A Difficult Diagnosis
- Australian TV star Edwina Bartholomew, 41, has emotionally shared her chronic myeloid leukemia (CML) blood cancer diagnosis live on ‘Sunrise’ morning show, which she joined in 2001.
- CML, a cancer of the white blood cells (WBCs), affects cells called the myeloblasts, which are the parents of the mature, infection-fighting WBCs.
- CML is classified into three distinct phases, primarily based on the number of immature white blood cells, called blasts, in a patient’s blood and circulation: chronic, accelerated, and blast phases, which have different treatments and patient outcomes.
- It takes time to cope with a diagnosis and part of that process includes how to talk to other people about what you are going through.
- While some people with cancer feel very comfortable talking about their diagnosis, others want to keep in private and both are valid approaches. Be respectful of a person’s wishes when it comes to how they are comfortable seeking support.
After delivering Friday’s programming, the former Dancing With the Stars Australia co-host, also known as “Eddy,” shared her emotional news before signing off.
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“Firstly, because everyone at home has been here for all the wonderful times, for the engagements and the weddings and the babies, for all of us. It felt right to share this with you too. Many of you have been in similar situations or much, much worse and come out on the other side stronger … and more resilient.”
Which is “exactly what I plan to do,” the journalist added.
Going into further detail about her diagnosis, Edwina said when she turned 40, she “made the decision to prioritize my health.”
Edwina’s Blood Cancer Diagnosis
Edwina got a skin check and mammogram, “even had an eye check,” which were all fine, then after a routine blood test last month, her doctor noticed her levels were “out of whack.”
Edwina stressed that she had had no symptoms, which is what it’s so important to do health screenings even if you feel fine. “I was tired, but, hey, I get up at 3am. So, no symptoms at all. No warning signs. That’s really common with this kind of cancer … you don’t have symptoms.”
“But if I hadn’t had prioritized my health and had the check, it would be a different situation. It is a lot to wrap your head around.”
To Share or Not to Share Your Diagnosis
“People should do what feels right to them,” psychiatrist Dr. Lori Plutchik told SurvivorNet in a previous conversation.
It takes time to cope with a diagnosis, and part of that process includes how to talk to other people about what you are going through. Some people are more open about their diagnosis for reasons that may include showing others battling cancer that they are not alone. However, other people prefer to keep their diagnosis close to the vest to avoid unwanted judgment from others.
Seeking Support Looks Different For Each Patient
“Going through a cancer diagnosis, through treatment, is often a very long process. And then, if you include after treatment ends where a person is in a kind of limbo, waiting to see if they are clear and get their scans. It may be three months or six months into the future. People are still dealing with uncertainty at that point.”
Chronic Myeloid Leukemia (CML
“It’s important for patients diagnosed with CML to understand that their prognosis is quite favorable,” Dr. Jay Yang, hematologist, medical oncologist, and leader of the Hematology Oncology Multidisciplinary Team at the Barbara Ann Karmanos Cancer Institute in Detroit, tells SurvivorNet. “With modern treatments, most patients will go on to live healthy and productive lives with a normal life expectancy.”
This cancer of the white blood cells (WBCs) affects cells called the myeloblasts, which are the parents of mature, infection-fighting WBCs.
These cells undergo a genetic mutation due to the formation of the so-called Philadelphia (Ph) chromosome. This mutation allows the myeloblasts to spill immature WBCs into the blood, bone marrow, and spleen. These immature cells are not fit to fight off infections, and their presence in the body causes a host of problems seen in CML patients. The phase of the disease will depend on the number of these immature cells.
“What happens with CML is there is a cross of your chromosomes, so there is a cross between chromosome 9 and chromosome 22,” Dr. Eric Winer, clinical director of adult leukemia at Dana-Farber Cancer Institute, tells SurvivorNet. This cross leads to the creation of a gene called BRC-ABL, which eventually causes the overproduction of cells seen in CML.
The Philadelphia (Ph) Chromosome:
The Ph chromosome forms when two originally normal chromosomes, 9 and 22 (normal cells have 46 chromosomes in total), break off and fuse together. This fusion brings certain genes together in an unnatural way. In the case of the Ph chromosome, the BCR and ABL1 genes are joined together, forming the abnormal BCR-ABL1 fusion gene. This gene produces a mutant BCR-ABL1 tyrosine kinase, which is a protein (enzyme) that stimulates the CML cells to proliferate in great quantities.
The Different Phases Of Chronic Myeloid Leukemia (CML) & Possible Treatments
The Three Phases of CML:
CML is classified into three distinct phases, primarily based on the number of immature white blood cells, called blasts, in a patient’s blood and circulation. These are chronic, accelerated, and blast phases. These phases have different treatments and patient outcomes, making them a key part of any CML diagnosis.
