These days in 2011 – An Anniversary we won’t forget

It’s funny that the “anniversary” of cystinosis in our lives isn’t June 6 – the day she was diagnosed.  Cystinosis happened in our lives far before we knew it – before we knew Aliyah was a girl, before we heard her heartbeat, before we even knew she was.  But the anniversary feels like the whole end of May and beginning of June, from the first realization that she was losing weight to the tearful phone call to the pediatrician, pleading for an earlier appointment, all the way through to those first long, fearful weeks ago home in a new lifestyle foreign and frightening to us.  Oh, life has changed.  It is now fresh, full, and joyful, even if it’s dictated by a six hour clock.  But oh, do I remember those days.

A letter to us – one year ago.

Dear Self,

I see you sitting there on the couch in your small townhouse living room, your two darling towheaded girls asleep upstairs. Your fear is so deep it burdens heavily on your chest, takes your breath away, stifles your voice.  You cannot ask the questions you are thinking – they are too dark, too fearful, and far too real.

The tests your baby had today are only the beginning.  You have several weeks ahead of you that will test your faith and hope further than its ever been tested.  Only now, looking back over the last few months, have your eyes been opened to the reality of little Ali’s health – her weakness, emaciation, inability to roll over or sit up and now, her increasing inability to even hold up her head.  Her chronic constipation and incessant thirst seem the least of your worries.  You wonder how in the world you could have missed all of this.

You have a long road ahead.  In the next weeks, you will weep many tears as your small, not-even-one-year-old daughter will be poked with needles, strapped to boards for ultrasounds and other invasive tests, and attached to heart monitors.  You will watch her throw up more than you think she can even take in, her little head hanging in limp exhaustion after each vomit.  You will lean your forehead on the white railings of the crib and stare at her, your vision blurred with tears, while the doctor tells you he’s “95% sure” and “it’s a diagnosis I wouldn’t want for my child”.  Every visit from the doctor or medical team will leave your head spinning with questions and information overload, and underneath all of it, your heart will break because the life you had hoped for your precious baby will not be.

You will not believe it when people tell you it will get better.  In your journal, you will record all of the 10 medications Ali must take in the tube through her nose, four times a day, every. damn. day.  You will record every time she throws up and every time you have to re-dose the medication.  In the small family room at the hospital, the pharmacist will patiently explain how to calculate the correct dose so you can mix powder with water and sustain Ali’s life, fighting the little crystals that threaten her cells every day. With a resolution you didn’t know you had, you will learn how to pull an ng-tube out of her nose and re-insert it, while she wails against you, not understanding this new life she faces.

In the dark of your parents’ basement, while someone else gives you respite and watches over your daughter, you will weep tears of fear and despair and hopelessness and guilt.

That first time you take Ali home for the day to celebrate her first birthday, and you fearfully give her all her meds without the aid of the nurses, and she throws it all back up?  That will happen again.  And again.  After the first few weeks, you will not be filled with fear but, armed with some experience and understanding, you will cope.  You will have months of sleeplessness – with two hour feeds in the middle of the night, constant vomits, going through 5-pairs-of-baby-pajamas in just as many hours – but those will pass.  The first Thursday night med night (med nights are on Mondays now) will take you 4 hours and you will only pack 4 days worth of medications, and you will look ahead at endless years of an onerous and discouraging weekly med-prep.

Clean syringes drying

But now?  Now you can do it all in about 30 minutes (not counting washing all those syringes), and while watching ‘The Bachelorette” too.  The tremendous fear of the internal damage that happens in Ali’s body when she doesn’t miss a dose is still daunting – but now you can accept your humanness when the alarm isn’t properly set and you miss a dose, and your world doesn’t melt around you if she throws up.

