UPDATE ON: Help! 31yr old newly diagnosed CLL.

UPDATE: hi everyone.... Just wanted to post an update on my brother and say thank you to everyone for all the info.... Really helped us have intelligent and productive conversations with our new doctor at Yale today.

We saw the new doc. He was amazing. We spent 2+hrs explaining everything anyone ever wanted to know about CLL. He answered all our questions and I feel my brother now has the information and confidence in his doctor to proceed with treatment. The doctor is reccomending FCR (I believe) chemo. He will have 6 rounds. He ordered a bunch of tests like the FISH and some others ( I forget the names) but he is giving my brother a month to think it over and return back hopefully ready to start treatment which he is reccomending right now bc of his age, and the magnitude of lymph node swelling he has in neck, axilla, and groin. Interestingly, and looking for any feedback from you guys on this. I started doing some digging into our fathers side of the family. We dont know him and have never had a relationship...so I didnt know the family history from that side but felt like I needed to. Anyway, our father (mine and my brothers) apparently has CLL, and his sister (our aunt) also has CLL... But no other members have it. I dont have more details then that right now but I plan to find out more like age of diagnoses (although I believe was 50/60yrs), chromosomal abnormalities etc as soon as I can. I couldnt find a ton on inheritence of CLL just that 10% of people with CLL have some blood cancer in their family. Thought it was odd that both him and his sister have it, and now my brother (at 31yrs old-young for this diagnosis) has it. Unfortunately, I tracked down this info after our apt with his great new doctor today but I will obviously bring this up at the next meeting. Any one with any studies/info/personal history anything on inheritence would be so so appreciated. Again thanks to everyone for all the help thus far!!!

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  • Hi Jade263,

    My father died from Richter's transformation at 83 after 14 years and many different chemo treatments. A few years later I was diagnosed at 51. As they say, "if I'd known then what I know now!" The local oncologist was not the best choice. Having a CLL specialist is a big deal! As I understand it, Cll is not inherited but you are more prone to it. And subsequent generations are affected earlier. I had an older uncle that had Cll but died of a heart attack. I'm the youngest of three siblings and the only one with Cll. I've heard Cll referred to as the "the good cancer..." NOT! But, this is a better time to be diagnosed than 20 years ago! Hoping all the best for you and your brother!

  • My father also had CLL and also died of Richter's. I was diagnosed at 54. Watch and wait for 8 years now.

  • My grandmother died of Waldenstrom's lymphoma and I had atypical CLL with M spike, a bit similar to Waldenstrom.

  • I am so pleased you have found a specialist in CLL, and that he gave you both the time you needed to ask your questions.

    My brother and I have cll but there is another who does not have it.

    I suspect it has come down from my great grandmother and my mother though they were not diagnosed with it due to the era they lived in. My Mum like me was always warmer than everyone else and used to get very tired., My grandmother died with persistant lung infections. Hence my suspecting they had CLL.

    I am so sorry your brother has it at such a young age, however, he should do well on FCR and there are new treatments coming along all the time so his future is looking good.

    Try not to get worried by all the sad stories, there are lots of good ones too.

    Have him eat healthily and exercise when he feels up to it.

    Be strong together,

    Best wishes

    Bubnjay1

  • My grandfather died of CLL in 1964 at age 65 or so...saw his death certificate. I foung this a few weeks after I was diagnosed at age 50 and it send a shiver down my spine! My brother had Hodgkins as a kid and was cured in 1966 or so. No doubt in my mind its genetic for some. There is a genetic study at MDA I believe for which I donated blood .

  • I know it's not CLL or related, but my husband's mother, uncle and aunt all had multiple sclerosis. Another uncle had Parkinson's and only aunt 'escaped'. Oh yes, it's not inherited, just the tendency ... and my husband has scleroderma, another autoimmune disease!

  • I know from my visit to The Ohio State cancer clinic (Dr. Jones) in 2014, that they are doing a study on the relationship between CLL & family relatives who have/had CLL. OSU helped to develop Ibrutinib. My aunt (dad's sister) had CLL and OSU wanted me to be a part of their study. I could not be in it as I lived too far (they are in Columbus, Ohio) away.

