, he won't be there anyway.

I'm really hoping that we won't have a repeat of our last NICU experience - but I guess we will have to wait and see. We are more informed now, as is the hospital, so fingers crossed we will have our little girl home as soon as possible :)

Friday, November 15, 2013

Maddy's NICU summary

I'm planning to write two posts - this one will be a summary of our experience with NICU with Maddy, the next one (which may or may not appear at any time before the baby is born) are things that I would do differently this time around - IF the situations were more or less the same. 

(For siblings with diastrophic dysplasia, there can often be differences in severity - and we don't know until this little one is born whether she will have the same issues as Maddy or different ones. Often siblings are similar - but not always. We are of course hoping and praying that this little one will be just as healthy as Maddy is, or even healthier!)

Here goes!

When Maddy was born, there were a lot of doctors in the room prepared for whatever the situation would be. At the time, we didn't know her diagnosis. We didn't even know for sure that she would not have a lethal form of dwarfism, although the scans towards the end of my pregnancy looked promising. As soon as she was born, she was very briefly shown to us before she was whisked away to special care baby unit for close observation/testing. We were left in the delivery room for around three hours after this due to the hospital being short staffed. 

After we did leave delivery and I got settled in the ward, Bernard went up to see Maddy. In her first few hours, she began to have some stridor (noisy breathing), insucking (where the ribs are struggling to inhale enough air) and blood acidosis (where she was not expelling enough carbon dioxide effectively). She had been moved into the NICU and was on CPAP. She did very well on the CPAP and her reliance on it was decreased over the next few days and was turned off when she was 9 days old. After that time, she had occasional use of oxygen but was never really reliant on it. We didn't get to hold Maddy until she was 12 days old, and we were only allowed to visit her between 3-8pm. Since we had another child at home who also needed our attention though, realistically we saw her on average for 1-2 hours every day.

Many people ask me why Maddy was in NICU for so long - particularly given that she was healthy and growing, and really needed minimal medical support in the four months that she was there, and the answer is quite complicated. There were two main issues:

1. Airway
From birth, Maddy was showing definite signs of either a narrow airway, or a "floppy" airway. The typical signs were the stridor and insucking. Her lungs were working fine (apart from the acidosis during her first 24 hours) but the airway was a concern. If an airway is dangerously narrow, even a bit of mucus could block the airway completely causing the person to suffocate without prompt and emergency intervention, sometimes in the form of a tracheostomy (where the person breaths through a tube in their neck). A "floppy" airway (tracheomalacia) is less severe and usually babies grow out of it without any intervention. It is important to diagnose exactly which you are dealing with and the severity - and usually this is done via a bronchoscopy.

2. Cervical spine
When Maddy was two days old, we were told that she looked as though she might have a cervical spine instability. It was difficult to diagnose via x-ray and the orthopedic doctors wanted to do an MRI to assess it further. Cervical spine instabilities are also tricky things as if the neck is moved in the wrong way, it could press on the spinal cord causing, as the hospital put it when Maddy was 2 days old, "instant death". 

The problem was that for the bronchoscopy, Maddy would have needed some manipulation of the neck and the orthopedic doctor would not give the ENT the ok to do this necessary test until they had first done the MRI. The MRI on the other hand would have required Maddy to be sedated, and the anesthetist refused to sedate Maddy without knowing the results of the bronchoscopy.

Reading back through my blog posts (if you're really interested, you can read from August-December 2010), it is amazing how early these issues started - I first posted about them when Maddy was only 9 days old. From the age of even a week old until Maddy was discharged at four months old, there was little resolution of these issues and no real plan besides "let's see how she grows and hopefully she will be better when she's older". 

So what changed when she was discharged? They did manage to figure out that they could do a "virtual bronchoscopy" by using a CT scan instead of the usual bronchoscopy procedure. This confirmed that all along she'd had a floppy airway rather than the "worst case scenario". In addition, I think that Professor Sillence's visit had something to do with her being discharged just three days later. 

And as for Maddy's cervical spine issues? After she was discharged, we were told that she never had the instability. But despite this, the same team of doctors gave us the same trouble when Maddy was being assessed for her cleft surgery at 17 months old. Just a week later, however, our orthopedic doctors assured us once again that Maddy's spine was perfectly stable and safe.

