Exploring the gray area between “Hell Yes!” and “Hell No!”

I’ve been living by a new motto lately, “If it’s not a hell YES, it’s a hell NO.” It’s been liberating.

Then last month I was asked if I’d like to represent the MPS IVA community at the University of Minnesota Medical School’s Mucopolysaccharidosis Newborn Screening Meeting, providing perspective on what a newborn screen would have meant regarding an early diagnosis for Cooper.

Do I want to travel and spend a few days in Minneapolis?
Do I want to catch up on work at night while I listen to Newborn Screening topics during the day?
Do I want to watch my family coordinate what appears to be more complicated than the moon landing to cover for me while I’m gone?
Do I want to re-live Cooper’s diagnosis, feel all the things, share my feelings and experience with a room (and internet) full of people?

Not really. But is it important? Yes. I’m here.

Tomorrow is my 15 minutes of fame. So, what would having known Cooper has MPS IVA at a few days after he was born (instead of at 16 months of age) have meant?

earlier treatment (weekly Enzyme Replacement Therapy infusions)
connecting with the right folks (National MPS Society, Children’s Hospital Colorado) earlier
less symptoms for Cooper (medicine would have had 16 more months to work)
no diagnostic odyssey

Cooper had symptoms at birth, but we and his pediatrician didn’t know the symptoms to look for. Cooper was diagnosed relatively early. I know of other MPS IVA families that have had a horrific journey finding the correct diagnosis, and in the meantime, missing out on years of treatment. MPS does irreversible damage. Treatment slows/stops the damage – it can’t reverse it. So if there is treatment, and a way to diagnosis it, let’s know about it right away, OK?

While in Minneapolis, I figured I’d treat myself to the Minnesota Wild hockey game. I’ve been to NHL games by myself in new cities before – this would be fun and exciting! I love ice hockey! But after the last session today, I came back to the hotel. I took off my shoes and flopped on the bed. I took a nap. I watched reruns of The Office. I played a game on my phone. I lounged on the bed for four hours. I beat myself up for not checking in on work, not participating in the happy hour, and not going to the Wild game. But I was reminded I needed to relax. I deserve it. So my pampering today looks like a hotel room with the remote all to myself. Not the beach or spa I have in my mind when I think of “getting away”.

After my lavish downtime this evening, I put my shoes on, found dinner and drinks in the hotel bar, and watched the Colorado Avalanche game while I caught up on work.

Tomorrow I’ll share my experience alongside others in our situation – we have a treatment but had to find the diagnosis on our own. Then I’ll go home and participate in what I call “re-entry”. Just like a spacecraft coming back to Earth with astronauts, I’ll jump back in to family life.

Cooper called me while I was at dinner tonight. He needed to know where the note cards were. He is working on his Ted Talk that every 5th grader at his school does. Cooper’s first line is, “It’s hard being a 3-foot-tall fifth grader”. He goes on to talk about his disease, all his surgeries and how he feels when people gawk at him. Our short conversation made me realize this mission to Minneapolis was a “hell yes”.

The bottom of the hill

This morning Campbell and I took the dogs on a walk. Our sweet old mutt, Roscoe, is almost 14 years old. His hearing is going and his back legs don’t work as well as they used to. But he loves going on walks and barking at anyone who walks by our house. I feel like he’s living on borrowed time.

Our dog walk route is down the street, turn left, down Dutch Creek to the park at the end of the street. At the end of the street today Roscoe‘s back legs gave out. Repeatedly. He wasn’t in pain, but it was heartbreaking to watch him try to get up again and again. He managed to remind those back legs how to walk again and we made it home (very slowly), but at the bottom of the hill, the floodgates opened.

