Chinese People are Nosey

Last Saturday we went to the Autumn Moon Festival in Chinatown.  Throngs of people showed up.  Perhaps it wasn’t a great idea to push a baby in a stroller, a toddler and a husband into this giant mass of people. 

Growing up in Hong Kong, the Moon Festival was one of my favourite events. I loved that it was a nighttime activity designed to include children.  I remember walking at night with my candle lit lantern.  All the kids held lanterns.  In fact the streets were all warmly lit by the glow of candle lights. To me, it had a similar feeling to Halloween.  It was the one night for kids to roam the streets after dark.  Rather than checking out other kids’ costumes, we admired each other’s lanterns.  And instead of candy we ate moon cakes. 

I just had to bring Mila and Niko to this Moon Festival. The streets were jammed packed with people.  I don't remember the crowds being this dense in Hong Kong.

When we stopped to get paper lanterns for the girls, I saw two women in their 60s hovering over Niko in the stroller.  Chinese people are not shy about invading your space, and they don’t hide it if they are curious about you.  If anyone has ever taken the #30 Stockton bus here in San Francisco, you would know what I’m talking about. 

These women were speaking in a Chinese dialect that I don’t really understand.  I could only understand a few words. 

“Does she have [word I don’t understand]?” one of them asked. 

“Yeah I think so, she’s got [word I don’t understand]” the second lady said.

They continued to talk about Niko, but I was both furious and embarrassed about this [word I don’t understand] that everything else they said became jumbled to me. 

I cut in, “What are you saying?”  I spoke to them in Cantonese.  They switched to Cantonese to respond to me (most speakers of neighboring dialects can easily speak Cantonese).

“Oh is she yours?” asked lady one.

“Yes,” I said.

“She looks… nothing like you. She’s so pale. And her hair is so light.” said lady two.

“Well the father is Caucasian.” I said. 

“Oh so she looks like the father,” said lady one.

“No she doesn’t look like him either.” I said. 

They continued to reprimand me for not dressing her warm enough.  They made me take out a blanket to cover her.  They grabbed Niko’s hands and feet and said that they were cold. 

“How old is she?” asked lady one.

I hesitated for a moment, “She’s 4 months.”

“What?!  Why is she so small?  The father is tall. She’s the size of a new born.  What’s wrong with her?” Blah blah blah blah blah…..

“Mind your own business!” I barked at them and walked away.

I should not have cared. That [word I don’t understand] should not have stung as it did.  Really. I don’t even know what that word means. But I let it bother me.  I cried about it later. 

People will always ask how old my baby is.  It’s involuntary.  When you see a baby, that question just bubbles up from somewhere in your intestine, travels up your esophagus, tickles the back of your throat and before you realize it, it just comes out like a burp.  “How old?” I ask it too. 

Kiril tells me to just say that she’s 2 months or something.  But I can’t.  I can’t lie. To lie about her age is to deny Niko of the progress that she’s already made so far.  To lie is to admit that I’m ashamed.  And I’m not ashamed... Not anymore.  I struggled with shame for a while to be honest.  But now that I’ve had more time to digest this, and more time to know Niko, I’m proud of her.  I’m proud that she’s living through this horrible condition.  I’m proud that despite all her setbacks she is so brave and smiles at the world.  So I won’t lie. 

I just have to figure out how to deal with questions.  I don’t want to end up snapping at people.  “How old is your baby?”  It’s such a harmless question.  And yet when someone asks right now, I want to spin around and say, “Mind your fucking business!”  And then I would look C-R-A-Z-Y! 

4.5 months, small but just the right size for me.

           

Bone Marrow Donation

I have to snap out of this morose mood that I’ve been in.  Life doesn’t stop just because I have a baby with a terminal condition.  Life goes on.  A good friend of mine reminded me that behind closed doors many people are in pain too.  And I have to remind myself that I shouldn’t be so melodramatic.  So it’s time to think about people other than our nuclear family.

Last Saturday I registered as a bone marrow donor.

My father has taught me the significance of donation since I was a small child.  He made a habit of donating his blood every so often.  In fact, the day before we left Hong Kong to move to the U.S. my father donated his blood as a farewell gesture.  And throughout my life, I’ve always donated my blood whenever there was a drive.

 

More and more these days, just the words “bone marrow” have a heavy significance to me and my family. One of the main reasons is because a bone marrow transplant is the only potential treatment for I-Cell, though highly experimental.  And the latest official conclusion from specialists is that a BMT does not benefit I-Cell patients.  Although my hopes were short lived, I had dreams that we could normalize Nikola’s lysosomal enzyme levels after a BMT transplant.  I understood that preexisting damage is irreversible, but I was hoping that progression of the disease would cease.  However we have decided to forgo the transplant after talking to the I-Cell specialist and other I-Cell parents have who opted for the transplant.  The pain and toll of the procedure outweighed the very small possibility of benefits. 

