Three years ago, a 45-year-old woman walked into Parth Shah’s office with anemia and a swollen spleen. She’d had two strokes and, for 30 years, couldn’t eat a full meal.
Nobody knew why.
Parth Shah, MD, a hematologist at Dartmouth Cancer Center and director of genome informatics at Dartmouth Health’s Dartmouth Hitchcock Medical Center (DHMC), had a fresh way to look at her symptoms. As part of DHMC’s joint clinic for genetic vascular disorders, Parth Shah, in consultation with DHMC clinical geneticist Nidhi Shah, MD, ordered comprehensive genomic and transcriptome sequencing via the DH-CancerSeq assay. That testing revealed PROS—a disorder so rare most physicians never encounter it.
PROS, or PIK3CA-Related Overgrowth Spectrum, is caused by mutations in PIK3CA, a gene that normally regulates cell growth. That gene is often linked to breast cancer, as mutations in it can drive the uncontrolled growth of breast cells. In PROS, however, the impact of mutations strikes in the womb, leading to the symptoms experienced by Parth Shah’s patient.
Diagnosis in hand, Parth Shah prescribed a breast cancer drug that counteracts PIK3CA. Within three months, his patient’s spleen had normalized and she could eat full meals again—completely transforming her life after three painful decades.
Five years of BabySeq data show that parents who receive genetic results do not “medicalize” their children: They don’t rush to emergency rooms more often or spend more on healthcare out of worry.
Nidhi Shah is also studying how well genomic medicine functions outside major urban hospitals. Since many families she sees live in rural New Hampshire and Vermont, she is keenly aware that an early diagnosis matters only if patients can realistically act on it.
Pattern Recognition
Not every rare disease presents at birth. Many surface slowly, after years of vague and disconnected symptoms. And the challenge isn’t only getting the right genetic test. Often, it’s identifying that something is wrong in the first place and getting the patient to the right specialist. That gap led Katharina Schmolly, MD, RES ’25 MPH ’28, founder and CEO of zebraMD, who worked in Parth Shah’s lab and trained under Nidhi Shah during her internal medicine residency at DHMC, to build something different.