Back when Dylan had his End Of Treatment scan 7 months ago, there was an area on one of the slides we were sent that concerned us. On either lung there were some small white spots that resembled nodules which would indicate new disease occurrence. We spoke to Dylan’s respiratory consultant who assured us it was probably areas of calcification (scarring) but he was also honest in saying there was no way to know for sure without a biopsy. So cue us on tenterhooks waiting for his follow up scans to look for any changes.
A brief overview of how Pulmonary Langerhans Cell Histiocytosis works is that the faulty gene causes the defective Langerhans cells to go an area of the body, presumably in an immune system response. These faulty cells then cause damage called lesions. A lesion is an area in an organ or tissue which has suffered damage through injury or disease, such as a wound, ulcer, abscess, or tumour. In other areas of the body it can be a hole or a rash or a cyst but in PLCH it causes a nodule. These nodules then progress over time and fill with air turning into small thick walled cysts then eventually large thin walls cysts such as Dylan has.
The cystic presentation is usually late stage disease and is rarely seen in children, the reasoning behind this seems to be that lungs usually tend to be a secondary or later organ to be affected by which point diagnosis has been confirmed and treatment has usually started and stopped the progression from the nodule stage. I am no doctor of course but this information has been taken from various medical papers by experts in the US.
Fast forward to this week’s scan which was an X-ray and we could see these white dots again. I don’t recall seeing the X-ray from when he had his line out as he was an inpatient but I also don’t recall EVER seeing these dots on X-rays before that clearly. However it then came to light that unlike every other X-ray he has had this one was done from the back, so this area must be further back in his lungs and obscured slightly from the front by the remaining cysts and scar tissue. Still slightly worried and not feeling very reassured Mike was scrolling through scans and low and behold those dots were actually on the first scan he ever had back in January 2016 if you scroll through different slides. So even if they are nodules they aren’t new ones, they’ve been there for two years and haven’t changed! This means they obviously aren’t active disease so I finally felt like I could breath again after 7 months of stressing out!!
But that is a good way to be really. We need to analyse everything and watch for changes.. in his behaviour, his sats, his energy levels and his scans. Because that is the only way we will pick up his disease reactivating in time to save him.
