Tuesday, October 2, 2018

Reality Bites...

A week ago yesterday Tess got sick. Very sick.

Like one minute she was good and then...bam!

And naturally this would happen on a day that I was orienting with a new home nurse for her. The poor girl walked through our door at 7am expecting to get Tess up and ready to take to school for the day and instead got a crash course on how to help Tess manage her cough, fever, heart rate and respirations. It wasn't pretty.

We spent the whole day moving Tessie to try to get her to cough because I knew her left lung was filling up. We did many breathing treatments, cough assist airway clearance treatments, and a whole lot of suctioning.

But Tess continued to get worse.  By the time Sheila came to help get Tess to bed for the night I had already called her pediatrician and pulmonologist for advice. Her pediatrician called in an antibiotic for her while warning me that it was most likely too late for it to work, and Sheila worked really hard to help Tess move that god awful junk in her lung. She and I did discuss taking Tess to the ER that evening but, and this makes me really angry, we knew that Tess just wasn't "sick enough" yet and we'd be sent right back home. What they don't understand is that by basically making Tess prove that she is sick enough to be admitted, she will have had just enough time for it to turn into pneumonia.

I was up a lot Monday night to try to help Tess breathe easier. I had hooked her up to oxygen and, by early Tuesday morning, was horrified to see her heart rate in the 150s, oxygen in the low 90s despite being on almost 4 liters of supplemental o2, and her respirations were between 70 and 80 breaths per minute. By 7am, when her nurse for the day got to the house, I had already given Tess her breathing treatments and knew we had to get her to the ER pronto. I quickly showered, packed up Tess's med bags, and called 911 while her nurse kept working on her. (If we had been on the island I honestly believe that they would have life flighted Tess off the island. She was in that much trouble).

Scarborough ambulance arrived within just a few minutes, packed Tess up, and loaded her into the ambulance and confirmed that I wanted to be taken to Maine Medical Center in Portland. I rode in the back with Tess.

The ER nurses, docs and respiratory therapists got busy and had drawn blood and done a chest xray before an hour was up. Her blood work showed an elevated white count and that it was bacterial while her xray showed a total white out of the left lung. She was immediately put on three strong antibiotics that covered everything from community acquired to aspiration pneumonia. During this time poor Tessie just felt miserable. She was also requiring deep suction using a nasal catheter and her oxygen dropped alarmingly at times. I was later told they suspected that her lung had at least partially collapsed.

By this point Charlie had gotten from the island to the hospital and helped soothe Tess while I excused myself to step out of her room for a minute for fear of bursting into tears in front of Tessie. I just couldn't handle seeing her struggle like that. It broke my heart.

After a consult with the pediatricians on call upstairs in the Barbara Bush Children's Hospital wing, it was determined that Tess should go to the PICU rather than the regular floor since she needed a lot more help and attention and basically a nurse ready to help at all times. They did tell me that they felt like, while yes, she was very sick, that she was being placed in PICU for the extra nursing help more than that they felt like was was in need of Intensive Care. Sort of like a mid-level of care. More than the regular floor could provide but not as intensive as the PICU made it sound.

During all of this, while still in the ER, they did an ultrasound of Tess's chest to be sure that she didn't have a pulmonary effusion on top of the pneumonia.  They were very concerned at the fact there was literally no air movement in her left lung. Thankfully, no effusion was found.

Tess did okay her first few days in PICU. Tons of breathing treatments, meds and just top notch care but she was still sicker than I've seen her. In fact, I made a point to tell all of her doctors that very thing. They all kept saying that her xray looked very similar to her xrays from her past two admissions for pneumonia and I kept telling them that, no, this time was very different because the past couple of times Tess was admitted, she was not requiring oxygen, wasn't feverish much, and was just better all the way around symptomatically. This time I needed a freakin' ambulance just to get her safely to the hospital from house which was only a ten minute drive away. I needed them to hear me.

This time was different.

