Sisterly Love

It's been a nice low key week around here. Back into a somewhat normal routine (whatever normal is anyways!).

Our Infant Team therapists from the hospital came and did a home visit this week. All three of them came, the Occupational Therapist, the Physiotherapist and the Speech Language Pathologist. They worked with Taylor's own toys and sensory items to do some fine motor stuff. Jeanette adjusted Taylor's standing frame, she has grown so much since we got it that it needed several adjustments. Then we had a little feeding session so they could watch Taylor have some thickened fluid by bottle. She drank a wee bit and they were pleased with how she managed it. Good good!

Marissa's morning cuddle time in Taylor's crib has resumed since we've been home, it is so very sweet to listen on the baby monitor as Marissa climbs into the crib, says good morning to Taylor, kisses her and talks to her. Warms my heart to wake up to that. This morning she got dressed first, did her own hair, and even brought her own pillow into the crib with her. She covered Taylor up with a blanket and put some stuffed animals around her head. So so cute.

Hope

Well...today was the big day. We managed to make it to the hospital on time despite the crappy roads and morning rush hour traffic. Barely!

FEES...Fibreoptic Endoscopic Evaluation of Swallowing

Poor Taylor, with the NG tube already in one nostril, the doctor shoved another tube down her other nostril, a tube with a camera on the tip. We then watched Taylor drink a bit of bottle on the screen. It was very interesting to see what actually happens back there when you are breathing vs. swallowing. I could see the "pooling" and "bridging of the airway" that they have been talking to me about where some of the milk pools and hovers over the airway instead of going down the esophagus when Taylor swallows. I could finally see what was actually happening when Taylor makes the gurgling sounds as she feeds. Despite the gurgling and pooling of the fluids around the airway, the doctor did not see an aspiration occur.

Great news. However just because it didn't happen during the 7 minutes of this feeding study doesn't mean it couldn't happen. So the recommendation by the OT is still to thicken the fluids to give Taylor better control, and that we can go ahead and start introducing oral feeds back into the mix.

What a relief! If we can get her eating and drinking again...safely...then at some point we'll be able to get RID of the NG tube. This is promising.

Next on the schedule today was the Neurologist. Dr. Bello was pleased to hear that the feeding study was positive. We're done with the Vigabatrin wean. He wants to continue the Phenobarbitol. We'll stay on the Phenobarbitol. Dr. Bello had dangled a little carrot in front of me a few weeks ago one afternoon while we were in the hospital. There's a drug called Levadopa, primarily used on Parkinson's patients, that he has used in certain cases like Taylor's. He wants to trial Taylor on a low dose. Because of her severe hypotonia (low tone), because of her severe intractable seizures (that do not respond to treatment), and because of her choreic movements (involuntary, abnormal, spastic movements that are not purposeful). He has treated a small number of similar cases over the years with l-dopa and has seen significant improvement in development and significant improvement in seizure control. Seems like a miracle drug, I'm in. Now that Taylor is stable, he has given the go ahead for us to start. The neurology pharmacist is going to put together a schedule for increasing the dose slowly over four weeks. He also needs to work with the dietician on the mixing instructions of this drug, because it has a carbohydrate content, and that will affect the ketogenic diet. It's never simple!

With today's happenings, I have hope.

Back in the Groove

Well, it's been a week and a half since we came home, boy time flies! I was settling into a nasty cold when we were discharged, so I was pretty much out of commission last week, finally feeling better now thank goodness.

Before we left the hospital the OT dropped in to see how things were going with Taylor. We hadn't been working on the thickened fluids by mouth because she was so congested from her cold that she couldn't breathe with a bottle in her mouth. The OT thought that it was better to play it safe for the next couple of weeks before the FEES study to continue with 100% tube feeds. So that's what we've been doing since we got home, and tomorrow is the big day, FEES study (Fiberoptic Endoscopic Evaluation of Swallowing) at 8:30am. Of course the city is hit with snow and bad roads right now, so it's going to be an adventure to get there on time in the middle of the morning rush hour. Sigh.

Since we got home Marissa has been quite interested in the whole tube feeding process. She told me that she wants to be my special helper, what a sweetheart. She likes to press the buttons on the pump. She knows how to turn it off when the feed is done. And she knows how to restart it when it beeps with a flow error. She gets quite annoyed when I beat her to it, she likes to do it herself. She's also very interested in flushing the tube after a feed finishes. We need to flush the line with water from a syringe after each feed, so Marissa is right in there wanting to help. She puts the syringe in the tube and flushes the water through, and she's very proud of herself every time she does it. I first let her do it last week one day. We were sitting at the dinner table while Taylor's feed was pumping through, and Marissa asked, "Mommy, is it time for me to milk Taylor?" Trying not to laugh I reminded her that it's called flushing Taylor's tube. Funny girl.

Going to call it a night now, early start in the morning to make it to Children's on time. Will update once we have news from the FEES study. Hopefully good news.

Snug In Our Own Beds

To say we're happy to be home is an understatement! What a blessing to finally be home with Supergirl, 25 sleeps at the hospital is a record indeed, a record we hope to never break!