First Birthday

You will doubt God.  You will question how he could be a God of love in a world of suffering, how people see you as strengthened by your faith when others you have met without faith seem stronger than you.  You will explore atheism and evolution and agnosticism, and you will let your doubt control you.  But you will also be surrounded by people who care, listen, truly hear, and pray.  You will not lose faith.  But you will walk around in the dark for awhile.  Perhaps for quite a long while.  That’s okay; God is God in the dark, too, and he can handle all of your questions and uncertainties.

You will not believe it when people tell you it will get better.  After all, what do they know?  You will be overwhelmed when you realize only 12 other Albertans struggle with this, and you will not appreciate anyone who tries to encourage you.  Cynicism is an ugly thing and you don’t wear it well, but you are so blessed to have friends who can tolerate you in your ugly moments!  It IS true that they may not know what’s like, but regardless, they’re right, it will get better.   Your three-year-old will help you administer supplements and vitamins, and will know words like potassium and cysteamine before her half-birthday.  You will manage endless rounds of vomit and countless loads of very smelly laundry without losing your own lunch!  You will learn early to trust both sets of parents to Aliyah’s care and thus will have some much needed sleep on occasion.  You will even go camping with all those meds – in a tent.   You still don’t wear a watch and you rarely miss the exact moment at noon/6/midnight/6 when the next dose is due.

So here we are, one year later, and while your life is entirely different, the little baby you thought you were losing is bursting with personality and energy and zest for life.  She is no longer immobile.  Her head doesn’t lag when you lift her up.  She is walking, running, dancing, learning to leap!  With the vocabulary of a four-year-old, she chatters your head off about anything and everything, with a sense of humor destined for some stage somewhere.  Her sleep is uninterrupted by meds or throwing up; she no longer pees through everything she wears; she doesn’t drink fountains of water and she actually likes food.  She is adored by her older sister, doted on by her grandparents.  And she has a far deeper faith than her parents.

Flying!

Your reality is not the same anymore.  Neither is Aliyah’s.  But it is full of hope, full of love, and full of dazzling joy.  You are surrounded by people who have cared for and prayed for and blessed you and your family; you have a new home; meds are not a daunting, hopeless, overwhelming, life-consuming task but rather just part of your daily life.  You still have questions – your daughter’s future is so full of unknowns – but then, whose future isn’t?  And truly, you are full of gratitude.  Gratitude for the science behind Aliyah’s ability to survive and be healthy within the diagnosis of cystinosis; gratitude for the technology and support of medicine; gratitude for the compassion of hospital staff and friends and family; gratitude to a God who gave you and your daughter a second chance you feared (this time last year) would not exist.  You have learned to relinquish control and rest in the now, the only time any of us have.

So on this long “first anniversary” of cystinosis, you recognize and understand the challenges.  You are aware of the unknowns that lurk ahead, of the trials that you and Aliyah – and Sara, as she experiences and copes with her sister’s condition – will each face.  But you do not mourn, not now.  You rejoice that your friends and family have patiently stood by and listened to your frustrations and fears, that your husband has been a solid rock even as he walks inside of his daughter’s diagnosis, and that God has faithfully guided you in the dark.

On this one-year-anniversary, you CELEBRATE!

What Map Do You Use?

We watch a lot of Dora the Explorer in this house.  My girls are permitted 30 minutes of Tube Time daily, for their entertainment and my sanity.  Dora’s usually Sara’s selection, and since she’s the oldest, she usually gets first choice, although the older (and louder and more opinionated, if that’s even possible) that my littlest gets the more likely we’ll be watching Go Diego Go more often.

Within the first few minutes of Dora, for you unlucky folks who’ve never had the privilege of meeting her, she sets off on an exciting adventure to a fantastic destination (like a fairy-tale castle or a treehouse party) and realizes she’s lost.

“Who do we ask when we don’t know which way to go?” says Dora.

(Jesus, I usually say out loud, but of course that’s not what my girls say).

“Map!”

And out comes this rolled-up scroll of a happy map, singing it’s happy “I’m the map” song (do you have it in your head yet?).  And this happy map navigates for them the very adventurous, dangerous-but-only-in-a-toddler-friendly-way path to their destination.

