Page 9 - Community Health magazine - issue 38

oung Ollie steals the hearts of
everyone he meets. He is highly
Yintelligent and knows what he wants
and with a purely magical smile, he’s sure to
get it. Ollie is not just any ordinary boy. He is
one in 10,000.
Mum Amy, 31, was enjoying an easy
pregnancy and together with husband Ben,
they couldn’t wait to welcome their precious
baby into their home in Wye near Ashford.
Three months after he was born, they
were in A&E at the William Harvey Hospital
and Ollie was diagnosed with Spinal
Muscular Atrophy (SMA) Type 1.
Amy said: “In hindsight there were
symptoms from birth. Ollie wasn’t feeding London Children’s Hospital. At that time that back. As his swallowing improves, we
properly and he was tummy breathing when only 15 children had received the treatment have let him try to feed himself – just like
asleep. As new parents, we asked questions in the country. any other child.
but were reassured it was normal.” “He may never regain the use of some of “He loves bananas but his favourite at
About 70 babies are born with SMA in his muscles but his movement has improved the moment is yoghurt. I love watching him
the UK each year and one in 40 people are and he has achieved milestones we never experience more each day, even if it means
genetic carriers. To put that in context, the thought possible.” food getting everywhere.
year Ollie was born, more than 681,560 Because Ollie has an unsafe swallow, he “Jo has been great, we know she’s on the
babies were born in the UK. had a percutaneous endoscopic gastrostomy other end of the phone if we need her and
SMA is caused by a mutation in the (PEG) tube fitted into his stomach which for us it’s important we normalise his feeding
survival motor neuron 1 (SMN1) gene, allows Amy and Ben to feed Ollie safely. as much as possible. It brings us together as a
which is responsible for producing a protein To support the family, a referral was made family and makes us all think carefully about
that is essential for motor neuron survival. to KCHFT’s Paediatric Dietetic Service. nutrition, calories and what our bodies need.
Motor neurons are nerve cells that control With about 70 referrals per week, the “She is part of an incredible network
muscle movement and strength. They service helps nearly 400 tube-fed children of people who are supporting us.
control muscle activity by sending signals across the county. This includes premature Physiotherapy, speech and language
from the central nervous system to muscle babies who may need short-term support, therapy, occupational therapy, portage
cells. In SMA, as motor neurons deteriorate, to more longer-term patients with and overnight support. Together with the
muscles stop receiving signals, which leads disabilities, anxiety or sensory difficulties. support from our family and friends, we
to muscle weakness and atrophy. Highly Specialist Paediatric Dietician Jo know we are not in this alone.”
Hulks said: “When I started my career there
‘I love watching him was one type of tube feed. Three decades SMA in the spotlight
experience more each on, there is a large range of tube feeds Last year, Ben spoke to the Scottish
available, including vegan, vegetarian and
day, even if it means food feeds that consider particular allergies. Parliament on the importance of new-
Using ‘normal’ food for tube feeding was
getting everywhere!’ not recommended as there was not enough born screening and the family has shared
their story with the UK SMA Newborn
research or evidence to show it was possible. Screening Alliance:
Ben said: “As Ollie wasn’t diagnosed “Blended feeding for tube-fed patients www.smanewbornscreening.org.uk/
until he was three-months-old, many of his is a relatively new approach. Every patient community-stories
motor neurons had already perished. This has different needs, we have to consider Ben also advised the National Institute
meant he wasn’t able to swallow much milk the nutritional value of each meal to make for Health and Care Excellence as a
and wasn’t putting on weight.” sure they are getting all they need. Some patient expert on Zolgensma and the
Ollie was admitted back to hospital and foods can’t be blended and may block tubes efficacy of pre-symptomatic treatment.
began tube feeding. and of course the families themselves need Amy has been asked by SMA UK to
In May 2021, the NHS treated their first SMA to be confident enough in planning and become a moderator for a parents’
patient with Zolgensma, a one-off gene therapy preparation to get it right. network of newly diagnosed children.
which has the potential – if administered “For some families, it’s exactly what they She will offer advice and support to
early enough – to give babies the ability to need. As a parent, we want to be able to parents as they come to terms with
sit, crawl and walk. Critically, it prevents them provide for our children and that includes what lies ahead for their child.
from having to be put on a ventilator. A life cooking them a dinner. Now with blended Ollie will be taking on a role as an
changing drug, which is also one of the world’s feeding, they can do just that.” ambassador for Designability, the charity
most expensive, at a staggering cost of £1.795 Amy added: “When Ollie was diagnosed, that provides his valued Wizzy Bug
million per single dose. I felt like I had missed out on being able to power chair.
Amy continued: “Ollie was the ninth child wean him, introducing him to new tastes Find out more about our Paediatric Dietetic
to receive this new treatment at the Evelina and textures. With blended feeding, I get Service: www.kentcht.nhs.uk/CND

www.kentcht.nhs.uk 9