My HFI Story: Carol Westwood

The following is a summary of my experience of having HFI. I have mentioned some specific events that I recall well, but there are countless others that I have not mentioned. 

My mum was apparently the first to recognise that, as a baby, something was making me vomit, have diarrhoea, and scream with “colic”. I was bottle fed with Cow and Gate and it was usual to add sugar to the bottles in those days. My mum found that if she left the sugar out of the feed I was not sick or uncomfortable. Apparently I once screamed with pain after being fed a can of Heinz Spinach baby food. I’d love to know what was in it! 

I developed an aversion to sweet tasting foods from a young age. I was brought up being able to eat what I wanted and was never forced to eat those things that I refused. I don’t remember eating sweet things specifically when I was young, but I must’ve tried sugary foods sometimes, as I remember feeling sick and vomiting every Christmas and birthday without fail. I remember always trying things that others were eating, like cake, ice cream, jelly, soft fruits etc., but never actually eating much of it. Even the limited amounts of sweet foods that I tried resulted in feeling or actually being sick and just very tired and unwell. My primary school cooks were very understanding. I recall one pancake day, they made me my own sugar free pancakes.  I was never made to eat the desserts. In secondary school I took a packed lunch. 

I recall being at my aunt’s house and playing with her grandchildren. I must’ve been about 3 or 4 years old. She bought us all a Milky Bar each. I remember not knowing what to do with it, as I had never been given chocolate (although it’s likely that I had been offered but refused it). I watched the boys rip into the wrapping and shovel it down. I copied them. I don’t remember feeling sick, but I do remember my aunt running over with a washing up bowl for me to vomit into. 

Throughout school years, growing up, I was often feeling sick and having diarrhoea. I remember one of the first words I asked the teacher how to spell was diarrhoea! I remember feeling sick in the mornings before school on a fairly regular basis. I’d have to go and lie down, but after a couple of hours I’d start to feel better. I think mum used to think I was faking it to stay off school! I wasn’t. I loved school. With hindsight I remember having cornflakes for breakfast and although sugar might've been sprinkled on them, it was more likely just the sugar in the cornflakes themselves.  

I recall being given a polio booster in school, on the obligatory sugar lump.  I held it in my mouth, then went straight to the toilet to spit it out. 

Feeling nauseous, and needing to have a lie down was fairly ‘normal’. On a couple of occasions  as a child, I remember having diarrhoea and being taken to see the doctor. I hated this as I knew he’d give me horrible tasting medicine which would make me sick. With hindsight again, of course it did! He’d prescribe kaolin and morphine - the usual treatment for diarrhoea at the time. It was sweet and vile, and made me vomit, thereby reinforcing that I had D&V or a "tummy upset". 

Eating at friend’s houses was always tricky. I’d try things that I was given, to be polite, but would only eat a little, and would often feel poorly. 

My brother was convinced that it was a psychological thing. He once said “If you were locked in a room with nothing but bags of sugar, you’d eat it). He did apologise for his actions and comments years later when he and I knew about HFI. On a day trip out when I was about 8 or 9 he bought peaches for everyone. I really wanted one, like everyone else. They smelt so lovely! He said he’d buy me one but if he did I must eat it all. I remember nibbling away at it and feeling sick. He commented that I was taking a long time to eat it, so I said “I’m enjoying it and I want to make it last”…. it wasn’t long before he had to stop the car for me to vomit in the hedge. 

At the age of about 9, I went to a pub with a friend and her parents. They bought me a glass of Coke. I had to stay in the car with my friend, so I drank it in the car (I hated the taste but thought I should drink it to be polite). Before long I vomited in the car park. 

I helped out at a youth club market stall at around age 14. The adult running the stall said "I've bought these lovely crisp Granny Smith apples. I felt I should eat one, to be polite. I didn’t finish it, but was really sick when I got home. Physically vomiting, shaking and having to go to bed. The nausea and vomiting were always accompanied by needing to lie down. If I was at home I would go to bed. I had no idea that this was probably hypoglycaemia.

Had a school trip to Longleat House and Safari Park (aged 13 or 14). In the coach on the way, I ate yellow and green Rowntrees fruit gums. They didn't taste quite as sweet as the other flavours. Felt dizzy and nauseous on a tour of the house, and had to be led down to a quiet area by the guide. I vomited before getting back on the coach for the journey home. On the way home, I sat next to a teacher, who tried to help me by giving me barley sugars, thinking it was travel sickness! 