One of the pulmonologists came in to talk about possibly doing a bronchoscopy and bronchial wash to help move all of the mucus plugs out of her lung but was concerned that, since she would need to be intubated for this procedure, and she was so sick, that he may not be able to take her off of the breathing equipment when it was over and thus would weaken her overall condition and we would have to make the decision on possible end of life care. He recommended helping Tess fight this on her own first and if and when the time came that she needed to be placed on a breathing tube due to her not being able to keep fighting on her own, that we would revisit the bronchial wash at that time since we would have nothing to lose at that point.

On day three Tess spiked a fever and just generally looked like crap for a kid who had been on steroids and three antibiotics for three days. It was also the highest her fever had been throughout this whole illness. Not good.

More xrays were ordered asap as well as another chest ultrasound (neither showed any new problems thankfully) which didn't really help us answer why the sudden fever but also let us know that she wasn't physically getting worse. We discussed the possibility of switching to even stronger antibiotics but in the end decided to stay the course to see what Tess would do.

By Friday night she was well enough to be moved to the regular peds floor and by late Saturday was able to be weaned off the oxygen.

She was discharged late yesterday but here's the thing, she is still very sick. As in, I have never seen her discharged from the hospital this sick. Like, I would normally be taking her IN to be seen like this, not home after being treated and discharged.

As scary as it is to think about, this may be her/our new reality. Tess was diagnosed with neuromuscular respiratory failure several years ago and, as her pulmonologist said to me before she was discharged yesterday, Tess is at the age where the disease starts to take hold which could explain why this particular pneumonia hit her so hard and she isn't recovering the same way she has in the past.  Last night was long and she needed some help managing her mucus and oxygen. She is acting scared at times because she can't catch her breath when coughing. Her oxygen and heart rate are still all over the place and she may need more oxygen at home now as part of her norm.

Frankly, it sucks. I'm hoping against hope that she just needs more time to heal up and will bounce back and be back to HER NORMAL baseline soon and that this is a temporary hurdle.

My gut has been saying otherwise. I don't think she's even close to being out of the woods with this pneumonia but god, I'm praying that I'm wrong. There are times she is looking so good and I'm like, Yes! She's kicking it! And then she's back down to low 02 stats and choking on her cough.

And if this does end up being the "new normal", all I can say about that is....

Reality bites.

But I'll leave you with this pic that her nurse took of her today when she looked so good because, dang, this kid is a freakin' Warrior!











Wednesday, August 22, 2018

Just another day in the life of Rett Syndrome....

I woke up this morning at my usual time of 6-ish, got up and opened the house up for Tess's home health nurse to arrive at 7. When Lindsay got here  we proceeded to go about our usual routine of getting Tess to the side of her bed, one person holding her while the other washed her and put on deoderant, briefs, bra, shirt, pants, etc. Then we transferred her together to her transport wheelchair that we keep upstairs so that Tess could begin her breathing treatment regimen.

I brushed her hair and put on her favorite movie as Lindsay got out the cart of equipment. And I do mean cart. It holds Tess's nebulizer, suction, cough assist, and chest vest.

Lindsay started with the usual xopenex via nebulizer to help open up Tess's airways as I wandered off to get myself somewhat presentable to take Tess to school by 8:30. After the xopenex came the hypertonic saline, again via nebulizer, and last, the dornase via nebulizer. The dornase is a medicine that is given to kids who have cystic fibrosis. Tess does not have CF but her lungs get "gummy" and can develop mucus plugs that lead to pneumonias similar to what happens to the lungs of a child who does have CF. All of this takes about 30 minutes or so.

Next is the cough assist. It's basically a machine that forces Tess to cough in order to move the crap that sits in her lungs by throwing pressured air down into her lungs then forcefully sucking it quickly back out. It's not pretty and it's not fun and Tess hates it. But man, that particular piece of equipment has quite literally been a lifesaver for Tessie. Then she gets 2 puffs from her flovent inhaler.

She gets this every day, twice a day when she is well. We do it 4 times per day when she is sick.

While all this is going on, Lindsay (or myself if I don't have a nurse on any given day) will mix up Tess's anti-seizure meds. 14 pills gets crushed and pushed into her gtube. FOURTEEN. And that's just the morning's dose. She'll get another 6.5 at 2pm and still another 16 at bed time. That's 36.5 anti-seizure pills per day.