I wept. Watching Roscoe was the pin that poked the balloon. I cried about him, but then everything came to the surface. I sobbed about Cooper’s surgery (I don’t want him to need it. I don’t want him to go through it. I don’t want him to have the anxiety and the recovery.) I sobbed about how I don’t want to go camping, and that ruins my family’s plans. (I don’t want to tweak my back. I’m scared of the uneven surfaces and my partially broken ankle. I am crushed that this makes me sound old and broken.) I sobbed about Johanna’s red Subaru and how it’s totaled. (Here’s the lunatic part – just this morning I saw Johanna’s post about the Subaru. Am I attached to the Subaru? No. Last time I was in the Subaru? 7 years ago. But I am channeling all of the emotions today and I feel you Johanna.) I cried about it all. And more.

How ironic is that? At the bottom of the hill, needing to climb all the way up. One foot in front of the other, I cried all the way home.

I try to be a robot – strong, doing all the things correctly. But today I am human.

I have so many emotions after my last blog post. It’s hard to put myself out there. Sharing our experience is the easy part – it’s processing the events – after they happen, again when writing, then again in conversations that takes a lot of energy. I bottle up the feelings that comes with processing. But the emotion is real and needs to happen to get me to the next step.

I am grateful for my community. Everyone who reads my ramblings, reaches out, makes me smile, hugs me, assists in our journey. Thank you.

Although we have this hill to climb, I know we have a support team. In my mind, our team looks like the support car that follows a Tour De France rider. But ours is a like a clown car, because there are so many people crammed in there. ♥️

Cover photo (Dutch Creek with rocks and blue sky) courtesy of Campbell.

WTH

What the Hell? I know life in general, let alone life with a child with a rare disease isn’t easy, but lately the shrug emoji is what I use most often to describe WTH is going on.

Two weeks ago Cooper’s surgery didn’t go as planned. As a matter of fact, it didn’t go. You can get details on CaringBridge, but after anesthesia, the surgical team stopped because the Neuromonitoring system lost signal on the right side of his body – possibly indicating spinal cord damage. It wasn’t bad, but it wasn’t good. We just opened a can of worms. And we have to muddle through all these slimy, entwined worms before we can move on. Cooper’s hernia bothers him daily – to the point of tears sometimes. His port is becoming harder to work with during infusion. The situation is becoming urgent.

The next step is to get an MRI on Cooper’s C-spine. Orders have been faxed in. I called and the radiology scheduler told me I had to go through a screening process to see if Cooper should go under anesthesia for the procedure. (Insert WTH and shrug emoji here.) I mentioned that Cooper did not need anesthesia for his MRI 3 weeks ago. I did NOT however tell him that the last time Cooper was under anesthesia he narrowly escaped paralyzation. Scheduler said, NO, the screening still needs to happen. I’m a rule follower. I know he needs to check the damn box before we get scheduled. So I was transferred to the “screening” number and left a message. 19 hours later and still no call back. The irony is that he shouldn’t go under anesthesia until we figure out what’s going on with his C-spine, hence the MRI! A growing circle of frustration right now. I’ll call again today.

In the mean time, let’s not forget about my oldest, the “unaffected sibling”. She’s growing up, she doesn’t want to talk with me about “stuff” unless it requires turning on the internet or ordering something on Amazon. I’m so stinking proud of her. Straight A’s, she gives her whole heart to hockey. She’s funny, and sweet (although I don’t get to witness that side much).

Campbell, #43. Watching her thrive here fills my soul.

My self care is struggling. STRESS EATING. There, now that it’s published, I’m accountable for it. Valentine’s Day treats didn’t help. But that’s over, they were delicious and I’m gracing myself. I’d like to work out again. I’ll get there – I threw out my back last week and with chiropractic help I’m getting back to normal. I want to plan a girls’ trip, I’d like to figure out family summer vacation plans, but my planning is paralyzed with fear until we get Cooper figured out. The next MRI is just a piece of the puzzle. It may lead to bigger things – bigger than the port and the hernia. It may lead to spine surgery. Which would mean another summer “vacation” in Delaware, and recovery at home, on the love sac, next to Velocity. Which is fine. We are blessed to have the right teams in place to help make decisions. I’m just a planner and this in between, waiting on checking the next box is torture for me.

