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Meghan Teague

Turning Personal Loss into Awareness for PPA2 Deficiency

Meghan Teague is a creative young woman with a close-knit family and a dynamic career. The 27-year-old, who lives in Oakville, Ont., works in event production as an audio-visual technician. “My job takes me all over Canada,” she says. “I get to work with all sorts of amazing people and in great locations.”

In her spare time, she enjoys being outdoors and all things creative. “I do a lot of hiking, biking, skiing, and running,” she says. “I also like to draw and paint, make my own clothes, and play piano and guitar.”

But behind her active lifestyle is a rare mitochondrial disease few people have heard of.

Growing up, the Teague family was close. Meghan and her three brothers (two older, one younger) were active kids, spending plenty of time together biking, playing  street hockey, and going to the park to play catch. “My siblings and I have always been really close,” says Meghan. “I would consider them my closest friends.”

From bike adventures with their dad to painting and playing the piano with their mom, the Teague children were supported in their athletic pursuits and encouraged in their creativity. But behind their idyllic upbringing, a medical mystery was brewing.

“Throughout childhood, my siblings and I had very negative reactions to medications and illnesses, from cough syrups and dental procedures to high fevers and viruses,” says Meghan. “We began to keep track of things ourselves, to pinpoint what caused us issues.”

At age six, after surgery for a broken arm, Meghan reacted badly to the anaesthetic and had trouble walking, sore muscles, chest pain, and vision issues for months. Doctors couldn’t figure out why. A few years later, one of her older brothers became seriously ill after consuming a small amount of alcohol. Doctors couldn’t explain the reaction, but Meghan’s mother suspected the incidents might be connected and urged her daughter to avoid alcohol as a precaution. Looking back now, Meghan believes her mother’s intuition may have saved her life.

In her second year of university, a new batch of mysterious symptoms appeared after she had experienced a viral illness and a concussion from falling off her bike: weakness, difficulty climbing the stairs, and chest and muscle pain that woke her up at night. Alarmed, Meghan sought medical advice but was told she had nothing to worry about. “After a few months I started to feel more like myself again, but I knew deep down that something wasn’t right,” she says.

Nearly a decade after her older brother’s incident, Meghan’s youngest brother Ben passed away at age 17 after consuming a small amount of alcohol. “It was devastating for our family,” says Meghan. “We were left with a piece of us missing.”

Medical professionals couldn’t find an explanation for his death. “We again had no answers as to why this was happening,” says Meghan.

After Ben’s death, their mother pushed for answers for years. Finally, after six years, doctors tested tissue from Ben and found genes consistent with PPA2 deficiency, a rare mitochondrial disease that affects how the body produces energy. “That’s when my other siblings and I were all genetically tested and found positive for PPA2 deficiency as well,” says Meghan. “Before that point, PPA2 wasn’t well-known in Canada, so our samples had to be sent to the U.S. to be tested. I was 26 years old when I was diagnosed, and it was 20 years after my first symptoms.”

PPA2 deficiency places individuals at increased risk of serious heart-related complications, including sudden-onset heart rhythm problems (arrhythmias) and weakening of the heart muscle (cardiomyopathy), particularly when the body is under stress. Muscle weakness and neurologic symptoms, such as seizures or developmental challenges, are also sometimes present. Triggers can include infections or viruses, fever, dehydration, intense physical stress, and alcohol consumption. “An accumulation of these triggers in PPA2 patients has been shown to cause sudden cardiac arrest,” says Meghan.

Over the years, she and her brothers have figured out what works best for them when it comes to managing the disease. “We’ve found healing primarily through rest and lots of fluids,” says Meghan. They’ve also learned to avoid triggers, rest fully when they’re sick or injured, and undergo regular cardiac monitoring.

Alcohol has been one of the most difficult triggers to avoid. “Alcohol could be lethal for me, or at the very least cause permanent heart damage,” says Meghan. “And many medications contain alcohol. I’ve tried to explain this to doctors and pharmacists, but sometimes they’re not very understanding. The condition is so uncommon.”

Avoiding alcohol socially has also been a challenge. “Because of the culture around drinking, even going to events or restaurants can be risky for me,” says Meghan. “Many people have misunderstood me and tried to pressure me to drink.”

Living with PPA2 deficiency has meant confronting a stark new reality. “Being diagnosed with a condition that isn’t well-known has caused me a lot of anxiety,” says Meghan. “I hate feeling like my future is unknown and out of my control. The little information available about this condition is bleak, and the death rate for PPA2 deficiency is very high. It’s hard to stay positive about it all.”

