HLH - share your voice


Share your voice - HLH Paediatric​

 Lili – by Mum

On February 1st, 2021, my life changed forever. My 14 year old daughter, Lili, was admitted to the Makarios Children’s Hospital, Nicosia, Cyprus, where she was diagnosed with HLH, a rare and devastating autoimmune disease, that can strike at any time, any age, and often with no warning symptoms.

We were lucky in that Lils got such a quick diagnosis of HLH, as there aren’t many medical professionals who have heard of it, let alone dealt with HLH, and treatment started immediately. Lils brilliant medical team, battled heroically against this vicious and extremely aggressive disease for 13 days, never giving up hope, celebrating every tiny positive step Lils made towards slowing down the destructive avalanche effect that HLH wreaks on the body, and I will never be able to thank them enough for everything they did for Lils.

On February 13th, 2021, Lils passed away as a direct consequence of HLH. Lils story doesn’t end on February 13th, 2021. I created a website: Lili Kyriacou HLH Awareness page in March 2021. Lils death from HLH is the driving force behind the Lili Kyriacou HLH Awareness Page, not because I’m a grieving mother and want to memorialise Lils, but because Lils ambition was to become a Euro MP, so she could make a difference and help people around the world, which is what this website is about. We want to be able to bring more information about HLH to both the general public and to medical professionals, as although there is some information available, it really isn’t enough. Our ultimate goal is helping and changing world understanding of HLH, and other rare blood diseases.

Violets in Bloom – A Beautiful Baby Girl by Mum

Violet Lilian Mornington was born on 3rd June 2008 at Samuel Johnson Hospital in Lichfield to Kerry and Ivan. A happy, healthy, sometimes very silly and a tiny bit naughty little girl, she completely rocked her Mummy and Daddy’s world. ‘Life was never boring with Vi, she was into everything, always on the go and making us laugh’.

Violet had so many friends firstly through her Nursery, Animal Crackers and then when she started school in September 2012 at St Stephens in Fradley, where she lived. She loved school and she loved her friends.

In 2013, Violet was coming on in leaps and bounds with her reading and writing, she had learnt to swim at her swimming lessons and mum and dad were planning on taking the stabilisers off her bike ready for the Summer. Things were good and Violet’s most favourite things in the whole world were her books and her teddy bears.

It all changed
On her 5th Birthday (03 June 2013) this all changed when Violet came home from school feeling a little unwell. We thought maybe she was a bit over excited about her birthday’. When Violet didn’t seem to pick up after few days and after several trips to the doctors, Violet was taken to A&E where she was immediately transferred to Birmingham Children’s Hospital where for several weeks she left Doctors baffled as to what was wrong.

Doctors ruled out Leukaemia and other weird and wonderful viruses, but they were completely stumped. They could not figure out why Violet’s bloods were so ‘bonkers’, as they put it.

They couldn’t understand why she was having raging temperatures up to six times each day, why her spleen had doubled in size and why she was too tired to even get out of bed to have a little play in the playroom.

Finally, after three weeks in hospital, they diagnosed Epstein Barr Virus associated Hodgkin’s Lymphoma and set about her first lot of treatment. However, it soon transpired that there was something more sinister going on with poor Violet and they suspected a rare blood disorder called HLH (Hemophagocytic Lymphohistiocytosis), a potentially fatal disease of the blood if not diagnosed and treated in time.

Despite starting chemotherapy treatment, the disease had gotten hold of Violet and on Saturday 20th July she started to have severe respiratory problems. After 24 hours of fighting so hard to stay with us, little Violet sadly lost her fight and she passed away on 21st July 2013. She died peacefully in the arms of her Mommy and Daddy.

Violet will be remembered and loved by everyone who knew her for her sense of humour, her beautiful smile, her strong personality, for her individuality and for the amazing courage and bravery she showed during her illness.

Isobel the Griscelli Supergirl by Mum

Isobel the Griscelli Supergirl by MumClippers Diagnosis
Isobel was 9 when a routine optician test showed there was an issue at the back of her right eye. Where the synapses meet at the back of the eye instead of a smooth oval there was a raised bump. Regular eye check-ups over 18 months showed no change in growth, there were no headaches or problems with vision and as she was not in pain, it was agreed that she would be discharged at the next appointment. However, 4 weeks before that appointment Isobel started to complain of having double vision. 2 weeks before the appointment her right eye turned in. At the appointment, she was booked in for a 20-minute MRI.