I think the happiest person in our house though is Marissa. She's been missing her little sister like crazy (and Mommy too). She's got loads of hugs and squeezes for the both of us and it sure feels good to be back under the same roof as a family.

Oh love!

Do I Hear Discharge?!!!

Yes I do, that's what the doc said when she came in this morning! Believe it or not I actually asked to stay one more night. Taylor's had a couple good days throughout this admission and something new would creep up. I would just feel more comfortable having one more good day under our belts before going home. The doc was okay with that so tonight is our last sleepover at Hotel Hospital! Taylor had a great day today. No issues. Making her usual noises. Moving around. Kicking. All good stuff. I feel much better tonight about leaving in the morning. I think Supergirl is ready to blast outta here and get back to some normalcy.

Two Sickies :-(

So, we didn't have the FEES study yesterday. The charge nurse called the ENT clinic first thing and asked when we could re-schedule as an outpatient. We were able to get an appointment on the 18th, so we decided to go with that. It's not too long to wait, Taylor should be all cleared up by then of her terrible cold, and then we can be more certain that whatever the result is, that it wasn't hindered by her congestion.

Taylor's eyes are looking much better today so the antibiotic drops are obviously helping. They were still red all the way around yesterday, but had stopped gooping up. Today the red circles have greatly improved. She hasn't had any fevers and hasn't needed any oxygen. All good. Her stuffiness seems better, but she still has an awful cough.

Unfortunately I'm sick now too, that was just a matter of time. Spent most of today in bed while the nurse took care of all Taylor's feedings. So grateful for that. Francis and Marissa came for a little while and brought me some Tylenol cold medicine, that always helps. With Francis playing Mom at home with Marissa, I never know what kind of getup she's going to show up in! Today she arrived in sparkly gold shoes, a summer dress, and a Halloween hairband. And I'm pretty sure her hair hadn't been combed. Too funny.

I think they may say we can go home tomorrow, but we'll see. I want to go home so badly, but I also want to feel comfortable with it. Taylor's had a couple decent days through this hospitalization, then something new creeps up. I'd hate to get home and have something else happen.

Pretty uneventful day really, other than a blowout of mass proportions this evening. It went all the way up her back and into her hair. OMG! The nurse put her right into the tub, while I changed her bedding. I actually threw out the sleeper!!!

Marissa brought a stuffed turtle from home for me (so sweet), I put it in Taylor's crib and she spent quite some time checking it out.

Two Steps Back...

It seems like we keep going one step forward, then two steps back.

Just after I finished the blog last night Taylor started to have to work really hard to breathe. She was really congested, her respirations were really fast and had indrawing in her chest. She also had another fever. The nurse paged a doctor. She was given back to back ventolin through a nebulizer, and they put the oxygen prongs in her nose. Given the recurring fevers, and the increased work of breathing, the doctors were concerned about aspiration pneumonia. They wanted to put her on antibiotics right away as a precaution. Because of the ketogenic diet there are specific antibiotics that have no carbohydrate content. Because it was after 1am at this point they didn't have access to it, and the emergency room didn't have it, so we were to wait until the morning. Poor Taylor was beyond tired at this point, she still hadn't fallen asleep and it was approaching 2am. They just kept re-examining her and asking the nurses to suction her. After she was breathing better I was getting more and more frustrated that the doctor kept coming back and prodding at her. I told her if she wanted Taylor to have a good night that she needed to be left alone or her seizures were going to get out of hand from sleep deprivation.

God bless Nora our nurse, she left Taylor alone after that, she didn't do the suctioning through the night that the doctor wanted. That would surely have put poor Taylor over the edge. And she slept peacefully once they left her alone.

This morning she got a dose of the antibiotics. And poor Taylor looked like she had been in a fight and lost. Poor little muffin has really goopy eyes and a crusty nose. And she developed red circles all around her eyes. Poor thing looks terrible and has had this tired sad cry off and on today. Swabs were ordered of her eyes.

When the docs came in for rounds they had decided to put the antibiotics on hold until they could confirm the need for it. There was also a mixup with the ENT clinic regarding the FEES study, it's scheduled for tomorrow morning at 8:30am, not today. The pediatrician is a bit concerned about whether or not it's a good idea to proceed with the study right now when Taylor is so sick. Would it be a true picture of the way Taylor handles her feeds? She said she'd give it some thought through the day and we'd talk more about it later.

This afternoon Taylor was sent down for another chest x-ray. I'm kicking myself for not bringing the camera down with me, they did it in the most interesting contraption called a Piggostat. I found some pictures online of what it looked like...

I talked with Dr. Vomeiro later in the day, the chest x-ray was fine, no indication of pneumonia. We decided that it's probably best not to do the FEES study tomorrow, but we'll wait and see in the morning how Taylor is in case she has some remarkable turnaround. The swabs taken from Taylor's eyes this morning are growing a bacteria, so she does have an eye infection in both eyes. As well as an ear infection in both ears. It's quite common for a secondary infection to manifest after one is already present. So antibiotics have been started, and drops for her eyes. Poor sweetheart looks like a train wreck.

