Not much has changed….

I just read my last post, because I couldn’t remember how Tiara was doing at that time or what I wrote well over 3 months ago, but after reading it, I realize not much has changed.   I continue to receive emails, messages, and fb posts from readers asking for an update on Tiara. I know it is kinda rude that I invited you all into our daily life and then ditched you, but please don’t take it personal.  Taking care of her has become more than a full time job and I can’t continue to write about our daily life because it has become too depressing for me.  So here is an update so at least you can stop wondering how she is doing, for a while.

Tiara continues to be very tired, lethargic and sleeps off and on most of the day.  She no longer enjoys coloring, cutting magazines or too much music.  She likes to watch family videos most of the day.  Our days are pretty much the same.  She wakes up around 7:30am.  We feed her a turkey sandwich, give her all her seizure medications, do her 2 breathing treatments and she is back asleep by 9am.  I lay with her on the couch reading or checking emails because she likes me to sit next to her.  She wakes up at about 10:30am and we drive to McDonald’s.  She starts to fall asleep again as soon as we get in the car but wakes up when she smells her 10 piece chicken nuggets and diet coke.  We drive around Mesa Verde and then she wants to go home.  We go home, I help her inside and she flops back into her spot on the couch.  I put on her oxygen, which she now wears most of the entire day while at home and she goes back to sleep.  I clean up a little and do some laundry but mostly I just sit with her.  We watch Hannah Montana, Camp Rock or Barney as she goes in an out of sleep. 

Her caretaker is supposed to arrive at 12:30 but we have been without consistent help for the past 2 weeks because her old caretaker went on disability for stress.  I guess watching Tiara sleep on the couch all afternoon was too stressful for her?  If a caretaker shows up, I go to the market, do my errands, pick up Trinity from school, fix dinner and the caretaker leaves.  She has refused to take a bath or a shower lately, so that has become very challenging.  It usually takes 3 of us, singing, laughing and making a game of it to get her into the shower.

So why is she declining physically, regressing developmentally and just getting worse?  This is a question everyone on her medical team has been working hard to figure out.  We met with a very experienced psychiatrist and he thinks she has encephalopathy.  Which is a general term describing that her brain is no longer functioning like it was before because of the constant seizures and high doses of seizure medications.  He described it as if she was in a constant state of delirium, which is why she is still constantly still pulling my hair, scratching and pinching.  She is very anxious and doesn’t really realize what she is doing and feels out of control.  He moved her medication times around and added another medication.  I am happy to report that since he did this I have noted a slight improvement in her activity level the past few days.

Three days after we saw the psychiatrist, we saw her neurologist.  She was extremely concerned about Tiara’s current condition.  She runs the TSC clinic at UCLA so not only does she look at Tiara’s neurological health but she reviews all organs affected by TSC.  Which is why she was very concerned when she saw that one of Tiara’s renal tumors was larger than 4cm.  If a renal tumor is larger than 4cm it needs to be shrunk before it bleeds or be surgically embolized.  There is a medication called Afinitor, which can shrink the tumors in her kidneys, help with her seizures, possibly reverse her developmental decline and potentially help with behaviors.  A magic drug, possibly?  We have talked about putting Tiara on this drug for over 2 years, so why haven’t we?  There are some very serious side effects which could be potentially life threatening for Tiara. 

This drug can cause some serious respiratory issues which Tiara might not be able to recover from if she were to have this side effect.  There is only a 10% chance BUT her doctor knows that Tiara is always the patient who gets the rare side effects.  So our plan is to get a high resolution CT of her lungs as soon as possible, have her consult with an adult Pulmonologist at UCLA and see what he thinks about the current state of her lungs.  Her Pulmonologist at CHOC tells me to keep her on oxygen if she needs it during the day but he doesn’t really give me a diagnosis as to why she is always out of breath and in need of oxygen.  We need to get an answer so we can determine how to proceed.

We also decided to have all the basics checked out, just to make sure we weren’t missing an onset of diabetes, high blood pressure, a thyroid issue or renal problems.  Shockingly, despite her morbid obesity, all her numbers were good.  I guess it is all the fruits and veggies I feed her.  Her daily McDonald’s run is the only bad food she ever eats.  The rest of the day it’s salads, veggies and fruit.  She will also being seeing her cardiologist on Tuesday.

As for me….  Before our regular caretaker left and Tiara started to get worse I had a few weeks of freedom.  I painted a picnic table turquoise for one patio, added a bunch of potted plants, refinished our original outdoor table along with the couch and chair, got new cushions for the outdoor furniture, picked up a few amazing finds at the thrift store and our house feels like the Goff home.  As Lou said “Now it looks like your house!”  So that was really fun. 

 

 

I also cut off all my hair a few weeks back just because I couldn’t stand to have it pulled much more.  I don’t like having it this short but it seems to keep me safer even though my neck has more scratches than usual. I really just can’t win in the injury department. 

 

I have also arranged to have a family picture taken by one of my best friends from college next weekend.  She is a professional photographer and I haven’t bothered to have her take our photo in 5/6 years?  Everyone in the family has moved around their schedules to make this happen, which really makes me happy. 

Also, all our family members have really been making an effort to stop by the house and visit Tiara more often, because it really makes her happy.  So with this sad and unpredictable time in our life has come good.  Family pulling closer together to support and love one another.  Oh and I forgot we celebrated Tiara’s 16th birthday and she had the best time ever.  We get to watch the videos from her party at least 20 times each day.  I have to go, she has been yelling at me to come sit on the couch with her.

 

Miss you all!

 

xoxo tiffani