Palliative care is more than you think

Well over 300 children and their families are cared for each year by Sydney Children’s Hospitals Network’s (SCHN) Palliative Care Services. There may be no cure for their underlying medical condition, but meeting with Paediatric Palliative Care means that a team of dedicated specialists will walk alongside each family and ensure that whatever time they have together, be it weeks or years, is supported. Paediatric palliative care – It’s more than you think.

The inter-disciplinary Palliative Care team works together with other care teams to provide psychosocial support, respite care, symptom management, end-of-life care and bereavement support. Dr Susan Trethewie, Department Head explains that their goal is to ensure that children with a life-limiting illness and their family receive the best and most individualised care to maximise quality of life, create joy, improve function and foster inclusion.

We offer specialised programs in addition to medical care, such as music therapy, hydrotherapy, and supported memory making programs both in-person and via telehealth. We also support health care professionals to facilitate difficult discussions about decision making and establishing goals of care when a child’s health declines.” Dr Susan Trethewie, Head of Palliative Care, SCHN.

The reach of the Palliative Care service extends well beyond the walls of our hospitals and hospice, Bear Cottage.The work of the SCHN team is state-wide, connecting families with community palliative care services and educating local teams to support the needs of children with life-limiting conditions, keeping them at home and close to family and loved ones as much as possible.

Matilda was diagnosed with CLN2, or Late-Infantile Batten disease, just before her fourth birthday and a connection with the Palliative Care team ensured the best possible quality of life for the whole family. But when her health began to change for the worse, the team was crucial in setting up community-based support for Matilda. Local palliative care service, Silver Chain and particularly nurse practitioner, Helen, supported Matilda and her family throughout her final weeks, Helen’s presence incredibly reassuring for Matilda’s whole family;

Silver Chain provided great care for Matilda, but I could also talk with them about what was worrying me, what I was fearful about. Anything beyond that, the expertise and liaison with the hospital team was invaluable, they worked together to fast track certain things for Matilda, so the combination was important. Helen’s presence reassured me that it was ok to be home with her and that I was supported, and the fact that I could call Silver Chain and they would come day or night, that was really comforting.” Liz, Matilda’s mum.

Helen’s former career caring for children with cancer well equipped her to step into Matilda’s team, however as community palliative care services are adult based, having the connection with SCHN services meant ongoing support, education and peace of mind.

Caring for children at end of life is very different to adults, and as families have such a long-standing relationship with the hospital Palliative Care team, establishing trust is really important. I called the SCHN team to discuss changes to medication or to confirm an approach to end-of-life care that is sometimes different to the care we provide to adults, it all worked well. Everyone is there for the right reasons, which is to deliver the best care for the child, where ever that may be.” Helen, Nurse Practitioner, Silver Chain.

Knowing that all the teams were communicating enabled Liz and her husband, Jeremy to keep Matilda comfortable at home, feeling empowered and supported. The result of good relationships and good communication with each of the treating teams,” adds Helen.

Bear Cottage, the only children’s hospice in NSW is also managed by SCHN and provides essential respite and end-of-life care for patients and their families. Like the foundations of the hospital-based services, a stay at Bear Cottage is about living life to the fullest, making precious memories – and in mum, Charmaine’s experience, providing a place where she and her boys can just “breathe”.

Charmaine and her boys first came to Bear Cottage two years ago. She was very reluctant at first and still in a state of exhaustion, shock and having difficulty processing everything. Charmaine’s four-year-old son Marcus developed debilitating and constant seizures soon after his first birthday,

“He has all types of seizures meaning he needs all-day care. If he gets too hot, he seizures; if he’s too cold, he seizures; if he doesn’t get enough sleep – yes, he seizures! The stress on me is hard to describe!”

After recommendations by hospital staff, and encouraged by other parents she met during hospital stays, she decided to see for herself.

“The Bear Cottage team make us feel like our whole family is special and cared for – as though we are the only people, the only ones who truly matter. Bear Cottage is not a sad place. When things are tough it is a place that allows you to live again – to live well as a family and enjoy each other’s company, no matter what we are facing.” Charmaine, Marcus' mum. 

