New Worries

Despite the fact Dylan has now finished treatment, things feel uneasy in our house. It feels a little like one door has closed but another one has opened and we are now looking more at the long term side effects and complications the disease has caused..

The recent CT scan showed very little improvement which was disheartening. In truth the reason we have previously seen such drastic improvement on scans is because the cysts were bursting. We can clearly see where large cysts once were by the scarring left behind. The cysts that are remaining are obviously not high risk of popping which is good in the sense that it takes away the risk of lung collapses, but it does mean they won't just 'go away'.

There is little known about Pulmonary LCH in children so there is nothing to really base a prognosis for Dylan on. In the few studies out there, some surviving patients have cysts remaining all their life, others they gradually resolve over the course of 20 or so years but it obviously would differ based on severity. For this reason he will be reviewed regularly of course but it worries us daily. He also has scar tissue where cysts once were which will have implications on how well his lungs work. He may continue to live a happy and normal life or these issues may impact on his breathing more as he grows and he may require a transplant still to buy him some extra time or improve his quality of life. People ask us his life expectancy and to be truthful we do not know and it is not a question we like to answer as it is still likely we may outlive our child, and that is a something no parent wants to ever face.

It may come back... relapse rates in LCH are unclear. There are lots of statistics out there but Dylan doesn't really fit into any of those as he has what is considered 'adult type' PLCH but to give an idea, the relapse rate for high risk organ LCH is approximately 54% with most relapses occurring within the first 12-15 months. Infact some people relapse within weeks of treatment finishing. Also unlike some other types of childhood Cancer.. LCH isn't limited to one system, just because Dylan had it damage his lungs doesn't mean it would happen again. It could quite literally come back anywhere and without doing regular full body MRI scans which are not practical there is no way of knowing until symptoms show. So every ache and pain could be a sign of it coming back in a bone, any rash could be it effecting his skin, any sickness or bug could be it effecting his GI tract, liver etc. LCH is a tricky little buggar.. described by a fellow 'Histio mom' as the honey badger of diseases as it does whatever the hell it likes and now our 'safety blanket' of Chemo has been taken away!

At one point before diagnosis was made, I was told Dylan likely only had 'months' rather than years left, so any improvement on that is a win for us and we will take every second we have with him as a blessing. It is impossible not to worry about the future, but you cannot let it impact on living life in the now.

But then sometimes even living in the moment brings worries to face daily and bridges to cross in the journey... For example, Dylan hasn't gained weight in the last six weeks which has me a little concerned. He has been constantly unwell lately, and fighting off illness can burn more calories so I am going to keep a close eye on him the next few weeks now his body should be recovering from Chemo and be able to fight off viruses easier. I am hopefully this is just a minor blip and not a signal of any underlying problems.

People will be expecting us to slowly go back to 'normal' I suppose now, but I don't think our life will ever be normal again. Every single day for us will be lived in the fear it is our last.. for we never know what is around the corner for any of us...

Surprise!

Back in February at Dylan's 12 month review we were told 'six more months' of chemo. I then worked out that 18 months is 78 weeks and counted on from that appointment. But I made a mistake...

Monday I made my usual three weekly call to the QMC to book Dylan in for his Chemotherapy appointment on the Tuesday. I had on my calendar that this was week 77 out of the 78 and would be his last week of actual Chemo as week 78 would be one of the weeks off. We were looking forward to marking these milestones together.

However it turns out I was wrong with my maths. I didn't count the first week he started treatment as week 'one' I just counted on from there. I also didn't count in the extra week he had before his 12 month review. So I was informed on the phone that LAST WEEK was actually week 78 so he was already finished Chemo. Dylan's consultant had even said 'no more Chemotherapy' in the diary.

This took me by complete surprise and I cried a lot once I got off the phone. I had been expecting Chemo this week, as had Dylan, so to be suddenly be told he had finished was a whole wave of emotions. I also felt a little robbed of the chance to celebrate his last dosage, it had been and gone without me realising it!

Obviously all this was pending Wednesday's pre booked CT scan to check the LCH is still 'Non Active' and we had a clinic appointment afterwards to confirm 'end of treatment' and go through details of what happens next.

