Lately, it Occurs to Me What a Long, Strange Trip it’s Been – Grateful Dead

Last night I had the first dream about my Dad since his passing.  It seems fitting as it is the night before Valentine’s Day and most of my memories from this holiday are of him always getting me some outlandish gift for my given age.  This was often an elaborate box of say 50 or 100 piece chocolates, which was thrilling to receive as a five or eight year old.  There was always an equally lavish card, huge in size with a red rose on the cover reading “To my Daughter….” in some fancy scroll. The card was always geared towards an adult, never a cartoon or whimsical kids card, which in an of itself I always found humorous even when I was little. 

In last nights dream, I was helping my Dad to stand from his wheelchair, something he hadn’t been able to physically do for roughly the last 10 years of his life.  He was in a hospital gown and as I held him up, I wanted to make sure it felt physically ok to stand, as that hadn’t occurred for so very long.  But he couldn’t understand me with my mask on (oh how COVID has permeated even the subconsciousness).   So I took off my mask and asked again “does it feel good to stand?” and a wave of complete peace fell over his face.  It was the same exact expression I witnessed when a nurse came in to give him a head message a day or so before he passed.  That was it, then I woke up. 

Hopefully this isn’t a surprise announcement, and I believe most readers know by now that I’m expecting a little girl soon.  Her due date happens to be very close to my Dad’s birthday. For a large chunk of this pregnancy, I’ve been reflecting on so many memories of the early years with Sasha.  Starting at the very beginning, protesting being told it was time to be discharged from the hospital and take her home.  I felt so unready.  Recalling Sasha’s first passport photo, how you can see the tips of my fingers holding her newborn head since we decided to take her to Southern France at just two months old (I’d mostly advise against this).  Those times around the age of 3 when Sasha would obsessively watch Elmo videos, standing so close to the TV so that when the VHS would pop out, she would pop it right back in again to hit replay.  “La La La La, La La La La, Elmos Song”….I can still hear that melody.

Then the harder years come into view, recalling the countless phone calls at work politely asking me to pick Sasha up from numerous day care centers and Montessori schools.  She’d thrown a wooden toy across the room again and hit someone in the head, or disrupted formal learning modules.  She wanted to play duck-duck-goose instead of sitting during circle story time. I can still see her walking back to the car each time with her characteristic chip on the shoulder gait that could almost be seen underneath the straps of her Dr. Suess backpack.  It was as if to say “that didn’t go so well but tomorrow’s a new day”.  I always wondered if she understood that she wouldn’t be returning there tomorrow. 

I reflect on all of these memories now through a new lens and think to myself wow, I’m about to embark on this journey all over again, twenty two years later, with such different context behind me and ahead of me. 

There’s been lots of time now for this surprise news to set in, and we’re so excited and (mostly) prepared for this imminent arrival.  The nursery is almost ready, such a peaceful space as I sit in that very room now while typing.  As I look over at this sweet space my eye wanders to the built in window seat that one of my dear friends and her Mom completely brought to life.  Last fall we went to a fabric store together to pick out seat cushions and pillow clothes, and while I had a vision in mind of something very different, when I came across a simple fabric of tan arrows crisscrossed with the black text “Be Brave Little One”, I instantly knew I’d found my fabric.  As I drove home with those yards of joy and creativity sitting in the bag next to me, a wave of emotions flooded in.  We are called upon to be brave in so many instances of our daily lives.  I know we’ll have to be brave one day as we navigate tougher waters with Sasha.  One day her little sister will likely experience a magnitude of loss that I can’t begin to imagine. Soon, I’ll need to be brave as labor approaches and all of its unknowns.   

But if there’s one take away from that recent dream with my Dad, it’s that wherever you may find yourself, remain tall, take in a deep breath and exhale.  I know with certainty that we have many great adventures ahead of us.  Together, we will learn to be brave. And despite any worries, the lyrics from one of Dad’s favorite Grateful Dead songs Truckin can help guide the way; “hang it up and see what tomorrow brings”. 

“Today you are you! That is truer than true! There is no one alive who is you-er than you!”  –  Dr. Seuss

Its hard to believe that another birthday is upon is.  Tomorrow as is with each day, Sasha will yet again amaze us all when she awakes full of smiles, eagerly enjoys her usual multicourse breakfast and sets out on her day.  But it won’t be any old day. Tomorrow is Sasha’s twenty-second birthday.

In the weeks leading up, I reflected one evening at how well Sasha was doing.  Yes, this year has had some major changes, but they feel major to us because we are so fortunate that Sasha’s baseline tends to run for so very long.  We typically go many years before seeing a dip from her plateau, and so it feels very sudden and unexpected when changes start to unfold.

When Sasha was diagnosed at the age of nine with Sanfilippo Syndrome, we were told the finish line would likely come during her teen years.  We’ve seen this reality come to pass far too often in the Sanfilippo world.

