Planning

Our travel plans are still not confirmed. It has been a bit of ring-a-ring-o'-roses lately! First of all, it was taking our doctor a while to decide whether or not he was going to sign the medical forms for us. Then I decided to look up prices and decide which airline we would fly - and we decided to fly a different airline to the one whose forms I had given to the doctor, so I sent him the new forms only for him to inform me he'd just filled out the ones for the other airline! After that, it took him another few days or so to fill out the form to the correct airline - at which point I felt confident making a tentative booking and submitting the paperwork to them. I initially was told that we could only hold the tickets for three days without paying but the medical clearance wouldn't be approved for another week at least - but after a bit of complaining and then talking to the right person, they decided to extend that for me so that I'm not having to pay for non-refundable tickets that the airline could potentially tell us that we are unable to use. After clearing the ticket issues up and submitting the medical clearance forms into them, it turns out that the doctor had filled something in incorrectly and we had to get him to fill it out again! He changed and signed his error and I resubmitted it, but the airline has told me that "(the) last email ... requested the Dr to fill a new one instead of correcting the old form. In case they don't accept this, I will request you to fill a new one."

All this back and forthing is a bit frustrating. I haven't yet asked the doctor to fill in a new form as I figure that I should have heard by now if the form actually was unacceptable - the first time around, I heard back in a matter of an hour, so fingers crossed! Our tickets are currently being held until Thursday, and the sale price is only valid until then, so I am hoping and praying that we hear back tomorrow or the next day! The airline may be able to hold the tickets for longer but I am unsure if they can keep the price the same for us - and if I compare to other airlines, they could go up by 40% after this sale! 

I am fairly confident that in the end, everything will go as planned. Bernard has already put in his leave from work and I'm already planning what to pack and the logistics of it all.

So we SHOULD be back in Australia from Easter until the end of May! About six weeks in total. We all really need this break, so please help us pray that all the paperwork lines up and that Maddy will be able to fly safely :)

Genetic Alliance

A couple of weeks ago, I was contacted by someone from the non-profit organisation Genetic Alliance. I presume that this person came across Maddy's blog and invited me to participate in a survey they are currently doing. They are surveying families with children with congenital conditions that were diagnosed either prenatally or in the first year of life. They are surveying not only parents, but also other relatives and caretakers too. I know there may be other families who are reading this blog who may be interested in this survey - my opinion (and one of the reasons why I started this blog) was that as we share our experiences, hopefully we can support some other families who are yet to go through the same issues!

If you are interested in the survey, you can read more about it here.

Eating and other news

We first introduced Maddy to solids last month. It has been a long process for Maddy, I think mainly complicated by the cleft palate. For the first few weeks, she would just spit the food out. Also, the food would often go up through the roof of the mouth into the nasal cavity causing her discomfort. She would then sneeze and all the food would go out her nose (and THAT can't be pleasant!)

I did a bit of research online trying to learn about how to successfully feed babies with cleft palates their solids and got a few good suggestions - one was that it is better for them often if the solids are not so watery, that way they can control it better in their mouths and swallow it instead of it going everywhere. (As an interesting side note, other people suggested making the food MORE watery so that if it goes out the nose, it is not as likely to get stuck and will not cause discomfort - and while that may work for other cleft palate babies, it didn't work so well with Maddy). Another suggestion was instead of feeding her in a sitting position, to feed her at a bit of a recline so that gravity helped the food to go to the back of the mouth where it could be swallowed. Both of these tips seemed to help Maddy a little, but we were still struggling.

It wasn't until last week that we had another appointment with the occupational therapist responsible for Maddy's oral motor training that she suggested a particular special needs spoon called the Beckman EZ spoon that we really saw major improvement. This spoon wasn't designed for use for cleft palate kids - but given the size and shape of the spoon, it is very easy to get small amounts into the sides of her mouth where she can easily swallow it. Immediately she went from eating very little to finishing entire bowls of solids! Although she still struggles with anything which has any texture, preferring very smooth purees.

Some mothers of babies with clefts have commented that their children didn't start eating great until after their cleft repairs - but since Maddy's cleft repair will be delayed until she is about two years old, we need to learn to work with it. (I don't want her eating purees until she's two!) The occupational therapist suggested getting a palatal obturator made to close the cleft so she can eat without food going into the nasal cavity, so we have a referral to dentist for that.

