Patient Perspective

Stella’s IPV Therapy Story

Patient Perspective

Stella’s IPV Therapy Story

Patient Perspective

Stella’s IPV Therapy Story

For many with spinal muscular atrophy (SMA), including 16-year-old Stella Turnbull, effective airway clearance therapy is crucial, as even simple respiratory illnesses can become dangerous. Diagnosed with SMA shortly after birth, Stella relies on intrapulmonary percussive ventilation (IPV) therapy as a key part of her daily routine. By offering a proactive approach to her at-home respiratory care, IPV therapy empowers Stella and her family to embrace an active and fulfilling lifestyle with confidence.

Stella’s SMA Diagnosis

When Sarah Turnbull’s daughter, Stella, was just one month old, she was diagnosed with spinal muscular atrophy (SMA). SMA is a disorder that affects the motor neurons, which leads to muscles weakening and shrinking over time due to inactivity. SMA has no cure, and when Stella was diagnosed, there was no treatment available. Sarah and her husband were given devastating advice: take Stella home and cherish the time they had with her, since most children with Stella’s diagnosis passed away by their first birthday. “Essentially we were told that for sure by the age of two [she’d be gone],” Sarah reflects.

Despite this difficult diagnosis, Stella’s family remained determined to keep her healthy and ensure the best possible quality of life. And, at 16 years old, Stella has surpassed the initial expectations given to her family.

While SMA presents many challenges for Stella, Sarah and her husband have found tools that help keep her healthy.

“Essentially she is trapped inside of her body,” Sarah explains. “Her brain works perfectly fine, but a lot of the muscles in her body don’t work.” She requires aids for swallowing and breathing, a gastronomy tube (G-tube) for feeding, and respiratory therapies aimed at protecting her lung function.

Navigating the Respiratory Challenges of SMA

Among the challenges that SMA brings, respiratory care is particularly difficult. The weakening of muscles essential for breathing, swallowing, and coughing makes managing secretions hard. This can lead to mucus buildup, creating an environment ripe for illnesses and the potential for choking, especially for young children. A weak cough further complicates recovery from illnesses, making even minor ailments potentially life-threatening, as they can progress into dangerous infections, like pneumonia. For Stella and her family, these obstacles have been especially challenging and frightening to navigate.

“We had multiple emergencies where Stella essentially choked on her secretions while in her car seat in our car and [we] nearly lost her,” Sarah recalls.

For the Turnbulls, and many other families of children with SMA, finding an effective way to overcome these challenges was crucial. They explored several airway clearance techniques for Stella, including mechanical insufflation-exsufflation devices, commonly known as “cough assist”. However, they felt as though they could do more. They moved on to trying vest therapy, or high frequency chest wall oscillation, but again, it didn’t seem sufficient for Stella.

“I just didn’t feel like [the vest] got into those lower lobes [of her lungs] and helped us to really clear as good as we could,” Sarah says.

What is spinal muscular atrophy?

Spinal muscular atrophy (SMA) is a disorder that affects the motor neurons in the spinal cord, leading to progressive muscle weakness and atrophy. Most cases of SMA are caused by a mutation in the SMN1 gene, which is crucial for producing the survival motor neuron (SMN) protein. This protein is essential for the survival and proper functioning of motor neurons. Without sufficient SMN protein, motor neurons deteriorate and fail to effectively relay signals from the brain to the muscles. As a result, the muscles are not stimulated and used, causing them to weaken and shrink over time.¹

Symptoms of SMA vary widely in severity and age of onset, and can include muscle weakness, impaired motor functions, feeding and swallowing difficulties, and respiratory issues.

Discovering IPV Therapy

Sarah and her husband first discovered intrapulmonary percussive ventilation (IPV) therapy when Stella was just a toddler, after hearing about it from other families of children with similar conditions. Once they gave it a try, they quickly realized its potential value in Stella’s daily routine.

The impact of IPV therapy became evident to the Turnbulls when Stella battled respiratory syncytial virus (RSV) — a respiratory virus that is particularly dangerous for children with SMA due to the risk of mucus plugging, which can obstruct the airways.

“She was just a toddler and really struggled with getting those secretions out,” Sarah says. “Unfortunately, we have seen a lot of kids succumb to RSV when they are quite young in this population…And so we are very thankful to have seen [IPV therapy] help her through that.”

IPV therapy has become a crucial part of Stella’s daily routine. She uses it daily in the morning and before bed, and increases her treatment frequency as needed — especially when she’s sick. This helps her and her family manage her care at home when possible, which in turn supports an active lifestyle with her family. Even more importantly, it helps to keep her out of the hospital: Stella’s pulmonologist has noticed a significant decrease in her hospital visits.

“We’ve noticed that she … often doesn’t need any other treatments; really, the IPV [therapy device] is kind of the one-stop-shop for her,” Sarah says. “It’s not something you have to keep putting her through. It’s just a treatment that she does twice a day and she can go about her day, which is great.”

We’ve noticed that she … often doesn’t need any other treatments; really, the IPV [therapy device] is kind of the one-stop-shop for her.

Embracing Life with Confidence

With the help of IPV therapy, the Turnbulls are able to confidently embrace each day. Stella’s proactive daily airway clearance routine has supported her active lifestyle, which includes school, extracurricular activities, and family vacations. Her parents feel like they are able to give Stella the same life experiences as their other children.

“IPV therapy has been very lifesaving for Stella,” Sarah shares. “When I look at all of the things we’ve added to help her get through her day, get through her illnesses, IPV [therapy] ranks up there with her ventilator — in terms of airway clearance, in terms of just giving us the confidence to go about the day with her and not worry about her plugging.”

One of Sarah’s greatest hopes for Stella was to change the course of SMA. Sixteen years ago, her family was told there was nothing they could do. Today, SMA is now included in newborn screening programs in every state across the U.S. — a development Stella and her family have played a significant role in making happen with their continued advocacy. Additionally, three treatments for SMA have emerged in recent years, offering newfound hope and possibilities for those affected by SMA.

“Because of her life…we’ve all just kind of grown not taking things for granted and just trying to really enjoy each day that we have,” Sarah says. “I count it a blessing that she’s part of our lives and honestly, I wouldn’t want her any other way.”

IPV Therapy at Home

Sentec IPV® Therapy is an effective, safe, and well-tolerated airway clearance therapy across diverse patient populations, care settings, and delivery interfaces.

Learn more.

IMPULSATOR

Trusted treatments in a home environment

The Impulsator is specifically designed for convenient use in home care and outpatient clinics, enabling patients to benefit from ongoing airway clearance therapy and lung rehabilitation in a comfortable environment.