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Adult Langerhans cell Histiocytosis and CMML
I was diagnosed with Langerhans cell Histiocytosis in May of 2016 after a biopsy of skin lesions. I had never heard of this rare disease and the NP at Mayo Rochester who called with results said she had had to look it up and she gave me some basic information. I soon learned much about this blood disorder of myeloid cell origin which affects only 1 or 2 adults per million population. It is more commonly a pediatric disease, although still rare. It can cause lesions on skin, bones, lungs, liver, or spleen. It can also cause problems with the central nervous system.
After a PET scan and other tests and imaging, it appears that mine is skin only. I did test positive for an acquired mutation of the BRAF gene that is found in up to 60% of patients with the disease. I have tried numerous topical treatments, including several strong steroidal ointments and cream, ointment to rev up my immune system and even nitrogen mustard in gel form. Although some helped, all topical treatments eventually, or very quickly caused worse skin irritation that the original lesions. Brachytherapy to affected skin in the groin area caused painful burns. For the last 5 months, I have been taking Hydrea, 500mg and then 1000mg per day.
This has resulted in the best response yet. I still seem to have flare ups and then subsiding of the skins, but much less severe. In June, 2019, a bone marrow biopsy was performed after blood labs had shown low platelet counts and high monocytes over a period of time. I was diagnosed with chronic myelomonocytic leukemia (CMML 0). This is another rare disease that is a myeloid disorder. My best hope is for a slow progression of this leukemia, since current treatments only treat symptoms. There are possible treatments being studied, but not yet available. Having any rare disease is frustrating since there is often very little money available for research because so few would benefit. It is also difficult to find providers who are knowledgeable about diseases so rarely seen! Any others have any experience with these disorders?
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I couldn’t let another day go by without popping in to see how you’re holding up, my friend. I think this has been a long winter for you so I really hope there is the renewal of the season all around you with bright sunshine and spring flowers. Do you have a lot of spring bulbs in your gardens?
Have you begun your online flower arranging class? 🌷🌸
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It has been a long time.times I feel I am doing nothing but then again,I am making each day as productive as possible
I now have my walk routine changed.I am now doing a 6 k most days,this time it is to the grave.gives me a real purpose and it is nice to visit and see the how the flowers are
Yes I am doing the online flower arranging
Really good
I buy my own flowers have the oasis and all the stuff needed
You would be very proud of me seeing my ‘artistic ‘ work
I wish I could you .Michael would be proud too knowing I did them all by myself
His memorium cards arrived yesterday.
Very difficult to do but they are so special and my daughters say he would be proud too.
The daffodils and tulips are doing well ,but I know he would be doing more as this is the time to plant.,but I’m not ready for too much yet
The robotic lawnmower we got last year is out now in the garden.the guy we bought it from called in and set it up for me,so kind.
He is greatly missed in the house,so quiet now,but the girls call and my daughter in Spain wants me to visit but I’m not ready to go just yet,she understands .She will be here for Easter
I hope you too are keeping well,I am talking too much about me.but thank God for good friends,means so much to me
Much love xx💖🌺☘️
I have a very rare lung disease, 1 in every 200K are diagnosed yearly and it is called; Pulmonary Langerhans Cell Histiocytosis. (Or PLCH) My body is creating more white blood cells than it can use, so they attack vital organs and tissues, lt is cancer- like, in that this can eventually cause tumors or damage to other organs.
I was diagnosed March 31st 2015.
I had to have a biopsy done to be sure, While I was under and as they were extracting, My right lung would not reinflate. I was placed in ICU {missing 4 days as they had put me in a medicated type coma}. Once awake and taken off some machines my lung collapsed, Long story short; I was revived twice and very blessed to be here today~.
I was sent home on 1.5 Liters Oxygen and perm disability. Using a Bipap at night as well as diuretics prednisone and anxiety meds. This slowed me waaay down, Once a social Butterfly to isolated mess.
It all started out as just not being able to catch my breath I was just doing dishes at work, I'd sit n rest, alot! {I woke one night choking,unable to breathe}. So after work I went to the ER, My Sats were 82% Respiratory Failure and The X Rays showed many small white cells in both lungs.
Steroid therapy, such as prednisone, is used to treat the LCH lesions. This is a bittersweet approach as well, as I have developed Type 2 Diabetes with it and now on a very slow progressive dose. Now on 38 Units Insulin & Ozempic for high blood sugars. {Update} Discontinued Ozempic ! Side effects were to much for me.
My heart goes out to those who are suffering any lung issues!, It is frightening. God Bless you.
Rene'
CURRENT INFO: Our understanding of the pathogenesis of PLCH is still in its infancy and will require further study of LCs in the context of human tissue and, hopefully, in animal models that reproduce the PLCH phenotype. Presently, smoking cessation, supportive therapy and, in advanced cases, lung transplantation, are the only therapies that can be offered to patients with PLCH. A deeper understanding of the pathogenesis of PLCH will be required before more effective therapies can be developed.
Most days I find myself...
Over thinking, talking to myself the tv the cat LOL
I also found a hobby, I had no idea I was good at, Hand Embroidery.
Given what I know now, I would recommend others...
Besides Eating More Veggies and Moving more, Research your disease and read up, things are always changing and there are always new medications in the works. Never give up Hope !
My life has changed in this way...
Socially mainly but also financially. Having a S l o w e r pace than I like.
When I'm having a bad day, I like to...
Sleep, Luv my naps too. Listening to worship music or soothing Instrumental.
I also fell and created a whole new mess; Now and it is Severe Lumbar Stenosis !, -But I'll save this nightmare for next time ~ Go with God amen