To cope with CML now, it well help to work even more closely with your doctors and to better understand what's going on inside your body.
When the CML Chronic Phase Ends
Chronic myelogenous leukemia is a disease of the bone marrow. As you may know, CML tends to get worse more slowly than other types of leukemia.
"During the CML chronic phase, your blood cell-making program simply shifts into high gear and pumps out more of the normal spectrum of blood cells," says Jerald P. Radich, MD, of the Fred Hutchinson Cancer Research Center/Seattle Cancer Care Alliance. As a result, your white blood count can increase by as much as 10 or 20 times, he says.
Today, medications can be very effective during the CML chronic phase, allowing many people to live free or nearly free of symptoms for years. These drugs are often very successful at blocking the protein made by the BCR-ABL cancer gene. It is this Philadelphia chromosome that basically drives everything at this stage of CML, Radich says.
But perhaps your CML was caught late or you were unable to take medications. Maybe your disease became resistant to medication, or treatment stopped working for a reason that is not yet well understood.
As you enter the CML accelerated phase or blast phase, other changes begin to occur as well. The Philadelphia chromosome no longer drives your disease alone, Radich says. "You've accumulated a number of other genetic abnormalities besides BCR-ABL," he says. These have also moved into the driver's seat. Now, abnormal cells begin to crowd out normal blood cells and platelets, which are involved with blood clotting.
Coping with CML now becomes a new kind of experience.
Understanding CML Accelerated Phase
What's happening now? This is a really short, but critical transition phase. "Untreated, the acceleration phase progresses to blast crisis in an average of 1½ to 2 years," says Bruno Medeiros, MD, assistant professor, hematology, Stanford University School of Medicine.
Blocks in blood cell differentiation allow abnormal blood cells to build up, he says. During this phase of CML, these so-called blast cells make up 10% to 19% of the cells in blood and bone marrow.
Symptoms like these may signal that something is wrong:
You may also develop anemia from a lack of healthy red blood cells.
Many patients, however, have no symptoms at all during this phase. Instead, your doctor may see changes on your lab test results.
"When you're not feeling any worse, it's hard for you to understand that your disease has changed," Medeiros says. But grasping this is essential, because it greatly affects your treatment plan.
Understanding CML Blast Phase
The CML blast phase is really a continuation of what started in the acceleration phase, Radich says. More and more blasts build up and the numbers of normal blood cells in the bloodstream decrease.
The blast crisis occurs when blast cells make up more than 20% of the cells in your bone marrow and blood.
During this phase, infections and bleeding complications are common and can be fatal, Radich says. You may have symptoms such as:
Treatment During CML Accelerated or Blast Phase
The goal of treatment during this CML accelerated or CML blast phase is to:
How do you do this? Your doctor may first put you on a medication called targeted therapy. This therapy targets cancer cells without harming normal cells. Called tyrosine kinase inhibitors, the one you take now may be different than the one you've already taken. Your therapy may include:
If a donor is available, view this therapy as a bridge to bone marrow transplantation, Radich says. "With tyrosine kinase inhibitors, you can slow down progression in these phases, but you cannot cure the disease." If you are in acceleration or blast crisis, you need bone marrow transplantation as soon as possible," he says. "And since that takes some time, you should be on a targeted kinase therapy or experimental agent until that time arises."
Even if you get a response to therapy in this phase, it's important to understand its stop-gap nature, Medeiros says. It's unlikely to provide a long-term benefit. "You try to maximize your response for the first few months of therapy while you're trying to identify a donor. And then you move forward to stem cell transplant as soon as possible. The sooner you do a transplant, the better your outcome tends to be."
During these phases, your doctor may recommend other types of treatment as well, including chemotherapy, hydroxyurea, or apheresis. Some treatments may help to address symptoms you're experiencing.
Monitoring CML Treatment Response
Symptoms don't tell the whole story. So it continues to be critical to watch your body's response to treatment during CML accelerated and blast phases.
Your doctor may order tests that were used to diagnose and monitor your disease during the CML chronic phase. They might include tests like these:
"Monitoring is typically done with bone marrow until people get a complete cytogenetic response," Radich says. Then it may be possible to do tests using blood until a bone marrow transplant is completed.
Coping with CML Changes
This may be a scary time for you. When you're feeling sick with CML, it is especially important to get support. Lean on others depend on family members to pick up more of the tasks around the house. Keep a list of chores and when others offer to help even those outside the family take them up on it. You may also find it helpful to join a support group or to "talk" with others online who are going through similar challenges.
It's also important to understand what's happening with your treatment. "Try to educate yourself about the disease and the goals of therapy," Medeiros says. Before you meet with your doctor, do your research and write down your questions. When you meet with your doctor, take notes. Be sure to alert your doctor right away to any changes in your symptoms.
Know that everyone is different when dealing with challenging medical conditions. So tell your doctor how much you want to know about the CML accelerated or blast crisis phases of disease. But, remember, working closely with your doctors and following instructions is now more important than ever.
Annie Stuart - WebMD
