Non-small-cell lung cancer (NSCLC) is the most common type of lung cancer. It’s rare, but some people have NSCLC with “ALK rearrangement” or “ALK mutation.” You might also hear your doctor call this type of cancer ALK-positive.
Understanding what that means can help you better manage your health. It will also make it easier to talk to your doctors about your care and treatment. Here's what you need to know.
What Is ALK Rearrangement (ALK Mutation)?
While you’re still an embryo, the ALK (anaplastic lymphoma kinase) gene tells your body how to make proteins that help cells grow. This gene finishes its job and turns itself off before you’re born. But in some people, the ALK later turns back on and becomes attached to another gene. This attachment, or fusion, causes a change in the gene that doctors call a mutation.
When the ALK gene is mutated, it tells your body to make a protein that doesn’t work the right way. This faulty protein makes cancer cells grow and spread. This mutation boosts your odds of several types of cancers, including lung cancer.
ALK-positive lung cancer is usually adenocarcinoma, which means that it starts in the mucous glands of your lungs.
Who Gets ALK-Positive Lung Cancer?
About 5% of people with NSCLC have an ALK rearrangement. Scientists don’t know what causes this mutation. It’s not thought to be inherited.
Unlike other lung cancers, ALK-positive cancer tends to affect people who have never smoked. It doesn’t seem to be linked to other causes of lung cancer, like asbestos or air pollution.
It’s also more likely to be diagnosed in younger people than other types of lung cancer. About half of people with ALK-positive lung cancer are diagnosed when they’re 50 or younger. Some get diagnosed in their teens or 20s.
What Does Stage IV Mean?
Staging means how far the cancer has spread to other body parts. Knowing this helps your doctor plan your treatment options.
The higher the stage number, the more widespread your cancer is. Stage IV is the most advanced form. It means the disease has spread to parts of your body that are distant from your lungs, such as your liver or brain. This type is very hard to cure.
About 90% of people who have ALK-positive lung cancer don’t find out until the disease has reached stage IV. With treatment, about half of people with stage IV ALK-positive lung cancer live nearly 7 years or longer. So if you have symptoms, see a doctor.
What Are the Symptoms?
Lung cancer may not cause problems until you’ve had it long enough for it to spread to other parts of your body. But talk to your doctor if you have:
- A cough that doesn’t go away
- Chest pain that gets worse with deep breathing, coughing, or laughing
- Weight loss without trying and loss of appetite
- Coughing up blood
- Shortness of breath
- A weak or tired feeling
When cancer spreads, as it has by the time it gets to stage IV, it can cause:
How Is It Diagnosed?
If you have NSCLC, your doctor should test you for the ALK genetic mutation. This will help them find the best treatment for you.
Your doctor will take a small sample of the tumor in a procedure called a biopsy and send it to a lab. The scientists at the lab will check the tumor's DNA for signs of the gene mutation.
This test doesn’t check your personal DNA makeup. That means it can’t tell if your children or other members of your family have the mutation.
Your doctor will also run tests to see how far the cancer has spread. You’ll get imaging tests to take pictures of other organs and body structures. Sometimes you might need another biopsy. If you get surgery to remove the tumor, the surgeon might find cancer that didn’t show up on other tests.
Are There Treatments?
Yes. Medicines called ALK inhibitors are the main kind. (You might also hear them called tyrosine kinase inhibitors or TKIs.) These drugs block the abnormal ALK protein so it can’t cause cancer cells to grow. Your doctor might call this “targeted therapy” because it zooms in on the cancer cells and disrupts their growth.
You should take these drugs only if a lab test shows you have the mutation. They won't cure you, but they can improve your quality of life and help you survive longer. They’re sometimes paired with other treatments like chemotherapy, radiation, or surgery.
In the past, the standard ALK inhibitor was crizotinib (Xalkori). Today, the standards are alectinib (Alecensa) and brigatinib (Alunbrig). Other ALK inhibitors include ceritinib (Zykadia) and lorlatinib (Lorbrena). All are pills that you take once or twice a day.