It is National Palliative Care Week 23-29 May 2021, and we are acknowledging the children across SCHN who have life-limiting conditions, and the hard work of dedicated staff and their parents who advocate for these children each and every day.

Visit this website for education and support resources created by the SCHN team, together with from colleagues across the state.

 

Remembering Matilda 

Matilda was born a healthy baby girl and arrived in the arms of her parents, Liz and Jeremy in August 2011. Liz had no reason to suspect anything unusual as her social, happy baby met her milestones and grew into an adventurous two-year-old. However, the family’s world changed one day in 2014 when Matilda had her first seizure.

“She had her first febrile convulsion at two-and-a-half. Then she had a second one, and a month before her third birthday, she began having seizures and was diagnosed with epilepsy,” said Liz.

About a year later, those seizures would be the basis of an even more shattering diagnosis, CLN2. Better described as Late-Infantile Batten disease, a form of childhood dementia.

“It was devastating. Batten disease is a rare genetic neurodegenerative disorder, it steals skills and ability and eventually the child's life. There is currently no cure,” said Liz.

While Liz, Jeremy, and new baby sister Rose navigated a scary and uncertain future and a new world of hospital visits, treatment regimens and medications, they were also encouraged to meet with the Palliative Care team.

“When you are faced with a child whose condition child is never going to improve, not many people can give you advice or support you. Having an early connection with palliative care was certainly confronting, but ended up amazing; they were the people that actually got it. They were able to give me advice that no one else can give, in both the ‘right now’ and also looking ahead and making sure supports are in place as things change and are changing for the worse. I think they’re amazing." Liz, Matilda's mum. 

Throughout the next few years Matilda and her family were able to take part in many therapies and services that helped improve her quality of life and that of the whole family. Matilda loved music and often engaged with the team’s music therapists, Liz says that although the way she communicated changed as she lost her speech and facial expressions, I saw her light up when she heard a familiar song.

“And not only were these experiences important for Matilda, if Rose was home the team would naturally engage with her to make it a family experience, it’s those moments that are truly valuable,” said Liz.  

When Matilda’s health began changing for the worse, the hospital’s Palliative Care team were crucial in establishing the best community-based support for Matilda. Palliative care service, Silver Chain and particularly nurse practitioner, Helen, supported Matilda and her family throughout her final weeks, Helen’s presence incredibly reassuring.

Helen’s former career caring for children with cancer well equipped her to step into Matilda’s team, however as community palliative care services are adult based and with typically less six children cared for by Silver Chain each year, having the connection with SCHN services meant ongoing support, education and peace of mind. 

“Caring for children at the end of life feels different to adults, a child and their family often have a long-standing relationship with the inpatient teams. You are coming into the relationship at the end of life, establishing trust is incredibly important for this reason. It’s about connection, relationship, dignity, comfort and impeccable assessment and good medicine if it is required. We work incredibly well with the children’s hospitals and Bear Cottage, I felt supported as a clinician at all times. Assisting in goodbye work and helping to create meaningful moments is an absolute privilege," Helen, Nurse Practitioner, Silver Chain

Knowing that all the teams were communicating enabled Liz and Jeremy to keep Matilda comfortable at home, feeling empowered and supported. The result of good relationships and good communication with each of the treating teams,” adds Helen.

Matilda peacefully left in January 2021. She will be forever nine.

Meet Marcus 

Charmaine is a busy mum of four boys. Her youngest is 4-year-old Marcus – a beautiful boy born with a rare and devastating genetic condition called FBXL4 Mitochondrial DNA depletion syndrome. It’s the rarest in the world and there is no cure.

Marcus’ birth was traumatic, with both Marcus and Charmaine almost dying during his delivery. “I met Marcus for five minutes after he was born and he was whisked away to spend his first weeks in the NICU. That was where our journey started,” she said.

