Wednesday, August 31, 2016

A Day in the Life (except really a couple of weeks...)

August 31st is here, and so ends SMA Awareness Month.  I was determined to get a final post in, and true to my nature, I have waited until the final hours.  The past weeks have felt more like a month or two, and I'm not quite sure how we fit everything in!  So instead of being all thoughtful and organized, I'll just walk you all through our exciting lives since my last post (when Isaac turned 2 1/2, just three weeks ago!).
Experimenting with sound- homemade instruments!

Isaac's appointment at Children's went fine, really well in fact.  He only glared at a few strangers and he actually spoke up a few times to let his sweet voice be heard by those well known to him.  We didn't have to make any changes, and he got a new mask/headgear for us to try.  We were excited about the fit of the headgear, because nothing goes between his eyes or on his forehead, but we were not excited about the giraffe-like print.  For one, not many people look good in yellow with brown polka dots. For another, why does any headgear made for a child need that much dye?  And for another, why do I need my kid to look like a poor excuse for an animal?  When Natalie was 2 1/2, she wanted to wear dresses and skirts and leggings and other normal things that kids wear.  She did not want to dress up like an animal every day, why should Isaac have to?  Anyway,  I have a complaint planned to the makers of the mask, but I don't imagine they will be changing the print anytime soon. I think it took them years of planning just to manufacture this one!

That evening, when we got home from the appointment, I noticed that the SMA Facebook world was going crazy over some "Expanded Access" clinical trial business.  I have never put much thought into these trials because Isaac didn't qualify.  But friends of mine whose children are older than Isaac, or as bi-pap dependent as Isaac, were commenting that their child would qualify for this expanded access, and they all seemed excited.  I wasn't sure what to think or even where to look for more information, and then my phone rang.  My friend Stephanie was calling to get me up to speed and to find out if anyone at Children's had mentioned the program.  What it boils down to is this: Biogen is a company that has been trialing a drug called Nusinersen to treat SMA Type 1.  The trial had gone so well that they ended the trial early with the goal of pursuing the Expanded Access Program (EAP).  That means that the drug is still not approved by the FDA, but that the criteria for getting the drug is opened up to more people.  Biogen's criteria for this EAP is that the person has to have infantile onset SMA and have been diagnosed before the age of 6 months.  So, pretty much everyone with Type 1.  There are no age restrictions, no bi-pap dependency restrictions, and they have enough of the drug for basically everyone with Type 1 in the world.  This is a treatment, not a cure, and there is still no guarantee that Isaac would benefit much from it.  Some kids who have been in this trial from a very early age are reaching motor milestones, some kids are getting stronger little by little and are able to use their ventilators on lower settings.  Children's Hospital was one of the clinical trial sites, and they are in the process of applying to become a site for administering the drug as part of the EAP.  The drug is a spinal injection and requires anesthesiology and at least a night in the hospital.  It is possible that sometime this fall, the drug will be available for Isaac and for every other kid with SMA Type 1 to try.  What? Seriously? What does this mean?  Jeremiah and I stayed up late that night, talking realistically about what it might mean, what it could mean, what we hope it means.  First of all, it means some serious fundraising to pay for the hospital costs- the drug is free, but the associated costs will not be covered by insurance (it's still an experimental drug).  Our thought on that was, who cares? Send us the bill, we will find a way.  Second, we thought, what if this buys Isaac some time? What if a cure is only a year or two away, and this treatment could keep him just strong enough to stay with us until it's here?  Two years ago, we would never have thought that a viable treatment was coming down the pipeline.  Then we started talking about what we hoped for, our big dreams for Isaac. What if this treatment does help him get a little stronger?  Jeremiah said he would love for Isaac to be able to eat something, to swallow it.  I said, "what if he got strong enough to be able to sit up a little bit, and control a power chair?"  And then "What if he got strong enough to be able to say our names?  If he could say "Mom, Daddy, Natalie...?" As we were talking, it struck me how small and unimpressive those goals would sound to most parents, and how insurmountable they sound to us, and how it only took a tiny, far-off glimmer of hope for us to speak those dreams aloud.  To speak, to eat, to move.
Zonked out on the drive home after his long appointment

