Watch what you say

*Warning: Sensitive topics, pictures, and language ahead*

It feels like we have a short period of good time with Greyson only to be slapped in face the next with awful time. The past few days have been incredibly hard. It's like one step forward and then 3 steps back the past few days.

Greyson woke up Monday with a little rattle in his lungs but nothing that was overly concerning. As the day went on it seemed to get a little worse. Tuesday he woke up congested and was breathing irregularly. He would almost gasp for air but not rhythmically. I had to leave for work around 10:30 in the morning. Adam texted me later in the afternoon that he had been suctioning and the stuff coming out was thick, white, but had a red tinge. We've never had that happen before. *picture ahead - just a warning* Once Adam sent me that picture I told him we should probably call hospice just to be safe. I figured it was just a little irritation or something but I didn't want to wait and be wrong. Everything with Tay-Sachs is a balancing act and sometimes I feel like we have everything evened out and other times we might miss something and that scale tips. Our hospice company sent out the on-call nurse and she came to do the assessment on Greyson. She said to do his nebulizer, shake vest, cough assist and suction a few times a day if Greyson can handle it. She gave him morphine to help calm his breathing and cuvposa to help dry up his secretions.

We've had these in our home since we enrolled with hospice but avoided using them. I think Adam and I were both afraid to use them. I know I was.. still kind of am. Cuvposa is great that it dries up Grey's secretions to help avoid him aspirating any of it that could cause pneumonia but it dries up everything else too. Like his eyes, mouth, throat, and anus.. everything. So it's a little scary. Morphine is scary too- not only is it a narcotic but it also comes with other side effects like constipation. Grey has had morphine once in his life up until this week. It was after his g-tube surgery when he was in pain and his heart rate was extremely elevated. He got constipated then so we had a feeling he would again once she gave him his dose Tuesday.

He was so gunky Tuesday night and we had a rough night of sleep. Wednesday I worked in the afternoon and I was able to sleep in a little. I ended up grabbing Greyson from his bed around 7:30am and had him sleep on my chest. He seemed to do better on his belly and upright. I went into work and came home at 5:30pm and he sounded so uncomfortable. I took Grey and made Adam get out of the house for a break. I knew he needed a break. Tuesday night was long again and I had to work all day Wednesday. I told Adam to just text me if I needed to come home.

Wednesday Adam's Uncle Rob wanted to surprise him by driving over from Michigan. I thought it would be a nice surprise despite Grey not feeling the best. Wednesday morning I woke Adam up before I left for work and told him I had a package that was going to be delivered around 11 that he would need to sign for. That way he'd be awake, downstairs, and dressed.... and he would answer the door if someone rang the doorbell! Rob kept me posted on his drive down and surprised Adam! I got home from work and Grey was just so gunky and congested. I decided to give him a nice warm bath with some Vick's to see if we could relax and get some of that crud out. Adam did his shake vest, cough assist, and neb earlier in the day. I know when I feel sick, a hot shower always makes me feel better. I changed his sheets, got nice loose and warm jammies, and drew his bath. We took a longer bath than normal and afterwards just snuggled in the recliner. Adam and his Uncle went to Cabela's and the grocery store to pick up dinner. It was warm Wednesday so Adam grilled out.

Thursday I was scheduled off work and we also had our regular hospice nurse scheduled to come. She came in the afternoon and Grey sounded the same. His oxygen level was low, his breathing was irregular, he was so gunky, and you could tell he didn't feel well at all. It was so hard to see him struggling still after days of not feeling well. We gave him another round of morphine and cuvposa, did a neb treatment and tried to give him some food. Our regular hospice nurse came over and did a listen. She said his lungs sounded like the gunk was mostly in the upper part of his lungs and his lower lungs sounded clear. We did the shake vest and cough assist and just made a plan for what to keep doing to help Grey clear this crap out. We gave him a feed after she left around 3pm. He slept most of the afternoon and around 9pm we noticed his stomach was still full from his lunch feed, which is unusual. He had a dry diaper and he hadn't had a real poop since Monday. We knew his tummy was probably full because he was backed up and decided to call hospice again and have someone come out.