On one hand, I am thankful for NICU in that Maddy was in the safest place she could be IF the "worst case scenario" ever happened. On the other hand, however, I have a lot of dissatisfaction in particular with the orthopedic team at this hospital. Also I think the hospital policies here in Hong Kong are harmful to families with children in hospital, particularly long term. This time around, however, there is much that I would ask to be done differently. I feel as though I'm a lot more educated now, and I'm also a lot braver. But more about that in a later post...

Wednesday, November 13, 2013

ECV decision

After much thought and discussion, we've decided to go ahead with the ECV. It has been crazy since my last doctor's appointment and I've been going back and forth on the issue. I emailed the dwarfism experts and they took a few days to get back to me (understandable, since I emailed on a Friday evening!). In that time, I thought I'd decided not to do it - why take the risk to the baby? And we would never have a guarantee that it would be 100% safe. Yesterday, I was ready to call the hospital and ask them to cancel it and just book the cesarean - but last night, I heard back from the dwarfism specialists. 

I was kind of hoping for them to say "No don't do it" - it would have made my decision a whole lot easier if they had! I had been preparing myself for a cesarean and could even see the positives in it. But they said basically if it is done gently and in the right conditions, it should pose no risk of spinal injury to the baby (the main concern specific to DD babies). 

Even after hearing back from them, I was not sure what to do - I'd already made up my mind not to do it. Maybe I should just go with my gut? However today I went for a check up with my wonderful obgyn and discussed it with him. He basically told me that if I have a senior doctor, discuss the concerns with him and have only gentle manipulation of the baby, it should be fine. I know already that I will have a high-risk senior doctor so hopefully I'm in good hands.

So next Wednesday (Nov 20), I'll be spending a full day in the hospital to see whether or not the little one will turn. There is a small chance of complications which would mean that she will be born by emergency cesarean on the same day. Then it's not far off 50-50 whether she will turn or not. If she turns, then we wait and she could theoretically be born as late as December 17 (10 days after my due date). If she does not turn, we will schedule a cesarean for the week of November 25. Whatever happens, I think I'll be glad that we at least tried to help her to turn... 


I also want to say thanks for those of you who are praying for us and sending us supportive messages and thoughts. It is greatly appreciated. Some things to pray specifically for are:
  • For peace in the whole situation. I'm mostly doing ok but getting a bit anxious about the delivery, recovery and hospitalisation. 
  • For the best possible outcome for the ECV. If she's meant to turn, that she'll turn. If cesarean is safer for her, then that she won't turn. That there will be no complications - I'd really prefer NOT to have an emergency cesarean but if that's what is best, then so be it. 
  • For our little girl's breathing once she is born - for her to be able to breath strongly and independently without any extra support.
Thanks once again!!

Sunday, November 10, 2013

ECV or no ECV?

On Friday, I had my 36 week check up at the hospital where I will be delivering this little one. As I knew that the baby was still breech (very obvious when there's a hard head sticking into your ribcage!), and since two doctors had previously advised me that it is not recommended to turn the baby manually, I was expected to come away with a C-section date which would be cancelled if and only if the baby turned naturally.

Instead, I came away with a "plan C" - the doctor who I saw (who is also a prenatal diagnostician who I saw in my pregnancy with Maddy) does not think that manually turning the baby (ECV) will hold any more risk than any other pregnancy, and it may be my best chance to avoid having a cesarean. 

So what is an ECV? If you are really interested, you can read in depth on Wikipedia. Basically, I would be admitted to the hospital early in the morning and they would carefully monitor the baby for 2-3 hours. Around lunch time, I would get some drugs to relax the uterus and a doctor would manually attempt to rotate the baby until she is head-down. After this, whether it is successful or not, I'd need to stay for another 2-3 hours in the hospital to monitor the baby in case of distress or other issues. 

And for the numbers: having an ECV holds a risk of 1-2% of immediate emergency cesarean due to complications such as damage to the placenta or cord, or fetal distress. There's around a 60% or so chance that it would work, in which case hopefully she'd STAY head-down and I'd go on to be able to deliver naturally without the need for a cesarean. And there's around a 40% chance that it would not work and I'd have to go ahead with the planned cesarean. 