10 days later, we received an evening phone call that no parent wants to have. There was a problem with the MRI, which showed inflammation spots scattered throughout Isobel’s brain, particularly behind the right eye and at the top of her spine. The doctors were unsure what it was, and we needed to come in the next day for more tests to be done on a children’s ward. Isobel, now 11, had a barrage of tests, including a 50-minute contrast MRI and a lumbar puncture. Within 3 days, thanks to a sharp-eyed radiographer, we had a diagnosis of Clippers, an auto-immune illness of the brain and one of only 60 known cases in the world. Isobel has tried various medicines over the last 2 years and the inflammation responds well to steroids, which she is still on; without them her balance and vision are severely affected.

Griscelli and HLH Diagnosis
Our wonderful Clippers specialist has been working closely with Great Ormond Street Hospital (GOSH) on Isobel’s case and over the last 2 years there has been genetic research to show that Clippers is not an illness in itself but a symptom of an underlying cause. Isobel, now age 14, went to GOSH to meet the team, have bloods taken and a lock of her hair to test for various genes. Recently we received the dreaded news that Isobel has tested positive for a very rare hereditary genetic condition called Griscelli, under the umbrella of HLH, a life-threatening auto-immune illness of the blood. there are 60 known cases of Griscelli in the world. The only hope of a cure is chemotherapy and a bone marrow transplant.

Over summer 2019 Isobel has had numerous tests, appointments and treatment discussions. She has been further diagnosed with ‘isolated neurological HLH’ one of 11 known cases in Europe as her HLH symptoms are so rare. Doctors say they have never seen anyone “so well” with this illness despite inflammation spots presenting in her brain and spine. Further treatment has been hotly debated between GOSH and Birmingham Children’s Hospital and they finally agree that she will continue to be closely monitored and the search for a donor match will go on. It is expected her symptoms won’t always respond so well to steroids and doctors want to be ready to move quickly if and when necessary. For now, we feel that we have had a reprieve and can plan some future events with family and friends without worrying we will get ‘the call’ for a Bone Marrow Transplant. Further donor events are in the pipeline for Isobel and for so many others in more dire need of a BMT.

Isobel isn’t the only one who has had tests! Kev and I had the results of our genetic tests. Everyone has 2 working Griscelli genes (RAB27A) which carry melatonin around the body. Amazingly Kev and I have only 1 functioning gene each and have passed on the non-working gene to poor Isobel. The chances of us both being affected were millions to one, the chance of passing it on were 1in 4. Myles is now having testing and genetic counselling to see if he is a carrier and to see if he could be a possible donor for Isobel.

People have commented on Isobel’s unusual colouring- white blonde hair and olive- toned skin since she was a baby- just like her elder brother. When the GOSH team wanted to test her for Griscelli (a genetic abnormality in gene RAB27) we were convinced she couldn’t have it, as Griscelli children are described as having very pale, translucent skin and Griscelli is under the albino umbrella. We were stunned when she tested positive. Griscelli is considered life threatening but Isobel is currently classed as atypical as the illness hasn’t attacked her body as it seems to in others.

Layla’s Rainbow

Laylas rainbow

I know all parents think their child is special, but Layla really was. Always ahead of her time. She was walking at just nine months, out of nappies by her second birthday and at 2 years old, you could pretty much have a full conversation with her. She had something about her.  Wise beyond her years almost like she’d been here before. She had a very loving, caring nature and always shown empathy towards others even at such a young age. She was popular at school and loved her friends. She’d put her arm around them and talk to them like she was the grown up and they were the child. She had an old soul for a little girl and often people would say she was like a ‘little old woman’. 

Layla also loved to sing and dance. She was a great little entertainer, always doing shows for us and she was funny with it too without even trying to be. She oozed confidence and charisma and would definitely stand out in a crowd. You’d always remember our Layla. With her wild ginger curls, infectious smile and her big personality, she stole the hearts of all who knew her. What we didn’t realise was, she was only borrowed to us for 6 short years and was needed in a better life. She was needed in Heaven. 