*Warning: This is the picture of how thick his secretions has been*

((That tube is usually clear. The red tinge is the blood we saw that made us call hospice in the first place.))

The nurse on call had to run to St. Vincent's hospital to get a child catheter kit and a suppository for Grey. She ended up getting to our house at midnight. As soon as her car pulled into our driveway, Greyson FINALLY peed. We laid him on the floor and she checked him out. We did the suppository and I scooped him up and snuggled with him while she charged and made her notes on his file and we waited. He didn't have any movement at about 1:45am so she left and let us know to just keep them posted. We had a scheduled visit with our regular LPN at 2pm so we were hoping by then he would have a movement. Grey was exhausted and slept SO well until noon today. He woke up and Karen (the LPN) came over. She typically does a massage for Grey but he was struggling to control his secretions when he was laying flat so she just checked him out and we did his neb, shake vest, cough assist and suction while she was here. We cleared a good amount of gunk and he seems to be doing better today. He's currently snoozing in his ppod chair while I take time to write this and Adam is taking time to play his video game.

A lot of times with Tay-Sachs kiddos- they end up dying from a respiratory infection like pneumonia. Keeping Greyson's lungs clear is incredibly important. We know we are going to lose our son, we know this. We just want to keep him healthy for as long as we can and keep him as comfortable as possible. When we got the diagnosis Adam and I talked and were both on the same page. We wanted Greyson to have quality of life over quantity. Something we shouldn't even have to think about. But this week scared the shit out of me. Part of that was the on-call hospice nurse Tuesday made a comment about how she thought Greyson had "turned the corner". That was stuck in our heads and truly scared the shit out us. He was struggling to breath and you could tell he has been just exhausted. It came on so fast.. he declined SO fast. I'm drained. Adam is drained. It was terrifying. I know we will never be ready when the time does come.. that is something I could never be ready for. But this illness came out of nowhere and felt like it got serious quickly. I'm careful at work to try and not bring anything home. Work has removed the mask mandate for employees but I still wear my N95 every day. I use sanitizer and always wash my hands when I get home with soap and water before I touch him. We don't really go anywhere else. I do online pick-up order for groceries 99% of the time and if we have shopping we need to do we try and go at off times when it won't be so busy. So this illness freaked me out. How did he get sick and how did it happen so quickly. It felt like he couldn't shake it like he could have before... which makes me worried that this will cause a bigger regression. I'm scared. I don't want to lose my son. I don't want to have to plan a funeral. I don't want to pick out an urn. I want to hold him and protect him. I want to take him to the park and let him play. I want to hear him call for me in the morning and he's ready to get up. I want to hold his hand, read him stories, and get him ice cream after a rough day. I want to hear him tell me all about his day with his friends. That emotional rollercoaster is back down in the trough this week. I'm emotional and overwhelmed. I don't have the energy to DO anything. I need to clean my house and get laundry done. I need to order groceries so we can have an actual meal for dinner.

I started to do laundry today. I separated all the clothes and threw in Grey's load first. I went upstairs while it was running and laid down on the couch. I ended up sleeping for 2.5 hours. I struggled to get up and stay awake even after that. My muscles feel sore.. my legs especially. It feels like I did a million squats yesterday.. that burn and jello feeling. I clearly needed the sleep after this week but I still feel just out of it. Adam is currently on spring break from school so we have both had a little more time to do things we'd like to do. I've been doing more crafting and he's had more time to play some video games that he really likes. Something to just turn your brain off for a bit. I recently made this for my sister-in-law. It's their mother's handwriting. Adam and Courtney's Mom Laurie passed away in 2016 and she would always tell them "I love you as much as a pinecone." They aren't sure where that came from but it's a saying that she would always say to them. Her favorite color was purple.. so I thought this was pretty perfect. I took her handwriting and cut it out with my silhouette cutter.

