Doctors perform High-Risk Surgery to give a better life to a 9-year old boy who suffered from rare Muscular Dystrophy
Pune, July 31, 2019: Nine-year-old Ashish (Name Changed) is all charged up as he starts his school back at Nagpur. Pretty incredible given his many struggles at a young age as he suffered and overcame comorbidity with a rare degenerative disease-Duchenne Muscular Dystrophy (DMD).
After being diagnosed with a rare disease of DMD, the family could do nothing but assess their options and see what the best course of action would be for him and his future. Suffering from DMD which was diagnosed at the age of 5, Ashish had progressively increasing bilateral Lower limb weakness and was on steroids. He also took stem cell therapy 1 year back. His comorbidity condition got worse due to enlarged tonsils and adenoids (swelling at the back of nasal passages) because of which he had difficulty in breathing and was very uncomfortable and not able to sleep due to obstruction of the air passage.
His problem could be solved by removing tonsils and adenoids through a surgical procedure. But doctors were not ready to operate due to the high risk condition of DMD. Without proper plan carrying operation was risky as chances of intraoperative and postoperative complications were very high which could have also led to the death of the patient.
Considering the high risk of DMD, initially, doctors were reluctant to carry out the surgery. Parents came with a hope to Surya mother and child superspeciality hospital and after thorough investigations of the patient for any cardiac and respiratory problems Dr. Sachin Shah, Director, Intensive Care Services took up the challenge to perform a high-risk surgery to give a better life to the patient.
A multidisciplinary team, comprising senior pediatric anesthetists Dr. Mohan Swami & Dr. Jayaprakash Galpalli, ENT surgeon Dr Smita Choutha and pediatric ICU experts Dr. AmitaKaul and Dr. Ganesh Shiwarkar, led by Dr. Shah planned a surgery. The patient underwent adenoidectomy& tonsillectomy, with bilateral grommet insertion.
Dr. Shah informed, “After the surgery, the patient was successfully extubated with good respiratory effort. Moreover, he did not require any post-operative ventilation. The patient was observed in PICU for one day after surgery to monitor for muscle weakness and rhabdomyolysis. After 24 hours he was started on oral liquids and discharged home on the third postoperative day. Currently, the patient is asymptomatic and able to sleep properly.”
Duchenne Muscular Dystrophy is a genetic disorder and a life-threatening condition as it results in progressive muscle degeneration and weakness. According to experts, muscular dystrophies are a group of diseases that make muscles weaker and less flexible over time. Due to a genetic disorder, muscles’ health deteriorates gradually. This rare disease is normally found in early childhood and children with DMD have a hard time standing up, walking, and climbing stairs. Apart from physical disability, children can also have heart and respiratory problems. Ashish’s condition, due to comorbidity, was deteriorating fast and required advanced medical interventions.
Dr. Sachin Shah further commented, “This is an unprecedented example of multidisciplinary tertiary care for a child that was possible in Surya Mother and Child Super Specialty Hospital with the state-of-art PICU, OT and clinical expertise in pediatric anesthesia and pediatric critical care. The other concern that could have surfaced was postoperative ventilation and the anesthetic management of such patients is complicated not only by muscle weakness but also by cardiac and pulmonary manifestations.”