(and yes, I realise that technically that all adds up to 101-102% - but I said "around 60%" and "around 40%"... ;) )

Currently, my ECV is booked for November 20 - only 10 days away. I'm still not 100% sure if it is the best course of action - mainly because I've now had two doctors tell me that it has additional risk to the baby due to the dwarfism and two doctors tell me that it does not. I've emailed Dr Mackenzie in the US and am hoping that he has an opinion one way or another. I also have another check up with my private doctor this week and will discuss it again with him as well. I can cancel the ECV at any time for any reason and go straight to the cesarean.

If we DON'T do the ECV (or if we try and it doesn't work), then we're looking at a cesarean the week of November 25 or so... just over 2 weeks away! On the other hand, if we DO try it and it works, this little one could be born as late as mid-December.

I'm finding that the closer that I get to the end, the more anxious/nervous I get about the delivery and hospitalisation. Please keep us in your prayers, particularly throughout the next month, as I'm sure it will hold many many ups and downs for us! Pray also that we will make a wise decision about the ECV - or better yet, that the baby will turn by herself and we won't have to even think about it any more ;)

Sunday, November 3, 2013

Best possible outcome?

I know that this baby has dwarfism and I am completely fine with that. It's funny that now that I have Maddy, my concerns about the long-term future of this baby are minimal. Most of my worries are the immediate, and a lot have to do with NICU. 

If we could have the best possible outcome from here (in the short-term):
  1. the baby would turn and I'd have no need for a cesarean (I really don't like the sound of the recovery from that!)
  2. when she is born, we'll find that she has no cleft palate and can breastfeed so I don't have to think about pumping again.
  3. her breathing would be efficient and regular, needing no assistance such as CPAP or oxygen.
  4. I'd be able to bring her home from the hospital with me.

What is the likelihood of the above?
  1. Possible - but I'm not really holding my breath for it... Some babies, even with DD, can turn as late as right before delivery. It is rare though - but not impossible. 
  2. I think of the above, this is the least likely. We haven't been able to see a cleft on the ultrasound, but we never saw Maddy's either. Cleft palate often goes along with a small jaw and restricted airways - and we already know she has a small jaw.
  3. I think this is possible - Maddy really needed minimal assistance, and I'm pretty sure that this daughter has a larger rib cage than Maddy did - so... fingers crossed!
  4. At times, this seems like a far off dream - I go between trying not to get my hopes up too much, and reminding myself that I need to prepare to come home alone again. 
Please keep praying for us as we wind down to the end of this pregnancy - there are a million emotions going through our minds at the moment. In some ways, having been there before makes it easier - it's not our first time at the rodeo! In other ways, we are not fresh this time around, we are a bit more battered, a bit more scarred - bluntly, I didn't agree with the way Maddy was treated in NICU back then and I don't want to go there again. Maybe I'm bitter, maybe I just plain don't trust the system so much. I'm sure if I'd had a chronically ill baby who needed high tech medical care, I'd be thankful. But I'm not - I'm resentful. And the closer we get to being there again, the more I see my own negative feelings and emotions about Maddy's experience...

Tuesday, October 29, 2013

Delivery options

Going into pregnancy for the third time, you would think I'd have the delivery stuff down pat - but life is not always that simple!

I already suspected that my spinal surgery in May could complicate things - in particular, with regard to whether or not I could have an epidural or not. In both of my previous deliveries, I had an epidural and I wasn't keen to do it without - particularly given my past need for induction which brought on labour painfully strongly and quickly! 

Yesterday I had an appointment with the anesthetist to discuss my options. She has recommended that I try not to have the epidural for a few reasons - first of all, having the surgery increases my risk of complications from the epidural; and secondly, the scar tissue which would have formed around the similar level to where they would put the epidural would make the epidural "unpredictable". Basically, I could have the epidural and possibly have little pain relief from it if the scar tissue blocks the flow of medication to those nerves - or maybe worse, I could have asymmetric pain relief, eg, pain relief to only one side of the body, or perhaps pain relief to the upper abdomen but not the lower abdomen. In short, the benefits are (potentially) reduced and the risks are much higher for me now. 

