July 8, 2016

So, we just returned (almost a week ago) from week 16 of the trial at CHOC.  So much has happened since we started the trial.

Katha now has hearing aids and she hates them.  She hardly ever wears them but will wear them all day for school.

Because I went through what the first infusion was like, I will go through what the latest infusion was like.

When we arrive they immediately know who we are.  Katha will volunteer to get her bracelet on and will laugh through getting weighed, measured and her blood pressure.  She will cringe when she gets her temperature done but that's about it.

Last time we went they had her oral medication ready.  She took it a half and hour before she got into the room where the infusion happens.  By the time we got into the room, she was almost already asleep.

She barely pulled her arm away for the I.V. and when I went to leave she tried to wave as I was leaving.

15 minutes later they came and got me.  She was already awake when I came back in and stayed that way while she had to lay down for a half an hour.

As soon as her half an hour was done she got up and we caught a taxi back to the hotel where she quickly went to sleep for a couple of hours.

She woke up at 4 and had fruit and a couple of buns then went back to sleep for another couple hours.

At 6 she woke up to have a bit more fruit but when she started to eat she started to cry and say her back hurt.  But, as soon as she laid back down she was fine and went to sleep until morning.

Katha isn't doing as well as we had hoped on the medication, honestly.  She is still really hard to understand, she is very unstable on her feet and her memory is really bad.  She is in a wheelchair almost full time now and looses energy very quickly.  I don't honestly know if she's getting a placebo or if the sedation is taking such a toll on her body that it's masking the effects of the medication.

Then, about a month ago the drug company running the trial sent everyone a notice that the budget needs to be cut, which means our travel expenses need to be cut.  The new rules state that only the patient and a caregiver can go on the trips.  There will only be one night in a hotel allowed and food budget will be cut from $75/person/day prepaid to $40/person/day reimbursed.  However, they are willing to make exception on a patient to patient basis for extenuating circumstances.

So, we have already been approved to stay 2 nights in a hotel for short trips, we are easily managing to stay under the $40/person/day budget.  However, we are still fighting to have all 5 of us go on the trips.  When we signed up for the trial I made sure that they knew I was unwilling to be a part of the trial if I constantly had to leave the boys at home.  They are considering this but we have not heard yet whether or not they will be willing to make an exception for us.

If they are not (it's decided by a counsel) we will be withdrawing from the trial and hope that if this happens they will reconsider us for the trial if and when they start a site in Alberta.  I really hope it doesn't come down to that but with Katha's already limited time, I'm not willing to separate my kids that much.  Plus, I need another person along because with my back, one wrong turn and I'm back to using a walker to get around.

So, here's where we stand with symptoms:

Enlarged Liver and Spleen: Still the same

Dysarthria (slurred speech): Katha is very hard to understand.  There are days where I can't understand a lot of what she says.  On the other hand, on her good days, she's witty and uses large words and will talk to anyone and everyone.  The last trip to CHOC she had a bunch of assessments and was telling the doctor about her cousin Kaylee that was with us on the trip.  Then she proceeded to talk about cousin Emma and how I always say "oh, my God, Emma!"  I responded that I don't and she told me that I do and that I need to "be more responsible."  She then proceeded to tell the doctors how annoying her brother are and how I am always screaming at them.  She kept all of us in the room laughing the entire checkup.

Dysphagia (trouble swallowing):  Katha's swallowing continues to do really well.  She still rarely chokes since starting the new mediation.

Basal Ganglia (holding limbs at awkward positions): This is consistent for Katha now.  When she walks, she walks on her tip toes.

Dementia (memory loss):  Katha is often very confused.  It took a full day to convince her that she's now in Grade 5 and only accepted it when I promised her a new pencil box.  She has to be reminded every day that she doesn't have school and she's starting to lose what day of the week it is.  Which, is really sad because it's the one thing she ALWAYS knew.

Seizures:  Still none.

Gelastic Cataplexy (falling down in response to a large emotional outburst):  Katha now appears to lose consciousness when she laughs.  For a brief second she looks like she falls asleep but her body bounces because she continues to laugh even though she's not smiling.  We went to see Finding Dory yesterday and she was laughing so hard she lost consciousness.  I think this is hilarious to watch but others seem to be quite upset when it happens.  Katha (like me) doesn't seem to be bothered by it in the slightest.

Sleep related disorders:  Katha spends more time asleep than awake.  When we go anywhere she wants nothing more than to go home back to her own bed.  The nice thing about it being summer is that there is no problem with her not waking up until 11.

Vertical Supranuclear Gaze Palsy (Trouble moving the eyes up and down):  Katha is still looking up really well.  Better than I have seen her do in years.

Hearing Loss: Like I said, she hates the new hearing aids.  She has been to the doctor several times to make sure they are not too loud and the doctor reassures me that they are actually a little quiet for her.  She spends most of her time watching tv with little to no volume and doesn't seem to have a problem hearing what's going on in the house.  We don't even usually have to call her for supper because she is already out at the table by the time we would call her.