A Warning

From the time we had received the trial BIPAP machine till the time the new machine was delivered, masking C was often met with difficulties, either he was unwell or something just had to break down. For that reason, we had decided that C could enjoy a little more days of natural sleep without machine intervention through careful monitoring. He was doing very well for the longest time I could remember. Drops in SpO2 were rare.

Friday night was different.

The first alarm was triggered before midnight. Oxygen saturation on the oximeter read 84% and it continued to dip by the second - 83, 82, 81...  77, 76, 75...

In the past, C lowest SpO2 was at 72% so I wanted to prevent it from reaching that. I began to gently stroke his chest hoping to wake him up from deep sleep (usually my touch would wake him and send his pulse rate & SpO2 back to normal level). This time, no response.

70, 69, 68... the drop did not stop. Worried, I hasten to wake him but to no avail. I grew desperate and started calling and patting him. My gaze were going forth and back, on C and on the dreadful numbers reflected.

62, 61, 60... finally, C stirred and I breathed in deep for the first time. His SATS started climbing and things were soon back to norm. I did, however, noticed that his pulse rate was fluctuating and SpO2 was at a lower than usual 96%.

Few minutes passed and the alarm sounded again. Two more dips ensued. I then alerted hubby to be on standby while I changed a new sensor probe to ascertain the erratic readings. I had the BIPAP and mask ready too. Things quieten down for the next hour and just as we thought it was over, the cycle started again (even though the drops were not as drastic). By the sixth drop, we were ready to mask him. C usually fights hard when on mask but this time round, he fussed only because we shifted him. He went right back to sleep shortly. No more beeping and no more heart-stopping moments.

Tonight, even though C is doing better but I do see slight drop and decided that he should stay masked. Wondering if it's the Monday jab at the polyclinic which is making him weaker?

Anyway, shall continue to do my night monitoring and I do hope tomorrow will be a better day.

Caelen Loves To Talk

Having seen how intelligent and creative Cayla is, we naturally held high(er) expectations for C. His diagnosis hit us hard and destroyed much of our wishes for the future. Ironically, C turned out to be quite a talker and that quickly filled the voids in our hearts and rekindled hopes.

C is currently obsessed with cartoons, balls and books. He loves to read and would tirelessly flip all of Cayla's books, one after another. He participates actively by spontaneously repeating some of the words we narrated, exhibiting a huge sense of achievement whenever he does that. What's more amazing is that C speaks to us in almost complete sentences (right from the beginning when he started talking at 1yo) and addresses himself as "我". How many 1yo child does that really?

Being a curious and eager learner, C expanded his list of vocabulary quickly. And this proud mama here just could not stop herself from sharing how much C has learnt so far.

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Baby / Ball / Balloon / Banana / Bear / Bird / Blow / Book / Bottle / Bubble / Bye Bye / Car / Chair / Close / Cover / Dog / Doll / Door / Duck / Egg / Hello / Hold / Jump / Knock / Milk / More / Monster / Morning / Night Night / No / No More / Open / Shoe / Sticker / Tap / Throw / Tiger / Turtle / Walk Walk / Want / Water

Donald Duck (Character) / Elmo (Character) / Gai Gai (Jalan Jalan) / Mickey Mouse (Character) / Moo Moo (Cow's moo) / Mouk Mouk (Character) / Mum Mum (Makan/Food) / Ng Ng (Poo Poo) / Orh Orh (To sleep) / Peppa Pig (Character) / Pers (Pampers/Diapers) / Pong Pong (Shower) / Quack Quack (Duck's quack) / Toot Toot (Pacifier) / Uh Oh (Oh No) / Wah! (Amazed/Excited) / Woh Woh (Dog's bark)

我要这个 / 我要那个 / 我要抱抱 / 我要拿 / 我要看 / 我不要 / 我要吃这个 / 我要milk milk / 我要喝水 / 我要去walk walk / 我要戴 / 粉 / 喂 / 眼镜 / 服 (衣服) / 丢掉 / 睡觉 / 爸爸 / 妈妈 / 姐姐 / 嫲嫲 / 爷爷 / 姑姑 / 公公 / 婆婆
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Below is a clip showing his love for balls and demonstrating his ability to link words "throw" and "ball" together.

http://youtu.be/nf_z8jdm8as

Honestly, we do not have concrete plans for C yet. But having seen his love for learning, perhaps it's time to consider the possibility of putting him in special school in the near future.

Weight Issues

It had been more than a month since my last post. Like many had said, no news is good news.

