One Cowgirl’s Fight Against ALS Rages On

Jaci Hermstad lost her life, but her strength may save others

Almost four years ago, we were introduced to the spirited Jaci Hermstad and her family. Diagnosed with the same aggressive form of amyotrophic lateral sclerosis (ALS) at 26 that had taken her twin sister Alex at 17, Jaci and her family were all too aware of her prognosis.

Thanks to some quick connections and help from Dr. Neil Shneider from Columbia University, Dr. Lauren Black from Charles River, non-profit group Project ALS and others, Jaci’s team of doctors was granted the right to administer an experimental drug under the US Food and Drug Administration’s compassionate use program. Just four months after her diagnosis, Jaci was administered her first dose of ION363, also known as jacifusen.

Though Jaci’s doctors saw measurable benefits from her treatments, unfortunately her disease had progressed too far. Eventually she succumbed to the disease that had taken her sister, and she died in May 2020.

Though she and Alex had lost their lives, the hope they inspired in everyone who worked on jacifusen could not be dimmed. As of now, Ionis Pharmaceuticals is conducting a Phase III clinical trial led by Dr. Shneider for up to 64 ALS patients with the same genetic mutation as Jaci and Alex. The Hermstad family generously donated Jaci’s tissue to research, allowing the team at Columbia University to publish this study on the results of her treatment. Her data, along with data collected from specially designed, genetically engineered mice, formed the basis for continuing research and clinical trials.

In remembrance of her daughters, Lori Hermstad has this to say:

Twins Jaci and Alex Hermstad are the faith filled, inspiring all stars in helping to pioneer this promising treatment and potential cure for FUS-ALS, their form of ALS. It is being reported the drug has shown encouraging results and is doing what they set out to do – which is extraordinary and phenomenal news. Even though it is bittersweet, we feel so happy it will hopefully help others. Helping others helps lift our spirits and continue in our healing.

I mention both girls because it’s important to note that if Alex had not fought for as long as she did, we would never have known the specific ALS gene we were dealing with for Jaci. Unfortunately, their form of ALS is extremely aggressive, and we were on a critical timeframe to get her the drug in time.

We feel at peace Jaci was so courageous and brave, willing to put herself out there to move forward with the drug process as she sadly, rapidly declined waiting for her chance. Without Jaci’s efforts and sacrifices there wouldn’t be a jacifusen trial, and others wouldn’t have this chance today for a possible treatment.

Our emotional, endless, fierce fight to save Jaci was never just about her. It was always about the love of humanity and to help future FUS patients be saved as well. Our hope is that Jaci and Alex Hermstad will always be remembered for what they did along this journey to help advance science.

Our wish and enduring prayer are that countless lives will be saved because a remarkable group of compassionate, kindhearted people united together for the good. There was immense determination, unstoppable belief and tireless aspiration from Project ALS, Dr. Shneider and Columbia team, Dr. Lauren Black at Charles River, and Ionis along with our family because we believed in a dream.

The heartache of losing both Jaci and Alex will never go away, and we wished more than anything Jaci was still here with us sharing in all the amazing medical advancements she was so much a part of. We find comfort in knowing the impact of what it is doing and that others can benefit and even be saved because of the help of Jaci’s heroic efforts.

Both Jaci and Alex donated their tissue to help advance research and medicine. Our girls are trailblazers and champions with all they did for ALS research.

It wholeheartedly helps us when Jaci and Alex are remembered and not forgotten. We are so grateful when people talk about them and share special memories. We can’t promise we won’t cry but we still wish to hear about them and know that their lives mattered.

Saying their names and remembering them brings validation and acknowledgment to them, yet also to us, which helps us in our healing, emptiness and grief journey. As parents we fought unceasingly, yet we had to face the painful realization that we couldn’t save their lives, so preserving their namesake and legacy will be a battle we will never cede.

They say grief is the price of love. We loved Jaci and Alex more than anything with our whole heart and soul. The heartache of losing both our girls will never go away and some days are much harder than other days but the best we can do is try to honor them by being strong for them.

When we go on each day, we allow grace. The emotional loss may slightly diminish over time, but the scars are everlasting.

You can help families like the Hermstads by donating to Project ALS and other ALS charities.