This is my PNH journey.
2010 Hypothyroidism and Polymyalgia Rheumatica. Prescribed steroids which immediately eased the pain but I had to reduce work to 3 days a week to cope.
2011 Scleroderma diagnosis: prescribed methotrexate while continuing on lower dose of prednisolone. Regular reviews by Rheumatology raised concerns at persistently low blood counts so in 2014 I was taken off methotrexate to see if that was the cause. No significant change in bloods – no action taken. Continued to monitor, stayed on steroids but new rheumatology consultant decided methotrexate had not made any difference to my scleroderma anyway.
Spring 2018: Rheumatologist observed blood in my urine at review, but no follow up of any kind taken.
December 2018: GP expressed concern at my blood test results after routine test and referred me to Haematology suggesting I might need an immediate blood transfusion. Consultant instead sent me for an immediate bone marrow biopsy and diagnosed PNH. I was referred to the specialist Leeds team in February 2019 (but saw Dr Hillmen at Birmingham QE outreach clinic) and given my first dose of eculizumab at Leeds 3 weeks later. Apart from a stonking headache after my first dose which lasted 24 hours and two sore arms from all the precautionary vaccinations I have never had any ill effects. The transfer to the 8 weekly ravulizumab was seamless and painless.
So how has it affected me, and how long did I have PNH before diagnosis? I reckon a good 4 to 5 years of anaemia and living with fatigue, taking early retirement and gradually giving up various activities. When it was picked up as an urgent concern by a routine GP blood test (I had a call by the out-of-hours service, they were so concerned) it was frightening. I was worried about the bone marrow biopsy and had to stop myself googling.
Just to finish, as soon as I met the Leeds (PNH National Service) team I felt a huge relief – reassuring and professional, and always available to answer questions. It took about 6 months of treatment before I suddenly realised I was feeling less tired and more able to engage again.