Adult Langerhans cell Histiocytosis and CMML

Posted by cblowers1 @cblowers1, Feb 27, 2020

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?

Interested in more discussions like this? Go to the Blood Cancers & Disorders Support Group.

Colleen Young, Connect Director | @colleenyoung | Oct 13, 2021

In reply to @christina67 "Actually he is feeling very good. This all started with an severe all over itch. We..." + (show)

Hi Christina, apologies for the delay in responding. You’re asking all the right questions and I hope you’ve received answers from experts in the meantime. Chemo drugs are mostly associated with cancer treatment, but can be helpful with other conditions, including auto-immune conditions. @becsbuddy and @loribmt may be able to tell you more about this.

Have you already contacted Mayo Clinic for a second option? They will walk you through the procedure of sending test results and medical records before your initial consult.

christina67 | @christina67 | Oct 14, 2021

I have spoken with them, but they are waiting for him to call and schedule something. He has his own business so it is not easy for him to get away for a week and we are quite a distance away. I am afraid that he feels so good that he is dragging his feet and a bit in denial of his possible diagnosis. Hoping to get something scheduled soon.

annettew | @annettew | Dec 30, 2021

I was diagnosed with LCH in the lungs in 2014. We did talk about transplants but not a great candidate for that for multiple reasons. Nobody ever mentioned bone marrow transplanting. Is there any research in bone marrow transplanting in with PLCH ?

Lori, Volunteer Mentor | @loribmt | Dec 30, 2021

In reply to @annettew "I was diagnosed with LCH in the lungs in 2014. We did talk about transplants but..." + (show)

Hi @annettew From my understanding, a stem cell transplant is not option for treating Pulmonary Langerhans Cell Histiocytosis. Since it’s centralized in the lungs it would require a lung transplant. It is a possibility when LCH is occurring in the bone marrow.
https://foundation.chestnet.org/lung-health-a-z/pulmonary-langerhans-cell-histiocytosis/?Item=Treatment
Are you currently in treatment for PLCH?

hearttoheart1 | @hearttoheart1 | Dec 31, 2021

New Year’s Eve 1986 Michael’s tragic accident
September diagnosed with esophigeal cancer
So sad
He is no longer here since his death on 03/12/2021
Thank you for your support and kindness to me over the year
So appreciated
Michael greatly missed😔
Happy new year to you all xx

Lori, Volunteer Mentor | @loribmt | Dec 31, 2021

In reply to @hearttoheart1 "New Year’s Eve 1986 Michael’s tragic accident September diagnosed with esophigeal cancer So sad He is..." + (show)

Thinking of you today, Miss Heart.

You’ve had a very trying year and filled with much tragedy.
I’m praying this next year brings a renewed peace and allows you to find some comfort in the memories of your amazing life with Michael. You both were through so much together.
That love endures.
Every time you feel a breeze, cool and uplifting, or warm and comforting, it’s the energy of Michael wrapping you in his love.
Each time you see a butterfly in his green house or on the flowers he’s cultivated, it’s the sprit of Michael winging its way to you.
The song of the Cardinal or a Robin in the gardens will be a signal from him that he is with you, my dear. His spirit is all around you.

And all of us in this wonderful Connect family are with you too. Please stay in touch with us.
When you’re ready there is a very supportive Loss & Grief group. We all need to feel that we’re not alone and this group is so caring and loving.
https://connect.mayoclinic.org/group/loss-grief/

hearttoheart1 | @hearttoheart1 | Jan 2, 2022

Your comments are so kind and inspirational
We may be very far aapart but your closeness is very near x💖
Thank you

Lori, Volunteer Mentor | @loribmt | Feb 2, 2022

In reply to @hearttoheart1 "Your comments are so kind and inspirational We may be very far aapart but your closeness..." + (show)

Good morning, Miss Heart. I’m was sitting outside, watching the sun bouncing off the waves of the gulf when a feather drifted into my view and landed on the balcony in front of me. When I was a little kid, I heard that a feather is the sign of a loved one sending us a message.
It instantly made me think of you today. I’m so sorry for your loss and want to know if you’re coping ok. I can imagine right now that you’re just going through the motions of trying to gain some normalcy to your life again. Are you doing ok?

hearttoheart1 | @hearttoheart1 | Feb 2, 2022

In reply to @loribmt "Good morning, Miss Heart. I’m was sitting outside, watching the sun bouncing off the waves of..." + (show)

How kind of you to think of me
I am not great today and yesterday
I had srarted to walk to the grave last week which I find comforting
I can get my walk done and it gives me a purpose to change the flowers and I like keeping the grave nice with fresh flowers
But I’m just so sad now,today
I have been busy doing lots of paperwork , finalising funeral payments etc
But he is greatly missed
I have decided to do a flower arranging course that starts online in March
I could then do my own flowers for my home and the grave
But today is a bad day
I’m doing my best
Knowing you are thinking of me so far away brings me comfort
Someone dear to you is looking after you today, with your little white feather🙏
Thank you 😘

Lori, Volunteer Mentor | @loribmt | Feb 2, 2022

In reply to @hearttoheart1 "How kind of you to think of me I am not great today and yesterday I..." + (show)

My dear friend, some days it’s all we can do to get through 5 minutes at a time. You’re doing amazingly well, I think. I love that you’re visiting Michael and making sure his flowers are fresh. Gardening meant so much to both of you and now it brings you a special comfort.
It’s really great that you’re going to take a flower arranging course. That will spark the creative juices and propel you forward. I think it’s important for you to get Michael’s greenhouse full of little green shoots, plants and flowers. It will symbolize new life and a personal growth while still being close to Michael. I have a feeling this will be a very quiet and very wonderful sanctuary for you. I’d love to see a picture someday. 🙂

And walking, well that’s been your solace for quite a while. It was just a little over a year ago when you first became a member in Connect and we talked for the first time. You’ve come so far since then and walking was an integral part of your recovery and control. I walk twice daily and feel down if I miss a day. It’s a way for me to focus and feel centered. I know it’s the same for you.

I do hope you know I’m here anytime you need to talk. I know I’ve mentioned it before but here’s the link again for our grief and loss support group. Sometimes it helps to talk with someone who is going through the same thing. https://connect.mayoclinic.org/group/loss-grief/

I found this today and it felt timely.
“Even now, as broken as you may feel, you are still so strong.
There’s something to be said for how you hold yourself together
and keep moving, even though you feel like shattering.
Don’t stop. This is your healing.
It doesn’t have to be pretty, or graceful.
You just have to keep going.” Maxwell Diawuoh

Sending you a gentle hug today. ♥️

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