Now I know we cannot reach for our only potential lifeline.  Niko cannot be saved by a bone marrow transplant.  But if someone else can benefit from a transplant, I’d like to do what I can to help.  I’ve always had that “donor” sticker on my driver’s license (in case something were to happen to me).  It never meant anything to me before.  Now the notion that someone’s life can be saved has a much clearer picture.  It’s not just a sticker on my license.  And I can donate more than just blood without having to croak.  

A week ago, I learned about a little two-year-old boy who has been diagnosed with a rare form of leukemia.  He is the nephew of a friend of mine from high school.  His only chance at life is to find a match for a bone marrow transplant.  With a BMT, ethnicity matters. And there is such a shortage of Chinese (or Asian) donors.  So I decided to register.  If I’m not a match for this boy, then my sample will be kept on the registry until I’m 62 years old.  I hope he finds a match.  

Niko kept me company (no surprise) while I registered.  

4 months

      

Speech Therapy

My brain is fried. I spend my days clutching Niko, apologizing for giving her this life.  I spend my sleepless nights in a seated position with my body twisted into very unnatural positions in my pathetic attempts to sooth her to sleep. I am a half lit flame right now.  A gentle breeze can blow me out.  What I crave so badly is some sort of omniscient helping hand.  Some guidance…… clear concise guidance.  I can see why some people turn to God, or Allah, or Buddha (or a rooftop with an AK-47) in times like this.   

Upon receiving this earth shattering news, I wanted to retreat into a dark place and ask “why us?”  I wanted to assign myself to solitary confinement.  Staring at one spot of a blank wall all day long sounded like a good time compared to the reality I had to face.  

Feeling so lost, I want the doctors and medical professionals to give me guidance.  I’m not looking for a miracle. I know that we can’t fix Niko.  I just want a voice of authority to tell me what to do.

In general, when you visit a doctor, he tells you what’s ailing you with terms that you can’t pronounce and prescribes treatment.  If it’s complicated, he makes a referral to a specialist.  They are supposed to know volumes more than you due to their long and expensive education.  In my case unfortunately, I do not have seasoned specialists to go to.  It’s tiring being on hyper alert all the time.  Because this disease is so rare, I research all day so that I’m more informed than the doctors.  I do my homework before each appointment to make sure that Nikola would get the most out of each specialist we see.   

Last week I met with a speech therapist.  The purpose of the appointment was to observe Niko’s feeding with the goal of improving her sucking ability and general oral motor control.

From the moment I saw this speech therapist I knew she was not going to be helpful.  She had a very quiet new agey feel about her.  Ironic for a speech therapist, her speech volume reached the same vocal frequency of a meek mouse at best.  She seemed rather shy and habitually backed into the dark dank corner of her cluttered office.

I brought about 5 different bottles with me, but she just asked me to try the Dr Brown bottle.  She observed Niko’s feeding for a short while.  She didn’t say much.  So I asked her what she was looking for.  She said she was counting the number of consecutive sucks Niko was able to do on that bottle.  This woman’s quietness could have been mistaken for a reluctance to share information.  She seemed to have a speak-only-when-spoken-to manner.  I was so frustrated because I had to keep pushing for answers.  Going to these appointments shouldn’t have to be so much work! 

So I kept asking questions.  How many sucks did she do?  How is she compared to other babies her age?  Why does she leak milk everywhere when she feeds?  Why does she scream at feedings?  She concluded that Nikola had a weak suck due to low muscle tones in her cheeks.  I kept at her.  What can I do?  Are there exercises I can do with Nikola’s jaw to develop her cheek muscles?  How should I hold her?  What bottle does she recommend?  And finally I got a real answer.  She passively suggested that I try the Haberman feeder; a special needs bottle created for babies with cleft palates and other feeding problems. 

We tried the Haberman feeder at her office.  Niko got one more suck out of this bottle over the Dr. Brown bottle.  Though she didn’t actually recommend this bottle, as she was a woman of very few words, I asked her where I could buy this bottle.  She directed me to a website.  Before leaving her office, I asked her if they sell this bottle at the lactation Center on the first floor (just to save myself the trouble and time of ordering online).  And she meekly said that they do sell them downstairs.  WTF!  This is what I mean.  Why direct me to a website when it's sold downstairs?  It’s like pulling teeth to get anything out of her.

