Hello Adam

my condolences on the loss of your mum in such a terrible way.  

CTCL Mycosis Fungoides is a strange beast.  Stage I A & IB are typically indolent, and you can get on with life and work with those stages.

At stage IIB, the skin involvement morphs from patches and plaques to tumours.  At that stage one should be considering stopping work, and realise that the disease is very serious.  As you correctly point out, the destruction of the skin barrier can cause death through infection.

The point I was trying to make is that disease progression is not automatic.  In about 75 to 80% of patients, the disease does not progress.  

However, if CTCL does progress to higher stages, it is simply awful.  The reason I stressed that most patients don't progress, is to not alarm the majority of newly diagnosed sufferers.  Many newly diagnosed CTCL MF patients use Dr Google, and see pictures of tumours like your mother had, and freak out.  I certainly did.

My CTCL MF has been stable and indolent for the past 9 years, and I haven't progressed above stage IA.  I have UVB light therapy periodically, and that sends the proliferating T-cells to oblivion.

Each patient is slightly different.  CTCL MF is not caused by a mutation of a single gene, but is caused by mutations of a basket of genes, maybe 15 to 20 genes.  Everybody's basket is different.

So, to the newly diagnosed reading this post, please don't assume that the terrible suffering that Adam's mum endured will happen to you.

Please find a haemo-oncologist or dermatologist who knows about the disease, and try and get yourself staged.

If you're staged at II B or above, the disease is not indolent, it's a very serious life-threatening condition.

I hope this helps.

I am sorry if I sounded like I was trying to scare anyone, I guess that I am in so much pain right now, I loved her so much, she was my best friend as well and my only freind. I was outcasted by "fake" friends years ago, it hurt my confidence and she was the only one that understood me and comforted me. It broke my heart seeing her on an oxygen machine and trying to get the fluid off her lungs. 

It's all my fault, I and my family should of took her into private healthcare at the first signs of her symptoms. She saw about 5 local surgery doctors before they took her seriously over the course of a year. I let her down, she would still be here like yourself if she was refered quicker to someone who knew what they were doing. NHS has let my Mum down, I just want to speak to her again but I can't. Another person on here I spoke to a few weeks ago, his/her mum also passed away in same circumstances where the NHS let their Mum down as well with poor/late diagnosis which resulted in poor outcomes.

I am glad you are still in remission but you have to understand as we have been told that finding an oncologist in this field in the UK is very difficult. Privately we would of, but there isn't an oncology center for about 100 miles from here. I am in so much regret right now of what I could of/should of done to save her life. 68 is too young imo and I am 32, I have lost both parents now. My Dad died in 2010 of an even worse condition, Bile Duct cancer which has a very poor prognosis. I even thought it was Psorasis because, you trust your Doctor's decisions. Obviously those decisions have haunted me since Mum's passing.

Doctors need to be trained better in diagnosing and refering patients because they are sending people with this disease to their doom. Doctors in local surgerys obviously don't have the knowledge but they should still refer you to an oncologist ASAP because they are playing with fire. 

Hi Adam

I'm sorry to hear about your pain and anger over the tragic loss of your mum.  Your suffering is obvious and raw.

Please try not blame yourself that it should have been identified earlier, because CTCL MF is such a rare cancer.

To illustrate just how difficult it is to diagnose, I read an journal article by Don. B. Cauthen MD that was published in 1994.  The title of the journal article is 'Family Practice Incidence Rates'.

In that article, the author estimated that the average doctor would have around 2,000 patients, and would for example see 6.2 cases of cardiovascular mortality per year.  The same doctor would see 1 case of tuberculosis every 5.6 years.

The author used the same methodology to determine that the family practice doctor would see 1 case of Mycosis Fungoides every 172 years.

In that context it is unsurprising that 5 local surgery doctors did not know what your poor mum had.

In my case it was discovered by accident.  I was seeing a dermatologist about some skin issues, and happened to mention the strange red patches on my lower legs.  Being a dermatologist, he took some skin punch biopsies and sent them to a dermopathologist.  The dermopathologist had a hunch, and applied the correct staining solution to the samples, and I was diagnosed in a fortnight.  That is exceptional.  It is not unusal to hear that Mycosis Fungoides can take 3 years or longer to diagnose.  

I hope that you overcome your grief Adam.

Hi Aronoel,

May i know how's your son now? what treatment does he have currently? Is his tumor shrink totally? Hope you can enlighten me. Thank you. 