In other completely irrelevant medical news, last week we had the MRI follow up appointment with the orthopedic doctor. We got there and he said "I'm sorry but we don't have the official report yet. I'll have to take a look at the slides myself", then after another 20 minutes of him playing around on his computer, he said "I'm sorry but it looks like the network is down so I can't get a very clear picture of the slides!" It was a bit frustrating, particularly after waiting for over an hour for that news! But the good news is that from what he COULD see, and what he could tell from doing a physical examination of Maddy's hips, they appear to be quite well-engaged now so we don't need to do anything different with them (unless the MRI showed something concerning, which he said was unlikely). We don't need to follow up with him for another six months, but when we do follow up, we will be taking another complete set of X-rays then. 

And for completely irrelevant NON-medical news, I'm still waiting on the doctor to get back to us about the medical clearance for flying back to Australia. He has the forms that we need to submit to the airlines but hasn't had the time to really go through them yet. Until he does that (or at the very least, we know he will - or we know someone else who will), we're not booking tickets. But we're still hoping to be back maybe around Easter time until the end of May. But nothing is set in stone yet...

And here are some pictures of Maddy's first swing at the park :) (doesn't she have the CUTEST leg dimples??)

Maybe not stuck after all?

In the last few weeks, I have been looking into holiday options that didn't involve flying. I had the brilliant idea of booking a cruise back to Australia and then cruising back to Hong Kong, hence taking the risk of flying out of the equation. However on discussion with Bernard, we came to the conclusion that it just wasn't a viable option, we'd have to be out of Hong Kong for five months since there are no regular cruises between HK and Sydney - and it is just too long for Bernard to be out of the office.

On Saturday, I was feeling pretty disappointed about all that - and since I've been a little confused about the whole "Maddy can't fly" thing, I decided to write to Maddy's NICU pediatrician for clarification on a few issues. To make it a little simpler, here was my email:

Dear Dr (name),

I wanted to ask you some more questions about Maddy and flying. I realise that via email may not be the best way to ask so if you prefer, I can call you or make an appointment to see you some time.

Is the main concern about flying Maddy's laryngomalacia? My understanding is that she has no problem with her lungs (although perhaps a smaller than average lung capacity?), only with her airway - is this correct?

If so, why is the reduced pressure of an airplane cabin a risk for her? Wouldn't it be an issue if the pressure was increased (ie, if she was going scuba diving) rather than being decreased?

Also, what medically needs to happen in order for Maddy to fly? Are you waiting for her to be stable for a longer period of time? Or are you waiting for the laryngomalacia to be completely better before saying that she can fly?

Thank you for your time,

Nicole

A day later, I got this response:

Hi Nicole,

There is actually no definite guidelines on flying for patients with upper airway obstruction.  I agreed that Maddy's lungs are fit for flight, it is just that the airway pathology has not yet been clearly defined.  Probably the reduction in airpressure to 8000ft and the reduction in partial pressure of oxygen should pose not much risk to Maddy. The reduced humudity may have some effect. Please just make sure she get her medical record clear and present this to the airlines to see if they need to prepare anything specific (they have additional oxygen via nasal canula).  The other main concern for them is whether their flight would be affected.

The chance is Maddy should be able to fly uncomplicated.  There is no particular risk of decompensation, but just in case Maddy gets some more airway problem (just like when she is on land) e.g (1) when she vomits (because of the change of airpressure, they have middle ear discomfort etc), (2) when the sputum becomes thicker due to the reduction in humudity (20-30% on board), the crew are confident to deal with this.

In summary, I would support you and Maddy to take the flight earlier than what I have recommended you, provided good preparation has been made.  It will be safe.

Best regards,

Dr (name)
 

So with this new information, we have been looking into the possibility of coming back home soon! Although just to be safe, we need to organise medical clearance forms for Maddy so that the airline will be aware of her medical issues. The doctor has said that likely the only thing we need to prepare for her is that we have oxygen available, just in case, although he says that it is unlikely that we will need it.

