Theme: Advance Care Planning
This event explores how completing healthcare advance directives for/with your child can support your family’s goals and wishes. Fiona Sampey and Amy Vance of Advanced Conversations share the importance of discussing and documenting healthcare wishes upstream of an emergent/urgent medical event, medical implications of and quality-of-life considerations related to advance directives, and currently available directives.
Theme: Advance Care Planning
Eighteen years ago, my world was turned upside down when my daughter, Waverly, was diagnosed with Sanfilippo Syndrome. One month later, my son, Oliver, was given the same diagnosis. My family was living in London, embarking on a new career adventure when everything we had planned was no more.
My husband and I felt an extreme need to be back stateside to settle down, make a home, and build structure for the chaos we found ourselves now living. We were in a position where I could forgo my career to become the primary caregiver, devoting my time to the daily tasks of mothering two children with complex medical care needs. My husband worked to provide our family with insurance and financial stability. Most therapy appointments, doctor and equipment clinic visits, Medicaid applications, insurance appeals, and school forms were my responsibility.
In all the literature we read and conversations we had with other families, we were naïve about the impact severe neurological impairment would have on the children’s body systems as they neared the end of their lives. My husband and I made countless difficult decisions about their care. However, the most painful and gut-wrenching decisions revolved around feeding.
We opted for a G-tube when Waverly was 8 years old. She had been having trouble swallowing for several years. We thickened and puréed, but after some choking episodes, we had a g-tube placed. I took photos of her perfect belly and wept knowing our decisions were causing her to undergo surgery and pain. As parents, we weigh the options and make the best decision we can with the information we have at the moment. We constantly ask ourselves, “What is the ‘right’ decision?”
We continued with pleasure feeds, allowing Waverly to taste her favorite foods. We watched for signs of distress around eating, like squirming and crying. We noted signs of pain as we started a feed and then relief when we stopped. We would decrease rates and reduce volumes to maintain comfort. She began having more diarrhea and vomiting, spitting up formula during feeds. Waverly was experiencing edema throughout her body. We consulted with her team and made recommended changes, but her system continued to decline.
We had difficult conversations with providers we trusted. We weighed their guidance with our philosophy and ethics. I relied on my mother’s intuition, but Waverly was our guide. We followed her lead. Waverly was signaling to us that her body was tired.
It took time to see that feeding her was causing her pain. We charted every feed, how many milliliters and over what period. We noted any movement or grimace that we recognized as pain. We stopped and started, gave breaks, and switched to Pedialyte. We dripped tiny amounts of food into her belly only to stop as her body showed signs of distress. My husband and I both needed to agree that it was time to stop feeding.
We transitioned into a hospice approach focused on comfort. We had time to invite loved ones into our home to spend time with her, continue to make memories and build her legacy. She died in our bed days after she turned 12 years old.
I spent a significant amount of time perseverating on our decision to stop feeding her. I would be rendered inconsolable, and overwhelmed with guilt. My husband would walk me through the events, logically laying out the cause and effects, and why we came to our decision. Repeatedly organizing the facts allowed my emotional responses to quell. I needed that framework to assuage any guilt and confirm the decisions were made to alleviate the suffering of our daughter.
And we knew we had to do it all over again with Oliver.
We were practiced. Waverly once again smoothed the path for her little brother. We noticed the signs earlier and knew what adjustments to make. Our medical team was also more versed having navigated Waverly’s decline with us just three years prior. The decisions came quicker and with a tinge less doubt. Oliver died a few weeks before his twelfth birthday.
My guilt was lessened with Oliver’s death. I felt more confident in our decisions. I also felt more supported by all of those around me. My expertise was validated by Oliver’s care team.
As parents of children with rare diseases, we are often the experts in the room, explaining the nuances of our children to those more credentialed than we are. We bring binders and share literature to inform those caring for our children. We were fortunate to have a medical team who invited us to have a seat at the table for all decisions. And if they weren’t open to that, we found another provider who was willing to say, “I don’t know” and “let me consult with others”.
Having abandoned a career and spent fifteen years as a full-time caregiver, I wanted to build upon my children’s legacy. I wanted to work in a field supporting children like mine. I returned to graduate school and became a pediatric social worker and grief counselor. I work for a community-based hospice and palliative care agency in Washington, DC.
As a pediatric hospice and palliative care social worker, I support families as they navigate complex decisions. I see my role as ensuring full parental participation and advocating for the parents’ goals of care. I am in their homes, supporting the entire family system, building trust and rapport, ideally early on so a relationship is established as decisions become more complex.
I have utilized the Courageous Parents Network both as a parent and as a professional. The educational videos were especially helpful in normalizing my experience as a mother. And I found it validating to hear others vocalize my thoughts and feelings. As a social worker, I return to the same videos with the families I support, hoping for a similar outcome.
