The Good Doctor

There has been uproar in the Histio community today over an episode of The Good Doctor that aired last night. An earlier promo had shown that Langerhans Cell Histiocytosis was going to be featured so there was excitement at first over the potential awareness this would bring. However the episode itself was flawed and did not accurately portray LCH leaving a lot of angry patients and parents.

*Disclaimer.. I haven’t actually watched the episode myself and after what I have read I don’t intend to. My comments are based on what friends have said happened and the synopsis.

The diagnosis was given as an alternative to the original diagnosis of Osteosarcoma and was described as not being a Cancer like first thought. We all know this is incorrect and LCH is a rare Cancer and after years trying to get it classified correctly we are sick of having to fight those ‘at least it’s not Cancer comments’.  It is CANCER and AUTOIMMUNE so it is the ‘honey badger’ of diseases and does whatever the hell it likes. It is not limited to blood, bones or skin, it can damage what it wants essentially...

It was also described as being easily treatable with prednisone which is a steroid pill. This is also incorrect as LCH requires Chemotherapy and is often not easily treatable with patients requiring multiple types over many years or even gene therapies for persistent recurring cases. Sufferers may need organ transplants because these rogue cells DESTROY the body and it isn’t always reversible. Sadly you can also can die from this disease whether or not people want to hear that.

But why such upset over a TV show? Lots of medical shows are inaccurate after all..

For those of us wanting awareness, it brings the wrong type of awareness. It marginalises our struggles and makes patients, and those caring for people with this disease, look like ‘hypochondriacs’ or liars. It creates a ‘my disease is worse than your disease’ mentality and downplays the downright hell our children (and adults sometimes) have to go through.

There would be uproar if Leukaemia was referred to as the ‘easy’ Cancer on a TV show because everybody knows what it is and that it can kill. But because nobody has heard of this disease, our concerns are met with comments such as ‘calm down’ and ‘it’s not that serious get over it’.

And as a parent of a child that nearly died REPEATEDLY because this CANCER destroyed his lungs I can tell you it damn well is serious!

#RANTOVER

I Can't Wait To Watch You Grow

Most people don't want their children to grow up. They do grow so fast it's true, and in the blink of an eye they go from tiny babies to school age, and then before you know it they'll be teenagers! It's a sentiment I do share believe me.. but I also can't wait to watch mine grow!

When you've faced the fear of loosing your child it really does throw a whole new light on everything. I am so lucky I get to watch my children grow into adults and that is amazing and something not to take for granted. Yes it is wonderful when they are little and you should cherish every second of it. But don't feel too sad when they go up a clothes size, start a new year at school, have another birthday.. because growing up is something not everybody has the chance to experience.

I thought that Dylan would be 'forever two' when we nearly lost him just weeks before his third birthday. Now instead of wishing he would stay little like I use to, I am just so excited to watch him grow and get stronger! I cant wait to hopefully watch him go through all the stages of school, find love, find a career, get married, maybe have children of his own etc. because he was nearly robbed of all those things.

Childhood diseases such as Cancer claim so many lives a year, and it's easy to live oblivious to this if you've never seen it happen close to you. So many children won't have had a 'first day at school' this last week. So many won't next year either. Lots never get to ring that 'end of treatment' bell. Some wont reach adulthood. Many more may not get married or have their own children. These things are not a 'right' in life and are not guaranteed, they are a privilege in my eyes. Such milestones should be celebrated with joy not sadness.

We had an appointment this last week for Dylan at Great Ormond Street hospital that has been weighing on my mind since he finished treatment. Despite the disease being 'non active' I have always worried about the damage left behind and what it meant for his future. I have a huge fear of outliving my children and I was so frightened that once treatment had finished we would be told Dylan still needed a lung transplant because of the extensive damage. My fears however were quashed by Dr. Helen Spencer who quite simply said "I'm not going to transplant him he's far too healthy"! Yes the scans don't look spectacular but Dylan has proved at his appointment that he can manage with what he has, and very well indeed!! They are so pleased with him that they don't even want to see him next year unless there are any issues. If he continues to improve and the disease doesn't reoccur there is a strong chance he will be discharged from GOSH in two years and won't need a transplant. I walked out of that appointment feeling like the weight of the world had been lifted off my shoulders! Now just to pray the LCH doesn't ever come back of course.

My beautiful boys.. you are growing up SO fast and I cannot wait to watch you grow into the wonderful young men I know you will all be someday. You make me feel like the luckiest mum in the whole wide world, and I can't wait for you to show me what you will achieve each step in your journey through life. <3

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.."

Feeling Isolated

I'm starting to really see the effects Dylan's illness has had on Bryce as we approach the end of term activities. I'm lucky that he is a very outgoing boy and will often just invite himself into the group to play without a second thought, but as an outsider I can see the awkwardness of the situation. They all have their little cliques and friendship groups and I feel we are slightly isolated from those.

