This is a post I’ve debated many times. I’m not much of a sharer of personal information. You won’t find anything about this on either my Other Worlds or my personal Facebook page, because I figure “why would anyone be interested?” Also I don’t want to be ‘that guy’ who seems to be posting about health issues all the time, and most of all, I don’t want to make excuses. I want to be ‘normal’.
However. I have decided to make this post here for a couple of reasons.
1: It does affect my work. Sometimes a little, sometimes a LOT. My health varies, and that really affects my life, and I have to learn to accept that and deal with it.
2: I figure there might be other people with ongoing health problems out there who might like to read about how someone else is dealing with their own issues. I’ve found it helpful myself at times to read about how other people cope with things.
What is Cystic Fibrosis (CF)?
It’s complex, and annoying. Most Simply, it’s a genetic condition caused by two faulty genes. You get one from each parent and both need to be faulty for a person to have CF (at least this is my simplistic understanding of it, genetics not my strongest area!). This defect in these two genes causes a whole slew of health conditions which collectively are known as Cystic Fibrosis. This means you’re born with it. It can’t be contracted via any means (it’s not like AIDS for example) and it’s not like Cancer (which develops from healthy tissues). It’s a genetic disorder that you’re either born with or not.
A person with CF can experience different symptoms at different times, and not everyone with CF has the same severity of symptoms.
There’s a lot more information online. A LOT! I learned early on not to read any of it, mostly because it’s either too specific to the patient, or too general to help me much. But, if you want to know more, there’s a lot more to learn, I won’t bore you with details here.
It started with blood, lots of it. And fear.
I have what is called “Adult Diagnosed Cystic Fibrosis”. It’s more rare, but it’s also more mild than ‘standard’ CF. A person with Adult Diagnosed CF is diagnosed after the age of 18, more usually CF diagnosis occur between birth and a few years old as symptoms manifest and need treatment. Had I been smarter, braver or trusted my GP more, I would likely have been diagnosed in my late teens or early 20’s. However, it wasn’t until I was feeling extremely unwell, coughing up huge globules of blood daily and generally feeling like I was about to die that I went to see my GP, and then only reluctantly, and because he was a super GP he immediately sent me for a lung Xray that afternoon.
The next day he phoned me to tell me it wasn’t lung cancer (which was the most obvious fear we both shared but didn’t say), but he said my lungs showed anomalies that he didn’t understand, so he referred me to specialist at my local hospital. This is another thing that makes my GP awesome, because he sent me to see a specialist immediately.
That specialist sent me for tests, including a FMRI scan, blood tests and lung function tests. Over several months, lots more tests, stays in hospital and lots of medication, I was a little better, but still very ill. My lung function was 70% of normal (at 50% they start thinking about lung transplants), I was still coughing up blood fairly regularly and few of the treatments did much. I was suffering repeated lung infections and was still unwell. just before my 30th Birthday my specialist consultant at the hospital told me that the results of my FMRI was in, and that I had Bronchiectasis, which is a type of lung damage. Again my simplified explanation of this is that it’s lung damage that leads to a compromised lung immune system, which leads to further lung damage and if untreated, a painful death. After more treatment and hospital stays (and more specialists consulting and trying out meds and things) my new consultant said that he wasn’t convinced they’d found out why I had bronchiectasis. It’s typically the result of some form of damage, it’s linked to inhaling damaging particles, or scarring from pneumonia (which I had had as a child, but my specialist explained how this damage he saw in the FMRI didn’t match what he would expect from childhood pneumonia scarring). So he sent me for some more tests, including HIV (or more specifically AIDS) and Cystic Fibrosis, both of which could be the root cause of the lung damage I have.
CF tests are for children!
The HIV tests were a simple blood test, of which I’d had a LOT over the past year so that was no issue. The CF test though is what’s called a ‘sweat test’. Someone with CF excretes more salt in their sweat, which is actually measurable! So a cheap and quick CF test is to use a small clinical device that’s strapped to your arm and passes a small electrical current through a patch of skin (you can’t feel it at all). This makes that bit of skin sweat, which the device collects. That sample is sent for a test and very rapidly you can get an indication as to whether someone may have CF. The amusing thing is that this test is usually given to young children, so the nurse who administered it on my was really shocked when I walked in. We joked about how it was easier on an adult since I didn’t fidget as much, but we had a few minutes of consternation because the arm bands that hold the device are made for children, she ended up having to MacGyver two bands together to make it fit round my arm.
