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...