So it was a few weeks ago, as I sat at the dining room table with Sasha bathed in gratitude at her enjoyment of dinner, when a wave of inspiration came over me.  It was as if Dr. Seuss suddenly came to sit beside me, for to my surprise a simple poem about Sasha’s upcoming birthday wrote itself as we sat at the table.  I turned into scribe, and quickly emailed the poem to myself to save for Sasha’s big day.

Happy Birthday Eve, Sasha.

Twenty-Two

Is it true?  Is it true?
Tomorrow you’re turning 22?
What is it you’d love to do?
Go high up in a hot air balloon?
Or for a whale watch on a pontoon?

Perhaps you’d like to travel far….
Or stay up late, for shooting stars
Would you like to buy a horse?
Or ride a dune buggy, tearing up some golf course?

What favorite foods shall we eat?
I bet you’ll say, “nothing but SWEETS!!”
Or would you prefer to sleep in late?
Or watch the sunrise, over a lake?
Whatever it is, you choose to do..

We’ll gladly give it all to you!! 

 

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i·den·ti·ty

/ˌīˈden(t)ədē/

The fact of being who or what a person or thing is.

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For the first time in perhaps ever, I saw a flash of what the end might look like.

I was walking from the kitchen into the dining room to bring Sasha her dinner when I received this rapid flash, mental images, a series of photographs and short video clips, careening through my minds eye. It was Sasha nearing the end of her valiant struggle with Sanfilippo Syndrome.

I’m not really sure where this came from. I know there have been reports during this time of stay-at-home orders of people experiencing vivid dreams, impacted sleep. Still, COVID aside, we’ve witnessed changes this year. Sasha has begun falling. We recently moved and so I was more than happy to blame these events on transitioning into a new house. I much preferred the idea that Sasha was trying to adjust to a new floor plan rather than consider that perhaps Sanfilippo Syndrome was starting to take hold.

I am grateful for the group of Sanfilippo parents I know, some I’ve met in person, some who have become pen pals, some who are both. At times I reach out to pose questions regarding their experiences. It saddens me that my probing initiates a fellow Sanfilippo Mother reflecting back on what are unimaginably painful memories, and so I’m choosing to tread lightly with these cherished resources. But through some messaging I’ve come to learn we are now in the next phase of this syndrome.

 

There’s been a variety of issues happening all at once regarding Sasha’s mobility. I wish not to bore you with clinical details but it helps me to process. She’s having a right foot drop and a right foot drag, so she is often tripping over her own foot. More routinely now, Sasha doesn’t lift her foot high enough to clear the floor beneath her or to pass through a threshold. Her left leg on the other hand, is presenting an interesting phenomenon where it’s as if that hip joint doesn’t want to rotate, impeding her ability to pivot.   This means when she turns, its as though her left foot is glued to the floor beneath her, like a well rooted tree. The right foot overcompensates, kicking out sideways to find balance and then boom, she falls directly onto her side without breaking her own fall. This has me particularly worried as hip issues are common with SF Syndrome, and her landing straight on her hip could be disastrous. This is no time to be headed to an ER. In addition, when standing still. Sasha falls straight backwards.  It reminds me a bit of old black and white footage of buildings imploding. At first these nuances were overwhelming to keep track of and to anticipate. But a lightbulb finally lit for me, conceptualizing that I need to be right next to Sasha, holding her hand at all times if she’s not seated. And so now things have become more straightforward.

Many weeks ago, while driving I caught a beautiful segment on NPR.   The discussion focused on the benefits (and setbacks) that can take place in this time of social isolation. Research is still young, with some schools of thought pushing for idleness and what can be learned there, while others suggest that being productive is paramount. A nursing student phoned in to discuss the emotional struggles she’s faced as this societal shift took hold. Her timeline of having just finished nursing school, a time where she found herself hyper focused on a sole goal (school’s successful completion; I recall those years so well!) to then preparing for the upcoming massive shift of entering the work force. But then, suddenly and dramatically, everything as she knew it came to a standstill, causing her great duress. She eloquently described how her identity felt suddenly and vastly so unknown, so unclear, freefalling, uncertain.

This era we’re in the midst of with Sasha has brought out the best in me and the worst in me. Seldom a day goes by where I don’t vacillate between anger and grief. I’ve become nearly fixated on physical activity; hiking, yoga, biking, jogging. Its an outlet for my worries and one filled with purpose; I must remain physically and mentally strong for Sasha, to remain competent in her care. I’m training for the unknown. I need to be ready for her ever shifting weight.

I’m sure many of you could identify with feeling like a sample size of one in those studies discussed on NPR that day. If posed the question to reflect on the benefits or challenges of idleness versus the comfort and sanity that often comes with being productive. I’ve learned that idleness helped me identify not only how to ask for help, but what would actually be helpful in this new chapter with Sasha. We’re house hunting yet again to find a home more suitable for the future. My Case Management team’s recently placed an ad seeking caregivers, and we have physical therapy consults coming up soon to address Sasha’s physical decline and to assist with customizing a lightweight wheelchair. I recognize that my identity has become fully immersed now as nurse and caregiver, but have started taking the steps to summon back all the other facets too.