Diagnosis at 31. Cystic Fibrosis confirmed.
All this took around a year. Just before my 31st Birthday I got a letter saying the sweat test had come back positive and I needed to go for a genetic test. This is a simpler process (for me), just a blood test. That’s then sent away for genetic analysis, they specifically look for the two CF genes.
I got a letter not long after saying the genetic test was positive, and along with that my referral letter to the Adult Cystic Fibrosis ward at Birmingham Heartlands Hospital.
First visit to Heartlands, and my first experience of being a CF patient.
So. I was terrified, honestly. I had this big thing called Cystic Fibrosis, it was so big that there’s literally two specialist wards at Heartlands (and other large hospitals around the country). I knew very little about CF and had made the mistake of googling it after I got the letter about the sweat test. Generally doing this about health issues is counter productive and pretty scary (didn’t know that ’till later though).
Firstly, I should mention that with CF there is a BIG risk of what’s called cross-infection, that means someone with CF can very easily infect another person with CF with bacteria that will cause chest infections just by being in the same room, the additional danger of this is that Pseudomonas, a very nasty bug that is antibiotic resistant and causes severe illness in people with CF is pretty easily spread, and if a CF patient develops an antibiotic resistant strain, then inadvertently passes that strain to another patient, it literally cuts their lifespan considerably shorter. This is very dangerous when you have people with differing severities of lung conditions going to the same hospital ward. To combat this CF wards have private waiting rooms, when I go I get given a room by the reception staff where I wait and all the CF specialists come to that room. The same with staying in hospital on the ward, I get issued a private room and they are very careful not to let patients mingle.
So, my first visit. I sat in the waiting room and honestly I can’t remember what order things happened in. I was seen by a specialist CF nurse, a CF dietician, a CF physiotherapist, a Social Worker and a specialist CF Doctor. They each explained to me a little about the specific area they dealt with, what I could expect moving forward, and I was asked some initial questions on my health. I was also given a fantastic book about Adult Diagnosed CF, which I later read cover to cover several times, I was given information sheets covering specific areas of health and a folder to keep it all in, I was given a list of contact numbers for each specialist, and most important, I was made to feel comfortable and like I was in exactly the right place being seen by people who knew exactly what they were doing. I’m actually getting teared up thinking about it, because I was pretty terrified, (and honestly still am a lot of the time), but I couldn’t have asked for better treatment.
My most significant meeting that day (I think I was there for about 4 hours or so) was with Doctor Whitehouse, who was (and is) brilliant. She’s hyper efficient, reassuring and very patient, which is really important to me because I hate going to the doctors, I hate talking about myself, I hate talking about my health and given the choice I generally say “I’m fine”, even when I’m really not.
She asked a lot about my medical history, which as it turns out, is littered with indicators of Cystic Fibrosis. I’d always been what a lot of people call a ‘sickly kid’. I’m 6’5″ (almost 2m tall), but despite my height (which is often seen as a sign of robust health) I’ve spent a lot of time unwell through my childhood, teens and 20’s.
The thing with CF, which I learned at that first meeting and subsequently as more of my health history became relevant, is that when it’s fairly mild, individual symptoms of it essentially look like a range of other illnesses. It’s only now, having the CF diagnosis and knowing what the typical symptoms are, that we’ve been able to look back and see the trail of ill-health left by CF.
Not going to bore you with every detail, so here are some highlights, and it’s still long:
As a young kid I had frequent chest infections. Bronchitis, sniffles, lots of colds and flu. Basically if it was going round, I caught it. Not entirely uncommon for a kid. However, I even had colds in summer (which many people believed I was making up, because “you can’t get colds in summer”). I had a runny nose so often that an aunt (rather unkindly) called me Tom Smiff (a play on my last name Smith), a nickname that fortunately didn’t stick outside that branch of my cousins. I had the aforementioned pneumonia when I was around 6 or 7, I was extremely ill for a couple of weeks, including a hospital visit for a week that I don’t remember, I was delerious for most of it. I have a fairly vivid memory of a doctor looming over me towards the end of illness and saying “Don’t do that to us again, you scared us there!”.
