Myoclonic Jerks Return

In September 2012, Vera had fits presenting in the form of Myoclonic jerks for the first time. They went away after she was started on Epilim.

Less than 2 years later, they are back again.

Again she stares at nothing with a stunned look. This time, the pattern is non-stop clapping interspersed with the jerks. She goes into 'The Mode' once she is left alone.

We should have increased her Epilim dosage as she grew but we didn't. Lesson learnt.

Now let's hope that we can move up quickly to a dosage that will put this under control and I can have Vera back to her normal self.

A Good 2013

I did a quick check and found that there were fewer posts in 2013 compared to 2012.

It's a good thing. It just means that things are going well with Vera, so I have less drama to blog about. 

In 2013, she had 2 hospital stays - one in June and another in December - both for gastro-enterological infection, with prolonged bouts of Diarrhea.

So far we are averaging 2 hospital stays a year, so I'd say she's doing alright. 

Here are some updates that we are very thankful for: 

1) Fits: Vera's myoclonic jerks (fits) have not resurfaced since September 2012, when she was put on fit medication Epilim. The course of treatment is 2 years, so by September 2014 if she stays fit-free, we can be on our way to weaning off the medication. 

2) Breathing: Vera's biPAP breathing requirement setting has remained largely the same. Results from the sleep study done end of 2012 are out and her requirement is 15/5. This is lower than the 15/7 she is currently on. This is the first time the settings have gone down instead of an expected increase. This is a reassurance that she is well supported for her breathing during sleep, or that she is growing stronger and doing more of the breathing work herself.

3) Gripping: Vera has starting to grip simple objects with her right hand. She has gradually become more open to holding things, even if it is for a few seconds. 

4) Kicking: She has discovered the joy of kicking, and loves it best when in the pool. Feeling her move so freely and joyfully in the water makes me wish I could bring her swimming every day. 

5) Visiting: We brought her out to more CNY house parties this year. When she was young, she hardly visited, for fear of infection. We're happy that she's stayed healthy throughout the festive period. 

So 2014, please be good to us. To Vera's fans who have been following us over the years, you have a good 2014 too!

Not Fits, Not Spasms

Just 4 good days of Vera back to her normal self and we’re back with her in the hospital.

She has started having the 'Episodes' again: Eyes looking to the corner, hand jerks, stiff legs.

Consistently once a day, each lasting for up to 1 and a half hours.

This time round, the EEG was put on her for a longer period, and managed to capture an Episode.

I am calling it an ‘Episode’, because, as it turns out, what we are seeing above turns out not to be fits. No seizure activity shows up on the EEG when she was having the Episode.

So what is it then?

The senior neurologist’s take is that this is a development of her Trisomy 18 brain. Making her lose some control, or altering some of her ‘regular’ movements. This may become her 'new normal'.

The good news is that, since it is not seizures, we need not panic each time it occurs. This takes a great weight off our shoulders. (If it were seizures, we have to give medication in 5 minutes, or there will be some brain damage.)

The good news is also that the EEG shows that it is not spasms.

However, it is clear that her EEG is not normal – which is totally expected due to Trisomy 18. There is evidence of seizure-triggering activity, which means she is highly prone to having seizures. She will be on anti-epileptic medication Epilim for a minimum of 2 years.

Daen’s Arthritis

Meanwhile, the swell on Daen’s ankles have subsided, I would say by 70%. It remains to be seen if it will go away completely. He still walks with a visible limp.

Daen is also getting well acquainted with hospitals. He looks forward to going to “chea chea Vera’s hospital” NUH, and knows his hospital is KK.

The pronunciation bee can, at 2 and a half, say the word ‘Rheumalogist’ & 'Photosynthesis'. Perhaps a career in medicine or botany (think long big words) may be suitable for him.

A Good Sleep

Vera is discharged today after 5 days in hospital. 

She comes home with Epilim, targetted to reach 3ml 2x/day.

She seems rather drowsy most of the time, but we understand it takes a while for the body to acclimatize to the medication. 

Let's hope she responds well to the Epilim and it keeps the seizures at bay. 

Mai Mai, who helped look after Vera in her beginning months, visits.

Able to smile for the camera again.