Just before his first birthday, Marcus developed debilitating and constant seizures. “He has all types of seizures meaning he needs all-day care. If he gets too hot, he seizures; if he’s too cold, he seizures; if he doesn’t get enough sleep – yes, he seizures! The stress on me is hard to describe!”

Marcus’ condition means that his body doesn’t produce enough energy in his blood cells to efficiently run all his organs and brain. As Charmaine says “it’s like running a house off a single battery”.  He tires easily, is unable to sit up, needs to be machine-fed and is unable to drink orally. He has poor muscle tone, limited sight and his hearing is affected. 

“The doctors told me they didn’t expect Marcus to live beyond the age of three and I believe he probably would have died by now if he hadn’t absorbed some of this positive attitude from me, as well as all the love we continue to give him. He wants to be part of everything and my wish is for Marcus to enjoy many special times with me and with his brothers. He loves his family and never lets us down. He is my refill – which I know is also a big burden for him to carry. And it’s hard to accept that he is also someone I could lose.” Charmaine, Marcus' mum. 

Inspired by Marcus’ strength, Charmaine resolutely perseveres and as a single parent, with Ayden aged 11, Ethyn aged 9 and Josiah aged 14 to also care for, she remains passionately committed to ensuring all four boys receive the love and attention they deserve.

Charmaine and her boys first came to Bear Cottage two years ago. She was very reluctant at first and still in a state of exhaustion, shock and having difficulty processing everything. After recommendations by hospital staff, and encouraged by other parents she met during hospital stays, she decided to see for herself.

“I made the decision when I came that I was going to hand over Marcus’ care. Despite the guilt, I know that I have to put my trust in someone and at Bear Cottage I can do that. It really is also the only place I can get a full night’s sleep.

“The boys are able to do fun things together with Marcus, such as music and craft, games, spa days and walks to the beach. At Bear Cottage, there are people who truly care about our family. 

It makes us feel like our whole family is special and cared for – as though we are the only people here, the only ones who truly matter. At Bear Cottage, you forget you’re AWAY from home because you feel like you ARE at home."

“It’s also important for all my sons to meet other families facing similar issues. They are learning things in this environment that some kids will not learn in a lifetime – compassion and kindness. Because Marcus’ needs are taken care of, it means the boys also get to enjoy quality time with me alone, which can’t happen at home without being interrupted. 

“It also allows us to make memories which are so important – the photos from our stays at Bear Cottage are so precious and a reminder that all my boys are special. The friendships I have made with other families are also amazing, because they often check up on us, and offer plenty of practical advice and support –- they are the ones who really understand what we are going through.”

 “My message to anyone is, Bear Cottage is not a sad place. When things are tough it is a place that allows you to live again – to live well as a family and enjoy each other’s company, no matter what we are facing.” Charmaine, Marcu's mum. 

Palliative Care – it really is more than you think.

The Warrior of Love

Marcus the Mito Warrior should never be underestimated.

His love for others never berated.

He is gentle, resilient and kind,

Yet one of the strongest boys ever you’ll find.

Yes he has been dealt a real tough blow

Yet he deals with it, the best anyone can know.

 

I absolutely cannot forget the day

When ten teams gathered in a room to say,

If Marcus survives, it's going to be tough.

Charmaine, you know, life will be rough

There's so much wrong, that cannot be righted

It was then, the warrior spark was ignited.

 

The warrior fought every battle he could,

With fortitude and honour as good boys should.

The blows kept coming but he bounced back strong,

Taking all that loved him all along

Through unfathomable depths all around town

His rollercoaster adventure going up and down.

 

The warrior’s battles are known afar

A legend that makes him an Tik Tok star

His smile crushes every bad thought

Inspiration this big, cannot be taught

His weapon, a huge smile and cheeky big grin

If you believe in the warrior, you'll always win.

This poem was written by Dr Kaustuv Bhattacharya, SCHN Staff Specialist and it sums up Marcus and all the brave and resilient children who visit Bear Cottage – they and their families are the true ‘warriors of love’ we like to celebrate and champion during this special week.