I’m reading at night instead of scrolling on my phone. Check out “Present over Perfect”. It helps me slow down and connect with what is really important TO ME, not what is important to others. Which I desperately need. So in the vein of self-help, I’ll list what I’m grateful for: Velocity, home, my health, supportive family, community, out of this world friends, hockey, fun times, my job, modern medicine, garage door repair guys. You may ask, “Garage door repair guys?” Ask Brian about how I backed into the garage door. And now I’m slowing down? Perhaps I should have slowed down a while ago. But this is where I’m at, where we’re at. All I can do is make adjustments (chiropractor joke, hahaha), embrace the now and move forward.

Cooper and Velocity, at work, and at play

Update: after speaking with radiology (thank you Caylee at CHCO radiology who heard my cry for help), we are scheduled for an MRI on this Friday!

Today Was Rough

And I can’t sleep.

While at infusion today I envisioned a blog post about how things are great, and I’d casually mention I broke my ankle three weeks ago playing floor hockey with Cooper. And he’s doing great, so excited to be on a baseball team this summer. And Campbell is happy, completely enveloped in ice hockey and loving it. And my ankle is healing. The walking boot has slowed me down, but with all the playoff hockey on, it’s OK to put my foot up for a while.

But I didn’t blog because I was too busy playing NHL ‘18 on the hospital’s Xbox with Cooper. Or watching him play. It’s his new favorite thing to do while at infusion all day, since he doesn’t have school work (or arguing about doing school work) to take up the time.

Check out the bottom left – Cooper created player #18, Cooper Tippett

It’s been a long time since we’ve had a rough day at infusion. But due to the butterfly needle sitting a bit weird in his medi-port today, we had a couple awful moments. Pressure on his port from trying to hep-lock him made him go through the roof. The pressure of the infusion all day was just fine – but the pump wouldn’t work with the syringe of heparin, which meant we had to hold him down, peel off the dressing (sticker covering the needle – always the worst part of infusion day), reposition the needle, push the heparin into his IV and then remove the needle and be done. As it’s always been, he’s so anxious about the process that the mere act of touching the needle and discussing what we needed to do made him so upset and frantic, he was literally sick to his stomach. So when we tried to comfort and distract him as our skilled, gracious and caring nurses maneuvered the needle, I had flashbacks of last summer when Cooper had his first cast removed, before his second hip, knee and ankle surgery. Last summer Coop screamed at the top of his lungs as the doctor started the saw – he thought they were starting surgery and didn’t realize/listen to the fact that they were only removing the cast. Screaming. Tears. “I can’t do this anymore!” “I don’t want to do this!” All again today.

Coop recovered very well. He held gauze over his blood spot as it dried up. His tears disappeared as he said goodbye and that he’d see everyone next week and play more NHL ‘18. We gathered our things and walked out of the hospital into the fresh air. That’s where Coop lost it. Just big tears as we stood on the sidewalk, waiting for Velocity to pee.

At home, Coop continued to feel better. He said he’s sad. I hear you buddy. I’m glad you’re not mad, or scared. I’m sad too.

So I’m heartbroken and I can’t sleep. Everyday I’m thankful there is a treatment for Cooper’s rare disease. I’m sad that Cooper has to live with it. I’m sad that Campbell has to live in his shadow. Campbell’s hockey gives her a place out of that shadow. Cooper’s upcoming YMCA baseball season will give him a place further from the medical rare disease space. But it will always loom near him. Like the fact that he’ll be playing baseball with 1st and 2nd graders, because they are the size of my 4th grader. He’ll make new friends with the kids, and he’ll be excited. But that takes care of this year. Next year he’ll be the same size, and the next year, and the next year. His problems and his differences won’t get easier or less noticeable. Just like today – we think we’ve got this infusion thing under control and we get a curve ball. Another reminder that I’m not really in charge, I really have no control over anything. It’s time to remind myself to put this in God’s hands and move on. Do what I can, and move on. Perhaps sleep.

