Even in the womb (being a breach baby), Otis decided he was going to do things his own way. We had a normal start to life as a family, as normal as could be in a not so normal world full of lockdowns and coronavirus fears. Otis was meeting all his milestones with ease and we really felt as though we were nailing this parenting business.
Otis is 2 years old now and has grown to be a wonderful little boy with a smile that can melt your heart into a puddle of joy. We are in awe of his loving, gentle and kind nature, his playfulness and tight cuddles. Otis hasn’t had many opportunities to play with other children but he will be sure to capture the attention of adults and flirt relentlessly with nurses and consultants regardless of the distress of having a cannula inserted. He is so brave and forgiving.
Otis has a love of music and the sea. He will happily strum his ukulele and sing or make a dash for the water at any opportunity. He loves nothing more than to make people smile, Otis has so much happiness to share.
When Otis was 13 months old he had his 1 year immunization jabs and a visit from the Health Visitor for his 1 year review. She was happy with his development and we discussed his night feeding. She explained that night feeds were no longer necessary and that to continue was more for my comfort than for his benefit. We took her advice and my partner settled Otis when he woke for his feed instead.
The following morning our lives changed forever. Our little boy woke as normal and I brought him to our room for a feed as I would usually do. Although this time he didn’t take his feed and went back to sleep in my arms briefly. I assumed he may have been more tired than usual due to the change in our routine. When he stirred I took Otis to his room to change his nappy and clothes. On the way he let out a groan and vomited green bile-like liquid down my arm. Otis was never a sick baby so this immediately caused concern. I lay him back down and he became unresponsive. We rushed him to the hospital.
Upon arrival Otis was taken out of my arms and taken away through the Emergency Department to the resuscitation room. Before we could follow we were warned that there would be a lot of people with Otis and it might be a shock. Going into that room was like a gust of frenzy. There must have been 10 or more medical staff from different departments all frantically trying to revive our little boy. He would not wake up. Tubes, monitors, leads, everywhere. It was like being in the middle of a film set except there wasn’t a director calling cut. We wanted this to stop, it didn’t feel real but the scene just kept playing. It was horrific.
No one could explain what was happening or why it was happening. The more questions we asked, the darker the answers we received. I asked if he would wake up and if he might die. The response we received was not what we wanted to hear. I stopped asking questions after that because everything was out of my control. We needed to keep strong and pray for Otis to be ok.
It was explained to us that it looked as though Otis was experiencing metabolic acidosis and they administered bicarbonate to try to balance it out. At this point Otis was scoring 7 on the Glasgow Coma Scale. Coma. Our little boy was in a coma. It was decided that he needed to be intubated to keep him stable and transfer him to the nearest specialist children’s hospital which was an hour and 45 minutes away. Otis was taken by ambulance and we followed behind by car. I don’t know how Simon managed to drive us there. We were both so scared that Otis might not make the journey. When we arrived at the Paediatric Intensive Care Unit (PICU) we were given an opportunity to hold Otis’ hand while he lay quietly being monitored with leads connected from head to toe. He was so still, I recall quietly feeling for, hoping for any slight twinge in movement from his fingertips to mine, any indication that Otis was still with us. Hope was all we had, it was everything we had.
The following morning after staying at the hospital, I went to see our little boy. Due to Covid the PICU ward had a one-parent policy for visits. I was met by a nurse who told me Otis had caused quite the scene by removing the intubation himself. He was awake!! Flooded with relief I held our little boy so close but delicately as he was wrapped in a variety of cables. We are so grateful he woke up. Our fears didn’t end there -- what we didn’t know was that they were just beginning.
We spent two weeks in the hospital while Otis was being monitored, tested and given time to recover. The metabolic team had given Otis the diagnosis of VLCAAD, a condition where very long chain fats cannot be processed by the body. After NG tube feeding training and guidance on how to manage and treat this condition we were sent home together and started to get our heads around adjustments we were to make together as a family.
Simon received a call 3 months later from our metabolic consultant. She explained that following blood results Otis does not have VLCAAD but will need further testing from the genetics team to establish what it could be. We were back to square one with no idea what we are facing. Full exome sequencing testing took place and another 4-week wait to find out what we were up against. “Hope for the best, expect the worst” we were told.
June 9th 2021 we met with our metabolic team and genetic counsellors who explained to us that Otis has been diagnosed with TANGO2. We had no idea during the meeting if this was the best news or worst news as no one had ever heard of this disease before. You would expect your doctors to arm you with knowledge and to answer any questions about a new diagnosis. However, it soon became apparent that we would be joining Otis’ medical teams to try and research this rare disease and keep them up to date with anything we learn. We are all still learning.
We are so grateful to the TANGO2 community for your support and to the TANGO2 Research Foundation. It is thanks to you, the scientists and our fund and awareness raisers that we continue to have hope for Otis and his fellow warriors. There is nothing more we wish for than to discover treatment for this condition so Otis can live a fulfilled life full of health and happiness and continue to share the vast amount of joy he so loves to give.