Dr. Vomeiro is going to call the ENT clinic first thing in the morning to ask when we could re-book the FEES study as an outpatient. If it's going to be too far down the road, then we'll go ahead and do it tomorrow. But if they can get us in in a reasonable time frame then we'll re-schedule it. I have been quite anxious to get these feeding answers, but the test is pretty invasive and uncomfortable under the best of circumstances, so to subject Taylor to that right now seems unfair.

If we do not do the FEES study, then they will keep Taylor here until she turns towards recovery, maintains her oxygen sats and stops spiking fevers. Then they will discharge us and we will maintain the NG feeds at home until our re-scheduled FEES study appointment.

So we shall see what transpires in the morning.

Brighter Day

I'll start with the good news...yes there is finally GOOD news :-)

Got woken this morning at 7:30am (no that's not the good part) and told a porter was on their way up to take us to EEG. That's the 4th EEG Taylor's had in the past 20 days! Naureen got her hooked up in record time and started the recording. I sat down beside her to watch the screen. She wasn't too concerned about Taylor doing the sleep part today since she's slept the 3 previous times, and her seizure activity is the same during wake and sleep. Once we were done and Taylor was cleaned up, I carried her into the hallway to wait for the porter and saw Dr. Bello sitting in the next room looking at Taylor's EEG. He saw me and said right away that he's very pleased, there's a 70% improvement from last weeks chaotic activity!!!!! Taking the Sabril down has definitely been the right call. Her dose was reduced to half on Friday night, and down to a quarter as of tonight for about 5 more days, then done. Hopefully by then Taylor will be back to her baseline. Maybe even better than her baseline...but let's not get ahead of ourselves!! A 70% improvement from last week is huge and definitely worth celebrating!

Poor pickle is really suffering with her cold though. Very congested. Very snotty. Ick. She's been suctioned a few times to get stuff out and I'm sure at this point she's beyond DONE with things being shoved up her nose! But she's such a trooper, has been relatively pleasant all day.

This afternoon she was settling into a nap, so I decided to do the same myself. Managed to sleep for a couple of hours...both of us! I think we both needed to crash, it was another rough night last night. She was up at 2:30am very congested, heart racing, crying, and making the strangest movements that actually had me wondering if it was a new kind of seizure. She had both legs pulled up and very tense, her cry was different, and she was almost vibrating. This went on for what seemed like forever, but maybe about 10 minutes. When the nurse put the probe on Taylor's finger, her oxygen sats were 81, they should be in the mid 90's. She turned the oxygen mask on and we held it over Taylor's nose and mouth. She slowly perked back up into the 90's and when we took it away she maintained it. Anyways, after Taylor finally settled, she started letting out some very loud gas. Funny. So we chalked it up to that, and I felt better that the movements weren't seizures. She was fine the rest of the night and all day today.

With all her congestion and working harder at breathing, we were taken down for a chest x-ray earlier tonight just to check out her lungs and make sure there were no signs of pneumonia. Thankfully there weren't. Keep up the good work Supergirl or we're never gonna get out of here!

Gave Taylor a nice bath tonight to clean up her lovely EEG gunked up hair. I thought baths were supposed to be calming? She's wired tonight. As I type, it's now 11:15pm and she's just movin' and shakin' in her crib.

Anxious for the FEES study tomorrow. Don't know the time yet, and hopefully her being sick isn't going to prevent it from happening. Fingers crossed.

Day 19

Taylor woke up with a cold today. She's full of boogers and has a nasty sounding cough. I think her dear sister passed that on. Marissa it's nice to share, but some things are better kept to yourself! Now we're on isolation.

We haven't been too successful with the thickened oral feeds today, as soon as she starts sucking on a bottle, she can't breathe because she's so stuffed up. So I haven't pushed that since lunchtime, just giving her a break and using the tube today.

I had the rest of the NG tube training today. Just call me Nurse Natasha, I can officially perform all steps in NG tube feeding...inserting and removing the tube, checking placement, flushing, administering meds, setting up the feeding bag, running the pump, cleaning and storing the pump bags, and doing syringe feeds when necessary away from home. The home nutrition nurse brought me the pump and pole today that we'll be taking home as long as Taylor's on NG feeds, pump is worth $1500 and pole on wheels is worth $800. She also brought two weeks worth of supplies...NG tubes, lubricating jelly, pump bags, extension sets, 10cc slip tip syringes, 30cc slip tip syringes, 60cc slip tip syringes, transpore tape, pink tape, duoderm, and tegaderm. We will then order a month's worth of supplies at a time and pick them up from the warehouse down south near us. This is all covered, we don't have to pay anything. We are so fortunate to live where we do.

Waited a long time today before seeing the doctor. She didn't come by until nearly 4 o'clock, and the OT came with her. Due to the change in the way Taylor has been managing her pureed meals, they'd like me to not give them until we have the FEES study done. Unfortunately it didn't happen today, we have been scheduled for it on Thursday. So her 3 meals will be replaced with 49 grams of 36% whipping cream and 46 grams of unsweetened apple juice. Through the tube. So until the study happens, Taylor will be taking everything through the tube apart from a small amount of thickened fluids 3 times a day by bottle.

