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Introducing PI -

People with primary immunodeficiency, otherwise known as PI, have immune systems that do not work properly.

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Introducing PI -

This means that they are more likely to pick up an infection, and once they have picked up an infection they are more likely to become very sick.

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Different types -

There are around 400 different types of primary immunodeficiency, each of which varies in severity. The more severe the type of PI, the earlier it is likely to be detected.

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Varying diagnoses -

Indeed, some people do not find out they have PI until they reach adulthood, whereas others are diagnosed as very young children.

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Rare -

Although there are a high number of primary immunodeficiencies, the majority of them are very rare.

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Statistics -

Overall, it is estimated that one in 500 people in the United States is born with a primary immunodeficiency.

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Life with primary immunodeficiency -

Living with PI can be difficult, but there are treatments that can help the immune system work better. The most effective treatment depends on which type of PI a person has.

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Symptoms -

There are a number of signs and symptoms of PI. The first is contracting infections—such as ear infections, sinus infections, or pneumonia—more frequently than normal.

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Longer and more serious infections - In people with PI, infections also tend to last longer and respond less well to treatment. Antibiotics, for example, may have to be given via IV, rather than orally.

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Hospitalization -

PI also increases the likelihood of developing a severe infection that requires hospitalization, or contracting an infection that most people don’t get (sometimes known as an opportunistic infection).

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Other symptoms -

Other symptoms to look out for include digestive problems, such as chronic diarrhea, and lack of weight gain (in the case of an infant).

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When to see a doctor -

If you or your child has frequent, recurring, and/or serious infections, or infections that do not respond to treatment, you should speak to your health care provider.

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The importance of early diagnosis -

Early diagnosis is important, because it can help prevent infections that may cause long-term problems for you and/or your child.

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The role of genetics -

Primary immunodeficiency often has a genetic cause, which means that it can run in families. In some cases, for example, it is due to a genetic disorder called DiGeorge syndrome.

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If you're pregnant -

If you are pregnant or planning a pregnancy, it is important for both you and your baby’s health that you share any family history of PI with your doctor.

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If you receive a diagnosis -

Equally, if you or your child is diagnosed with PI, it is important that you share this information with your family members.

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Treatment -

Although there is no cure for most cases of PI, treatment can help delay or prevent some of the health problems it may cause.

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Possible complications -

Indeed, if left untreated, PI can cause serious health problems, including organ damage and even death.

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Preventing infection -

If you have primary immunodeficiency, it is very important that you take steps to prevent infection. These steps include washing your hands correctly and taking proper care of your teeth.

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Maintaining healthy habits -

Maintaining healthy habits, such as following a proper regime of diet and exercise, is also important, and it is a good idea to avoid people who are sick, as well as crowds.

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Vaccinations -

People with PI should also ask their doctor which vaccines are safe for them to take. In some cases, vaccines such as the MMR (measles, mumps, and rubella) vaccine are not safe.

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Antibiotics -

In terms of treatment, what is suitable depends heavily on which type of PI a person has. In some cases, antibiotics may be prescribed to prevent certain infections.

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Therapies -

There are also treatments that can help the immune system work better, such as immunoglobulin replacement therapy and interferon-gamma therapy.

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White blood cells -

Doctors may also take steps to increase a patient’s white blood count (white blood cells play an important role in the functioning of the immune system).

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Stem cells -

Stem cell transplants are another possibility, in which your body gets working immune cells from someone else (a donor).

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Gene therapy -

Finally, in the case of a genetic cause, gene therapy may be used to replace the gene that does not work properly with one that does.

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Status quo -

As it stands, there are a number of treatments available to boost the immune system in the case of various immunodeficiency disorders.

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Looking ahead -

However, primary immunodeficiency is still an area of active, ongoing research, so we can expect improved treatments and a better quality of life for patients in the years to come.

Sources: (CDC) (Mayo Clinic)

See also: All about ITP, the autoimmune disease affecting children and adults alike

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