In 2004 I was diagnosed with idiopathic subglottic stenosis (iSGS) after two years of misdiagnosis. Initially I was wrongfully diagnosed with asthma, investigated for a heart murmur I didn’t have, and told I was having panic attacks. Eventually, through sheer determination and seeing many doctors, it was discovered I was breathing through an airway the diameter of a cocktail straw. I ‘wheezed’ on both the inspiratory and expiratory breaths (known as a stridor), which sounds a little like how Darth Vader breathes. Every little action was exhausting, making the bed almost impossible. I could not talk while walking. I struggled to eat and breathe. This rare disease is autoimmune in its nature, and impacts women 98% of the time. It acts by building up scar tissue in an area of your airway, just below the vocal cords, which closes up over time. Two women in a million will be diagnosed with iSGS. There is no cure, only various treatments which can put the disease into remission. I have had 21 dilation surgeries since diagnosis – these are simple day surgeries conducted under a general anaesthetic which use a laser and/or a balloon to stretch or dilate the area of the trachea which is closing up. I have also had a major tracheal reconstruction surgery, which involved removing the whole area of airway which was scarring in the hope of getting a longer period without surgery, but unfortunately I got a new stenosis (narrowing) after less than three years. Treatment for this disease is largely experimental and undertaken by an Ear Nose and Throat (ENT) doctor experienced in laryngology. Not all ENT doctors are experienced in laryngology nor have they all heard of this disease, therefore many patients continue to be misdiagnosed even after meeting a doctor in the right specialty! Some patients get a year or longer between operations, whereas others get six months or less. If a patient falls pregnant, it can often accelerate the disease, indicating there is perhaps a hormonal element to it. It is a challenging disease to diagnose and treat, with patients usually becoming good friends with their doctors as they continue to see them for many years. It is certainly a disease that requires more research to understand its nuances and to help control this disease.
