Amanda Kenvyn, 33, has the rare condition granulomatosis with polyangiitis – which causes inflammation of the blood vessels in her nose, sinuses, lungs, throat and kidneys
A mum-of-one has told of her rare condition that attacks her organs and means she needs to have 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. She started with 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 was diagnosed with GPA and has 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. 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 there’s nothing else that can be done for me.” 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.
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 up 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 doctors and she told me it could be this disease.”
It is believed only around one in 100,000 people are diagnosed with GPA in the UK. Amanda says she was ‘totally confused’ when she was told she had it, because it’s that rare, she had never heard of it. She said: “I was totally confused. The first thought I had was ‘why me and what have I done to deserve this?’ because I thought I was going to die.
“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 and now I’m finding it so 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?”
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 so much’ and she feels like she has a ‘new face.’
Amanda, of Scunthorpe, Lincolnshire, 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 8pm every night. 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.”
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 my son have been amazing and have always been there for me at my lowest.
“I’ve had some thoughts about not being here and 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.” You can donate to the fundraising page here.