RIP Julian

Another I-Cell child passed away yesterday after battling respiratory infections.
This I-Cell family lives in Sydney, Australia (only minutes away from where I lived while I was studying in Sydney). He was the only person currently diagnosed with I-cell in Australia.

My heart breaks.  These poor little sweethearts, gone before they can even put weight on their legs.    
Rest in peace Julian.  September 9, 2011 - January 29, 2013.

More photos

I just want to purge my phone of all these photos and videos to free up some space.  I'm sure no one is interested in seeing so many photos of Niko, but this blog is pretty self indulgent.   

I used to get really sad when I see siblings (especially sisters) play together.  I mean, I still do.  I had dreams of Mila and a healthy Niko running around, laughing, fighting, protecting each other, keeping each other company, growing up and growing old together.  And now I'm really treasuring the sister time they share together.  

Floor time with Mila.

A very unusual sight around my house - this is the first time Niko actually tolerated tummy time.  A few days ago she stayed on her tummy for about 5 minutes.    

Off the Charts Small

Last Friday we went to another skeletal dysplasia clinic.  We met with 10 different specialists.  They were all worked up about her weight.  "She's off the charts small" they all said.  She is now 12lbs 13oz.  Though they were alarmed about her weight, they were casual about the solution.  They suggested that we try the G-Tube, or at the very least an NG-Tube.  Like it's no big deal.  

It's as if they were saying, "Oh your hairstyle isn't working for you, try bangs."    

Inserting a tube, by surgery, directly into Niko's stomach and feeding her through a bag isn't no big deal.  

Even the NG-Tube, which does not require surgery, is painful to think about.  An NG-Tube is a thin tube that runs up your nostril, through the nose, past the throat, and down into the stomach. 

And they delivered this suggestion like, "Oh if you don't like the scissors required to cut bangs, try a hair clip."  I understand that these are options to consider if Niko is not feeding by mouth.  But so far, she's feeding quite well, a lot better than even a couple months ago.  She takes about 20oz to 27oz of formula or breastmilk a day.   

I know Niko is small.  Yes off the charts small.  But that chart doesn't apply to her.  She shouldn't be compared to healthy babies anymore.  In the world of I-Cell children, Niko is where she should be.  I think that I-Cell kids reach an ultimate weight of anywhere from 13lbs to 20lbs.  I made it very clear to all the doctors that I'm happy with where she is.  Her stomach is completely full after a feeding.  And when I push her to feed more, she spits it up. She knows when to stop.  I'm completely fine with her staying on the small side as long as she's "healthy" for her length and able to meet her very modest milestones.   

She actually looks the healthiest she's ever looked.  She's doing very well.  She has made huge improvements over the last month.  She's now very engaged and social.  She makes direct eye contact with people and smiles often.  She can extend her arms and reach for objects.  This is her newest accomplishment and a pretty big deal in my opinion because soon she can potentially chose her own toys or fetch her pacifier.  I would love for her to gain as much independance as possible so that she can enjoy the world she lives in and make her space work for her. She now bats at objects and plays with toys.  If only she can improve on her sleep then we'll be over a huge hump. 

Here she is reaching, batting and using both hands to bring toys together.  

She's learned to suck her thumb at short intervals.  She's on her way to self soothing!

Rolling over, almost

I know I mentioned that Niko had rolled over once at physical therapy.  Well that was an embellisment.  The therapist allowed Niko to roll to her side, and then she placed her hand behind Niko's back.  Then eventually Niko continued to roll over to her tummy.  So while the rotation was "on her own" because the movement was made only by Niko's efforts, the therapist's hand was there to prevent her from returning to her starting position.  She did this on both sides.

Yesterday, she rolled over on her own… sorta.  She was lying on our sofa/bed.  The bed has a fold in the center which dips in just about an inch or so.  Not at all noticable when sleeping on this bed, but for a 24 inch 12lb 13oz baby girl, it's just enough of a boost to allow her to roll over.  She did this many times.

  

Update on Amber and Gabrielle

A few of my friends have been asking about these two sweet girls, so here's an update.  They are both back home from the hospital.  That's a great relief.  Whew!  Amber was on a C-pap and Gabby was on a bi-pap while at the hospital.  They both looked like mini 13lb darth vaders.  Now they are both home and back to getting oxygen from the nasal cannula.

Gabrielle is back to her normal self more or less.  Amber is still on morphine and maybe using the C-pap at night.  I'm glad to hear that they are back to bringing smiles to their families.  I know that both families really thought that they were going to lose their baby girls this time.

