Spinal Muscular Atrophy: The Jones Family’s Story of Hope

Skylar with Spinal Muscular Atrophy SMA - 3 Months Old

We had the pleasure of meeting Ashley and Kyle recently after several people told us how much we would love connecting with them. Their reputation preceded them, and we were so grateful to talk with them about their journey through parenthood, caring for and making decisions for their terminally ill daughter, and holding onto their marriage after their devastating loss. After hearing them humbly share their hearts, we were struck by their strength and the fact that they are fighters, fiercely devoted, and selfless. It is our honor and privilege to introduce you to them and share about their beautiful little girl who they loved so well.

Receiving the Diagnosis of Spinal Muscular Atrophy

Skylar’s story starts when she was about a month old. During a well check, the pediatrician observed that Skylar had low muscle tone and was below the charts for her weight. Ashley showed him that Skylar’s arm was always bent at the elbow and wrist, as well. She anticipated that the doctor was going to recommend some physical therapy and possibly some supplemental formula. Instead, they were sent to a number of specialists who eventually delivered the devastating news that Skylar had Spinal Muscular Atrophy (SMA) type 1. Ashley and Kyle’s world was turned upside down.

Skylar’s characteristic bent arm was the beginning of her muscular degeneration. Similar to ALS, SMA gradually takes away the ability to move, eat, or breathe. SMA has no cure and is the leading cause of death in infants. Most babies diagnosed do not live to see their first birthday.

Ashley quit her job and became a stay at home mom and Skylar’s full-time caregiver. The gravity of Skylar’s condition was soon evident when, at 3 months old, she lost her ability to swallow.

Living with SMA: Holding onto Hope

As their hopes and dreams for Skylar’s life and their family shattered, they grappled with how to digest the news of her SMA diagnosis. Ashley shared that they took the appropriate time to cry and grieve their new reality, but they didn’t want to stay there. She reflected, “A diagnosis changes your world, but you change with it, and it doesn’t define you. Our daughter was dying. We could stay in that place, which was miserable, or we could try to make the most of our time together. We wanted fun and happy memories in spite of our hardships. As a family, we grew to appreciate every moment — even the hard ones. We allowed our trials and suffering to make us better instead of bitter. We know life is short and we wanted to make the most of the opportunities we had.”

Skylar with Spinal Muscular Atrophy SMA - 7 Months Old

In the early days of Skylar’s diagnosis, Ashley and Kyle were introduced to a new world of medical terminology and became acquainted with how to use at-home medical equipment that would be crucial to their daughter’s survival. The first intervention for Skylar was having a g-tube surgery with a nissen fundoplication. Before the surgery, Ashley hired a photographer to come and take family portraits in case Skylar didn't make it through the surgery. She made it through the surgery wonderfully, and over the next year, another photographer donated 2 portrait sessions and beautiful photo books to their family. Ashley and Kyle were so grateful to know they would be able to draw on the memory of the days they had with Skylar.

Their new normal was closely monitoring Skylar. Regularly, Ashley and Kyle had to suction Skylar’s mouth to prevent aspiration, use a cough assist to clear her airways, and use a shaker vest to help loosen up her lungs. As she grew weaker, Skylar used the BiPAP machine to assist her breathing. All of these were done in conjunction with multiple physical therapies that Ashley did with Skylar each day. Ashley explained, “It sounds like a lot, but it became a normal routine for us and was just part of life.”

With all of the equipment that was part of their daily life, Ashley and Kyle wanted to prioritize caring for Skylar’s heart and emotions with the same diligence that they applied to her physical care. They asked each other a profoundly insightful question, “How do we parent a child who still has every normal desire a kid would have at her age, but has the inability to communicate verbally or physically?”

Though unable to form sounds or use her limbs, Skylar had the most expressive eyes. If eyes are the window of the soul, Skylar’s soul is breathtaking. Even from birth, people commented on her sparkling blue eyes and long lashes. Her eyes communicated volumes and told her parents of both her discomforts and her delight and affection for them. She began using her eyes to look at what she wanted, and this became a daily staple for Ashley and Kyle to be able to parent, care for, and communicate with their daughter.

Ashley and Kyle also relied heavily on other SMA parents to get tips on communicating and to learn little things that had been helpful to their children. One family suggested a swing that their child loved. Due to Skylar’s needs, the suggested brand was not an option for her, so Kyle made her a custom swing that could accommodate her. She loved it! Another insight came when Ashley realized that most infants play with their mother’s hair. Since Skylar couldn’t reach for or grasp Ashley’s hair, Ashley leaned down and helped Skylar stroke her hair. The light and smile in Skylar’s eyes showed her happiness in connecting with her mom in that special way.

Living with Spinal Muscular Atrophy

These moments layered with the unending care that they lovingly gave Skylar amounted to a deep love and connection between Skylar and her parents. As their devotion grew, Skylar’s body became weaker. She had initially been using the BiPAP to help her breathe at night. It progressed to being used additionally at naptime, then every four hours, every two hours, and eventually 24/7. Other systems in her body started showing distress, and shortly after, Skylar passed away at 21 months old.

Navigating Grief: Committed in the Pain

I cannot comprehend the depth of pain that Ashley and Kyle felt in saying goodbye to their beautiful daughter for whom they had poured their lives out in selfless love and care. In our conversation, it was evident that they fixed their gaze upward to the Lord and drew strength from Him. Their arms were empty, yet they desperately clung to His promises of hope and sustaining strength to carry them. Their faith certainly didn’t erase their pain, but it comforted them and carried them in it.

