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Which CLL treatment approach might be best for your individual disease? This animated video walks through important considerations that help guide treatment decisions, including genetic testing results, lifestyle factors and patient preference.
|How to Be A Partner in Your CLL Care||Key CLL Treatment Decision-Making Factors|
Hi, I’m Christy. I’m a nurse practitioner and I specialize in chronic lymphocytic leukemia, or CLL. With a variety of available treatment options, CLL patients often wonder which approach might be best for their individual disease.
Before we walk through the information that goes into choosing a treatment approach, I want to remind you that this video is intended to help educate CLL patients and their loved ones and shouldn’t be a replacement for advice from your doctor.
So, how is a treatment path determined?
CLL physicians will typically consider several key factors to help guide the decision.
When many CLL patients are first diagnosed, their medical team may use an approach called “watch and wait” or “active surveillance.” This means that treatment won’t begin immediately. Their healthcare team will monitor their CLL via in-person visits and lab testing. And, some patients may never even need treatment, depending on their individual situation.
But, if bloodwork indicates advanced disease, enlarged, bothersome lymph nodes develop, or, if symptoms like fatigue and night sweats are negatively affecting a patient’s daily life, then it may be time to treat the CLL.
Physicians typically consider a patient’s age, overall health, and existing conditions before they suggest an approach. There are also several tests on the CLL cells that may help guide treatment decisions.
Physicians use immune globulin heavy chain gene, also known as IGHV, mutational analysis to determine whether a patient is IGHV mutated or unmutated.
In IGHV mutation analysis testing, being “mutated” is a favorable finding.
If a patient’s IGHV status is mutated, and, depending on other factors such as age and overall health, the physician may recommend a treatment called FCR. FCR stands for the drugs used in this approach, which are two chemotherapy drugs combined with a targeted treatment that is a monoclonal antibody.
However, it is important to realize that due to side effects and other risks, chemotherapy is not for everybody. Non-chemotherapy treatments work very well for IGHV mutated patients as well as unmutated patients.
If a patient has unmutated IGHV, then a targeted treatment or a clinical trial might be more effective.
Molecular testing, also known as genetic testing, can identify specific genes, proteins, chromosome changes, and other factors unique to your CLL.
The results can provide your healthcare team with information related to prognosis, risk and which therapy may be most effective in treating your disease.
One of the most widely used tests is call a FISH test and it looks for specific changes in the chromosomes of your CLL cells. These specific changes can help understand how well certain treatments are likely to work for you.
For example, patients with the chromosome abnormality “17p deletion” may have higher-risk disease and will not respond well to chemotherapies such as FCR. An oral targeted treatment approach or a clinical trial will be more effective in patients with 17p deletion.
There are several types of targeted treatments that are currently approved to treat CLL including:
- Monoclonal Antibodies, which work by targeting specific proteins on cancer cells.
- And, Kinase Inhibitors, which work by blocking proteins that tell the cancer cell to grow and survive.
- A combination of treatment approaches may also be considered.
Before you start any treatment, it’s essential to ask your doctor if you have had relevant CLL genetic testing, including FISH testing, and what the results could mean for you.
Finally, one of the most important factors that your healthcare team will consider is YOUR treatment goals.
It’s very important to consider a treatment’s course and potential side effects.
With the many options available today to treat CLL, you will be able to get effective treatment. How your treatment choice affects your other health conditions and your lifestyle is essential.
Remember, you are a partner in your care and have an active voice in finding the best treatment for you.
When treatment is discussed may be a good time to consider a second opinion or a consult with a specialist. If you don’t feel supported or an active member of your team, then it is always best to get another opinion if you are able.
So, how can you put this information to work for you and help improve your care?
- Talk to your physician about what you’ve learned.
- Ask about testing mentioned in this video and whether you need to be retested over time.
- Discuss clinical trials with your physician.
- Visit credible resources to stay up to date on CLL information.
Visit powerfulpatients.org/cll to learn more about CLL.
Testing for mutations can influence treatment options available to patients with MPNs and provide a more in-depth understanding into their essential thrombocythemia (ET), polycythemia vera (PV) or myelofibrosis (MF) diagnosis. MPN specialist, Dr. Srdan Verstovsek reviews three key mutations that may impact treatment timing and approaches.
