The Disease


We have had a ‘good week’; when you talk to anyone who has been or is going through chemotherapy (or anyone who has cared for someone on chemo) they are likely to talk in good/bad days/weeks.  This is the cycle you sign up for when you scrawl your name across the consent form.  It’s easy to get carried away when you have a good week and want to do loads of things.  I’ve lernt from past mistakes that it’s best not to commit to any plans at any stage during a cycle of chemo because you just don’t know what might be around the corner.

For those that follow us on social media you will have seen that the main side effect at the moment is from the steroid ‘prednisolone’.  The appetite is unreal! Turkey mince madras with rice in a tortilla wrap is in high demand, as are Weetabix minis! Jack has managed to function as normal in school (with a few added snack breaks) and we are comfortable with the at home medication regime.

When I decided to start writing this blog I promised to share the good, bad and ugly.  I also pledged to raise awareness of paediatric cancers more generally and to reach out to other families in our current position.  I feel that although this doesn’t really count as a diary entry as we are having a ‘good week’ I ought to use this forum to talk more specifically about Jack’s diagnosis and what the disease is.

Langerhans Cell Histiocytosis (LCH)

In 2014 LCH was re-classified by the World Health Organisation as a cancer. This is significant because when Jack was first diagnosed it wasn’t.  I really struggled with this at the time because everything about the diagnosis, the treatment and so screamed BIG C.  Trying to explain to anyone that Jack has a disease that’s like a cancer but isn’t officially a cancer was just tough.  It made it harder to discuss with people and I almost felt like we didn’t belong on the oncology ward with all these children who had REAL cancers. Anyway, the clever people who know their shit when it comes to this have agreed (in the main) that it needs to be classified as a cancer.  Even as I am writing this another Mum has been in touch to say that she has had a nurse from Macmillan advise they may not qualify for some levels of support as LCH isn’t a cancer.  This just drives my determination to raise awareness more.  I had a similar encounter last week with the lady from the oncology outreach team who has been allocated to Jack.  She had put on the report for Jack’s school that LCH wasn’t a cancer. When I saw her in the hospital I advised her of the mistake and she said ‘well, it isn’t REALLY a cancer is it?’.  It took all my might not to go in to lecture mode but she had the initiative to go away and question her own knowledge with her peers and when we saw her the next day she had an updated ‘factsheet’ from CCLG and apologised for the confusion. DEEP BREATH Rachel, I am glad that she and her colleague are now updated but the classification changed in 2014.  How many patients/families have they wrongly advised since then?!

The controversy about its classification came from the fact that LCH can spontaneously resolve.  Sadly, this isn’t the case for Jack’s allocation of the disease.  I’ve spoke to loads of parents online and more recently in person about the disease and various treatment plans and I’m yet to find anyone who has had the same presentation or the same treatment.  This just adds to the confusion.  For example, I met a Mum and her daughter recently at Alder Hey.  The daughter had been diagnosed aged 2, had Diabetes Insipidus (like Jack) and had successfully completed the first line treatment of Vinblastine and Prednisolone for a year.  The daughter has a small lesion in her skull currently and her consultant was following the ‘watch and wait’ approach.  This blows my mind as Jack also has skull lesions, how do they know that one is OK to watch and wait and one isn’t?

Langerhans comes from the doctor who first described the cells in the skin that are similar to LCH cells.  Histiocytosis refers to the histocytes which are part of all of our immune systems and are found in many parts of the body.  The histiocytes in LCH are abnormal, they are dendritic cells that in a ‘normal’ person stimulate the immune system, in LCH patients they over stimulate the immune system.  They are usually found in different parts of the body, including; bone (which Jack has) , skin, lungs, liver, lymph glands, spleen and pituitary (Jack had this last time but no changes reported on the most recent MRIs).  There are different groups of LCH depending on which organs are involved and how many of them.  So Jack is Multi-system, Low-risk as bone and pituitary aren’t seen as ‘risk organs’.