Her seizures are not controlled despite all of that.

After her morning breathing treatment today, just as we were finishing up and getting ready to start the process of getting her on the chair lift and then into her real wheelchair that stays downstairs, Tess went into a seizure.

Lindsay called to me and we began timing. This was a big one. Her breathing was affected, her heart rate shot up to 160 beats per minute and she was drenched in sweat. We gave rescue meds at 4 minutes and again at 12. She still continued to seize. She finally stopped at about the 20 minute mark.

Needless to say, Tess spent the rest of the day in bed while I continued to hear heart alarms as she twitched from the after affects of all of those meds that had been pushed into her little body.

Tonight her temperature is slightly elevated and her heart rate is still a little too high for her so now  wait to see if it's all after affects of this morning's seizure or was the seizure actually a sign that something worse is brewing. Time will tell.

I'll also get to spend the rest of the night worrying about SUDEP.

Just take a second to imagine that. And then imagine it happening on a regular basis.

This is one small part of what goes on in the day of the life someone with Rett Syndrome.

This is just part of the reason I am fundraising for Rett Syndrome. No kid should have to go through this, especially considering how close we are to finding a cure.  Please consider donating to Tess's Team Page.  (hover over Tess's Team and it will link you to her page or you can copy and paste the link below into your search bar).  We are grateful for ANY amount you can give. And please consider sharing either this blog post or the link to Tess's Team Page on your own social media accounts! Thank you! :)

http://events.rettsyndrome.org/site/TR/Strollathon/General?team_id=1074&pg=team&fr_id=1114





Wednesday, March 14, 2018

The Green Envelope On The Fridge...

A couple of week's ago Tess had a check up with her pulmonologist at Maine Medical Center. Knock on wood, the kid has had an amazingly illness free winter in an almost too good to be true fashion. Her doc was very happy with how she was doing, we adjusted some daily breathing treatments, and then things turned pretty serious. 

He asked me if Tess had had the flu vaccine this winter and I replied absolutely, she had it at the beginning of November (which was a little late for her but with moving off the island, we missed the island clinic one).  The doc was happy to hear because, as we all know by now, this year's flu season has been more deadly than most and children like Tessie are hit the hardest. 

I told the doc that I had a huge fear of Tess getting this flu in particular due to everything I'd been hearing about the deadliness of it and then asked if he thought I was overreacting with my fear of it. 

The short answer? No. I was not. 

I then asked the question that haunts my nightmares. If Tess does get this flu, did he think she would survive it. 

The short answer. Again, no. 

ARGH! 

Now before anyone starts to think this guy is a big jerk with no bedside manner, I can assure you he is actually an amazing doctor with a huge heart for these kids and their families. He had even fit us in on a day when he doesn't normally see patients because he wanted to be able to spend an hour and a half with us. Like, he had literally blocked that off in his calendar. He's a good doc and more importantly, a good man. 

What he did say, and which I already knew in my heart, was that the chances of Tess surviving this flu were very slim. Especially since Tess's lungs and body have already been throughs so much so it's just not the same as a healthy child getting it. And, even seemingly healthy kids were dying from it. 

This led to him bringing up the topic of an Advanced Directive for Tess and if we had one. He knew we had met with Maine Medical's Palliative Care nurse a couple of times in the past and had even filled out paperwork called  My Wishes that does deal with end of life care and what that might look like for your child. 

When he asked if Tess was still a full code I very emphatically responded with, "YES!" I want every measure to be used to try to save her if she crashes. 

He looked at me very kindly and asked me gently did I fully understand what that meant? I was confused by the question because, hello! Of course I knew what it meant....CPR, many, many meds, possible life support (temporarily until she gets strong enough to breathe on her own again), etc.

He then asked me if I knew just exactly what CPR entailed. Well, I have watched an awful lot of Grey's Anatomy and have taken several CPR courses so, yeah! Of course I knew.

Except I didn't know. Not really.