The new NeuroJourney tool is invaluable. I wish I had access to this when I was parenting my children. However, I am thrilled to have this instrument available to my patients and their caregivers. I have introduced several families to the website, exploring the impact severe neurological impairment has on their child’s body systems. The website provides information and tools to parents and providers.
I have found the GI section especially helpful as it addresses a caregiver’s innate need to nourish their child. It provides a guidebook for one of the most challenging decision points. By using this tool, I have watched families recognize the signs of feeding intolerance and decline in their children. It opened the door to an honest conversation about prioritizing comfort and adjusting goals of care.
My hope, as both a bereaved parent who held onto guilt and as a therapist who supports caregivers experiencing guilt, is that this tool can aid in adjusting to changes and navigating decisions. This information has the potential to improve caregivers’ confidence in their choices by providing clinical data and reducing the potential for a more complicated grief process.
Theme: Advance Care Planning
Part of our role as parents is to protect our children while slowly teaching them how to protect themselves. When they are toddlers, we hold their hand in parking lots, knowing they don’t have the knowledge they need to navigate the danger on their own. But when they’re teenagers, we trust them to walk alongside us, navigating the danger on their own. A chosen few of us are called to parent our way through procedure rooms just the same.
My daughter, Izzy, was three years old when she was diagnosed with cancer. In those early days, I took the lead, telling her what was wrong and what we were going to do about it: “So, once they take the rock out of my tummy it won’t hurt anymore?” Over ten years and six relapses our conversation evolved into one she had a voice in: “What will happen to my body after I die.” That evolution didn’t happen in one moment but in a million tiny moments combined.
Izzy’s cancer would never stay away long. So for us, treatment became the only way Izzy could stay alive. At the age of ten, she started asking to have a say in what treatments we did. For years, decisions had been made about her body without her consent and she simply wanted to be part of the conversation: “You talk about me like I’m not even sitting right here.” Her medical team agreed it was time, and soon Izzy began participating in the conversations we had every three months after scans.
At each appointment our doctor would give us the results and tell us what he thought was best. Then ask Izzy if she had any questions. She always did and they were always pretty much the same: “Is there an option where I can just go to school and not do treatment?” The truth is, she knew the answer. But her ability to ask the question gave her what she needed to process the answer. “They say I have options but what they really mean is I can do treatment, or I can die. And that doesn’t seem like much of a choice.” But in the end, it was a choice. And it was the choice she made.
The last few years of Izzy’s life the disease ravaged her bones, often making her unable to walk. While treatment always destroyed the disease in her, after so much toxicity it began destroying all of her. This meant ‘good days’ were a thing of the past. When we weren’t in the hospital for treatment, we were in the hospital controlling the damage of treatment. It suddenly became hard to assess what the real ‘danger’ was. Izzy said: “Fighting cancer your whole life isn’t the same as living.” She was right and together we began to wonder what death could be for her.
In January of 2021, we made the decision to stop treatment. To leave the hospital and go live life the way she wanted before she died. Izzy walked towards death with curiosity and hope, believing it could offer her the one thing this life no longer could: freedom.
For most of Izzy’s life I fought to keep her here. Holding her hand and teaching her to identify danger and navigate it safely. But in the end, she let go, teaching me that danger isn’t always the thing we think it is. Teaching us all that having to fight to stay alive is not the same as living.
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Molly is a Writer, Speaker, and End of Life and Grief Coach in the Indianapolis area. She spent a decade in Pastoral Care before spending the next decade caring for her daughter throughout her cancer journey. She has combined both experiences to help others navigate unimaginable journeys. You can find her writings and teachings on IG at: the.grief.writer, or online at: mollymattockscoaching.com.
Theme: Advance Care Planning
Theme: Advance Care Planning
Theme: Advance Care Planning
The mom of a baby with Krabbe disease recounts her son’s end of life. It was anticipated and peaceful, and yet ‘it was a total shock.’
Theme: Advance Care Planning
The mom of a baby with Krabbe describes the way she considered her decision-making in different emergency situations (related to his breathing), and working with the EMTs. “It was like being in a sadistic game show. Which option do I pick?” As his condition progressed, her decisions changed. (This video is complicated but clear.)
Theme: Advance Care Planning
The mom of a boy born very prematurely, who lived with serious medical complexities for 14 years, speaks so tenderly about how she had to really listen and watch to understand her son’s wishes for himself which meant his end of life. “I was like, okay, yeah, he’s done with this. And we’re done with this. You get to that point where it’s like, I’m not going to allow him to suffer any more than he has to. Because as much as I wanted him to stay there on life support that wasn’t what he wanted. … We will never be ready.”