The reality of missing Skylar set in immediately, and though they had prepared for this time in counseling, it was so painful. Ashley wrote about it in her blog in the days following, “The quiet in our home is deafening. Ever since Skylar was a couple months old, there has usually been a constant noise from her equipment, her movies, or her sounds. Not only is it quiet, but it's so lonely...it’s sad to see her empty bed begging us to come snuggle up in it. It's hard to stand in the silence because your mind starts to re-visit things. I try to think about her bright eyes welcoming me as I walk into the room, her sweet little hand hugs as I sat with her to watch movies, and her little giggles as I did the most ridiculous things. It usually ends in tears as I realize that I will never get those moments again even though I am so grateful for the memories. Missing Skylar is what brings the tears. We know she's so much better off in Heaven and so we're not sad for her. We're just sad we're not with her. We know that our lives were meant to go on and we still have purpose to our lives — so we keep going.”

As they navigated the waters of grief, Ashley and Kyle discovered that they handled grief very differently from one another. Ashley commented, “Kyle and I grieve so differently. That was really hard because it felt like our marriage was going in different directions because we were going in different directions emotionally. Then we realized that just because our grief is going in different directions doesn’t mean our marriage is ending or that we can’t get through this together. That realization helped a lot.”

Ashley and Kyle were told soon after the SMA diagnosis that the divorce rate is 90% after losing a child. Kyle remembers, “I was really thankful going into it that we’d been told that marriage was going to be a big challenge. I was thankful we heard it and had the humility to realize that we are not so much better than everyone else that has gone through this. We knew we should really take that statistic into consideration. Even preparing ourselves for it by going to counseling while we had Skylar and after she passed was incredibly helpful, but it was still very challenging. It was still a choice.”

In that first year, they had to learn how to communicate and respect each other’s needs. They created a safe place with each other that allowed them to share their feelings openly without fear of anger or rejection. Sometimes they just asked each other really practical questions to understand what the other needed: What does this mean for you right now? Do you want a hug? Do you need to cry by yourself?

Kyle continued, “The bigger choice was not to say, ‘We’re faithful to each other,’ but to make our marriage what it should be and could be. And we’re still working on that in some areas.”

They had really practical conversations to not only survive grief and marriage, but as Kyle said, to make their marriage and their life together all it can be. Ashley started a business during this time and realized that the SWOT analysis and goals she was making for her business would be helpful in their marriage, too. After talking with Kyle about it, he surprised her with journals for their anniversary to write out their strengths, weaknesses, how they are similar, and how they are different. They asked questions like, “How can we be better? What goals are we both willing to sacrifice to make this work? What do we love doing together? Let’s make more of that happen.”

Ashley and Kyle’s commitment to their marriage is so beautiful and such a reflection of the hope that they carry. Ashley recalled, “I found hope in God, through other people's kindness, and believing that there is a greater story in the works and that we are but a small part of it.”

Looking Ahead: Love Not Lost

Ashley was keenly aware that her suffering had a purpose, and a year after Skylar’s passing began to seek out what that was. She realized that the photographs of Skylar from the three gifted portrait sessions were an immense comfort to her. By simply looking at them, she was able to be present with Skylar, to look at her, talk to her, or just hold her and cry. Ashley explained, “Being a photographer myself, I saw the value in photos, but it wasn't until Skylar was gone that I understood the depth of what a tangible photo could do for those left behind. They helped me grieve, brought me comfort, and bring me joy now. As her memory gets farther away as each year passes, those photos bring her close again.”

Skylar with Spinal Muscular Atrophy SMA - 16 Months Old

Ashley knew she wanted to start something new, and because of the way that the photographs of their family had meant so much to her after losing Skylar, she started a photography business. In addition to portrait and wedding photography, Ashley also offered to give a free portrait session to anyone she heard of who was facing a terminal diagnosis. As she began donating more of these sessions, she knew something bigger was taking shape.

She soon formed an organization, Love Not Lost, to photograph people facing a terminal diagnosis to capture and preserve their memory for those they love. Love Not Lost provides free portrait sessions through their network of volunteer photographers. They take the best photos from the session and design a beautiful photo book to help people through the grief and healing process. It's their mission to support people in grief and build a community that strives to love people better.

Ashley added, “People frequently ask me how I do what I do... The suffering and grief I've been through, along with the suffering I've joined others in is hard. And it hurts. But there is great joy and much hope, too. I know that this gift can help people through some of the darkest times they will face in life, when many find themselves feeling helpless and alone. It is truly an honor to come alongside of people who know their time is short to capture and preserve their memory for those who love them. I can't think of anything else in life for which I am more qualified and have a passion for more than this. It is why I created Love Not Lost. Death stings, but it's only because there is great love, and that love is never lost! Everyone deserves to be remembered.”

Today marks five years since Ashley and Kyle lost Skylar. The memory of her beautiful, bright eyes compels them to remember the bigger story they’re a part of in spreading hope. Their faith and commitment is an inspiration that no matter the circumstance, we can find purpose and healing.

SMA Type 1

Love Not Lost has already touched so many families, and they are preparing to roll out a formal grief training curriculum to equip more photographers and reach more families. If their story has touched you or you know someone who has lost a loved one, we highly encourage you to check out their crowdfunding page and support them in any way that you can!

Photo Credit: Sarah Harms Photography and Tessa Marie Photography