Dr. Srdan Verstovsek:
So, what we know is that ET, PV, and MF, these are these three, and we will use the abbreviations for simplicity, are so-called classic myeloproliferative neoplasms. Myeloproliferative means that it is disease of the bone marrow where cells grow without control.
Now, with ET we have high platelets, but ET is the disease of all the cells. In PV you have high red blood cells, and in many patients you have high white cells and platelets. In myelofibrosis, it’s paradoxically many patients present with too few cells because of reactive bone marrow fibers or fibrosis that limits the growth of the cells.
So, these are the three diseases that have underlying problem, same problem in these three conditions, which is high activity of proteins inside the bone marrow cells, proteins inside the bone marrow cells.
A cascade of protein that makes cells grow without control. We call this a JAK-STAT pathway. I had patients; they say JAK-STAT highway. It’s active all the time. This is a protein JAK too, and then the JAK2, and then other, so we call it JAK-STAT pathway.
It’s super active. Active in normal person when we need to make blood, but in the diseased person, active because of acquired mutations that affect that highway, JAK-STAT pathway or highway.
It makes it work all the time, that’s why we have so many cells. And there are three mutations, which are part of diagnostic process. You test for these. You can test in the blood or in the bone marrow sample, and these are JAK2 mutation, calreticulin mutation, and the MPL mutation.
They are almost always exclusive of each other, and about 90 – 95 percent of patients will have one or the other. They are still very few patients that have none of these three, which is interesting. And we are, in others, looking for other reasons in these few patients.
But one of the three is present, and it’s part of the diagnostic process as well. I didn’t emphasize this before, but it is present as a part of the bone marrow evaluation. That’s where it goes. And it is therefore, helpful to test for it. But one can test for other mutations. Many patients have many other mutations that have nothing to do with the JAK-STAT pathway, and that in large part is responsible why people have different disease ET, PV, or myelofibrosis. We explain this that way because of other genetic abnormalities, other abnormalities that we cannot really describe yet.
Genetic is not the whole picture. There are other parts, I’m sure, in bone marrow environment, in other factors they control the genetic expression, and so on, that contribute why a patient with JAK-STAT hyperactivity has ET, and why another has myelofibrosis.
We don’t really fully understand that. And of course, there is a plethora of patients in between that are not all the same. So, genetics do carry a lot of weight in what happens with the patients, and we do test for that, in addition to testing for JAK2, calreticulin MPL. We test for multiple others. That’s routinely done in academic center. It’s very valuable, and it should be standard practice.
The main utility of widespread testing for additional mutations is to assess the prognosis of the patients. If we are looking at the bone marrow blood chemistry and physical exam, a splenomegaly, and presence of this driver mutation, the JAK, calreticulin or MPL.
We call them driver mutations. They drive that highway. If that is the complexity of the diagnosis, then the next step is, as you remember, the patient will say, “How long I’m gonna live?” Well, obviously, that information comes from the historical experience, and I always emphasize that. But there is valuable information from historical perspective to some intelligence to tell the patients what to expect in general terms. Since the introduction of the genetic testing in academic centers, we have enhanced our ability to prognosticate. Initially, ten or more years ago, we would be looking at the age of the patients, how the patient fares, the symptoms, the anemia, or white blood cell count, or blasts.
These would be kind of common prognostic factors for assessment of the outcome of the patients. But now, we add information on the presence of one or the other of the driver mutations, and the presence of the number and types of these other additional, which call them somatic mutations that have nothing to do with JAK-STAT pathway.
And you can see now how the prognostication also has the flavor of complexity, and it is really not that easy, and we keep moving forward. That prognostication effort is keep moving to assess the outcome of the patients better and better for one particular reason.
If we have we a sense that a patient, based on this prognostic scoring systems, have a poor outcome, which we define as the life expectancy less than five years, then that patient should be referred to the [stem cell] transplant.
And transplant should be done because the benefit of a cure, and the risk of dying through transplant procedure – unfortunately, that’s the reality, is just justifiable if the prognostic scoring system tells you that the life expectancy is less than five years.
That’s the main role for the genetic complexity testing. Looking also at the chromosomes that might be broken. That’s done on a bone marrow sample. And dividing patients in prognosis scoring groups to guide the decision making on the transplant.
