Why does Tatyanna have a feeding tube?

As Batten Disease progresses it affects the body’s ability to chew, swallow and digest food.  Children often often problems aspirating liquids into their lungs or choking.  Although Tatyanna never had problems with either, she simply quit eating.  We wonder if this was out of a sense of self-preservation.

How old was Tatyanna when she got her g-tube?

Just before her 8th birthday.

Were there ever any complications?

Nothing major.  In the beginning she did have some granular tissue build up around the site but it has been fairly smooth sailing for the last two years.  Although the rate at which she tolerates feeds has slowed, we’ve been fairly lucky  in that Tatyanna has not had issues with things like vomiting or reflux.

How long was the adjustment period following the surgery?

Truthfully, the emotional preparation before the surgery was the hardest part.  The difference in Tatyanna after we began tube feeds was huge and left us wishing that we had the operation done sooner.

Was not doing the surgery ever an option?

In Canada, tube feeds are an extra and a parent could “opt out”.  But for us this was never an option.   I have no doubts that we would have lost our daughter within months had we not gone through with the surgery.  Despite the terminal nature of Batten Disease, it was obvious that she still had plenty of life left to live, having a g-tube put in only served to increase the quality of her life.  We’ve had three fairly awesome years filled with fun times and plenty of laughter  during which  she has been completely dependent on a gtube for nutrition.  It was the right decision for us.

What do you do when you go out or travel?

We haven’t let tube feeding stop us from going places or doing things. We just pack a bag of supplies, some hand sanitizer and off we go.

What does Tatyanna eat?

At the moment Tatyanna uses a formula called Compleat.  Out of all the formulas we have tried (Nutren Jr, Jevity) we found that she has done the best on Compleat.  As a mom I like it because it contains real ingredients like chicken, peas, beans and fruit…for some reason that just makes me feel better.

Does she eat anything by mouth at all?

No, nothing….really.

To this day, she does still enjoy the occasional lollipop. 🙂

Were there ever any issues with Tatyanna not being able to eat while everyone else was?

Because we didn’t have issues with aspiration we always had the option of offering her food when we were eating.  Despite our efforts, she would refuse every time.

Where do you get your supplies?

In our province all supplies and formula are covered by Manitoba Home Nutrition as well as Home Care.  We have a standing order that gets delivered monthly for formula and other supplies are ordered as needed.

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Please feel free to ask if you have any more questions!

Of all the transitions that we have made on our journey as parents to a child with Batten Disease, g-tube surgery was the hardest to prepare for emotionally.  Tatyanna was my little foodie.  She could spend hours watching the food network channel and was eager to try new recipes.  She would turn down McDonald’s for sushi (unless the offered toys happened to be either Star Wars or My Little Pony) and happily wolfed down bowls of curry and plates piled high with salad.  The thought of her losing something she so clearly enjoyed was hard to take.

As the disease began to take its toll, her swallowing remained unaffected.  She had no problems with choking or aspirating but rather required increasing amount of help in getting the food from her plate into her mouth.  We were hopeful and perhaps a touch naive…perhaps she would be able to hold on to this ability.   And then, without warning, she simply quit eating.  Just like that.  Meals that were once a source of comfort became increasingly stressful as we struggled to get enough calories into the few mouthfuls that we could convince Tatyanna to eat.  We began to add calorie rich whipping cream to her morning oatmeal and I spent hours looking up recipes that would maximize calories and nutritional content.  But, it didn’t take long before it became very  clear  that our efforts were not enough and her weight began to drop very quickly.

By the time we were able to get her in for surgery she was so very thin.    Looking back at old photos is painful; she looks like a little old man; her cheeks bones were hollow, her eyes more sunken.  Although she was still smiling, her fatigue was evident.  In fact, during the week leading up to surgery we thought that she had finally lost her eyesight as she was no longer interested in movies or books.  In hindsight, she probably just didn’t have the energy.

The surgery itself went perfectly and aside from the expected post-op pain, her recovery was smooth as well.  Within a few days she was taking 3 bolus feeds per day and we were released from the hospital with a van full of supplies and equipment to begin our new normal.

When you are dealing with a degenerative condition, the “new normal” often refers to regression….but not this time.  Deciding to go ahead with the surgery was the best decision we ever made.   In the weeks following  Tatyanna began to fill out, her eyes were brighter and she began to do and say things that we hadn’t seen in months.  It was like getting 6 months back.   Meals were suddenly stress free -I actually got to eat with the family again!  Previously I would spend meal time feeding Tatyanna and would eat after….after sometimes being and hour or more later.  If we happened to be in a restaurant I would often order mine boxed to go at the end.   Although we thought that she would be able to eat by mouth for pleasure, she never did really eat again.   She simply sat at the table and enjoyed family meal time….without the food.  The daily medication routine was also suddenly easier as all her medication could go directly into her stomach via the tube.  No more trying to mask yucky tastes, no more worrying about whether we had lost a pill during her protests or wondering how we were going to wake her if her body needed extra rest.

It was awesome.

Perhaps someday there will be words here…but for the moment,looking back and attempting to write everything that happened feels entirely surreal. Did we really go through all that? Sitting here, with Tatyanna beside me, smiling and happy it all feels so very far away. Any yet, there is a part of me that knows it was only just the beginning of what’s to come.

all photos taken with an iphone5 during our stay at the children’s hosptial January 6 -January 30.