 

“In separateness lies the world’s greatest misery; in compassion lies the world’s true strength..” Gautama Buddha

It was 13 years ago and I still vividly recall helping my daughter navigate through the dark, cavernous parking garage. Locating our black Subaru I hoisted her up into the back passenger seat. Buckling her in the car I beamed as I told her “I think we’re about to finally get some answers”.  Sasha was almost 9 years old and yet her diagnosis was still unknown.

As we headed home from Dartmouth Hitchcock Medical Center that afternoon after meeting with a new specialist, the very long list of rare diseases swirled through my mind. I was so optimistic we’d find out that this one, whichever one that she had, would be something treatable. I felt simply elated the entire drive home.  I remember convincing myself that some deficiency requiring vitamin injections was the likely culprit. I kept smiling back at Sasha in the rear view mirror. Our lives were about to dramatically change. It never entered the recesses of my mind that she might instead have something devastating.

I don’t know why, but for some reason this specific memory is the one that kept surfacing last year as I contemplated my elevator pitch for Rare Disease Week on Capitol Hill. In November, I’d responded to an email from the National MPS Society volunteering to advocate before my state’s Congressmen in February. It was an exciting opportunity given my love of politics (being my first college major) and rare disease advocacy. It was equally exciting as a Mom.

I have so many reflections from the special series of days spent in Washington, DC. So much was learned, with the most educational experience stemming from the two other MPS Moms I’d met from New Hampshire. However these Moms children did not have MPS III, but instead have MPS I. It was so unique being able to hear their perspective about a disease similar to Sasha’s and yet also very different. I listened intently while they shared personal insights of the hurdles that still remain for this MPS Syndrome. They shared with our state’s health advisory staff alongside sharing with me.

MPS I, Hurler-Scheie Syndrome, if diagnosed very early, does have treatments. This is not a simple intervention however, and before the process can even begin a bone marrow transplant must occur. I marveled at what these families must contemplate and endure when their child is deemed a candidate. As with any condition requiring bone marrow transplant, the immune system must first be suppressed for acceptance of foreign matter to take place. The sacrifices and fears a family confront when approaching such treatments are not easy ones.

When I spent those remarkable days with these two MPS I Moms, they shared with me these stories, and the steps required for their children to eventually receive enzyme replacement therapy. They shared some of those same stories during our meetings on Capitol Hill. And for some reason, specifically the stories of clinical isolation, resonated with me. A lengthy isolation period must take place to protect a bone marrow recipient from viral and bacterial pathogens. These isolation periods can last months to well over a year. They often take place in a hospital room where there is no familiarity in terms of routines, people, food or environment. Couple that with a most worrisome time facing tremendous medical risks and consequences.

Little did I know when I was so moved by their stories that the pandemic of COVID-19 was soon set to explode. Today, our nation and our world has a similar, universal sense of concern. For many there seems to be a teeter-tottering of emotions, vacillating between “this is absolutely ridiculous” to “this is absolutely terrifying”.

These are trying times.

When I reflect with fear at the thought of Sasha potentially contracting COVID-19 I then think of those who already have a positive test result. I think of those two Moms who went such great lengths for their children. I am reminded that what is being asked of us is not insurmountable. And maybe now I know why that parking garage story kept replaying, over and over again in my mind. Because I again find myself thinking, with such high hopes, that maybe we will find an answer to this thing.

 

I was lucky to attend two Rare Disease Genetic conferences near the end of last year. Some of you are yawning, I can almost see it! But try to stay with me and hear me out, as these were deeply compelling experiences. So much came from both conferences in terms of those speaking at the podium to the equally amazing individuals in attendance I was so fortunate to meet. I may have to write about these layered experiences in installments as they were vast and ran deep.

The topic of this fall’s conference was Improving Health Care Experiences in the Rare Disease Community and I love that chosen title. It serves as a gentle reminder that while the list of rare diseases is vast, individually we can feel like and are, a healthcare minority. There’s nuances to Sanfilippo alone that I’m still learning, and I am fortunate to have a community of resources through social media and long standing friendships for vetting inquiries. But what if you were a health care worker who has never seen a particular syndrome? There is no way to know the myriad of factors that can take even a parent, fully invested 24/7, years to understand. So what if you were to treat dozens of rare diseases over the course of your profession that you had never seen before.  Double that with the limited resources of practicing somewhere rural and one can see the benefit of these conferences.

Both events, one in October and one in December, were well rounded, and I did not overlook the fact that the Executive Directors of both non-profits hosting these events were Registered Nurses. Inspiring! Attendees enjoyed an interactive session with a Rare Disease Legislation Advocate who imparted education on how to lobby locally and beyond for rare disease legislation. Then came the medical heavies – geneticists speaking to telehealth and the potentially risky uptick in “DIY” genetic testing. I learned so much from the geneticist giving a deep dive into the phases of clinical trials and from the attorney who, as a single Mom to a daughter with a rare disease, chose to create the advocacy resource group she felt was much needed in her home state. She spoke to the early years of her child’s diagnosis when she had so little to work with and so few were able to help her navigate an already overly demanded system. You could hear the air leave the room when she shared with us that her daughter had passed away since her last public speaking engagement.  I remain awe struck by the continuous examples I see of parents who do not close the door to their mission even when the unfathomable occurs.