I have not been thinking about Niko's mortality lately.  And I'm glad I don't.  It's not a pleasant thought.  I'm conscious that she has I-Cell and I focus on her conditions and therapies.  But I don't think about her short life span anymore.  I don't immediately equate sickness to death.  However the scary week these two girls have had (and another one year old I-Cell girl, Lucia, was also hospitalized at the same time) is making me face reality.  I have to appreciate this time with Niko.  She is hands down the sweetest little thing I've ever met.  She melts my heart everytime she smiles.  And I want to hold her in my arms tightly for as long as I can.  

Rough season for I-Cells kids

This is Niko's first winter.  And so far so good, knock on wood.  Just a couple fevers here and there.

I have been mindful about protecting her from sick people, but not careful enough.  I think I'm about as cautious as any mother would be of a regular baby.  I don't put Niko into the arms of sick people.  But that's about all I do.  Mila is in preschool, a potpourri of God knows what kind of germs and viruses. And when Mila comes home from school she's all over Niko like a windshield wiper.  But I have to remind myself to be a bit more paranoid, especially during this season.  A bad cold, the RSV virus, the flu… any of those could be deadly for Niko.

The two girls I mentioned in previous posts are very sick.  And I'm pale with worry.  I've been thinking about them all day for the past couple days.  I'm nervous as if they're my own children.

Gabrielle has influenza A.  She's been in the ICU for a couple days.  She was desatting to the 40s with a heart rate in the 200s.  They had to bag her to bring up her oxygen saturation.  X-rays revealed patchy spots in her lungs.  They tried to do a NPW but that caused heavy nose bleeds.  And more disconcerting, Gabby started to have seizures and loss of consciousness.  She's on medication now and they are prepping a bi-pap ventilator for her.  My heart aches for this little girl.  I hope and pray that she will kick this nasty thing and return back to her normal sparkling self.  

Amber is still on the C-Pap machine and lots of heavy medication.  An X-ray of her chest revealed that she's got what is called a "white out".  An X-ray of a healthy set of lungs should look nice and black.  In Amber's case, it's completely white with two tiny patches of gray.  White means fluids.  The fluids are leaking from her heart.  She is in gradual heart failure.  The parents are hopeful that they can bring her home, enjoy time with the family, and then take things one day at a time from there.  Of all the scary sad things Amber's mother wrote, the one detail that pains me the most is that Amber cannot put her pacifier into her mouth because of the C-Pap mask.  That kills me!  

With so few I-Cell kids out there, I feel like they are Niko's sisters.  I hope they both find relief very soon.          

The Boy in the Moon

I have more than a handful of friends who love and care about Niko.  But I have two friends who can truly relate and understand the world that I am in.  They are in similar, but very different, situations.  Much like Tolstoy's quote about happy and unhappy families, all healthy children are alike, but unhealthy children are sick in their own ways.

One of these two friends gave me a book over Christmas.  I have been devouring this book.  I'm surprised that I am able to find any time at all to read, but I'm almost half way through the book.

It's called "The Boy in the Moon", written by Ian Brown.  It's about the incredibly difficult journey of caring for a special needs child.  There are so many decisions to be made without any single right answer.  It's not so much about making good choices.  There's bad and then less bad.  The child in this book, Walker Brown, has a super rare condition called cardiofaciocutaneous syndrome (CFC).  What I’ve learned recently is that most “conditions” are super rare.  With the exception of down syndrome and autism, everything else that goes wrong with your child’s health is mind boggling.  Any condition that is considered abnormal causes most people to recoil in horror.  Abnormal conditions are always bad.  You don't see "special" people who were born with genetic mutations that allow them to fly, to never age, or to have self-cleaning epidermis that eliminates the need for bathing.  

Children with CFC are moderately delayed mentally.  They are often small in size for their age.  Most CFC kids can’t talk or eat on their own.  They are fed through G-tubes.  They are not compatible with toilet training.  Some CFC kids (including the one in this book) self mutilate by punching themselves in the face, bashing their ears in or kicking at their own genitals.  But many learn to walk and have normal life expectancy. The author is a journalist so the writing is smooth, honest and revealing.

Of the many striking topics in the book that caused me to pause and think, my mind went back to this: are we so different?  Have we become “that” family?

The following are a couple paragraphs from the book.

* * *

But let me ask you this: is what we've been through so different from what any parent goes through?  Even if you your child is as normal as a bright day, was our life so far from your own experience?  More intensive, perhaps; more extreme more often, yes.  But was it really different in kind? 

We weren’t disability masochists.  I met those people too, the parents of disabled children who seemed to relish their hardship and the opportunity to make everyone else feel guilty and privileged.  I disliked them, hated their sense of angry entitlement, their relentless self-pity masquerading as bravery and compassion, their inability to move on, to ask for help. They wanted the world to conform to their circumstances, whereas – as much as I could have put words to it – I simply wanted the rest of the world to admit (a minor request!) that our lives weren’t any different from anyone else’s, except in degree of concentration.  I realized I was delusional.  People often said, “How do you do it?  How are you still capable of laughing, when you have a son like that?”  And the answer was simple:  it was harder than anyone imagined, but more satisfying and rewarding as well.  What they didn’t say was:  why do you keep him at home with you?  Wasn’t there someplace where a child like Walker could be taken care of?  Where two parents wouldn’t carry the whole load, and could have a moment or two to work and live and remember who they were and who they could be?