There have been multiple emails going back and forth between me and this particular doctor and I don't know whether it is due to language differences, or maybe my keenness to return home is clashing with his precaution - but I still do not fully know if and when we will actually get on a plane and return back to Australia. I need to arrange for someone to actually fill in the medical clearance forms for Maddy, and then for the airline's medical team to approve them. We need to book the flights, arrange leave for Bernard from work and for Lana from school (although she doesn't need "approval" so much, but I want to try to work something out so that we don't have to pay the monthly fee for the month that we are planning to be back home). And I'm trying to make sure that we do everything in the right order so that IF for some reason it doesn't work out, we aren't stuck with flights that we can't use or anything like that.

Right now, I need to get Maddy's medical records from the hospital. Really, I should have gotten them when she was discharged but I didn't for whatever reason. Then I need to find a doctor who is willing to give Maddy the medical clearance. I hoped that our pediatrician would be able to, but he seems to be wanting us to go back to the ENT and have her sign off on it instead of him. But last time we were at the ENT and asked her, she also referred us back to the pediatrician so I'm hoping that one of them will be willing to "put their money where their mouth is" so to speak (both have said that flying should be safe for Maddy already)! If not, I'll have to wait until we get Maddy's medical records before we can take them to a private doctor for their opinion, which will make the whole process take longer.

So I'm trying to not get my hopes up too much about this - but I REALLY REALLY REALLY want to get home... 

And just because every blog should have a picture, here you go ;)

Socialising

Maddy and Luke - Luke is 2 days older than Maddy and already 20cm (8 inches) taller.

I arrived in Hong Kong over four years ago knowing nobody. Even through my first year here, I didn't really make many friends. It wasn't until I fell pregnant with Lana and joined a "Due Date club" that I started meeting a lot of other people who were in a very similar situation to myself. I've always said that having Lana was the best thing I could have done for my social life here in Hong Kong ;) Today, some of her friends' mothers are my best friends here. 

When I first found out I was pregnant with Maddy, one of the first things that I did was to find another "due date club" of other mums due around the same time as me. We were meant to have our first meeting just before Christmas when I was about 8 weeks pregnant but for one reason or another, I couldn't make it.

Before the second meeting, I had found out about the complications in the pregnancy and at the time, losing Maddy was a very real possibility. The last thing that I wanted at that time was to go and meet other excited pregnant mothers who were actually going to be bringing babies home from the hospital while I had to plan a funeral! I was still included in the mailing lists but I couldn't bring myself to meet any of them.

I don't think that it was until I was about 30 weeks and we knew that Maddy most likely had a non-lethal form of dwarfism that I got up the courage to meet with this mother's group - and at first, it was very emotional for me. I was still "mourning the loss of my healthy child" in many ways, but I found that many of these mums already "knew" me through this blog. They had been following our story and were so supportive towards me.

During Maddy's first four months, I only met up with the other mums and babies once or twice. It felt somewhat strange going to these gatherings without a baby - and besides, I was so busy with going up the the hospital that it was hard to make time for social things anyway!

Since Maddy has been home, we've been making up for lost time though and trying to meet up with them at least once a fortnight. It's so great for Maddy to play with other kids her age - I figure that she will need to live in the real world and make friends her age sometime - so there's no better time to start making those friends than right now :) And in the process, I'm making some great friends too.

Plus, how cute is the picture of Maddy with Luke?!!

February 28 - Jeans for Genes Day

When I was growing up, the school that I went to participated in the annual "Jeans for Genes" day. I had no idea what it was about at the time - everyone wore denim and had to pay $2. It was some sort of fundraiser for something to do with genes, but I wasn't exactly sure what or why. But I didn't really care since it was a good excuse to be out of school uniform! (I went to a school which was very strict on uniforms. It wasn't a "dress code", it was an actual uniform. A plaid dress with a little tab tie and all).

Turns out that this fundraiser is still going on 20 years later, renamed as "Rare Disease Day: Wear that you Care" and it is a fundraiser for over 7,000 rare diseases such as Diastrophic Dysplasia which affect 250 million people, mainly children, around the world. Why mainly children? Because many of these rare diseases have life expectancies of only twenty years. Or ten. Or five. Or not even one year. We are so fortunate that Maddy's life expectancy should not be affected by her rare disease.

Rare Disease day is coming up on Monday 28th of February, so be sure to sport your denim! And if you can, you can make a donation to help research and cure some of these rare diseases.

