THE EARLY DAYS
As the weeks went on at home, the signs that something was really not right for our son became obvious. We had a tiny baby boy who could not drink properly from the bottle and who didn't breastfeed well, he spent a lot of time screaming out in pain during feeds. It seemed that he could either feed or breathe but was not able to manage both at once. He looked unwell, was not particularly responsive to his surrounds and he was often struggling with his breathing. Alarm bells were going off in our minds. We continued to have contact with our local paediatrician, trying to gain an understanding of what was going on. Finally it all became too much to manage at home, and a trip to the local emergency department when Jakob was 3 months old resulted in him being admitted to hospital due to low oxygen saturation levels and for the issues with his feeding. The need for oxygen therapy was new, and questions were being asked as to why this was happening.
After a number of days with no answers he was transferred to the Royal Children's Hospital (RCH) in Melbourne for further investigations. On arrival at the hospital Jakob went into respiratory failure, he was rushed to the emergency department. He required intubation and a ventilator was used to support his breathing. He was moved to the Neonatal Intensive Care Unit (NICU). Chest x-rays revealed Jakob had changes in his lungs, and the suspected cause was aspiration (taking milk and saliva into his lungs). He had developed aspiration pneumonia and was unable to breathe properly because of this. He spent a few weeks in the NICU being stablised, then moved to the Special Care Nursery and finally into a general medical/respiratory ward. Many specialist teams became involved in his care, investigating his symptoms, completing numerous tests and hypothesizing what was happening for Jakob. These tests included a skeletal survey, an MRI, a lumbar puncture, ultrasounds, x-rays, swallowing studies and numerous blood tests used for genetic and metabolic testing. After 8 weeks in hospital we had a child who was medically stable enough to come home on continuous oxygen and with naso-gastric feeding, but with no definite answers as to what was 'wrong' with Jakob.
Once home, our life of therapy appointments, medical reviews and daily medical interventions continued. Doctors had identified that Jakob has differences in the way his bones form and grow, leading to his small size and proportional differences in his arms and legs. His skull shape and formation is also different. There are issues with his lung functioning. He has a hearing impairment. He has a large portwine mark across his face, over his head and down his back. He has hypotonia (low muscle tone). Nothing however could be found to tie all these symptoms together. We continued to battle issues with feeding and vomiting. A planned admission was required when he became too unwell to continue to feed at home. Trying to keep Jakob well was what our life as a family was all about.
At about 8 months of age Jakob started to show signs that he was becoming unwell again. This time it was related to his breathing. After a month of trying to manage him at home Jakob required hospitalisation. This admission was to be a long one. His respiratory function declined rapidly and he needed much higher flow rates of oxygen. He became extremely susceptible to viruses. Doctors suspected micro aspiration again. Jakob required stays in the Paediatric Intensive Care Unit (PICU) on three occasions, following episodes of respiratory failure. He again needed breathing support, this time in the forms of high flow oxygen and C-PAP. In between the admissions we had two failed discharges home, each time having to return by ambulance within days of leaving the hospital.
Eventually, and reluctantly, a decision was made for Jakob to have a fundoplication. A procedure in which the fundus (top right hand part of the stomach) is wrapped around the lower end of the esophagus to create a tightening. The idea is that it makes it more difficult for the stomach contents to come back up out of the stomach and for the person to then aspirate on them, something they felt may be happening for Jakob. At the time of the fundoplication a percutaneous endoscopic gastrostomy (PEG) was also performed, inserting a tube directly into Jakob's stomach for feeding. This was a high risk surgery for Jakob and he required admission to the PICU post surgery. One month after Jakob's surgery, and after 4 months in hospital, he was able to come home again.
To learn more about Jakob's progress post his hospital admissions and his more recent progress, click here.
After a number of days with no answers he was transferred to the Royal Children's Hospital (RCH) in Melbourne for further investigations. On arrival at the hospital Jakob went into respiratory failure, he was rushed to the emergency department. He required intubation and a ventilator was used to support his breathing. He was moved to the Neonatal Intensive Care Unit (NICU). Chest x-rays revealed Jakob had changes in his lungs, and the suspected cause was aspiration (taking milk and saliva into his lungs). He had developed aspiration pneumonia and was unable to breathe properly because of this. He spent a few weeks in the NICU being stablised, then moved to the Special Care Nursery and finally into a general medical/respiratory ward. Many specialist teams became involved in his care, investigating his symptoms, completing numerous tests and hypothesizing what was happening for Jakob. These tests included a skeletal survey, an MRI, a lumbar puncture, ultrasounds, x-rays, swallowing studies and numerous blood tests used for genetic and metabolic testing. After 8 weeks in hospital we had a child who was medically stable enough to come home on continuous oxygen and with naso-gastric feeding, but with no definite answers as to what was 'wrong' with Jakob.
Once home, our life of therapy appointments, medical reviews and daily medical interventions continued. Doctors had identified that Jakob has differences in the way his bones form and grow, leading to his small size and proportional differences in his arms and legs. His skull shape and formation is also different. There are issues with his lung functioning. He has a hearing impairment. He has a large portwine mark across his face, over his head and down his back. He has hypotonia (low muscle tone). Nothing however could be found to tie all these symptoms together. We continued to battle issues with feeding and vomiting. A planned admission was required when he became too unwell to continue to feed at home. Trying to keep Jakob well was what our life as a family was all about.
At about 8 months of age Jakob started to show signs that he was becoming unwell again. This time it was related to his breathing. After a month of trying to manage him at home Jakob required hospitalisation. This admission was to be a long one. His respiratory function declined rapidly and he needed much higher flow rates of oxygen. He became extremely susceptible to viruses. Doctors suspected micro aspiration again. Jakob required stays in the Paediatric Intensive Care Unit (PICU) on three occasions, following episodes of respiratory failure. He again needed breathing support, this time in the forms of high flow oxygen and C-PAP. In between the admissions we had two failed discharges home, each time having to return by ambulance within days of leaving the hospital.
Eventually, and reluctantly, a decision was made for Jakob to have a fundoplication. A procedure in which the fundus (top right hand part of the stomach) is wrapped around the lower end of the esophagus to create a tightening. The idea is that it makes it more difficult for the stomach contents to come back up out of the stomach and for the person to then aspirate on them, something they felt may be happening for Jakob. At the time of the fundoplication a percutaneous endoscopic gastrostomy (PEG) was also performed, inserting a tube directly into Jakob's stomach for feeding. This was a high risk surgery for Jakob and he required admission to the PICU post surgery. One month after Jakob's surgery, and after 4 months in hospital, he was able to come home again.
To learn more about Jakob's progress post his hospital admissions and his more recent progress, click here.
