Ezra's story

“When Ezra was diagnosed with spinal muscular atrophy (SMA) type 1 - the most severe form of the condition - there was no treatment and it just looked like he was going to die. It was the worst possible news. 

“But then they told us about a treatment that had just been licensed in America which we could get under an expanded access program. They couldn’t tell us about outcomes or prognosis, because there wasn’t any long-term data - normally no one with SMA type 1 lives beyond the age of two. They said some kids in the trials were walking, some weren’t, but no one really knew anything then. 

“Ezra is now in year four and he goes to mainstream school in his power chair. He’s completely peg fed and has lost his swallow; he’s ventilated overnight; he’s got a severe scoliosis and will need spinal surgery at some point; he has a very vigorous regime of respiratory physio, he has a cough assist machine and we have to use suction catheters to clear his chest, because he’s not strong enough to do a big cough. 

“All of that is with the treatment. The important thing with these drugs is you need to give them to children as early as possible, before the symptoms start, then they can follow normal development patterns. If Ezra was treated as soon as he was born, he would be walking, running, eating, breathing – living a normal life.” 

When Demelza first offered support, Portia wasn’t sure a children’s hospice was the right place for her family. 

“Demelza was introduced to us on one of the many intensive care visits we had when Ezra was a baby, and to be honest, I was very against it. The suggestion robbed me of my hope, sending us to a hospice. So I said no for a very long time, until he was probably about three years old, and more stable. We went and had a look round, and actually it wasn’t what I thought a hospice was like at all. It looked fun!” 

From then, Portia started bringing Ezra and his sister Martha to Demelza to stay. 

“I’d get to take Martha out and we’d have a really nice time. That’s one of most important things Demelza has done for us - enabling me to have more time with Martha. She’s missed out on so much. Martha is 12 now and has just started secondary school, and she really values just having her mum to herself for a bit. At the weekends and holidays, I have to be next to Ezra all the time, he can’t do anything without me facilitating it. She calls herself the ‘glass child’ – she feels like she’s see-through, that no one sees her. So Demelza’s help means we get time together and the chance to do things that otherwise we can’t do, like trampolining or ice skating, that inaccessible stuff that Ezra doesn’t even want to watch, because it’s not fair. So, for me that’s been the most valuable thing. 

“Demelza have also been a great help when we’ve needed extra support. We’ve been refurbishing our house forever, but one time my husband was pulling down some walls, 

and all the dust meant we couldn’t stay at the house, so me, Martha and Ezra moved out and came to Demelza for two weeks. That was amazing - it was a real lifeline and we had a great time.” 

Ezra also loves visiting Demelza’s hydropool – a safe place where he can build strength and enjoy a moment of physical independence. 

“Ezra loves coming to Demelza. He loves the pool – swimming is his absolute favourite thing. Even though he has breathing and swallowing troubles, he can swim underwater for about 20 seconds, he can do whole widths – he’s amazing in the pool. It’s easier for him to move in the pool and he’s got floaty suits so he can be more independent and just bob off – he loves it. 

“Being in the pool is more or less the only physio he does, and it’s just the best, because when he’s supported with his buoyancy suit he can even take steps. And respiratory-wise it’s been fantastic – he’s learning to hold his breath, I think it’s improved his cough, his ability to get better with colds, and the volume of his voice as well. The respiratory benefits of swimming aren’t talked about enough.” 

Ezra’s sister Martha is supported too – from having fun with arts and crafts to meeting other siblings. 

“Martha loves being at Demelza too. She loves all the arty stuff, making crafts and going to the cinema room. She went on one of the sibling residentials - she really enjoyed it and made a lovely little friend. She wasn’t sure about it at first and didn’t want to stay over so I just took her up for the day, but now she wishes she had stayed because she had such a lovely time.” 

Demelza also visit the family at home, giving Portia more time with Martha or helping her to balance the pressures of both life and work. 

“Demelza also come out to our house for a few hours – they’ll come and entertain Ezra at home while I take Martha out, or if it’s during the school holidays and I’m working, I can just get on while they look after Ezra downstairs. Ezra is hilarious. His favourite things right now are fighting, swords, Zelda, volcanos, tornadoes, space, rockets, dinosaurs - anything like that. So he usually wants to fight with swords or play hide and seek - his Demelza nurses just get involved! We’ve got a really good team. It’s usually the same nurses each time which really helps him build that bond with them.” 

They have all found real community in the other families at Demelza too. 

“We normally do the Easter egg hunt at Demelza and the Christmas events too. They’re so great with gifts and understanding that Ezra can’t eat, so they make an extra effort to do something a bit different for him – it’s not just an easter egg or a candy cane which he can’t enjoy. Because I’m quite well known in the SMA community I often get approached by other families at these events, which I don’t mind at all – it’s brilliant to help build that community. 

“It’s something that I wish I’d had when Ezra was little. He was one of the first ones to live following treatment – I think there was just one other little boy in America whose social media used to give me hope. So it’s nice that I can do that for other people. There’s lots of us now! It’s massively helpful to have other people around who just know what it’s like.”