  • Jade,

    From the way you posted it sounds like the doctor is preemptively recommending FCR before the FISH test result. Be certain that the FCR protocol is matched to a FISH tested CLL profile that indicates an indolent set of markers that excludes 17p- or 11q- or has 3 or more defective chromosomes making it CK (Complex Karyotype).

    WWW

  • Hi there... Yes it was pre-emptive. He order FISH at visit on Friday and stated plan was FCR as long as FISH came back showing he is a candidate. I forgot which one he said if we couldnt do FCR. Will find out when we go back in a few weeks. Thanks for your reply!!!

  • Jade's mother here ( Lynn ), and it is my son that has the diagnosis. The FISH test came back and he has 13q and 11q deletion ( no 17p ) looks like 40-48% on the 11q and something called ATM with a poor prognosis. In all the research I have done, and we are all doing a tremendous amount, that is what I have seen, FCR is not recommended and CLL does not respond to it well with the 11q deletion.. I would be interested to know more of your thoughts on this. His doctor has already scheduled a pre-op appt to have a port put in.....he is resisting this port and chemo aggressively.. eats healthy, is not tired, hikes about 3 hours a day, what is you experience or knowledge on the FCR and 11q deletion?

  • Hi Lynn,I spoke with your daughter Jade a while ago, I was diagnosed myself last October age 38 and this was very unexpected to me as well. I didn’t have anytime to come to terms with it and had to begin treatment immediately due to severe anaemia caused by this.

    I was fortunate to be accepted on a trial in the U.K. with one of the new targeted therapy treatments (ibrutinib) and has worked extremely well. On my last appointment my blood results were nearly perfect with a very small trace left of the Cll. I was just like your son adamant I didn’t want chemotherapy despite my haematologist insisting it was the so called gold standard.

    Jade did say you’d managed to get to see a specialist, can I ask what treatment plan they discussed with you and why he’d need a port? I was offered this and refused but I only needed it due very low blood count which was a struggle to get a needle in

  • Hi Stuart!!! Thanks for replying to my mom... Idk if she responded... But I actually contacted Dana Farber who is running a familial CLL study with ibrutinib in conjunction with FCR. They told me this before seeing his results so we will have to wait till Tuesday next week to see what they are saying now. I might be mistaken but I am not sure ibrutinib is being offered here as a first line treatment. I believe they will make him try something else first (At least one dose) unless he can get into a study. Dana Farber is doing one now but Yale where we live I think just finished one and isnt offering it. Anyway, I could be wrong or misunderstanding. I will post an update after we see Dana Farber Tuesday. Thanks, as always for the info & help!!

  • While it is true that your son does not have CK defined by 3 or more involved chromosomal defects, the fact that he has an 11q deletion with damaged or missing ATM (Tumor suppressor Gene) is not reassuring that FCR is a good option. My concern would be that FCR will likely kill off the 13q- expressed CLL cells but leave some of the aggressive 11q- expressed cells to reemerge stronger than ever.

    11q- patients typically manifest with bulky nodes and the nodes are where the CLL cells do their dirty work of proliferation and remodeling of the microenvironments in which they live. Clonal evolution is the unknown risk factor in your son’s case that is more likely than if he were just a 13q deleted patient with an IGHV mutated status. If his IGHV status is unmutated (likely) the risk of short remission is even greater.

    Kinases inhibitors like Ibrutinib work very well for the vast majority of patients and specifically in clearing the nodes of disease. Ibrutinib now has a stellar track record extending into the 7th year of use for some patients. I have been on Ibru for 6yrs and 3months.

    In strategizing for the future it may prove very important to avoid the known toxicity and likelihood of short remission using FCR vs the better tolerated but longterm use of a kinase inhibitor like Ibrutinib. Ibrutinib appears to work even better in treatment naive patients than those beat up by prior chemo.

    I am not medically trained or in any way credentialed to give medical advice so take this post as a layperson’s perspective.

    WWW

  • Hi there, thanks so much for your reply and in depth information. We are actually not sure if they are still reccomending FCR. His doc had mentioned FCR before his FISH results were back and so we are waiting to hear what the final say on treatment is when we go the first week in October. He is also getting a second opinion from Dana Farber cancer institute on Tuesday next week. They are running a familial CLL study and mentioned using ibrutinib in conjunction with FCR... This was again before they had seen his FISH results. Right now we are just waiting to see what they will suggest when we go.... But my understanding is here in the US patients cannot just go on Ibrutinib first line... They need to have at least one dose of another medication first.... I could be mistaken though. At any rate thank you so much for the information thanks to all of you we will be well informed when we see his docs next. Appreciate the help!