Oh, if it were only so easy.

Seems to me that we are all on our own personal quest to some unknown destination, and that happy little map in Dora?  He doesn’t usually show up on our radar.  Sometimes we don’t really (seem to) need a navigator; the little boat of our lives floats us along the tranquil waters of a great career, or an easy loving, or a circle of fabulous friends.  But at other times it’s like our little boat has been thrust into tumultuous waters and we really don’t know which way to go.

The first time I white-water rafted down the Nile (doesn’t that sound smashing?) proved one of the most thrilling rides of my life.  I had triple-thick blond Amazon Woman extension-braids in my hair, a dark Ugandan tan, and a ton of courage…before I saw the white water.  Um.  Do I have to get in that little rubber dingy???  But I did, with 8 other scared passengers, and we paid excessively close attention to the instructor and his instructions before we headed downstream.  Now, white-water rafting (which may be boring to kayakers but is a wild-ride for we-who-dare-only-in-our-imaginations) on the Nile is a mighty different thing than white-water rafting on a Canadian river, largely because a Canadian river is full of rocks (that’s why the water’s so bubbly) and freezing cold.  You don’t want to fall in.  They tell you not to fall in.  But on the Nile, the white-water comes from the incredible depth of the water and the convergence of rivers, and the water is balmy and full of alligators.  Supposedly.

And the first rapid we came to, our guide said, “So, do you want to stay in the boat or fall out?”

What?  My boat of chickens voted to stay in the boat.  Which the guide honored.  The first time.

The second time, we thought the guide had listened to us when we said we wanted to stay in the boat.  He told us to paddle left.  Paddle hard!  And we did!  But those bubbly, churning, frothing waves just kept coming closer!  And suddenly our boat was vertical on the horizontal and we were flung into that mass of water.

It was like being in a washing machine.  I did not appreciate my extensions at that particular moment.

Later when we climbed into the boat, we actually thanked our guide.  It was terrifying…but enormously fun!  When it was over, that is.

Navigation of this life sometimes feels like that…like the guide knows where we’re going, and has instructed us for various situations, but we are headed in blind and don’t appreciate the ride until it’s over.

So often when we’re charting these waters we feel alone.  We know so few who have rare diseases or conditions!  Did you know that a rare disease is typically classified as one that affects less than 1 in 2000?  That means that a rare disease affects 18,000 or fewer in Canada.  Cystinosis affects approximately 50 people in Canada.  Talk about a lonely boat!  And I live in this lonely boat as a parent of a child with cystinosis, and not as a person with cystinosis myself.

There are a lot of things that make living with a rare disease/condition difficult, frustrating, frightening, and lonely, don’t get me wrong.  We struggle with questions that parents shouldn’t have to struggle with:

How will our daughter cope with foul-tasting medications?
How long will she have muscle strength?
Will she ever be able to have biological children?
Will she feel nauseated for most of her life?
How will she cope with “being different”?  Even when others don’t know she is?
Who, when so few are affected, will contribute to the research for better treatment and, dear God, please, a cure?

And others that I can’t even bring myself to write.

Eight months ago, when we boarded this lonely boat, our Guide seemed oddly silent, not giving us “fair” warning when we were tossed into the waves of heartache, disappointment, fear, and aloneness.  And yet, we had been prepared.  Words of encouragement, guidance and instruction permeate our Map.  Things like

“Be strong and of good courage, for the Lord your God is with you wherever you go.” (Joshua 1:9)

“Be strong and take heart, and wait for the Lord.” (Psalm 27:14).

“…we also glory in our sufferings, because we know that suffering produces character; and character, hope” (Romans 5:4)

“In this world you will have trouble.  But take heart!  I have overcome the world!” (John 16:33)

Such words glimmer like little stars on the pages of Scripture.  Verses that speak of hope inside of despair, love destroying fear, life breaking free from death.  We have a Navigator.  He left us a Map.

