Lily

The subtype of JIA discussed in Lily's story is Still's disease (also known as Systemic JIA). If you have this subtype, please see our dedicated Still's disease website at www.stillsdisease.co.uk for more information.

"I was a healthy, happy 14 year old girl then one morning I woke up and I couldn’t move. You don’t like your job? Leave it, find something you enjoy. Don’t like the course you took? Change it. Unhappy with your life? Change it. Life is too short."

My name is Lily. I’m 17 years old and am from Newcastle. 

In 2022, when I was only 14, my life changed forever. One morning I woke up and I could not move. After discovering I had suffered from a viral infection a red, hot and itchy rash began to appear. After nights in agony not being able to soothe this itch, the only thing that would calm me was high doses of opioids. It was soon discovered that I had developed Systemic JIA, which is also known as Still’s disease. 

After spending weeks in hospital, I was discharged on a high dose of steroids and a weaning plan to reduce the steroids over time. After a couple of months my rash began to appear again. That’s when I started my short-lived journey on methotrexate. The side effects for me were unbearable. I then trialled tocilizumab which was also relatively short lived as it was not controlling my disease effectively. I was covered in rashes and my joints felt like they were trying to burst beyond my skin. 

Feeling so helpless, being pumped with extraordinary doses of steroid just to keep my head above water, we had to try something new. Whilst on tocilizumab, I was admitted to hospital for something as simple as the common cold! The news was then broken to me that I was starting the treatment of anakinra. This broke my heart due to the idea of injecting myself once a day. Whilst on anakinra, I felt like I had finally reclaimed my life. A drug that was finally controlling my raging, angry disease. I could see my friends, retain energy and enjoy my life - as I was supposed to do all along. 

Having started Anakinra around April 2023, I was admitted to hospital in the following August with swollen glands. I had developed glandular fever. Glandular fever turned my world upside down as I developed Macrophage Activation Syndrome (MAS). MAS is a rare but very serious complication of Systemic JIA / Still’s disease, and ended up being something much harder for me to contend with than Systemic JIA / Still’s disease. Feeling helpless in my bed, missing my summer holiday, I was absolutely gutted. How could something that developed so quickly take so much of my freedom? As I was discharged from the hospital, it was decided that I would take 200mg of anakinra (this is two injections as opposed to the normal dose of 100mg for my weight and age) This did help, however a higher dose of immunosuppressants meant a further risk of infection. In the following October, I developed a Campylobacter infection in my gut (I haven’t quite experienced physical pain like it). I was taken back into hospital and put on IV antibiotics which helped me overcome the horrible infection. Dropping around 7kg in a week I was so very weak and once again I found myself really upset and thinking “Why me? What did I do to ever deserve this?”

I was once again put on a higher dose of steroids. I was also combatting the side effects of that both wonderful and awful drug. I will say, it has saved my life multiple times while making me despise myself simultaneously. The hair thinning, the weight gain, the mood swings - I couldn’t bear to look at my face in the mirror every day. However, I recognise and appreciate that they were extremely necessary in helping my immune system to recover. 

The following December, I was once again admitted to hospital due to elevated liver enzymes. It was starting to feel like it was during every holiday that I was admitted. I was again put on IV steroids and began on cyclosporine with a whole host of other drugs to try and help. After a while, I was discharged. 

You’d think that by then, after all of the help everyone had provided, maybe my body would leave me alone – ha… if only! The following April, marking two years since I was first diagnosed, I was admitted once again due to another MAS episode. By this time, my mock exams had begun. After having already moved school and missing so much of my education, I was truly anxious. 

I was unable to sit my mock exams as my body had failed me once again and there was nothing I could do about it. After having glandular fever, I was sweating constantly and was far too embarrassed to go into school. After my campylobacter infection I felt far too weak. Not to mention the endless days of flare ups, itchy skin and begging for something to take the pain away. Now it was my mock exams. Everything I’d fought for was crumbling before my very eyes. 

Having such a fantastic team of consultants backing me all the way, they took a risk at my request. They allowed me to sit my actual exams after being told I was in no fit state to do so. Weekly IVs of high dose steroids and late LATE nights of revision. I wanted this more than anything and I wouldn’t rest until I got it. With inflammation levels still incredibly high, I began to sit my exams. That was until my third maths exam. I woke up that morning and looked at my arms. Before my eyes was a mottled rash, not the raised rash I’d usually get if I were to flare up. A harsh yet dull pain in my lower back and a swollen face. I woke up my parents and made it very clear to them that there wasn’t a chance I could make it into school for this exam that day. Me being me, so determined to fight my own body, called my headmaster out to my house. I sat that maths exam and achieved a grade 7 - my kidneys were failing as I sat that paper. The next morning I was rushed to A&E, my hands together in prayer. A cannula was put in and morphine was pumped into it. I was asked to give a urine sample after the doctor took my bloods, but I was unable to. 

The next thing I knew, each and every one of my consultants were gathered around my bed holding an oxygen mask with an expression on their face that I still often see in my sleep. “We’re taking you to intensive care.” These six words made my heart drop to my stomach. I was taken to ICU. My mouth so dry and my appetite gone, I was told I was not allowed to drink anything as I was going to theatre the next day. That’s where they would put a central line in me. I was given dialysis, one session much longer than the other. From this, my kidneys began to work efficiently again - yet my head didn’t cooperate. I woke up in my hospital bed, looked to my left where I saw my mother and my nurse. I looked to the clock, asked my mother when the consultants were coming in (it was 9pm and I believed it to be 9am) the next thing I knew, I was convulsing on the floor with the emergency alarm ringing in my ears. I haven’t felt fear quite like it.