  • It is argued by some CLL experts that FCR is potentially curative in some patient profiles with defined indolent CLL. In that scenario a patient receiving 6 cycles of FCR is done with TX in 6months.

    With Ibrutinib there is no expectation that as a monotherapy it will result in a cure although many get deepening remissions the longer they are on the drug (my experience). In a desire to achieve a "cure" or at least a durable remission the idea of adding a second or third agent is being tried out. Ibrutinib can certainly be used frontline without a 2nd agent. One gentleman I met at OSU has been on Ibrutinib as a frontline therapy for very close to 7 years with zero side effects and doing extremely well.

    Generally speaking it is assumed by most that a combination of drugs will be needed to cure CLL or any cancer for that matter.

    My preference would be to avoid the toxicity of FCR to wait for a combo of targeted therapies.

    WWW

  • Jade

    Ibrutinib is FDA approved firstline in the U.S. and has been for sometime...

    You have been misinformed...

    Link

    janssen.com/us-fda-approves...

  • Thank you I must have misunderstood what his new doc was saying. Thanks for the link!

  • There are many papers on Familial CLL. My sister and I both have CLL, but I was diagnosed 18 years before her.. we are only 4 years apart... I'm also a Richter's patient.. but familal links and studies in this area are non-existent.

    Look at the work of Goldin and Susan Slager, Richard Houlston etc.

    The watershed study was done just after WW2 and is refered to as Pedagree 14.

    Also the Bronx study from the 1950s saw the first correlation between Famillial CLL and Ashkenazi Jews from central Europe.

    There are many studies from the Nordic CLL Group and Hovon...since they have the best and most complete databases on blood cancer, often going back to the 1930s... look for studies from Sweden and Denmark... Upsalla University does a lot of research in ths area...

    It all on PubMed...

    This topic comes up frequently on Facebook, and it is quite surprising how many CLL patients have a family history if not of CLL, then of a lymphomas, usually, Burkett's, Follicular, WM or some other NHL.

    My feeling is that the 10% figure is low, probably 15-20% in actuality... but still no one gene has been found, but we have over 20 very possible candidates.

    ~chris

  • Thank you Chris. I will look for those older studies bc I found recent data to be lacking... Maybe 5 studies I found and they werent super consistent. Anyway, thank you! One more question i saw you talking about and now I cant remeber if I asked or not. I forgot to talk to my brothers doc about the ECGC studies at mayo that you guys helped pay to support. I am wondering if he can take the green tea extract just normal dosing while he waits the month to go back. I already told him he shouldn't be taking the doses I saw some taking like 800mg because it can cause the liver function increase. I also told him he needs to call his doc to discuss next week and make sure its ok. I'm just curious if anyone has any effect from a dose of 250-500mg (of actual EGCG) I know this dose is usually tolerated ok. I read the studies, seems like CLL patients were doing this while watch and wait status? Or in between treatments... Am I mistaken? Was this done in place of any traditional treatments? I am really hoping my brother will stick with the plan to start treatment as the doc reccomended in one month. I am just curious what type of patients were using the ECGC. His lymph nodes in his armpits are about the size of a baseball... His neck lymph nodes are very large also but maybe slightly smaller than the baseball and give him pain when he opens his mouth. I am just curious if this might help a bit.

  • EGCG ...you can't buy the clinical trial purity and over the counter extracts vary in dose and quality...

    Its use is no longer recommended by Dr. Neil Kay, who ran the trial, simply because nobody knows what's in these OTC capsules and tea from China and India are known to have high levels of banned pesticides...

    Further, the Mayo study was done on very early Stage 0,1 CLL and it showed some effect in about 60% of patients. It sounds like your brother is perhaps stage 3/4.

    The dose of Polyphenon E was 2000mg twice a day, and you need liver monitoring.

    Certainly some early stage patients feel it has helped them, but how much is unknown.

    If he decides to try it, approval from his doctor would be wise.

    ~chris

  • Thank you Chris exactly what I was looking for .... As always appreciate your help and knowledge!