I think I'm going to try and figure out a fundraiser for my family. I can make things with my cutter to sell or find a company to help make tshirts to help spread Greyson's brave story and Tay-Sachs awareness. I want to do something to share our story with as many people as possible.

This was Greyson Thursday night when the on-call nurse was here. This was at 1am, waiting for the suppository to kick in. He was so tired but trying so hard to stay awake.

More snuggles from earlier in the week:)

These are two things I also wanted to share..

With St. Patrick's Day being this week.. I wanted to share this graphic. Adam and I are both Irish. Tay-Sachs was not even a discussion. So we are 2 in 100 people that happened to fall in love, start a family, and happen to be Tay-Sachs carriers. It just boggles my mind how two Yoopers who happen to be Irish are carriers.

This is one I saw this past week on Instagram under MamaRaisingRare. It hit me like a ton of bricks. We have so many appointments, specialists, nurses, care coordinators.. we've had two longer hospital stays.. but he's never EVER been to a park.

This was before Greyson got sick this week. We don't know if it's a seizure or just a muscle twitch/spasm. He doesn't seem bothered by it.. but we call it his "Joey" (from Friends!) How You Doin?

This is the shake vest. The vest inflates with air and he's attached to the machine by long tubes. The machine is programed already and runs for 20 minutes. It's 4 - 5 minute different speeds. You can always stop or pause if you need but it starts slow and harder, then works up to faster and less hard to hit different parts of the lungs. Grey honestly doesn't mind it and typically finds the second set of 5 minutes pretty darn funny.

This was before Grey got sick- he was telling Dada stories!

This was a TikTok I made the other night before he got sick again too. He loves his lights so much, they are so calming to him.

Adam and I have been talking the past few days about our wishes for Greyson's final good-bye. We really don't want to do a typical funeral. We want to do more of a celebration of life. It's so hard to even think about. How do you plan for your child's death? I just know I want my funeral to be more of a celebration of my life, not a stuffy funeral home funeral. We don't want a mass or anything like that. To be honest, we have lost our faith in this diagnosis.. I don't know how strong it was prior and I don't want to get into it much but we are angry with "God" and angry that this is happening to our son and all of the other children who haven't even had a chance to live their lives. I've said it before but honestly, what kind of God damns a child's life before it even begins. We are still angry and right now my focus is taking care of Greyson, spreading his story, sharing what we've learned with this diagnosis, and hopefully helping other rare families in their own struggles. After this week, I think we've only felt these emotions stronger.

Our one year anniversary of the diagnosis is coming up and I can't help but feel just so sad at how much has changed in the past year. Greyson has lost so much. Adam and I have gone through so much. We've had anticipatory grief- and had to learn what that is and what it truly feels like. I have CTSD (chronic traumatic stress disorder.. because it's not post yet, thank goodness).

I learned that term from this article: https://community.today.com/parentingteam/post/dear-special-needs-parents-its-time-to-stop-saying-you-have-ptsd