Now I'm not actually that scared about labouring without anesthesia - however I AM more worried about the way the hospital's policies make you labour. With induction, in my experience, they were very insistent that I stay in the bed at all times and very strict that I had continual fetal monitoring. With Maddy, the midwife freaked out when she saw my file and demanded that I lie in one position, not even allowing me to roll to my other side since the monitors kept losing Maddy's heart beat! Since the it's likely that I might need the induction again (judging from past experience!) I know I will have to fight in order to be mobile during labour - particularly if I'm going to try without the epidural. I'll also need to have better strategies for natural pain relief.

In addition, this baby is STILL breech at 34.5 weeks! I remember reading in my pregnancy with Maddy that it was common for babies with skeletal dysplasias to be breech since their legs are shorter and kicks are weaker, so it is harder for them to turn. I went back through my blog and found that Maddy had turned by around 30 weeks so I'm starting to worry about this little one! I've asked my DD support group and many of their little ones were breech too.

Up until today, I had assumed that they would do an ECV to manually turn the baby, however I had two doctors today (both my private obgyn and the public hospital prenatal diagnostician) tell me that they would not recommend it for a baby with skeletal dysplasia. 

So basically, if the baby does not turn within about 2 weeks, I will be scheduled for a cesarean. Here, they schedule the cesarean between 38-39 weeks to reduce the chance of the mother going into labour and then requiring an emergency cesarean. So for me, that would probably be the last week of November. 

I'm not thrilled with the thought of having a cesarean either - it does have pros and cons though. Firstly the pros: I think if she is born earlier (but not premature), her chances of being home for Christmas are greater. In addition, I AM able to have a spinal anesthesia so it kind of solves the pain relief situation. I like the idea of scheduling it in some ways, particularly with the added stresses of having a baby with special needs. The hospital will be more "prepared" for her arrival. And given the choice of elective cesarean or emergency, the elective cesarean is far more preferable for everyone involved.

The cons - the big one is the recovery. I had great recoveries the previous two times and was up and walking immediately. Particularly with Maddy, I was going in to the hospital every day (sometimes multiple times) and really forgot at times that I'd just given birth. I know the recovery from a cesarean is slower and more painful, generally speaking. Also, I think at this hospital, they don't "allow" you to walk for 24 hours after the surgery - so it will be more difficult for me to go up and see the new baby who presumably will be in the NICU. In addition, a cesarean means a 5 day hospital stay as opposed to a 3 day stay - and I don't relish the thought of being in the hospital for a minute longer than I need to be! 

All in all, I feel less prepared for this delivery than I did with either of my previous two, and so much depends on whether or not the baby turns. I guess we'll have a completely different experience either way - and I'm not sure whether I like either one of them!

Please keep us in your thoughts and prayers - and I will try to update more frequently with news of this little one, who could be with us within the month!

Monday, October 7, 2013

Maddy's orthopedic update

Three months ago, when we were in the US, we had an appointment with Dr William Mackenzie, an orthopedic surgeon who specialises in skeletal dysplasias. He had a few suggestions as to how to treat Maddy's feet, hips and spine. 

In Hong Kong, we see not one orthopedic team, but two. Our spinal team deals with all spinal issues, and the pediatric team deals with everything else. After we returned home, we brought Dr Mackenzie's thoughts to these teams to hear their thoughts.

Our pediatric team was completely on the same page as Dr Mackenzie. For her hips, we will continue to observe them as she grows - her hips are not great, both the head of the femur (thigh bone) and the socket of her hip joint are misshapen but for now, she can use them and any surgery could theoretically worsen that. When she is older, she will need a hip replacement, maybe around age 30 or so. For her feet, our orthopedic doctors agree that the current orthotics don't provide enough support for Maddy's ankles and so this week, she will be fitted for dynamic AFOs.

Dynamic AFOs, pictured above, are basically an "ankle-foot orthotic" with a hinge at the ankle - that way the ankle can bend forwards and backwards, but not sideways. Maddy sometimes has a habit of walking on the inside of her foot, even when she is wearing her orthotics (the TVB Pearl documentary picked up on this and made it look like she always walks like this! She doesn't, only sometimes. But regardless it's not good for her ankles and these will provide better support for them). 
Onto the other area of concern: Maddy's spine (see this post for a lovely x-ray of it!). Dr Mackenzie's opinion was that we should start casting Maddy's back to potentially delay the need for surgery. Eventually she would need growing rods to support/correct her scoliosis but the best thing would be to avoid that for as long as possible. The spine has been the major concern for me and was the cause of a nice little meltdown the other day.