Feeling the texture of a balloon.

What we love about NUH Pediatric ward is the greenery that surrounds it. The beds face Kent Ridge Park and there's a big playground next to it. It's a great place for children to heal in.

Daen visits his chea chea

Myoclonic Seizures

We spent the weekend in hospital, and today being a Monday, they were suppose to schedule an EEG for Vera. Beth called me at 8am to say they were already preparing her for it.

We were expecting perhaps by end day, but there was just one slot and Vera got it. We would have had to wait until Friday for the next available slot. 

Vera undergoes her first EEG, which reads her brain activity. By reading the waves, doctors can ascertain which part of her brain is showing "irritations" which predisposes her to fits. 

They recorded her brain activity while awake (10 minutes) and then gave her some sedatives to put her to sleep to get another 15 minutes of activity while sleeping. Because the activity that causes fits is usually in the period going into sleep and coming out of sleep.

Waterfall of wires to plug us into the mysteries of the brain.

Within minutes, the Neuro team was in the room to answer all my questions and bring clarity to the matter. Her neurologist Dr S came, read the EEG, saw the video I posted and shared her observations.

The results show 3 areas of her brain with abnormal activity:

a) a point on right side which presents as either muscle pull at mouth or hand jerks;
b) a point on left side
c) a point at left occiput (back of head)

Dr S confirms that the video shows a cluster of myoclonic seizures as well.

Her plan is to start her on Epilim. And this will be at least for a period of 2 years. If she remains 'seizure free' for 2 years, then can we start to wean her off the medication.

Her dosage is currently at 1ml twice a day. They will progressively up it to 3ml twice a day, over the next few days, to gradually get the body up to the required dosage for weight. The side effect of any fit medication is drowsiness. So we pray that the doctors will find the right dosage that would be just enough to control the  seizures without making her too dopey while she's awake.

I do still want my excitable little girl : (

I thank God for the speedy team effort this morning, diagnosis and medication. I will write more later about the excellent team at NUH. Vera is now drugged out by the hydroclorate, which was the medication I had intended her to get when I checked her in. I know that with this, she will have a very good long sleep and really get the rest that she needs. It is a short-term medication, but we've seen how helpful it is in resetting her sleep clock.

Meanwhile, since she is well-monitored in the ward, the Respi team will attempt to titrate her from 13/7 up to her recommended setting of 16/7. Given our past 3 failed attempts to even reach 14/7, this leap, if successful, will be nothing short of a breakthrough.

So many miracles already have shown themselves through this little girl, from the day she was born. Her chiropractor said to us, 'She is a miracle'. I now know she is a gift from God, so bursting with multiple miracles that I just cannot not see.

Is It Seizures?

Some special needs moms I know watched the video of Vera in the previous post and said it looked like fits.

I've learnt to listen well to these moms.

So this morning (Saturday), I took Vera down to Emergency. It's been almost 2 weeks since the height of her so-called 'possessed' episode, and she's still not back to her usual happy self. Instead, she's drowsy, dazed, crying to be rocked to sleep, then repeatedly crying during her sleep. She's basically behaving like she's sick.

It's the weekend, and I wasn't going to risk an ambulance run should the 'episodes' strike again (we know how things like to happen during the weekend). I have had enough drama to last me for a good while.

My instincts said: "Check her in, get her monitored, ready to respond with meds should a seizure strike, and more likely a Neuro doc will address her issues sooner." It turned out to be the right move. There will be a senior Neuro doc in the ward tomorrow (Sunday), and we'll most likely get some clarity.

I will update more then.

Thanks everyone for your support.

Arthritis, Stitches & Spirits

Where to begin? 

So much has happened over the last 1 month. 

It started in early August with a suspected sprain on one of Daen's ankles. It was swollen. 

A pronounced bulge around his left ankle that is warm to the touch
and skin that's now brownish in colour.

We brought him to Chinese sinsehs. The swell remained. 

We brought him to A&E at a children's hospital. They took an X-ray and said that there was a hairline fracture. They put his foot in a bandage and told us that the swell would go away within a week of resting the foot. 

Rather happy to be staying at home and getting to watch
more TV than ever.