Now, we were able to look back and see that these were likely indications of how CF weakens the immune system of the lungs, and therefore leaves me more susceptible to chest infections. Though obviously it would have taken a medical maverick to leap to that conclusion in the 80’s just from a kid having a slightly above average susceptibility to chest infections.
In my pre-and early teens I still suffered from frequent colds and ‘bugs’, but I’d gotten so used to them that I pretty much ignored them, but at this stage I developed crippling abdominal pains that would literally leave me crunched up in an agonising ball and in some cases screaming. these would come and go, varying in severity, sometimes lasting all day, sometimes several days in a row. I got so ill with these that my mum took me to my GP many times (this is my childhood GP, the reason I stopped going to GP’s even when I later moved to a new one as an adult). He decided I was making it up to get out of school, though I missed so much school from illness anyway that I really didn’t need to make it up, at that age I literally didn’t understand why anyone would pretend to be ill, I already hated it. Eventually, and after some stern words from my mum over several visits my GP started taking my symptoms more seriously, and ultimately came up with the idea of an allergy (after ruling out Appendicitis). Ironically, he was sort of right, since we ended up cutting out milk, which was pretty much the only part of my diet that contained a lot of fat (this is significant later). My mum was a vegetarian, and although she didn’t impose that on me, and I ate meat, I was never into fatty meat at all.
Now, we were able to look at these stomach problems (and after clinical tests that involved samples I won’t describe!) and see that I’m Pancreatic Insufficient. Essentially meaning that CF has caused my pancreas to produce far fewer digestive enzymes that normal, this makes it extremely difficult to digest fat in foods (and the associated foods). This leads to all sorts of extremely uncomfortable blockages and issues with bowels and digestion. In addition, CF causes even further issues with bowels and digestion. Often one of the first indicators of CF in infants is an impacted bowel. While I didn’t have anything like that, I was suffering the early stages of CF related bowel problems. I found it really interesting that this massively influenced my diet as I grew up, I naturally started avoiding foods that contained a lot of fat and by the time of my diagnosis I was severely malnourished (when my vitamin results came back the doctor said “I don’t really know how you managed to walk in here, I’ve never seen levels this low”). I now take Creon, which are pancreatic supplements every times I eat anything that contains fat, I have to do maths every meal to take the right amount.
In my teens I started to struggle a lot with physical activity, sports were hard (previously had little problems with that) and my GP called it Asthma and gave me an inhaler, which did help some.
In my late teens I had something of a very unexpected breakdown. I almost collapsed a few times at work, and they sent me home and to the doctors. My GP (still the crappy one from before), called it Glandular Fever and gave me a sick note. After a couple of weeks I was worse. I was very weak, almost instantly fatigued even after a good sleep, I felt awful and generally like I was on the verge of a bad flu, but it never quite developed. Generally just very weak and dizzy, short of breath, that sort of thing. My GP called it ME, and sent me to a specialist at the hospital, who said there was nothing I could do but wait for it to pass.
I quit my job (because I felt guilt about them paying me on the sick for what was turning into months) and fortunately I was still living at home so it wasn’t so bad, I had no energy to do anything anyway. I spent about 8 months feeling pretty bad, and then I started feeling better, slowly, and finally I was OK again.
Looking back now, I feel this was my body suffering from years of CF illness that wasn’t being properly treated (because no-one could possible have known then). I think basically the 8 months or so of doing very little essentially gave my body a rest and time to recuperate, I wasn’t continually ignoring illness, I was literally convalescing.
In my early 20’s I was doing better, though I had started to develop a chronic cough. I was about 20 or 21 when I first started to cough up blood, although it was fairly infrequent and I ignored it as ‘one off’ occurrences. I did continue to get my little illnesses, to the stage where my close friends were getting exasperated with me constantly staying at home instead of going out partying. This is the time when my headaches started too. At first they were infrequent, but as my 20’s went on both my headaches and my cough got worse.
By my mid 20’s I was underweight, had a constant bad cough, chest pains, pains in my knees and ankles, frequent stomach pains and discomfort, my skin was pretty bad, spots all over the place and I had huge spots on my face that welled up for days under the skin before erupting. I was barely sleeping because I hurt so much and I was finding everything difficult, work, life, everything was a continual struggle. I had a headache literally every single day that ranged from annoying to debilitating. However, I didn’t actually know any of this because I’d gotten used to it all, it all happened so slowly that I literally didn’t realise that other people didn’t feel the same. When I did complain about a headache, or various pains, my friends agreed, they too felt such things, because everyone does. What I didn’t realise was that they didn’t know I meant continually every day, and I didn’t realise they didn’t mean that.
