My Story, pt. 3 — the one with Mr. T

and now, the shocking conclusion!….

But things weren’t all normal.  Hope never cried.  One of the quirks of Prader Willi.  When she finally had the traditional “1st Birthday Party Meltdown” it was the first time that most of the family had ever heard her cry, and Grace in particular was very upset by it – she had never seen her sister cry like that.

I spent six months hooked up to a breastpump, and for the first three months it was a constant cycle of pumping every two to three hours, then trying to get Hope to eat from the bottle before giving up and feeding her whatever was left through the NG tube.   To put that into perspective…

12:00 – 12:20: getting organized for pumping, pumping, detaching from the pump, putting the milk away for the next round.  Usually Tammy would get up and get my supplies for me, and cart them away when I was done.  I just sat there like a zombie cow, which basically, I was.  Tammy would also change Hope’s diaper during this time.

*Incidentally, did you know that there are different sizes of breastpump shields?(the cup thingys that actually attach to the breast)  Well I sure as hell didn’t!  Not for several weeks anyway.  In the hospital they just give everybody “medium”.  I had never used a breastpump before, Grace had gone from the boob to a sippy cup.  Every night (or darn close to) I would go to Target or Babies R Us looking for the perfect bottle/nipple combination for Hope and to stock up on disposable milk storage bags.  One night, at Target, I stood in front of the Medela section and noticed the rows and rows and rows of breast shields.  I must have stood there for five minutes, thinking to myself, Why do they have all of these, do people lose them?  How could you lose a breast shield? Then I realized they had different letters.  “F–K ME!” I shouted (seriously, I shouted this out loud, in Target, in the baby section — I was really tired).  Like an idiot, I left the store without buying any, went home and looked up the Medela website and realized I was pumping with a shield at least one, probably two sizes to small — which explained why my nipples were raw, swollen, and why there was a little layer of my SKIN on the inside of the breast shield after every pumping.  So I went back to Target and got the right size.  Within days my milk production (which was pretty good to begin with) tripled, we were in business.

12:25 – 12:30:  Until I got ahead of Hope’s consumption and/or convinced myself that I wasn’t depriving my child by feeding her “previously frozen” breastmilk, we had to wait until I was done to get the bottle ready.

me & my girls in "Command Central"

12:30 – 12:50 (or later):  Attempting to get Hope to drink from the bottle.  This was no easy task.  Hope lacked the stamina, the muscle strength, and the coordination to suck efficiently.  But we had been given a deadline by our pediatrician, if she wasn’t off the NG-tube in two months, we’d have to look at surgery for a G-tube.  Around 2 months, our PWS specialist gave us a bit of a reprieve, “She’ll take the bottle, cancel the surgical consult.”  So we continued to try.  Keeping her awake was a major challenge, we’d been instructed to tap the end of the bottle while she sucked, and while it may have helped to keep her awake, I’m not sure if it helped with coordination and rhythm.  So I’d sit, rock back and forth in the chair, and “tap-tap” the bottle in the rhythm of a heartbeat.  God it sucked.  There was some debate over how long to let her try, too long and she’d be burning more calories than she was ingesting, too short and she’d never get the hang of it.  These attempts ranged from 15-45 minutes over the course of the first few months.

12:50-1:10:  After we had given up on the bottle, whatever was left would have to be tube fed to Hope through her NG-tube.  That process included placing her back in her crib and checking the placement of the tube by pumping a little bit of air and listening through a stethoscope to make sure the air was going into her stomach and not her lungs.  We also had to attempt to “draw out” stomach contents – to make sure that she had digested the last feeding, before we pumped more in.  Once those things were done, we’d tape a big syringe to the crib, behind and above her, connect the syringe to the NG tube, then pour the remaining milk into the syringe and give it a little pump to get it going.  Then we’d wait.  When the syringe was empty, we’d disconnect, check the diaper again, take everything to the kitchen, wash and get ready for the next round.  Which would begin in 50 – 110 minutes, with me at the pump again.

We had to use this car seat insert wherever Hope was, otherwise her head would flop uncomfortably to the side. She also had to use an incline pillow, a wedge, to remain partially upright because she had a slight case of GERD and a slow digestive system. There is really no explanation for that outfit though.

That was life for two months solid.  At three months it started to spread a little, I could go three-four hours between pumpings, because I was way ahead of Hope in my production versus her consumption.  But Hope still needed to eat every 2-3 hours, and wouldn’t wake or cry to do so, it was entirely up to us to maintain the schedule.

Oh, and Hope absolutely refused to take a bottle from anyone but me.

But we managed to wean her off the tube between 2 and 3 months and avoided the G-tube surgery.   At 10 months Hope rejected the bottle completely in favor of solid foods (just like Grace had).  This would have been great except she couldn’t drink from a sippy cup either.  So we had to feed her jello to get the fluids into her, but there’s not a lot of calories in that, so the rest of her meal started with a tablespoon of butter.

