Kirstyn T.

After ten years of infertility issues, Kirstyn was on her way!  She turned 4 on Saturday Aug 17 & mom said her birthday was bittersweet as it is every year.  Like so many PA families, her parents were told in the first week that their daughter would likely not live through her initial crisis.  When Kirstyn was born she had ABO incompatibility which is basically severe jaundice, so she was transported to Shands NICU to get her liver to kick in.  The first 7 days there, Kirstyn became very lethargic & stopped eating.  One of the nurses even scolded mom saying she couldn't take her child home until she proved she could feed her.  The next day Kirstyn crashed, falling into a coma.  Ammonia was over 700 & she spent 9 weeks in that NICU, during which time she had a core team of nurses assigned to her because of her rare condition & bleak prognosis.

After that, she went the entire first year without further need for hospitalization, but has had 4 since then.  She's had three blood transfusions of packed red cells.  She was diagnosed with mild cardiomyopathy at 18 months, so she has an EKG & EEG every 6 months.  Stomach bugs are the hardest thing, causing 2 hospitalizations due to excessive vomiting, ketones & high ammonia.  December 2005 she spent 2 days in the hospital for a terrible ear infection that was first misdiagnosed as seizures because she was vomiting then zoning out.  March 2006 she was in overnight for a respiratory infection & high ammonia.  Her mom said “Kirstyn gets sick really quickly!  Once she reaches moderate ketones, she will need to go straight to the hospital or her body will begin rapid decompensation.”

Kirstyn had OT & PT her first 3 years through an Early Intervention program.  At the age of 3 she broke her collar bone in school because she didn't reach to catch herself during a fall due to her low muscle tone.  She's clumsy when she walks, but has always been very active.  Her mom said “If she gets red faced, we have to give her extra calories or she will spill ketones within an hour of hard play.  We learned that the hard way during her 3rd birthday party at the park.”

Last year at age 3, test results showed Kirstyn had reading & comprehension skills of a 6 year old, & counting & number abilities of a 5 year old.  She's going into her 2nd year of pre-k, geared toward medically impaired children with no cognitive delays.  She's quite bold & independent & it's very difficult to keep her on a task she doesn't want to do.  She loves to sing, draw, & paint.  She has very low muscle tone in her hands, so she really resists anything that involves use of hands, like using scissors.  Due to her low muscle tone, Kirstyn still receives PT through school. She has a lot of behavioral issues & doesn't like to listen.  Her school has recently considered testing her for ADHD, even though they're also considering testing her for the gifted program.

She's just started her first activity, tap dancing & loves it!  Recently Kirstyn learned to ride a bicycle with training wheels, which she got for her birthday.  Playing on the computer is one of her favorite past times.  She doesn't like her little brother Cason (20 months), & often asks her mom to “Please send him back to the Little Brother Store.”  She picks out all of her own clothes & doesn't care what people think : )  And, she loves to do housework.

Kirstyn eats 100% by mouth except when she's sick.  With a packed lunch from her mom, she eats so much better to at school with friends around.  Her mom said “We always know when she's getting sick because her first symptoms are always spilling ketones and she stops eating.  For this reason she has a g-tube“.   Like many young children, she has a weird diet consisting largely of french fries, pancakes, hash browns & loves to eat at McDonalds & Bob Evans!  Her mom said she likes ketchup on mini frozen waffles & calls them mini pizzas & would eat ketchup on everything (anything mom lets her put it on!)  She doesn't care much for sweets, only an occasional M&M.  At parties she'll tend to lick a bit of icing to feel like part of the group. 

As far as potential complications from PA, she's doing amazingly well.  As of this year her doctors said she only needs labs 4 times per year!  Her restricted amino acids typically run around the low end of normal, & she’s given a Valine supplement because it's always run below normal.  Her Glycine is typically around 800. 

Marsha, Kirstyn’s mom, expressed her sincere appreciation for the support she’s received from the other PA families through PAF, saying “We’ve been blessed to be part of this support community, which I’ve turned to for help on many occasions.”  She said they’ve even met a few other PA families because they live so close to Walt Disney World, so look her up if you’re planning a trip!

On behalf of Marsha, Ronnie, Kirstyn, & Cason Tripp

marmarsh38@yahoo.com

Florida

Written by J Mouat; jenmouat@gmail.com