In the United States, a rare disease is defined by a prevalence of < 200,000 patients. There are approximately 7,000 unique rare diseases affecting an estimated 25 to 30 million people in the United States. So researching these conditions is incredibly important.

About 5-10% of rare disease diagnoses are lung-related, such as Alpha-1 antitrypsin deficiency, Pulmonary arterial hypertension, and Lymphangioleiomyomatosis (LAM). These conditions may be foreign to most, but to the people who suffer from them and the researchers who study them, they are all too familiar. 

Restoring Hope for Rare Respiratory Disease Researchers

Rare respiratory disease can cause  barriers in a patients life, and the rarity of their disease can be isolating. Unfortunately, researchers of rare respiratory disease can feel isolated as well; With only a few cases, clinical data and research opportunities are limited in both clinical research and treatment development.

Luckily, advanced lung simulators have transformed how we study rare diseases, making it possible for researchers to  simulate specific respiratory conditions, test potential treatments, and gain insights into disease progression—all without needing access to numerous patient cases. With such limited cases available, lung simulators offer a lifeline to researchers.

Using Lung Simulators to Replicate Unique Respiratory Conditions

Lung simulators offer researchers a unique opportunity to study rare lung diseases by replicating the human lung’s complex respiratory mechanics.  This includes specific symptoms and disease progression. With this information at their fingertips, researchers can complete a controlled and detailed study of each condition.

That being said, not all lung simulators are created equal. While many test lungs perform just a handful of simulations and are not fully to scale, limiting their usefulness, using a simulator like The Training Test Lung®, allows you to simulate realistic residual lung volumes and lung capacities. The range of settings for compliance and resistance on the TTL® and PneuView® systems allow researchers the advantage of moving and “feeling” like a real lung or lungs when ventilated.

For example, if a researcher is studying the progression of Idiopathic Pulmonary Fibrosis (IPF), a disease that causes lung tissue scarring, they can set the lung simulator to emulate a reduced lung volume and decreased elasticity, mimicking the lung’s stiffened state. This allows researchers to observe the lung’s behavior under disease-specific conditions, providing a safe, repeatable way to test potential therapies or examine how respiratory function deteriorates over time.

The Benefits of Lung Simulators on Rare Lung Disease Research

Breakthroughs in the development of technologies, including lung simulators, further the goal of personalized and precision medicine, providing major benefits in researching rare respiratory diseases. These benefits include: 

Modeling Diseases Accurately

Lung simulators allow researchers to model disease symptoms and progression by controlling a number of variables, such as tidal volume, respiratory rate, and airway pressures to reflect a range of patient profiles. This ensures that treatments are truly personalized to each disease’s specifications for optimal results, which is essential when studying diseases with unpredictable progression.

Providing a Safe Testing Environment

For researchers working on treatments or interventions for rare diseases, lung simulators offer a controlled, risk-free environment to test new ideas and hypotheses before advancing to human trials, and help make the connection between theory and clinical experience in a safe setting.

Collecting and Analyzing Data

Some simulators, like the PneuView® system, allow for real-time data display and data export. Researchers can obtain data and waveform displays of pressure, volume, flow, timing and more, even when using simple ventilation devices like BVM’s, emergency ventilators, and CPAP systems. PneuView Software allows researchers to get in-depth visual feedback on devices and techniques. It also provides a mechanism to collect and review results.

Creating Breakthroughs in Research

Simulation capabilities like these have led to breakthroughs in respiratory research, helping develop safer, more effective treatments and empowering researchers to pursue insights that otherwise might have required extensive patient studies.

The Future of Lung Simulators in Rare Disease Research

As technology surrounding rare diseases continues to evolve, so does the potential for lung simulators in rare disease research. Advancements in software, real-time data analytics, and AI integration are making simulators increasingly useful and have the potential to unlock new possibilities in the research of rare respiratory diseases. 

Lung simulators in particular play an essential role in developing personalized medicine, where treatment approaches can be tailored to individual patient profiles based on specific respiratory needs and limitations. This customization can be incredibly useful in the case of studying rare diseases.