It is so easy to forget.

In celebration of Mobility: Aliyah Update #2

Playing in the leaves before her cold

October and November was a long silence on this blog.  Aliyah got sick.  It started with a common cold – I fondly called her my little snot faucet – during which she sneezed, coughed, snuffled and sniffed but was otherwise her normal happy self.  Within two weeks, it mutated into an ugly virus that caused all kinds of heartache and worry.  Aliyah couldn’t hold down food, didn’t drink (that’s a huge indicator of Aliyah’s health as she normally drinks like a horse – and pees like an elephant), and lost all the beautiful pounds she put on in September (so, really, only one pound – but on a wee smidge like her, that’s a big deal).  I wanted to be all cool and collected and manage this flu without too much fear, and made it to the peak  before we did a little trip to the ER.  Of course, that was the worst night of the flu and she was on the upswing after that.  She did learn to tell us when she has to “pook,” and now, every time we pray she reminds us to pray she doesn’t “pook” – which is endearing and sad at the same time.  At any rate, as of November 14 she’s been healthy and gaining once again.

So, every 3 months we get to make the long trek up to the Children’s Hospital for a loooong appointment that starts with bloodwork and ends with a lively discussion with the doctor regarding Aliyah’s current health and any concerns we have.  November 27 was our second of these “cystinosis clinics” and SUCH good news came from that!  First of all, she no longer needs iron or activated Vitamin D, the first of which makes her sick and the second of which is very expensive.  Her sodium phosphate was decreased and all her other electrolyte numbers, even after being so sick, were “brilliant.”  She had started gaining weight (but was still below previous weights).  She is now en route to 20 pounds, hopefully nearly there.

But the best part came today, with the phone call from the cystinosis nurse.  Aliyah’s cystine levels, which were 3.8 in June and 0.7 in September, are now at

0.02!!!!

Merry Christmas to us!  That is definitely the best gift we could have received.  What that means is there is very little damage currently occurring in her cells.  We are thrilled!

In addition to all this fabulous news, we have been hoping to eliminate the medications from Aliyah’s regime, and as of two weeks ago, she no longer needs her anti-nauseant medication.  This is another great “drop” from our medicine cocktail!

In other Aliyah news:

She started crawling two weeks ago, and has the cutest little wiggly-bum when she does it.  And she started walking on teetering toddly legs last week!  While she still doesn’t prefer walking (it is, after all, fairly slow and involves lots of falls), we often find her walking ’round and ’round the couch, giggling to herself.

Aliyah's Great Strides

Last week, her G-tube was replaced with a Mic-Key (Peg), which is a tube that we hook-up and take off when she is done, which definitely makes for easier movement.  The initial exchange was painful, but now she tries to put it in herself.

Aliyah still talks up a storm – her new obsession is “na-na-niles” (crocodiles) and Santa (on who’s lap she happily sat).

On the parental front, Bob and I are managing to get more sleep as well.  We’ve figured out a feed schedule whereby the midnight meds only take 15 minutes instead of 45, so I can go to bed at a normal time and wake briefly at midnight before sleeping the night through.  And since we have sleepers (yesterday our girls slept until nearly 9am), I’m quite well rested.

All in all, a very positive month.  Aliyah’s nose has started running again, though, so here’s hoping it stays just a cold…

Proud as punch

 

It’s the little things

A friend of mine recently made an interesting comment to me.  I was just administering some of Aliyah’s vitamins, and she said (something like), “I was just thinking how normal you seem.”

Ha!  If only she knew.

I just shrugged modestly (because of course, while I am super-confident super-mommy, I must not give that away, and I can’t think of witty comments spur-of-the-moment anymore – you need to be well-rested to do that).  Later on, while contemplating our family’s normalness (or lack thereof), I realized a few things.  Firstly, we do have a major challenge (cystinosis, not Aliyah) to live with daily; secondly, we are totally normal (because who does not have some kind of major challenge to live with?); and thirdly, we are totally not normal because we have a lot of funny (or weird) little cystinosis-related things that make our days unique.

