Title: My HLH story by Emma

At the beginning of September 2019, I became unwell, little did I know at the time that it would progress to me ending up on an intensive care unit –

It all started off with flu like symptoms – aches, pains and a temperature. However, over time these symptoms progressed – I could barely walk without help, I was having rigors – uncontrollable shaking alongside the fever and instead of improving after 10 days, I was getting worse.

I had such difficulty getting into the system, my symptoms resembled a young girl suffering from glandular fever – a common condition that affects people my age. It took 6 contacts with a healthcare professional before I was finally admitted to hospital. After 6 days on an acute medical ward and all the standard tests coming back negative, I was discharged home with a diagnosis of pericarditis (fluid on the heart) which was the only thing picked up; they thought it was due to an unknown virus that would soon resolve itself.

Oh how little did they know. I returned for repeat blood tests as my infection markers had been very high, when I returned, I was still suffering with all the symptoms but my bloods were much better! This was actually the start of the HLH destroying my blood cells, we just didn’t know it yet.

A couple of days later back at home, I had the worst night I’d experienced so far and found my blood pressure had plummeted and my body was covered in a rash, so my parents called an ambulance. I was seen by so many different specialities including dermatology, cardiology, endocrinology, infectious diseases, acute medical, haematology and eventually ended up on intensive care.

The scans had shown nothing so far but my bloods weren’t good as the phagocytic process had begun (my white blood cells attacking my own healthy cells). My ferritin levels (the protein that stores iron in the blood) had risen to 28,000 – this should be no higher than 150 and I had other signs that were indicating to HLH.

I had a bone marrow biopsy performed which confirmed this and was transferred to a critical care unit within a London Hospital.

At this point, I was terrified. Nobody could tell me I was going to survive this or get better and no one knew what was driving the HLH – a cancer like lymphoma, an infection or a rheumatological disease such as Adult Onset Stills Disease (AOSD). Being a physio who works in an acute hospital usually, I had knowledge of what normal blood results are and so I knew mine were far from it. I thought this must be what it feels like to be dying. I couldn’t turn over in the bed, lift my head, feed myself or do anything.

However, as soon as I arrived in London, I was commenced on a biological drug – Anakinra and amazingly my body responded to this fast. An infection driving this was ruled out by exhausting blood tests, cancer was ruled out by a PET scan and so by process of elimination, plus the signs I was showing initially (muscle/joint pain, pericarditis, a rash) I was diagnosed with secondary HLH, driven by AOSD.

I had to have blood transfused and, with the help of the physios and occupational therapists, build my strength up to do everything for myself again – from standing and walking to using the toilet and showering. I was so weak after being bed bound for weeks. But I was so thankful to be alive and improving. I am still on Anakinra to manage my condition, but I am back home with my family and back doing the job I love.

Despite feeling so lucky, my story still highlights that if more people were aware of HLH and I’d been diagnosed earlier, I probably wouldn’t have been left fighting for my life. I am one of the lucky ones but our voices need to be heard and more awareness needs to be raised, in order to prevent future patients ending up on critical care units or losing their life to this cruel disease.