With two young sons – James, three years and Samuel, 15 months – life for Rachel and Paul Lockyer was just like that of every other family with a lively toddler and a new baby – hectic and happy.
But things changed dramatically one day that led to both James and Samuel being diagnosed with an exceptionally rare medical condition that transformed the lives of the whole family.
Rachel says that Rennie Grove’s children’s hospice at home nurses mean the world to the whole family and explains how they have supported James and Samuel and the whole family through some very difficult times.
“James was a really articulate little boy who was meeting all the developmental milestones expected of a three year old. Then one morning I got him up to get him ready for nursery and he kept falling asleep. And literally our world changed just like that.
“James became very ill and went into hospital in October 2017 and had to spend many months there whilst the doctors tried to find out what was causing his illness. Eventually he came home January 2018 but we were in and out of hospital for a long time after that as he kept getting infection after infection.
“It was a really difficult time for us a family and for a long time we were never really together because one of us was with James in hospital and the other was looking after Samuel who was still a very young baby.
“For a really long time no one knew what was wrong with James but then in March 2018 he was diagnosed as having XLP1.
“XLP1 is a rare immune condition that affects 1 in every 1 million males. It is passed on genetically and in almost all cases it is carried by females and passed to men who develop the symptoms following a trigger – often caused by an infection. In James’ case he developed extensive encephalitis (inflammation of the brain) which has been life changing for him and us.
“Because it was discovered that I was the carrier, Samuel was tested and also diagnosed with XLP1. He was just 20 months old.
“Early diagnosis of XLP1 can be treated with a bone marrow transplant so both our boys underwent this serious procedure and we are so thankful that as a result Samuel has gone on to develop normally despite him being in hospital for many weeks and undergoing such a traumatic procedure.
“James also underwent the transplant but his brain damage was too extensive to repair and he now needs constant care and attention 24 hours a day.
“We were discharged from hospital at the end of January 2019 shell-shocked from everything that had happened to us. It is hard to put into words how tough it was.
“Our community nurse arranged for nurses from Rennie Grove Hospice Care to visit and carry out an initial assessment. Very quickly after that two nurses from the charity’s children’s hospice at home team started visiting and things changed for us right away.
“The nurses helped us in so many ways – providing clinical care to the boys and offering a listening ear and practical help to me and to Paul. Their twice-weekly visits meant that I could get breakfast, have a shower and get dressed – all those things that you take for granted but that were almost impossible for me whilst looking after two sick children on my own when Paul had had to go back to work.
“James is fed by a central line that he tries to pull out all the time and can now walk so he needs someone with him at all times to keep him safe. Samuel is a typical toddler and as all parents know that is a full time job in itself. So when the nurses came I could give Samuel my full attention or do some things around the house knowing that James was safe.
“Rennie Grove nurses coming in twice a week was a life-saver.
“Sam Howard (who is now Rennie Grove’s Director of Nursing) visited us. She worked at Great Ormond Street Hospital for many years and knows a lot XLP and bone marrow transplants as the hospital carries out research into the condition. So I didn’t have to explain anything to her. She also knew all about James’ feeding method so everything about James Sam knows.
“She was able to give all the Rennie Grove children’s nurses full information about their condition post-transplant – she knows everything! It’s an amazing coincidence that she works in our area and has been able to give me so much information and be someone to talk to who really knows James’ condition.
“Once Samuel started to get a bit better we changed to one nurse visiting each time and they started taking James out a lot which was wonderful. It was really good for James because it got him out and gave him a change of scenery and was good for me too as I had a little space. I felt confident when the nurse took James out as I didn’t need to explain what needed to be done in terms of his medication or his central line.
“Rennie Grove nurses are a lifeline in many ways and have given me the opportunity to do the things that most people will consider a chore, like emptying the dishwasher or putting on a load of washing. They have also helped in practical ways too. For example Terry spent a lot of time contacting hospices and other services to try to find somewhere that might be able to take James for respite care. Because of the way he fed it’s difficult to find anywhere that has the facilities to care for James. I simply would never have the time to spend on making calls like that.
“Rennie Grove nurses have given us so much more than nursing support and it’s so reassuring to know that it’s Tuesday morning or Friday afternoon and Rennie Grove is coming.
“More recently things have changed significantly as James – who is now five - has started school full time and Samuel – who is now just over three- goes to nursery three days a week. Rennie Grove nurses are still there for us during the school holidays and are great at doing their best to squeeze in a visit if James’ 1:1 carer at school is away which means James cannot go in.
“That’s another good thing about Rennie Grove they can get things moving quickly whereas other services tend to take weeks before anything much happens. Of course there are times when they are fully booked with other families and of course we understand that but basically if they can, they will.
“It’s been an exhausting two years for everyone – including our extended family. But James and Samuel are our children and we will do whatever we can for them.
“Rennie Grove nurses mean everything to me – I feel that I simply would not have coped without them. With Rennie Grove you know that twice a week someone is going to be here. And even now with the boys at school and nursery, knowing the children’s nursing service is there is just like having a security blanket. You think: “It’s OK. Rennie Grove are coming soon and I know they are coming to help me.””