Sophie is one of a set of twins born extremely prematurely at just 24 weeks. Both my girls survived but not without complications and a difficult NICU stay with various challenges for them both. Within the first couple of weeks Sophie suffered a collapsed lung and a significant bleed to her brain. She was closely monitored under the care of the NICU staff but at that point there was no way of telling if the bleed had caused long term brain damage.
At 8 months old Sophie was diagnosed with Cerebral Palsy after it became clear that Erica (Sophie's twin) was achieving milestones that Sophie couldn't, like pulling to sit, hands to mouth or rolling. From here she had an NG tube for feeding for approximately 2yrs until she had her first operation and a gastrostomy tube fitted. Within this time period Sophie was also diagnosed with Auditory Neuropathy, Epilepsy, Extropia and scoliosis.
In 2011 Sophie was really struggling with reflux which was causing her to aspirate (milk onto her lungs) and causing serious chest infections like pneumonia. A decision was made for more surgery a 'nissens fundoplication' this was surgery to tighten her oesophagus to stop the reflux. The surgery went well but Sophie had an adverse reaction post surgery and her stomach and intestines shut down. She could no longer tolerate any feed into her stomach and was at risk of losing more weight. To maintain Sophie's nutrition she had another operation to fit a Jejunostomy. This is a feeding button directly into Sophie's intestine, bypassing her stomach.
In 2013 Sophie had major surgery to have a Baclofen Pump fitted to help with her spasticity and tone as a way to keep her comfortable and improve her quality of life. By this point she had also started to have regular Botox injections in her upper limbs. Between 2012 and 2014 Sophie had many emergency stays to the surgical ward following blocked buttons that then needed surgery to repair. In 2014 Sophie had several operations all at once for her stomach and intestines resulting in 8 hrs in theatre and the need to be ventilated in intensive care post surgery.
In the summer of 2014 Sophie became very unwell with her gut again and was closely monitored by the surgical team but deteriorated very quickly with cellulitis and pain. From here she was put on TPN for all her nutrition as her stomach and intestines had shut down again. Sophie throughout the years, due to prem birth and many procedures, has always had poor access with her veins so a decision was made to fit a PortaCath. (A central line to Sophie's heart) Sophie had her TPN administered via her port to give her body chance to recover before returning to her specialist milk feed. She still has the port and we use it for IV antibiotics or bloods when needed. At the same time Sophie was introduced to the long term ventilation team to monitor her breathing as her scoliosis and respiratory rate had changed significantly over the last year. The team started Sophie on CPAP ventilation over night to help her lungs and tiredness levels during the day.
In Feb 2015 Sophie was starting to obstruct her airway so my husband and I were trained to insert an NPA (nasopharyngeal airway) to keep Sophie's airway open in an emergency. Sophie really started to deteriorate at this point. We noted changes with her airway, pain, tone and seizures. In June 2015 with inexplicable episodes Sophie was admitted to intensive care with suspected status dystonicus. She remained in intensive care for 5 wks with many changes including pressure support ventilation 24/7 and a massive increase in controlled drugs. Sophie's life was turned upside down again. It took a lot of co-ordination as initially the intensive care team didn't think it was manageable or safe for us to care for Sophie at home but with the support of Sophie's respiratory team and a Wellchild nurse we were able to bring Sophie home just before her 10th birthday.
Sophie requires intensive care at home and in the community and it is 24/7, she has to have someone with her at all times as her airway is unpredictable and unsafe when teamed with her dystonia which can be extremely challenging and painful for Sophie. Since her stay last year Sophie has also been on clinical trials for medical cannabis and due to the deterioration of her spine we are now in discussions with her team regarding spinal surgery for next year. We have been told it will be a massive undertaking for Sophie but one that could improve her quality of life greatly.
So this is Sophie and all of her medical challenges but all of that aside (and I'm hoping you can see from her picture) she is without a doubt the strongest and happiest person I know. I tell people regularly she has this amazing zest for life and is intrigued by everything and everyone around her. Sophie is a fighter and enjoys life to her fullest, we just have to manage the risks and care safely so she can get out there and be who she wants to be!