Mom was here for a short time this morning and brought with her something special for Taylor. She got a plain white diaper shirt, had it dyed with the Supergirl logo in pink, and finished it off with a few pink Swarovski crystals. She needs her bling! It is so cute, thanks Mom!!! I had Taylor in it today, but Francis got chocolate all over it when he was here before I got a picture of her in it! So I took a picture of the shirt by itself and used Photoshop to remove the chocolate mark...

Daddy's Experience

The days are starting to blur together. It's been 18 days in hospital. That's too many. It's taking its toll on us all, me especially today. Very grumpy, very frustrated, very done.

Poor Francis had a pretty traumatizing experience with the tube insertion today. I don't think he was too worried about doing this procedure...until it took him about 5 times before it was in. Having to subject your screaming daughter to that miserable experience once is awful, let alone 5 times. But he kept at it, and finally got it. Way to go honey, you did it. I think it took Francis longer to recover than it did for Taylor.

All bundled up for the tube insertion

Having to hold Taylor down is the worst part of it all

Daddy snuggled his sweetie for a long time after it was all over

It has been back and forth, back and forth, back and forth on whether or not we need a pump. Bottom line, yes we do. If I have to syringe 5 feeds a day into her tube, give her three thickened feeds a day through a bottle, and three oral meals a day, that's all I'll be doing is feeding ALL DAY. I believe that finally at the end of the day, after my conversation with a very understanding doctor, it has been approved for us to go home with a pump.

During rounds this morning I said that I was concerned with the fact that Taylor has starting drooling excessively. I'm talking serious drool. Way beyond anything that could be attributed to teething. And she has significantly changed the way she's eating her solids, the pureed meals. She takes a spoonful, swallows, then pushes a bunch of food mixed with saliva out of her mouth while she sort of suckles her tongue. Over and over and over. This is not normal, and I don't want to get home and be concerned that she's not managing her swallowing safely.

So they requested another OT consult, and she came by at lunchtime. She watched Taylor eat and agreed that there has been a definite change from last week and she thinks that we should look at having a FEES study done. This is a Fiberoptic Endoscopic Evaluation of Feeding. A flexible fiberoptic endoscope is introduced transnasally to the patient's hypopharynx where the clinician can clearly view laryngeal and pharyngeal structures.  They can evaluate the sensory and motor status of the pharyngeal and laryngeal mechanism.  Food and liquid are given to the patient so that the integrity of the pharyngeal swallow can be determined.  Information obtained from this examination includes ability to protect the airway, the ability to sustain airway protection for a period of several seconds, the ability to initiate a prompt swallow without spillage of material into the hypopharynx, timing and direction of movement of the food through the hypopharynx, ability to clear the food during the swallow, presence of pooling and residue of material in the hypopharynx, timing of food flow and airway protection, sensitivity of the pharyngeal/laryngeal structures and the effect of anatomy on the swallow.

This will be very interesting to watch. The frustration today was around when this should be done. More back and forth, back and forth. I think it should be done now, but they are questioning whether we should wait until Taylor gets back to normal. What is normal for Taylor right now, I have no idea. And we could wait forever for that. They think it's possibly related to the med adjustments, but the feeding/drooling issues started in the middle of last week, and the Sabril wasn't cut in half until Friday night. Interestingly enough she has been more her 'normal' self, behaviour wise, since the Sabril went down. And I THINK the seizures are coming down. I'm almost hesitant to say that out loud. Anyways, after much back and forth (too many players involved and they're not communicating with each other) and frustration, the FEES study will either be done tomorrow or Thursday.

Thankfully we had a few brighter parts of the day. Thanks Carol for the bag of giggles this morning, Jane for the care package this afternoon, and Mom for the suppers. Taylor napped this afternoon in the funniest position...both arms, one leg, and her other foot's toes all sticking out of the crib, so I'll end tonight's blog with a funny picture...

Slumber Party

I was feeling so sad last night about Marissa and how all of this is affecting her. When I was chatting with Carol on Facebook she suggested that I ask the charge nurse if Marissa could have a sleepover.

So this morning I tracked down the charge nurse and she said if it was just for one night it would be okay. I called Marissa and asked her if she'd like to have a sleepover at the hospital with Mommy and Taylor. She thought it was a good idea, so her bag was packed and she arrived here just after 3 o'clock!

Daddy, what have you done to my hair?!!

I had big plans...I thought we'd go downstairs and have supper together, go outside on the playground for a bit, and come back to the room to watch a movie and eat popcorn. She's got a cold and combine that with a 4 year old and you get lots of whining! Kim was here visiting me, and Jeff came along with Francis and Marissa. When they all left, Marissa and I went down to the playground for a bit, then she was hungry so we went to find some supper. Slim pickings here on the weekend. She seemed happy to have some hard boiled eggs along with a fruit, cheese and cracker plate. So we sat down and she ate two bites of egg, a grape, a cantalope cube, and a bit of watermelon. That was it and she was whining to go back up to the room because she was tired. So I packed up the food to bring back up. She got into a pair of hospital PJ's and crawled into bed. Her typical bedtime is 7:30pm. This was like 5:30pm! She asked for the movie so I put it on and popped the popcorn and she was asleep about 5 minutes later. Craziness!!!