On November 24th, 2017, I had a call from school to pick Layla up as she wasn’t feeling very well. When I arrived, she was lying down with temperature. I thought a bit of Calpol and cuddles from Mummy would do the trick and had no doubt she’d be back at school after the weekend.

Unfortunately, that wasn’t the case. By the Sunday evening, she’d become so lethargic and spiked a temperature of 40.9. We also noticed she had come out with an unusual looking rash. We made our way to A&E. After her first blood test, her CRP levels (which detect inflammation in the body) were 300 plus. Which suggested an infection and a very nasty one at that. We thought at that stage we were dealing with Sepsis or Meningitis.

She was transferred to Bolton Royal where she was treated for over a week with antibiotics, but Layla wasn’t showing any signs of improving. They’d ruled out Sepsis and Meningitis after a series of tests and Layla’s illness was becoming more and more of a Mystery. The Doctors at Bolton told us there was nothing more they could do for Layla and the specialist’s teams at Manchester Royal Infirmary wanted Layla there. I could see in the doctors and nurses faces that even they were starting to worry and the fact they called us an emergency ambulance to MRI made me think we were dealing with something life-threatening.

The team at Manchester were brilliant and reassured us that she was now in the best care and they would make her better. But Layla’s health continued to deteriorate, and she was placed in a high dependency unit while doctors continued to run more tests as they still didn’t have a confirmed diagnosis. 
On Saturday 9th December, Layla took a turn for the worst and was rushed into intensive care and put into an induced coma. The docs warned us then, she may not survive but against the odds, she stayed strong and continued to fight. Whilst in a coma, the docs took a sample of Layla’s bone marrow and shortly after that they were able to diagnose Layla with an extremely rare blood disease called HLH which is short for hemophagocytic lymphohistiocytosis. Of course, we’d never heard of it but as we started to research, it didn’t make sense. How could this happen to our Layla? She was singing and dancing at a Little Mix concert only two days before. She was never sick!! she was everything a normal happy healthy little girl should be! 

We were told to expect a long road to recovery for Layla. At least an eight-week hospital stay and months of chemotherapy. We prepared ourselves for this and was prepared to do whatever it took to make our girl better. We just wanted her to wake up. 

On the 15th Dec, her 6th Birthday, she opened her eyes. We put up balloons by her bed and her brother and sister and grandparents all came to see her. It was a happy moment but also very sad that she was spending her birthday in the hospital and so poorly. Not quite the birthday we had promised her. However, the docs were really pleased with Layla’s progress and moved her into an oncology ward which would become our home until Layla was well enough to come home. 

Unfortunately, the very next day, Layla’s health deteriorated quickly and drastically, and she ended up back in Intensive Care and put back in an induced Coma. Layla was bleeding internally, and they sadly couldn’t stop the bleed. The machine by this point was the only thing keeping her alive. It was the worst two days of our life. On the Sunday night, while we were sleeping, the hospital called us to tell us that Layla was unstable and basically prepared us for the worst. We called our parents who arrived shortly afterward and we all stood round Layla while they fought through the night to save her. It was not meant to be, she was too poorly. It was time to say goodbye. 

Our beautiful daughter passed away at 5.05am on the 18th December and our lives changed forever. 

There’s a chance Layla may have survived had they diagnosed her sooner. Therefore, I am very passionate about raising money for this charity to help fund the research needed to uncover the causes of HLH, diagnose early and hopefully finding a cure. We hope that no other parent has to go through what we’ve been through. 

Thank you in advance, Michelle, Paul & family and of course Layla xxxxx

HLH the impact on our family by Andrea & Paul Scholes.

Share your voice – Your story HLH

The views expressed below are those of the writer and do not reflect views and or opinions of the charity any products mentioned are available from a variety of retailers.

Title: Paediatric HLH – our worst nightmare…

Intro: Debbie-Sue Price re-counts the terrible moment she found out her son, Aaron, had a life-threatening immune disease, HLH, and how all parents should listen to their gut instinct when it comes to a poorly child…

My son Aaron got chicken pox last year in May, two weeks after his twin sister. Five days later, when the spots had scabbed over, I thought he would be returning to nursery but that night Aaron spiked a fever of 39+. Despite giving him Calpol, his fever didn’t subside. We made numerous trips to our GP’s surgery and local hospital every day for a week until they finally admitted Aaron.

