An Introduction and Guide for the Newly Diagnosed Patient
A CLL Primer
Here is the scenario. You have just been diagnosed with this nasty stuff called CLL, probably following a routine blood test. After you got over the initial shock of it, you logged on the web, looking for information and support, and you found our site.
At this site you will find people who care, people who are well informed, who share information and feel they are stronger because they work together as a group for the common goal of improving therapy options for CLL patients. This is a strictly volunteer effort by people with no ties to any medical or pharmaceutical establishments.
We try to stay pretty much on topic in our discussions, not much chit chat or off-topic conversation, and the bias is definitely towards patient empowerment, the latest developments, new clinical trials and critical evaluation of the results of such trials. We also tend to be pretty hard nosed about the miracle cures, some touted in TV infomercials, that claim to cure everything except the condition of your wallet.
Below is a quick "cheat sheet" of stuff you probably need to know right away, as you begin to get your arms around this crisis in your health. As time goes on, you will learn more, and feel more confident in the choices you make. With the progress of the genomic revolution, the science of immunology and our understanding of CLL are changing so fast that many of the less informed oncologists and hematologists are getting left behind. No one has a higher stake in this matter than you do - it is your life, your body, your health. I am absolutely convinced active participation and understanding of this disease and all of the available options open to you can make the difference between good health and quality of life for many years, versus poor choices that may cost you dearly. This is what makes patient education and empowerment so important for this disease.
Some oncologists will hand out that guff about CLL being an "old man's disease" and "the good cancer to have", when they first break the bad news to you. That is a load of rubbish, but look at the bright side: you do have a little time to get your bearings, learn more than you have ever wanted to know about blood cancers and, with any kind of luck, this supposedly "incurable" cancer may well be cured in time to get this monkey off your back.
Step One: It is absolutely essential that you get an accurate diagnosis.
Chronic Lymphocytic Leukemia (CLL) is characterized by an uncontrolled accumulation of large numbers of white blood cells (specifically, B-cells) in your blood, bone marrow, lymph nodes and other organs. There are also other blood cancers, kissing cousins of CLL, so to speak, that also have the same basic pattern, for example Mantle Cell Leukemia (MCL), Pro-Lymphocytic Leukemia (PLL), various varieties of Non-Hodgkin's Lymphoma (NHL). Some of these are much more aggressive cancers, and may need more immediate attention, others less so. On a personal note, when my husband was first diagnosed after a routine blood test, the local oncologist told us he had advanced NHL, life expectancy of about four years if we were lucky and that we should start chemotherapy right away. Wrong on all counts. As it turns out, he has early stage CLL, relatively benign form. Two years after his diagnosis, he is now in better health than he was before the diagnosis, probably because he now takes better care of himself after this wake-up call. We fully hope he will die of ripe old age some day.
I cannot overemphasize this point: the first and most important step is to get a rock-solid diagnosis of your situation. Get a second or third opinion, as many as it takes until you are sure the job is done right, and you know the exact name of your enemy. If you need references to oncologists who specialize in CLL, there is a group of doctors in some of the best cancer centers in the country, that have formed a group called "The CLL Research Consortium". You might want to get in touch with one of these experts.
Step Two: CLL comes in many flavors, it is important to know which one you have.
Some of the recent research is focusing on the fact that not all CLL patients have the exact same type of disease. Some have what has come to be labeled as "smoldering CLL". These are patients who clearly have CLL, but it is of a variety that will not do much of anything for a long time. For these people, the best choice may be no therapy at all, or if they choose therapy, it should probably be one of the newer, non-chemo variety, perhaps an immunotherapy regime with monoclonal antibodies like Rituxan, or one of the new vaccine trials. The idea is that it does not pay to treat patients with aggressive therapy if they are not likely to need it in the first place!! Other patients, not as lucky, may have a more aggressive form of CLL and may need to be monitored more closely and may receive therapy sooner than the first group. Doing too little in this situation could be as dangerous as doing too much in the former case.
Some of these words, immunotherapy, Rituxan, monoclonals, etc. might sound like so much gibberish to you right now. Not to worry, you will learn more about these things as time goes on, and I promise they won't seem so strange in a little while. The point to take away from this section is this: after making sure you have CLL, it is important to know what variety of CLL you have. As you can see, it is not a good idea to treat a "smoldering" CLL patient with aggressive chemotherapy, treatment that may be no walk in the park and may also cause some harm to the rest of the functioning of the body. On the other hand, you would not want to ignore or neglect a more aggressive form of the disease that needs to be taken care of. One of the biggest emotional hurdles faced by cancer patients is getting a good reality check on their specific situation.