And I don’t want to forget the little things, because they are what make us laugh, cry, and overcome!  Here are just two of the funny little life-changes we face.

Syringe-Stair-Walking

Aliyah has become mobile.  Rather suddenly.  She isn’t walking yet, at 16.5 months, but she bum-scooches everywhere, her particular favourite location being the stairs.  And actually, she’s a pretty darn good climber already, largely because when she gets to the top (with me closely behind her), she wants to climb back down again (on her feet, which is only slightly frightening), and when she’s back at the bottom, she turns around to begin all over again.

What does that have to do with syringes, you ask?

Well, if I had any common sense, I’d do all of Ali’s liquid vitamins sitting at the kitchen table.  But since she likes motoring around, I’ve started just pulling the tube out of her onesie and popping in the vitamins in, one by one.  And since she beelines for the stairs every time, I’m left with a little baggie of vitamins climbing up the stairs, trying not to pinch the tube (and thus wear a lot of strange smelling vitamins) while I administer these tasty treats.  I’ve learned that it takes 6 stairs, minimum, to empty all of those syringes into her g-tube.

I’ve also learned that smelling like potassium isn’t so bad.

Urine Enough Diapers Already! (haha, get it?)

About eight months ago, I was a cloth diapering tree-hugging mommy, doing my best for the environment by diligently covering her little bum with cloth.  Then she decided water was “the thing” and breastmilk was no longer interesting.  Within days of Ali becoming a water-lover, I was wondering what the heck I was doing wrong with her diapers – she just soaked through them constantly!  And they shouldn’t have been leaking, since they’re BumGeniuses and no small cost (well, unless they’re from Kijiji, which mine are).  Now I understand the problem.

Aliyah is a pee-tsunami.

She doesn’t trickle, this baby of mine.  No, she’ll be dry one minute and flooded the next.  Today, in fact, she drenched through 4 pairs of pants and onesies, never mind diapers, and not for lack of being changed!  She drinks like a camel (only more often) and pees like an elephant.  She did manage to pee on the toilet for the first time yesterday, by her own will, so hopefully this incredible pee torrent will lead to swift potty training.

We change her diaper before bed, at midnight, and at 6am, and she’s wet every time.

Sorry, dear landfill, I can no longer do my part to save you.

Anyone want some Bum Geniuses?

In celebration of chub: an Aliyah update

It has now been nearly three and a half months since Aliyah’s diagnosis, and life has certainly changed for our family!  Our little munchkin has gone from frighteningly tiny and lethargic to a still-tiny-but-not-scary-tiny bundle of ridiculous, non-stop energy and laughter.  Here’s the update.

The food update

Some have told me that a baby/toddler will never starve himself, no matter how little he seems to eat.  In Ali’s case, that does not ring true as many cystinosis kids have a really hard time eating.  To this day, I’m not quite sure why: is it cystagon that suppresses the appetite, or cystinosis itself?  Not sure.  Regardless, one of our earliest concerns about her was how incredibly little she would eat at any given meal.

That concern continued for a long time.  Many families whose cystinosis children have g-tubes have been really concerned that their baby will not take food orally.  For the last three months, we have laid off oral feedings with Ali, choosing instead to hope that she’ll gain weight through tube feedings.  We would still sit her at the table with food, but her preference by far was to toss it over her high chair rather than put it in her mouth.

Well, in recent days, that has begun to change.  She has definite favourite foods now: spaghetti, carrot sticks (to suck and slobber on and then try to feed to mommy – gross!), yogurt (vanilla only – just like her sister), cheerios, peaches.  At supper time she practically falls out of my arms to get to the food!  She also loves to drink her formula – through her syringe.  Sometimes she’ll drink half the dose!

Bottom's up!