That Saturday was the candle lighting for SMA Awareness. We lit our candle for Isaac, and another for Holly (my friend Britten's angel baby), and I was so touched by all of the pictures that people posted from all over the place, wherever they were, in honor of Isaac.  I went to bed smiling, and woke up smiling and teary eyed when I read all of the comments and posts.  Thank you, each and every one of you, for your support and prayers and love and light.

After that weekend, my friend Erin came to visit with her sweet baby, Magnolia. I can't even begin to tell you how sweet that baby is, so just believe me she is just happy and beautiful and has the best smile ever.  Erin and I haven't seen each other in four years, but it's one of those friendships where we can just pick up and catch up and enjoy every minute of it.  Isaac and Natalie loved having them here, especially Magnolia.  Isaac spent most of the day watching her, obviously getting a kick out of her smiles and sounds and excited arms that she hasn't quite gotten control of yet.  They flew back to Portland, and I hope I get to visit them there someday!

The next week was a blur. Working, Jeremiah getting sick again, then not working (he had a terrible cold and probably viral conjunctivitis, so I stayed home from work to keep him quarantined), crying while I washed the dishes because I was so stressed out about him being sick again and learning that our food benefits were cancelled because I had been working so much that I made enough not to qualify anymore, only to have another week of not earning enough to pay the bills, and then calming down because we have managed for the past two years to pay the bills and get plenty to eat and to do some amazing things and God takes care of birds in the fields so I should just finish cleaning the kitchen and take a deep breath and calm the heck down.  So I did. Then Jeremiah went to the doctor, got some antibiotics for yet another sinus infection, a diagnosis of another ulcer, and a prescription for an abdominal scan to determine if he needs his gall bladder removed.  Sigh.  We finally tried that giraffe mask, and I decided that I hated it less than I thought I would.  Except then, about 24 hours after we put it on him, Isaac got a mucous plug in his nose and started to turn blue on us.  Jeremiah and I got him stable in what was probably less than a minute. We attributed the plug to waiting a couple of hours after Isaac woke up to do his breathing treatment.  I hadn't slept most of the night because Isaac had slept terribly (he was constipated), so when Isaac woke up, I plopped him, Natalie, and recovering Jeremiah in front of Saturday morning cartoons and went back to bed. None of us needed that adrenaline rush.  It had been a year since we last saw his lips that color.   That afternoon, he did it again, another rush to get the suction machine, to clean a plug out of his nose, to cough him, to get him stable.  Later that day, Jeremiah had to give Isaac a suppository to help him poop, and the sweet kid fell asleep on the floor, watching a bike race with his daddy.  When he woke up, we loaded him onto his stroller and walked up and down our block, up and down, up and down, trying to calm our nerves.  We put him to bed in his old mask, and he was obviously happy to have it back, determined to try the giraffe again on Sunday.  On Sunday morning, I took the kids to church (Jeremiah had been told by his doctor to just 'stay away from people' while his immune system tries to recover), Isaac in his spiffy new mask, and to the farmers' market downtown.  He was in a great mood when we got home, and Natalie wanted to play with him.  We got him situated on his sheepskin rug on the floor, Jeremiah was resting on the couch, and I was doing laundry and dishes.  Natalie was reading books to Isaac, and at one point she came to the kitchen to tell me that Isaac didn't like the books she was reading.  I told her he was probably whining because the stories were over, like he does whenever someone finishes a book.  She said "Oh...okay" and gave me a forced little smile. A couple of minutes later, I had finished fighting with our washing machine (an ongoing battle of 'to spin or not to spin, no seriously, just freaking spin') and came back into the living room to check on the kids.  That's when I heard Isaac's telltale cry, the "help me, this is horrible" whimper.  Natalie was trying to make him happy with toys, but she didn't realize that when he feels like he is suffocating, no toy is going to make him happy.  Jeremiah, dozing on the cough not two feet away, couldn't even hear Isaac's quiet, distressed sounds.  Isaac's lips were dark purple, his eyes were closed, but at least I could hear him. I rolled him over as I shouted to Jeremiah, who literally jumped off the couch and to the suction machine before I could even finish saying "Isaac's not okay."  Thank God Jeremiah is quick on his feet and thinks clearly at times like these.  He told me to get the pulse ox, the cough assist, turn up the oxygen- all things we keep within a few feet.  Natalie quietly slipped away into her room, then peeked out of her doorway while we got Isaac cleared out, breathing normally, and relaxed.  We thanked Natalie for telling us that Isaac wasn't happy, and she said that she knew he wasn't because he had that funny look in his eyes.  We told her if she ever sees Isaac's lips turn that purple color, or she sees that look in his eyes again, she needs to tell us what she sees, to tell us more than that he isn't happy, so that we can help him right away.  We haven't kept his pulse ox on him during the day for almost two years, but I can tell you it will be a while before I go fight with the washing machine for five minutes, thinking Isaac is happily entertained in the next room.  One minute intervals or the pulse ox is on.  Yep, this is life at our house.  Needless to say, we now blame the giraffe mask for those three plugs in one weekend, all in his nose.  The nose piece didn't fit quite right, the medium size was too short, the large caused a really high amount of air leak.  We will try it again, someday, for short periods of time. Someday.
Please note the brown polka dots, which aren't even giraffe print.
 Also, notice his awesome hair- I liked that I could mess it up without a strap being in the way.

