Initial Diagnosis

We were referred onto the Paediatric Neurologist based in CUH, Dr Niamh McSweeny, and got an appointment for a month later.  During this month of a waiting period, we had many assessments and scans carried out.  On the 2nd of February, we met with Dr Niamh McSweeny in CUH.  It didn’t take her long to diagnose Rose with SMA because she easily recognised the symptoms that I listed above, it was so easy for her to see these symptoms that at times had made me feel like I was going crazy.  I used to think I was over-reacting, or maybe I was under reacting but I had definitely been questioning myself over these last few weeks and months.  Karen paused, I will never forget that moment when she said it to me, I suppose I had googled it so many times and it was always the one that came up so I was surprised but I wasn’t surprised either, my heart just sank when I heard her saying SMA. 

I’ll never forget her words, she said, ‘I highly suspect that she has this condition called SMA’.  I didn’t know if I wanted to cry or whether I was going to faint so I said, right, lets pull ourselves together.  I asked her what does this mean for Rose and she just said ‘Look, 2 years ago if I was giving you this news, there would have been nothing that I could have offered you’, there were no treatments then, there was nothing that could have been done, basically Rose’s life expectancy would have been 2 years.  She added ‘However, we are where we are today, there is a treatment that has been approved’.  This treatment is called Spinraza and Dr McSweeny along with Dr Declan O Rourke, Neurologist, Temple Street got Rose on the expanded access programme of it.   The expanded access programme was run by Biogen, the pharmaceutical company responsible for the development of this drug.  The programme’s goal was to determine it’s efficacy before the HSE would agree to reimburse for future treatments.  This expanded access was just about to run out and Rose was one of the last of a handful of infants that qualified for this trial. 

By the time we had to wait for testing and genetic bloods and things like that to come back, Rose was 11 months old receiving her first treatment of Spinraza.  Dr Niamh Mc Sweeny ran the genetic bloods to officially confirm SMA which would normally take 2 weeks to come back.  Our results, however, took 4 weeks to come back which gave me a brief sense of hope that maybe the diagnosis had been wrong because normally you think that if there was a problem that you would hear sooner rather than later.  So I started to relax thinking ‘Ah sure look, it’s been 4 weeks now, she must have got her diagnosis wrong in her initial assessment’ and then I remember, she rang me on 28 February 2018 to say ‘Yes, unfortunately, it has been confirmed, Rose has SMA’.  Dr O Rourke in Temple Street had a place waiting for her to start, Rose was going to start her treatment and the progression would be halted and she would hopefully gain some strength as every child responds differently. 

We got her date to start Spinraza, it was going to be on 05 April 2018.  So between getting the diagnosis from Dr Niamh McSweeny and starting the Spinraza treatment, it was only a 5 week wait.