When we arrived back in Hong Kong, I saw one doctor on the spinal team. I discussed Maddy's case with him and he recommended against the casting for various reasons. I mentioned Dr Mackenzie's thoughts to him and asked if he could contact him and his response was "I could contact him but it wouldn't make me change my opinion anyway." This response bothered me - but as he was going to refer me to maybe the most senior doctor on the team, I figured I would just let it go and see what the other guy had to say.

I was really excited to meet with this senior doctor - he is very well respected, is a professor and has made some amazing and exciting contributions to spinal surgery. The appointment with him went very well, I thought.

At our first appointment with the spinal team back in January of last year, a doctor there mentioned that Maddy had a hemivertebra. At that stage, it was just a "kink" and the scoliosis had not yet developed. What is a hemivertebra? Have a look at this picture:

Basically, a hemivertebra is when only half of the vertebra develops. As a result, you see this kind of a "kink" in the spine and the spine then curves into a scoliosis so that the body isn't held at this strange angle.

Since the hemivertebra had been mentioned before, I didn't question it when this professor brought it up again. Basically, his plan would be within the next 18 months or so, to remove the deformed vertebra and fuse the two vertebra either side of it together. Doing so would remove the source of the scoliosis and theoretically, it would improve the curves in Maddy's spine. He also commented that casting was not likely to be effective in the upper thoracic region where Maddy's main curves are, and that this was our best option at the current time. We planned to do a CT scan to get a better picture of the hemivertebra and to be able to prepare for probable surgery.

I left the appointment feeling quite positive - of course I dread the thought of spinal surgery, but this kind of surgery is far less major than putting rods along her entire spine, and it is a plan - I like to have a plan! I neglected to ask this doctor if he could consult with Dr Mackenzie as I thought I would go home and ask his opinion on this plan myself.

I emailed Dr Mackenzie and waited for his reply. When he wrote back, he told me that he does not believe that Maddy has a hemivertebra, that he would still recommend casting, that he would avoid surgery for now and that he would not recommend having the CT scan as he was quite sure it was not a hemivertebra anyway (it is best to avoid unnecessary CT scans as each scan contains the same level of radiation as something like 100 x-rays). He also confirmed that casting was probably not going to correct the curves in Maddy's upper spine (where her major issues are) but that it was likely to have a positive effect on her less severely curved lower spine.

It is a hard place to be, stuck between two differing opinions of two highly qualified doctors. Not knowing exactly what to do, I emailed our doctor here in Hong Kong with Dr Mackenzie's thoughts and asked if he could please consult with him as to what the best plan would be. I also had probably my biggest melt-down since I was pregnant with Maddy (can I blame pregnancy hormones again?) As a parent, I just want what is best for Maddy and sometimes it is hard to know exactly what that is. Our spinal team here are very good at what they do and they are highly specialised in the spine and in scoliosis. They don't, however, have a great amount of experience in diastrophic dysplasia. Dr Mackenzie on the other hand has maybe more experience than anyone in diastrophic dysplasia - however he deals with the whole body - spine, feet, knees, hips... I figure that if these two experts can communicate together we'd have the best of both.

Ultimately, though, Dr Mackenzie is not our doctor - he is just consulting with our doctors here. And if they (for whatever reason) choose not to avail themselves of his expertise, we cannot force them to communicate with him. I think that our doctor here may try to contact him though - I sincerely hope that he does.

After a few days and a lot of thinking, I've realised though that we still have time to figure this out. As much as I'd like to know the plan right now, the simple fact is this is still up in the air. As for casting, knowing that it will not really help her main curve that much makes it less urgent to me - I am happy for now to leave that alone.

Maddy may or may not have a hemivertebra. The CT scan definitely would help to clear that up so I think if these two experts cannot agree, we would be foolish not to proceed with the CT scan. As to what that shows and what the outcome will be there, we'll figure that out later. "Wait and see" - that seems to be the story of our life!