We were referred right away to the Rheumatology department (what the #%@? is Rheumatology) and within an hour Daen's ankles were ultrasound scanned and he was diagnosed with JIA: Juvenile Idiopathic Arthritis. 

On BOTH his ankles. 

What? My 2 year old boy has ARTHRITIS?

The doctor explained. It is an auto-immune disease whereby his body's immune system mistakenly attacks itself, mostly at the area between the joints, causing the surrounding tissue to become inflamed and therefore swell with water retention. 

The only other shock that equalled this was the day we learnt about Vera's diagnosis. 

Vera has a diagnosis, a life-theatening one. Okay, I can accept that. Now Daen too? 

So now I have 2 medically-challenging kids on my hands. When you're a seasoned traveller on this path you know what that means. More anxiety, more heartache, more waiting for improvement, more hospital visits, more medication to secretly administer, more side effects to deal with....the list goes on. 

After one month of mounting question marks as to why he has been limping has led to this.

Arthritis is categorised as such: 

Mild: In 4 joints or less. 

Serious: In 5 joints or more.

Severe: Whole body. 

The doctor put it this way, "If he's going to have arthritis, this is the best type to have,"

By that time, I was a wreck, and that did little to console me. 

It is now 3 weeks since that dramatic day.

Thomas the train set from his Godma help us to keep
him from moving for 24 hours after the steroid injection.

Daen has had a dose of anti-inflammatory steroids injected into both ankles under sedation. He has to be on anti-inflammatory drugs (Indocin) for the next 6 months at least. The swelling has subsided, but not fully. He doesn't limp now, but is still wobbly.

"My ankle is better, I don't need a bandage anymore" the boy declares happily.

Then 3 days after the injection, bam!

Just as I was slowly recovering my composure after the climax of the injection and subsequent 24-hour bedrest for the boy, he runs, trips, falls and cuts his head on a sharp corner. 

Ian picked the wailing boy up and blood smeared all over his T-shirt.

Blood coming out from the head provokes a different response as compared to blood coming out from say, your leg. 

Head = Brain. Blood = concussion. I went berserk. Stark mad. Even Vera falling off the bed didn't feel so crazy. There wasn't blood oozing out directly from her head. 

Holding an ice compress to Daen’s head, I dashed out the house with him in my arms as if I was the ambulance. Ian broke a few driving rules. 

When we reached the A&E, I was determined to cut the queue. I shouted, "This boy’s head is bleeding, please help!"

We got speeded up. 

The gash was pretty deep, needing 2 layers of stitching: 2 beneath, 4 on top. But it wasn't a concussion. His brain is okay. He's going to be okay, I repeated to myself.

The boy fell in and out of sleep, I kept talking to him, comforting him. When my mother ("Grandma is a nurse" the boy knows) arrived, I offloaded him and burst into tears. 

It's been a week since the fall, and the wound is healing well. Daen can point you to the ledge where he cut his head. 

You'd think the story ends here.

Well, here's the straw that broke the camel's back: 

At the exact same period that we went through Daen's injection and fall, Vera started behaving strangely. 

It started with very bad sleep. We had increased her biPAP pressure upon her doctor's recommendation, based on the sleep study findings. We had to progressively increase it from 13 to 16 (Inspiration rate). We upped it to 14. 

A few days of bad sleep progressed to 2 whole nights of no sleep at all. She would stay awake, crying because she was so tired but she just couldn't sleep. Instead, she would stare fixated at the same spot at the ceiling. Her breathing would quicken, her legs would tense up and her hands would jerk frequently.

It happened to be the peak of the Lunar 7th Month, otherwise known as the Hungry Ghosts Festival. Every year, Vera has had  bad sleep during this period in past years, but this time, her behaviour completely freaked us out.

When she refused to even let us carry her, or touch her hands, or look at us, we decided the best immediate course of action was to get her out of the house. We brought her to my grandma's place for the day. My aunt and grandma prayed for her. She finally slept in the car back home. 

We reduced her setting back to baseline. More family members came to pray for her the following day. I started to pray.

I think back now on how the events of the past month have just been beyond me. And it deepens my belief that there is a higher power orchestrating my life, our lives - Vera's and Daen's included. How their different diagnoses will pan out, is way out of my human hands.