Written by Sally and Haley Welch, volunteer
“If I was paid a dollar for every time I was told that ‘no one has long term severe neutropenia,’ I’d be a rich woman.”
Sally, a woman in her 40s from London, has dealt with neutropenia her entire life – but for more than 20 of those years, her condition has been a mystery to her. The constant severe infections and attempt to justify them with various aspects of her lifestyle left her feeling estranged not only in her own body, but from the confusion of peers and medical professionals alike.
If you haven’t heard of neutropenia, you’re not alone – it’s an extremely rare illness that affects fewer than 1-2 per million people worldwide. Being diagnosed with neutropenia means that the number of neutrophils a person’s body has circulating through the blood is significantly lower than that of a healthy individual. Not having enough neutrophils means that a person is much more prone to infection than the average individual – and when a person with neutropenia does get an infection, the symptoms are far more severe than what healthy individuals experience.
The turning point that helped reveal Sally’s condition to her came in the form of a bug bite. The bite appeared one day on the back of her leg, and it just wouldn’t go away. She recalls, “In fact, it just kept getting bigger and angrier all the time. Within hours of noticing it, I went to bed, woke up and had a thigh the size of a watermelon! A hot, red, heavy, pulsing thigh that was so swollen I couldn’t bend my knee – most unattractive when you’re 23!”
This wasn’t the end of the incident. After going back and forth to the A&E in London, she suffered a ferocious temperature, nausea, and fatigue. Sepsis reared its ugly head, and she had to be taken to a bed in isolation. This triggered an 8-month bout of going in and out of the hospital. “My neutrophils were extremely low, but this was simply noted as ‘one of my symptoms’ and not really taken into account, as ‘no one is severe neutropenic,’” Sally explains.
Sally was finally properly diagnosed when she was referred to a hematologist at another hospital in London. It was a relief to finally feel understood. She says, “The day I found out what I had, I cried – for a very long time. My Mum cried for a very long time too. Living with something invisible and yet which impacts your life in so many negative ways is entirely rotten.”
Though getting the correct diagnosis lifted a huge burden in her life, there are still many obstacles Sally faces. Because neutropenia is so rare, it’s often misunderstood and stigmatized. Sally is no stranger to this. It’s difficult when she finds herself having to explain her condition to others. She shares, “It is so easy for people to either look heavenwards like you’re fabricating some mythical illness that just means you want to work from home or have a duvet day, OR they talk to you like an idiot and think you can’t possibly have a life and survive. It’s a difficult one, as I’m one stubborn human – I kind of like that side of my personality, but it also means that often inwardly I’m screaming because I hate having an illness I have no control over, and when it strikes, it can render me very fragile indeed.”
Fortunately, Sally’s workplace is incredibly understanding of her condition. But this isn’t the case for all people with neutropenia – and Sally, alongside the National Neutropenia Network, aims to change this. It’s hard to raise awareness for a disease that is so rare, but that only gives a stronger incentive to those who live with it.
Treatment for a disease of this nature is difficult to cater to the individual, but it is possible. Sally gets periodic Neupogen G CSF injections that allow her to take control of her neutropenia. Finding the sweet spot for injection frequency took years to calibrate, but Sally was finally able to find what worked best for her.
The injections solve a mighty part of her struggles, but Sally must still fight the disease with humility and courage. She shares, “There are times I have to sleep my entire weekend away or just hit a wall physically and fall into an utterly imperfect mess of a human. I allow myself these times, but this has been hard to swallow when I have to let people down last minute because I just don’t have the oomph to be sociable or put on a front. I have worked full time in a stressful and intense job for the entire time since my diagnosis – I will not let this win and I will not give in!” She has also follows a healthy diet loaded with veggies and fish.
Today, Sally manages her neutropenia well. She exclaims that she has officially achieved “Neutropenia Rockstar status!” She does note that the side effects of the medicines she takes have intensified as she gets older. “I can sometimes only manage hot tears on a particularly bad night post-injection. I take them on a Friday, so as to limit any interruptions to my working week, and, on occasion, I get fierce flu-like symptoms – a spike in my body heat that’s uncontrollable, my body stiffens and I can’t fully extend my limbs, I feel like hot knitting needles are being spiked into my long bones and sacrum, and I shake uncontrollably.” Sally’s husband is aware of the effect these episodes have on her, and he knows how to help her as best as he can. He gathers plenty of blankets and speaks calming words to ease her pain. When she can’t sleep after an injection, Sally will listen to Michael Sealey’s sleep hypnosis, which helps her shift the focus from the pain her body is in to the soothing voice in her earbuds.
“I tried for many years to ‘be normal’ as I didn’t want any judgement for having a chronic condition – especially one that is so utterly unknown and rare. I now tell those who need to know, and I also share a link to the National Neutropenia Network website so they can educate themselves. It’s important to spread the word, as we may be a small minority, but this is an illness which is long term and needs careful management and understanding.” Sally credits her Mum for being her biggest supporter from the start. She has never failed to be by Sally’s side and know exactly what to do at every step throughout her journey. She is incredibly grateful to her employers for being more than understanding when she needs to complete her workday from home.
Sally readily shares how neutropenia has changed her life. “You have to adapt – it’s not a choice. But adapt is the word – not give up or give in. This is a long-term sentence and it is incredibly daunting, but it is manageable, and with the help of us all, it will become more known, understood, and supported. I have found inner strength that I never knew I possessed. I have become less afraid to simply say ‘no’ if I can’t muster the energy. I am less fearful of offending or upsetting friends. If they are friends, they will stay by your side and understand, and hey, maybe even turn up at yours instead with a bottle of wine and their pajamas! Don’t hide the fact you live with a chronic condition, but also don’t let it define you.”
Sally’s resilience and transparency not only make her a Neutropenia Rockstar, but a necessary catalyst for the visibility that neutropenia needs.