Step Three: Get the right diagnostic tests done.
This is where it gets complicated, and there is no way to avoid the jargon.
The first test ordered is usually a blood test, called a CBC (Complete Blood Counts), which looks at the numbers of the various kinds of cells in your blood. In addition to the CBC, the doctor may also order a blood electrolytes test, to make sure your kidneys and liver are functioning well, the electrolytes are in good balance. Less frequently, and perhaps only if you are getting frequent infections, an immunoglobulins test may be ordered, to measure the level of these important infection fighting proteins in your blood. Reading one of these lab reports is not easy since they are loaded with acronyms that make no sense. You might want to click your way to the section of this website titled Your Charts to see what these test mean. The Excel workbook named Records.xls provides six worksheets containing tables and charts to help track your medical data. Each of the table headings are commented: as you slide your cursor over them, you get a brief description of the term and what it means. You can download these Microsoft Excel based chart templates, they will make it easier for you to track and monitor your test results.
The CBC may tell you and your doctor only that you have elevated numbers of lymphocytes (B-cells) in you blood, seen as high "WBC" (White Blood Count), and higher than normal percentage of these lymphocytes in your blood. This could easily be due to a simple infection. In fact every time you come down with a bug of some sort, your WBC will ramp up because your body is marshalling its disease fighting armies (of which B-cells are an important part). An increased WBC by itself is not an indication of cancer. You and your doctor will have to look at finer details to make that determination.
One of the blood tests that is done to make this determination is something called a "flow cytometry". This tests looks at the lymphocytes in more detail, specifically B-cells, since we are talking about B-cell CLL. B-cells that are malignant, whether they be CLL or some other type of blood cancer, have very distinct sets of markers on them, markers that are not present on normal cells, or not present at the same levels, or in the same combinations. These markers are called "cluster designations" or "CDs". There are more than two hundred of these CD markers that have been identified and studied.
One of the things the flow cytometry looks for is the level to which your B-cells have the classic pattern of CD5, CD19 and CD23 markers, since this combination of markers is a seen in all CLL patients. The flow cytometry will also look for other markers that are indicative of some of the other types of B-cell malignancies we mentioned above, in order to make sure it is not one of these close cousins. The results of the tests for the CD markers are sometimes reported as "dim", "moderate", or "bright". Other labs may use the notation of "1+", "2+" or "3+" for saying the same thing. The comments on the spreadsheet headings in Your Charts give you some more information to understand this part.
Sometimes the doctor may ask for a CAT scan or PET scan, if there is concern that some of the internal lymph nodes are large, or placed such that they may threaten important organs or squeeze shut major blood vessels. These scans can also show if any of the other organs, especially the liver and spleen are presently involved. I personally do not like getting tests done that I do not need, so you might want to ask the doctor to explain to you why these scans are being requested. Besides being expensive (it does not matter who pays for it, you or your insurance company, in the final analysis the only ones who pay for everything are the patients and taxpayers - you) some of these tests can be quite a drag and who needs the additional radiation exposure if they serve no real purpose in your case. In any case, it is an excellent idea for you and your doctor to get used to the idea of you asking questions first.
In my husband's case, the local oncologist also ordered a lymph node biopsy. This is a minor surgery, but nevertheless surgery that needs general anesthesia, and significant discomfort later as the incision heals - besides being expensive. The expert we saw at M. D. Anderson later on was aghast that the biopsy was ordered so lightly, since there was absolutely no need for it and nothing could be learned from his lymph node biopsy sample that could not have been learned more easily from a blood sample. I guess the moral is this: you need to be pro-active in asking why specific tests are being done, and equally assertive in asking for other tests that may not get done if you stay passive.
In addition to the blood tests, and perhaps scans, another test that is sometimes requested by oncologists is a bone marrow biopsy (BMB). There are two schools of thought on this one. Some CLL experts swear by this test, since it gives them a clear look at the bone marrow, the most important site of the disease for CLL patients. Other experts feel that blood based tests are now so sophisticated, it is not necessary to go through the more expensive, somewhat more painful, and certainly more complex bone marrow biopsy. Our expert at M. D. Anderson felt a bone marrow biopsy early in the diagnosis stage would give a reference point to compare to later on, sort of like getting your first breast mammogram or colonoscopy when you turn 50. If you get the BMB done at one of the consortium centers, where they do literally dozens of them every day, the technicians are experts at doing it with little or no discomfort. Really, the description of the process of obtaining a sample of bone marrow sounds a lot worse than it is in reality.
