The building blocks of proteins and vital energy sources for cell repair, growth, and development in the body. Some are “essential” to receive from diet, and non-essentials are made from metabolism of other amino acids in the body (e.g. tyrosine is made from the breakdown of phenylalanine). Inherited metabolic conditions involving protein occur when the body cannot break down certain amino acids. External reference: National Library of Medicine.
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flok’s method of evaluating protein values for specific foods to ensure our community has reliable nutrient data for dietary tracking, as product labels do not provide information down to the specific amount of an amino acid. Analysis is completed by testing a food product in two separate laboratories to detect small amounts of amino acids, before a dietitian reviews and calculates approximate values. The six studied amino acids include isoleucine, leucine, lysine, methionine, phenylalanine, and valine. External reference: flok
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A technology that analyzes data to quickly form insights. In healthcare this technology can be applied to diagnose, make predictions, monitor, and treat individuals. Machine learning is a specific type of AI used in healthcare. flok's data platform uses this technology to build models with potential to predict clinical biomarkers and health outcomes. External reference: foresee medical

Also called “biological markers”, biomarkers are measurable indicators of processes occurring in the body. In inherited metabolic conditions, these indicators can be used to identify the presence and progression of a diagnosis; they are often identified through a blood sample. E.g. An elevated leucine level in a newborn’s blood may be an indicator of MSUD. In a diagnosed person with MSUD, elevated leucine can result in a metabolic crisis. External reference: Science Direct
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Biological, physiological, and behavioral data from an individual. flok app pairs with a phone’s native health app to capture this information, such as heart rate, sleep duration, activity and calorie burn. External reference: Baylor College of Medicine
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The breakdown of complex molecules into their smaller forms, such as protein into individual amino acids. In inherited metabolic conditions, this may occur during an acute illness, infection, or fasting as the body begins to break down glycogen, fat and protein as an alternative energy source. Most conditions require a “sick-day protocol” and guidance from a registered dietitian to prevent a catabolic state, as it can be very serious and require hospitalization if it leads to metabolic decompensation. External reference: National Library of Medicine
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An inherited metabolic condition caused by reduced activity of the cystathionine beta-synthase enzyme causing elevated levels of homocysteine and methionine in the blood. Without proper treatment, individuals with HCU experience developmental delay, and have increased risk for blood clots, stroke, osteoporosis and lens dislocation. Treatment requires a lifelong low-protein diet, methionine-free formula, and medication to lower homocysteine levels in the blood. External reference: NORD
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Brenda Winiarksi founded Cook for Love when her daughter was diagnosed so that no one with a protein-limited diet would ever have to limit their enjoyment of food. With credentials from the Chef Training Program at the Natural Gourmet Institute for Health and Culinary Arts, she developed not only a library of recipes, but a supportive and creative community that loves to cook low-pro. In 2018, Cook for Love became a project of (then) National PKU News and remains a key part of flok. Backed by the nutritional engine of flok, CookForLove.org allows community members to create and share recipes with confidence.
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Collects data from the flok app with user consent to better understand the lived experience of individuals with inherited metabolic conditions as it relates to diet, physical activity, mood, symptoms, and other provided health information. The platform supports predictive modeling and has potential to predict clinical biomarkers, outcomes, and guide future research and treatment development. External reference: flok
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A set of genes an individual carries. It can also refer to the variant of a gene at a certain place in the DNA where the gene is located. E.g. A mutation of the gene for phenylalanine hydroxylase (PAH) causes PKU, and the specific variation of the mutation is its genotype. External reference: NIH Human Genome Research Institute
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A group of genetic conditions that affect the body’s ability to convert or breakdown substances in food into energy and remove their toxic waste from the body due to a faulty enzyme. There are two categories of these conditions: enzymatic deficiencies related to fat and carbohydrate conversion, and enzymatic deficiencies in specific amino acids that break down food. They can impact multiple organs and systems in the body with clinical consequences ranging from mild to severe. These conditions require early detection and lifelong treatment for optimal health outcomes. Treatment includes a protein-restricted diet, medical formula, and for some, medications or vitamins; there are currently no cures. flok serves the following amino acid-related conditions: Classical HCU, MSUD, Organic Acidemias, PKU, Tyrosinemia, and Urea Cycle Disorders. External reference: Cleveland Clinic
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*flok uses the term inherited metabolic conditions
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The primary treatment for inherited metabolic conditions to prevent toxic levels of amino acids and their metabolites in the blood and brain. Depending on the condition, an individual will limit intake of a certain amino acid found in natural protein. Limitation of natural protein in the diet requires addition of a medical formula free of the offending amino acid to ensure adequate protein intake for normal growth and development. Diet may also include modified foods that resemble standard foods but have reduced protein levels to meet nutritional requirements (e.g. pastas and breads manufactured by medical food company).