I bought this bottle right away.  And guess what!  Niko is bottle-feeding now.  This bottle is a miracle.  It doesn’t require Niko to actually suck like other bottles.  It works by gum and tongue pressure, most similar to breastfeeding.  So I take back all my negative thoughts of quiet mouse lady.  She has offered the most useful tip of everyone we’ve seen to date.  Niko now takes the bottle (about 60 – 70ml per feeding). 

Maybe someone else can feed her eventually.  So far I’m enjoying Niko as my new appendage, but I’m looking forward to a little freedom.  I’d like to take a long walk, baby-free, one day soon.    

A couple Niko shots to keep this blog fun.

         

Meet Claire

Last weekend, we met Claire.  She is another I-Cell girl.  She is 4 years old and lives in Cupertino.  I found this family on Facebook.   

With such a rare disease (and not to mention unfamiliar to our doctors), one can feel totally isolated and helpless.  When we received our diagnosis, I scoured the Internet for information.  After reading the first four or five medical articles on the subject, I can see that they offer the same bare minimum information.  I wanted a better vision of the life we should expect.  I wanted to know what is Niko’s baseline.  Is she doing well for an I-Cell baby?  Is she severely affected?  I want to know how I can help her along the way.  I can only get this kind of information from other parents who are going through this or have lived through this.  So I started to search for blogs regarding I-Cell.  I found a couple blogs.  One woman mentioned that she met other families through a Facebook support group.  And when I joined the group, it was the jackpot.  I think almost all the I-Cell families are in this group (and not just limited to the USA).

I was so thrilled to have found another family in California.  And not just California, they are in Cupertino, about 1.5 hours away from us.  To give you an idea of what a miracle this is, I found a woman in Australia who has the only I-Cell child currently recorded in the country.  She feels so utterly isolated.  (Ironically when I lived in Sydney, I was only minutes away from where she is now.) 

I contacted the parents of Claire right away and we set up a time to meet.  We visited their home in Cupertino.  They have an older healthy daughter who is about 7 years old, Claire who is 4 years old, and a set of twin boys (unaffected by the disease) who are 2 years old.

We learned a lot from them.  It was very interesting to see Claire, and to see what a 4-year-old I-Cell kid can do.  I understand that I-Cell kids are all different and can function at many different levels.  But I found this meeting extremely helpful.  The parents were so warm and generous with their advice.  While bizarre, it's touching to meet another I-Cell family.  Kids affected with I-Cell have very similar features and hence they look like they are related.  That physical similarity coupled with the exclusiveness of I-Cell makes us instantly comfortable with each other.  They shared the ways they were able to cope with this consuming disease.  They talked about the help they were able to get from California Children’s Services and the referrals they were able to get for Early Start, which is a state funded program for various forms of physical and occupational therapies.

Claire’s family agreed that I can share her photos.  

When she is outdoors, Claire uses this walker to get around.  It puts her in a semi-seated position.  She's quite fast.

Claire does not walk or crawl.  To get around the house, she does torpedo rolls on the floor and is lightening fast.  

Claire's eyes started to become a little unfocused.  She got glasses but never wears them.

Claire is very good at sitting (a huge feat for I-Cell kids).

Four years old and rocks the best hair of any kid I know. 

Mila with their other kids.

Neurology

We saw the neurologist yesterday.  I actually didn’t find this appointment at all helpful.  The doctor was rather young and the room was windowless, hot and depressing.  Of course I asked her if she’s seen any I-Cell patients before, and she said no but that she read about it once while in school.  I suppose I respect her honesty but that just made her unqualified to see Niko in my opinion. 

During the “neurological exam”, she listened to Niko’s heart, looked at her eyes and ears, held her hands and tested the reflexes of her knees and elbows.  Nothing different from a regular baby-wellness checkup with her pediatrician.  She told me to expect mental delays and that she didn’t expect Niko to walk or talk.  She then suggested that we come back in a couple months.  I had to ask, “Pardon me, but we’ve seen a lot of specialists and have many more upcoming appointments.  This is all so new to me.  Can you please tell me what you were looking for in this exam?”  In other words, “What the hell is the point of this appointment?”  She explained that she was looking for Niko’s developmental skills like ability to track with her eyes, muscle tone, etc. 

I asked if she would be able to judge by examining Niko (now or in the future) whether she is severely affected or a highly functioning I-Cell child.  And she replied that there is not enough information in the very small pool of I-Cell patients to provide this kind of answer.  I felt like we could have easily reversed roles in that room.  I could have been the doctor answering her questions.  She didn’t know anymore than me.  In fact she knew much less regarding bone marrow transplants.

We have so many more appointments coming up with every possible subspecialty department.  In order to avoid being a “hospital family”, I have to weed out appointments that are not helpful.  This is one of them.  After meeting with each department, I plan to keep the relationships with the specialists who will provide useful information and treatments that will improve Niko’s quality of life.  The rest I will eliminate.    

