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Introducing PI
- People with primary immunodeficiency, otherwise known as PI, have immune systems that do not work properly.
© Shutterstock
1 / 29 Fotos
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.
© Shutterstock
2 / 29 Fotos
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.
© Shutterstock
3 / 29 Fotos
Varying diagnoses
- Indeed, some people do not find out they have PI until they reach adulthood, whereas others are diagnosed as very young children.
© Shutterstock
4 / 29 Fotos
Rare
- Although there are a high number of primary immunodeficiencies, the majority of them are very rare.
© Shutterstock
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Statistics
- Overall, it is estimated that one in 500 people in the United States is born with a primary immunodeficiency.
© Shutterstock
6 / 29 Fotos
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.
© Shutterstock
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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.
© Shutterstock
9 / 29 Fotos
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).
© Shutterstock
10 / 29 Fotos
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).
© Shutterstock
11 / 29 Fotos
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.
© Shutterstock
12 / 29 Fotos
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.
© Shutterstock
13 / 29 Fotos
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.
© Shutterstock
14 / 29 Fotos
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.
© Shutterstock
15 / 29 Fotos
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.
© Shutterstock
16 / 29 Fotos
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.
© Shutterstock
17 / 29 Fotos
Possible complications
- Indeed, if left untreated, PI can cause serious health problems, including organ damage and even death.
© Shutterstock
18 / 29 Fotos
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.
© Shutterstock
19 / 29 Fotos
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.
© Shutterstock
20 / 29 Fotos
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.
© Shutterstock
21 / 29 Fotos
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.
© Shutterstock
22 / 29 Fotos
Therapies
- There are also treatments that can help the immune system work better, such as immunoglobulin replacement therapy and interferon-gamma therapy.
© Shutterstock
23 / 29 Fotos
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).
© Shutterstock
24 / 29 Fotos
Stem cells
- Stem cell transplants are another possibility, in which your body gets working immune cells from someone else (a donor).
© Shutterstock
25 / 29 Fotos
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.
© Shutterstock
26 / 29 Fotos
Status quo
- As it stands, there are a number of treatments available to boost the immune system in the case of various immunodeficiency disorders.
© Shutterstock
27 / 29 Fotos
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
© Shutterstock
28 / 29 Fotos
© Shutterstock
0 / 29 Fotos
Introducing PI
- People with primary immunodeficiency, otherwise known as PI, have immune systems that do not work properly.
© Shutterstock
1 / 29 Fotos
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.
© Shutterstock
2 / 29 Fotos
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.
© Shutterstock
3 / 29 Fotos
Varying diagnoses
- Indeed, some people do not find out they have PI until they reach adulthood, whereas others are diagnosed as very young children.
© Shutterstock
4 / 29 Fotos
Rare
- Although there are a high number of primary immunodeficiencies, the majority of them are very rare.
© Shutterstock
5 / 29 Fotos
Statistics
- Overall, it is estimated that one in 500 people in the United States is born with a primary immunodeficiency.
© Shutterstock
6 / 29 Fotos
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.
© Shutterstock
7 / 29 Fotos
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.
© Shutterstock
8 / 29 Fotos
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.
© Shutterstock
9 / 29 Fotos
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).
© Shutterstock
10 / 29 Fotos
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).
© Shutterstock
11 / 29 Fotos
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.
© Shutterstock
12 / 29 Fotos
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.
© Shutterstock
13 / 29 Fotos
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.
© Shutterstock
14 / 29 Fotos
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.
© Shutterstock
15 / 29 Fotos
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.
© Shutterstock
16 / 29 Fotos
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.
© Shutterstock
17 / 29 Fotos
Possible complications
- Indeed, if left untreated, PI can cause serious health problems, including organ damage and even death.
© Shutterstock
18 / 29 Fotos
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.
© Shutterstock
19 / 29 Fotos
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.
© Shutterstock
20 / 29 Fotos
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.
© Shutterstock
21 / 29 Fotos
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.
© Shutterstock
22 / 29 Fotos
Therapies
- There are also treatments that can help the immune system work better, such as immunoglobulin replacement therapy and interferon-gamma therapy.
© Shutterstock
23 / 29 Fotos
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).
© Shutterstock
24 / 29 Fotos
Stem cells
- Stem cell transplants are another possibility, in which your body gets working immune cells from someone else (a donor).
© Shutterstock
25 / 29 Fotos
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.
© Shutterstock
26 / 29 Fotos
Status quo
- As it stands, there are a number of treatments available to boost the immune system in the case of various immunodeficiency disorders.
© Shutterstock
27 / 29 Fotos
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
© Shutterstock
28 / 29 Fotos
Demystifying primary immunodeficiency and why it occurs
World PI Week is upon us
© Shutterstock
Unless you know someone who has primary immunodeficiency (PI) or you are that someone, you probably haven't heard of it. To a large extent, the clue is in the name: people with PI have deficient immune systems. More specifically, primary immunodeficiencies cause their sufferers to get more sick, more often.
Fortunately, there are treatments available and measures people can take to prevent complications, although there is currently no cure. Thankfully, the majority of primary immunodeficiencies are rare, although this also means awareness is generally low.
With this in mind, check out the following gallery to learn all about primary immunodeficiency and why it occurs. Click on for more.
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