Play Hard, Play Fair, Give Back

On the heels of MPS Awareness Day I am overwhelmed by the outpouring of support for Cooper, his situation and our journey. MPS Awareness day always sends me reeling. Usually I have a “wear purple!!” campaign and everyone posts photos of themselves wearing purple and wishing Cooper well. Instead of a purple campaign this year I was busy trying to generate interest in the MPS Society virtual gala and bidding on the auction items I had procured. Alas, the Gala and auction were a success and we toasted in celebration with a tiny group of vaxxed friends and family. But the excitement of the gala and MPS Awareness day is just one part of the past week.

This past Monday, the MPS Society organized virtual Hill visits to advocate on behalf of our MPS and ML affected individuals and families. We asked that our representatives and senators support newborn screening legislation and the STAT Act. We also asked our Senators to create a resolution to make May 15 National MPS Awareness Day. Senator Bennet’s office agreed to do the resolution!!! From what I understand, it’ll be presented to the Senate and a press release will be coming this week. HUGE WIN! So excited for a Colorado Senator to step up for us!

My mom, myself, Jamie, Jack and Mary (from Senator Bennet’s office) for the MPS Society Advocacy Day

Last weekend we were blessed to become a part of the Dawg Nation family. We all had the most wonderful night, being OUT, watching hockey, talking hockey, making new friends and finding old friends. We were surrounded by people lifting us up, celebrating and supporting Cooper. I felt like I was in a dream! We are excited to be involved and pay it forward with Dawg Nation.

Cooper with fellow Dawgs and Colorado Avalanche greats, Jan Hejda and Milan Hejduk

Of course these wonderful things happen and all the while sweet Velocity is by Cooper’s side. Everyday since February 14, 2020 she’s here. This expertly trained ball of white floof is part of our family, free of charge, as Cooper’s Service dog. Every week at infusion, she’s there. Every doctor appointment. Everyday I am reminded of the generosity and compassion of the staff, donors and volunteers behind Canine Companions.

Cooper and Velocity waiting to check in at the hospital for infusion.

I’ve mentioned it before, but I need to mention it again. The hospital staff is our adopted family. They see to it that Cooper has a great day, every infusion day. They’re his hockey team, his biggest supporters, and Velocity’s biggest fans. They are there with an ear to listen and empathic smile as I share our ups and downs. They’re along for this journey with us and they take care of the Tippetts. We leave every week after infusion and although we are relieved to go home after the long day, we look forward to being back with friends again next week. We are forever grateful for the compassionate care and buddies we have at Children’s Hospital Colorado.

School (noun): an institution for educating children. I am overjoyed that school for my kids means so much more. A safe place to be heard and seen. A place with friends, staff and curriculum they enjoy. An extension of home where the adults there are looking out for their wellbeing just as much as we do. Campbell is thriving at her “hockey school”. She’s made great friends, developed leaps and bounds as a hockey player, and is challenged academically. Of course the engineer in me is proud that math is her favorite subject! Cooper’s school continues to lift him up every day. His team is the best. I can’t put it any other way. Modify the bathroom for him? OK. Lower a swing? done. Need a HC accessible switch on the door? done. iPad robot in the classroom so that Cooper can attend school virtually while at infusion and recovering from surgery? sure! (We knew all about online school before COVID.) Special seating for each classroom/lunch situation? done. Speakers in the classroom or a speaker for the teacher so he can hear better? check. Customize the special class outings so he feels a part of the fun? yep. And then we’ll touch base and they’ll suggest ways it needs to be changed so it can better. Of course the IEP and Special Ed department are to thank, but this group goes above and beyond to make sure school is everything it can be for Cooper. I’m constantly full of joy and appreciation for Cooper’s experience as an elementary school kid.

Campbell and her classmates celebrating after her goal

So what am I getting at? My heart is so full. Our bucket is so full. We are living life to the fullest, and we need to give back. I know I do things to give back. But I feel I need to do more. Visit my new page, Extraordinary Organizations. Donate your time, talent or cold hard cash to these organizations that have lifted us up, and do so for countless other families as well. Share their missions. In the meantime, I’m going to carve more time out of my day to be of help where I can.

Thank you for lifting us up. For reading my rants. For sharing our story. My new mission is Dawg Nation’s motto – Play Hard, Play Fair, Give Back.