It was a good job that my husband and I did keep going back, as we soon learned that the chicken pox had sent Aaron’s immune system into overdrive and he had secondary Haemophagocytic Lymphohistiocytosis (HLH).

HLH is an immune disease and is most common in babies and toddlers; however, it can affect people of any age. It can become fatal rapidly and without effective treatment most patients will die.

The treatment for HLH is chemotherapy, high dose steroids and, in some cases, a bone marrow transplant. Thankfully, HLH is rare – there is a one in a million chance worldwide of getting it – but because of this doctors and parents might not spot the early signs so it’s important to raise awareness.

The initial symptoms of HLH mimic the flu and many parents’ stories begin the same way: “our child is sick, not getting better and we are being dismissed with “it’s just a virus, your child needs more antibiotics and time; they’ll be fine”. It’s important to keep in mind the most common symptoms of HLH are a fever, enlarged spleen, low blood counts and liver abnormalities.

Aaron was transferred to Birmingham Children’s Hospital (BCH) and stayed there for three months; with two months in Intensive Care and High Dependency. Our Christmas came early last year when Aaron was discharged in early October, albeit with a bag full of drugs that he had to take each day. We celebrated with a Halloween party.

My husband had to take Aaron to BCH twice a month for his chemo until he completed his course just recently this month.

I don’t want to cause panic but if you think something is seriously wrong or your child has a fever despite medication for over five days (a fever is classed as over 37.5). Speak up and trust your instincts. If your doctor hasn’t already done so, ask for blood tests (including a Ferritin test) and a complete blood count (CBC).

Aaron has been very lucky. If it wasn’t for our cousin, Shahenda, a paediatrician in the US, who spoke directly to our consultant in hospital and insisted on vital blood tests, Aaron might not be with us today.

Share your story with the media

By raising awareness of histiocytosis in the national and local press we can reach more people. We rely on personal stories to help us engage readers around our cause and bring our stories to life.

If you are interested in sharing your story on our website or in a magazine, newspaper or on TV please send a 100-word summary of your story along with your name, contact details and a recent photo to Histio@histiouk.org. We regret that we are unable to respond to everyone, but we really appreciate you getting in touch.

Why should I share my story?
We want to help as many people as possible during their histiocytosis experience – patients, carers, families and communities. By raising awareness of what we do in national newspapers, magazines, on TV and in the local press we can reach more people.

We rely on personal stories to help us do this as they engage the reader and bring the story to life. For example, if we provide research for a newspaper showing that histiocytosis can affect people financially it has more of an impact if it’s accompanied by an emotive quote or story of someone who has been directly affected and helped by us.

Your story can encourage those who see it to get help if they are worried they have the symptoms of histiocytosis or may inspire them to donate or fundraise for us. Some people who have shared their story find it a cathartic experience. It makes them feel good to give something back.

Where would my story appear?
As well as our website, we work with a range of media from broadcasters like the BBC, national newspapers like The Times, Daily Mail and Guardian and women’s magazines like Woman’s Own and Good Housekeeping.

They all have different requirements for their stories so we would approach those which we think are most appropriate, depending on your story.

Do I have to be identified/pictured?
Yes, all of the magazines and newspaper ask that the people they interview are pictured. Some ask you to send/email them photos to use while others send a photographer to take photos.

Will I have a say over what’s written?
By sending us your story you agree to its publication on our website or on or in any publication that may collect news feeds or create editorial content.

Request by journalist for contact details will be agreed with you in advance of disclosure.

Please be aware that whilst some journalists offer to read your story before it goes to print to check facts it depends on the publication and this cannot be guaranteed.

What happens next?
The journalist will try tell you when your story will appear so you can get a copy and tell all your friends. We ask the journalists to include information about Histiocytosis UK Support’s campaigns and services and you can feel proud that you’ve helped deliver our message.

If you are interested in sharing your story in a magazine, newspaper or on TV please send a 100-word summary of your story along with your name, contact details and a recent photo to Histio@histiouk.org. We regret that we are unable to respond to everyone, but we really appreciate you getting in touch.

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