On a youth club visit to London around 14-15 years old, I stayed with a family in Hounslow. We were given tomato soup for our meal. I ate most of it to be polite.  I then felt nauseous and had diarrhoea during the night. 

At the age of 15 or 16, I was admitted to hospital with acute central epigastric pain. I was working weekends in a holiday camp shop. My boss had given me a glass of Martini as a 'treat' when he and his wife had one. I hated the taste, but again felt I had to drink it to be polite.  I can't recall what I had eaten, but it may well have contained some sugar. In hospital, I remember feeling desperately tired but being made to get out of bed when all I wanted to do was sleep. I do not recall having blood sugar tested at any stage. A provisional diagnosis of gallstones was made.

At around 17 years old, I went to a cousin’s wedding. Had a single glass of champagne for the toasts. Didn’t like it but drank it. I don't recall what I actually ate at the event, but I do remember having to stop the car to be sick on the way home.

At the age of about 17, I had worked out that the only common denominator for feeling sick was fructose. At age 18, I started nurse training and I had access to the hospital library and found information about “Fructosaemia”. This seemed to sum up what I had, although I wasn't sure. The nursing tutor wanted a volunteer to have a Barium swallow, to demonstrate to the rest of the class. I volunteered as I thought it may highlight some problem with my gut that would explain what was wrong.  I don’t remember finding anything mentioning HFI in the hospital library.  

In my teens and early 20s I was always embarrassed to say that I couldn’t eat fruit or anything sweet. Consequently I used to try being ‘polite’ and eating what I was given but generally refusing fructose containing foods. When I had a polio booster at the start of my nursing training it was offered on a sugar lump, but I asked to have it directly in my mouth. The comment from the doctor?… “There’s always one…”.

At around age 19, I stayed with a friend in Cambridge. We had a meal in the hall…some sort of beef stew. It tasted ok. Then I had nausea and vomiting and felt unwell in the night. Later I found that it had been cooked using port. There would doubtless also have been onions and probably tomato in it as well.

I made myself feel sick every year from childhood until diagnosis, without fail, by eating not quite ripe blackberries, loganberries, wild strawberries, gooseberries and raspberries. I didn’t eat loads, but enough to make myself feel sick. If only I’d had a diagnosis early! I wouldn’t have touched them!

For all of my life, I have regularly suffered with gut pain at night - I still do. I find that a hot water bottle on my stomach helps a little, and I will take one to bed with me every night of the year, even during hot summer weather. 

When I worked on the wards as a staff nurse, doing regular night duties, on one occasion I'd eaten a big meal before work. I then had a meal of savoury mince in the canteen on my break. I didn't think about what might be in the mince, but it tasted ok. When I got back on the ward, I remember feeling really tired and woozy. I sat down on a box in the corridor. The nurse that I was working with saw me and asked if I was ok. She took my blood sugar. It was 2. I had a couple of glasses of milk (always my go-to if feeling ill). My blood sugar soon returned to normal levels. I had no idea at all at that point that ingesting fructose if you have HFI will make you hypoglycaemic!

At 25 years old, I was doing Health Visitor training. A paediatrician gave us training on rare disorders to be aware of. He described my experience completely. I spoke to him and he advised me to go to my GP, get a referral for formal diagnosis of HFI and have genetic counselling before having children. The GP was brilliant. He referred me to a gastroenterologist and genetic counselling team. The GP did bloods for liver function test and the results came back showing indicators of liver damage. I thought I was accidentally killing myself by causing damage to my liver, and not long after, I had my first episode of depression. 

Age 25. Gastroenterologist disbelieved me. Told me I didn’t have it, but had an irritable bowel and should go away and eat all those things that I thought made me ill and I’d be fine. In relation to the abnormal liver function test, he asked how much I drank.  When I told him it was maybe half a bottle or can of lager on a hot summer’s day he said “So how much do you really drink?” He told me to cancel the genetic counselling appointment as I didn’t need it. I cancelled it. I believed him about the fruit and IBS! I went out of the consultation feeling pleased! I tried eating melon!! I'd always wanted to eat fruit!! The consequence of course was nausea and gut pain, along with the associated tiredness and just feeling ill. When I went back for review and told him it was making me ill, he told me that “Fructose Intolerance is so rare, of course you don’t have it. But as you’re a nurse we’ll bring you in to do a fructose tolerance test to prove to you that you don’t have it”.