While she was sleeping I fed Taylor her supper. I kept her in her wheelchair for a bit so that her food would settle, then when I noticed she was pooping I quickly scooped her out and into the crib. Too late, it was up the back of her shirt. I put the rail up and swung around to grab the wipes and a diaper when I noticed tubing on the wheelchair. OH CRAP. I looked over at Taylor and...NO TUBE. It had been caught in the chair and came out when I took  her out. Perfect, I have to insert another tube. I guess it's only fair, it was my fault for not checking before I pulled her out of the chair. Before going through that crappy task I decided to give her a quick bath since she was free of  the tube. Then our night nurse came in to hold Taylor while I put the tube back in...took 3 times tonight before I got it in right, first two ended up in her mouth again. Sorry sweetie.

A request had been put in for us to get a pump to take home for her feeds. Normally at home you do gravity feeds, but because Taylor's formula is so fatty it moves through the tube super slow and a gravity feed would take FOREVER. Tonight the nurse came in and said they had gotten a message that we were to use a syringe to do the feeds. That's not too bad because we'd be kind of tied to the house with a pole and pump and 5 feeds a day. This way we can be out and just bring a syringe and her bottle. But it's tricky wondering how quickly to inject the formula into the tube. It would be so easy to squirt it all in over a few minutes, but that would be way faster than her drinking time, so her stomach may not be able to handle that. So it's a slow process injecting 5mL at at time and trying to pace it. But it's only short term, so we'll just deal with it.

I woke Marissa up around 9pm to put her on the toilet and then she didn't go back to bed, she was hungry and thirsty and wanted to watch Treehouse in her bed. Then she asked to cuddle in my bed, so we climbed in together and cuddled for a quite a while and watched Treehouse. So happy she's here. Now she's going between her bed, walking over to the crib to kiss Taylor, and coming over giving me the biggest hugs ever. This is turning out to be a great night together. Just us girls. I love my girls.

EEG News

As I said last night, I needed some time to digest the outcome of yesterday's EEG.

So, here we go...

There was no improvement whatsoever in Taylor's EEG yesterday. It shows a diffuse slow spike and wave pattern during wake and sleep. This goes along with the severity of the epilepsy. The diagnosis of Lennox-Gastaut Syndrome has officially been made. It's not a pretty picture, I attached a link to the name and you can click on it to read more.

Taylor has the three classic features of Lennox Gastaut:

1. frequent generalized seizures of different types
2. diffuse slow spikes and waves on the EEG
3. cognitive dysfunction

As my friend Carol (whose daughter has the same diagnosis) told me last night, it's just a name, they're still the same girls they were a month ago.

When I watched the EEG screen yesterday, I thought it looked the same as it had on Monday. So when Dr. Bello came in yesterday afternoon, I can't say I was really surprised that there had been no improvement.

The technician actually let me take pictures of the screen, so for those of you who are curious this is what it looked like...

And this is what a 'normal' EEG looks like...

Dr. Bello thinks there are two reasons why the seizure activity has suddenly increased:

1. The Vigabatrin could be making it worse. This is the medication Taylor's been on since she started having the Infantile Spasms at 5 months old. He is weaning her off it quickly over the next week. As of last night the dose has been cut in half.
2. The natural history of the condition: developmental delays, onset of Infantile spasms, multiple seizure types, settle into Lennox-Gastaut.

Taylor's having significantly more of her myoclonic seizures which present as a startle seen usually as a jerk of her head and her eyes roll up. Now there's a lot of eye flutters with the rolling, which are also seizures. And most recently the atypical absense seizures, spells of staring and zoning out.

She's not yet having the typical tonic seizures of Lennox-Gastaut, which involve stiffening of just a few muscles or the whole body.

If weaning off the Vigabatrin does not bring her back to her baseline seizure activity, it is going to get a lot more difficult to control. So we are hoping for the best here.

Supergirl

Marissa wouldn't go to dayhome this morning, told Daddy she was too sick to go and she wanted to cuddle. So with me being in hospital with Taylor, he had to stay home from work and take care of the sickie. Apart from a cough I'm not too sure she's all that sick, I think that Mommy and Taylor being gone for so long is really starting to take its toll on our monkey.

Later in the afternoon they came to the hospital to visit. I found out there were Halloween festivities happening down on the main floor so Marissa had come dressed up in one of her princess costumes. I had Mom swing by the house on her way here to get Taylor's costume too. I stumbled upon a pink diaper shirt a little while ago at Old Navy with the Supergirl logo on the front...and since that's what I call her, I clearly had to get it. So rather than a regular costume, Taylor is going to be the 'Supergirl' that she already is wearing the supergirl diaper shirt, some pretty pink tights with hearts all over them, finished with a pink tutu!

Marissa ran across the cafeteria when she saw me and jumped into my arms, it was the best greeting EVER. Missing my little monkey. She had fun running around to the different stations trick-or-treating, but I think between being tired, having a cold, and the sugar rush...she started to rapidly expire when we came back up to Taylor's room. That's to put it nicely. So her and Daddy left...she crashed on the car ride home.