And then, something happened. For those who may recall my Simple Sketch blog, a similar episode unfolded when a research geneticist came to the podium and prepared his slides, with the cover image being a painting that I know well. Andrew Wyeth’s Christina’s World. Some members in my family particularly love this painting, and one of my brothers has a large print framed in his living room. We see a dark haired young adult, perhaps a child, sitting in a pale pink shirt dress in a seemingly endless field of muted grass. She is looking off in the distance at a large, almost gloomy yet strikingly beautiful home at the edge of the field.

After learning about thismost interesting scientist speaking at the podium, how he studies mutations in mice and can be commissioned to create mutations for private study while working from his beautiful lab overlooking the ocean in Maine, he came back to the image of Christina’s World. He asked the audience what we saw. How old was this girl? Was this her own home she was looking at from afar? Is she healthy, so to speak? He explained that little was known about this image, but it was thought that Christina was the neighbor to the painter Wyeth. As the story goes he would see her crawling through the endless grass from her homestead to, do what exactly? Escape? Feel freedom? Experience autonomy? To simply…breathe?

It was thought for years by Art Historians that Christina suffered from polio, and in recent years it has been suggested that instead, she had the genetic condition Charcot–Marie–Tooth disease. This is a degenerative condition where the muscles, most often in the arms and legs, weaken as a form of motor and sensory neuropathy takes hold. I reflected on Christina, wondering how long, in terms of time, was her journey to this destination? Where was her family? Did she have caregivers? Did she attend school?  It has been said that Wyeth would watch her from his own window next door and likely wondered some of these questions too. It struck me.

On New Years morning, at my insistence, Mike and I always write our goals for the upcoming year. I then take our lists and fold them into the tiniest bits of paper and stuff them far back in some random desk drawer and pusporsely forget all about them. We then pull the lists out and unfold them one year later, marveling at how often each goal comes true.

This year on New Years Morning, surprising even myself, I posed a similar yet different question. Instead of asking about goals and objectives for 2020 I simply asked, “what are your hopes and dreams”? Mike looked at me like I had two heads. We are so goal and task oriented in this culture that we often stop thinking this way once we exit our teens or twenties. “What are your hopes and dreams”? We had no clue. We both sat there dumfounded, our heads spinning. We still joke about that moment but continue to ponder the question both in jest and in sincerity.

In that same vein, I keep thinking about Christina…what were her hopes and dreams in the moment of this painting?  And then of course I think of Sasha. What are hers?  We all have them, and its inspiring when art and science merge to remind us why.

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• To read a bit more about Christina’s World you can click here: https://www.moma.org/collection/works/78455

“If we could see the miracle in a single flower clearly, our whole life would change”. Buddha

Its hard to believe that three months have passed since I stopped working in order to be at home with Sasha. In special education here in New Hampshire, on a student’s 21^st birthday they “age out” of school and are officially done. This can seem sudden, and so I’ve always been grateful that Sasha has a summer birthday. And while I’ve had plenty of time to prepare for this new era, its still been a huge adjustment for both of us. (I feel like I say that often–“I had plenty of time to prepare, but…!”).   2020 I’m looking at you!

This summer was a whirlwind as it included Sasha’s birthday, two end of school year parties (one in June when the entire population was present and one in July on her official last day of summer school), a getaway to celebrate Sasha becoming 21 (such a milestone!) and of course the Sunrise Ascent on Mt. Washington and all its training, organizing and fundraising. All of this coupled with my Dad’s passing made it so there was little time to dwell on what I had been down about leading up to in months prior. Sasha has always loved school and I was sad that her lifestyle would change so drastically.

We are fortunate that in the state of New Hampshire there are options in the models provided for families of adults with special needs. After much consideration (and changing my mind back and forth as my regional service agency can attest to!) we decided it would be ideal to start out with me being Sasha’s “staff” to help establish this new chapter. This means that I run her day program and oversee her activities and scheduling along with being her actual staff member. Our days include a scheduled wake up time (at my insistence) along with Adaptive Sports (hiking right now which will switch over to alpine skiing soon) a weekly social and wellness hour also run by Adaptive Sports in tandem with the Boys and Girls Club, equine physical therapy and visits with friends, former colleagues and her former school aide who visits each week. In between we’re hitting up our favorite coffee spots, or taking small road trips to Target (which used to be distance prohibitive).

On my end, while no longer working, I remain engaged with personal and professional interests by being a Board Member of two organizations (Adaptive Sports Partners of the North Country and New England Regional Genetics Group) while taking a chemistry class at home. This keeps me aligned with my ever present (though perhaps not too realistic) goal of attending grad school. And I do get respite, so I’m still able to go for weekly hikes which is so important to me!