Michigan Instruments’ Commitment to Furthering Rare Disease Research

Lung simulators have transformed the research landscape for rare respiratory diseases, offering researchers the ability to replicate disease conditions, conduct safe testing, and explore new treatment possibilities. As technology advances, these devices will continue to play a pivotal role in rare disease research, bringing hope and potential solutions to patients affected by these challenging conditions. 

With devices like the TTL® , SBL and PneuView® , Michigan Instruments has been at the forefront of technological innovation for decades, providing researchers with the tools they need to conduct critical work in rare respiratory disease research.

Contact us today to see how Lung Simulators from Michigan Instruments can help propel your research forward.

The computer-controlled ventilators we know and use today operate on basic principles that are more than a century old. While the history of respiratory therapy dates back, there’s no denying that the advancements in the field have come a long way since then! 

What is Respiratory Failure?

To fully understand the miracle of respiratory therapy, we first need to grasp the gravity of respiratory failure. 

Respiratory failure is a condition that makes it difficult for a person to breathe on their own, resulting in the lungs’ inability to supply blood with oxygen. As breathing is an essential function of life; without sufficient breathing, our tissues and organs cannot work properly. 

According to NHIBI, when we inhale oxygen from the air into our lungs, we exhale out carbon dioxide, which is a waste gas made in the body’s cells. Improper breathing leads to a buildup of carbon dioxide. This buildup can damage tissues and organs and slow oxygen delivery to the body.

Some of the different causes of respiratory failure include:

• Nerve and muscle disorders
• Lung and airway diseases 
• Fluid buildup in the lungs or pulmonary embolism 
• Infections in your brain or spinal cord, lungs, or airways
• Blocked airway when food or another object gets stuck in your airways
• Chest or back injuries
• Severe scoliosis
• Severe allergies to food or medicine
• Lifestyle habits, like smoking or using drugs or alcohol 

The Ventilator: The Medical Device You Hope You’ll Never Need

When encountering respiratory failure, the use of a ventilator is an extreme life-saving measure.  These are used when a patient’s lungs are not functioning properly on their own. Ventilators give a patient’s body a much-needed rest in addition to support, allowing their bodies to heal.

The use of ventilators goes back quite some time, and there are different types of ventilators on the market today. But how has technology changed over the years? 

Whole-Body Ventilators Lead to The Iron Lung

In an article written by Robert M Kacmarek, it is noted that the negative-pressure ventilator was the predominant device used to provide ventilatory assistance in the 19th and early 20th centuries. 

• 1838: The earliest ventilator was described as a full body ventilator, or as a “tank ventilator”. The ventilator was an air-tight box, with the patient in a sitting position while negative pressure was established by manually pumping air into and out of the box. 
• 1904: Following the Tank Ventilator were similar types of manually operated negative-pressure ventilators, like Sauerbrach’s negative-pressure operating chamber in 1904. In this chamber, the patient’s body, except for the head, was maintained inside a chamber which was large enough so that the surgeon was able to perform surgery while also in the chamber. 
• 1907: Johann Heinrich Dräger and his son Bernhard introduced The Pulmotor, one of the only early devices for positive pressure ventilation in 1907. The Pulmotor was a transportable device that used a face mask to deliver oxygen until a set pressure was reached in the lungs, at which point the device switched to assisting the patient with exhalation. 
• 1928: The Iron Lung, originally designed and built by Drinker and Shaw, but manufactured and sold by Emerson, was one of the most widely used ventilation devices of the early 20th century. It used negative-pressure techniques and worked by changing the pressure inside an airtight container. It expanded and contracted the chest, pulling air in and out of the lungs, and providing much needed support in the ICU for children suffering from polio for decades.

Advancements in Positive Pressure Ventilation are Made 

Just after the Polio epidemic of the 1950s, there was a movement away from negative-pressure ventilation.

This was mostly due to:

1. Volume-targeted ICU/anesthesia ventilators beginning to appear. 
2. The development of small, compact, intermittent positive-pressure breathing (IPPB) devices.
3. The use of negative-pressure ventilation became too much, as they were large, heavy, and cumbersome.