Certainly, she does not eat enough to sustain herself, and still gets the full tube feedings while we attempt to grow into the growth chart, but it is a definite improvement and encouragement.  People often ask if she’s allowed to eat through her mouth.  Heck yes, and anything she darn well pleases!

The Growth Chart Update

That said, I guess chub is a strong word for our tiny little Ali, but truly, she has gained about 1/4 of her birth weight in 3 months!  When we were admitted to the hospital two weeks before Aliyah’s 1st birthday, she weighed 11 lbs – barely more than some babies’ birth weight!  At her 1 year check-up (3 months late), the scale shouted 16.5 lbs!  And counting!  Every gram brings her that much closer to that beautiful day when she can face forward in the van. (:

The Cystine and Electrolyte Update (**and, to me, the most important of the updates**)

Almost three weeks ago, Aliyah had her 3-month cystine appointment, the time when her blood is tested for cystine levels in her body.  The first test on June 3 revealed her levels at 3.8 – when they should be well below 1.  Today, the pharmacist called to let me know Ali’s white blood cell cystine count is now 0.7!!!  A phenomenal drop – but not quite the target level of 0.4, so now our cystagon levels are on the rise again.  Apparently it’s due, in part, to the speed with which little Ali is gaining weight.  No complaints here!

Our doctor decreased two of her supplements, charted her in the 3rd percentile for height and weight, commented on her incredible astounding vocabulary.  (She has several dozen words that she uses on a regular basis.  They include whale, book, puppy, bubbles, bath, more, thank-you, and various animal sounds).

The Motor Development Update

Two weeks ago, our home care nurse visited, bringing with her a visitor – a physiotherapist to look into Aliyah’s lack of mobility.  Now fifteen months old, Aliyah still had not rolled from lying down to a sitting position, nor did she crawl (a source of frustration to me; she just grins and lets Sara bring her everything she wants, the little queenie).  Within two days of encouraging particular movements, she has, today and for the first time, sat up by herself!  (<– Note: that was last week, now).  While camping last weekend, Aliyah discovered transitioning from sitting to standing, but didn’t seem interested in continuing the exercise until today, when her bath consisted of up-and-down, up-and-down from bum to feet, with a lot of happy and proud giggles.  One of the most fun things I hear these days is, from Sara, “Yay!  You did it, Ali!”  and an echoing, “Yay!” from Aliyah when she accomplishes such a feat (she loves cheering for herself and does it quite regularly).  Now, she is beginning to scooch on her bum from one place to the next, and has started pulling herself up on any piece of furniture nearby.  This is SUCH a joy – and now we have to baby-proof the house again!

It’s not all easy.  Some days I miss her flat, perfect tummy – with no tube in it.  Her hair and skin and breath has started to release the faint chemically sweet odor of cystamine – and I miss that fresh baby smell.  She still throws up on a fairly regular basis (particularly if she bonks her head pretty hard).  Bob and I look forward to a break in the night-routine, and know that Aliyah won’t get that.  I wish, sometimes so deeply that it hurts, that she did not have this horrible disease.

But she is growing – she is happy – she is a fighter and a cheerleader and a laugher and a breath-takingly wonderful baby girl.  We are so thankful for her, and so proud of her.

We named her Aliyah, which means “to ascend” in Hebrew.  Our prayer is, indeed, that she rises high above her condition and lives life triumphant, joyful, and free.

Her next appointment is the eye appointment next week – and then in November another cystine check.  And so the routine carries on.

Parenting Aliyah

Some of you have asked what our lives look like now.  Since it’s changing so rapidly, it’s kind of hard to say!  The pathway on what started out as a seemingly impassible mountain is beginning to widen, the slope to decrease, and the pace to relax, so we can freely say everything is looking smoother from here, although it isn’t without bumps.   But parenting Aliyah is a joy beyond words.  For her sake, I would erase cystinosis in an instant.  But I would take her any way she comes, she is just that amazing.