Side note:

Yesterday I had my first “what’s wrong with your child” experience.  I was in the hospital elevator.  A nurse walked into the lift.  I was carrying Niko in her car seat.  The nurse looked and smiled at Niko.

She casually asked, “How many weeks?”

“She’s four months” I said.

“Oh, was she premature?”

“No, she’s got…… a condition.”

“Oh.”

And then she inched away from me and pressed her body against the other side of the small elevator.  This wasn’t an afraid-of-catching-a-disease-from-thy-neighbor inching.  It was more of an awkward-wish-I-didn’t-ask inching. 

I didn’t blame her for asking.  Niko is small for her age.  But this was a nurse in a pediatrics hospital.  If she reacted awkwardly, then this is an indication of the strange looks and reactions we’ll get as Niko’s condition progresses.       

The Stuff That Mila Says

Three weeks ago

Mila:   Mamma why are you crying?

Me:  [choking]

Mila walked into the kitchen.

Mila:  Oh no, you too?!  Why are you crying papa?

Kiril:  We are worried about Niko. She’s very sick.

Mila:  Well worry about me too.  I have a tummy ache from eating too much sugar.

Two weeks ago.

Mila to Niko:  When you grow bigger, you will be a great ballerina.  I will teach you how to dance and we will go to ballet class together. 

Me:  [heartbroken]

One week ago.

Mila to Niko:  When you grow bigger we will play tennis together.

Kiril:  [heartbroken]

Yesterday.

Mila to Niko:  I’m so happy that I have a new friend.  When you get bigger, I can play with Marganit and Nuella (neighborhood girls) and you will be our new friend.

Today.

I’m so grateful to have Mila who warms our hearts and makes everyday beautiful.

    

Pulmonology

We had an appointment with a pulmonologist yesterday.

So far Niko is breathing fine.  Her oxygen level is at 99%.  Even though everything checked out fine yesterday.  The pulmonologist had a lot of concerns.  So I asked him my usual question, “Have you worked with any I-Cell patients before?”  To my surprise, he answered, “Yes.  One.”

He is in the Pediatric Subspecialties department and he sees a lot of children with metabolic storage disorders.  And though the other various forms of metabolic storage diseases (like Hunter syndrome or MPS) require different treatments, the symptoms are very similar.  Of the doctors we've seen, this doctor is, by far, the most knowledgeable with regards to I-Cell.  More importantly, he seems to know quite a lot about the specific details involved with caring for Niko.

He talked with us about the 3 different factors that will eventually affect Niko’s ability to breathe. 

1)  Her small chest.  Her chest will eventually become too small for her lungs to properly expand and thereby restricting sufficient oxygen. 

2)  Overgrown tissue.  One of the many symptoms of I-Cell is overgrown tissues and organs.  Due to the flawed cellular enzyme storage, she will likely develop thickened valves around her lungs, obstructing her airways. 

3)  Reflux.  With so many organs (potentially enlarged) packed into a tiny body, acids will get pushed up and can enter her lungs. 

He also talked a lot about practical advice.

Sleep position.  Side sleeping is best for her on an elevated bed to prevent reflux.  He made a referral for a sleep study.  I’ve read from other blogs that this is a rather unpleasant experience.  I better prepare myself.  

Reflux and Gas treatment.  We are starting Niko on Zantac to hopefully calm her reflux and get me some much-needed sleep at last.  (Niko wakes up every 30 mins on average!)

Respiratory Rate.  We should occasionally track Niko’s respiratory rate (the number of times her body expands and contracts with her breathing per minute).  It should be around 45.

Insurance.  I was surprised that he spent so much time talking to us about this.  I’m sure it’s because he sees a lot of families go down this road and the financial burden the unfortunate conditions have caused.  We will likely need equipment for Niko.  Oxygen, walkers, and other equipment that I don’t care to think about right now.  This doctor really took the time to discuss ways we can get help or change our insurance plans, because currently equipment is not covered in our plan.   

Therapy.  He talked about how this can sometimes break people or their marriage.  He suggested we try counseling.

Social worker.  He is contacting a social worker for us to see what kind of help we can get (i.e. caregiver, insurance, financial, etc.). 

Next steps.  I will be the first to admit that I’ve been really lost since receiving the diagnosis.  My brain is pretty fried despite the fact that I’m trying to put on a good strong face for Mila, Kiril and family/friends.  I’m certain that this doctor does not know everything.  But he is the first person to actually suggest a plan regarding our next steps.  He’s putting dates and appointments in our calendar to see other specialists.  Though some might prove to be unnecessary, I’d rather see these specialists and weed out the ones we do not need to revisit than miss some crucial steps.