Visit The Global Genes Project for more information on Rare Disease Day.

To make a tax deductible donation, click on your appropriate country: Australia, USA, Canada, Hong Kong - or to find your own country, search for it here.

In future years, I'd love to organise a group for World Rare Day - maybe at the girls' school, or at Bernard's work or something like that. This year I liked the idea, but I didn't have the motivation to put wheels on it. Ah well, there's always next year :)

Stuck

I've been avoiding writing this post for a few days now. I was so hoping that at our appointment on Tuesday that the doctor would approve our plans to travel back to Australia. A couple of months ago when Maddy was discharged, he told us to not travel for a couple of months. Since then, the ENT said that Maddy would most probably handle being in a plane. In addition, her breathing is getting better and better all the time - she rarely has the stridor any more now. But it wasn't to be - the doctor told us that we should put our plans to travel on hold for another 3-6 months at least.

Disappointed doesn't even really begin to describe what Bernard and I are feeling at the moment. You see, last Easter we were planning to go to the US but had to cancel it because of my "high risk pregnancy". We thought of going to Australia in the pregnancy instead but it didn't work out. Since Maddy was born, we were initially wanting to go back in October for Lana's birthday, and then after Christmas and into January, and more recently we were starting to think about March, but each time it has fallen through. We haven't had a good break for over a year. Bernard hasn't been back home for over two years. Lana and I haven't been back for eighteen months - last time we were back, she wasn't even walking yet!!

The doctor admitted that chances were that Maddy would most likely cope well with being on a plane - but since they know she has had airway issues, IF there were any complications on the flight, her airway could completely close up and there is absolutely nothing they could do for her on a plane. Oxygen wouldn't help because it wouldn't be able to get into her lungs anyway. It could be a life and death situation. So he advises that it is better to wait a couple more months and then possibly repeat the CT scan to reassess her airway and keep our plans to travel on hold until then. He also advised that we should probably do a couple of short-haul flights before we go back to Australia (a nine hour flight) as well.

I've already had six months experience with this doctor and we have butted heads a bit over the time. I haven't always agreed with his management of Maddy's care when she was in NICU. I still believe that she could have and should have come home between two weeks to a month old and IF she had any problems after that, we could have brought her back. This particular doctor is VERY cautious and conservative. I asked him on Tuesday "If we had another child with DD with similar symptoms to Maddy, how would your care of that patient be different now that you have learnt more about the condition from Maddy?" and his response? "It would practically be identical. There may be a chance that if the feeding went well, we may let the child home slightly earlier if the parents were CPR trained, but there is very little we would do differently." It really makes me think that if we DID have another baby who also happened to have DD, I would prefer to just move back to Australia. I think that the NICU situation there would be much more to my liking, and I'd rather be stranded there than here as well if we were unable to fly!

It is so frustrating to be stuck here, and this issue really brings back a lot of my frustrations with NICU as well. When Maddy was in NICU, it felt like her release just kept getting pushed back later and later. There were times when it felt like there was never any plan to release her. And each time that this doctor tells us "Wait another couple of months to fly", it feels like he'll probably NEVER say she can fly. 

In the meantime, we have SO much time off that Bernard can take (over 7 weeks) - and so little that we can do with it. There are not that many places you can go to from Hong Kong that don't require flying. We have already been to Macau and we spent a couple of days at Disneyland too. We could go to some places in China, but the places that are close to Hong Kong may not be so clean and child-friendly. There are no cruises that depart and land in Hong Kong - but we could possibly do two cruises back to back - say one from HK to Singapore and another one back to HK.

Being stuck here is really hard for both Bernard and I. And for me, it makes me realise more and more that Hong Kong is not "home" for me. I do like it here and we have made some wonderful friends here (some of who have already moved back to their homes, and others who are leaving HK shortly). If it was completely up to me (and it's not), on some days (but not every day), I would love to just call it quits here and move back to where I DO call home. But that plan is not in our immediate future at the moment.

We have another appointment on June 2 with the respiratory team, including the ENT, the pulmonologist and a couple of other doctors that I can't remember right now. I can and will ask them again about flying then - but my past experience with them says they will likely just defer that decision to our neonatologist. Our next appointment with him isn't until July 12. So chances are, we may not be going anywhere before Maddy turns one.