Hope's story by mum, Caroline

"Hope was given her name before she was born. She has a brother called Sam, who was born and went to heaven before she came. Sam was just 16 hours old when he died from a diaphragmatic hernia. It was Christmas Day.

"I was pregnant again a short time after, and from the beginning Hope was very much wanted and needed," shares mum Caroline.

"Her name has taken on another meaning now, of course, but we named her Hope because she was our ‘hope’ after Sam. And everyone who knows her knows she is the ultimate in hope, and very much lives up to her name."

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At the hospital we were under the same consultant, and he said it was the unlucky lottery of life – to have two children with two completely unrelated, rare conditions.

Hope was born with a life-limiting mitochondrial disease called pyruvate dehydrogenase complex deficiency. It is a very rare and complex metabolic condition, which affects Hope’s ability to process carbohydrates or make the energy her body needs to work properly. It means Hope is unlikely to ever talk or walk by herself, and she is registered blind.

A very poorly baby

Hope was a lovely baby, but she was a very poorly baby.

She was only 4lb when she was born, so she was small, but she seemed fine at first. We had a few weeks at the start when we were blissfully unaware really.

Hope started to smile at five weeks – and she really has always had the best smile.

Then at around 3 months old, her body started jerking oddly. I quickly realised that these jerks were seizures, and Hope was having around 200 of these a day. It was really awful.

She was given epilepsy medication to keep the seizures under control and it did help, but it made her sleep all the time. She seemed to regress at that point in her development, and her sight seemed to go then too.

When I stopped breastfeeding and started Hope on solid foods fully, that's when she got very seriously poorly.

For the next few years, we were known to be frequent flyers to the hospital. Hope would have really big seizures, and we’d have to call an ambulance and then she’d be rushed into hospital. Then she would get terrible chest infections and have to have antibiotics. That was really scary because I used to think her little body can’t carry on like this.

It was also hard on our older son and daughter, Max and Jordan. They’d lost their brother, then they were excited to have a baby sister. And then their baby sister came along and she was really poorly. They have never had that normal toddler to play with; everything has been different for them.

A ‘miracle’ diet

Once we got Hope's diagnosis of mitochondrial disease, a lot of pieces fell into place. What used to happen is Hope would go into hospital and they’d be giving her IVs with sugar water because that’s what normally makes everyone better, but that makes Hope so poorly because she can’t process carbohydrates. So, she was going in and getting better from the infection but metabolically she was crashing because they were giving her something that her body can’t tolerate. It was completely the wrong fuel.

One day after Hope’s third birthday, we started her on a low-carb, high-fat ‘ketogenic’ diet, and it was like a light came on. Suddenly, she was reaching for a mobile in her cot that she’d not paid any attention to before. She started rolling again and sitting up. And she just really had another lease of life.

Incredibly, she’s been seizure-free since November 2012, just because of her new low-carb diet!

The ketogenic diet isn’t easy – we prepare everything fresh and by the gram and even avoid common brands of sun cream and bubble bath which have topical carbohydrate ingredients in them – but it’s absolutely worth it. The change in Hope has felt like a miracle.

“Paused at the nine-month phase”

I’ll sing Hope's praises and say how much she can do, but really she is like a giant baby. It’s like we paused at the nine-month phase, and she’s still dependent on us for everything.

She can't drink as much as she needs because of reflux, so every 45 minutes during the day we have to give her water via a tube into her tummy. Hope also has sleep apnea, so she often stops breathing and wakes several times a night. She uses a wheelchair to move around and a standing frame. And she still plays with baby toys and teddies that sing. We normalise it, but after 16 years it’s a lot.

The reality is that Hope has this horrible life-limiting condition and there is no cure. She’s stable now but Hope’s future is unknown, and things could change overnight.

I don’t normally like to leave Hope. In 16 whole years, I think I’ve had three nights away from her. I recognise that’s not normal, but that’s how we’ve stayed well.

We really feel like we’ve been swept up by Julia’s House since we were first introduced last January. A carer that used to come to our home in Swindon had sadly stopped working due to cancer, and we had nowhere else to turn to for regular help. Without Julia’s House, I don’t know how we would have coped this year.

I'm really happy with the care we’ve received. I like the way that the care team’s paperwork and care plans are so detailed, and everything is so tight – it makes me feel like it doesn’t leave room for mistakes to be made, which is reassuring and has helped me to step away.

At Julia’s House, I know there are nurses and carers who really understand Hope; they know how to look after her, how to communicate with her and how to make her happy and comfortable. Hope needs to build trust and feel confident in the people who are looking after her, and it’s important for me too. So, we’ve spent a lot of time with Hope’s named nurse, Molly, and the other nurses and carers on sessions, getting to know each other.

Hope’s greatest love

You’ll never meet anyone that loves stories as much as Hope. She would read stories (and we largely do) from the moment she gets up to the moment she goes to bed. And when she goes to bed, she has a Yoto player and a Toniebox to read stories to her through the night, and if they go off then she wakes us up. She literally just wants stories all day.

Wherever she goes, Hope doesn’t leave the house without her book bag, which has her favourite stories inside. And she likes to read her favourites over and over again. She does have lots of favourites, fortunately! She likes Michael Rosen and Julia Donaldson mainly. She loves We’re Going on a Bear Hunt, The Gruffalo, books with animals and books with the sound buttons along the side.

Hope’s registered blind but if you put all of her books in front of her, she can absolutely find the book that she wants. And if you put a book just out of her reach, she can see it. It is a bit of a joke that her sight is quite selective. She’s a really determined little girl, which stands her in good stead, I think. She’ll work really hard on things.