Theme: Advance Care Planning
The parents of a son with a very rare genetic condition recount an experience with intubation that was extremely painful but also incredibly informative as they faced the final days of their son’s life and chose to put his comfort and peace first when they made the decision not to re-intubate him.
Theme: Advance Care Planning
Dr. Scot Bateman, now a PICU MD, wrote this poem as a 2nd year resident. If ever we need evidence of how providers can be open to and touched by their patients, here it is. If ever we need evidence of how impactful honesty — delivered gently and lovingly — is to the care that children and parents seek, here it is. If ever we need evidence of the courage of children AND clinicians in the face of the limits of modern medicine, here it is.
Thank you Scot for writing this beautiful tribute to Emily, and to letting us share it here on CPN these many years later.
***
A Death Smile
I told Emily a joke every day I knew her
A dying child is still a kid
Who can and wants to smile
An 11 yr old with relapsed T cell lymphoma
Bald, steroid chubby
Bed bound from weakness
Innocent and open
Loved and loving
Radiating hope
And trust in me, a second year resident
Admitted as a “last ditch”
Racking her body with one more round
Of energy sapping chemicals
Hoping
Day after day, I try to treat
Expected and unexpected complications
Learning as much as I can
About medicines and side effects
About central lines and infections
About tumor cells and growth
Emily knows and appreciates this
As much as every new joke
But the tumor ravages on
Nothing seems to be working
Her pain is real
She knows that I see this
The jokes are for her
“Why do Gorillas have big nostrils?”
Palpable anticipation of her laughing is fun
“Because they have big fingers”
Her laugh is hearty and genuine and infectious
But the jokes are for me too
She gets equal pleasure seeing me smile
It metastases to her spine.
Morphine drip becomes our main therapy
“It’s not good, is it?” she says through knowing tears
“No, it’s not,” is all I can honestly say
She contemplates that answer
Suddenly, she asks
Curiously, earnestly, and fearfully
“Scot, what is it going to feel like when I die?”
My shock at her question
Induces a surreal state of mind
By stretching my preconceived notions
Of doctoring and caring beyond my boundaries
No one taught me how to answer that in medical school
Words used particularly with kids
“You can fight,”
“Be strong,”
“Hold on.”
Come to mind
But they’re hollow
Her look demands honesty
We both know she is dying
I pause and ponder
The unknown and the unknowable
Hoping the words come out right
“I think you will probably feel like you are falling deeper and deeper asleep,”
Says a slow, serious voice that quivers
“You will find it harder and harder to wake up and see all of us here,
But finally you won’t be able to wake up.
Instead, you will be in your dreams from then on.”
The words hang in the air
As she stares at me
With her own lie-detector
Her first words astound me
In their nobility and unfailing hope
“I like my dreams,” she finally says, resolutely
She starts crying uncontrollably
But I sense relief and a peace
Acceptance not fear
And I see just grace
My eyes have not been dry since her initial question
They let loose as well
I marvel at the depth of my sorrow
That our medicines and best intentions have failed
A child
But these tears calm me too
By finally being able to help this little girl
Somehow
Even if it was only hope in a dream
A few days later
Slipping in and out of consciousness
Emily starts saying goodbye
When she can
To family and friends who cared
One by one they leave her room
Shaking their head in disbelief
Wiping away tears
Later, alone with me in her room
Barely able to open her eyes
She wearily asks for a joke
As a joke
It is hard for us to smile
She motions for me to come closer
I lean in
“Scot,” she whispers
“You are going to be a great doctor.”
I look at her in utter amazement
Fumble for words of thanks
And feel so incredibly and deeply honored
By this beautiful gift
Yet, humbled beyond belief
Incredulous that her faith in me
Lasted longer than her body
My feeble definition of a good doctor
Altered and expanded forever
That is all she said
She drifted off into her dream world
And passed away that night, peacefully
A huge void existed the next day on rounds
One that was difficult
To fathom or interpret or describe
But in that emptiness
Was the beginning of a fullness
A spark of inspiration
That grows and grows
And blesses my life as a physician
Thinking of her now
Tears well up in my eyes
Such a tragic loss of a beautiful spirit
But ironically her memory
Enlivens each day with hope
Giving me the courage to smile
Can there be such a thing as a death smile?
Theme: Advance Care Planning
After reviewing this Guide you will be able to recognize the value of advance care planning (ACP) as a tool to enhance communication with families, build trust, engage in shared decision-making, and improve the care of medically complex children. You will also be able to consider strategies to introduce families to ACP, and to support them in reflecting on their values and goals throughout the course of the illness (not only at end of life).
Theme: Advance Care Planning
Matt and Myra, parents to Havi and Kaia, talk about what it felt like to discuss Havi’s end-of-life and what brought them to their decision not to bury her.