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A type of artificial intelligence in which models are built using data to identify patterns and make predictions. In the context of flok’s data platform, these models have potential to predict clinical biomarkers or health outcomes. External reference: National Library of Medicine
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An inherited metabolic condition caused by a deficiency of the branched chain alpha-keto acid dehydrogenase enzyme complex that disrupts metabolism of the amino acids leucine, isoleucine, and valine. If left untreated, individuals with MSUD experience developmental delay, pancreatitis, and osteoporosis. Even with early and consistent treatment, individuals are at risk for metabolic crisis from acute illness or infection that can result in coma and death. Treatment requires a lifelong low-protein diet and formula free of leucine, isoleucine, and valine. External reference: Cleveland Clinic
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Includes both medical formulas consumed to provide essential nutrients minus significant amounts of the amino acids that cause toxicity in a particular condition, and low-protein modified foods manufactured by a medical food company.
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An acute process that occurs from the buildup of toxic metabolites in the body, generally related to catabolism or increased consumption of a food component that the body cannot break down. Triggers include fasting, illness, and infection that lead to catabolism, then metabolic crisis. Symptoms may include vomiting, lethargy, seizures, and respiratory problems; hospitalization is needed for specialized management. Of the inherited metabolic conditions supported by flok, MSUD, Organic Acidemias, and Urea Cycle Disorders are prone to an acute metabolic crisis. Also: Hyperammonemia
​External reference: Up to Date
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A collection of information on a condition from onset to resolution, or end of life. They provide insight into condition characteristics, demographics, environmental factors, and other things that impact a condition’s progression and outcome. They also enhance understanding of daily life with a condition, highlight areas of unmet medical need, and inform research priorities. Natural history studies are beneficial to developing drug and treatment interventions. Flok’s app and data platform support the creation of natural history studies, as few exist for inherited conditions of protein metabolism. External reference: National Library of Medicine
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A class of inherited metabolic conditions caused by deficiencies in enzymes that break down certain amino acids, which vary by condition type, resulting in a buildup of acids in the body. Without treatment, individuals with OAs experience poor feeding, developmental delay, muscle stiffness, and difficulty with coordination; presenting symptoms and severity vary by type. The four main types are Isovaleric Acidemia, Maple Syrup Urine Disease, Methylmalonic Acidemia, and Propionic Acidemia. Treatment varies by condition type, but generally requires a lifelong low-protein diet, medication, and medical formula. External reference: Science Direct
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The observable or measurable characteristics associated with a genotype. In inherited conditions of protein metabolism, the gene variants (genotype) of a condition may correlate with age of onset, severity, protein tolerance, or condition subtype, among other factors yet to be studied (phenotype). External reference: NIH Human Genome Research Institute
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An inherited metabolic condition caused by deficiency of the enzyme phenylalanine hydroxylase resulting in elevated levels of phenylalanine in the blood. Without early treatment, individuals with PKU experience profound developmental delay and behavioral dysregulation. Treatment requires a lifelong low-protein diet and phenylalanine-free formula. Some individuals are responsive to medication that liberalizes the diet by increasing the amount of protein the body can metabolize. External reference: Medline
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flok’s process for providing accurate and reliable estimations of protein amino acid values in store-bought food. First, estimation is supported by nutrient data provided by the food brands when available, then a flok dietitian determines amino acid:protein ratios in a product and compares it across similar foods in the flok food database for consistency. External reference: flok
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Refers to the “safe” protein an individual gets from metabolic formula – the protein with the offending amino acid(s) removed. For example, an individual with PKU might have daily protein tolerance of 8 grams, but intake 45 grams of PE from phenylalanine-free formula.  
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The amount of protein, or other amino acid, that an individual with an inherited metabolic condition is allotted within a given day to maintain therapeutic biomarker levels. A protein value is determined by a dietitian and varies between conditions and individuals based on genetics, medications, and other factors.
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An approach to counting protein or amino acids, unique to those with PKU and Tyrosinemia, that reduces the daily tolerance or amount by up to 30 percent, so that certain food items are not counted towards the daily protein/amino acid limit. This dietary approach is meant to make protein tracking easier and should only be used under the guidance and support of a registered dietitian. External reference: flok
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An inherited metabolic condition caused by the deficiency of the enzyme fumarylacetoacetate hydrolase resulting in elevated levels of tyrosine and succinylacetone in the blood. There are three different types with varying severity and symptoms. Type 1 is the most common and severe; without early treatment, individuals with type 1 experience developmental delay, corneal crystals, and liver and kidney failure. Treatment requires a lifelong low-protein diet, tyrosine and phenylalanine-free formula, and medication to lower succinylacetone levels in the blood. External reference: Medline
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A class of inherited metabolic conditions caused by defects in one of the enzyme or transporter molecules that break down nitrogen in the bloodstream, causing a toxic accumulation of ammonia in the body. Without treatment, most individuals with UCDs develop cerebral edema, neurological problems, and coma. Treatment usually involves a lifelong low-protein diet and medical formula; some require tube feeds and nitrogen-scavenger medications to prevent ammonia buildup in the blood. Even with early and consistent treatment, individuals are at risk for hyperammonemia from acute illness or infection that can result in coma and death. External reference: National Library of Medicine