The beaming smiles of two-and-a-half-year-old identical twins Ruby and Sophia are enough to brighten any room. But even when the girls are smiling broadly, parents, Kevin and Lyndsey, live with the anticipation that the twins could go into full body paralysis or even stop breathing at any moment. But with the lifeline support of Martin House Children’s Hospice, Kevin and Lyndsey now calmly manage such episodes sometimes multiple times each day.
Kevin and Lyndsey’s lives were turned upside down when the twins were diagnosed as babies with alternating hemiplegia of childhood, also known as AHC. AHC is a genetic condition affecting the movement of salts in the brain, causing paralysis to one or both sides of the body. The condition is incredibly rare. Ruby and Sophia are two of just 50 children in the UK who have the condition and their mutation is thought to be one of the most severe.
“When the girls are well, they’re unstoppable. They’re very cheeky and love to crawl and climb everywhere! But the next minute, they could have an episode and struggle to sit unaided, or even swallow,” says Lyndsey.
“At times, it can be really hard to explain the impact of their condition, and how quickly we can suddenly find ourselves in an emergency situation. We’re so used to fighting for them because the condition is so rare.”
“You just freeze”
The condition was diagnosed in the summer of 2019, when the family spent June to August continuously in hospital. At one point the twins were in different hospitals, which led to lots of anxious running between bedsides. Pinderfields Hospital in Wakefield carried out the genetic tests to find out the cause and worked with clinical nurse specialists from Martin House to introduce the idea of hospice care to the family.
“You hear ‘hospice’ and you just freeze, but we took the opportunity to have a look around and find out more about what was offered to us,” says Lyndsey.
“The first time we stepped into Martin House was emotional, as it’s not what you expect for your child. But we got past the preconception and accepted that we are dealing with this.”
Apneas are a symptom of the condition, where the children suddenly stop breathing. These can last up to five minutes and can happen up to ten times a day, with Kevin and Lyndsey needing to resuscitate them. They also have periods of ineffective breathing, which can last much longer and led to the girls being intubated last year.
“We have no medical background and never thought we’d have to do CPR, let alone on our children. Each time it happens, you ask yourself ‘is this it?’ They can be crawling and suddenly turn blue. We’re both calm and we don’t panic. You detach yourself, because you have to get on with it.”
“It was November 2019 when we visited for our first stay at Martin House, and it was just brilliant. We were apprehensive about leaving them in the care of someone else, even though we were staying, too.
“We spent three nights at Martin House, and that was the first time we’d had a good night’s sleep for at least a year. A night without a SATs monitor beeping is everything! It was a chance for us to spend some quality time as a family without worry. There are so many opportunities to play, and enjoy music and art.
“It’s somewhere people understand you totally. I’ve often felt like I have to explain to others why they vary so much in how they are and what they’re able to do. I’ve been a part of baby groups and there’ve been parents stressing over things like their child’s first haircut, and for us, Ruby’s first haircut was in hospital so the doctors could insert a cannula into her head.
“At Martin House, you can talk to other parents who experience something similar and you don’t feel so alone. We’ve found Martin House to be quite the lifeline. Your stay comes just when you need it, when the responsibilities are taken from you. Martin House makes you feel like it’s not just you.”
The world turned upside down
Last year, the whole world was turned upside down, but despite the challenges of the pandemic, Martin House was able to continue its support for the family throughout the year, with an emergency symptom control stay to help with Ruby’s sleep, and an emergency stay for the girls when Lyndsey had to go into hospital herself.
“The girls are still having a lot of AHC symptoms – we had to resuscitate Ruby eight times in 24 hours a couple of weeks ago – and their developmental delay is becoming more apparent now they’re older. They aren’t walking or talking yet.
“They are lovely though, and have made so much progress recently! Sophia has learnt to drink from a straw recently, and Ruby has learnt to open doors and can now escape any room at will!
“We are still really appreciating the support from Martin House, and we’re so impressed by how joined up everything is. When we came in for symptom control, the doctor at Martin House had already emailed the girls’ consultants at both Great Ormond Street Hospital and Pinderfields Hospital and had formulated a plan of action to discuss with me.
“As always, it really was a discussion, with our views very much sought and considered, and the medication he suggested has helped. They ask how we are, as well as the girls’; it’s a real source of support. We’re looking forward to a stay this month which includes a trip to Whitby for a boat ride!”
There are no limits to the support that families like Ruby and Sophia’s need from children’s hospices like Martin House.
More and more families need the help of children’s hospices, and the hospices need your support to continue.
Help us ensure children’s hospices are not pushed to their limits by donating today.