Some days it’s hard to reconcile “what was” with “what is”. Just over five years ago we stood in one of the tiny examining rooms of the children’s clinic in the city. Tatyanna went around the room and, without prompting, shook hands with each and every medical professional that was standing there, gave them a smile and a chirpy “nice to meet you.” I knew when they would not meet her eyes that what we were facing was bad.

They handed us a lab requisition and said they would see us when the results were in.   Any attempts at receiving any sort of clarity were met with a dismissive “we’re just going on a hunch,  we’ll let you know.”   But when I looked down at the folded paper in my hands, the words “batten disease enzyme” were written in bold black.

The blood draw was performed with out a single protest from my little girl and she hopped off the chair with a “thank you” and left the lab tech standing there, an incredulous look on her face and the vial of blood that would seal my daughter’s fate in her hands.

Five years ago, my little girl had hopes and dreams. She loved dancing, music, horses and the colour pink. We couldn’t mention the words beach or swimming or she would ask nonstop at 5 or 10 minute intervals when exactly we would be going on said outing. The chances of her wearing an assortment of mismatched bathing suits for the duration of the wait was extremely high.

For five years she has fought the beast that is batten…it is a fight she will not win. We recently came home from a 31/2 week stay in hospital. While we are grateful to be surrounded by family, home does not mean fixed.  This time home means that nothing more could be done.

We would very much appreciate your prayers right now.

Facebook status from today:

The moment that you wonder if you will ever find your footing under the new normal is also the moment that you spill a bowl of tortilla soup over your beside table….the table on which your phone was placed and contains two drawers filled with junk that were left cracked open…to remind you that you simply don’t have a choice. On the upside? The life proof case that I just put on my phone works! Life proof=Janelle proof. I can appreciate that.

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I may need a day or two before I am able to  resume more regular posting.  I’m totally cheating by posting a photo that I took in the days before this last hospital stay.  Ronweasely has resumed his position by Tatyanna’s side and has been caught trying to work the new normal to his advantage.  Perpetual myoclonus provides a continual, albeit rough, source of chin scratches if you lay your head down just right.  Cats, they have no shame.  His sense of opportunism might be irritating if he wasn’t so cute and fluffy.  Also, Tatyanna didn’t seem to mind.

It’s been a long three and a half weeks (made even longer by the hospital’s lack of internet access but we are finally home.  Once I have a few moments to collect my thoughts I will update….with more iphone photos. 🙂 For now, I will take advantage of the fact that today is the first and leave you with this blog entry that i wrote in June 15, 2007, very aptly titled, “like trying to put a billy goat in a shopping bag”

I should have know that it wasn’t going to work. After a week of increasing seizure activity, we finally got a call from the EEG department. The lady on the phone suggested that they like to schedule the pre-school appointments for the afternoon because that is when they nap. Umm….right. I tried to explain to her that my daughter does not sleep and runs around like a caffeinated weasel until her battery dies around midnight. The receptionist did not believe me, brushed off my concerns with a “I’m sure it will be fine, we’ll see what we can do.” and scheduled the appointment for 2:00. We showed up at the hospital at 1:45 as suggested and proceeded to wait for 45 minutes. As the seconds ticked by you could see Tatyanna start to unravel. The novelty of sitting on each and every chair in the waiting area only lasts for so long. By the time we finally got called in I was peeling her off the walls and dragging her out of the ornamental trees. It wasn’t pretty. Once in the appropriate room, I was told that she would have to lay still for 25 minutes without moving. I think I might have laughed at this point. He, being the astute, EEG tech quickly realized that this really wasn’t going to work. It might have been the fact that Yanna was trying to jump off the bed yelling “I NO SLEEP!!!!!” but I could be wrong. Needless to say, we had to come up with plan B. “We deal with these types of kids lots” he said  “and it never works with “them”. ” He then apologized that they had even tried to set up the appointment for 2:00 at all. The plan B is that we reschedule for tuesday at 9 am, keep her up as late as possible and then wake her up at 4am. Hopefully by 9:00 she will be tired and more receptive to the mild sedative they are allowed to give. Again, I am skeptical as nothing ‘mild” seems to knock her out.  I speak from experience.  The energizer bunny has nothing on my girl. It should be interesting to see how this works.

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Also thank you so much for the encouraging emails and comments.  Your thoughts and prayers really do make a difference to my family.

Barely one week later and we are back on the PICU in Winnipeg due to another status seizure. Same pediatrician in Brandon, same life flight crew and same nurse on the unit.   tatyanna has since been moved to a regular ward after a couple of nights in the PICU and it looks though we will be here for several days while they try to get her seizures under control and determine if there is an underlying cause.

I will try to update more when I have access to a computer and  not a  phone that is insistent on making some rather bizarre autocorrections.  Yes iPhone I really did mean say the word look   The first time you switched it for lollikens it was funny.  But every time?  Notsomuch.

We’ve only been home for a few hours and when I attempt to put words with these photos, they just don’t come.
Perhaps tomorrow after I have had a chance to process the events of the past 5 days I will be able to come up with something slightly more detailed than: “big seizure. but all is good now.” I am numb physically and mentally and think that I just need a few minutes to catch my breath.

My girl. She is definitely a fighter.

(At the time these photos were taken, Tatyanna was stable and the seizure had finally stopped. Due to the medication given, she was unable to breath on her own and had to be intubated. Our smaller hospital does not have a PICU and so transport via life flight to a larger centre for monitoring was necessary.  Once she was breathing on her own again we returned via ambulance back to the hospital closer to home.  Although the picture doesn’t show it, she smiled for the entire two hour ride.  I think I did too. )

All images taken with the iphone 5.