And while our days have balance, I am quickly seeing how much Sasha misses school. Her days were much fuller being out of the house from 8:00 to 3:00, along with the benefit of being independent of me during those hours. Sasha saw more people, had a routine and had more purpose. Lately I’m observing a lowering in her mood and overall demeanor, and so I’m taking note.  In the long run we may determine its best that someone else staff her day program as opposed to me. My state of mind has changed too, with it varying from feeling much more relaxed to a slight panic setting in, almost a feeling of dread, induced by not having the schedule, structure and purpose offered by working in my given field.

Yet I am grateful that we had a summer to initiate the closing of one door as we opened the next. At Sasha’s (final) end of the school year party, I was overwhelmed by the outpouring of love and affection from her school staff, her current and former caregivers, her bus driver and her friends. Her school physical therapist who has worked with Sasha for many years, was able to attend that last party and was so sweet as she knelt down beside Sasha’s chair to present her with the most beautiful pot of Gerbera daisies. She then gently initiated one last PT session. It was a beautiful day filled with emotion.

As we drove home, the daisies in my lap felt particularly poignant to me. In being one of my favorite flowers with its vibrant colors and purity in their simplicity of shape, I felt it mirroring Sasha in many ways. It was also an ironic choice, as it has been one of the few flowers I’ve never had a green thumb with, and so I was determined to find a way to make this one thrive.

Two days later we took off for our much-anticipated New England road trip to celebrate Sasha turning 21, with our final destination being the aquarium and ocean in Mystic, CT. When we returned home, I was shocked to see that our fuchsia and tangerine daisies that were doing so well before we left had completely died. I tried a few things over the course of that week to no avail. I was so sad as this was a special gift and once again, it was also becoming my nemesis flower! As a last ditch effort I decided to change its placement, offer it scant water as I’d read to do and instead of hovering, I simply let it be.

To my amazement, a week later our daisy was in full bloom. Its shape and colors were slightly different, no longer as perfect as it once was, but it was truly alive and well. I marveled at this moment and took it as the lesson I believe was intended. For me to do the same in this new chapter with Sasha. To see where this path takes us and with gentle reminders, to try and let it be. We will find our way, make adjustments as needed, and find the right balance in order to flourish and succeed.

“These are days you’ll remember
Never before and never since
I promise
Will the whole world be warm as this”  – Natalie Merchant

Some of you may recall April of 2015, when barely a year into my new nursing career I’d truly had it with northern New Hampshire’s long winters.  And so it was then, on a lark, when I applied for a nursing position at Duke University Hospital.  I was both stunned and elated (and quite frankly frightened) when I received a response and was suddenly on a plane to Durham NC for a job interview.  I felt like it went well as I answered critical thinking and clinical questions and integrated with the nursing staff on the units as I shadowed for the afternoon.  When I later sat in an enormous conference room with floor to ceiling windows overlooking the city below and my recruiter suggesting I not unpack my bags upon return to New Hampshire but instead, keep on packing, I had a good feeling.

Upon accepting the job that June, there was almost a two month wait before I was to relocate.  This gave me time to study, visit with family and friends and research the area’s special needs resources. What I didn’t have time to prepare for was the sense of loss I was feeling as I prepared to loosen the ties of a brand-new found friendship with the incredible organization Adaptive Sports Partners of the North Country.

To backtrack a bit further, during the last semester of nursing school a dear friend of mine checked in on how Sasha was doing.  As we chatted, she asked if I had ever engaged in any outdoor activities with Sasha.  I explained why that tended to be challenging, but given Sasha’s boundless energy at the time, I knew it could be a wonderful thing to offer.  My friend had a sibling involved with Adaptive Sports Alpine Skiing program and so was able to provide detailed information about how amazing this group was.  I told her after nursing school wrapped up I’d be looking into this.

And so as my winter doldrums overtook me that April back in 2015, I was just a few months into getting to know Sandy Olney, the passionate Executive Director of ASPNC.   She assessed Sasha thoroughly and started us on some hiking experiences.  But it was the months leading up to the detailed preparations for our first Sunrise Ascent on Mt. Washington that truly changed the trajectory of life as I knew it with Sasha.  I started meeting the most amazing people, all volunteers with ASPNC, and witnessed the commitment level of this organization. I saw sights with Sasha I never dreamed possible, sights that simply would not be available to us without human horse power, very specialized equipment and equally special people.  Every time I returned home from an adaptive adventure, the 10,000 Maniacs song “These are Days” would start playing in my mind.  I was  hooked, and felt such sadness in knowing that the very day after our first Sunrise Ascent on Mt. Washington, I would then get into a car to drive to Durham NC to start my new job that next week.

For many reasons,  in knowing how much I would miss my family, my friends and New England, 10 days before my departure I realized I couldn’t do it.  Or that I wouldn’t do it.  A huge part of that decision was in wondering if I would meet up with an organization quite like ASPNC ever again.  These dozens and dozens of amazing volunteers, interns and staff had become extended family to me.  My new job was going to be intense with full time training for months before I switched over to a schedule of  “three twelves” as we say in nursing.  I knew it would take time to settle in, and I wasn’t sure when and if I’d resume these kinds of adventures with Sasha ever again.

