Time is the wisest counselor of all – Pericles

Origami Spiral by Pan Albers, 2019

November is here and well upon us. It’s hard to believe that it was back in late August, eleven weeks ago, that Sasha had her g-tube placed. It mostly feels like a lifetime ago now. I know of many parents who have said they can’t remember life without the g-tube, and I’m hoping that we get there. They celebrate the ease a g-tube brings; being able to travel more with meals being much more portable. Time opens up for many families as feedings having the potential to be hands free for a caregiver. Food preparation virtually ceases, no more pureeing foods and no more dual food prep – simultaneously cooking foods for the majority of the family along with other items that puree more readily. The quality-of-life metrics promise to be abundant.

For now, though, I have to be honest. I have great disdain for this device. The looks of it, the discomfort it causes Sasha and the representation that Sanfilippo Syndrome is really here now. I see Sanfilippo Syndrome almost more than I see Sasha most days, and I am trying to find some common ground within that reality. I will not let this become my new norm. I remind myself time and again that Sasha is in there, that she is the same Sasha post August 27th that she was on August 26th.

Following suit with her character, Sasha was a true champion throughout the procedure, stoic from start to finish. She tolerated the anesthesia well, the surgical procedure well and resumed eating orally within just a few hours. The nurses were absolutely amazing with Sasha. They were inquisitive and kind, asking thoughtful questions and wanting to know her story starting back so many years ago. As each nurse got to know Sasha, you could see them become increasingly comfortable caring for her. When we wheeled out of PACU after an extended period waiting for a room, the PACU nurse asked who uploaded Sasha’s profile picture to the patient portal: “because that doesn’t look anything like her” she stated incredulously. I smiled and told her she was right. At that moment I knew that after just four hours together, she deeply got Sasha. While I walked behind her stretcher headed to Med-Surg, I uploaded a new profile photo to the patient portal. It was from just moments prior, when Sasha had looked so present during her recovery in PACU.

It was difficult to know the best way to navigate the lead up for this particular situation, and it wasn’t until just the two of us were driving to the hospital that I spoke with Sasha about what was about to occur. For those who may not know, Sasha is significantly developmentally delayed, but we speak with her as we would anyone else. I sometimes think she understands incoming communication but simply cannot express outgoing communication in the way that most do. What I do know is that she comprehends intention. And so, in that pitch-black commute and solitude of a rural New Hampshire byway, I told her what we were driving toward. I told her I was sorry we had to get up so early. And I told her I hoped, I thought, that this would be a good thing. I’m not sure who I was convincing.

I am still grappling with the guilt of making a decision for Sasha that, many years ago, was something I ensured her I would never do. Shortly after her diagnosis in 2008, my care model was to keep invasive interventions at an absolute minimum. To my mind, that meant no g-tube. This was a non-negotiable. It was easy to have that bravado when Sasha, at nine years old, was eating enthusiastically and running all over the land. It continued to be easy for 15 or so more years, and I became complacent that we would never approach that bridge, where oral intake would slow and became work, taxing Sasha’s energy.

My staunch position became harder to maintain as Sasha began losing weight, while medical professionals urged me to consider the g-tube. “It’s an insurance policy” they reminded me. “You never have to use it”.

That last statement, so far, has been true. I have yet to use Sasha’s tube, other than maintaining daily water flushes to ensure that the line remains open (think of it as your home’s plumbing, you never want a clog)!. Yet I know that soon, I will need to use the g-tube for nutritional supplementation. I am bracing myself that, at next week’s weight check during her annual physical, it will likely confirm what I already suspect. And so, that first statement will prove true as well. I now have an insurance policy.

Like me, Sasha does not like the g-tube. It is uncomfortable and she often pushes my hand away during wound care. She still has some bleeding and looks deep in my eyes every time I access the tube. Questioning. On my best days I am thankful for it when she’s eating rather slowly, knowing it will come in handy soon. On my worst days in an irrational state of mind, I quietly consider scheduling an appointment to remove the whole darned thing, hoping to reverse time.

This past Sunday, I attended the funeral services for a very special young man named Sam, just 28 years old, who so valiantly battled MPS 1; Hurlers Syndrome. I met his amazing Mother during a 2020 trip to Washington DC for Rare Disease Week on Capitol Hill. This Mom, along with one other special MPS 1 Mom, and myself, had come to represent New Hampshire to push for legislation to add an MPS disorder to our state’s Newborn Screening panel. In an instant, I was greeted with enthusiasm by these two seasoned Moms when I stepped off the plane, swept into their worlds of advocacy work and adventure. We spent three days together, rushing up and down the steps of capitol hill, scurrying across wide, gleaming stone floors hurrying into meetings with the staff of our congressmen and congresswomen. We sat and shared the medical stories of our children with these complete strangers, knowing our time together on those deep velvet tufted couches was short. It was a time of such bonding. We knew we would gather again one day.

Meer weeks after our DC trip, a pandemic hit. Then, a surprise baby was born. We had plans of going for a walk with Sam’s Mom at our nearby outlet stores as soon as “life settled down some”. A chance for her to meet Sasha and for us to meet Sam, a formidable individual who himself had lobbied on Capitol Hill sharing his medical journey of 38 surgeries and a bone marrow transplant. Alas, time marched on as it insidiously does and eventually, Sam became quite unwell, with extended hospital stays lasting well over a month at a time. Yet I simply could not believe the news when I read last month that Sam had in fact succumbed to MPS. My count-out of eleven weeks since Sasha’s g-tube surgery seemed so insignificant, so superficial as I contemplated the same calendar that Sam’s Mom now looks at every single day.

And so, I am mindful to celebrate the moments in between. The moments when Sasha walks a full circle in our cul-de-sac, even if it requires some breaks. The moments when Sasha smiles just in being together in the kitchen while I prepare dinner. The moments when Nika asks to go into Sasha’s room to play with her hair supplies, or when Suki rests her head in Sasha’s lap. And soon, there will be moments when Sasha and I will explore the good that her g-tube may bring.