C had been well so far, no major concern except during our last visit to KKH, Dietitian commented that he was underweight. Being his caregiver, I knew this was coming. We had been desperately trying to improve on the situation for a while now. C was also probably going through the "milk and food strike" growing-up phase. His milk intake took a plunge for no particular reason and was just more interested in play than food. Otherwise he was all well and happy.

The changes expected from us?

• Need to add more cooking oil in his food and milk (maybe I'll skip the milk part. I honestly did not want to upset his tummy or cause any indigestion)
• Need to meet the minimum 150ml to 180ml per milk feed, 4 feeds a day (I faced problem in squeezing in 4 feeds, 2 meals from 7am to 9pm. What could I do if C refuses to drink?)
• Need to switch from giving blended to soft chunkier food (Didn't they last advised me to continue with blended food as tolerated? Why risk choking on foods he cannnot chew and swallow?)
• Need to stop syringe-feeding him water and to use a straw / cup instead. That way I have better control over how much he drinks (fact: syringe gives me more control than letting C drinks from a straw or cup).

That did not go down too well with me because they were adding on issues on hand instead of offering solutions to my problems. I'm just going to try my best, stick to what works best for C.

Rejoice!

Always been a side-sleeper

C's respiratory doctor had returned from leave. We took the first opportunity to explore the possibility of reducing BIPAP pressure settings so C could quickly resume usage. Dr B was very understanding and see to the changes immediately. Pressure was dropped from 15/7 to 12/6.

First night of trying yielded surprising results:
09:00pm   Mask on
10:30pm   Fell asleep
11:00pm   Woke in state of shock, heart-rate climbed to 170
11:20pm   Returned to sleep
07:30am   Slept till next morning

Second night was a breeze:
09:30pm   Mask on
09:45pm   Fell asleep
07:00am   Woke up

It definitely brought about hope and peace of mind knowing that C is quick adapting and is able to breathe properly during sleep.

Mid-week, we made another trip to KKH for a scheduled (video)fluoroscopy. C being a glutton, completed the procedure effortlessly. Nom nom nom, doctors monitored the motion x-ray display and voila! he's done in less than 10 minutes. Doctors observed droplets of milk entering and exiting the top of his airways, C is at risk of asphyxiation. Taking preventive measure, we were instructed to change his bottles and teats to one with extremely slow flow (so slow that C became impatient as he took 20 minutes to finish a feed). While drinking poses a risk, doctors were somewhat satisfied with his ability to take blended food. In fact he fared better eating than drinking and hence agreed that C continues to eat as tolerated, which is just as well since C loves to eat.

Other than going for appointments, what has C been up to lately? Well, he is pretty much growing up like a normal child. He prefers to be up and exploring than lying on his bed. We thus try to accommodate whenever possible, carrying him around teaching him new things. C is also fighting to be more independent too, wanting to hold his own bottle (provided it's not too heavy for him). See how elated he was doing that!

I hope to see more milestones coming. Surprise me baby :)

 

Before I go, this entry is for tonight:
10:10pm   Mask on and fell asleep

Blessed. Good night peeps!


P.S. Uncles & Aunties: Thank you for your generosity and kindness. Little C is very thankful and blessed to be loved by all.

New Gadget

Ta-da! A delivery on April Fool's Day! Latest addition to our collection of gadgets - a handheld oximeter which cost me $1,200 and the box of probes (far left in pic) cost another ridiculous $640. My jaw dropped when sales rep quoted a whopping $3,200 for a table-top oximeter!!! That, is another heartache for another day... when my heart is stronger (I doubt).

To cool things off a little, we have decided to shave off C's hair. Weather is way too hot and humid for his liking. How's the new look? I thought he is still as handsome.

C has been making tremendous progress in his linguistic skills. For a one-year-old, he is already using short three, four-word sentences e.g. 我要这个，我要那个. His vocabulary is quite impressive too as compared to Cayla at his age. I suppose his language skills compensates for his lack of mobility and he is able to tell us what he needs and wants. He does the talking, we do the walking haha. See, God is fair. Mommy is really proud and can't wait to review his progress in few months' time!