Eddie (aka Mad Hatter), our favourite porter

Granny

Grandpa

Sparkles the clown dressed a little differently for Halloween

When I talked to Francis tonight he was pretty choked up. Marissa had been acting differently all night. Very cuddly and kept looking at him with a forced smile. When he put her to bed she did the same thing and he asked her what was wrong. She broke down crying and said how much she's missing Taylor. Oh my heart nearly broke. This is alot harder on her that I really realized.

It's looking like we're here until Monday for sure. We have one more teaching session before we can go home with the feeding tube, and Francis has to do an insertion. Monday at 11am is the time scheduled for that. So hopefully we can then be finally discharged and have our family back under the same roof.

I did my second tube insertion late this morning when the Home Nutritionist came. It was a little less scary today, although the first one coiled up in her mouth. Quickly got it out and the second time I got it right. Phew. Having some issues with the end caps coming undone and leaking out stomach contents everywhere...GROSS. So we tried a different tube today that apparently the cap stays put better, but it's a one week tube rather than a one month tube. It was a little easier going in than the monthly tube. Not thrilled at having to put in a new one every week, but it's not going to be for too long, so we'll get through it if it ends up working better.

From the teaching session we were taken to Neurophysiology Clinic for a follow up EEG to see if the Phenobarbitol has brought the seizure activity down. I'll blog about that tomorrow. It's late, I'm tired and need to digest the results first.

Signing off for tonight...night night!

"The Plan"

Taylor had a pretty good day. She didn't have to go through another tube change, so that definitely worked in her favour. But that meant that Francis didn't get his turn. He was here at 11am for our second teaching session, but the Home Nutritionist didn't show. That was a bit disappointing. We found out later in the day that she was sick at home.

The OT came this afternoon to discuss "The Plan". So there are three concerns from the Swallow Study report:

1. She had a micro aspiration from her liquids
2. She is pooling the solids at the back of her throat
3. There is bridging of the airway

Both 2 and 3 put her at risk of aspiration. Bridging of the airway means that the pooling food is hovering over the flap right over the airway. The recommendation of the report is just that her liquids be thickened! This is great news! On Tuesday night when they came to talk to me, the impression they gave me was awful and I had her needing a tube for liquids for the rest of her life. Not so!!! There is a carb free thickener called SimplyThick that can be used on the ketogenic diet. We are going to thicken her fluids with it to a nectar consistency. Taylor has no history of aspiration pneumonias, so we are prepared to accept the risk and take all the appropriate steps to minimize this risk:

• She will continue to be observed by the Occupational Therapist and Speech Language Pathologist here at the hospital at our regular Infant Team appointments. They can watch for any feeding concerns.
• We are going to more closely monitor her overall health, how many colds she gets, if we start to notice increased work of breathing, lots of coughing, and signs of pneumonia.
• We are going to thicken her fluids
• We are going to make sure her feeds are as safe as possible: properly positioned in appropriately supported seating, making sure that she is very alert when feeding and not having lots of seizures

If we aren't prepared to accept the risk, the alternative is to tube feed her. They usually only use an NG tube for a few months, and then surgically put in a G tube in her stomach. We're just not prepared to do that at this point, and it isn't the recommendation either.

This SimplyThick can cause a lot of gas and discomfort, so we have to start this in very small amounts. We're going to give her two ounces of thickened fluids three times of day through until Monday, with the rest of her fluid going through the tube. This will gradually increase until all her fluids are thickened. So we will be using the feeding tube short term until she is up to her full fluid intake being thickened.

I like this plan :-)

I can live with this plan :-)

I gave her the first two ounces of thickened formula at suppertime and she handled it very well, without dribbling anything down her chin. This was nice to see, because when she drinks her bottle, quite a lot spills out of her mouth, almost like it's too hard for her to manage.

That's about it for today.

Teaching

Well it's been quite a day!

Unfortunately Taylor had another rough night, up crying from just after midnight to 2am, and then again quite early in the morning. The nurse vented her tube and quite a bit of air came out so I think her tummy was just full of gas and she was uncomfortable. I'll have to remember that trick next time it happens. She finally pooped on her own, so hopefully the continuing constipation issue is beginning to resolve itself.

Taylor's ketogenic dietician came by this morning, so I shared with her all the aspiration stuff from yesterday. I did feel better this morning after my emotional night. She helped me out with some questions to ask:

1. Could Taylor's swallowing have been affected by the tube, therefore affecting the result of the study, and thus the aspiration?
2. How clinically significant is this aspiration when she has no history of aspiration pneumonias?
3. Could her newly increased seizure activity play a role in her current ability to swallow?

She was very helpful in my feeling much better about the outlook of all of this. There is a carb-free thickener that we could use to thicken up her fluids, so it would not interfere with the ketogenic diet. That is good news. This could possibly be as simple as thickening up her formula, which will have to be done gradually. Coming home with a tube is still our reality, but maybe it's just going to be for a short term.

