Yen Ling Wants To Keep Her Hair Long

Yen Ling grows up like any other little girl, she wants to keep her hair long. She wants to tie up her hair just like the pretty girl in her school. However, I did not want to make it an easy job for her. I had recently set up a challenge for her that is to hold herself straight up all the time. Then only she can have her hair as long as her wish.

Due to her un-noticed Pompe problem (weak muscle) since young age plus the Malaysian's style of putting baby to sleep in the "sarong" in day time, she had gradually developed into haunch back due to the lack of support from the "sarong". While she was a baby, the symptom did not show up; the problem slowly took it's place. It only apparent when she was about 3 years old.

She will keep herself straight up every time when we asked her to for a while, then she will go into the posture that she feels easy with. I tried to tie her up using a correction stripe but she would refuse, she would be moving around not cooperating. At that time, she still can't really see why should she torture herself for. I must admit the correction will not be a pleasant task for her.

Recently I had ask for doctor's advice and she was referred to physio therapy. I was glad for the referral. The therapist started to look into her back muscle and commented that it was quite badly hunched. However, it was not as bad that she could improve or recover if she exercise enough to realign her muscle.

Now, every 2 weeks besides the enzyme infusion, Yen Ling will have her physio therapy appointment in GHKL. This way, she will save her time.

After 2 rounds of the therapy sessions, the therapist was quick to point out that her trunk muscle was not as great too. She noticed that Yen Ling leg muscles was still not very strong. It was like inter-related muscle problem.

According to the therapist, Yen Ling will need to strengten her back, thigh, waist and leg muscles. It's really the whole body exercise.

Now, besides the once every two week exercise in hospital. Yen Ling was asked to do the exercise every night before sleep. That's the only standard time slot that I could fix up for her so that it will be consistent. Because, we have enrolled her in for Chinese Wushu lessons in the hope to strengthen her muscle. Earlier, I have started swimming lesson for her but her body did not cope well with the pool. She will be complaining of cold and run away from the pool. Guess it's not the time yet for her to learn swimming.

Whatever the plans lined up, at the end only Yen Ling can decide whether she will have her long hair or not. I am hopeful as her is quite a strong minded little girl.

Wish her luck.

Wrote in 17 Jul 2008

Wei Ling Can Stand Up On Her Own!

Since Wei Ling started walking, it was last January that she acquired this skill. However, her lower limbs and waist muscles are still not strong enough to hold her from squatting position to standing position without any support.

I told her that she will need to acquire this skill if she wants to go to school like her brother. So, I started to get her to hold on something and pull herself up. However, it was a very tiring exercise for her; she will refuse most of the time.

So, I asked her to crawl on the floor instead. It was more fun that way and she was more willing to do it. This way, she strengten her knee and waist muscles. Still it is not good enough to give her the energy to stand up on her own.

Then I asked the physio therapist in the hospital how to make her stand up on her own. He said she will need to strengten her overall leg muscle. He taught me to get Wei Ling to sit properly and lift up and hold the alternate leg one at a time.

However, Wei Ling did not like it very much too. So, I ask her to do lots of crawling. As time went by, she slowly strengten her lower limbs muscles. She started walking faster and faster as if she was running. Though she was not in her running posture.

Later, I taught her how to do squatting. Initially, her knees are not strong enough, both knees will fell to the floor. Slowly, I convience her to do more whenever I have a chance especially during the night that Yen Ling and Sze Hong was practising Chinese Wushu. It's much easier when we have a big open space and everyone was exercising.

Then, I taught her to hold on to her own knees & slowly moved herself up. That way, she will be able to stand up on her own. Every little step that is easily conquered by any other normal child will typically take weeks to months of practices.

For months, Wei Ling was only able to hold herself quite high up but not high enough for her to lift up her entire body. If she moved on further, she will fell back down.

In mid Jun 08, Wei Ling stood up on her own without any support finally. We camered down the wonderful scenes and gave her a big applause. She was so happy. She still falls down few times every time before she could stand up on her own. But that's a great break through for her. It's another important milestone chart her.

Now, it's getting easier to ask her to go wee wee or doing 'big business' on her own. Still, when she is lack of energy or the case is too urgent; she will still look for adult's help.