I've had to learn more about suction, shake vest, cough assist, nebulizers, medications, feeding tubes, gtubes, enzymes, genetics, physical therapy, speech therapy, pulmonology, hospice, respite care, palliative care, complex care, nephrology, GI/nutrition, adaptive equipment, insurance, Katie Beckett waiver, CLTS waiver, social security, etc- than I ever thought possible. I mean that list could keep going. We've watched our son go from rolling over and sitting up with assistance, to not being able to hold his own head up anymore. He can't move like he used to. He has to eat through a gtube. It feels like we've all lost so much. One of my fellow Tay-Sachs Mama's said we're no longer Mama Bears.. we are now Dragon Mamas. We have this fire that gets awaken inside of us with this diagnosis and we learn how to harness it. But the amount of times Adam and I have had to fight for things for Greyson is astronomical. Fight for adaptive equipment, fight for services, fight for basic care that we should have had to! Fight insurance approvals, fight for hospice to come in, fight for a supportive CHAIR for my son to sit in. I will always remember the week after we got our diagnosis I took off from work. Adam and I spent the entire day on the phone trying to find help and find specialties to help us. We didn't have a social worker because our hospital "doesn't provide that". So we had to do everything on our own. We had to find programs and apply, we had to sift through applications, forms, and insurance jargon to get things set up for Greyson. It was a black hole of "What do you need?".. I don't know, what do you provide? We need help. We need HELP. It was infuriating. Everyone kept passing us off to the next person and then to the next. I remember asking for palliative care or hospice right after the diagnosis and being told no, you don't qualify right now. We had to fight for Greyson's ppod chair- why? because insurance only covers ONE seating device for 5 years. They considered Greyson's wheelchair to be a sitting device. Technically we could bring his wheelchair inside and he can sit in there. Oh yeah because the wheelchair is oh-so-comfy. We had to fight with our DME provider to keep Greyson's food pump. We had to remind his doctors so many times to order his equipment and continue to follow-up to make sure we didn't get lost in the shuffle.

We've also gained a lot too in this past year. We gained a lot of support from our fellow NTSAD families, support from friends, family, and community. We have both decided to go back to school to make a difference. I've decided to make it my life's mission to educate about rare diseases and to share Greyson's story.. as painful as it is sometimes. But having an outlet to release some of this grief has been so needed for me. I may not always have the right words or have proper grammar, but this blog isn't about that. It's to help me process and share my son's life with those who want to be a part of it.

This week has been so hard and I'm honestly hoping we are coming out the other end. Grey is currently sleeping in his bed, hooked up to oxygen but his levels look amazing. His O2 is 100% right now and his heart rate is 110. It was up around 140 this afternoon! His breathing sounds more rhythmic and calm. We didn't need to give him any morphine or cuvposa today at all. Fingers crossed tomorrow will be a better day. I'm off work until Thursday now and I'm so thankful the way my schedule worked out. I needed to be home with Grey and Adam.

- Saturday- I ended up falling asleep in Greyson's glider before posting an update. Today was so much better. I spent 99% of the day snuggling Grey in our recliner in the living room. Adam and I had a hard time sleeping last night and I was up a few times checking on Grey. I was in his room at about 6am because he sounded gunky and I was trying to suction him. Once I cleared it I fell asleep in his glider again. We did his meds and food at 8am and got out of bed around 10am. Adam and I both took MUCH needed showers. Grey woke up around 11am and we did another nice warm bath and began his treatments. We did his nebulizer, shake vest, cough assist and some suctioning. He sounds soooo much better today. His lungs sound clear and his breathing is more rhythmic and normal. Our LPN came over and did a massage after we finished and she said he sounded 110x better even from yesterday. She said his lungs sound clear and his respirations were down from yesterday. He isn't working so hard to breathe and is more relaxed.

Here is a picture of us from this afternoon. Both clean, clean jammies, now snuggles!

He was still pretty sleepy today but that's a combo of seizure meds, being sick. and Tay-Sachs I'm sure. I'm currently sitting in his room with his light globe on, he's sound asleep as I type this out. I wanted to post the blog and make sure I try and keep everyone updated on how he has been doing. We were honestly scared while we were in the middle of his illness. He was drained and it felt like we couldn't get our Grey back. But today was the first real day that he was back. You could tell he felt better and he loved his massage. He's feeding better and his pluming is finally working ;) we had lots of diaper changes today! Whoo-hoo!

This is my current view :) My sweet boy cooing in his sleep, feeding pump running to provide him nutrition while his body rests, and his O2 is at 100, heart rate is at 114 right now. I usually sit in here every night while he falls asleep. It's my quiet time. Even though I'm not snuggling and I'm usually scrolling on my phone or trying to read my book, I love this time of night. I love this view and his sweet noises. I love his lights and hearing his breathing just slow as he drifts to sleep in his big boy bed.

Here is to another good day tomorrow and healing thought for my baby boy.

xoxo,

Kim