These are the new pit-stops on the Walker Highway of Life (haha).  (If you have cystinosis or a child with cystinosis and are reading this, I’d love to know how your schedule compares…).

Meds and Feeds

5:45am-6:30am (or so) – Bob’s shift

11:30am-12:10pm (ish) – Crystal’s shift

4:45pm-6:15pm (kinda) – Crystal or Bob’s shift

11:45pm-12:15pm (thereabouts) – Crystal’s shift

Each dose includes some or all of the following syringes:
Supplements: calcitriol (breakfast only), potassium, dicitrate, sodium phosphate, carnitor, iron
Medication:
locec (supper only), timotheprim (midnight only) cysteamine.

Each feed starts with about 60 mLs of formula and the supplements (6 at breakfast, 5 at all other times).  It takes about 15 minutes to administer into her g-tube (which is a tube in her stomach.  The meds taste icky and Aliyah doesn’t like to eat, although, lately, she’s been sucking the formula out of the syringe.  A good start).

About an hour after the first part of the feed, Aliyah receives cysteamine and the last part of the feed (also 60mL).  Sometimes this last feed is given in one dose, sometimes divided into 2 smaller doses, depending on how she’s responding.  For awhile there, she would throw up 3-4 times a day, but since the new antacid has been introduced, that has decreased significantly.  As in, about once every two weeks and usually in conjunction with a missing nap.

Sara likes to administer these feeds, under great supervision.  She’s my “little nurse.”  Hopefully, she grows into deep and active compassion, gentleness, and empathy through this!

Our little nurse

In between feeds, life is pretty normal.  We go to the park, play in the pool (yay summer!), clean the house, colour, dance.  All the good fun times of a toddler, her baby sister, and an entertaining (and entertained) mommy.

A busy calendar

Daily preparation of cysteamine, the primary medicine, by mixing pills and water and drawing syringes.  She’s currently on a 112.5 mg dose/6 hours and we’re tapering up again because she grew!

Monthly trips to Shoppers for an antibiotic Aliyah requires (preventative) and her antacid.

Weekly (Thursdays, so far) med prep nights.  It takes 2 people 1.5 hours to pull 130 syringes full of meds.  The time it takes is shrinking, though!

Weekly (at the moment, not forever) visits from the nurse to weigh Aliyah.

UPDATE: Aliyah weighed just under 13 lbs when we first were admitted to hospital June 1.  Today, she weighed in at 15 lbs 13 oz – almost 3 lbs in two months!

Every six weeks, ordering food supplies and formula and picking them up from the warehouse.

Quarterly (Sept., Dec., March, June) visits to the Alberta Children’s Hospital for the cystinosis clinic, blood tests whereby Aliyah’s cystene levels are evaluated.

Quarterly trips to Shoppers Drugmart for a boatload of meds.  Must say, the pharmacists’ eyes widened a bit when they saw Aliyah’s file.

Yearly order of colour-coded syringes, which are (thankfully!) sent straight to our door.


I know it sounds like a lot.  But if I’d written up the schedule in the first few weeks that we were at home, it would have been several pages long with multiple dosage times!  It is getting easier to manage and the times for all of the preparation less cumbersome.  The hardest part is actually not the schedule, but the realization that by the time Aliyah is administering her own meds, she will never again experience more than 6 hours of sleep in a stretch.  Rarely does either one of us do both the midnight and the wee sma’s dose; we have been able to leave the girls’ with our parents overnight for a “vacation” from the meds.  Aliyah will always have to do both and will never get a vacation.  Even the hope of a delayed release drug is hard to hang onto, as it will be ridiculously expensive and not covered by insurance.  But she is a trooper; it will be her routine; she will be a strong, compassionate, and napping young lady!

I look forward to rewriting this as it gets quicker and easier.  Eventually, according to one mom, we will notice that she has cystinosis “four times a day, five minutes each.”  Can’t wait!