But then on Tuesday we had some other sad news. My parents' dog had suddenly gotten very poorly over the weekend and it was discovered Tuesday morning that he had Cancer and was bleeding internally. Devastatingly it was already too late to help him, any attempts to remove the Cancer would have been in vain and he would likely have suffered so my parents' had to say goodbye to their beautiful German Shepherd.

This affected us all and tinged our mood with sadness. How could we possibly celebrate the very next day if we got some good news? What if we got more bad news? Emotions were running high and I was feeling angry, so angry with Cancer and the fact it seems to keep picking on us. Those that know me personally know that we've had a lot of Cancer in our family prior to Dylan. Some people go through life never facing hardship or illness and others seem to hit it every way they turn. I see so many families loosing to these diseases that celebrating feels almost bittersweet.

But then Dylan ringing that bell would be a huge 'F*CK YOU' to the disease that tried its hardest to kill him and would for us be in memory of those no longer with us.. 

So here we are.. Dylan's scan results were a bit anti-climactic this time with very little change but they are stable and there is no signs the disease is still active which is the main thing. We are not sure what will happen with regards to the remaining cysts or damage and scar tissue. We should have a review with the transplant team at GOSH and our respiratory doctors to talk about those issues over the next few months.

We have no idea exactly when the LCH activated but after an almost 2 year journey (from first symptoms) that has included; 18 Months of Chemotherapy, 26 Chest Drains for Pneumothoraces, a 5 Month stay in Hospital (with 23 days of that spent on Life Support), and 5 very VERY close calls.. TODAY our amazing superhero Dylan finally got to ring that pivotal End of Treatment Bell at Nottingham Children's Hospital signalling no more Chemotherapy!

He will still keep his Hickman line in situ for blood tests and until his repeat scans are done to check there is no relapse now that Chemotherapy has stopped, that should be reviewed at some point in November.

We are of course hopeful that the LCH doesn't EVER come back and that he can get his 'wigglies' out before Christmas, then hopefully we can start to think about putting the last 2 years behind us and focus on the future! <3

What Causes LCH?

LCH is caused by mutations in a cell signaling pathway known as the MAPKinase pathway. Key genes mutated in this pathway include BRAF (65-70%), MAP2K (20%), and other rarer genes, all of which lead to abnormal activation of a gene known as ERK. These mutations are “genetic accidents” which occur during DNA copying in dendritic cells and are not inherited. The maturation stage of the dendritic cell will determine what type of disease a patient will develop. If the mutation occurs when the dendritic cell is still in stem cell stage, this early precursor may go to any organ in the body-especially liver, spleen and bone marrow. Mutation in a more mature dendritic cell will lead to LCH in a variety of organs, but not the “high risk” ones mentioned before. An even more mature dendritic cell carrying this mutation may go to only skin and bone.

BRAF is the most common mutation in a cell. The mutation actually causes the cells to keep producing even though there is nothing telling it to turn on. There are receptors that usually tell the cells to multiply and when there's a mutation like the BRAF mutation it (the switch) is stuck on the "on" position. That's why the lesions develop.

They don't know why they develop in the places they do and they don't know why some form lesions and others eat away at things like bones or even white matter in the cerebellum. There is another theory that the faulty genes can cause the cells to multiply in an immune response, such as to infection. They rush in to try and protect the area but instead these immature defective cells cause more harm and damage that particular system. So with Dylan, for example, it is suspected that a chest infection and swollen lymph node he had early 2015 triggered his immune system to respond and sent these defective cells to his respiratory tract and lungs.

The same theory goes for adults that smoke. If they have one of the faulty genes then in theory the body sends these langerhans cells in to protect the lungs from the chemicals etc in cigarettes but of course they do more damage. Doctors do not think smoking itself actually causes LCH, it is the immune system's response that can cause it.

So in theory anything can trigger these rogue cells. There is no 'remission' for LCH only 'active' or 'non active' disease and it can reactivate at any point. Doctors are still trying to combat 'relapse' or reactivation rates by increasing the length patients receive Chemotherapy or by trialling new inhibitor drugs for those with particular gene mutations that haven't responded to Chemotherapy.

So when the question is asked.. is LCH cancer? The answer is vague. It is now classed as Cancer officially even if it it not fully recognised as one. It is an autoimmune disease at its baseline, that acts cancerous. So both, in the words of Gregory House (House M.D. episode 'Merry Little Christmas')..

"Also known as 'you got your Cancer in my Autoimmune' disease.."