She also loves animals, and being outside, and the wind. One of Hope’s favourite things is the wind blowing in her face. She’s quite wild and feral really.

Hope loves swimming and water as well. She loves to have a bath, and she does quite often at Julia’s House when she’s there. She likes the really nice big bath in the hospice, and she uses a neck float so she’s just floating in the bath. It’s a nice part of the routine that the Julia’s House nurses and carers do at the hospice for her. And that’s a massive help for me if Hope has had a bath that day and I can take her home in her pyjamas and she’s ready for bed.

Giving Hope her voice

We were told that Hope would never walk or talk... but she kind of can. She’s just started using a walker and although she can’t talk, she can communicate really well.

She’s got a special communication book called a Pod which helps her talk about the weather and her feelings, and she can say what she wants and choose nursery rhymes and stories. She’s just moved onto an electronic device which helps her to communicate as well, so we’re all learning how to use that – including our Julia’s House nurses and carers.

We’re all working really hard to give Hope the voice that she has inside her head and give her the freedom to communicate.

Hope loves the quieter toddler sessions at the Julia's House children’s hospice – she’s the noisy one then, and she loves all the stories and nursery rhymes, so it’s ideal for her!

When I arrive and it’s a nurse or carer we know really well, Hope will reach out and they’ll be using her Pod communication book and getting her out of her chair and reading her favourite stories. I’ll say bye then and I can see that she’s fine. I know that she’s really happy and well looked after.

And it’s a huge weight off for me, as I can hand over all Hope’s medications, her blood ketone testing machine, her feeding kit, her lunchbox, and properly hand over to someone else who is specially trained and will give her the very best care for the next five or six hours. That’s the only time I can ever do that, and it’s such a lovely feeling.

There for the whole family

It’s not just Hope who is supported, it’s also me. Without Julia’s House, I’d be more overwhelmed and stressed and tired, and that would have an impact on our whole family.

It’s a huge relief that I can see those regular dates in the calendar, and I think to myself, ‘Just get to that point and I can relax, and I can have a day to myself.’ Because those days are rare.

Hope goes to school a couple of days a week and they tend to be my busiest days because I end up squeezing in everything that needs to be done – usually a lot of Hope-related admin and jobs – but the days at Julia’s House are my days. They’re my days to go for a run, to meet up with friends or just relax and have a coffee and read a book. So, they’re definitely a highlight of my month, just knowing that they’re coming up. A day to recharge.

I’ve had a really lovely couple of complementary therapy massages, which helps relieve the aches and pains that come from caring for a child with complex needs. And the Family Support Worker at Julia’s House has helped us get a voucher for our heating bill, through Together for Short Lives. We haven’t applied for any grants, but it’s helpful to know that there are people there who know how to access those things and can help if we need it.

And when it comes to the school holidays, that’s when I’ve noticed a massive difference. Always having a day at Julia’s House each week over the half-term breaks and summer holidays has been a life saver for me because there’s so much more to do when Hope’s not at school.

Over winter, I think I’ll be less inclined to go for a run along the canal in Devizes, so we’ll probably have more respite sessions at our home. The home sessions are lovely because Hope’s nurse and carer can give her tea and a bath and take turns to read her stories before putting her to bed. It means my husband Chris and I can go for a nice dinner, which is something we never do together.

Time to recharge and re-connect

Hope actually needs somebody to look after her all the time. So, our weekends are just my husband and I taking it in turns to look after Hope. I go for a run, and he looks after Hope. He goes shopping and I look after Hope. I have some lunch, and he looks after Hope. He goes into the garden, and I look after Hope. So, we never spend time doing something together and that’s the same when we go on holidays. We don’t spend a lot of time together because we’re always tag-teaming, just talking about when she last had water.

When Hope had her first overnight hospice session at Julia’s House, it was really significant. Hope is 16 years old and when she stayed overnight at Julia’s House, that was mine and Chris’ second night together ever away from her in her whole life, which is crazy when you think about it. But we just don’t have those opportunities with people who can look after Hope.

I wouldn't have thought I would be ready to leave Hope overnight after just six months at Julia’s House, but I knew she would be absolutely fine with the nurses and carers there. And Hope had a really lovely time.

It was our wedding anniversary – not a milestone year and actually we’ve lost count now – but we wanted to celebrate and make the most of the time. So, we dropped Hope off at Julia’s House and then zipped straight off to Bath to enjoy an afternoon at the Spa, followed by some shopping and dinner and then a night at a hotel. And what was really incredible was being able to wake up at our leisure – although it was still very early – and make ourselves a coffee in bed. Just to put the kettle on, instead of turning off the SATs machine and prepping medications, was such a treat. We went for brunch and then headed over to the hospice to pick up a very happy Hope. It was so very perfect. It was an expensive weekend in the end, but we wanted to go big after all these years and treat ourselves, and now that we’ve done it once and it was so lovely, we’ll definitely do it again.

When you have a child with a life-limiting condition, you’re not thinking too far ahead. Nothing is guaranteed. You live in the moment; you’re making the most of the now. Julia’s House helps us all to make the most of the now, with Hope. Their regular care and support has been a wonderful gift to our family, and I can’t thank them enough.

Hope's story by mum, Caroline

"Hope was given her name before she was born. She has a brother called Sam, who was born and went to heaven before she came. Sam was just 16 hours old when he died from a diaphragmatic hernia. It was Christmas Day.

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