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!

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6 Comments (+add yours?)

  1. Monique Carriere
    Aug 10, 2011 @ 14:29:54

    That is such a nice keepsake for all of you to sit down and read , and update as life and cystinosis changes!! Glad to hear the positive changes for Ali!! Dr Midgley is a fantastic DR and I think his cystinosis patients are near and dear to him!! What I look forward to is the 12 hour cystagon being approved and getting a better nights sleep!! How did your camping trip go?

    Reply

    • Crystal
      Aug 10, 2011 @ 21:31:18

      Thanks, Monique! It totally is therapeutic for me, plus I want Aliyah to be able to see the process one day and how thrilled we were as things became easier for us and for her. Camping was fantastic – I’ll post about it in a few days.

      Reply

  2. Nettie Wiens
    Aug 10, 2011 @ 14:36:55

    God is HUGELY undertaking in this strange journey you have in hand. You attitude as a family is incredible, and the teamwork is beautiful, taking a mountain together, finding that amid the climb that sometimes seems unending, there is strength available in the hard secluded places, and there is beauty, laughter and life-giving moments, that can always be found. Aliyah truly is such a precious little person that she just reaches into everyone’s heart and teaches us so much just by who she is! Hats off to ALL of you, for becoming a medical team!
    Love to you all – mom and dad.

    Reply

    • Crystal
      Aug 10, 2011 @ 21:31:55

      Thanks, Mom! Aliyah is full of beauty and joy and cute chubbiness now – and we’re so thankful for our family and how much support we’ve had! Love you.

      Reply

  3. Karen McCullagh
    Aug 11, 2011 @ 22:23:20

    Ah the life and love of Cystinosis affected families. Sounds much the same as our schedule except meds only take about 5 minutes now. We order the pedia-sure and supplies only once every 2 1/2 to 3 months so that cuts down on the trips into Calgary. You have adjusted amazingly well to this new reality and I see nothing but blue skies in your future. Monique, you are so right about Dr. Midgley and the rest of the ACH Nephrology clinic, they are angels from heaven, each and every one. We are blessed to have these wonderful children in our life to remind us daily of just how precious life is.

    Reply

    • Crystal
      Aug 11, 2011 @ 23:36:31

      Thanks so much, Karen! I was so encouraged meeting you and your son that day in the hospital when everything was so overwhelming. I think we are adjusting pretty well – it helps that Aliyah is such a sunny, chipper little thing, and that we have the most incredible Nephrology team I could possibly imagine. I have nothing but amazing things to say about them all.

      Reply

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