On 17 June 2012, our family welcomed a healthy baby boy who weighed in at six pounds and nine and a half ounces. We were all delighted. When he was nearly three months old Jack started to become unwell. He vomited constantly and became dehydrated. My husband, James, and I took him to the hospital where it was determined that Jack had gastroenteritis. However shortly after the diagnosis, Jack began to have seizures.

The hospital arranged a number of tests to try to establish what was wrong. In the end the tests came back to show that Jack had Menkes disease. Our entire world fell apart in that moment.

Menkes disease, we learned, meant that Jack’s body could not metabolize copper, an essential mineral without which Jack would die, probably within three years. There was no cure and in the meantime Jack would become progressively sicker and be severely disabled. We were told to go home and enjoy him while he was still here. In a few days a community nurse would call out to the house. That was it.

24 Hour care

SuperJackWe drove home with our boy. He looked the same. We had some medicine to give him every day for his seizures but other than that nothing had changed. As the months passed Jack’s seizures became progressively worse, he became floppy due to low muscle tone (Jack could not hold his head up or hold a toy), his body temperature was permanently low (our heating was always on), he began to develop frequent chest infections, his ability to feed diminished until eventually he needed to be fed through a tube, his digestive system started to fail and he vomited several times a day.

He cried constantly, a condition we were told, called cerebral irritation. I call it misery.

We also had some practical problems to think about. My plan was to return to work and to place Jack into nursery before he was one. That was something that we could no longer do. Jack’s disability was getting worse and a nursery would not be able to look after him due to his problems. If I couldn’t work how would we pay the mortgage? If Jack did outlive his three-year life expectancy would our house be suitable for all his medical equipment and the severity of his disability? These were problems for which we had no answer because the thought of losing our son was by far the greatest burden to bear.

Jack needed 24 hour care. One of us slept in his room as he was at risk of choking and suffocating on his vomit. His seizures needed monitoring and treated with rescue medicine and therefore the person on night duty (James or I) rarely slept at all. The hollow pained crying was very stressful, not because of the noise but because we knew our child was suffering and there was nothing we could do. It was emotionally and physically exhausting.

A helping hand

Our own health was suffering due to a poor diet, stress and complete inactivity. I was confined to the house as Jack was too sick to go out. The community nurse suggested the Northern Ireland Children’s Hospice for respite care. They could offer some overnight stays throughout the year to give James and I a break. We gladly accepted and were offered a tour of the facilities a few weeks later.

On the tour we were instantly impressed. The facilities were fantastic; they even had a heated hydrotherapy pool so Jack could go swimming (an activity Jack grew to love!) There was a sensory room (by now it had been established that Jack was blind). Each child had his or her own bedroom and one-to-one care during the day. Parents could stay too, we had our own separate accommodation so that we could get that much needed sleep but see Jack whenever we wanted.

After our first stay we counted down the days to the next. Over the next year Jack’s condition continued to deteriorate. Seizures became worse requiring several blue light ambulances to hospital and some moments when we thought he was gone. Chest infections and pneumonia became more severe and more frequent, requiring long hospital stays where we were told to expect the worse. Every time we brought Jack home we seemed to have more equipment to pack into our small terraced house.

As well as his medicines (13 or 14 at the peak), we had his NG feeding pump, stand, boxes of syringes and giving sets, pH testing strips, surgical gloves, a suction machine (for clearing Jack’s airway of mucus which had to be done frequently), two oxygen condensers (a large machine for giving Jack oxygen which he now needed 24/7), six portable oxygen cylinders, one large emergency oxygen cylinder and a machine for measuring Jack’s oxygen saturations. Our house was a hospital only without the appropriate design.

End-of-life care

When Jack was a little over two years old, another severe chest infection and emergency ambulance brought us back to the hospital after a record six month stay at home. By now Jack had a six-month-old younger brother and the shifts that James and I took at the hospital to care for him became much more strenuous with another little one to look after at home. We were running out of steam. At the same time the doctors gave us the news that Jack would not recover. His digestive system was failing meaning he could no longer tolerate milk in his stomach. His chest was not going to get better.

We were given the option of bringing him home to die or the children’s hospice for end of life care. It was an easy decision. Jack had always been happy at the hospice, what they could provide we could not and what we had at home just wasn’t working. A few days later our family moved into the children’s hospice where we lived together for the next six weeks.

On 4 November 2014 my husband and I held our baby boy in our arms as he took his final breath. He looked peaceful and was no longer in pain.