In addition to the qualitative CD marker report we discussed above, your oncologist may also order a more thorough quantitative flow cytometry, including looking for some of the prognostic indicators that will give him and you a better understanding of your specific situation. You can find a wealth of information on many of these prognostic indicators (CD38, beta-2-microglobulin, IgVH gene mutation status, CD23, FISH, chromosomal aberrations, etc.) both on our website and our discussion group. If your oncologist is not the type that is inclined to order these more expensive tests, I suggest you get yourself up to speed quickly on what they mean, so that you have a good set of arguments to present to him/her as to why these tests are both necessary and prudent. Without this prognostic information, and a reasonable understanding of what these prognostic indicators mean, it will be difficult for you to make the right therapy choices.
Right now, many of these terms don't make a lot of sense to you. But keep up the process of learning, and they will begin to make sense to you. We all started at the same point, mind going blank when the oncologist said the fateful words for the first time, "you have CLL". What the heck is CLL? Never heard of it! was my first response. Many of us have now traveled the same path that you will, in the months and years to come, to the place where we now feel a lot more comfortable with the jargon, and a lot more secure in the choices we make for our own healthcare. Here is a useful link to an "FAQ" maintained by the ACOR organization: http://cll.acor.org/cllfaq/cover.html. It gives short and sweet answers to many of the questions you may have right now.
Step Four: What to do while you are waiting:
No question about it, like most things in life, dealing with CLL is a question of hurry up and wait. Drives me crazy. But there are many things you can do, while you are waiting for the umpteenth little detail to fall in place. Think of this as a weather advisory. A storm is brewing, it is coming your way. With luck, it won't be a real bad one, with luck you will have plenty of time to prepare. But it really is prudent to prepare. If your body is in good shape, well nourished, well exercised, well rested, unstressed, it is likely to handle both the CLL and the possible therapies that become necessary a whole lot better. Exercise and nutrition are always popular topics on our discussion group. You will also find a lot of information on some interesting chemoprevention approaches that you might want to learn about, as well as some serious cautions about snake-oil sales pitches to steer clear of. When in doubt, raise the question on our discussion group. Some one will chime in with a response, setting the record straight, giving you valuable leads on where to look for more information, etc.
Bottom-line: What it all means:
The old paradigm for CLL was that only old people got this disease, there was no cure for it, so the best thing to do was to keep the patients in "watch & wait" mode as long as possible. Many oncologists still swear by this approach, waiting till the disease gets to the point where the patient is beginning to experience serious "B-symptoms", then treating the patient with drugs that give short term remissions, make the symptoms easier to bear. No attempt is made at curing the patient, the drugs are considered to be palliative and not much more than that. This approach made a lot a sense just a few years ago, since there really were no drugs that could cure CLL, and many of them were quite toxic to other systems in the body, causing quite serious and sometimes permanent harm. It was often a case of the cure being worse than the disease, so the logic was clear: wait till the last moment to initiate any treatment. The drugs available even a few year ago showed no survival advantage, in other words, patients who took these drugs did not live any longer than those who did not.
The scene is changing now, especially since CLL patients are now being diagnosed at earlier age, possibly because of more widespread use of annual medical exams and routine blood tests. It is not as much of a penalty to a 75 year old patient to be told he has CLL with an average survival expectancy of 7 years. The same message given to a 40-50 year old man/woman with children to raise, careers to pursue and mortgages to be paid, now that packs a bigger punch. "Watch & wait" followed by palliative therapy is no longer good enough, if it ever was.
The good news is that for the first time it is possible to plan for a long and healthy future for many CLL patients, with survival statistics that match "normal" people of the same age and sex. Many of the patients diagnosed with CLL today will die of plain old age, heart attacks, traffic accidents or whatever, causes that have nothing to do with CLL. Some of this is because of better prognostic tests, giving doctors a better handle on whom to treat, when to treat and with what. The other side of the coin is that the advent of monoclonal antibodies (Rituxan, Campath, to name two) have revolutionized the treatment options open to CLL patients. Either as frontline and single agent therapies by themselves, or in combination with other immune system modulators or standard chemotherapy drugs, these new drugs have made it possible to get better responses, deeper remissions, and higher percentages of responding patients, all with lower toxicities. Last but not least, we have come a long way in improved support for the "side-effects" of CLL: better drugs to handle opportunistic infections, better ways of combating disease-related anemia, reduced platelets and other auto-immune diseases.
All in all, the future looks bright, and I am betting my money on things improving even more in the years to come. In the meantime, there is a lot you can do to help yourself. It is your job to stay as healthy as you can, learn as much as you can, take charge of your own healthcare the best you can. CLL Topics is honored to help you in this fight.