When everything first happened with Dylan it put a stop to plans we had for Bryce as well. He wasn't able to attend birthday parties as we were stuck in ICU with Dylan, so the invites soon stopped. He couldn't attend the out of school clubs because we couldn't afford it at the time due to the costs of hospital travel etc, plus we were already relying on other people to drop him off and collect him from school, we couldn't really ask they give up their evenings and weekends as well to ferry him to football or Beavers for example.

Once things settled down and Dylan came home I signed Bryce up for Beavers and every school subsidised club going but he struggled to mingle with the other children as they had already moved on and formed their new friendship groups whilst our lives had screeched to a halt. The one thing we didn't sign him up for was football because I was very aware how 'behind' all his peers he would be unlike with Beavers where they all do the same activity each week. 

And that was probably my biggest mistake.. he hates that he doesn't go to football with his friends but it's just not feasible either with Mike's shifts. All his 'friends' attend clubs he doesn't or have been spending time together outside school when he was stuck at Nanna's house for months on end. They seem to all have formed a strong bond as have the parents, people I thought I got on well with and vice versa barely speak to me these days..

When Dylan was first poorly I really struggled with the school run (I still do on bad days) and didn't want to talk to anybody at first so I kept my head down and stayed in the corner, but I can see how this hasn't helped because I just don't have a relationship with the parents of Bryce's friends. I stay in my comfort zone and only talk to the two or three people that I know which isn't helping Bryce. Whilst their children ARE friends with him, they aren't the friends he would play with at say lunchtime, because they are all girls and have their own games and groups of friends of course.

I do the same with Dylan though. I don't really talk to anybody at nursery because I hate talking about Dylan's illness and that is all people want to talk about!!! And with Logan.. I've actually stopped taking Logan to playgroup the last two months because I'm sick of being introduced to people as ''Dylan the boy who collapsed and nearly died's mum." Ugh!!

I am starting to feel very isolated. I've kept people at arms length because of how I've been feeling, and in turn people have done the same to me because nobody knows what to say to that depressed 'Cancer mum'. Everybody has gone about their lives meeting up, doing playdates, football, days out, nights out etc. while I've been stuck staring at four walls of either a hospital ward or this house.

I only hope that moving to Junior school may provide some fresh faces and a fresh start for Bryce. And I need to try and pull myself out of this depression and try for my children's sake to make friends. Feeling pretty terrible as a parent this week that's for sure..

Chicken Pox & Chemotherapy Patients

Why is the chicken pox virus so dangerous to chemotherapy patients?

It is quite simple really.. patients undergoing chemotherapy have a weakened immune system therefore cannot fight off the virus and it can cause serious complications such as pneumonia and blood poisoning such as toxic shock and sepsis, which can be deadly because these complications use up neutrophils quicker than the body can produce them.

Chemotherapy slows the production of blood cells, killing them off as they grow and divide. The white blood count typically goes down first because these have the shortest life span of the blood cells. Part of our white blood cells are made up of neutrophils which are used to fight off infections. If these drop below 1 then a person is less likely to be able to fight off an infection. If they drop below 0.5 a person is at serious risk of become extremely unwell from a minor illness such as a cough or cold. A persons counts are usually lowest 7-10 days after chemotherapy and they can take 2 weeks to recover by which point another dosage of chemotherapy is due for some patients such as Dylan for example.

In a person with damaged lungs or lung disease such as Dylan again, any illness that carries the risk of progressing to pneumonia can be devastating. Dylan nearly died last year from pneumonia because he quite simply does not have enough healthy lung tissue to cope with infection. You'd be surprised how many people out there have lung problems.

It is extremely frustrating to explain why chicken pox is so dangerous over and over. This doesn't just apply to chemotherapy patients either. If you go out into the world with active chicken pox or shingles you are risking EVERYBODY with any underlying health problems, this includes pregnant women, premature babies, newborn babies, elderly, those with lung problems, heart problems and other chronic conditions to name a few.

Please think before you go out into the world when unwell even if you feel fine. What may be minor to one person can be deadly to another. You never know if somebody has an underlying condition, are in the early stages of pregnancy, whether they care for an elderly relative or whether them or a sibling of theirs is receiving chemotherapy. It's not worth risking other people's lives. I do understand we all have busy lives with jobs to do and children to get to and from school but please ask for help in these situations. It may be a slight inconvenience to you, but imagine the inconvenience of having a child seriously unwell in hospital for months on end. Or having to split siblings up and have family take time off work because one has caught something that is too high risk to be near the other. PLEASE walk a mile in our shoes before you put your own on and head out that door in the morning. Thank you.

And on a side note, those saying we should keep him at home... REALLY?! Would YOU keep an active four year old at home all day everyday? He already missed out on five months of his life confined to a hospital bed last year and we've had to fight hard to get him back to where he should be for his age. Plus HE fought hard against all odds to be here today and he deserves to live his life and enjoy it!! A few simple precautions is all it takes from others, and that is all we ask for.