Home / Health / Genetic Tumors Devastate Family Across Generations
Genetic Tumors Devastate Family Across Generations
4 Oct
Summary
- Von Hippel-Lindau syndrome causes tumors and cysts in various organs.
- Eight family members tested, four positive, three with developed tumors.
- KEM Hospital uses amlodipine to manage blood pressure in VHL patients.
A 48-year-old Andheri resident, Kamal, and his family have been battling Von Hippel-Lindau (VHL) syndrome for two decades. This autosomal dominant genetic disorder has claimed his father and afflicted Kamal, his sister, her daughter, and now his 13-year-old nephew. The condition causes tumors and cysts in organs such as the brain, kidneys, and adrenal glands.
Kamal's own journey with VHL began in 2009 with a brain tumor, followed by more in 2016 and kidney lesions by 2019. He finally received a diagnosis at KEM Hospital, Parel, in 2019. Genetic testing has confirmed VHL in four of eight tested family members, with three already developing tumors.
Younger relatives, like 13-year-old Harsh and 23-year-old Bhagwat Giri, have experienced severe symptoms including dangerously high blood pressure, fever, and seizures, necessitating urgent treatment. Bhagwat underwent surgery at KEM Hospital in 2021, and his sister Vaishnavi was operated on recently after exhibiting similar signs.
Dr. Tushar Bandgar, head of endocrinology at KEM, explained that adrenal gland tumors (pheochromocytomas) are common in VHL, causing surges in blood pressure. Surgical removal of these tumors carries significant risks due to potential blood pressure spikes. The hospital is using amlodipine as a first-line preoperative medication, showing promising results in managing patients, an approach that differs from traditional methods with more side effects.