CPR would be painful. Chances are they would break her ribs and sternum. They would push cardiac meds. And if they did manage to restart her heart, chances were very good that this would not be an isolated incident. That it was a sign of her body truly wearing down and would happen again. Maybe not that day or week, but also not too far off.  He also said that if they got Tess back, she would most likely need much more help medically speaking than she does now. If Tess ever needs life support due to an illness, that most likely she will never come off of it and would have to live the rest of her life hooked up to a ventilator in order to live. He then added, "And we don't know if we get her back if it's going to be the same girl you know now and in all honesty, I don't think it will be." 

I was just shaking my head no the whole time he was talking. No way in hell was I going to allow anyone to hurt Tessie even if it was because they were trying to save her. Not after everything she's been through and would have gone through to get to that point of needing CPR. I couldn't imagine intentionally inflicting more pain on her. Not on my watch. And the bottom line for me, for us, is Tess has EARNED the right, when her time does come,  to pass away with ease and as much dignity and comfort that we can give her. She deserves it. It would simply be selfish of us to allow anything else. When I told the doctor that I also said that I can't imagine ever signing a DNR on her. It's like saying "I give up" and I will never give up on Tessie. 

He told me there was a form that we could fill out that was a step by step of what we would allow for crisis care for Tess and that we could tailor it to say whatever we wanted.  He felt, given the crazy flu season and Tess's fragile health, that we shouldn't hesitate to get one filled out so that it would be in all of her doctor's computer systems and we would have a copy as well. After that, he set up an appointment for us with Palliative Care again and, after dropping Tessie off at school on Monday morning, we went to fill out that horrible, but necessary, piece of paper. 

Charlie and I have always said that the quality of Tess's life would always be the most important thing. Tessie has a good life. She loves her life. Her family. Oreo. Her school and friends. If she gets so sick that those decisions do need to be made, then Charlie and I have made them together with only that in mind. That Tess deserves whatever is best for her. Not us. If it was up to me, I would say DO EVERYTHING JUST SAVE HER! But that would be selfish. That would be for me, not for Tessie. And that's just not fair. 

In the end Charlie and I opted for a modified Full Code. Do everything you can to save her but no CPR or cardiac meds are to be given. We will  not have her hurt even more during a time when she will already be suffering and needs us to be strong for her. We will have her use breathing support if the doctors believe she has a chance of surviving as well as any and all other meds that could help her but that's where we are drawing the line. At that point it will be up to Tess and God. 

And I pray we have the courage and strength to remember this if and when this terrible time ever comes. 

In the meantime, the Advanced Directive sits in a green envelope that's hanging on our fridge because apparently paramedics are trained to look for them there. As you can imagine, having that staring at you everyday and knowing what's in it brings it's own heartache but I think the bigger heartache would be dishonoring Tess in any way. 

But it also sits next to happy things. Life affirming things like Blake and Bobby's Christmas Card and Ellie's Dean's List paperwork along with, on the other side, an art project of Tessie's. 

So the green envelope's heartache is lessened with daily reminders of the good stuff in life along with the hope and prayers that it will not need to be opened for a long, long, long time. 




Thursday, March 8, 2018

You've Come A Long Way, Baby...

When you first truly understand that your child will have major, lifelong disabilities, it is absolutely devastating. There is just no way to sugar coat it. All of the hopes and dreams that you have for your baby are suddenly crushed beneath the weight of words like, Intellectual Disabilities, Global Developmental Delay, Spastic Quadriplegia, Hypsarrhythmia Infantile Spasms (that eventually will change over to Intractable Seizure Disorder) and so many other terms that just break your heart and, for a while, your spirit. There will be anger and fear and so much sadness that you don't know how you will possibly be able to handle this new world. A world you never in a million years thought you would have to live in.

You attend more meetings than you could ever imagine and make friends with therapists, doctors, social workers and counselors because they are the people you now talk to the most in this new world.  You cry and rage and even try to pretend that it's not as bad as it feels like it is because if you admit it really is bad, it feels like you are betraying your child whom you love with all of your heart.

