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Vaccinations are particularly important for people living with paroxysmal nocturnal hemoglobinuria (PNH) because simple infections can trigger severe disease flare-ups. Having PNH can also make you more vulnerable to infections. Getting vaccinated gives people with PNH a head start in preventing infections that can worsen their condition.
Vaccines are a key part of staying healthy. This guide will help you understand why vaccines are important for people with PNH.
When you have PNH, getting a cold or the flu isn’t the same as it is for healthy individuals. These infections can cause flare-ups or worsen your PNH symptoms.
While your immune system is normally your best defense to fight infections, when you have PNH, a bad infection might weaken your immune system.
The complement system is a part of your immune system that plays an important role in inflammatory responses and the health of your red blood cells. Proteins in your complement system help fight infections and clean up damaged cells. It’s your body’s first line of defense against getting sick.
During a PNH flare, the complement system attacks and destroys your red blood cells much faster than usual — this is known as severe hemolysis. This red blood cell attack can cause anemia, meaning that you’re likely to feel more tired.
If your PNH is linked to other blood problems like aplastic anemia (a type of bone marrow failure), an infection can add stress to your body that it can’t easily handle. Red blood cell deficiency can make people with PNH more likely to develop blood clots (thrombosis) and abnormal platelets (cells in the blood that help stop bleeding). People with PNH may also be more likely to develop infections because of low white blood cell levels.
Getting vaccinated helps prevent people with PNH from getting sick when their immune systems aren’t working properly.
People with PNH must work with their healthcare providers to understand which vaccines they should get. Partially, this is because PNH medications called complement inhibitors increase the risk of severe infection in people with PNH. Eculizumab (Soliris) and ravulizumab (Ultomiris) are examples of complement inhibitors used for people with PNH.
Complement inhibitors are treatments that work by blocking the part of the immune system that destroys red blood cells in people with PNH. They help prevent damage to red blood cells. However, this action also reduces the body’s ability to kill certain bacteria, which can leave people with PNH more vulnerable to infections.
One of the most important vaccines to get if you take complement inhibitors for PNH is the meningococcal vaccine. People who take complement inhibitors are 2,000 times more likely to get meningococcal disease than people without PNH. This vaccine is important because meningococcal disease can lead to meningitis (infection of the layers covering the brain and spinal cord) and sepsis (spread of infection into your bloodstream).
Treatment with complement inhibitors disrupts the part of the complement pathway that’s known to be a key defense against bacteria like Neisseria meningitidis — the bacterium that causes meningococcal disease. Blocking this pathway increases the risk of developing meningococcal disease in those with PNH. This means that people with PNH who are taking complement inhibitors have a weaker defense against meningococcal disease.
The timing of getting this vaccination is critical. If possible, people with PNH should get their first dose of the meningococcal vaccine at least two weeks before starting a complement inhibitor. This will help build up the antibodies needed to protect your body before you take your PNH medication.
To stay protected from meningococcal disease, people with PNH will need to get booster shots as needed. As long as you are taking a complement inhibitor for PNH, you should get booster shots every two to five years.
People with PNH should work with their medical team to keep track of the timing of these vaccines.
Because of the increased vulnerability to infection with PNH, it’s important to protect yourself from infections that can cause PNH flare-ups.
The pneumococcal vaccine is designed to stop the bacterium that causes pneumonia. This vaccine is especially important because pneumococcal disease can invade parts of the body (like the blood) that are usually germ-free. There are a few different types of vaccines that a person can get for protection.
The influenza (flu) vaccine is the best protection against the flu. While this vaccine is generally safe for most, a few people with PNH have had adverse reactions to the flu vaccine. Talk to your healthcare provider to see if a flu vaccine is safe and recommended for you.
There are two main types of vaccines — inactivated or nonlive vaccines (containing the nonlive version of the germ) and live-attenuated vaccines (containing the weakened live version of the germ). Unlike live-attenuated vaccines, which use a small, weakened amount of the real germ, nonlive vaccines don’t use living germs.
Nonlive vaccines are considered safer for people with PNH because these vaccines are less likely to increase the risk for adverse reactions. Live vaccines can be risky for people with a weaker immune system. Because your immune system is often weakened due to PNH or PNH treatments, live vaccines aren’t safe, as they can cause infection.
Nonlive vaccines include meningococcal, pneumococcal, flu, and COVID-19 vaccines. Live vaccines include the vaccine for measles, mumps, and rubella (MMR; combined in one vaccine). Talk to your hematologist (blood disease specialist) before getting any vaccines.
The COVID-19 vaccine is safe for people who are immunocompromised and is strongly recommended by the Centers for Disease Control and Prevention (CDC). COVID-19 is an acute respiratory infection that can trigger PNH symptoms.
Researchers have found that the COVID-19 vaccine is safe for people with PNH, even those on complement inhibitors like eculizumab or ravulizumab. Staying safe requires staying up to date with COVID-19 booster shots (every six months).
The CDC recommends an updated COVID-19 vaccine for everyone 6 months and older, including those who are moderately or severely immunocompromised.
Other vaccines that may be worth getting based on your age and risk factors include:
The CDC provides a helpful adult immunization schedule that you can reference.
Your PNH diagnosis is unique, and only your healthcare team can give you the right information. Your healthcare team will be aware of which medications you’re taking, along with any additional problems you might be facing. It’s very important that you discuss individual risk factors with your hematologist before you receive any vaccines.
Together, you and your healthcare providers can decide which vaccines are best for you, depending on your unique circumstances.
On myPNHteam, people share their experiences with paroxysmal nocturnal hemoglobinuria, get advice, and find support from others who understand.
Which vaccines did your healthcare provider recommend? Let others know in the comments below.
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