As patients, we normally rely on our doctors to tell us which tests and medications to take for the betterment of our health. Rarely do we question them since they know a whole lot more than most of us when it comes to medical ailments and overall health. However, that doesn’t mean you can’t find out more about the various suggestions doctors make.
If you have an ailment in the body and your doctor finds it hard to determine exactly what it is, they will likely ask you to get either a CT scan or MRI done. The tests are used to provide a detailed view of your internal body to help determine the ailment. We breakdown the two for your better understanding:
CT scans provide imaging using x-rays at different angles. This scan is more in-depth as compared to an x-ray. X-ray tests use a beam of radiation from a set angle and display the image. Since a CT scan uses a series of radiation beams at different angles, it slices the same image up, giving a 3D view so doctors can understand the ailment better. With the help of a computer, an image is produced. CT scans can help determine ailments such as cancer, bone injuries, and chest and lung ailments.
Magnetic resonance imaging (MRI) uses a magnetic field instead of radiation and provides a more detailed image of the body which also includes soft tissues along with the internal body. It is used to help diagnose the following:
- Brain injury
- Damaged blood vessels
- Spinal cord injury
- Multiple sclerosis
- Bone infections
- Damaged joints
- It can also be used to ensure that various organs are healthy.
Both methods are noninvasive and rely on heavy technology. But when it comes to CT scans, more and more hospitals are opting for mobile CT scanners, which make it easier for them to manage.
Getting Ready for the Test
Preparing for CT Scan and MRI is slightly different. With CT scans, your doctor may recommend you take a contest dye. The dye helps highlight the scanned region more and is generally consumed when scanning the abdomen. It is important to notify your doctor if you have any allergies because you may react to the dye. If you’ve previously had reactions to prednisone (a steroid), iodine, or seafood then the doctor should be immediately notified. Other than that, the doctor may ask you not to drink or eat several hours before the test.
For an MRI, the one thing you need to make sure is that you are not wearing anything that can be detected by magnets. This means, no jewelry, watches, hearing aids, glasses, and other items that may have a metal can be worn during the test. In some cases, a gadolinium dye may be recommended which is injected into the hand or arm. The dye highlights certain details in the imaging and rarely results in any type of reaction. The test can be lengthy for some as it takes anywhere from 30-45 minutes, so if you are claustrophobic, you may want to discuss that with your doctor since you are required to stay in a closed space for that period.
- CT Scan: You will be asked to put on a robe and remove jewelry and other metal objects so they don’t have any impact on the image produced. The scanner itself is a doughnut-shaped machine and you lie on a flat table in the middle. The table starts to move back and forth and x-ray tubes fitted into the scanner send out beams and different angles. They pass through your body to the other end of the scanner. The test is painless but make sure you are comfortable because you will be asked to stay still as the scan is going on.
- MRI: The MRI machine is a long narrow tube that is open at both ends. Like in a CT scan, you lie down on a flat movable table that slides into the tube. As you slide in, the table stops at the specific part of the body being examined and a magnetic field is created and radio waves are directed to the body. The machine does make tapping and thumping noises, so the technician will likely offer earplugs to block it out.
Understanding the Test Result
After getting either a CT scan or MRI done, you will need to consult your doctor. Unless you are a trained doctor, the images will make little to no sense to you. You will need to consult a radiologist that can explain the results to you. In case of an ailment, they will usually recommend you consult a specialist, depending on the ailment, that can assist you further.
As a patient, it is important for you to understand the tests and treatment doctors recommend. Most of the time, you can consult your doctor and they will be more than willing to give you the information you need. Knowing makes it easier for you to undergo the tests and treatments with a little more ease.
Scott has been working in the radiology field for over 30 years. He finds the biological phenomenons found in humankind fascinating and appreciates the incredible use that diagnostic imagery has to save lives. Other than acting as the President for Catalina Imaging, Scott enjoys spreading the word on new insights and breakthroughs in the radiology field, specifically the impact that mobile imaging has for patient care.
Dr. Gareth Morgan, Director of the UAMS Myeloma Institute, discusses new oncogenes in myeloma and importance of testing at the time of myeloma diagnosis to set a treatment plan at the American Society of Hematology (ASH) Conference 2017 in Atlanta.