A diagnosis

Hemophagocytic Lymphohistiocytosis

Yeah… What the title says!

HEE-moh-Fa-goh-SIH-tik  LIM-foh-HIS-tee-oh-sy-TOH-sis

HLH for short.

I walked into the neonatal unit the day after Arlo had his bone marrow aspirate. I was there unusually late that evening. That afternoon I had popped into Liverpool for supplies, nappies and cotton wool for the most part, but also some food and supplies too. I had taken the bus there and back and before going back to the flat I called in to see Arlo, feeling incredibly guilty about nipping to town for an hour or so and not being with him.

The white lights seemed harsh against the dark sky outside. Normally at this time, unless there was a reason for the bright lights, they were dimmed because it was night-time and the babies had to get used to day and night even in the neonatal unit. The fact the lights were on signalled there was something happening. But there was nothing immediately obvious going on. I walked to Arlo’s bedside which seemed to be busy, but with nothing in particular; nothing to worry about. Arlo was having some medication given and some infusions changed, but as with everything in neonatal, it required two members of staff to make absolutely sure the medication was correct and the minuscule dosage was perfect for his weight. There was a calm but electric atmosphere around Arlo, yet I couldn’t quite work out why or where it was emanating from. The consultant was consulting with the nurses around Arlo’s bed. The consultant was The Professor or ‘Prof’ as he was known around the unit.

He looked up and saw me, and made towards me immediately. Then I realised the excited energy was coming from him.

“We have some results back from the bone marrow aspirate…” The initial thought that flew, unwelcome, into my mind, was that they had found nothing. Arlo was a mystery after all and wanted to continue to be… I had already written off what was coming next, ready to question the doctor what was the next level of investigation.

But what he said next stopped me in my tracks…  

“…We have a diagnosis.”

It was one of those moments where time stood still. I was desperate to know, yet terrified of what I was going to hear at the same time.

If something was so incredibly rare, that had had everyone scratching their heads for so long. If it was that rare, would there be a cure?

“Hemophagocytic Lymphohistiocytosis...”

I glared at him. I thought I was getting used to the medical jargon that was thrown at me. But it became clear to me that this was no longer the case.

“…Or HLH for short.”

Well that was slightly more manageable for me to tell people.

He then went on to explain what HLH was.

The reason for the HLH diagnosis was because they had seen his red blood cells being eaten by other cells, the body’s defence mechanism. This defence mechanism had gone into overdrive and along with cleaning up Arlo’s body and fighting the germs that may have snuck in; they were eating Arlo’s blood cells too, therefore were stopping him from producing any white blood cells and munching any red blood cells that were produced. This was the reason for so many blood transfusions.

This link may explain it better than me...

https://youtu.be/Vxoy5328MZc

Of course he wasn’t going to give it all away, in true Arlo style, it was INCREDIBLY rare and not one of the medical professionals at the hospital had ever heard of it before. It was a lot to take in, but it the endless downpour of information wasn’t over yet. They had already done their research and found out the recommended first line of treatment. It was a steroid called Dexamethasone, but there were risks.

One of the risks could be that Arlo could be more susceptible to infection, plus other side effects which could be risky. But the only other option was leaving Arlo without any treatment to see if he would, himself, start to produce some white blood cells. By the point of diagnosis Arlo was over 4 weeks old and had never had a white blood cell to his name. I knew in my heart we had to try it, but I knew I had to phone Day so we could make the decision together. Although it was said it may leave Arlo more susceptible to infection, the doctor agreed that he couldn’t really be much more prone to infection without any white blood cells. He also informed me it may actually help Arlo’s lung development, so that may actually be a bonus.

A quick phone call to Day followed, where I summarised what the doctor had said. The decision was swift; we had to give it a go. Nothing that had been tried so far had worked.

Returning to Arlo’s room I gave the doctor our consent. We had to try. We had to give him everything we could to help him to develop some white blood cells and give him the ability to fight infections and stop his body from attacking itself. The first dose would be administered to him in the morning; there was no time to waste.

After the decision was made and the wheels were in motion, I realised the nurses had moved on to doing Arlo’s cares while I was talking to the Doctor. It always upset me and, if I’m honest, angry when someone else did Arlo’s cares. It should be me changing his nappy and doing everything he needs, not them. This was amplified because I felt so helpless, most of them time I just sat and watched the doctors and nurses as they cared for Arlo, giving him everything he needed through his wires when it still should be me. He should still be nestled inside my stomach; cosy, warm and safe, and because he wasn’t, I wanted to do as much as possible for him and it hurt to watch others do it.