 You will see other babies and toddlers your child's age and silently compare what your child ISN'T doing. It will hurt so badly because it will make you feel even worse and you are so irrational in your grief that you're almost mad at these babies who are developing "normally". And when you notice that feeling you will give yourself a mental slap because WHO DOES THAT?! And what does it say about you that you, even for a nano second, feel like this? You are lost in a storm tossed sea of  emotions. Joy one minute, anger the next, fear the next, and so on and so on until you are sure no one can possibly survive this type of storm. Yet you hear stories every day in your new group of friends of people who, not only are surviving, they're thriving in it. You'll wonder how they ever got to that place and if you ever will.

You may even avoid bringing your child around other kids their own age or, in my case, ask the people closest to me who had kids Tess's age to not tell me all of the new, fun, and exciting things their kids were doing because I just couldn't take it emotionally. And your true friends and family members will not only understand, they'll go out of their way to try to protect you and make you feel okay about it even though you know it's not okay to ask that of them (forever thankful especially to Kris and Ali for protecting me during those years). Then one day, you'll see those same "normally" developing kids alongside your clearly delayed child and your brain won't compare. You won't marvel at the differences. You won't even feel a little bit sad about it. It won't even be a blip on your radar. You'll be able to not only listen to all of the awesome things your close friends and family members kids are doing, you'll WANT to hear them and go out and support them however you can. You will know in your heart that disability is just a word and that your child is just exactly who they are supposed to be in the world and you will marvel at them and what they have taught you.

  And soon enough those days turn into weeks and then years and you will realize you did survive the storm. You learned how to navigate those rough waters and you mastered the art of sailing through them. It wasn't pretty a lot of the time and you didn't come through the storm the same person as when you got caught in it, but that turns out to be a good thing. And you'll feel pride in yourself and the unimaginably hard work it took to get you there.

All of this to say that Tess had another wheelchair fitting this week. I remember pretty vividly the absolute devastation I felt when I first heard the word "wheelchair" in regards to Tess. It felt like I had been sucker punched. Like all the air was just sucked out of me. I didn't want her to look different than the other kids and I most certainly did not want her to have to have a wheelchair.

After I put it off for as long as I could without admitting the real reasons I was putting off to the people who said she needed it, I  ultimately picked out the one that looked the most like a baby carriage (since Tess was still small enough not to need a "real" wheelchair just yet). I hated that chair with a vengeance. Even though it was the least wheelchiar-y looking, it was bulky and ugly and obviously made for a child who had special needs. A wheelchair to me meant no hope of her ever walking. It was a symbol of everything I was grieving as I was trying to grope my way through the loss of the baby I thought I was going to have.

A few years later Tess had grown enough to need another wheelchair. This time the process was much easier. I was years into the Special Needs world at that point and was starting to leave the grief behind most days and only see the good stuff thanks in large part to some really good friends I'd made because of Tessie (shout out to Buh and Kellie!). They came into my life at a time when my faith in God was really being tested and, without judgement or preaching at me, helped bring me closer to God again, and I am so thankful for that.

At any rate,  I picked out a pink chair for Tessie and asked that they embroider her name on the seat back. When the chair came, I still had a pang of, "I can't believe this is my life.", but for the most part, it was just nice to see her in a properly fitted chair. I wasn't happy about it but I wasn't particularly unhappy about it either. More emotionless over the whole thing.

This past week I went into Tessie's new school where I was met with her PT, her teacher, her aide, and the representative from the durable medical equipment company that we use. This visit was actually fun and full of laughs. I was practical in my decisions of what we needed in a chair (it had to be able to roll easily across more than just pavement unlike her current one, and needed the best possible safety features for van transport).

Then I looked at Tessie and told her it was time for her to choose her wheelchair colors. She got to pick the color for the edging on the seat back and bottom as well as the frame. After giving her several color choices my little diva decided to pimp her ride with pink edging and an orange frame. I was cracking up at just the thought of what this thing is going to look like. The representative (who has done ALL of Tess's chairs and has known us since she was 2 or so) looked at me and said, "Please don't put those two colors together! Do you have any idea what this will look like?! Let me just make it all pink for her!". I laughed at him and said, "Nope! Tessie gets to choose what she wants in her ride and that's what she chose so make it happen for her!". He was shaking his head and chuckling as wrapped up the wheelchair fitting and I told Tess to have a good day at school then left and drove home; still chuckling to myself over her color choices.

