Myelofibrosis is a form of cancer that affects your blood. The disease leads to a buildup of scar tissue in your bone marrow, which is where your body makes blood cells, that keeps it from making normal red blood cells.
About one person in 67,000 will get myelofibrosis, most of them over 50. It usually happens in people who have a change in their genes that control how their body makes blood cells. But in some cases, there’s no clear cause.
Managing Your Symptoms
In many cases, myelofibrosis doesn’t cause any symptoms until it's damaged your body’s ability to make red blood cells. When that happens, it’s harder for your cells to get oxygen, which leaves you feeling weak and short of breath. This condition is known as anemia.
If you have anemia, your doctor might try to treat it with:
- Blood transfusions, which give your body more red blood cells to carry oxygen
- Drugs like steroids and hormones, which can help boost your ability to make red blood cells
In other cases, the first sign of myelofibrosis is that your spleen is larger than it should be. Your spleen is an organ near your liver and stomach that filters out and destroys old or damaged blood cells.
When you have myelofibrosis, your body tries to make blood cells in places like your spleen or liver. That can cause a buildup of blood cells in those organs and the blood vessels that feed them, making them swell. Your doctor may recommend the chemotherapy treatment hydroxyurea to help ease that swelling.
If your spleen swells, your doctor also might give you a drug called ruxolitinib (Jakafi). It stops the genes that may be causing your myelofibrosis from working. It also may help with some of your symptoms, like fever, night sweats, and weight loss. But it can cause side effects like anemia, dizziness, and bruising. It also may weaken your immune system and make you more likely to get infections.
If your spleen has grown so large that it’s affecting other organs, your doctor might recommend surgery to remove it. She might also recommend other treatments, including chemotherapy or radiation, to shrink it back down.
Stem Cell Transplants
The only treatment that can cure myelofibrosis is a stem cell transplant, but it doesn’t work for everyone. It uses stem cells from another person (a donor) that develop into blood cells to replace your bone marrow.
Before the transplant, your doctor will do some tests to make sure you're healthy enough for the procedure. Then a surgeon will put a long, thin tube into a vein in your neck or chest that will stay there while you're getting treatment. It's called a central line, and it's how your doctor will get medicines and the new stem cells into your body.
After that, you’ll have chemotherapy or radiation to destroy your bone marrow and weaken your immune system so it's less likely to fight the new cells. (This is called the conditioning process.) Possible side effects can include:
- Nausea or vomiting
- Hair loss
- Mouth sores or ulcers
They're much less common, but some serious effects can include infection, bleeding, or problems with your heart, lungs, or liver.
When the conditioning process is finished, you’ll get new stem cells through the central line. You'll be awake for this, and it doesn't hurt.
After the transplant, you'll stay close to the hospital for several weeks or possibly months so your doctor can keep a close eye on you. It can take that long for your body to start making a healthy number of red blood cells.
In the meantime, you may need to be given more red blood cells or platelets. You also may get medicine to help with issues like nausea or diarrhea.