How long do I have to live?
This is most people’s first question, along with “what can I expect to happen.” The doctor’s answer is called a prognosis (an educated guess about the likely course of your disease and how long you might live). Because each person is unique, and each person’s disease is different, a prognosis can be difficult to make. Also, how a disease progresses over time is unpredictable, and less is known about rare diseases than common diseases.
Whether you are an aplastic anemia, MDS or PNH patient, you need to talk to your doctor about your prognosis. This may be hard to hear, but getting a prognosis will give you key information about the treatment decisions you need to make so you can plan for the future.
Prognosis guidelines based on current data
With standard treatments, about 8 out of 10 aplastic anemia patients get better. Standard treatments include immunosuppressive therapy with antithymocyte globulin (ATG) with cyclosporine and promacta, or a bone marrow transplant. The chance for recovery depends on many factors, including how severe your case is and how you respond to treatment.
There are many different subtypes of MDS, which are identified by testing the blood and bone marrow. Your MDS subtype is a key factor in a doctor’s decision about your treatment and your prognosis (an educated guess about the likely course of your disease and how long you might live).
To figure out a prognosis for a given patient, doctors also use a prognostic scoring system. The most common one used today is the International Prognostic Scoring System-R, or IPSS-R for short. This system looks at three things:
- Number of low blood counts you have
- Percentage of young white blood cells (blasts) in bone marrow cells
- Number of cytogenetic changes (abnormal gene changes) in bone marrow cells
This prognostic scoring system tells your doctor how severe your disease is and how likely it is that your MDS might become acute myeloid leukemia (AML). It also gives your doctor a general idea about how long you might live.
With current treatments, patients with lower-risk types of some MDS can live for 5 years or even longer. Patients with higher-risk MDS that becomes acute myeloid leukemia (AML) are likely to have a shorter life span. About 30 out of 100 MDS patients will develop AML. Most patients with AML need treatment soon after diagnosis because the disease often progresses fast. The initial goal is to put the patient into remission. The long-term goal is to cure the disease, although this is not always possible.
Many people with PNH live for decades. People with PNH who develop blood clots in key parts of the body or develop MDS (myelodysplastic syndromes) or AML (acute myeloid leukemia) may have a shorter life span.
Treatments available for PNH are helping people with PNH to live longer. Older research you may have come across state that PNH patients live an average of 15 to 20 years after diagnosis. More recent research shows that the PNH lifespan has climbed over the past 20 years. It’s possible that PNH patients – who rarely develop MDS or AML – will soon have a lifespan that is normal compared with people their own age.
It is always best to talk with your treating doctor about your prognosis. Remember, each patient is different.