New to all this!

[LAinNYC]

My husband was diagnosed at age 59 and has been "watch and wait" for 3 years...he was recently told his spleen is enlarged (no other lab results have been made available to us yet) and is likely to need treatment soon. I've been catching up on all the research I can so we can make sense of any recommendation that's made and try to advocate for the best approaches possible. It seems at least in the U.S. that clinical trial options are mainly focused on people older than 65 who've relapsed or been refractory following chemo-immunotherapy, and the newer targeted therapies won't be available as front-line therapies for a few years. Terrifying!

[Cllcanada Top Poster CURE Hero]

Very true for ibrutinib and idelalisib ... but there are many new drugs in clinical trials for CLL... ibrutnib isn't the only small molecule...

However, ibrutinib 17p/TP53 deleted should be FDA approved next spring, then for others as phase 3 data matures...Wall Street says commercialization in early 2016... but just a guess...

These guys have a timeline you can view...

seekingalpha.com/article/12...

Then there are some who feel ibrutinib cost will be an issue...

~chris

[AussieNeil Administrator]

I take it that you are in the US, in which case you are in the best place in the world when it comes to opportunities to enter trials - which doesn't necessarily mean that a trial is the best option for your husband.

To save you and your husband possibly unnecessary worry, I recommend you do at least the following:

1) Get all the facts from whoever is assessing your husband as to why he is likely to need treatment soon.

2) If your husband is not seeing a CLL specialist, make sure he sees one for a second opinion. (Again in the US, you are spoilt for choice, though I appreciate travel and medical coverage may be an issue.)

3) Share what you've been told here - feedback from others should help you with the big decision on how to proceed.

Many of us are living with enlarged spleens and are still on watch and wait (in my case over 4 years). There are many factors that go into deciding when to start treatment. With the spleen it comes down to whether the enlargement is causing any problems due to its size or its effect on the different blood cell lines. Having the wrong treatment, starting treatment too early and starting treatment too late are all dangers you need to guard against.

While many of us are hanging out for safer, gentler treatments, waiting too long for them to arrive can be more dangerous than going with a proven and readily available chemo-immunotherapy option. Take some comfort in why your husband doesn't qualify for trials currently open - the drug companies are purposefully selecting out that part of the CLL population that hopefully will do better on the newer therapies. Turning that around, your husband is likely to do well on existing therapies.

Given treatment may be on the horizon, I'd recommend your husband use this time to improve his health and fitness - there's evidence that fitter people handle treatment better.

Neil

[LAinNYC]

Such a relief to be able to post about this...with the test results 3 years ago (13p deletion, mutated IgVH) I (foolishly?) was able to put CLL mostly out of my mind, so we were both unprepared for any of this. It's really helpful to hear that an enlarged spleen by itself doesn't necessarily require treatment. My husband is not prone to getting sick with colds, flu, etc and is very fit, so we're doing all we can to maintain what we can control. Our health insurance is good for another 17 months and our plan is to purchase new insurance through the exchanges coming on-line in January...and I've read that clinical trials will have to be covered under the new law. In the meantime, he's asked me to go with him when he sees his hematologist next month, and I'm planning to go with lots of questions!! Including which CLL specialist to consult with as a next step...

[lankisterguy Volunteer in reply to LAinNYC]

Hello LAinNYC,

If your screen name is any indication that you are in New York City, I hope you will immediately make an appointment with a NY based CLL specialist for a second opinion.

You do not need permission or a referral in most cases (unless you are in an HMO that requires referrals). My own favorite is Dr. Richard Furman at Weill Cornell NY Presbyterian 525 East 68th St., Payson Pavilion, 3, New York, NY 10065 [phone] FAX[phone], Dr. Furman is the medical advisor of a similar online group to this one and is an excellent communicator and diagnostician. You can call the phone number and make an appointment through Rachel, his secretary and see him next week if you choose. They will bill your insurance directly.

Your husband has a legal right to have copies of all tests and medical history records, you should request copies and have them available to share with other doctors. You can be a proactive advocate for your husband and both of you should consider your doctors as consultants you employ at will, so treat them as such. Expect & demand to be informed and involved in all decisions.

Clinical trials have a complex reimbursement system that can be explained by the trial staff, but in most cases current insurance plans will pay for the parts that are medically necessary and the study sponsor pays for the remainder.

Len

[LAinNYC in reply to lankisterguy]

Thanks, Len, Dr. Furman's name keeps coming up, so it's good to get another positive recommendation. I'm still finding my way to being supportive without trying to take over the choices, decisions, etc. As much as I can!!

[Quarry]

Hi

I might be in similar situation (53, diagnosed last New Year) and main problem is enlarged spleen (no other lymph enlarged). My bloods are ok, though haemoglobin down at 11 (not a huge issue). Spleen enlargement was increasing (cause by it destroying red cells, but compensated by bone marrow producing more reds, so overall more or less stable....at least a good sign that narrow is healthy)

As enlargement doubled over last 3 months (to over 8 inches by a very non scientific method), we decided to treat. First treatment is high level of steroids (80mg/day, so it is v high). Can be a lot of side-effects, but only major problem for me so far is I can not sleep! Perhaps I am lucky re side effects, or maybe a young body copes better.

Good news is after 3 weeks, spleen enlargement has halved: indeed haemoglobin is up to over 13 too. So steroids doing their job. I have another 3 weeks on the high dose, before I think a further 10+ gradually reducing the dose - then hopefully my body will re-start its own steroid production.