Many people who take ALK inhibitors develop a resistance to them. Your cancer cells often mutate within 1-2 years after you start using a particular ALK inhibitor. When this happens, the medicine stops working.
Your doctor can then prescribe a different ALK inhibitor, increase the dose of your current ALK inhibitor, or consider chemotherapy or radiation. They might suggest biopsying the tumor again to see exactly how the cancer cells have mutated. The results will help them decide which drug to prescribe next.
What Are the Side Effects?
Like all medicines, these drugs can cause other problems. The most common include:
These side effects are usually mild, but not always. Stomach problems tend to be more severe with ceritinib. In rare cases, some people have to stop taking the drug.
A small number of people get pneumonitis, an inflammation of the tissue in the walls of the lungs. It can be life-threatening. If you get it, your doctor will likely tell you to stop taking the drug.
Before you take these drugs, your doctor should do an EKG to test your heart. ALK inhibitors have been linked to changes in the heartbeat or heart rate that aren’t explained by other health problems or medications.
What About Chemotherapy?
Your doctor might start you on chemotherapy before you get tested for the ALK rearrangement. If you’re ALK-positive and already on chemo, some experts say you should stick with it for several cycles -- as long as you can handle it and the cancer doesn’t spread.
If the cancer does spread, your doctor may switch you to an ALK inhibitor.
What Other Treatments Might I Need?
This depends on how far your cancer has spread.
If it has reached your brain, your doctor might suggest surgery or radiation. But newer ALK inhibitors are helping to reduce this need.
Your doctor will prescribe other treatments to keep you as comfortable as possible. These often include:
- Medicines to ease your pain, shortness of breath, and other symptoms
- Procedures to remove fluid buildup from around your lungs to help you breathe better
Be sure to tell your doctor about all your symptoms so you get the right treatments.
How Often Will I See My Doctor?
This also depends on the stage of your cancer. Your health care team will let you know how often you should come in for checkups. Keep all your appointments so your doctors can keep up with how you feel and how well your treatment is working. They may order regular follow-up tests, like:
- Blood work
- Lung tests
- Imaging tests to take pictures of the inside of your body, like CT scans or chest X-rays
Can a Clinical Trial Help Me?
Scientists are looking for new ways to fight lung cancer. A clinical trial may be a good option if what you’re doing isn’t working, especially if you have stage IV cancer. Ask your doctor about any clinical trials for ALK-positive lung cancer. Clinicaltrials.gov is another good place to try. Try typing "ALK-positive lung cancer" in the search box.
Remember, you’re at the heart of your health care team. Taking a proactive approach to care and treatment can help you feel more in control. Deciding on the right treatment for cancer is a personal choice. It’s OK to ask questions if you’re not sure how a medicine or therapy might affect you, and to tell your doctor how you’re really doing -- physically and emotionally. It’s normal to have strong feelings when you’re dealing with cancer. Your doctor can put you in touch with a counselor and support group so you can talk to people who understand what you’re going through.
Where Can I Find Support?
A lung cancer diagnosis can bring up many emotions. Each person will feel and react differently to the news. Individual or group therapy can help. Your doctor can refer you to a therapist.
A support group can also help you deal with the process and learn more about your condition. Several support groups and mentorship programs offer help to people with ALK-positive lung cancer. These include the ALK Positive Group and the American Lung Association’s online support community. Your doctor can help you find in-person groups in your area.
How Can Palliative Care Help?
Lung cancer and its treatments can cause fatigue, stress, and discomfort. Palliative care helps you manage these symptoms and side effects.
Palliative care isn’t the same as hospice care. It’s a common part of cancer treatment, meant to make you more comfortable throughout the treatment and recovery process.
It might include nausea or pain medication, extra oxygen to make breathing easier, or counseling. You can start palliative care when you’re diagnosed, and continue as long as you need it.
Palliative care teams often work at hospitals, clinics, and long-term care facilities. Some might visit you at home. These teams might include doctors, nurses, social workers, chaplains, and nutritionists.
If you’re interested in palliative care, talk to your health care team.