PARENT'S PERSPECTIVE

Written by: MICHELLE L. LANGE, OTR/L, ABDA, ATP/SMS

Kory, Elizabeth and their two boys are a typical busy family in Missouri. Kory worked in communications at a nonprofit, and Elizabeth is a school psychologist. The family was excited to welcome their third child, Asher. After Asher’s midwives noticed some progressive weakness and loss of reflexes, his pediatrician admitted him for a neurological and genetic evaluation, and he was diagnosed with spinal muscular atrophy, type 1 (SMA) at just 1 month of age. Within a week of his diagnosis, he began treatments to help slow the progress of this disease; but in the meantime, Asher also developed a cold, was intubated and had to be airlifted to the St. Louis Children’s PICU. Eventually, he underwent a tracheotomy to manage his chronic respiratory issues and copious secretions. Today, he has a mostly stable airway but still sometimes needs suctioning, mechanical coughs, ventilation, and occasionally, supplemental oxygen. Asher also has a gastrostomy tube and is not currently eating by mouth. He was born with a right club foot which, in the past, would most likely not have been treated as improbable ambulation. As newer medications and gene therapies have changed the prognosis for children with SMA, his parents decided to have it surgically corrected. Finally, Asher has developed some scoliosis and wears a TLSO about 60% of the time. Due to Asher’s medical needs, Kory left his work position and has been a stay-at-home dad ever since (he likes to call himself the “CEO” of Asher’s complex care).

Asher is now 3 years old and can sit independently and stand with assistance. His primary language is American Sign Language (ASL); however, he is beginning to vocalize around his trach often, especially when using a Passy Muir valve. He also has a communication device to help him speak clearly. He is in preschool and loves “Blippi” videos and nature documentaries, being outside, taking walks and swinging, anything with wheels, and music. And, he is an amazing power wheelchair driver!

Asher received an adaptive stroller as an infant (See Figure 1). This not only provided a dependent mobility base and postural support for Asher but also supported his extensive medical equipment and supplies. Before age 2, his family was already exploring a manual wheelchair to provide some independent mobility for their youngest son. The Panthera Micro allowed Asher to propel short distances, exploring his environment at peer level (See Figure 2). If the family went anywhere, they brought along a wagon to carry all his equipment and supplies. He also began using a gait trainer, which provided limited mobility and weight bearing. Asher works with Keith Jolicoeur, ATP, CRTS ® at Alliance Rehab and Medical Equipment. It was very important to Kory and Elizabeth to have everyone on the same team. If someone was not on board or seemed disinterested or unconvinced about giving Asher a fair shot at independence, they were not eager to work with them.

Kory and Elizabeth were the first to bring up power mobility. Despite having some independent mobility, Asher was getting frustrated he couldn’t keep up with his brothers and peers. They knew Asher would need to navigate school and keep up with the other kids in the future. His parents brought this up to his therapists who arranged for Asher to try several devices, such as an adapted ride-on car and a Permobil Explorer Mini, while at therapy. Although he did well with each of these options, his parents realized Asher would eventually need a power wheelchair to truly get him mobile in his community; he was ready now, and there was no reason to delay.

Asher proceeded to trial five different pediatric power wheelchairs in the therapy gym during the summer of 2020, between the time he was 18 and 24 months of age. He was able to drive using a joystick placed at midline, primarily using his left hand. During this time Asher’s skills improved, and his therapists had plenty of justification for their Letter of Medical Necessity. The family narrowed down the field to a few options, which Asher also trialed at home. His driving was best in the home with the Quantum Rehab Stretto, which he seemed to navigate intuitively. He was able to navigate hallways and corners even before the paperwork was filed! His parents also appreciated the small footprint, as he had quite a bit of equipment to carry around.

The team discussed the recommendations in detail. Kory and Elizabeth appreciated being involved in each decision. Asher appreciated picking out colors! A power tilt, recline and elevate were recommended, as well as an appropriate seating system. Keith and the team at Alliance worked hand-in-hand with Asher and his parents to custom fabricate all of the extra equipment-toting pieces that were not available for the chair from the manufacturer.

The power wheelchair was funded on the first submission, and Asher received his very own power wheelchair in March 2021 at age 2 ½ years (See Figure 3). Asher didn’t receive very much mobility training as he had practiced quite a bit during the power wheelchair trials and was quite proficient. After initial programming, Asher began practicing with his family.

Before Asher began trialing power wheelchairs, the family had moved into a home with a ramp at the garage door entrance and off the back deck. Once they were comfortable, the power wheelchair would be funded, his parents began looking at accessible vehicles. They were able to purchase a full-size van and convert it with a lift right before a very special extended family vacation in summer 2021 to Disney World! The family drove Asher, his brothers, his beloved medical assistant, his power wheelchair, and lots of equipment and medical supplies to Florida. Despite his young age (nearly 3 years old at this point), Asher was able to drive with supervision about 50% of the time the family was in the park (See Figure 4). The primary reason caregivers took over driving the rest of the time was because of large crowds.

When asked about power mobility limitations, Kory stated Asher can drive up the driveway and ramp into the home, but it is hard to open the door. It is also hard to drive up to the dinner table, as the armrests and joystick are level with the table surface. The power wheelchair is bulky at preschool, and Asher prefers to be in the gait trainer in the classroom, because it is easier to get up to a table next to a peer. Asher doesn’t use the recline as much as anticipated, as he has ”too much stuff” behind the back of the wheelchair.

When asked what the family likes about the power wheelchair, Kory states they haven’t had a lot of issues. The power wheelchair has performed nicely, is an appropriate size for Asher, and works well in the house. Programming the driving parameters was critical, and

he was very appreciative of Alliance doing such a good job. Kory also likes they can mount Asher’s communication device and other items readily to the Stretto. The power wheelchair created a level of stress-relief for all of his caregivers, as well, especially when facing rougher surfaces and inclines where Asher struggles to stay safe and mobile in his manual devices. And since much of his emergency equipment, such as the vent, has a designated space on the power wheelchair, it is easier and less time consuming to leave the house. Packing up to go anywhere used to take up to 2 hours – and now takes about 30 minutes.

Asher is an adorable little boy who is exploring his world and participating not only because of his own amazing skills but also due to his parent’s desire and passion for him to reach his full potential!

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CONTACT THE AUTHOR Michelle may be reached at MICHELLELANGE1@OUTLOOK.COM

Michelle Lange, OTR/L, ABDA, ATP/SMS, is an occupational therapist with 35 years of experience and has been in private practice, Access to Independence, for over 15 years. She is a well-respected lecturer, both nationally and internationally, and has authored numerous texts, chapters and articles. She is the co-editor of Seating and Wheeled Mobility: a clinical resource guide, editor of Fundamentals in Assistive Technology, Fourth Edition NRRTS Continuing Education Curriculum Coordinator and Clinical Editor of NRRTS DIRECTIONS magazine. Lange is a RESNA Fellow and member of the Clinician Task Force. She is a certified ATP, certified SMS and is a senior disability analyst of the ABDA.