* * *

My answer to the questions:  are we so different?  Have we become “that” family?  I would like to say no, we are not different.  We are normal.  But I don’t agree with the author of the book.  It’s foolish to pretend that our lives are not any different from everyone else’s.  While I certainly do not want any kind pity, we are different.  We have become “that” family.  I’m painfully aware of this now.  I see people looking over and smiling at Niko.  And a moment later the smile drops from their eyes while their lips remain frozen.  The lower half of their face cooperates with the façade that is their smile while their eyes betray them.  And I immediately know what they’re thinking, “I must not let them see my shameful uneasiness.  I must not upset “that” family.”  They have already compartmentalized us into a subgroup in their brain.  We are “that” family.  And what exactly is “that” family?  Group: “Special Needs” family.  Subgroup:  person(s) with deformities. 

I used to think that people with “special needs” or “abnormalities” belong to the other side of the line.  I didn’t really think that I was better than them.  I just subconsciously thought that abnormalities happen to families with abnormalities, certainly not to normal people like me or my friends.  I thought that the “special needs” family was a race within itself, as defining as the colour of one’s skin.  Unfortunately I know now that no one, nor any group of people, is immune to it.  And it happens a lot more often and to a lot more people than they are willing to admit or share.

When we go to all those hospital appointments, I know that the doctors and specialists see us as the “parents of an I-Cell child”. And I want to grab them and shake them and say, “No we are normal people!  This is a fluke! This should not have happened.”  But it did.

This fluke that has happened greatly impacted me in two ways.  First is the overwhelming love that I have for Niko that is often too painful to bear.  The second is my need to protect her… from stares, from questions, from judgment.  I see her as my vulnerable broken little angel.  And the protective maternal instinct is at an all time primal high. 

Little Amber

My heart is breaking.  

When I first learned that Niko has I-Cell, I searched everywhere to get my bearings.  The first blog that I found was Amber's Adventures.  Amber's was the first face I saw and the first child to introduce me to what is now a very familiar world of I-Cell.  It is through her blog that I discovered the online community of I-Cell families (with whom I communicate almost on a daily basis).  By reading about Amber I learned about the fears and also the joys of bringing up an I-Cell child.  I learned how to revel in the small stuff.  Small hands ringing bells, small mouth eating food, small face wearing a frown.  Better yet, a small girl wearing a smile.  A small child celebrating a birthday.  

Amber is now very sick.  She, along with the other I-Cell kids, fight viruses everyday and many stay at the hospital often.  But this time, we are all worried.  She is in serious condition.  It seems that Amber's little heart is failing.    

A note from her father.
"Had a really bad night finally got her calm enough to sleep for a couple hours around 2am. We have her on CPAP now and she is holding her sats but her heart rate hasn't come down from the 160s at all even with constant morphine and ativan. Most of the time she has been very agitated and hasn't stopped coughing and wheezing with the exception of a little while at the peak of her medication this morning. We are waiting on cardiology now to better assess how bad her heart failure is and how long they think her heart can handle the sustained stress. Once that happens we will need to decide which direction to take from here. ( if possible to get her heart rate back down and get her home for a while longer or to medicate her more heavily to give her the maximum comfort possible until her heart stops.) Thank you all for your thoughts and prayers it means a lot."

Dear Amber, I wish I can give my arm, my leg or my kidney to protect you, to protect all our i-cell children. If I could, I'd do it in a second. I hope you will be become I-Cell free calmly and peacefully.

I'm shattered.  Im a wreck. I'm in tears thinking about this girl, a girl who just turned three years old, who most likely weighs no more than 13 pounds.  A girl who has done nothing to deserve this.  She was innocently born with the same disease that is destroying my daughter's body.  I-Cell is so fucking cruel.  I hate you I-Cell!

Sister Time...

Winter in Santa Fe

We just came back from our winter break in Santa Fe.  Niko loved the snow, the crisp air, the winter light and the attention from family.  

Her favourite part of the day was the walks in the cold cold air, even when it was 5 - 10 degrees below freezing.

I love her chubby cheeks here.

Mila went nuts playing in the snow covered garden. 

I love snow covered adobe homes.  Hardware outlined by snow.

Niko seemed to have notice that the houses were not freestanding structures, rather they grow naturally out of the earth to provide shelter from the cold.  

My feet in virgin snow.