Hi, I know your post is old but wanted to see if I could ask you a question or two. I’ve had MF for about 8 years now and it has slowly progressed. My Dermatologist sent me to have UVB Narrowband light treatments. I was surprised to hear the Dr say I needed to go for the rest of my life. I’m in the Patch phase. I’ve now been for fifty (50) treatments but it has been a struggle. They keep telling me I’m at a low dose (650) but I continually get burned (about 4-5 times very badly). In addition, the treatments “knock the stuffing” out of me.....groggy, tired, fatigued, useless. Not sure if it’s related but now my feet are swollen. It looks like fluid that won’t move. Usually I’m fine in the morning but by nighttime both feet are badly swollen. I’m a bit desperate and actually considering stopping the treatments.......If you’re still there can you give me your thoughts, suggestions, etc., perhaps relay some of your experiences?

Thanks,

Geoff

Hello Geoff

I'm lucky in comparison - my skin type tans easily and I end up with a rediculous tan.

Even if you were a red head with extremely fair skin, I would be surprised to hear that you were getting burnt though.  The whole process is designed to avoid sun burning, by gradually increasing the dose, calibrated against your skin type, usually with an algorithm (computer chip) controlling the time dosage.  Well that's how my dermatologist's light chamber works.  

The staff encourage reporting of sun burn, and they dial down the dose for a session or two before the gradual increase starts again.

it could be worthwhile seeing if you can find another light chamber at another facility, so that you can compare and contrast equipment and staff expertise.

i've probably had 120 sessions over the last 10 years.  In my case I can have no patches for about two years, and then need UVB treatment.  Some years I have had preventative UVB treatment when I start to get tingling skin sensations, but no patches are visible.  But the UVB then makes the patches visible.

i subscribe to a US list server for CTCL MF patients, and have never heard of swelling of the feet.  I'd definitely report that to the dermatologist, and your GP, as something else might be happening Geoff.

Tiredness is sometimes reported as a side effect though.

With regard to the lifelong sentence, yeah, our DNA has been damaged and changed by something.  It can be old age, industrial chemicals like benzene, or other factors.  The exact causes are not known, although the disease was documented before the industrial revolution.

My understanding is that it's not a single mutated gene that causes CTCL MF, but rather a basket of mutations of multiple genes, which is why some folks have an awful time, but others have an indolent condition.  Nearly every sufferer is slightly different.

Obtaining good advice is bloody hard, because this wretched disease is so rare.  My first dermatologist wrote letters to the nearest teaching hospital and made such a nuisance of himself that the Haematology Department staged me.  I'm stage IA.  That required a bone marrow biopsy, multiple blood tests, DNA analysis, and a PET scan.  While I'm British by birth, I reside in Perth, Western Australia.  The reason for mentioning that is that the official protocols for staging (and the expense associated with it) vary from country to country.  Haemo-Oncologists can also be your primary health care doctors, particularly for more stubborn cases.  Radiologists can also get involved in some cases - so it can be a real multi-disicplinary arrangement.  The centre of excellence in Australia is in Melbourne, a couple of thousand kilometers from where I live.  It is probably worth finding out if you can be assessed at a centre of excellence in the UK.  Easy to say, but I have no idea whether that is achievable Geoff.

I hope your condition improves.

Regards - Mori

Hi Tracy

Targretin (Bexarotene) is apparently effective for folks with higher stages of CTCL MF, and yes I have heard of the side effect you mentioned.

In the USA it's very expensive.

I take my hat off for your son - and congratulations for his marriage.

Radiotherapy is surprisingly effective for this disease - it can "Nuke" tumours, and Total Skin Electron Beam Therapy can help many sufferers.

The disease is difficult to comprehend - in the early stages one can keep on working.  In the higher stages it changes into a different beast.

Regards - Mori

Hi Geoff,

I saw your recent post on here and it rang bells with my experience if UVB. I’m in the UK and have only been diagnosed for 8 months. I didn’t have swollen ankles but all of the other symptoms you mentioned including burning. I wasn’t happy with the treatment and the dermatologist wanted to move me straight into PUVA. I therefor transferred my care to a hospital which is a centre of excellence. The dermatologist specialises in curtaneous lymphomas and although it is slow progress they are thorough in staging my condition properly befor continuing treatment.  I am going to have a lymph node removed for testing in 10 days as two in my armpits are enlarged. I’m hoping the results are not too bad.

Are you in the UK?

Jayne

Hi Jayne, thanks for your response. I’m in Florida in the U.S. i’ve had 52 UVB treatments so far. I insisted the Dr office lower the strength of the UVB and it’s been going better. I’m insisting on a lower dosage and gradual increase to avoid burning. I see the Dr tomorrow. Each session “knocks the stuffing” out of me but the Dr keeps saying it’s not the UVB. That part doesn’t make sense to me.