Shailza and Daniel Leaver have two beautiful boys just a year apart in age. They smile and giggle and win hearts just like any other 4 and 3 year old but sadly neither Riley nor Stanley is able to sit, walk, talk or even feed properly. Both boys have been diagnosed with a very rare genetic condition involving a duplication of an X chromosome which has left them with very poor muscle tone and requiring constant care. The condition is so rare that not even the hospital specialists are able to predict what the future looks like for Riley and Stanley. It is simply a question of taking each day as it comes. Shailza shares her story about life with her boys and how the Rennie Grove Children’s Hospice at Home team has become her lifeline. . .
I was really happy when I found out I was pregnant with Riley. I’d had a few problems conceiving and had almost given up hope it would happen naturally so when it did, we were delighted.
I had a normal pregnancy but developed gestational diabetes which led to labour being induced at 39 weeks. It was a long and difficult labour lasting four days and when Riley was finally born with the help of a forceps delivery, we were both exhausted. There were a few early problems; Riley didn’t cry properly which led to him spending a few days in the special care baby unit, he also didn’t latch on and he had bad jaundice but we were told everything was fine and it was just a result of the long labour.
Once at home, Riley grew normally and was a very good baby. I had postnatal depression which was linked to my disappointment about not being able to breastfeed and it was not until he was about 4 or 5 months old that I started to think things might not be right. Riley became seriously ill when he was 6 months old and my GP sent us to the children’s Accident and Emergency department where the consultant said something was wrong and was concerned that he was not holding his head.
Genetic testing
We were referred to specialists who started to ask about our family history. It was only at this point that I connected some events that had occurred in my family and realised that there might be a hereditary genetic problem. There followed a raft of genetic testing for members of my family and the disorder, which affects only males, was identified.
By this time Riley was 8 months old and I was already 20 weeks pregnant with Stanley. We had found out that he was a boy and we knew that there was a chance he would have the disorder too, but it was only a chance and as it wasn’t possible to test Stanley at this time, I couldn’t consider a termination – there was a chance that he would be fine.
The extent of the effect of the condition on Riley was becoming apparent at this stage. He could not sit or roll over and it was clear that he was not going to be able to walk. He also did not develop speech and could not do anything for himself. Life became a constant round of hospital appointments and managing Riley’s care around the clock which was exhausting.
We were referred to the Rennie Grove children’s service and they began a weekly three hour respite visit when one of the charity’s nurses was able to take over all of Riley’s care needs to give Daniel and me a break. They also helped us liaise with other healthcare professionals involved with Riley.
Stanley arrives
Stanley arrived three weeks early by emergency caesarean as he was breach. The hospital checked him for muscle tone and they said he was fine. He was a strong baby; he was alert and active and latched on and breastfed well. He was very different to Riley and I convinced myself that everything was ok. I didn’t even ask for a genetic test.
When he was three and a half months old I took him to India for my sister’s wedding and the minute my Auntie saw him, she said he was the same. She could see it. I was heartbroken and as soon as we returned I discussed it with the Rennie Grove children’s service nurse who was by that time helping us with Riley. I was very emotional but we decided to ask for the genetic test which confirmed what we had feared, Stanley was affected in the same way as Riley.
The nursing team then took Stanley onto their books too and continued with their weekly respite visits. Both boys developed distressing difficulties with swallowing and feeding which led to them needing naso-gastric tubes and ultimately they both had gastrostomies and were fitted with a feeding device that goes straight into the stomach.
The boys had the operations at different times and the team supported us through them both by providing extra care at home so that both Daniel and I could visit the hospital.
At the point when Stanley was diagnosed we were living with Daniel’s parents but we badly needed our own space and Sarah, our nurse, was able to help us with a housing application and just before Stanley’s first birthday we moved into our own flat.
Learning to cope
It was at this point that worries for the future really hit me. I felt very scared about what would happen and what our life would be like. Would the boys die and how would Daniel and I cope?
But we are coping. The boys’ smiles really keep us going and the children’s nursing team play a large part in helping us cope too.
It is immensely reassuring that the team is only a phone call away, 24 hours a day, every day of the year. Although Sarah provides most of our care, the whole team is fantastic. We have called them at night for help and advice. Daniel works nights and there have been two occasions when I have become really unwell and have been unable to care for the boys. The Rennie Grove children’s nursing team have come out without hesitation on both occasions to look after the boys until Daniel was able to get home and on one occasion continued to care for Riley and Stanley while Daniel took me to hospital.
Knowing that there is help at hand 24 hours a day is immensely reassuring. I have also called them at night for advice when the boys become ill and I was unsure about whether or not to call an ambulance. They will always offer advice and come out if needed and if the boys are in hospital they will come and visit.
Part of the family
Sarah has become like part of our family. While I do sometimes use her three hour weekly visits to have some time to myself, on other occasions we plan to take the boys out. I can’t manage to take both of them out on my own so we might plan a walk or a swimming trip. I am determined to give the boys as much quality of life as I can and they really enjoy these trips out and they absolutely adore Sarah.
I am also able to talk to Sarah about new symptoms that might develop and she advises me accordingly. For example, it was Sarah who looked at Stanley’s tonsils when I said that he was not sleeping properly and she spotted that they were big and recommended that I speak to the consultant about it. They then did a sleep study and diagnosed sleep apnoea and Stanley has now had his tonsils removed.
About a year ago Riley developed seizures which is an extra worry and as the condition is affecting the boys in a similar way, we are now waiting to see if Stanley will be affected by this too.
Caring for both boys round the clock is exhausting. They need medication, water and feeding at regular intervals throughout the day and they need to be turned three times a night, and now with the seizures, I also have to monitor Riley’s temperature. If I’m lucky I will get three or four hours of sleep in between caring for them. But if I’m worried or they are unsettled, I often sleep very little. There’s so much to think about that I do find it hard to switch off and sleep even if the boys are fine. I have had Reiki Therapy through Rennie Grove which has really helped with this. It taught me some little breathing exercises to help me fall asleep quicker and they actually do work!
Lifeline support
The boys’ needs are increasing all the time, but with the help of Rennie Grove we are accessing as much extra support as we can. We have just moved again into a house and are waiting to have some major adaptations done to the property to install a lift for the boys and add an extension which will include level access to the garden so we can take the boys outside. Riley and Stanley will also both start school soon.”
The children’s nursing service has been a lifeline for us. It is so important to have someone you can rely on and who you know won’t let you down when you are in real need. It is so reassuring to know that if we ask for help and advice, it will be given without hesitation, whatever time of the day or night.
We are just taking each day as it comes. We don’t really know what the future holds but with Sarah and the rest of the Rennie Grove nursing team just a phone call away, we face that future with much more confidence than we would otherwise.
There are 54 children’s hospices across the UK, all of whom provide lifeline care to children like Riley and Stanley. If you’d like to support their work this Children’s Hospice Week please make a donation through our website – all funds we raise this week will got to our National Fundraising Scheme which is distributed to children’s hospices across the UK.