As Meghan does her best to move forward carrying a new weight on her shoulders, she also makes sure to honour the past and her youngest brother’s memory.

“My little brother was an amazing person and it hurts me to know that people can’t meet him now,” she says. “Ben had a huge heart and was kind to everyone he met. He went out of his way to make things easier for other people. That’s just the type of person he was. Whether it was helping our grandma with her physiotherapy exercises, helping our dad fix the family car, or helping me practise for my driving test, he was always there for the people he cared about.”

Meghan shares that Ben did well in school and was a skilled defenceman in hockey. “He was looking forward to university and dreamed of becoming an engineer,” she says. “At just 13, he started his own business, Bengines, doing small engine repairs. People would travel from all over to have him fix their lawnmower or snowblower, and everything he was doing was self-taught.”

His life was tragically cut short by a hidden disease that few people understood. “Ben had so much potential and it breaks my heart that he didn’t have the chance to fulfill his dreams,” says Meghan. “Nobody should have to lose their life over something like this.”

Meghan looks back at the years of mysterious health issues with no answers from a new perspective since her diagnosis. “I believe the MitoCommunity really needs  to be listened to and their symptoms seriously considered by medical professionals,” she says. “We don’t need to be made fun of by doctors for having symptoms that they don’t understand. We don’t need our symptoms to be downplayed or minimized. It shouldn’t take someone’s death to be taken seriously.”

She encourages others to advocate for themselves and to stand up if they know something’s wrong.

Meghan also advocates for better screening of PPA2 deficiency. “I hope one day we’ll have preconception screening for this condition,” she says. “It’s especially dangerous for infants and young children, and the heart damage sustained can affect them for the rest of their lives. Many families across the globe have already suffered the loss of multiple children and babies to this disease. Preconception screening could help better prepare families and healthcare professionals from the start.”

Meghan was finally diagnosed after two decades of symptoms, but by then it was too late for Ben. “I hope that telling my story might help another family out there that recognizes these symptoms in themselves,” she says. “Most people have never heard of PPA2 deficiency. More awareness of it may save someone like my little brother.”

Research in MitoNutrition

Mitochondrial disease is complex and highly individualized, so it’s no surprise that nutrition research is moving toward more personalized, diagnosis-informed approaches. While we don’t yet have a single “best diet” for mitochondrial disease, growing evidence is helping clinicians better understand when nutrition strategies may be helpful, for whom, and why.

This is an encouraging direction. As research evolves, it strengthens the possibility of more refined nutrition guidance, better symptom management tools, and ultimately, improved quality of life for adults living with mito.

One of the most hopeful shifts in the field is a move away from one-size-fits-all advice and toward precision care, where nutrition strategies are tailored to a person’s specific mitochondrial diagnosis, symptoms, and metabolic risks.

This approach recognizes that different genetic conditions can affect energy pathways differently, and nutrition strategies need to match those differences (for example, fasting guidance in FAOD versus other mitochondrial conditions).

Researchers continue to explore therapies aimed at supporting mitochondrial function more directly, including targeted supplements and “metabolic support” strategies that influence how cells generate and use energy. For example, NAD⁺ biology and NAD⁺-boosting compounds (such as nicotinamide riboside and related approaches) are being actively studied across conditions involving mitochondrial health, with emerging clinical trial results in rare disorders showing mixed but evolving findings. NAD⁺ (Nicotinamide Adenine Dinucleotide) is a coenzyme found in every cell, crucial for energy metabolism, DNA repair and cellular signaling.

It’s important to note that many of these approaches are still under study, and what’s appropriate can vary widely by diagnosis. This is why specialist guidance remains essential as new options emerge.

Another rapidly growing area is the connection between gut health, the microbiome, and mitochondrial function. A 2024 review focused specifically on mitochondrial disease highlights the “mitochondria-microbiome” connection and explores how diet and microbe-produced metabolites (small organic molecules produced during metabolism) may influence mitochondrial pathways, opening up new possibilities for supportive therapies and research.

For many adults with mito who experience gastrointestinal (GI) symptoms, this research is especially meaningful because it validates that GI function isn’t “separate” from energy, it may be part of the same interconnected system.

Nutrition is a powerful tool for supporting daily living with mitochondrial disease.