Recently, at a routine neurology appointment at Dartmouth Hitchcock, Sasha’s beloved neurologist (who practiced at Duke prior to his position in New Hampshire), asked if I’d regretted that decision.  I told him that a small part of me always would.  I still struggle with the long winters of northern New England and at times with the desolation of living so rural.  I know deep down I would have done well at Duke and would have had infinite opportunities to progress both professionally and academically.  And my Dad, who held education and opportunities like these in the highest regard, was disappointed when I changed my mind.  But this past Sunday, during our fifth attempt at the Sunrise Ascent on Mt. Washington (read about previous ascents and attempts in my August ’18 Chasing the Sun post) I remembered why we stayed.

Two years in a row now I’ve experienced the sheer joy of climbing the highest peak in the Northeast with Sasha.  who was full of smiles and wonderment the final 2 miles as the weather and landscape suddenly changes with its elevation., She  engaged in friendly competition with the other mules and participants (and visibly does not like getting passed by another team).  As is true to ASPNC, this experience was done with incredible equipment and even more incredible individuals.  Each year our team sees previous “mules” return to us and countless new ones jump on board. And this 2019 Sunrise Ascent was extra special for many reasons.  It was the 10th annual and also the first Sunrise Ascent with our new Director, Thomas.  We are so fortunate that in Sandy’s recent retirement, someone else who is passionate and committed, and wanting to do right by Sandy and by our community, has taken the reigns.

It was also an emotional day as the sole team who created this special event 9 years ago continues to be present with their loved one for the climb.  And so this marked the tenth ascent for a special man afflicted with the same condition as my Father’s.  I reflected on the sorrow I’ve carried in wishing I’d taken Dad on an Adaptive journey,  but was deeply comforted when I encountered a former Team Sasha mule, who couldn’t join our team this year as she had created one of her own.  As we chatted on Sunday, I learned that her own Dad had been given just a year to live in the winter of 2018, and had promised her that if he surpassed this prediction, her would allow her to bring him up Mt. Washington.  It was like a circle in some ways had been closed in seeing someone else do for their Father what I had hoped to do for mine.

And finally, when Team Sasha’s name was announced at the Awards Ceremony as top fundraising team, something I’ve always dreamed we could accomplish, more reasons for why we stayed began to surface.  I suddenly heard Natalie belting it out: “These days you might feel a shaft of light make its way across your face, and when you do, you’ll know how it was meant to be”.  I hope that Dad knows he was a huge reason as for why I stayed too, and that we will continue to climb mountains for him, in memory of the mountain he climbed so full of grace.

 

 

This summer will be  a period of transition, as I knew it would be with Sasha aging out of the public school system this coming July.  And while I had much time to contemplate the eventual reality of other shifts to the family dynamic, I still found myself ill prepared for the recent loss of my Father, who passed early Saturday morning, June 8th.

As I ponder these new phases, which I’ll share more with you in the coming weeks, I thought I’d first offer some sentiments about my Father as I survey this new chapter in parenting Sasha.

Below is a link to my Dad’s obituary as well as the words I spoke at St. George’s Greek Catherdral yesterday morning.  I want to thank Father Mike Wilson for providing my brothers and I both the opportunity to speak as well as such compassionate support and genuine kindness during this time.

http://www.tributes.com/obituary/read/William-J.-Makris-107178187?fbclid=IwAR2Cmxg-WR2vHT8AwfV3BtbCz9d2BeTX3g0u8cP9CNBXQ2Fdf5R5kpffKoA

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Good Morning. Thank you all so much for coming today.  I realize that we’re all still in the early days of navigating this loss, but for me at present loosing someone like my Dad feels mostly like a ship that’s anchor has been lifted while also being informed at the same time that the ship has no captain.  And yet if our parents do a good job, they provide us with the tools to navigate this life on our own, with strength, knowledge and independence, which I know my Father (and my Mother) have done.

Still, having someone like my Father around was very reassuring.  He was a quiet leader in that he wasn’t one to offer direct advice yet you always knew what he felt the prudent decision was.  He was stoic but ever since I was a little girl, I considered him to be such a dynamic individual.

Dad had an interest in so many things.  The New Yorker Magazine, US and World History, politics and volunteering for campaigns, researching products in Consumer Reports Magazine and driving the Kancamagus Highway. He loved The Tour De France and German Automobiles, LL Bean and the Atlantic ocean,  New England sports teams, Fritz Wetherbee,  A Prairie Home Companion, and 1965 Mustang Fastbacks.

Throughout these past several weeks I’ve of course reminisced on so many memories with our Dad.  Visiting the LL Bean flagship store in Freeport Maine, driving the hair pin turn on the Kancamagus highway (which he would explain to me was one of the very few times you won’t gas it going into a turn). Studying audio products in stores like Lechmere’s and Service Merchandise – with me as a young child never understanding the concept of entering a store and leaving empty handed.  When on longer road trips he used to quiz me on recognizing out of state license plates.  Being more of an adventurous personality he would buy me gifts such as Archery sets, cross country skis and 20 gear racing bikes.  (I’m not sure what he expected of me there).  We’d grill steaks at the ocean and then catch an air show at Pease Airforce Base or go to the movies on dreary days.  Every Valentine’s Day he would buy me a box of chocolates and for as long as I can remember up until he could no longer speak he called me sweetheart.