Chronic Phase Symptoms:
Although most patients are symptom-free during the chronic phase, some may experience:
- Weakness
- Unintentional weight Loss
- Fever
- Shortness of breath
- Night Sweats
- Enlarging spleen causing belly fullness and pain
- Bone pain
Treatment:
At this early stage of the disease, the main therapeutic goal is to improve a patient’s symptoms, if present, destroy the Ph chromosome-containing cells, and prevent the disease from progressing to the accelerated and blast phases. Targeted therapies are often effective during the chronic phase.
Targeted therapies are drugs that target the unique features of the cancer cells. In this way, these therapies can cull cancer cells without causing collateral damage to healthy tissues. For CML, the unique protein product of their BCR-ABL1 mutation, BRC-ABL1 tyrosine kinase can be targeted by drugs called tyrosine kinase inhibitors (TKIs). These oral drugs prevent the tyrosine kinase enzyme from working, killing the CML cells in the process.
Currently, there are 5 TKIs available in the market:
- Imatinib (brand name: Gleevec)
- Dasatinib (brand name: Sprycel)
- Nilotinib (brand name: Tasigna)
- Ponatinib (brand name: Iclusig)
- Bosutinib (brand name: Bosulif)
Accelerated Phase Symptoms:
Patients may experience some of the same symptoms as patients with chronic phase disease. These include weakness, weight loss, fever, night sweats, enlarged spleen, bone pains, and shortness of breath with minimal activities. Accelerated phase patients experience these symptoms more frequently than those with chronic phase CML.
Accelerated Phase Treatment:
TKIs can be used for accelerated phase CML, although they are usually less successful at controlling this phase than the chronic phase. The disease usually comes back within 2 years on TKIs alone.
Allogenic stem cell transplantation can provide longer relief and is potentially curative. However, this is complicated by the difficulty of finding an appropriate bone marrow donor. As it is an involved, intensive treatment, patients must additionally be “suitable” for a transplant, with a good social support system, financial security, and a complete understanding of the risks of the procedure, which can include death.
TKI therapy is often used as an “induction” therapy for eventual stem cell transplantation. This means that drugs, usually bosutinib, dasatinib, or nilotinib, are used to temporarily control the disease until a transplant can be performed.
Should patients not qualify for transplantation, they could be enrolled in clinical trials.
Blast Phase CML Or Blast Crisis
If the accelerated phase is left untreated, it will eventually transform into blast phase CML.
Like the accelerated phase, there are several sets of criteria for the diagnosis of the blast phase. However, the WHO criteria are most used in clinical practice:
- More than 20% of blasts in the blood and bone marrow
- Involvement of tissues and organs besides the blood and bone marrow
- Bone marrow biopsy showing clumps of blasts within the tissue
- New chromosomal abnormalities
International Bone Marrow Transplant Registry and ELN criteria are less commonly used in clinical practice in the USA.
Blast Phase Symptoms:
Patients with blast phase CML are more likely to experience symptoms than patients with any other phase of the disease. In addition to the symptoms mentioned before, patients may experience:
- Anemia (low hemoglobin) due to a lack of RBCs
- Easy bruising and bleeding due to severely reduced platelet numbers
- Extreme vulnerability to infections due to a lack of normal, infection-fighting WBCs
- Treatment:
TKIs can be used as an initial treatment for the blast phase CML. However, they are considerably less effective during this phase and may only work for a few months.
Oftentimes, chemotherapy is combined with TKIs for better disease control. Hydroxyurea is an oral chemotherapy drug that can rapidly reduce the number of WBCs in the blood and reduce the size of an enlarged spleen. If blast phase CML becomes unresponsive to TKIs, then the chemotherapy drug omacetaxine mepesuccinate (brand name: Synribo) may be used. It is given via an injection under the skin every 7 to 14 days. Unlike, TKIs, chemotherapy is indiscriminately toxic (affecting cancer and normal cells alike) and carries its own side effects, such as nausea, vomiting, tiredness, weakness, fever, infections, diarrhea, and low hemoglobin (anemia).
Usually, the purpose of starting a blast phase patient on TKIs is to control their CML at least partially while a stem cell transplant is arranged. Unfortunately, a transplant during this phase is less successful than during the other phases and carries its own risks, as described above. There is some evidence that a transplant is likely to be more successful if imatinib or dasatinib are working.
Ultimately, a patient may progress despite all of the above therapies. For such patients, clinical trials may be the only option.
Key Takeaways for CML
- Most new cases of CML, 80%-90%, are chronic phase at the time of diagnosis.
- If left untreated, the chronic phase can progress into accelerated followed by blast phases.
- Tyrosine kinase inhibitors (TKIs), which block the BCR-ABL1 tyrosine kinase, are the first line of defense against CML and are especially effective in the early phases. They become less effective in more advanced phases.
- Chemotherapy is often combined with TKIs to increase their therapeutic effect, especially for blast phase CML.
- Stem cell transplantation is the only cure for CML, but this process is demanding and carries significant side effects.
- If the patients do not respond to any of the above treatments, they may enroll in clinical trials, which test new and upcoming treatments for efficacy against CML.
Contributing by SurvivorNet staff.
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