But here's the real news: I didn't, even for a second, feel bad about this new wheelchair. It didn't even cross my mind to feel sad or mad or any of the other emotions I have felt in the past. I was just proud of Tessie for being so adamant about her color picks. And, I have to admit, control freak that I am, I am pretty proud of myself for not trying to get Tess to change her color choices or, like I would have in years past (and I'm not necessarily proud of this), simply vetoing her choice and getting the colors I wanted.

Yup, I've come a long way, baby!


Side Note: I use the word "you" at the beginning of this post a lot. Obviously the experience of having a child with special needs is different for everyone and I'm not trying to assume that mine is the same as everyone else's though I do think many of us go through a lot of those emotions.









Saturday, January 13, 2018

Time to Bloom

Since Tess began school here in Scarborough, her teachers have put her through her paces with what is called "Goal Work". Goal Work is exactly what it sounds like. Tessie works on meeting goals so her teachers can get a feel of where she is at academically.  With help from her new teachers, we rewrote Tess's most recent IEP because there were no academic goals in her old one and the girl is crushing everything her teachers are asking of her as if to say, "Pfft, that's all you've got?!".

And Tess's teachers aren't shy about letting me know how smart Tess is. When it has felt like for so long that you're one of the few people who really knows that, well, I'm here to tell you, it feels pretty dang good. Plus, Tessie is also hearing all of these things said about her and she is so proud of herself that it just about melts your heart to see because people that she wants to work hard for are validating that she is indeed smart. Very smart. She's not just cute and funny and loving and all the nice things that are normally used to describe her.

Nope, what gets talked about the most by her teachers is how smart and clever she is. (They also say the other nice things but, being teachers, focus more on her academics.)

She knows all of her numbers, at least 1-10 by sight, she knows all of her letters by sight, she knows her primary colors by sight, and she knows all of her body parts by sight.

So the next step will be having her work on word recognition (which she has begun to do). For example, one of her goals is to be able to recognize her name when she sees it. They are also working on her being able to recognize the words: Reidy, Mama, Daddy, Blake, Ellie & Oreo. Once those goals are met, new ones for her to be able to recognize functional words will begin. Words like: help, hurt, stop, etc. In other words, Tess will be reading! I'm not sure if she'll ever be able to read more than one word at a time but I have learned not to underestimate this kid so the sky's the limit on where this could take her. Can you even imagine how much bigger her world would be if she could learn to read a book on her Tobii Eye Gaze Device all by herself?! As an avid reader myself, this thought excites me a lot!

She is also wowing them in Speech and PT. As I told my FB friends the other day, Tessie stood INDEPENDENTLY, with no hands on her and nothing used for balance,  during her most recent PT session. It lasted only about 3-5 seconds but she did it! And I think it may be the first time EVER that she has done that. Again, can you imagine your child not being able to stand/walk their whole life without someone giving them nearly total assistance and then one day, bam, they're standing on their own under their own power at fourteen years old?! I don't care if it was for 3 seconds or 3 hours, the kid did it! Of course, when she realized she was doing it her PT said she looked sort of startled as if thinking, "I'm not supposed to be able to do this!", and then buckled her knees.

I get a little thrill every time I think about her standing up by herself and when Ellie and I asked her about it, she was clearly super proud of herself which makes me even happier.

Soon, Tess will start having some of her PT sessions at a pool and she will start horseback riding in the spring. I can't wait to see what she amazes us all with next.

Moving to Scarborough was absolutely one of the hardest and most gut wrenching decisions we have ever made and it's been tough for a lot of reasons, but I can say without hesitation, as far as Tessie goes, it's one of the best things we have ever done for her, not just for her schooling, though that's the reason we moved, but as it turns out, for her own sense of self worth and I can't think of anything more important than that.

There is a saying that goes, "Bloom where you are planted." Well, we're literally watching Tess blossom and it's one of the best things I've ever seen.