When I was back on my bed, I needed to use the toilet and asked to be assisted to it. Whilst literally sitting on the toilet, my head flew back and once again, I found myself on the floor convulsing with the emergency team, many doctors, nurses, resus teams and neuro specialists - you name it – all gathered around me. A later MRI revealed I had suffered from a build up of fluid due to a surge in blood pressure - you could say the dialysis worked too well. Once again, I found myself in intensive care. I remember looking back, it was the day of my prom. Whilst my beautiful friends were all dressed up, there I was. Lying on my bed with a drip in my foot, a mid line, pic and central line, a catheter and greasy hair. I’ve had some lows in my life but I’ve never felt quite as defeated as I did in that moment. I would go on to spend 10 weeks in the hospital. During that time I underwent a lumbar puncture, a bone marrow sample, being attached to an IV drip of anakinra 22/24 hours a day, anti-clotting jabs and a real cocktail of medications.

By this point, my proactive consultants had ordered a new medication from Switzerland. A wonderful drug called Emapalumab. This drug is not easily accessible and had my consultants not applied for it when they did, I may not be here to write this. My ferritin levels had reached >100,000, something unseen by Great Ormond street and the rest of the United Kingdom. Not knowing how I’d react to this drug, many precautions were taken. The whole team of wonderful nurses and consultants watched carefully, staying with me through the entire infusion to make sure I didn’t react badly. This fabulous treatment did in fact give me my life back. After suffering with MAS, each of my inflammation levels plummeted over the 8 weeks I received this treatment. I can’t thank my consultants more. 10 weeks in hospital and talk of a bone marrow transplant when you’re at the lowest point of your life is something I would not wish on my worst enemy. Let alone only being allowed to leave my room for two hours. Seeing my vast improvement, I didn’t need pain medication anymore and was slowly allowed back home for nights. 

Finally, I was able to move to subcutaneous injections rather than being attached to a machine.  I was discharged before exam results day, something I’d begged my consultants for since the first day of admission. Despite not being able to sit my second physics and chemistry paper, I received all grades 6-9. During my time in hospital, I arranged a meeting with the CEO of NHS England, Sir James Mackey. This gave me something to sink my teeth into. I did get to meet him and the wonderful Lucia Pareja. It was an absolute pleasure and we discussed the inequalities in funding of certain sectors and new upcoming drugs that aren’t allowed to be approved. I thoroughly enjoyed getting to meet Sir Jim and Lucia and I am so grateful to have their viewpoints as one of these new drugs is what saved my life. Being in hospital and so close to passing for that amount of time really tests your faith. It makes you take a step back and wonder what really matters in your life. 

Before that point, I was planning to take my A levels, however, I have taken the year out to recover from the trauma that I went through. Our lovely psychologist from the hospital, Jenny, came to visit me and my family every day during my admission. This helped greatly. What I went through isn’t an experience that I will be forgetting anytime soon and to everyone who has heard of my case, it is really one in a million. For a long time after, I struggled to find a point in anything - if I nearly passed and we all do eventually, then what is the point? I took up a very nihilistic point of view and felt so guilty for not being happy that I was well and wanting to make the most of it. I fought this feeling over time - there was no point in feeling guilty. What I went through was bound to change me and thinking that way helped me to come to terms with what had happened. It helped me to understand my priorities - being happy. 

I am currently off steroids (woooo!!) and am down to two injections a day and on no other pain medication. Since being diagnosed this is the longest I have gone without being admitted to hospital.

My disease has made me a different person and I often wonder who I would be if none of it had happened. I won’t ever know. I am proud of everything I have overcome and want to inspire people by sharing my story. A sixteen year old girl should never have to have gone through what I did. That’s why it is so important that we keep researching and funding new medications. Rare diseases need to be recognised. Just because they are rare doesn’t mean they make people suffer any less. My grandad showed me a quote that has resonated with me ever since being diagnosed: “You don’t know how strong you are until being strong is your only choice.” Make the most of your health. 

I was a healthy, happy 14 year old girl then one morning I woke up and I couldn’t move. You don’t like your job? Leave it, find something you enjoy. Don’t like the course you took? Change it. Unhappy with your life? Change it. Life is too short. Make new friends, fall in love, laugh and smile and never stop being kind.

If you would like to share your JIA story, please get in touch.

For information and resources about Still's disease (Systemic JIA), please see our dedicated Still's disease website at www.stillsdisease.co.uk

If you are concerned that a child or young person may have JIA and would like to know more, please visit www.thinkJIA.org

For support at school, please visit our Parent Zone. Schools can request an information toolkit to support children and young people with JIA in their setting at www.jarproject.org/toolkit 

Many of the resources we have developed can support children and young people at school and with explaining their condition to others such as our "I have JIA" cards. You can access these here.

For support with Mental health, please see our Mental health and wellbeing page here.  

You can also help us be there to make life better for children and families affected by JIA by supporting our work at www.jarproject.org/fundraising