This treatment has a 70% chance of working long term (so key for me is 6 to 12 months time...). It appears being young helps cope with the steroids / ?will it help long term in stopping spleen growth coming back I don't know. Indeed, I am not sure why the steroids work - they must be causing a switch to flick somewhere to stop red blood destruction (assuming the CLL cells somehow caused the 'switch' to move to 'destroy')- it is a question I have for haematologist next meeting.

If the growth comes back, second line is some form of antibiotics, but my haematologist is not really optimistic they will work (if steroids don't work, you are in a 'difficult to treat' category). The only option then is to have the spleen removed.....

There is a great paper re Treatment Guidelines for CLL somewhere on this site (written for haematologists, but I think should be compulsory reading for GPs too for their awareness), and I have it: but can not see how to attach to this entry! However, if spleen enlargement is your husband's only problem, he should be in similar path to me I guess.

Timing of treatment is personal/discussion with haemotologist: there is no set spleen size to start.

Hope this helps

Andy

[AussieNeil Administrator in reply to Quarry]

Thanks Andy for that informative response on what is being done to manage your enlarged spleen - I trust you'll continue to do well.

I think the link below is what you are after?

Guidelines for the diagnosis and treatment of CLL

bloodjournal.hematologylibr...

Neil

[Quarry]

Neil

It is not the one I was thinking about, but a good article none-the-less! Thank you

One I had in mind is a British Journal of Haemo article.....Details are:

Guidelines on the diagnosis, investigation and management of chronic lymphocytic leukaemia

David Oscier,1 Claire Dearden,2 Efrem Erem,3 Christopher Fegan,4 George Follows,5 Peter Hillmen,6 Tim Illidge,7 Estella Matutes,8 Don W. Milligan,9 Andrew Pettitt,10 Anna Schuh11 and Jennifer Wimperis12, Writing group: On behalf of the British Committee for Standards in Haematology

ª 2012 Blackwell Publishing Ltd First published online 11 October 2012

British Journal of Haematology, 2012, 159, 541–564 doi:10.1111/bjh.12067

I have pdf, just can't work out how to get into this comment box! If you can provide guidance on how to overcome my IT incompetence (copy/paste does not work), I will paste in!

Andy

[AussieNeil Administrator in reply to Quarry]

Andy,

It's not you, it's this website. It won't allow you to add attachments, but it WILL enable you to add in a link to the source elsewhere. Just remember that if you need to copy a link from elsewhere in this site, because the link is abbreviated, you have to capture the hidden link. In Firefox, you right click the link and select 'Copy Link Location', then paste. Here's what you were after I believe:

Updated guidelines on the diagnosis, investigation and management of Chronic Lymphocytic Leukaemia, published as early view on-line on the 11th October 2012 in the British Journal of Haematology.

onlinelibrary.wiley.com/doi...

[Quarry]

Neil

Thanks and this is the article! I had help in understanding the article from a GP friend, who also learnt a lot re CLL, which in turn has helped them understand/support their CLL patients better! I am lucky as my haematologist is one of the authors

Flow diagram on P 554 is relevant to discussion above. It reminds me, I have NOT had a bone marrow aspirate 'to confirm autoimmune' bit (and so despite what article says it is not essential at this stage as a good haematologist can pick it up from bloods/other symptoms)........

However, I have been warned I won't escape the marrow aspirate for ever (I think more linked to CLL development rather than autoimmune bit)......and that the procedure is not without serious discomfort.....(which is why she is keen to delay procedure until really necessary to future treatment option decisions)

Andy

[AussieNeil Administrator in reply to Quarry]

Your comment on the bone marrow biopsy procedure along with a recent discussion on the CLL/SLL yahoo group has prompted me to ask this community what their experiences have been. Here's your chance to find out where to go/who to request for the least discomfort!

Neil

[LAinNYC]

We got good news yesterday, husband's labs were stable enough to remain on watch & wait, he'll re-do bloodwork at the end of the month, & then onto a CLL specialist.

[AussieNeil Administrator in reply to LAinNYC]

That IS good news! And now you know more of what to ask when you meet the CLL specialist. Later still, you'll have many more facts plus some advice from someone who should know what they all mean. If you do end up seeing Dr Furman, you can get a feel for his expertise and style by checking out his videos. Search for "dr richard furman weill cornell video" if you haven't already.

Neil

[LAinNYC]

I'll definitely read the articles to help prepare; also, if anyone has resources re: nutrition, I haven't found well-researched material.

[AussieNeil Administrator in reply to LAinNYC]

I recommend you ask a separate question about nutrition, but be prepared for plenty of anecdotal comments. There are the occasional papers on the effect of known biologically active extracts on CLL, but you can appreciate the challenges of doing a proper trial given the difficulties of providing a known strength and dose of what's under trial. Eating a healthy, balanced diet incorporating a wide range of foods and tread warily about eating high concentrations of anything that is touted as boosting your immune system is a good starting point.

[AussieNeil Administrator]

Regarding nutrition, check out this information from CLLSA's website:

cllsupport.org.uk/eatingwel...

[LAinNYC]

Still getting used to the new site/format; I posted an update that my husband is now in the hospital for an infusion; new symptom of fatigue, and hemoglobin at 6.4, so his doc wanted him admitted immediately and infused for fear of a cardiac "event"...we'll meet again with him tomorrow, and the "watch and wait" recommendation may be over. Also learned that his igvh is unmutated, so I'll look into getting more specific info about the implications of that marker on any treatment recommendation. Was a bit dismayed by the response to my "second opinion" request, but part of my job as patient advocate is not to take "no" for an answer if it seems wrong. Onward....