They don’t know yet what causes LCH but recently it was discovered that LCH cells carry a mutation, Jack was tested positive for the somatic BRAF mutation.  For anyone who has wondered if the disease is genetic, this is how we know it isn’t, these mutations happen after conception.  They aren’t sure what causes the mutation but the findings have meant that in recent years they have started researching new targeted therapies. It’s also worth noting here that  The ones of interest to us are the BRAF inhibitors.  I have asked each of Jack’s consultants if he qualifies for any trials related to these new therapies, it is an option to be considered as far as I can see only when other ‘standard’ protocols have been tried and failed.  So basically, if he relapsed again after this time and more organs were involved they may try the inhibitors.  Having spoken to some parents whose children are on these therapies there’s plenty of positive experiences.  The only issue is, they don’t know when to stop the therapy.  I understand that reactivation occurs very quickly in most patients as soon as the therapy has stopped, they also don’t know enough about the long-term effects of this type of treatment and so the risk is not easy to assess.


These obviously depend on which organ is affected and if there are more than one. In Jack’s case he presented with the symptoms of Diabetes Insipidus (passing large amounts of wee and drinking loads of fluids, in Jack’s case he drank 8 Litres of water in 24hours while we were admitted with him in hospital) this was because the LCH had damaged his pituitary gland.  Other symptoms include; enlarged lymph glands, poor appetite, irritability, bone pain or swellings in the skull, skin rashes such as really bad cradle cap or nappy rash that don’t ease with the normal topical treatments can also be LCH.  There may also be discharge from the ears, breathing difficulties, upset tummy and even jaundice.  As you can see these symptoms are pretty generic and many ‘normal’ children will go through phases of having most of them which is why its so often mis-diagnosed until serious damage has been done.  In order to get a diagnosis various tests need to be done, usual blood and urine, X-rays, MRI and ultrasound examinations may also be used and in most cases a biopsy (taking some tissue) to confirm.


As I mentioned earlier some kids are just put on ‘watch and wait’ and the disease disappears without treatment. I haven’t heard of many of these cases in real life but I suppose you wouldn’t bump in to the parents in hospital if no treatment was happening! If the disease is single system, the approach is surgery, steroids and a drug called indomethacin (which is also used to treat arthritis). If the disease is multi system then chemotherapy and steroids is the standard.  The length of time on treatment depends on the child (and the doctor treating them I’ve found!).  When neither of these approaches work then the new ‘inhibitor’ therapies are introduced.

At the moment there’s an international study happening called LCH 4.  They are enrolling 1500 children and will collate and analyse the results.  Jack didn’t qualify to be included in the actual study but the consultants all agreed he would follow the same regime.  This could be a good thing as the protocol is 6months of intravenous chemotherapy plus steroids and then one of two maintenance pathways.  As Jack isn’t in the study the maintenance part is open to discussion depending on results from tests at the end of the 6 months.

Follow Up

Most kids recover completely from LCH.  If you ever hear me talking about it I often say ‘the prognosis for Jack is really good’ and it is. However, many are lefy with persistent problems and recurring disease and for some LCH can be life threatening.

The consultant who was in charge of Jack’s care initially had him in clinic every 3 months after he finished treatment.  They check height and weight and ask general questions and of course they are the point of contact if you have any concerns or notice any changes.  Reactivation of LCH is unfortunately common but treatable.

Hopefully that’s a bit easier to digest than the reams of medical journals you stumble across when you google LCH.

I find myself increasingly drawn by the idea of learning more about it on a serious level, Dr Daley anyone?!

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I'm a 34 year old Mum of two from Liverpool. My son Jack was diagnosed & successfully treated for a rare blood cancer called Langerhans Cell Histiocytosis in 2014. He has recently relapsed so I have decided to share the journey. My reason for being so open this time round is three fold; I want to raise awareness of childhood cancer, I want to reach those parents & families who are just getting a diagnosis & (selfishly) I find writing therapeutic and feel that sharing the story will help me get my own head around things.

One thought on “The Disease”

  1. Wow…what a read. I’m glad they have changed the status of the disease & classified it as a cancer…that must have been a tough one to deal with for you guys. Dylan’s cancer is also rare as it’s b-cell & he was stage 4 when diagnosed. It felt a bit like they threw everything at him because they hadn’t seen anyone with his cancer for 17 years. It’s a mental mine field but knowledge is power. I know what you mean about the “good weeks/ days” it’s so tempting to run with it as like you say, you never know what’s around the corner.
    Sending you lots of love xx

    Liked by 1 person

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