Keeping an I-Cell child alive

Caring for an I-Cell child is difficult but rewarding at the same time.  Niko is still a baby, and at her healthiest right now (her geneticist keeps reminding me).  So caring for her is not extremely different from caring for a normal baby.  Perhaps caring for Niko is more similar to the care of a newborn baby who is not quite ready for life yet. However as I-Cell children grow they demand more attention, equipment, medication, etc.  

The following is written by the father of another I-Cell child, Gabrielle.  She is 2 years old.  She's beautiful and has the wisdom of generations in her eyes.  She loves music, flowers, the beach and older boys (She's got brothers).  She brings great joy to everyone she touches and fills her parents with more love than most people can bear. Her parents are optimistic and inspirating.  But they are not exempt from the daunting task of code-red care giving. Fear and worry cast a shadow over their lives everyday. Her father describes what it's like to care for little Gabrielle.   

Beautiful Gabrielle

* * * 

Keeping an I-Cell child alive is a lot of work.

Fear of oxygen cords popping off. Chronically checking the tank regulator's dial to ensure it hasn't been nudged ... landing between two settings that shuts the valve off... shutting off our daughter's air supply.

Did we bring enough O2 for the trip?

Lying in bed, dead tired, almost asleep, and hearing your daughter cough, hoping it's not a bad plug. The cough turning into an abrupt silence. 5 seconds pass. An eternity. The sat machine goes off. Levels dropping. Jumping out of bed. Lift her up. Suction machine. Crank up dial on 3-foot-high O2 tank that takes up a good portion of her room.

Feeding her mushed up food knowing that every cell of nourishment won't be stored by her body properly. Every bite progressing the disease. The work of it all. Both for her and for the parent.

The brittle bones. The scoliosis. The kyphosis. Other kids running around and jumping. Wincing and hoping that they don't land on her and crush her. One wrong step. Oh god.

The hands that don't work properly. Those beautiful hands that hold the entire world within them. The ones that need braces put on them when she sleeps so she doesn't lose her range of motion due to the disease contracting them permanently.

The deafness. The near-sited partial blindness. The dislocated hips. The rib-cage and sternum that breaks your heart every time you look at it. Wondering how much pain is she in? Any? Is she just used to it?

Turning the sat machine off some nights at 4:45 in the morning b/c you just want to sleep for a couple hours. Hoping that she'll be okay when you wake up. But the guilt you feel when you see her lips a pale blue in the morning.

The fear of flus and fevers and other people's colds. Knowing that your daughter is a bad cold away from passing has become normal. A crazy normal. But not wanting to bubble your family away in protective fear forces you to come to peace with this reality. Someone's bad sniffle could take your baby girl's life. The worrying becomes normal. A normal not many can understand.

The cries. The bone-chilling, heart-breaking cries that make you want to give your life to take the disease away from her. Cries that – as much as you try not to – make you want to cry with her.

The sadness. The long goodbye that you don't want to end but know has to.

The life lessons you didn't think you would learn until your 70's or 80's, if at all. Seeing the finality of everything by coming to grips with your child's mortality.

The distance in perspective that grows between you and what seems to be everyone else ... except for other parents who've endured the chronic sadness of slowly losing their child to something beyond their control.

Keeping an I-Cell child alive is a lot of work. It makes me appreciate my own health. The good health of my other family members. It's a phenomenal gift so many of us take for granted. I wish I could give it to my daughter.

8 months old and a night to remember

It's now 2013, we just spent two weeks in Santa Fe, Niko saw snow (heaps and heaps of snow) for the first time, she now can reach for toys with precision, I discovered a great Hakka restaurant, there are three new cafes serving third wave coffee in my hood, the Chinese are building a new town in the Peruvian Andes 14,000 ft above sea level, blah blah blah.  All of that would have been news worthy in my world, but it's immaterial compared to what happened last night.

Yesterday, Niko turned 8 months old and she slept through the night!  This was the first time in her entire life.  Last night she slept from 9:30pm to 6am.  Up until now, she's been waking up about 5 to 6 times a night.  I suspect that Niko and my bed have a secret alliance, with the sole purpose of seeking satisfying humor by ejecting me from bed repeatedly, night after night, eyes blood shot, hair pasted to my face, skin the colour of cracked concrete, and my night gown soiled by sour milk. The routine involves fixing her bottle, then topping her off on the boob, followed by rocking her for 10 to 20 minutes before she drifts back to sleep.  Repeat 5 to 6 times a night.

I'm realistic.  I don't dare to wish that she will grant me another such night.  But I took many photos of her this morning to capture this event, this feeling.  This is the face of a little girl who slept through the night.

The morning after, I want to remember you like this always.  

Oh! It's midnight.  She just woke up.  After one heavenly night, the old routine continues.