But more than anything I so enjoyed Dad’s quick wit.  I will never forget the time about 12 years ago when I excitedly told him we were getting our first puppy.  At the time my daughter was quite the handful, and upon hearing this news Dad looked around at no one in particular and said “A puppy?!  Why not get her a baby alligator and give it a fighting chance!”.  There was also the first visit with Dad after my daughter was born, when I asked what he’d like to be called.  “Pappous? “I offered.  “Grampie or Grampa?” I suggested.  “Maybe Gramps?”.  He looked at me slightly annoyed and simply responded “How about Bill”.

I think Dad would be pleased to know that I have a genuine interest in almost everything I’ve mentioned above (with the exception of the Tour de France, to be honest there is only so much one can watch of the chase for the yellow jersey).  My husband Mike can attest to my researching a vaccum cleaner purchase for nearly 9 months and there are ongoing jokes at my place of work about my Fritz Wetherbee fascination.

As Dad’s disease progressed, while none of us around him ever dwelled on it, it did take from him the ability to engage with so many of these interests.  On my last visit at the VA just before the approach of his final descent, a beautiful spring day had finally bloomed. I walked with Dad through the flower gardens and told him what each flower was.  I mentioned how there were clearly many more varieties yet to bloom and that we could come back and enjoy them throughout this spring. We then meandered to the upper parking lot where we stopped to look high above us at the state seal, US and military flags.  Dad was clearly moved.  I told him that next time I came down I’d bring my dog Stoli for the visit and sneak in some Metaxa (Greek Spirits).  (For the record I haven’t done so in many years).  Dad seemed pleased and I was looking forward to this last chapter with him.

Unfortunately that last chapter was cut a bit short.  But Dad provided his most everlasting life lesson in this difficult time.  He showed me that we can make plans but that they might not always pan out.  He lived that most teachable moment every single day for 25 years.   I hope he knows how strong this made us as his children, that the ships anchor will one day again be lowered and that we are forever grateful for his example.

 

 

 

 

 

“Awareness is like the sun. When it shines on things, they are transformed.”
― Thich Nhat Hanh

Without any intention to offend, I’m going to be frank. In general (and increasingly so in recent years), I’ve not always been the biggest fan of “(insert cause here)— day”. Partly this is because the “market”, for lack of a better word, is getting oversaturated. Think about it for a moment, we have national food days (avocados and donuts…I love you both but  – really)? National pet days (I feel like I should love my dog with the same verve and authenticity every single day, right)? And if you don’t believe me, you can check out these 1500+ (insert your cause here) days at this website: https://nationaldaycalendar.com/. Woah.

But there are deeper reasons too for why I sometimes balk at awareness/notoriety days. They may unintentionally trivialize something that is strongly important to a given sect of society, creating a brief flare up of interest only for it to then fizzle out for the next 364 days. The resulting tone can feel superficial. Additionally, emotional videos, photos and editorials may be blasted throughout social media, making some individuals feel uncomfortable or worse, triggering a loss from long ago or very recent.

And so, with International MPS Awareness Day fast approaching on May 15^th, I’m having the same inner debate that I always have this time of year. Do I make a call out for purple?  Do I write a blog post?

But there is a strong list of reasons to celebrate MPS Awareness Day, for which I’ve decided to compile a list.  Here it is:

1. The Great Unifier: As parents we all have differing philosophies on raising our kids and parents of MPS children are no exception. Yet on this specific day, we are all bound together by what is most important, the health and wellbeing of our children, as well as our sincere reflections in memoriam.
2. Acknowledgement – It’s a wonderful feeling for a parent when your child gets acknowledged for being special; perhaps their team won a state championship, or your child made the honor roll. For an MPS parent, seeing a sea of vibrant purple proudly worn at schools and in workplaces can evoke a similar pride.
3. Education – due to the phenomenon that is social media, information is being shared at a rapid, (almost frantic) speed. This can be a great thing. It can also be a challenging thing. Sometimes people will glean a bit of a headline and run with it, leaving parts of the story behind. This is especially true in science and medicine and is no different for the parent of a child with MPS. International MPS Awareness Day brings about the chance to have conversations. In recent years, for example, I’ve been asked “what is this comparison I’m seeing between Sanfilippo Syndrome and Alzheimer’s Disease?” (Answer: the outward presentation of SF is quite similar to the stages of regression seen in AD, however the pathophysiology is quite different). Another question I’ve received: “Is MPS and Sanfilippo Syndrome one and the same?” (Answer: Sanfilippo Syndrome is just one of many forms of MPS, here is a complete list):

• Hurler/Scheie syndrome , MPS I H (Hurler disease), MPS II-(Hunter syndrome), MPS III A, B, C, and D (Sanfillipo syndrome), MPS I S (Scheie syndrome), MPS IV A and B (Morquio syndrome), MPS IX (hyaluronidase deficiency), MPS VII (Sly syndrome), MPS VI (Maroteaux-Lamy syndrome)