I had two admissions for fructose tolerance tests. For the first one, I had a newly qualified House officer with me. I was given an intravenous infusion of fructose. I remember feeling ill, shaky and tired, and vomiting. When I went for the results, I was told that "The doctor didn't take blood for phosphate levels so we'll need to repeat it. On the second visit, the registrar stayed with me. I was visited by the head of pharmacy, who had prepared the fructose infusion, and also visited by the head of biochemistry, who would be carrying out the blood tests. This time I didn't feel quite as ill, but I certainly didn't feel well! When I finally went back for the results, they were pushed across the desk to me with a “You’d better read it for yourself”…. ‘This patient has a moderate degree of fructose intolerance’… Followed by the gastroenterologist's comment, “You know what you can and can’t eat. Just carry on as you always have and you’ll be fine”. Not a hint of an apology or acknowledgement that I'd been right all along. 

I paid to have a genetic study with 23&me. My results showed two copies of mutations for HFI. I didn’t know which ones. I felt that at last this was some validation. 

When I was about 40 years old, I had a holiday at Centreparcs with friends. Over a couple of days I ate a combination of different foods in different restaurants, and didn’t check ingredients but relied on my taste buds to avoid sweet tastes. A combo over the couple of days of tuna baguette with mayo (with sugar), ‘healthy’ porridge (later found to have apple juice in it), jacket potato (baking potaotes often have more fructose than some other varieties- I no longer touch them) with tuna (mayo with sugar), mussels (later discovered there was coconut milk in the sauce). I was really poorly. I remember spending most of one night lying on the floor in the toilet, where it was cool, but also where I could easily be sick.  My friends wanted me to be seen by the doctor on site - I worried them, but I didn't go to the medic! Lots of vomiting, nausea, needing to lie down and sleep. With hindsight, I'd love to have known what my blood sugar level was.  

In my 40s, I had been visiting my mum, who was by then in a care home. It was about 60 miles from where I lived. On the way home, I bought a bottle of supermarket own brand Lucozade equivalent. Didn’t finish it as started feeling sick. After I got back home, I vomited a lot and felt very shaky and tired. The drink had contained Invert glucose syrup. As a child, regular Lucozade was a drink I could tolerate well and I didn’t perceive as tasting sweet. Since Lucozade have replaced some of the glucose with artificial sweetener it now tastes too sweet and I hate the taste. Before it changed ingredients, I used to keep a small bottle in the car in case I felt sick from inadvertently ingesting fructose and getting low blood sugar/feeling woozy. 

My sons each carry 1 copy of the gene. During pregnancy, my daughter in law also paid 23&me for a test just in case!! We are not at all related, however it was found that she also carries an HFI mutation and as such both she and my son were seen by the NHS genetics service. The care and understanding that they had from their local service in Wales has been wonderful. Their daughter was tested at birth and is just a carrier, however until the results were back, the paediatrician in charge of her care was really on the ball! She was knowledgable about HFI, and kept the maternity unit staff fully informed to avoid all sources of fructose until DNA results were back.  

I’m now much more careful about my diet. However 2 or 3 years ago, I tried to eat a more “healthy” diet. I had much bigger portions of what I considered ‘safe’ veg, e.g. avocado, salad veg, kale, spinach, cavolo nero, kimchi, houmous, broccoli, olives, asparagus, radishes. I was having much bigger portions than usual. I was happy that I was losing weight. I was waking feeling sick in the night every night, and every morning. I assumed I must have reflux. A fellow HFI friend suggested that I was eating far too much fructose. I ‘cleaned’ my diet to try having no more than 1gm fructose per day from vegetables. Of course, I started feeling much better! 

I still find that many people react with disbelief when I tell them that I can't eat fruit and veg, or sugar.

Now in my 60s, I have learned to keep to a safe HFI diet. No fruit, only a small amount of green leafy vegetables, and a mainstay of meat, fish, dairy products and a few lentils and pulses. It is really difficult to avoid fructose completely, especially if eating away from home. I still get stomach pain at night fairly often. My GP referred me to a specialist metabolic service in Cambridge. Finally, albeit in my 60s, I feel listened to, understood, and validated. I also feel safe, knowing that regular checks are undertaken to make sure that I am not incurring any liver, kidney or other damage to my health. 

I wear a Medic Alert bracelet and carry a Rare Disease Alert Card with me. This has contact numbers for the Specialist metabolic team in Cambridge.