Does anyone have Cold agglutinin disease that has CLL/SLL

[Saratoga]

Cold agglutinin is a rare autioimmunine disease that is seem with people with a lymphoma or leukemia. My husband has SLL with is very similar to CLL but has secondary cold Agglutinin .I have not seen many people with this rare disease and need to get to talk to others.. Many seem to live in Norway with only about 1200 cases in the US.. If anyone has knowledge please respond, your help with many questions we have will be greatly appreciated. The two hospitals and doctors from both saratoga NY. And dana Farber in boston don't know much about it. He is in the hospital as we speak and having trouble trying to get his red blood count stable... Many thanks in advance for your help...

[Ronmar]

I have CLL with-secondary cold agglutinin disease and have undergone FCR chemotherapy in Melbourne Victoria Australia 7 months ago. I am in partial remission at the moment and just waiting for something to happen. Happy to answer any questions u may have. Regards Ron

[Saratoga in reply to Ronmar]

Ron, you are probably the only person I have found. My husband is presently in the hospital and will be out tomorrow. He had to start chemo last month not because of the sll lymphoma but because of cold agglutinin . He had an optic nerve stock in eye about a month ago and doctors in boson advised to start rituxan/bensamustine in order to put the cold agglutinin to rest. Lymphoma was in watch n wait with no pres ent issues. But two weeks after his first treatment, he started getting night sweats, and last week he woke up in middlle of night so weak, rbc dropped to 7.2, passed out and went to ER. He had 102.7 fever, low blood, dehighdrated, etc... Drs felt he got infection due to low resistance from chemo. We are so overwhelmed. Hope you are doing well, what has your experience been with the cold agglutinin, did you find a dr that you feel are knowledgeable. Pks let us know. Wishing you all the best.. Cathy and Mark

[Ronmar]

Hi Cathy and Mark, Here is a little of my history. My cold agglutinin disease was discovered in 2008 so I was on watch and wait until 2012 when I developed another condition called Raynauds disease which then led me to having a bone marrow biopsy which diagnosed the CLL. My haematologist advised that I should have the FCR chemo not because of the CLL but because of the CAD(cold agglutinin disease). While I was having chemo my Dr. told me to basically lock myself away and not to have any contact with people as my immune system would be very low and could easily get an infection. While I was having chemo my rbc dropped to 7.3 so had to have a blood transfusion but all went well after that. Well chemo is no walk in the park but I survived and my Dr. seems to know what to do. My rbc is up around 12.8 and steady at the moment. I have heard there is a drug called Ibrutinib that can control CLL but it costs $100,000 Aust. per year. Like you I could not find anyone with this combination of diseases so I wish you well and keep in contact. Regards Ron

[Ewuraja in reply to Ronmar]

Dear Ron,

I just stumbled across your exchange with Cathy and Mark. My husband has been diagnosed wit cold-agglutinin-disease and has cll/sll .

When he met up with a haematologist he was given three options: sit and wait, chemo but told it would be like trying to kill a mosquito in a house with a cannon ( might not get the mosquito but may demolish the whole house) or a course of steroids. The steroids did not work and had bad side effects, such as confusion, inability to focus, shortness of breath, etc.

The doctor has now suggested chemo, but we don't know the details, as my husband did not want to even talk about it.

Just wondering what your treatment was, what sort of chemo did you have, what if any side effects, etc.

regards, Ewa

[Ronmar in reply to Ewuraja]

Hi Ewa, I will attempt to answer as much as I can as the "chemo" brain does not seem to work as well as it used to. The chemo I had is called FCR which is 6 weeks of chemo over a 6 month period. This was back in 2013 and their are a few more options these days so you have to do research or better still find a haematologist who has experience with this combination of diseases. I was lucky and found one who treats 3 of the 8 known cases in Australia. You have to remember that every case is different and the side effects will differ from case to case. As for me my main side effect was heart burn but with a change in diet and tablets it was controlled to a certain degree. The good news is I am now in full remission and my third anniversary will be in October. I hope this helps and feel free to contact at anytime and ask specific questions and I will try to answer them. As you are probably your husbands carer my wife Maureen would be happy to help with any questions you may have also. I presume you can read the posts between ourselves and Cathy and mark. The golden rule with CAD is stay warm do not eat or drink anything cold. Kind Regards Ron.

[Ewuraja in reply to Ronmar]

Mark, thank you for your response, i just logged on now as we were both sick.

I am glad that the chemo worked for you and you are better.

My husband, we have since been to the doctor who on this occasion said that he would not recommend chemo. So we have had three different ideas from him one after the other, no chemo, chemo, no chemo.

My husbands CLL/SLL was diagnosed only because one of the tests he was sent for was a bone marrow biopsy, to find out why he has the

cold-agglutinin-disease. The leukemia was not showing up in the blood tests because it is at stage 0 or even less.\

The doctor said he will now watch and wait, my husband is to have a blood test once a month and see him once every three months.