I used to marvel at my friends who could go to work all day, then go out in the evening, or do some activity, play sports, then at the weekend go do more stuff. By this point my life was literally a blur of just trying to make it through the next day. The weekend was spent trying to pull myself together for next week. I was struggling socially, making very poor life choices because I just was at the raggedy edge. I felt like I was failing at life, and I literally had no idea what I was doing wrong, I just couldn’t keep up.
And then, things changed.
The first time I went on powerful antibiotics in conjunction with various other meds to treat other symptoms I actually cried when the pain started to go away and I realised that it shouldn’t hurt to move – CF related infections can cause swelling in soft tissues, which is a type of arthritis, which is why I hurt so much. When my cough eventually started to go I was stunned by how much energy it expends to cough all day, and how much more I had now. When the dietitians worked with me to correct my malnutrition and get me back to a more healthy weight I was shocked that I could do more, I felt better. I actually felt like I could do the same things as my friends, I suddenly realised that they weren’t super-people able to do these astounding things that I couldn’t – I had been assuming for years that everyone felt as I did, and yet they were able to get on with life and somehow I couldn’t. Turns out they were just doing normal things, and over the course of two years of treatments and lots and lots of medication, I started to be able to lead a more normal life, and do those same normal things (to some degree), but more importantly understand why I struggled at times.
And here we are, in 2016.
Now, in 2016, I’m sitting here writing this with mild chest pains that I’ve had since I woke up, my fingers hurt from mild arthritis caused my soft tissue swelling (another sign that I’ve got an infection again) and I’ve just realised I’ve not eaten for over 15 hours (loss of appetite is another sign of a chest infection for me), I weight myself yesterday and I’ve lost 6kg in the last couple of months without realising it, which is not good. I’ve spent the last three hours doing the first part of my daily medication routine, and I’ll have another hour to do soon, then tonight another couple of hours, a total of 4-5 hours every day to just stay well, when I get ill that increases. I take over 30 tablets every day, I do chest physio to clear my lungs of bad stuff (which is pretty gross) and I have to use a nebuliser 6 times a day just to stay relatively well. I’ve been on continual antibiotics of over a year to keep the infections in my lungs contained.
I am frequently ill. Mostly it’s a low level that just needs a day or so rest to recover from, but I consider myself “really well” if I go for a week without some sort of feeling unwell.
I have spent the last three months with varying degrees of cold/flu symptoms, which have fortunately not manifested into a full blown chest infection yet (thanks to the two different antibiotics I’m on!), but I’ve still felt pretty crappy for the most part, and I seem to be getting worse. Fortunately I have my next clinic appointment in two weeks, so I can hold on ’till then and see what the Doctors say!
I go to the hospital ever 2-3 months for checkups, the great team of specialists continually monitor my health and tweak my meds and last year I didn’t have to stay in hospital at all (firs time since I was diagnosed I’ve done a whole year just as an outpatient).
However, although that sounds pretty bad when it’s all written down in one place, overall things are much improved over where I was a few years ago. I feel generally positive and I enjoy life a LOT more now, though often I feel frustrated because my brain works fine and it’s just my body that lets me down, and I feel annoyed at the inconsistency of my illness. I don’t know how I’ll be next week, I may be so ill I need to be hospitalised, I may be totally fine and indistinguishable from someone without CF unless you see me doing meds, I may be slightly unwell but generally able to carry on, who knows! And that’s actually the hardest thing.
The average life expectancy for someone with CF in the UK is 41 years. For someone who is Adult Diagnosed, that’s usually longer, however CF gets worse as you get older, so there’s a trade-off and barring some medical breakthrough, my health will decline, I will ultimately need oxygen support and without a lung transplant, I’ll die. With a lung transplant I’ll get a new set of meds and medical issues, but a few more years life. I’m currently 34, I honestly don’t know what I’ll be like next year, or even in six months.
So there you have it. I have Cystic Fibrosis, and now you know a little more about me.
Thanks for reading 🙂