The rest of the week was fairly uneventful, if I recall correctly.  By this time, we figured Isaac had probably caught a cold, though it didn't seem too bad (normal heart rate, oxygen, and lung sounds, just extra gunky in his sinuses). Last Friday, after Jeremiah's ultrasound/abdominal scan, my dad came down from Estes Park to help us drive up to their house for a long weekend.  I have wanted to get up there to stay all summer, and finally the stars aligned. We arrived Friday, late afternoon, and unpacked, only to need to do another impromptu breathing treatment on Isaac, whose oxygen only got down to 80% this time!  Victory!

On Saturday, we took the kids fishing at this little trout pond that's just a few blocks from my parents' house.  Please watch this video, it's hilarious.  I had just said the words "Okay, it's time for us to leave," when Jeremiah shouted "we got one!"  He and Isaac reeled it in, and the rest is history (the fish is history too, Jeremiah cooked it for dinner that night).  I only wish I had gotten a better view of Isaac's face the moment after the fish flopped onto his chest...

Later, I took Natalie on a trail ride up at the YMCA.  It was a beautiful day, and a nice ride, but Natalie wanted to know why the guide "just won't stop talking."  When we got off the horse, she said she felt crooked.  I told her I felt crooked too, and that was when we decided we won't ride a horse together again.  On Sunday, we went to Mass at the absolutely beautiful church, Our Lady of the Mountains.  We went out for brunch, then home in time for Isaac to take a much needed nap.  On Monday, we went up to Rocky Mountain National Park.  Jeremiah pushed Isaac all the way around Bear Lake. Natalie informed us that she does not like the national park, because it has too many trees, like a forest, and there are bad creatures in the forest, like snakes and bears.  She liked Sprague Lake better, fewer trees, flatter.  Anyway, it was another beautiful day, perfect weather.  We had a nice picnic, Isaac fell asleep on the walk around Sprague Lake, and Jeremiah and I wished we could have walked around it another 4 times. Nurse Jessica drove up on Tuesday to spend the day.  We wanted to take Isaac swimming at the YMCA, but thought we would start the morning with another trip to the park to walk around the lake again.  We investigated the accessible backcountry camp site, with Jeremiah and I day dreaming of what we would need to make a night of camping happen- batteries, oxygen tanks, big tents, air mattresses, etc etc etc.  That was about the time Isaac started to whine.  We thought he wanted to fall asleep, so Jessica suctioned him and we went about our way.  We got back to the parking lot, where we planned to have another picnic, and suctioned him again.  He had so much saliva and mucous that we just couldn't get it all out, and eventually he started to turn colors again.  There we were, in the parking lot, in the national park, praying that the portable suction would hold its battery power, while Jessica held Isaac's head and coughed him, I suctioned and suctioned and suctioned and suctioned, Isaac gurgled and cried and gurgled and cried, Jeremiah kept us up on his numbers and unpacked equipment, turned up oxygen, got out our supply bag, my mom dug through the bag for a different suction catheter and the afrin (his nose was bleeding again) and kept an eye on Natalie.  I couldn't believe how much we suctioned out, how much Jessica had to just reach in his mouth and help him get out because the suction machine couldn't keep up.  All the while, people walking by, cars driving past, Natalie telling us that Isaac is so mad because of the boogers.  