I got to Exceptional Parents Unlimited (the Family Resource Center where Hope would receive early intervention services, including Physical Therapy, Occupational Therapy, and Speech Therapy) when Hope was around 3 months old.  I took a tour, and knew immediately that this was where we wanted to be.  It was extremely important to me that Grace would be able to come along and participate.  I didn’t want Grace to be “left behind”, and believe me, she hasn’t been.  Some day, she will be absolutely shocked to learn that we actually come to EPU for Hope, not for her.  I joined a support group, I basically just did what I was told, our early intervention teacher said, “Okay, you get her all week, she’s mine now.  Go to support group now” and I did.  I’m glad I did.  It saved me.  I still saves me and I know that long after Hope transitions out of EPU, I’ll still be coming.  I was beating myself up a lot when we first started, and I think one of my turning points – maybe the first was when our teacher looked at me one day and said, “What part do you honestly think you could have done better?”

Hope has had to have two sleep studies so far, to look for sleep apnea - because it is a condition that can be aggravated by HGH. The first one was at two months, and I was too much of a wreck to take pictures. This one was several months after she had been on the HGH, just a precautionary follow up. Apnea has fortunately not been an issue for us thus far.

I’m always kind of surprised to be asked to tell my story because frankly, I don’t think I have much of a story to tell – I am still very, very early in this journey.  We still struggle to get enough calories into Hope.  I can’t believe the crap I have to feed her now.  And it breaks my heart really, “Please don’t develop a taste for Kraft Macaroni & Cheese,” I think to myself.  The food seeking and obsessing over food may be starting, we can’t really tell if it is or if we’re just being paranoid because now, finally, Hope can say “I want milk.”  But it doesn’t matter, she’s still underweight.  We just can’t get her to eat enough.  It’s ironic.  It’s cruel.  And I admit that inside, I’m really angry about it.

Most of the time I just feel like a bundle of fears for the future, like I’m constantly waiting for the other shoe to drop.  But there are bright spots.  People talk about finding the blessing in the disability and I can say that, for me, I feel so lucky that I’ve gotten to have a baby for so long.  Though Hope walks and talks on her own now, and the growth hormone has done a good job helping her develop some muscle, she is still very “baby-like.”  She’s soft, kind of floppy, and melts right into you when you hold her.  She still needs and wants to be held.  The baby-babble phase came later but has stayed longer.  I won’t have any more kids, we’re done at two, and that was something that was really hard for me to give up – but we’ve got to be able to feed the ones we’ve got, so I feel lucky that Hope has been a baby so long, and will be a little longer too.

But I worry about the future.  I was teacher, I am a huge proponent of and believer in public education but I don’t know how I am ever going to be able to turn Hope over to any school.  Schools, like society, are saturated in food.  Will she steal another kids lunch?  Will I keep her fit and lean only to watch her weight spiral out of control the moment she goes to school?  We’ve been conditioned to believe that weight control is all about willpower.  For Hope it’s not.  The part of her brain that tells her “I’m full” is not active.  It’s not that it is “less active” or “slightly active” it is NOT activated.  The genetic data that she is missing is what turns that on.  She doesn’t have it.  On top of that, her body composition – the abnormal ratio of body fat to muscle mass is a condition that will persist throughout her lifespan, meaning she only needs half or less than half of the calories that a typical person needs to maintain her body weight.

I am still in a lot of denial about our future.  There are certain words and phrases that, when I hear them, I just shut down.  ‘Group home’ is one – I can’t even go there.  I exercise and diet now, because maybe then I’ll outlive her, and I won’t have to worry about what happens to her after I’m gone.

I remember when we first got the diagnosis, and how shattered I was.  People were always saying “God never gives us more than we can handle” – and I would always think to myself “I wish God wasn’t so fond of me.”  But there is something to that I suppose. I’m training for my first marathon now.  I run very slowly.  My goal is to finish before the start of next year’s marathon.  But I don’t think I would have ever even had the thought to try had it not been for what has happened.  You discover strength you didn’t know you had, love you didn’t know you were capable of, and a resilience and perspective you couldn’t have gotten any other way.  You learn that even the smallest victories are worth celebrating and that sometimes just getting up and out the door is a major triumph.  You learn quickly not to measure success by milestones, and simply to appreciate the joy in just making the journey.

The End.

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This entry was posted in Postcards from Holland, PWS, Scrapbook and tagged . Bookmark the permalink.

2 Responses to My Story, pt. 3 — the one with Mr. T

  1. Bretagne says:

    Thanks, as always, for being able to put this experience into such eloquent words.

  2. Jen says:

    Hey! i was wondering if I’d lost you forever after my last hiatus — hope things are well.

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