Hurray!!!

Wrote in 14 Jul 2008

Something Joyful to Share...

Another victory story for another Pompe family. Yeah....This time it's in Sarawak. This family also has got 2 Pompe children. It's the story of a sister and a brother. The mother is a single parent. She is such a brave and loving mother, she takes up the whole responsibility even though it is such a tough one...

Both Bong's sibling were borned to be as normal & as healthy as any other kids. They had their mischiefs and were going to kindergarten as any other kids too. However, Mdm Bong noticed something was wrong with her elder girl. She started to show weakness on her lower limbs, she would fall down very easily and she started to get sick quite easily too. Soon, the younger brother also started to show similar symptom. Both their health condition deteriorate slowly.

Only after 2 years of finding the root cause (from 1 discipline to the others) that Mdm Bong was finally given a name for her two siblings. They were diagnosed to have Pompe (Glycogen Storage Type 2), a very rare inherited metabolic disease. This was not an easy moment, she almost collapsed as the doctor told her that her child might have an early death as compared to other kids. However, the doctor told her too that there is a group of scientist and doctors that are doing researches to come out the with humanised enzyme to treat the problem. That was in year 2001...

However, this family was not that lucky as the research target was on "Infant" group. They belonged to "Juvenile" group, they could not be taken in for human candidates for the enzyme trial runs. Even though they were diagnosed to have problem, nothing can be done except waiting. Their body started to deteriorate more rapidly that they finally lost the ability to walk & breath on their own.

Finally, after waiting for 6 solid years; they were finally given a second chance. They started on Enzyme Replacement Therapy (ERT) Jun 08. I am very sure if they follow the doctor's advice strictly, great improvement will be seen. Let's us all pray for them that they are going to have very positive response to Myozyme that one day they will be able to walk & breath like any other normal person. Their response is critical in deciding if the drug supply will be a continuous one...


Wrote in 14 Jul 2008

The Dream

Have you ever have a preview dream ? I do...

I know I dream everyday but I almost instantly forget totally about it the next morning as if there is no dream at all. But I just could not forget about this particular dream. It's really a scary one.

I dreamed of having a baby girl with deformed limbs. The baby girl looked quite terrible. Then this baby talked to me while she was still in the womb. She was asking me not to abort her due to the physical disability. I told her that I won't as I just couldn't do that... She was happy with the reply and had a big smile. Then she told me not to worry for her as she will be fine, she can take good care of herself. She told me too that her existence will help her sister and a lot more. Before I could ask her more, I woke up in the middle of the dream. The dream was so real, I felt as if I was really pregnant again. Though I acted so calm in the dream but I was frightened truly by the thought of the baby's disability when I woke up.

I have decided to keep the dream as the dream, so I told my hubby that we will stop production since we have a pair of children with different sexes. However a promise is a promise, the more I would like to run away the more that I got it. I was pregnant 2 months later.

It was the most difficult pregnancy I ever had, the mental stress was huge. I thought of the dream almost everyday but I dared not tell my hubby or anybody about it and didn't know what to do except for carrying on with the pregnancy.

The dream had haunted me till the day when Wei Ling was born. When she was delivered with 4 complete limbs as normal as her other two siblings. I was relief, I thought everything will be alright, it's just a dream.

After she was a year old, she would be sick almost every month. I almost lost my sanity taking care of her for the first few months, it was truly difficult as she would not be sleeping through out the night. Luckily my mom stayed with me for 6 months to help out. Else, I would definitely collapse and things will be totally different now.

Finally, the dream revealed it's true color when Wei Ling was 1 year old. She was diagnosed with Pompe (Glycogen Storage type II), a very rare inherited metabolic disorder. It was really due to her existence that we came to know that Yen Ling is also having Pompe.

The dream was just trying to prepare me to go through the challenge. I understand now why it only took me such a short time to accept the fact that my girls are having inherited disease as compared to others. I just realized I have started prepared myself even way before the pregnancy.

Now, I would just prayed that the last part of the dream will come true that both girls will be fine. That everything will be well taken care of with or without me and my hubby.

Wrote in 3 Jul 2008