Wegener’s Granulomatosis, a rare disease that is 1 in 10,00,000 children

A 7-year-old girl was admitted to Aster CMI Hospital with alarming breathlessness and significant pallor. A detailed medical evaluation revealed a rare, life-threatening autoimmune condition, Wegener’s Granulomatosis (now known as Granulomatosis with Polyangiitis or GPA), which causes spontaneous bleeding in the lungs, leading to acute respiratory failure.

Due to the severity of her condition, the Pediatric Intensive Care Unit (PICU) team, in collaboration with the ECMO team, ECMO is a complex and high-risk procedure, particularly for a child of this age, but with the parents’ consent, it was deemed necessary to stabilize the heart and lung function.

“As a team, we understood the extraordinary challenges posed by her condition, which was compounded by her young age. ECMO was our best option to support her vital functions and give her a fighting chance,” said Dr. Arun V, Lead Consultant for ECMO. “The journey has been challenging, but her resilience and the collective expertise of our team have brought us to this remarkable recovery.”

The treatment progressed, complications arose due to prolonged ventilation, which included severe sepsis and the need for tracheostomy care. The MICU team, managed the pulmonary hemorrhage, acute ventilatory crisis, and kidney injury that were part of the illness, working tirelessly to keep the child stable. To address the underlying autoimmune disease, the child was started on immunomodulation and plasmapheresis, which helped in controlling the disease’s progression. Over time, and with the support of ECMO, the child’s condition improved, and she began to respond positively to the treatment.

“The courage and determination shown by this little girl are truly inspiring. It has been a privilege to witness her incredible progress,” said Dr. Karthik Arigela, Consultant in Pediatrics and PICU. “This case highlights how cutting-edge technology combined with skilled multidisciplinary care can lead to life-saving outcomes.”

The child is now on the road to recovery and is preparing for discharge. While she will require ongoing oxygen therapy at home, her story exemplifies the extraordinary possibilities of modern medicine.

The patient’s recovery is an indication of the coordinated efforts of the multidisciplinary team of doctors at Aster CMI Hospital. This successful case further reinforces our dedication to pushing the boundaries of medical science and providing the best possible outcomes for our patients, said Mr. SGS Lakshmanan, COO of Aster CMI Hospital.”

Aster CMI Hospital continues to be a pioneer in offering advanced medical treatments and remains dedicated to providing exceptional care to its patients.