Second Challenge

In March, Caelen went for a series of follow-up appointments with various doctors. Here's an update:

• Respiratory - Sleep Test results showed overall oxygen level at 93% (less than ideal of above 95%). Lowest oxygen level at 73% (frequent drops observed). Usage of a BIPAP machine during sleep is necessary to improve his breathing.
• Chest Therapy - Senior therapist checked and noted that he is not using full lungs capacity to breath.
• Physiotherapy - Weak trunk and limps. Need to continue with therapy at home to at least maintain and hopefully improve his strength.
• Occupational Therapy - More than satisfied with his fine motor skills and cognitive development. Praised that he is a smart boy and is able accomplish complicated tasks suited for older tods.
• Feeding - Doctors were concerned as he was no longer chewing his food and he seemed to have slight problem swallowing. Caelen was thus scheduled for a videofluroscopy early next month. Doctors also noted that his weight is not climbing as much as it should and I was ordered to thicken his milk as well as putting him on Valens Carborie.

After numerous trips to the hospital, Caelen came down with a fever 5 days ago with no other symptoms. Kaelyn's daddy and mommy (another special family) were kind enough to come check on Caelen (to ease my worries) and loan us their portable oximeter for monitoring his condition. That very night, Caelen's temperature shot up to 40 degrees with heart rate at 213, his vitals triggered the alarm. Drowned in the beeping of the machine, hubby and I sponged our boy for close to an hour, finally bringing his temperature and heart rate down to desirable level. Miraculously, his fever was completely gone the next day.

Caelen subsequently developed a cough in days that followed. We started putting him on BIPAP to regulate and aid his breathing. He cried incessantly each time we hooked him to the machine, we even tried to calm him by playing his favourite videos. Unfortunately, he vomited on different occasions, with one particularly bad that he erupted like a fountain. That I'll need to check with his respiratory doctor on the cause.

For now, his MMR inoculation this week had been postponed. He is on BIPAP (during sleep) for as long as he could tolerate. Otherwise, sometimes I think he fared better without.

Caelen Is One!

Overnight Sleep Test at KKH

On 28 Feb, Caelen underwent a Sleep Test at KKH. This test is often conducted for patients with sleep apnea or related sleep disorders. However, in the case of SMA patients, it is used to identify their sleep patterns, access respiratory functions and to also understand how the changes in airflow and pressure aid their breathing while asleep.

In order to take detailed readings of the above mentioned, technician needed to wire him up to a few machines. Took us almost an hour and a half to complete this delicate task with Caelen screaming and crying the entire time (not a great experience). He continued to protest for the next hour that followed, leaving me emotionally and physically drained. But the night ended well with him knocked out, totally exhausted.

We celebrated Caelen's first birthday cum fundraising the Sunday that followed. It was the best celebration ever and fundraising was a success! All these not possible without the help from families, friends and lovely people.

Heartfelt thanks to:

1. Mr Rahul Sharma & Ms Beh Hui Shiang the venue sponsors (very lovely place) for this party
2. Ms Isabel Lee-Shorrock the resourceful planner who has helped in ways I could never thank her enough
3. Many friends who came and helped out with the logistics and setup
4. Families and friends who came for the birthday party
5. All kind donors and supporters for the fundraising program

Special thanks to kind sponsors:

1. Mr Case Woo & Ms Tammy Sim for designing the 'Thank You' cards
2. Mr Tan Jit Khoon - Winson Press Pte Ltd for printing the 'Thank You' cards
3. Mr Bernie Lee - A'zone Corporation Pte Ltd for the customised Sesame Street backdrop
4. Ms Rena Tan & Partner - Sugarena :: Candy Buffet Stylist for the candy table of Sesame Street treats
5. Pan Pacific Singapore for the Sasha's Bears
6. Ms Annabelle - One-Stop No. 1 Entertainment for the lovely Sesame Street balloon sculptures and face-painting session
7. Mr Joe Lim - Wow Let's Party for the helium balloons & Elmo balloon bouquet
8. Mr Eisen - Birthday Party SG for the fabulous entertainment
9. Ms Samantha - Hey, Cupcakes! for the pretty and delicious Cookie Monster cake
10. Ms Renae Lim - Say It With Sugar for personally baking and decorating the Sesame Street cookies
11. Ms Vivy Toha for the organic Nutrifresh soya milk sachets
12. Mr Alex Pua - BellyGood by Tung Lok Group for the professional service and logistics arrangement

Finally, hubby and I did our first live TV interview with Channel News Asia AM Live today, a special arrangement by Mr Kenneth Mah of RDSS. For those who missed the live telecast, this is the link to the video clip http://www1.channelnewsasia.com/amlive/2014/03/03/interviews-rare-diseases/

Raise awareness, bring about changes and new hopes for all rare diseases patients.