Rounds didn't really have much new on the list, phenobarbitol started last night for the seizures, and a follow up EEG to happen Friday. Taylor's ketones were way too low this morning (1.5 when they should be over 3, ideally in the 4's). In discussion over rounds they discovered that the phenobarb had been ordered as a liquid, which is a syrupy, liquid full of sugar, and therefore NOT ketogenic diet friendly. Crap. So they corrected the order and tonight it was sent as tablets to be crushed and mixed with water. Only other thing of note from rounds was that what has to happen before we can be discharged, is parent teaching with the NG tube.

I had dodged that bullet for 12 days since the tube first went in, but today it caught up with me. We know now that we are definitely coming home with a tube. For how long we don't know, but indeed we are. So the time to learn how to remove it, put it in, and use it has arrived.

My Dad was here visiting when the nurse from Home Nutrition came down to do the first of three teaching sessions. She told me that the last part of today's teaching was to have me remove Taylor's tube and put a new one in. O.M.G. I asked her if she brought a bottle of wine for me to consume after I did this. Sadly, her answer was no.

So she went through the steps with me:

1. Wash hands
2. Cut Duoderm in a rounded teardrop shape about the length of your thumb and apply to cheek with narrow end just under the nostril
3. Cut Tegaderm in half lengthwise
4. Measure and mark tube - Place the end of the tube on the tip of the nose and measure to the ear lobe and then to the midpoint between the breastbone and the belly button. Make a small mark on the tube with a red Sharpie marker. Do it again to make sure your mark is in the same place, if so then make a solid mark around the tube with the Sharpie.
5. Bundle baby - Using a blanket, tightly swaddle with arms tucked in to her sides
6. Remove old tube - Peel the tape and duoderm off all together starting at the outside edge farthest from the nose, once it is all off and the tube is free, pull the entire length straight out with one quick motion.
7. Lubricate tube - Close the cap on the NG tube, and cover the tip with water-soluble lubricant.
8. Insert tube and tape - With child lying on her back and head straight, gently insert the tip of the tube up and angled in, then lift arm and start pushing the tubing in quickly (while holding your breath because at this point you will be FREAKING OUT). If the tube curls in the mouth, don't be alarmed (yeah, whatever!), just pull the tube out and start again. If you are not able to forward the tube, do not force. Pull back slightly and try to insert the tube again. When the red marker line reaches the nostril, the tip of the tube should be in the stomach (and you can breathe again). Have partner hold in place at nostril and across cheek while you tape it in place over the duoderm.
9. Check placement - Withdraw fluid from the stomach by attaching a 30mL syringe to the end of the tube. Using a 30mL syringe is important because if you use a smaller one it could collapse the tube. If you withdraw stomach contents, it means the tube is in the stomach, and placement is good. Gently inject the stomach contents back into the tube as they contain important body fluids and enzymes that your child needs. Flush with 5mL water to clear the tube. If you are not able to withdraw any stomach contents, try again lying on left or right side or sitting up. If still unable, inject 5mL of air quickly into the tube and try again. If you are still unable to withdraw fluid from the stomach, remove NG tube, reinsert and check placement.
10. Cuddle baby (**to apologize for the trauma that you have just put her though!!!!**)
11. Coil tube and tape to clothes behind the shoulder (this is to avoid chance of strangulation)
12. Enjoy a LARGE alcoholic beverage (okay I put this one in myself 'cause I'm sure it would help)

Happy to report......I....DID....IT!!!! And I have a witness, Dad was here to back me, thanks for sticking around for the excitement! Francis...study up on these steps, tomorrow it's your turn!

12 days ago I thought, "there's no way in hell I'm going to put one of those into her". But today I did it. I was shaking like a leaf when it was over and cried, but I did it. Yay for me. Oh the things I never in a million years thought I would learn and do, little Taylor is teaching me along the way. You've brought out strength in me little girl that I didn't realize I had. I love you so very much.

After that was over Taylor's Physiotherapist Jeanette, and her Occupational Therapist Andrea came to our room and took us down to the sensory room for half an hour. Taylor was surrounded by all sorts of different lights, a very nice distraction after subjecting her to such trauma with the tube change, and a nice change of scene from the hospital room. Thanks you two for the little break!

The inpatient OT didn't make it up to see me until 4pm, so we have set a meeting up for 2:30pm tomorrow. I'm a little disappointed that a feeding plan hasn't been organized yet, but it was still a productive day with the teaching and all.

Mom arrived tonight with her arms full. She found some toddler size winter sleepers at Costco, very exciting! Cinnamon buns for breakfast, mmmmm. More laundry all clean and folded. Turkey soup for tomorrow. Banana bread, trail mix, tea bags, sugar and a teacup. A bag of my favorite oatmeal cookies from the bakery at Sobeys. Cutlery, cause I can never find forks around here. Beef stew for her and I to have for supper and a stainless steel water bottle filled up with......RED WINE!!!! I'm sure we violated some sort of hospital policy with that one. At any rate, we enjoyed a delicious supper here in the room complete with a paper cup of red wine. Lovely!

All I Can Do...