By understanding the principles of MitoNutrition, balanced meals, adequate hydration, symptom-aware strategies, and appropriate supplements, individuals can make informed choices that help support energy, resilience, and overall quality of life.

As with all aspects of mito care, nutrition works best when it is personalized and team-based, guided by healthcare providers who understand both the diagnosis and the individual. Because every mitochondrial condition, and every person living with mito, is different, there is no single approach that fits all.

You are not alone on this journey. Small, thoughtful nutrition steps, taken one at a time, can add up to meaningful support over time, helping you navigate daily life with greater confidence and care.

Nutrition and Specific Mito Disorders

As mitochondrial disease represents over 350 different diagnoses, it makes sense that nutrition can play different roles depending on the underlying condition.

In some disorders, nutrition strategies are focused on preventing metabolic crisis; in others, the goal may be to reduce catabolic stress, support muscle health, or complement diagnosis-specific therapies. Broad clinical care standards emphasize individualized planning and avoiding metabolic stressors (like prolonged fasting), with diagnosis-specific guidance helping further shape nutrition approaches.

For FAOD, nutrition is often a central part of disease management. Because the body many not be able to use fats effectively for energy, maintaining a reliable fuel supply (often emphasizing carbohydrates) and avoiding fasting is critical. Clinical and guideline literature repeatedly highlights:

MELAS can involve high metabolic demand and multi-system symptoms (including GI and nutrition challenges), so nutrition strategies often emphasize maintaining energy balance and preventing catabolic stress during illness or poor intake.

Targeted supplementation is sometimes discussed in MELAS, most notably arginine and/or citrulline in relation to stroke-like episodes. The literature includes supportive reports and reviews, but consensus guidance also notes limitations and ongoing debate about strength of evidence and clinical use.

In MELAS, nutrition care often focuses on steady intake, illness-day planning, and discussing targeted therapies (like specific supplements) with a mitochondrial specialist because recommendations vary across clinics and individuals.

When muscle involvement is prominent, nutrition strategies often focus on supporting muscle maintenance and minimizing fatigue by ensuring adequate overall calories and protein, especially if appetite is low or unintentional weight loss is present.

Some research in mitochondrial disease populations (including those with muscle symptoms) suggests that insufficient intake is common, and that higher intakes of macronutrients (including protein) may correspond with better outcomes like muscle strength, lower fatigue, and improved quality of life, though this doesn’t mean “more is always better,” and personalization matters.

For mitochondrial myopathies, nutrition support often prioritizes “enough energy + enough protein,” using practical strategies (small frequent meals, energy-dense additions, smoothies) when fatigue or GI issues make intake difficult.

POLG-related disorders can present very differently from one person to another. Nutrition strategies commonly emphasize preventing catabolic stress (especially during illness or reduced intake) and maintaining consistent energy availability, an approach aligned with broader mitochondrial care standards.

In some POLG-related conditions, seizures can be a major concern, and dietary therapies (such as ketogenic-style approaches) may be considered in specialized contexts (typically for difficult-to-treat refractory epilepsy) under expert supervision, with careful monitoring and individualized risk–benefit discussions.

For POLG, the key nutrition themes are often “avoid catabolic stress, plan for illness, and create individualize dietary approaches with your mito specialist and care team,” especially if seizures or significant GI/nutrition issues are part of the picture.

Because mitochondrial disease includes many different conditions, nutrition approaches are rarely one-size-fits-all. Some disorders rely heavily on nutrition strategies to prevent metabolic crisis, while others focus on maintaining energy balance, supporting muscle health, or managing symptoms. Across many diagnoses, maintaining consistent energy intake and avoiding metabolic stress are key themes. Individualized guidance from a mitochondrial specialist or dietitian is important to determine the best approach.

Fasting and Mito

Fasting can significantly change how the body fuels itself. When you go without food for longer periods, the body shifts away from using incoming carbohydrates and begins relying more on stored fuels (including fats, protein/muscle). For individuals living with mito, where energy production may already be less efficient, this shift can add metabolic stress and may worsen symptoms for some individuals.

Clinical care standards for mitochondrial disease specifically emphasize preventing catabolism (a state where the body breaks down stored energy and tissue) and recommend avoiding prolonged fasting, particularly around illness, procedures or other stressors.

Because mitochondrial disorders are highly variable, always consult the specialist overseeing your mitochondrial disease care before trying fasting, intermittent fasting or any restrictive eating approach. The “right” strategy must match the diagnosis and the individual, not what is trending online.

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