Although, letting someone else do his cares this time it was for a reason, a very important reason we were moving forward, but it didn’t hurt any less. The nurses must have picked up on my feelings as they stepped aside and I smothered the cream all over Arlo’s body to sooth and moisturise his skin. He seemed so settled with no idea of anything that was going on around him at that moment, of all the decisions that were being made all around him. As settled as he could be with wires connected to almost all his tiny limbs, the puff of the ventilator and the beeping of all the machines that surrounded him. When I looked at it like that I felt the tears burn my eyes. I still had to fight the urge to snatch Arlo up and take him away from all this craziness. I just wanted the world to stop turning and spend some time with my boy without all the beeping machines, medicines and decisions to be made. To take him to a place where we could all be a family as we should be, where we would be part of a different type of craziness; the craziness of our family home. None of this should be happening; we should be like every other family. We had been on this road for 4 weeks, but the road had been more of a treadmill, it kept turning over, occasionally there was a slightly different pattern underfoot, but on the whole it was a treadmill. The same thing day in, day out over and over again. Now things were shifting, with a diagnosis I dared to hope that we may start moving forward with a plan and treatment in place. I looked down at this tiny fighter, who was already fighting so hard to stay with us and desperately pleaded that this medication would help Arlo to develop some white blood cells.

As I left Arlo for the night, I whispered for him to keep fighting and walked back to the flat I felt almost excited! I was excited about pushing forward and solving this so we could bring our baby home.

By the time I returned to the flat which was only a short 5 minute walk, however, I was starting to feel scared and alone. Everything Arlo had was so incredibly rare I didn’t know where to turn. Once again we had no one to turn to for advice or support. It wasn’t the physicality of being alone, because even though I was, I had people I could call or message whenever I needed. It was that we were alone on this journey, it wasn’t that we wanted people to tell us they had been there before and that everything was fine. I wanted to know the good, the bad and the ugly. I just wanted someone with experience to speak to.

Sitting down for a late tea, I received a message from Ciara at Little Heartbeats. She was like a guardian angel and had been messaging me all along; since that fateful scan, home bed rest, hospital stay, Arlo’s birth and along the neonatal journey, every step on the treadmill.  I shared with Ciara the diagnosis Arlo had been given, she (along with every single other person I told) had never heard of it, but she told me to leave it with her. I didn’t hold out any hope.

After expressing I retired to bed. I felt enough was enough for today. I had briefly googled HLH earlier on, but being given the news so late in the evening, the words swam on the page in front of me, no information able to penetrate my already oversaturated brain. I expected to fall into a fitful sleep; instead I was out like a light into a dark, heavy sleep, having to drag open my eyelids to express in the depth of the night.

The next morning, back by Arlo’s side, I felt stronger and ready to fight, just like he was every single minute of his life. Sitting there gently holding his hand, I received a message from Ciara. Not only had she found a support group for people with HLH, but she had found a newspaper article dated just a couple of days before hand about a little baby from Ireland who was being treated in Great Ormond Street Hospital in London. She sent me the link to this article, along with the Facebook group that was set up in his name. His name was Shaye, he had HLH and he was born at 31 weeks gestation. Immediately, after reading the article I sent a message to the Facebook group, receiving and almost instantaneous reply saying the message would be passed on to Shaye’s mum.

Here is that very article!

https://www.irishmirror.ie/news/irish-news/irish-baby-born-rare-life-9093239

I sent all of this through to Day, we both felt so excited.  

All of this was down to Ciara at Little Heartbeats; this was something I was not in the frame of mind to be doing at that time. She is someone who does so much for so many, yet is so often overlooked. I am truly grateful for her putting me touch with Shaye’s mum.

Later that day I received a message: “Hi Sarah I hope you see this message. It’s Shaye’s mum…”

Through a true friend who wanted to do anything she could to support me, a mum who had been through PPROM and that she had only met once, together through the power of social media I had found someone who would in some way, shape or form know how I was feeling.

And there we were, the connection was made and two mothers, two families, on the same journey were connected; no longer alone.

What a 24 hours!