4. Outreach: This day can forge new family-to-family, peer-to-peer and professional- to professional connections or any combination there within. Through such a concentrated flurry of videos, posts and articles on social media and in the news, new connections can be made.
5. Volunteerism: a day such as this one can generate a tremendous movement of good will. Sasha has personally experienced entire classes of schoolmates dressed in purple, enormous purple banners being made for awareness photos, purple ribbons being sold at the front desk of her high school, newspaper articles featuring her story and purple teams of mules pulling her up Mt. Washington.  I myself have witnessed friends (and the children of friends) sending messages and photos dressed in purple, or watched a striking video of my own yoga teacher in her studio dressed in purple, doing a beautiful silent yoga sequence in honor of this day.  I have witnessed the coworkers of friends, people whom I have never met, join in to wear purple while all sending well wishes together in videos.  Awareness Days at their finest can act as a spring board to generate an interest in a cause and volunteering that continues for a lifetime.
6. Marking of Time: For many MPS families, the 15^th of May is a day that has become sacred. Parents might find themselves mentally revisiting their diagnosis journey, reaching out to other MPS families to see how they’ve been faring or posting updates on their CaringBridge pages and Blogs (shameless plug!)
7. Financial support: Again, due to this influx of heightened interest, non-profits such as the National MPS Society, Ben’s Dream and the Purple Lemonade Stand for Courage and many others may see a spike in sponsorships and donations. Since MPS is what’s referred to as an “orphan disease” (ones that are so rare the result is less federal funding in research dollars than for more common conditions) this makes it especially important.
8. Aiding in Diagnostics: as hard as this is to ponder, there are stories of parents of not yet diagnosed MPS children who, upon seeing a photograph or reading an article related to MPS, then realized this might very well be their own reality. MPS Awareness Day, by raising visibility, can also assist in diagnosis.

These are just a few of the reasons to consider wearing purple on MPS Awareness Day on Wednesday, May 15^th.  Each reason listed is incredibly important and stands alone on its own merit. But perhaps above all, the love that is felt through the purple gestures made by friends and families across the globe, the photos that are shared, montages that are made and stories that are written might be the biggest reason of all. Thanks so much for reading today and if you have a reason that you’d like to share as to why you honor MPS Awareness Day, please post it in my comments section! And thanks for getting ready to bring out your purple!

 

“O flock of heavenly cranes…cover my child with your wings.”

I am often asked how it is that I remain positive in what many consider to be the midst of sadness that is Sanfilippo Syndrome.  People comment that I am “always happy” (which I often am). When I’m not happy on a given day or in a given moment, I rebound quickly. For me, a poor mood won’t last even an hour. Yet my answer to that question, “how is it that you are always so happy” is almost always the same (and is also true). “When Sasha is OK, I’m OK”.

I’ve been such a fortunate parent in this utterly unfair world of Sanfilippo Syndrome, and I reflect on that daily.  Sasha’s stability has included unusually long periods of plateaus.  Her pattern is often this; Sasha tends to have the slightest shift in a given function, weather that be swallowing changes, mobility decline or memory loss, and at first it merely cracks open a door only to then shut that door instantly.  Then (thankfully), the functional decline is not seen again for quite some time.  But when it is seen next, that door remains ajar, the frequency of events is now sprinkled throughout a given week, here or there.   It may subside for many days but returns again soon enough.  “New normal” has now arrived.

It is when that door first just barely cracks open that I am (mentally speaking) paralyzed.  The noises of the world around me start to whir as I suddenly realize that I have taken everything, everything, for granted.  Slight panic sets in.  I mentally review her list of doctors and determine who  would be best to touch base with.  I scrutinize environments, wanting to assign responsibility elsewhere, to try and gain some control. There must be something that I can alter to then “fix” this latest symptom.   In short, I am no longer OK because Sasha is no longer Ok. She’s shifting.  She’s not the same.

And so this week I’ve had to reflect on the notion that Sasha’s walking is simply not the same.  She trips through thresholds, she slips on smooth surfaces, she falls when someone is right next to her, holding her hand to support her.  Her leg strength gives out, her vision fails her, her depth perception can’t be trusted, her coordination is delayed.   It can’t be pinned on one thing.  I can’t solely improve the lighting, or remove all of the thresholds, or put anti-skid socks on Sasha and have these falls simply go away.  I can do all of those things listed  to mitigate these events, but I can’t take away Sanfilippo Syndrome for her.

In the days leading up to this post I guess you could say that I wasn’t really all that OK.  I was sad and fearful for Sasha’s future.  I so wanted the folklore of my beloved origami to be true, that by folding 1,000 cranes,  wishes could be granted.  That as I took in images of flocks of cranes, their symbolism of longevity would be honored.  I don’t know if such a mythical leap can be made, but what I do know is that I can continue to reflect on what the Japanese consider to be a bird of happiness, and hope that those reflections aide in shutting a door to allow a return back to a prior normal.