Rare diseases as a category aren’t that rare if you consider they impact 30 million Americans and 400 million people around the world. Not to mention their families, who often become caregivers. Yet, with nearly 7,000 rare diseases (identified as of today…), each is unique. Many are so unique physicians lack knowledge on how best to care for these patients – in fact, most rare diseases take an average of 4-5 years to even diagnose.  

This all gets more real when a rare disease meets your family. My daughter, Taylor, was diagnosed with a rare neurological condition, agenesis of the corpus callosum (ACC), when she was 8 months old. Given her brain malformation, it was somewhat expected when she had her first seizure about a year later. After years of heartbreaking and terrifying seizures, we finally found the right medication mix for her that largely controls her seizures. Finally, when she was 13, we received another surprise – after years of genetic testing, she was also diagnosed with an even more rare chromosomal syndrome, Mowat-Wilson. 

Truveta Chief Growth Officer, Lisa Gurry, shares her family’s personal diagnostic journey with rare diseases and the role Truveta can play. (Video filmed in 2021)

Our experience isn’t unique. Along the way, well-intentioned physicians and a small army of specialists struggled to learn more about her condition, which many of them had never seen. They weren’t sure how to help her – so we had to learn through trial and error.  

Taylor was my foremost inspiration to help build Truveta. I saw the potential instantly. What if those physicians could have easily learned from patients like Taylor? What if researchers interested in these conditions could easily find similar patients to study? What if care pathways were instantly clear because the healthcare community could easily learn from others? 

Now, Truveta makes this possible. In Truveta Data, there are 3,700 de-identified patients with ACC. Almost 4 million with epilepsy. And, incredibly, 63 patients with Mowat-Wilson (likely many more undiagnosed, as the condition didn’t exist until 1998). Researchers can uniquely study each of these conditions individually and for conditions that overlap, which is common. I can’t wait for researchers to learn more about these conditions and help the next family like mine.  

These are just a few of the thousands of rare diseases researchers can learn more about from Truveta.  

Advancing knowledge of rare diseases

Rare diseases, often cause patients to suffer needlessly due to lack of knowledge about their condition, with less than 10% of rare diseases having an approved treatment available.  

Enter the availability of nearly 100 million patient journeys from across the US, with daily updated data from more than 30 health system members through Truveta. Truveta Data is the most complete, timely, and clean data on US health, with billions of data points to enable statistically significant research on any drug, disease, or device. Now, instead of taking multiple research grants, full-time staff, and potentially years of combing through data to build a patient population for a rare disease, those populations can be found in near real-time.  

And, Truveta Data makes it possible to find enough patients to study via the aggregate, de-identified dataset. Individual health systems may have only 5 or fewer patients with a particular condition, an insufficient population to make meaningful strides in disease research. Truveta’s breadth and depth of data make it possible to learn more from statistically representative populations. 

Read on for a few more examples of rare disease datasets available for research today with Truveta.  

Truveta Data: Amyotrophic lateral sclerosis (ALS)

 Amyotrophic lateral sclerosis (ALS), aka Lou Gehrig’s disease, is a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord. There are approximately 32,000 ALS patients in the US, which sadly is usually fatal within 5 years of symptoms onset. Current treatments slow the progression of the disease, but none reverse or cure it. With 13,000 ALS patient journeys, Truveta offers a large population for study, relative to the rareness of the disease. 

There are currently four treatment options available for ALS, two of which – Relyvrio and Qalsody – were approved within the last year. With Truveta Data representing the full diversity of the United States, researchers can analyze daily updated data for the full medical record, including newly launched medications, without months-long delays. With Truveta’s rich data extracted from clinical notes, researchers can learn about nuanced clinical concepts like patient-reported outcomes, quality of life, and ventilator parameters. 

Truveta Data: Cystic Fibrosis

Cystic Fibrosis (CF) is a progressive, inherited genetic disorder that primarily affects the lungs and digestive system. Once considered a children’s disease, the predicted survival rate has increased over time thanks to universal infant screening and new therapeutics which have profoundly changed the course of this disease. The predicted survival age is now  56 years old, up from 38 years old just 10 years ago.

Excitingly, there are approximately 80+ companies and 80+ drugs in the R&D pipeline for CF today, meaning now more than ever, researchers will benefit from access to patient data representing the full diversity of the United States. Truveta Data enables capturing of the entire patient journey for those living with CF (over 11,000 total patients in Truveta Studio); from time to initial diagnosis, to prescription of CFTR modulators (the breakthrough medications that have significantly increased longevity for these patients), to outcomes including hospitalizations, lung transplantation, and clinical improvement.

With more than 17% of daily clinical care across all 50 states from 800 hospitals and 20,000 clinics, plus comprehensive social drivers of health data, Truveta offers the most complete and representative data to study CF and other rare diseases.  

I am so excited to see how Truveta can help these patients, their families, and the healthcare community who cares for them.  

 – Lisa Gurry  

 

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