My eyes were swollen, my lips cracked. I felt like I was having a heart attack

Nicky Bowen has hereditary angioedema (HAE) which can cause severe swelling

My first attack was at the age of four, although I don’t remember it. One morning, I couldn’t get my shoes on as my foot was so swollen. That’s how my parents knew that I had hereditary angioedema (HAE) – a rare disease that I’d inherited from my dad. HAE is caused by a deficiency in C1 inhibitor (C1-INH)  in the blood and it causes huge swellings anywhere in the body. My next episode was when I was seven or eight and my hand swelled up. As a child, I’d never known anything different. My dad used to say: “We just swell up now and again, but I still go to work and you still go to school.”

But he would also add: “But if your face swells up, you’ve got to go to hospital in case it cuts off your breathing.”

We don’t know too much about our family history of HAE, but joining the dots, it was probably responsible for the death of my dad’s mother five days after he was born. Family members vaguely remember that she would have funny episodes where her hands or face would swell and no one knew why. My dad was told that she died from complications after the birth related to “abdominal pain”. His own diagnosis came at 22. He’d been in and out of hospital with swellings that came and went, and one day, he saw a doctor who had been at a conference where HAE had been raised. A blood test confirmed it.

Nicky’s parents found out she had inherited her dad’s HAE when she was four

The attacks got much worse when I hit puberty as it can be linked to hormones. At that point the swellings were in my stomach, so every month I would suddenly look six months pregnant. I would throw up for 24 hours, literally every half an hour. The pain is crippling – having now given birth, it’s like full on contractions for a whole day. The next day, I would be worn out and drained. On day three, I would be feeling a bit better and by day four, I would be back at school.

I was 18 when I had my first facial swelling. I’d been on a night out, woke up at a friend’s and realised I couldn’t feel the inside of my cheek with my tongue. I looked in the mirror and saw that my mouth was swollen. I called my mum in a panic, petrified it would reach my airways and she took me to hospital. However, on that day, the doctor decided that I might be having an allergic reaction to something I’d eaten and gave me hydrocortisone which made the swelling 10 times bigger.

My whole face was enormous. My eyes were swollen, my lips cracked. I looked like the elephant man and felt like I was having a heart attack. In the end I was given a blood transfusion which was the only treatment at the time and stayed in hospital for four days. It’s such an unknown disorder that another time I went to my GP with a swollen face only to be told that I must have squeezed a spot, and he didn’t want to see me again that day. I ended up in A&E again.

Nicky: ‘I went to my GP with a swollen face only to be told that I must have squeezed a spot, and he didn’t want to see me again that day’

By my mid-20s, I would have attack, after attack, after attack. I might wake up with hands so swollen, I couldn’t move my fingers. They would be set in whatever position they’d been in when I’d gone to sleep, as if in plaster. For a day, you can’t hold a knife or fork or pen, you can’t drive. When my children were young, if I had an attack, I wouldn’t be able to change a nappy. The attacks in my feet would make them double in size. All I could wear were my husband’s crocs. Walking on them could be so uncomfortable if they’d swollen into an odd position – sometimes my toes would be pointing towards the ceiling. I would have to stay in and crawl from A to B.

That period was really my rock bottom. I remember being told by one specialist that stress, worry, hormones or happiness could trigger it – it’s not an allergy but basically anything can spark an attack. It was unpredictable and so overwhelming. You feel you have no control over your health. There were so many things I stopped doing to make sure I didn’t swell up. I wouldn’t ride a bike in case the saddle made me swell in a very uncomfortable place. I wouldn’t go to the gym. I wore flat shoes. I was always anticipating the next attack and stopped wanting to go out at all.

Nicky’s hands and feet can swell up to almost double the size

At 25, I’d got a civilian job with the police-force but it was becoming so hard to manage. I remember lying on the sofa around that time in tears and realising I didn’t have a life. My self-esteem was low, my body image was low. To this day, I don’t know what I did to pick myself up, but I do know that I gave myself a shake, and a good talking to. I began to realise that I had to find ways to live with it. Things really changed when I started forcing myself to get up and leave the house and do a lot of walking.

But the real life-changer was when I reached my late 30s and was given medication to raise my C1-INH levels that I could store at home and trained in how to administer it myself if I had an attack. Now if I medicate as soon as an attack starts, it goes away and I can carry on my day. If you treat an attack, it doesn’t build up and come back somewhere else a few days later. I haven’t had a bad attack for 10 years.

Nicky’s daughter Rhiannon who was diagnosed with HAE at four

I’ve been with my husband for 27 years – we worked together first so he has always known about my HAE. When our daughter Rhiannon was four, she was given a blood test and we learnt that she had it too. I remember being so upset. I’d desperately wanted a child. Maybe it was selfish when there was a 50 per cent chance that she would get it.

When I realised I had passed it on I felt devastated. Knowing my daughter had it was much worse than having it myself. Questions filled my head. How will I prepare her? I might not be there when she had her first attack. I might be at work, she could be at school. How could I show her that when it happened, she would be okay?

Nicky: ‘[Rhannon] is 21 now, she’s been to uni, she’s a primary school teacher and HAE hasn’t stopped her from doing anything’

Rhiannon was 10 when she had her first attack – and I wasn’t there. She was at school, I was asked to pick her up as she was vomiting so much and we did end up in hospital. She is 21 now, she’s been to uni, she’s a primary school teacher and HAE hasn’t stopped her from doing anything. She says that she’s a “puffer fish”. One minute, she’s nice and flat, swimming around, then suddenly, she’ll blow up and puff out. Then 24 hours later, she’ll be flat again. Mentally and physically, she manages HAE so well. You can’t let the illness control you. Getting up and keeping on is the only way.

As told to Anna Moore

Visit the HAE website for more information.

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