Cystic Fibrosis and Airway Clearance Therapy

Cystic fibrosis (CF) is the most common fatal recessive hereditary disease that affects one in every 3,000 newborns. It creates dense layers of mucus in the lungs, causing airway obstruction and trapping.

Airway Clearance Therapy (ACT) is the treatment for cystic fibrosis (CF) that loosens sticky mucus, making it easier for patients to clear their lungs. Typically, ACT lasts about 1 hour and needs to be performed 2 times per day.

Excess mucus can lead to recurrent respiratory infections, lung failure, and irreversible damage. ACT removes mucus and minimizes infections, thus slowing down the rate of lung failure.

CF Management

Cystic Fibrosis requires lifelong management starting from birth. Home management, overseen by parents, is practiced until age 13. The "transition" period, spanning from 13 to young adulthood, prepares patients for independent adult life. From age 13 onward, individuals assume responsibility for their condition with guidance from their CF team.

Challenge

The "transition" period can be challenging, especially as patient compliance with ACT declines, leading to the risk of lung deterioration.

Project Goal

The overarching objective is to empower CF patients to enjoy their lives while adhering to treatment, emphasizing reducing lung failure to maximize life expectancy.

Outcome - Moii

Moii is a new ACT management approach for adolescents with CF. Beyond mere mucus expulsion, Moii focuses on enhancing efficiency and ability through the training of airflow control and chest mobility. The primary objective is to promote independence and liberate patients from relying on equipment, offering a more adaptable and seamlessly integrated ACT approach to daily life, thereby increasing adherence.

Research

In this project, I have been fortunate to receive support from various sources, including the Swedish Cystic Fibrosis Association (RFCF), CF Center Gothenburg, CF Center Uppsala, Fonden Citronfjärilen, Genia APP, and 28 patients and their families. Their assistance has enabled me to gain insights into the disease, the social environment, prevailing conditions, and related policies.

Analysis Process

The investigation revealed that low adherence is not attributed to a singular cause but rather arises from a complex interplay of factors. Some of these contributing factors encompass management methods, footprint, technique utilization, the learning curve associated with technology, and psychological aspects, among others.

CF Managing Footprint

In Sweden, there are four CF Centers, all concentrated in the south (Stockholm, Uppsala, Gothenburg, and Lund). Due to the unique nature of CF, each patient has their own CF team at the CF center they belong to. Patients visit the CF Center every six months for routine checkups. Due to geographic constraints, especially for patients in northern Sweden, CF Centers are unable to intervene in the patient's ACT treatment on a daily basis.

Airway Clearance Techniques

Positive Expiratory Pressure (PEP), Autogenic Drainage (AD), and Active Circle Breathing Technique (ACBT) are primary ACT techniques. PEP is often the choice for beginners due to its supportive equipment, while AD and ACBT offer more freedom but involve a longer learning process.

Equipment

PEP: The most common equipment is a PEP mask or pipe. By using the valves, patients can find the optimal pressure for their ability to stabilize airflow. However, the equipment adapts to the patient without challenging and training the patient.

AD/ACBT: AD and ACBT are typically executed with the assistance of an expiratory tube to facilitate mouth opening, or patients may choose to perform these techniques without the use of equipment.

Keys to Successful ACT

Airflow control and chest mobility are pivotal for effective Airway Clearance Therapy (ACT). While CF Centers provide guidance and visual feedback through instruments, home-based ACT lacks these supportive elements.

Problems regarding ACT

01: Patients desire greater freedom, yet the commonly used ACT techniques often provide minimal freedom.

02: A long and inefficient way to gain experience and learn advanced ACT techniques.

03: Patients lack motivation because there's no feedback or short-term rewards. The prospect of an extended lifespan resulting from consistent Airway Clearance Therapy (ACT) is less impactful for adolescents compared to the immediate enjoyment of their lives.

04: Airflow and chest mobility are not properly utilized in home Airway Clearance Therapy.

05: ACT brings negative emotions, reminding patients of their illness, which induces anxiety and resistance.

Opportunity

From PEP to AD/ACBT: What if we could create a program that helps patients start with supported PEP equipment, trains them to gain experience, and then allows them to progress to more advanced, un-equipped techniques, thus gaining more freedom?

Airflow and chest mobility evaluation: What if we could create products that provide instant feedback about their efficiency and offer guidance, eliminating the need to wait for 6 months to visit the CF Center?

Minimize negativity: What if we could design a progression process that brings positive energy?

Solution - Moii: A progressive ACT training kit

Moii is a novel ACT management approach designed for adolescents with CF. Beyond mere mucus expulsion, Moii focuses on enhancing efficiency and ability through the training of airflow control and chest mobility. The primary objective is to promote independence and liberate patients from reliance on equipment, offering a more adaptable and seamlessly integrated ACT approach to daily life, thereby increasing adherence.

The kit's collaborative components—the breathing device, chest mobility wearable, and app—facilitate a user-friendly ACT experience. The breathing device and wearable record training data, translating it into lightly gamified interactions in the app, while providing timely guidance and feedback. As training progresses, patients can reduce assistance by changing the parts of the breathing device, shifting their focus to breathing and chest movement, ultimately becoming free from equipment.

What sets this solution apart?

It considers the experience of ACT throughout the patient's life, enhancing the ability to expel mucus and potentially reducing the time-consuming daily ACT required.

It provides patients with the opportunity to set goals, potentially increasing their motivation for daily ACT.

It visualizes data collected from the breathing device and wearable in lightly gamified interactions, offering real-time instructional feedback and reducing patient boredom.

On a psychological level, Moii creates an ACT mate as a companion who witnesses the patient's growth, converting negative emotions associated with ACT into positive experiences.

Appearance

Aesthetically, Moii intentionally deviates from the clinical look of traditional medical products to reduce patient anxiety and foster a more positive and healing experience.