A MUM-OF-ONE has told of her rare condition that caused her nose to collapse and left her needing glass tubes inserted into her eyes.
Amanda Kenvyn, 33, has granulomatosis with polyangiitis (GPA), which causes inflammation of the blood vessels in your nose, sinuses, lungs, throat and kidneys.
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She started showing symptoms when she was heavily pregnant and still had them after she gave birth, but doctors told her it was normal and she would feel better.
Eventually, Amanda, from Lincolnshire, was diagnosed with GPA and since been forced to have glass tubes in her eyes to try and stop infections.
She also has surgery on her windpipe every six months, which she “hates” but knows she has to have it as “there’s nothing else that can be done.”
Amanda said: “I’ve had to have Lester Jones glass tubes inserted into both of my eyes to try and stop infections.
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“My windpipe is more narrow, and I’m having to have surgery every six months to re-open it.
“I know when I need it because I get really breathless, and I can’t sing a song, and I get really breathless.
“I hate it, but nothing else can be done for me.”
FIRST SYMPTOMS EMERGE
Amanda started with symptoms, including headaches, joint pain and a large abscess on her breast, when she was pregnant with her son Toby, who is now two.
She was reassured by doctors her symptoms would disappear after she gave birth, but she began suffering with more headaches and sinus pain after he was born.
The admin assistant was then unable to lift Toby out of his cot because she lost sensation in her hands, which is when she was diagnosed with the condition.
My physical appearance has changed so much; I’ve never been thin, but from the chemotherapy and steroids, I’ve got a new face, and my nose looks strange
Amanda Kenvyn
Amanda said: “I kept going back to the doctors, and they kept saying it was postpartum and that it would go away.
“I was going back every week because strange things kept happening – I had sinus pain and headaches, and I couldn’t lift my baby out of his cot.
“I lost the sensation in my legs a couple of times, so my husband had to rush me to hospital.
“I had a lovely doctor who said she didn’t know what was wrong with me and asked if she could reach out to other people.
“My bloods were coming back normal apart from my infection markers.
“Then one day, my friend told me to look at my nose, and it had completely collapsed – so I went back to the doctor, and she told me it could be this disease.”
It is believed that only around one in 100,000 people in the UK are diagnosed with GPA.
Amanda says she was “totally confused” when she was told she had it because it’s so rare; she had never heard of it.
“The first thought I had was, ‘Why me, and what have I done to deserve this?’ because I thought I was going to die,” she explained.
She added: “I had lots of questions, and I did a lot of googling – but it’s taken a lot of strain on my mental health, and I’m not the person I was before.
“I used to be a fun, bubbly and outgoing person, but now I’m finding it hard to come to terms with it.
“I’m worried that it will attack me even more, and I’ll die – because what would happen to my son?”
A NEW FACE
Amanda has had immunotherapy treatment and may need a three-month course of intense chemotherapy in the future to stop the condition from attacking her face.
But she’s been left devastated as her physical appearance has changed drastically, and she feels like she has a “new face”.
Amanda said: “I’ve had to go from full-time hours to part-time hours, and I suffer from extreme fatigue, so I’m in bed for 8 pm. every night.
I’ve had some thoughts about not being here, but he’s [her husband] managed to talk me around and save me
Amanda Kenvyn
“I’ve even had to move into a bungalow because I can’t go up the stairs because I get so out of breath – that was a nightmare in itself because I loved my house.
“I suffer with nosebleeds daily because the disease is attacking my sinuses and my face – if I cough or laugh too hard, then it starts bleeding.
“My physical appearance has changed so much; I’ve never been thin, but from the chemotherapy and steroids, I’ve got a new face, and my nose looks strange.
“The immunotherapy takes time to build up and I’ve been told it’s working because the condition is only attacking my face.
“I’ll be having another round, and then if that doesn’t help, then I may need three months of intense chemotherapy that cancer patients have.”
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Amanda’s husband Gareth, 36, set up a Go Fund Me to try and support the mum-of-one while she battles the condition.
She says the pair are her “purpose for carrying on” with life and they have both “always been there for her at her lowest”.
Amanda said, “My husband and son have been amazing and always been there for me at my lowest.
“I’ve had some thoughts about not being here, but he’s managed to talk me around and save me.
“I just have to look at my son, and I think I need to be here for him – he’s my purpose for carrying on.”
Granulomatosis with polyangiitis: Everything you need to know
Granulomatosis with polyangiitis (GPA), which used to be known as Wegener’s granulomatosis, is a rare condition where the blood vessels become inflamed.
Typically it affects older or middle-aged people, but all ages can have symptoms, even children.
GPA mostly affects the ears, nose, kidneys, lungs, and sinuses.
The condition can be severe, but with medication, sufferers can live relatively normal lives.
The cause of GPA is unknown, but it is thought to be linked to an issue with the immune system.
What are the symptoms?
It depends what part of the body is affected as to what symptoms are present, but these can include:
- Tiredness, high temperature, loss of appetite, weakness, weight loss and joint pain
- Lung problems, chest pain, wheezing and shortness of breath
- Kidney problems like blood in urine, high blood pressure, kidney inflammation
- Rashes, purple spots or lumps on the skin
- Ear, nose or throat problems like hearing loss, blocked nose or earache
- Irritated eyes or double vision
- Stomach pain, diarrhoea or blood in faeces
How is it tested?
It depends what part of the body is affected as to what symptoms are present, but these can include:
- Tiredness, high temperature, loss of appetite, weakness, weightloss and joint pain
- Lung problems, chest pain, wheezing and shortness of breath
- Kidney problems like blood in urine, high blood pressure, kidney inflammation
- Rashes, purple spots or lumps on the skin
- Ear, nose or throat problems like hearing loss, blocked nose or earache
- Irritated eyes or double vision
- Stomach pain, diarrhoea or blood in faeces
Source: NHS
