Noah was resuscitated at birth and had a tracheostomy at 2 months because of tracheomalasia and laryngomalasia. They thought that that was the only problem but they could not get Noah off the vent so in the end did a lung biopsy and found out about PIG. We tried a couple of attempts with the IV steroids (methal predisinone) but they didn’t make any difference and so we stopped them and started hydroxychlorequin. Noah was put on the long term vent and we escaped from hospital after 10 months (Alder Hey Childrens Hospital) and we are now under Leeds hospital. We have had Noah at home since he was 10 months. Whilst in hospital, he was initially very poorly in picu and having respiratory arrests all the time, then he was eventually on cpap in oxygen. When we came home, we upped the vent to Bipap which enabled us to get rid of the oxygen. He is now only on oxygen if he is poorly with a cold. He now has a speaking valve for his tracheostomy which allows him to talk (and have a peep of 5 which he needs) and is on the speaking valve / off the vent whilst he is awake and he is on the vent for all sleeps.
This is how Noah remains, each year we think that this is the year for us and we wean the vent down to off or almost off but he is not strong enough to manage yet and has very low sats so he goes back on and we try and try and try again. Maybe this year we will get him off the vent, Noah was very poorly last christmas with a bad bout of RSV and so they want him to manage throughout a winter with no need for added interventions (no vent anyway and minimal 02) if he picks up winter bugs. If he passes that bit, they will do a scope and see if his airway is firm enough to manage without the tracheostomy tube and if so we will try to remove the trachy. Only time will tell.
We remain with carers living with us day in day out who are a godsend – we have day and night cover with someone awake with noah the whole time. Noah is doing fab with his learning (which was delayed) and his talking on his speaking valve. He walks with the aid of a walker but can not stand up himself yet but he will do one day. Noah now attends full time mainstream school and is in the reception class which he attends with his carer. In September (2016) he will have an educational one to one start with him as well to bring him up to speed. He’s a clever little boy but it just takes longer for hi m to grasp an initial idea. We continue to try to wean his ventilation year by year and are now very low but not quite off yet. Noah likes to have his own plans and will come off when he’s ready. For now he’s a happy little boy loving life.