Olivia’s Type 2 Cleft Journey
Australia
By Olivia
I can’t remember a time in my childhood where my swallowing didn’t bother me. Aspiration (inhaling liquids into my lungs) was just there in the background. But as far as I understood, my aspiration was not abnormal. And as far as I knew, all of my birth defects were all fixed at birth.
I was born in 2005 in Adelaide, South Australia. My mum had polyhydramnios (too much amniotic fluid) during her pregnancy, but my parents were not told I would have any problems with my development.
Within 40 minutes of my birth, it was found that I had a tracheoesophageal fistula (TOF) and oesophageal atresia (OA) (Type B). OA meant that my oesophagus was not connected to my stomach and TOF meant that my oesophagus was abnormally connected to my trachea (windpipe).
At 6 hours old, I went into major surgery to repair my TOF and OA. My early days were spent in PICU, filled with complications and challenges.
At 1 month old, my type 2 laryngeal cleft was identified through a bronchoscopy. Initially doctors wanted to see if the cleft would self-resolve, but after repeated aspiration pneumonias, my cleft was repaired endoscopically at 4 months old. I also received a Nissen fundoplication and was gastrostomy fed until I was 3 years old.
From 4 years old until my early teenage years, my conditions played a small part in my life. I was able to play soccer and attend mainstream school. I still love watching and supporting the Matildas, the Australian Women’s soccer team! During these years there were subtle things that were not quite right. I would always choke on water when swimming and always struggled lying on my stomach. I felt as though I was drowning in my own saliva and would cough and choke. I assumed that this was all normal although in hindsight, it was aspiration.
At around 15 years old, I started choking when drinking water. I would aspirate a few times per week, which continued to worsen over time. Over a few months, I started aspirating everyday. I then started using thickener. I progressed up the thickener levels, until eventually I was aspirating even the thickest consistency, level 4.
After a month of aspirating everything, I presented to an emergency department for severe dehydration. In hospital, I saw an ENT and a speech pathologist, who both determined that it was unsafe for me to have any oral fluids, so I received IV fluids. A few days later, I had a bronchoscopy which identified a recurrent type 2 laryngeal cleft, thought to have grown as I grew throughout childhood. The ENT injected Restylane filler into my cleft to temporarily address my aspiration. The injection was a success, and I was able to drink thin fluids again. However, it started to wear off quickly, so I had another Restylane injection two months later.
At 17 years old, my medical team and I decided to place a nasogastric feeding tube. I received all my fluids through this tube. This made my daily life much simpler, as I didn’t need to worry about aspirating or not getting enough hydration. In later years, this changed to a PEGJ feeding tube as a long-term device.
The month after my 18th birthday, I had an endoscopic repair of my laryngeal cleft which improved my aspiration a lot. At first, I convinced myself that I was aspirating ‘the normal amount’, however I was still regularly aspirating. These aspirations felt different to some of the aspiration I was having before my repair, they felt deeper in my chest and less predictable. I knew it was time for another barium swallow.
As an adult, this barium swallow was conducted in a different way to all the ones I had as a child. There was no aspiration from my cleft, but it showed a source of aspiration at the level of my collar bones.
After a bronchoscopy at 19 years old, they confirmed I had a second tracheoesophageal fistula. This TOF had been there from birth and grew bigger as I grew. In August 2024, I had a successful open TOF repair (through my neck and thyroid). This was an invasive and challenging surgery. Thankfully, it has healed very nicely, and my team is confident that it will remain closed permanently.
I have ongoing conditions related to my airway and digestive defects. I have postural orthostatic tachycardia syndrome which was triggered by severe dehydration before my Restylane injection. I also have reflux disease, gastroparesis and tracheomalacia which will each be life-long. Gastroparesis means that my stomach is partially paralysed and holds onto food much longer than normal. I have a PEGJ tube which helps me receive extra nutrition and all of my hydration.
Advocating for myself has become a core part of my medical journey. I have learned to advocate for myself, speak up when things are not right and seek out doctors who take my concerns seriously. If I could give any advice, I would say to trust your instincts. Finding your voice to advocate for yourself or your child can be very challenging, especially if you do not feel listened to, however it is so important and can be empowering, too.
I am currently studying nursing at university and work as an advocate and advisor for the department of health in my state. I understand what a profound impact healthcare staff can have on a patient’s experience and wellbeing. I hope to use these experiences to make a positive impact in this area.
I am still learning to accept my unique path, but I am so grateful for the life I have right now even if it challenges me. The support of my family, medical team, incredible friends and other people living with the same health conditions have been and continue to be remarkable. My current medical team is full of compassion, empathy and expertise. I also can’t forget the infinite support of my parents and younger brother.