My son Xander

My name is Stacey Carpenter-Salmon and I am writing this on behalf of my son Alexander, who is now 8.

He experienced his first stroke at 5 years of age, underwent treatment for the past 2 and a half years, and he relapsed in January of this year with more small strokes.

In October of 2019, we (my husband Dean and I,) started to notice small physical differences with Xander. In the morning, his speech was slightly slurred, and he became very uncoordinated. He has ASD and is extremely articulate, and many people around him started to notice that his speech was changing. There was also one incident at this time when he suddenly said, “I can’t see out of my right eye,” yet it resolved immediately. It’s actually scary to look at all of these things now and see how long we waited to investigate!

After 4 weeks of strange symptoms, we took him to the GP to make sure “it was nothing neurological”.

The GP was amazing and sent Xander for an MRI, which he had 2 days later. Due to Xander’s ASD, the MRI was difficult, and it took 2 attempts to get him through it. The technician approached me when it was over and said, “We’ve found something, and you need to go straight to Emergency at Gosford Hospital- we have called ahead.”

This was terrifying and eventually later that night he was moved to Westmead Children’s Hospital, where we were told that he’d been having small strokes over the last few weeks.

Initially it was thought that he has Stenosis of the Internal Carotid Arteries and he started treatment with this diagnosis in mind. Xander went home two weeks later, and we spent the next 2 months (including over Christmas) getting used to a new normal and essentially waiting for more strokes.

In early January 2020 we noticed changes in the shape of one eye, and we took him to the GP and then Emergency. He was transferred in an Ambulance back to Westmead and we discovered a few days later that he had been having more small strokes.

Investigations through this hospital stay lead to Xander’s diagnosis changing to Childhood Small Vessel Primary Angiitis of the Central Nervous Symptom (sv-PACNS). From this new diagnosis, he was put on a treatment plan for the next 18 months, which included chemotherapy infusions and immunosuppressive drugs.

Due to severe oral sensitivities, Alexander was fitted with an NG Tube for 9 months and then he had surgery to fit a stomach PEG, to ensure he was receiving his medications.

From here, Xander’s treatment went pretty smoothly and while we were always on the lookout for new signs of stroke, he was very stable for 18 months.

In January of this year, Xander reached the milestone of finishing treatment, and he faced a final MRI to show that he was clear of new strokes.

However, from early December and into January this year, Dean and I had started to notice a few very subtle changes in Xander that reminded us of his very first symptoms in 2019. We monitored them, but knew we had the MRI scheduled and we felt that his treatment had been doing its job.

Exactly 1 week before the scheduled MRI, Xander woke up and his speech was obviously slurred and stunted. He was moving involuntarily (Chorea) and one side of his mouth was drooping. I immediately started to take footage of him talking and moving, and I sent it to anyone I could think of… his Paed. and all of my family etc., as I knew his Neurologist at Westmead was on leave. I called and left messages at Westmead too, but the wonderful Paed. called me straight away and encouraged us to drive straight to Westmead. Xander had an MRI on arrival and it took a few days to confirm that he’d had subtle new strokes.

Only 5% or so of children around the world have repeat strokes after treatment with this very rare disease, so he was very much in the minority.

Dealing with a relapse has been hard. While we are more ‘comfortable’ with the whole world of hospitals and medications, it’s terrifying to think that something has been going on again. We are back to a very heightened sensitivity to stroke symptoms, and we are hoping that genetic testing can shed some light on exactly what he is fighting, and why he is fighting it.

He is undergoing a different treatment protocol right now, which hopefully means no more strokes for good.

With his younger brother Jude (Xander on the right and Jude on the left)