  • Just a quick question I've been wondering, has any ever tried or looked into the possibilities of nettle tea and Cll, it has some very strong properties but also needs to be approached with caution as like anything natural can also cause some side effects.

  • I have never seen anything with CLL and nettle tea... there have been a number of things tried, gossypol from cottonseeds [AT-100], resveratrol from grapes, PIETC from watercress, silversterol from mahogany bark, neem tree oil, curcumin ... citrus pectin-derived galectin-3 inhibitor GCS-100...

    They have shown some effect but never enough to get too excited about. They have been considered in the between treatment 'maintenance' setting but not really used.

    hematologyandoncology.net/f...

    Probably the closest was gossypol it had a clinical trial, as did resveratrol as an adjunct to fludarabine...etc.

    ~chris

  • Thanks Chris

  • I experimented with EGCG and concur with what Chris has written. I would add that in my case I used a combination of oral capsules and lozenge EGCG which I began at 250mg per day and titrated to 1,100mg per day at which time I experienced liver pain and elevated liver enzymes. Pain and enzymes resolved after stopping.

    Charting the ALC (Absolute Lymphocyte count) It showed no benefit over an 8 month period. It should be noted that small leveling out shifts in ALC or even downward trends are part of the natural course of CLL and can appear in the short term to be a result of whatever alternative therapy one might be taking. This is why I waited 6 months to gage the disease progression before starting EGCG. The following eight months of EGCG use showed increased rate of disease progression. I attempted to buy the best quality EGCG but as Chris pointed out there is no assurance of consistency or purity.

    I well understand the compelling need to do something but as one data point in a pool of patients my attempts only depleted my wallet and did zero to slow the dance of my CLL Bear.

    WWW

  • My husband was diagnosed with CLL in 2013, 8 years to the day after his older sister was diagnosed with ALL.

    My husband has p53 and 17 deletions. Has 2 failed chemos and no richter transformation. He's just begun Ibrutinib treatment. Had his 3rd dose last night.

    His sister died in 2007 at age 59.

    My husband is 66 and has been treated for 4 and a half years now.

    I pray to God this doesn't show up in my children or grandchildren.

    Sincerely,

    Farrpottery

  • Hi there.... Im so sorry you have had such a terrible history with this condition. I hope your husband will have better luck with this treatment, and I too hope your children and grandchildren dont suffer also. My brother too is worried now because he was planning on having children. Im scared too that if my brother, my father, my aunt all have it I must too have a liklyhood. Anyway, I guess its best to not worry about things that havent happened yet.. Or at least try our best to not worry too much... It is hard to not I know. Anyway, thank you so much again for your reply and praying for positive news in your family moving forward!

  • Jade - one question your brother should ask is about the effect of his treatment on fertility. Depending on the treatment he may want to consider banking his sperm.

    My extended family has other lymphomas and many other cancers. I am one of six and the only one with cancer. My cousin - one of 4 in her family had breast cancer. In the third family the father had multiple myeloma, two sons have follicular lymphoma, ond one of their sons had AML. We have developed a family health history so that everyone, including future generations, is aware, but beyond that we don't look for trouble.

  • Can anyone help me search the site for any information on rashes with Ibrutinib?

    My husband is in his 3rd wk at 2 pills nightly. He's been doing great!

    More energy, no fatigue, spleens reduced in size, legs not swollen anymore...

    But he's beginning to develop a rash on his face and neck.

    Sincerely appreciate any help

    Farrpottery

  • Tell your brother to have his children and enjoy every moment of them. If we are going to have cancer, this is the most manageable and watch and wait kind to have.

    The one thing this HAS done is brought the entire family closer together.

    I think a lot of what we have been exposed to during our lives and what we eat makes s difference.

    My husbands Veterinary business had him exposed to dips and things no longer on the market because they cause cancer.

    Now they're linking duet Coke to leukemia in men. Aspartame is toxic. All the artificial sweeteners are toxic.

    How many of us ran and played in bug fogger machines in the 60's for mosquitos. They dropped murex on us to kill fire ants. It was carcinogen too. How many of our guys were exposed to agent orange during Vietnam.

    Please have your families and save the cord blood. Save the baby teeth. These are being used to get stem cells for cancer killers that attack the cancer cells only.

    God bless you and your family. Enjoy every day because we aren't promised tomorrow. None of us are.

    Much love to you and yours ,

    Farrpottery

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