The development of new positive-pressure breathing devices is credited to the need for pilots of late-WWII era jet planes requiring oxygen for their flights at high altitudes.

In the mid-1950’s, the Bird Mark 7 Respirator was developed by a former U.S. Army pilot, Forrest Bird. Many consider this device to be the first modern medical respirator, which led to many advancements in ventilator design and technology.

Non-Invasive Ventilators Through the Years

Though negative pressure, whole-body ventilators were most widely used at first, other non-invasive ventilators existed as well.

• 1780: The bag and mask manual ventilator is introduced by Chaussier
• 1887: A more sophisticated bellows with a mask was introduced by Fell
• 1907: Dräger’s Pulmotor was first introduced, and for its time, was fairly sophisticated. The pneumatically operated positive-pressure device has been credited with saving thousands of individuals over its lifetime.
• 1910: Green and Janeway create a device known as a “rhythmic inflation apparatus.” The patient’s head was placed into the apparatus and a seal was secured around the patient’s neck with positive pressure applied to the patient’s head. 
• 1957: The Bird Mark series of ventilators were originally created to provide intermittent breathing treatments as opposed to long-term ventilation. Soon after, they began to be used for life support in both noninvasive and invasive ventilation.

Continued Advancements in Positive Pressure Ventilation Leads to Invasive Options

Another key part of the history of respiratory therapy is positive pressure ventilation. The development of ventilators for positive-pressure invasive ventilation began in the 1940s, and with each generation, became more advanced. Early models provided volume-control ventilation but lacked patient-triggered ventilation. Examples included the Morch ventilator, a simple piston ventilator, and the Engstrom ventilator, more sophisticated with double-circuit capabilities. 

The first-generation ventilators didn’t incorporate PEEP until the 1970s. The second generation introduced patient-triggered inspiration and basic alarms like high pressure and low tidal volume. Intermittent mandatory ventilation (IMV) emerged during this period. 

Third-generation ventilators, with microprocessor control, marked a significant advancement with extensive monitoring, waveforms, and various ventilation modes like pressure support and SIMV. 

Fourth-generation ventilators are the most complex, featuring numerous ventilation modes, closed-loop control, and capabilities for non-invasive ventilation (NIV) and neonatal ventilation. They also offer extensive monitoring and specialized management packages for weaning and assessments.

Michigan Instruments is Committed to Continued Advancements

Michigan Instruments Lung Simulators play a crucial role in advancing ventilator technology through various contributions that enhance training, testing, research, and product development in the field of respiratory therapy.

• Training: We offer the Spontaneous Breathing Lung (SBL™), a sophisticated device that facilitates training for healthcare professionals and students. With the SBL™, users can create a wide range of breathing scenarios and patterns, making it an invaluable tool for educating professionals on new respiratory devices, ventilators, and therapeutic modalities.
• Testing: The SBL™ is instrumental in testing and troubleshooting devices designed for spontaneous breathing patients. It enables thorough assessments, such as detecting the start of a breath and avoiding breath-stacking, ensuring the optimal performance of respiratory devices.
• Research & Development: Our Lung Simulators are highly versatile for research applications. They have been utilized in diverse studies, from analyzing aerosol dispersion to evaluating the responsiveness of new ventilation. Researchers benefit from the precise simulation capabilities of Michigan Instruments’ equipment, enhancing the quality and accuracy of their research outcomes.

The SBL™ offers advanced control over breath rate, tidal volume, inspiratory time, and inspiratory flow pattern. Additionally, it has customizable settings and intuitive software, so users can simulate spontaneous breathing accurately. This makes it ideal for designing, testing, and training on non-invasive ventilation and oxygenation modalities.

Elevate Your Research Abilities with Michigan Instruments

Our commitment to innovation and excellence in respiratory care is evident in our comprehensive range of simulation tools and software solutions. We continue to empower healthcare professionals, educators, and researchers worldwide—contributing significantly to the evolution of ventilator technology and respiratory therapy practices.

For more information on our devices used in research environments worldwide, contact us today.