Oh, and the way out of the cold-agglutinin-disease is to move to a warmer climate.

He went to his GP today, one of the things he was complaining about is a pain in his leg, she sent him for a scan and it turns out he has a blood clot and she will put him on some blood thinning medication. I don't understand why they did not put him on that in the first place, and am grateful it is in the leg and not the head or on the way to his heart.

any thoughts-Ewa

[Ewuraja in reply to Ewuraja]

so sorry Ron for calling you Mark-ewa

[Ronmar in reply to Ewuraja]

Sorry to hear that both of u have been sick as one at a time is plenty to cope with. In my case I was on watch and wait for 4 years and in my research I found many people had been watching and waiting (worrying) for longer than that. There is a web site for CAD people and none of them mention CLL so it could be that you could still have a long wait. In my case I got to the point where my HB. got so low (around 80-90) that I was so weak and puffed out that it was decided to proceed with the chemo. The other consideration was that as I was turning 66 and relatively healthy i.e. strong enough to take a decent dose of the FCR. They don't often give the high dose of FCR to people over 65. So I guess u have to way up if u proceed whilst u are young enough and healthy enough to take a high dose of chemo which can give u a good result. I did what my haematologist suggested as I trusted him and was sick of walking around feeling like a wet rag. But remember it took 4 years to get to that stage. I don't envy the position u are in at the moment because watch and wait can be very frustrating. I hope this has helped and wish u luck with your decision. Kind Regards Ron.

[Saratoga]

Ron, I am so glad your therpy worked, not sure if you received my prior response. You don't know how much we truly appreciate you reaching out to discuss this. Yes, I feel your pain as there is so little info on cold agglutinin. Mark just got out of the hospital today after contracting and infection 15 days after his first chemo treatment, now I know why you were advised to isolate yourself when getting your treatment . I think you both would greatly benefit in talking with each other. Do you ink we could call you to, mark would like to have a conversation if you would permit us to call you. We don't expect you to make the call we would be happy to contact you on a particular day if possible. It would be interesting and very helpful to both of you.

[Contact details deleted to protect your privacy - Admin]

Please give us your contact info and we can arrange forme both of you to speak.

Wishing you and your family all the best today and always.

Cathy & Mark Libecci

[Ronmar in reply to Saratoga]

Hi again Cathy and Mark, hope Mark is feeling better after a good nights sleep. [Contact details deleted to protect your privacy - Admin] We also believe that if you ring at 5.00 pm your time we should receive the call at 10.00 am our time. Any day would suit us as we are retired and don't have a particularly heavy social calendar. Hope to hear from you soon. Regards Ron.

[Saratoga in reply to Ronmar]

Good morning Maureen, it is so nice hearing from you again as well. Surprised enough mark is still sleeping. I will save your email and phone number, that time of day is perfect for us as well. Let me talk to mark and I will let you know when we will call. Looking forward I to finding out how Ron realized he had cold aggulinitin and the process of treatment..

Background of mark... We are both 55 yrs old.. Used to live in New Jersey.mark played ice hockey for years.. About 5 yrs ago when he came off ice someone noticed his cheeks, ears and nose was black and ble.. Went to several des all said it way raynauds and no big deal. Keep face covered in cold and you will be done. So for yrs whenever we went skiing o very cold weather he would protect himself with a mask and all would be good. Fast forward to this past feb he had a stomach flu and didn't feel right. Went to dr who did blood and we found his RBC was 8.6.. All he felt was very tired we thought job..

Well after several dr and tests, we find out he has SLL lymphoma and cold aggulinitin.

Same as Ron there was no need to treat SLL watch and wait.. But about a month ago, mark had an optic nerve stroke in left eye. Dr strongly recommeded chemo rituxan/ bensamustine twice a month for 4-6 months. In order to put CAD to rest.

July 16 was first treatment seemed to handle very well only tired until last wed we ended up in ER... RBC 7.2 fever 102.7, kidneys failing,5 blood transfusions, blood would go up.. And then he bounced back and is home now... What a nightmare... Looking forward to hearing Ron's story and please let's keep in touch.. The support I have found with you and my be able to compare what des are doing and stories is priceless to me.

Since my husband doesn't go on web sites it's always me talking to people. Mana many thanks.

Cathy

[Ronmar]

Hi Cathy Maureen here Rons wife Like you I think it would be good for mark and Ron to Chat but also I wondered if you had an email address as I would love to have contact with someone else whose partner is going through this I am Rons full time carer and there are so many things need to be done for someone with CAD [Contact details deleted to protect your privacy - Admin]

Maureen Walsh-Martin

[Saratoga]

Maureen, I agree and look forward to speaking with you as well. [Contact details deleted to protect your privacy - Admin]

It is such a learning experience. There is a doctor in Norway that is supposed to be the expert on CAD...I am trying to reach out to the hospital he is with. I will give you all,the info I can. Going to try and get some sleep, it has been a long day since Mark got out of the hospital earlier today. I will save your email and write you tomorrow.

Best regards, cathy