...Is Love You To Pieces. The song keeps going through my head thinking about my sweet Taylor. Francis and I had tickets to a Calgary Philharmonic Orchestra concert tonight with Chantal Kreviazuk. It was a crap day today for bad news and I really didn't feel like going but Mom had come to the hospital and she made us go. I guess it was a good distraction. Chantal has such a beautiful voice, and that particular song tonight had me a little choked up.

So...I blogged this morning about yesterday's EEG, and the disappointing results I got this morning when I saw Dr. Bello. Unfortunately that wasn't the only bad news...

Taylor had her Video Fluoroscopy (Swallow Study) this afternoon. Initial results that we were told right away were that there was some question on how Taylor managed the bottle at the beginning and they were going to take another look at it. As for the pureed meal, she is definitely 'pooling' some of it at the back of her throat. Basically she's swallowing, and most of it goes down the right way, but some of it is lingering behind. This puts her at risk of aspiration. I was feeling pretty down about this because I so wanted the result to show that she was swallowing everything nicely and that there was no risk of aspiration happening.

Getting ready for the Video Fluoroscopy

All strapped in and ready to eat

The machine came right up beside Taylor to x-ray her throat from the side

Here's Taylor! Live on x-ray.

The gals from the test came back to see me around 5pm tonight. They had reviewed the study with the Radiologist and Taylor had in fact silently aspirated a small amount of liquid from the bottle feed. My heart just sunk. They have strongly advised that we stop giving Taylor thin liquids orally. I asked if this could improve and what they told me was that it is unlikely. That it is pretty rare for them to see improvement because the swallowing muscles are ones that you cannot strengthen. I felt like they had just sucked any hope out of me, and that Taylor will need to be tube fed liquids forever. The SLP (Speech and Language Pathologist) said that the congestion she heard build in Taylor's throat from the time the study finished to us putting her back in her wheelchair, told her that Taylor was aspirating again. How can I absorb all of this?

So they leave the room and I'm left here alone with everything running through my head. Taylor is coming home with a tube. I have to learn how to use it and put it in. She'll never drink again. Tears came, lots of them. I called Francis but he was in the car with Marissa and her friend so he said he'd call me when he got home. I went to the kitchen to melt the butter to mix into her supper...they told me I can still feed her the purees orally. As I was coming back to the room I passed the Pediatrician in the hall. She saw my red eyes and came to talk to me.

She does not want me to think this is forever. She said that it could be that with everything else happening with Taylor right now, increased seizures and all, that it's possible for her feeding to be affected by it, and maybe once she's back to her baseline, her feeding abilities may also improve. They are going to talk tomorrow with the team from the study, as well as our ketogenic dietician, and come up with an interim plan for how we are going to feed Taylor. There may be a possibility of thickening her formula so that she could take it orally. But then there's still her water requirement, and she definitely can't take that orally. So tomorrow's big priority is the new feeding plan.

What's next I really don't know, but I'm feeling pretty disheartened, discouraged, and sad tonight. I feel like it's another loss for Taylor and I just keep asking whoever out there that may be listening..."why her"? I SO hope that the pediatrician is right and that once Taylor is back to her normal self, this new challenge will resolve itself. But at the same time, the swallow study team didn't give me that kind of hope. I don't want to let them take that hope away from me, but tonight it's hard. Tomorrow is a new day, with that (and some sleep) I will try to face this next challenge with a more positive outlook.

On a happy note, before they came with the bad news, Taylor enjoyed another visit from Sarah the Music Therapist. Definitely the highlight of a difficult day. Thank you Sarah for bringing some smiles our way.

Taylor's exploring the drum

She enjoyed the chimes again

Nice touching Taylor

Spike and Wave

Taylor had a good day yesterday. She ate both meals and drank 1.5 bottles, with the remaining ones going through the tube. Main activity was her EEG. It was at lunchtime so Francis was here and came down to the EEG lab with us and waited until all the electrodes were attached. Then he had to head back to work with it being monthend. Taylor was lying nice and still for the test so instead of being up on the bed with her I put the bedrail up and sat down by the technician so that I could watch the screen. I've never done that before. Without knowing what a normal EEG looks like, I watched the screen and thought to myself..."her poor brain is crazy".

I asked the technician what the seizure activity looked like so she pointed it out to me, which was nice because I don't think they're really supposed to tell me anything. As I continued to watch I was shocked at how much seizure activity I was seeing on the screen while Taylor was sleeping. Every minute, sometimes a handful every minute.

It wasn't until this morning that I saw Dr. Bello who shared the results. There is markedly increased seizure activity from the last EEG which was just 11 days ago. Continuous spike and wave activity. Not what I wanted to hear. He told me not to get discouraged, this happens, and it's not necessarily a regression. But we need to get her back to where she was 11 days ago. So his recommendation is to proceed with starting Taylor on Phenobarbitol. Valproic Acid is apparently another very good option, but I do not like the side effects of that one at all, fatal liver failure...no thank you. So we're going with Phenobarbitol, that will start tonight.

So, now we're here for the rest of the week for sure, he wants to see how Taylor responds to the new drug, and they'll want to do another EEG at the end of the week.

Next up is the swallow study at lunchtime, hope that has better news for us.

I know he said not to be discouraged, but I can't help but be.