5th July 1998
I have a diagnosis! Finally, after years of illness and tests I know what’s wrong with me.
I found out today when I went to my outpatient appointment with Dr. Jenkins. He asked me to pull my chair closer to his desk so I could see my medical notes. There was a chart with all my blood results from the last few months. They seemed to have gone up and down in a wave-like pattern.
“You see this?” he said pointing at the chart. “This is a very rare sight, something that we would only see once in every million people. That’s why it’s taken a while to put the pieces of the jigsaw together. What we have here is called idiopathic cyclic neutropenia and yours is a truly classic case”
I felt like I should ask a lot of questions but I was too shocked. But then all kinds of questions started to run through my mind as he shut my folder and suddenly looked very serious.
“There is no known cure, I’m sorry to have to say. It’s a lifelong condition. All we can do is treat you with an injection called GCSF and try to control any infections you may pick up”
“What is this condition, and how did I get it?” I finally managed to ask.
“Cyclic neutropenia refers to your neutrophil count going up and down in a three week pattern. Neutrophils are the part of the blood responsible for fighting bacterial infection. When your counts are at the bottom of the cycle, at the end of the three weeks, this puts you at risk of potentially fatal infections. Idiopathic means no known cause”
“But it can’t have come out of nowhere”
“In your case, it has”
He told me I would be under the care of the hematology department for life. I could come to the hospital to get the GCSF injections or I could learn to do them at home.
I can’t take it all in. On one hand I am relieved that there is finally an explanation for why I’ve been so sick for such a long time. On the other hand, I now have a rare blood disorder. Today I feel fairly well, but this won’t last. The awful pattern of illness isn’t going away. I’m going to be ill again, again and again, too many times to even imagine or process. I’m one in a million. I feel so overwhelmed and so very alone.
“Did you say I could get a fatal infection?”
Dr. Jenkins grimaced. “Yes, I did. Your neutrophil count drops so low that if you got an infection at that time it could kill you within 48 hours”
I was too shocked to respond.
“But the GCSF should help to bring up your counts”
I wonder where the other “one in a millions” are. I asked if there were any other people with cyclical neutropenia I could talk to. Dr Jenkins said he didn’t know anyone else with this condition and he wouldn’t be able to tell me anyway due to patient confidentiality.
There was only one thing I could do. I called my Mum.
“You were one in a million before you got this illness,” she said and I laughed.
6th July 1998
Today I spent a good hour doing what I thought was fully comprehensive research and was shocked that there didn’t seem to be any support groups whatsoever in the UK. If I am one in a million, surely there must be about 55-60 other people with cyclical neutropenia somewhere? Where would I find them? I suddenly began to feel very isolated. Out of interest I typed some more common illnesses such as leukemia, AIDS and hepatitis into Google and all kinds of websites and support groups immediately popped up.
As my initial search for cyclical neutropenia had produced zero hits, I tried typing in “rare illnesses of the immune system” and one of the search results was systemic lupus erythematosus. I clicked on it and the home page of the UK lupus support website came up featuring pictures of lots of people smiling happily together, even though they had a rare disease. I wanted to smile happily with other people too, even though I had a rare disease. Why couldn’t I have got lupus? It’s still mysterious sounding and unusual and at least I’d have a bit of company. I kept going and surfed the net for another half an hour. Then I found it. There was one neutropenia support group, in Manitoba. The other side of the world!
It seems that this ‘one in a million’ status is a double edged sword. There is a small part of me which is glad to be original in my contracting of a disease. The last thing I would want is to be is boring and it’s kind of cool to be unique and different. However, there is a much bigger part of me that is terrified about having no-one to talk to who truly understands what I’m going through. It seems I’m now adrift in an ocean that stretches across to Manitoba. I’m aware of this region from a geography course years ago. I can remember that it is in the ‘near north’ of Canada and that an animal called caribou roam around there.
Years ago I became very drawn into the plight of the caribou after it was revealed they were too scared to go under the Alaskan pipeline; and this was then affecting their mating rituals. We watched a slideshow about them and they look a lot like deer. When I left the course I felt strongly that I should look into animal rights issues in the region and then promptly forgot all about it. Now the only support group for neutropenia in the world is housed there and I need to draw on its resources after being so laissez faire about its indigenous creatures.
After I got home, I composed a letter to the cyclic neutropenia support group as there was no email address. I asked for any information they have and also if they could put me in touch with other sufferers.
8th July 1998
I am ill yet again with an awful throat infection and mouth ulcers. This time feels different though, because now I know why and there is an edge, a sense that I want to try and do whatever I can to turn my life around. I cannot cope with the thought that I have to be ill, tired, depressed and lonely, or all of the above for the rest of my life. I’m only a few days into this lifelong illness and I am bored with it already. I can’t see how it is possible for me to live with it forever, when it is so limiting.
11th July 1998
I haven’t been out of the flat for 2 days as this throat infection is taking a while to improve. But, I have had what I think is a brilliant idea, after a very long session of heavy thinking today. The hospital advised me to take the GCSF injections “when I get ill,” this approximates to about every three weeks and coincides with my neutrophils hitting the bottom of the cycle. Then it takes me however long to build up my immunity again and fight off the infection. By this time, the neutrophils then begin their predictable descent once more. My neutrophils are only going up and down within a very small range and are never high enough to fully protect me from getting an infection in the first place. GCSF may well stop me from dying when I hit the bottom of the three week cycle, but I think there may be a better way to take these injections. What if I had them every two weeks, and intercepted the cycle on its way down? Thus, they would act as a prophylaxis and boost my neutrophil count before it dropped very low. I am very pleased and excited by this idea. I can’t wait to ask Dr. Jenkins about it !
9th August 1998
Dr. Jenkins looked at me as though I was a genius when I explained my idea. He sees no problem and thinks it is well worth a try. He told me that there were no real case studies of how to treat my condition successfully, so any ideas I had were fine. While we were talking I had another brain wave. I asked if there were any different dosages of GCSF and there are. I am on the lowest dose, thirty million units. There is a higher one of forty eight million units.
“Why am I on the lowest dose?” I asked, incredulous.
“We give people the lowest dose they can manage on” he answered.
“But my life is awful on the lowest dose”, I said. “It might keep me alive but it doesn’t give me any quality of life!
“Try the higher dose, then” he said.
Up until I was diagnosed with this illness, I thought doctors had all the answers. How wrong I was!
19th May 2016
Eighteen years later and I am still living life as best as I can with cyclical neutropenia and after many further self-directed experiments, I’m now on another brand of injection that I only have to take once a week. It took six months to hear back from the support group in Manitoba, who were able to provide a monthly newsletter but no details about other members.
I had no choice but to manage the bulk of the physical, emotional and practical side of this illness myself and it impacted every area of my life for a very long time. But going it alone taught me resilience, gave me strength and afforded me huge empathy for others suffering with chronic health issues. Life turns on its head when you are diagnosed with a long-term illness and so do your beliefs about who and what will help you get through it.
I am also hugely thankful that the internet has developed in the way it has. This has been the single biggest factor of positive change in being able to manage my condition. It’s opened a gateway of information and access to other sufferers and online support groups.