And once again, Isaac stabilized and was just fine.  Thank God Jessica was there, thank God my mom was there, that the battery held up.  We packed up and drove back to my parents house, got everything charged up, and relaxed a bit.  Sort of.  We had planned to go home late that afternoon, but opted to go earlier so that Jessica would be able to help us at home.  When I told Natalie that we were going to pack up to go home, she replied "But, I thought you said we were going swimming..." She understood, however, and decided it would be more fun to go swim at the rec center sometime, where she can play and touch the bottom.

The whole drive home, I had this tight bundle of nerves instead of a stomach, and my heart was pounding until we drove through Longmont.  Isaac did absolutely fine on the drive home, during his treatment that night,  Wait, last night. That was just yesterday. Jeremiah worked off some adrenaline by building part of our fence and going for a bike ride.  And then we all went to bed, and slept.

That brings us to today, the last day of August!  It was a long and eventful and memorable month. Our trip to Estes Park was stressful, but our daily lives are stressful. It was also a lot of fun, and so wonderful to just spend time with my parents and brother, to see Natalie ride around on my brother's lap in his wheelchair (that hip surgery business is no joke) and play with the dogs, to see Isaac catch his first fish and have daily sword fights with Grandpa at the dinner table, for my mom to bake with the kids, to see my aunt and uncle, and for all of us to just get out of our house and into the mountain air.  

I saw a woman at church on Sunday, who I noticed because she was striking in her manner and so beautiful.  She was probably 65 or so, with long white hair, dark blue eyes, high cheekbones and prominent jawline. She had the most serene look, with a smile resting on her face as she watched the altar.  Then, I saw her on Monday at Sprague Lake, greeting us as she and her husband passed us with a sweet 'good morning' and that same look of quiet joy on her face.  I saw her a third and final time, hiking around Lake Estes as we drove out of town. She was walking up an incline, face to the wind, eyes half closed, and her lips relaxed into that smile.  I took a moment to pray for her-why else would God put her in my path three times in as many days- and to wonder about her story.  I turned my attention back to Isaac for the rest of the drive home, but I kept thinking of her as I tried to fall asleep last night.  I had this feeling that she has known significant pain, suffering, stress, and trials, just as many people have.  But somehow she has come to place of joy, or perhaps she never left that place, despite anything else that might have happened in her life.  It is my prayer, for each of us, that we can endure our suffering with grace, use every opportunity to share a smile, and to feel joy in the presence of God, everywhere that we find Him.

1 comment:

  1. Ah Jenna...the last two lines are breath taking! Amen!

    And Good NESS...what an amazing time. Everything seems so lovely and so scary at the same time. Those two things don't seem to go together...but, like you said, that is your life! And a good one it is:-)

    It all sounds so wonderful and I feel like I was right there with you!

    You guys and Isaac are in our prayers every night and you always will be!!! Thanks for sharing your life with us...Sure makes mine seem simple and taken for granted some times!

    xoxoxo Renee


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