Primary Immunodeficiency

1 What is Primary Immunodeficiency?

If your immune system is weaken allowing infections and other health conditions to occur more easily it is called primary immunodeficiency or primary immune disorders.

People who are born with this condition are missing some of the body’s immune defenses that will leave them more susceptible to germs that may cause infection.

There are types of primary immunodeficiency that are mild and will go away on its own without any treatments, but there are types that are severe that they are discovered as soon as an affected baby is born.

There are treatments that can boost the immune system.

2 Symptoms

The signs and symptoms of primary immunodeficiency that may differ based on the type and that varies from one person to another include:

  • frequent and recurrent bronchitispneumonia, ear infections, sinus infections, skin infections and meningitis;
  • infection and inflammation of internal organs;
  • blood disorders such as anemia or low platelet counts;
  • delayed growth and development;
  • autoimmune disorders such as rheumatoid arthritis or lupus;
  • digestive problems such as loss of appetite, diarrhea, nausea and cramping.

If you have primary immunodeficiency, you might develop infections that a healthy immune system person will not get (opportunistic infections).

Consult your doctor if you or any members of your family have severe or recurrent infections that don’t respond to treatments because early diagnosis may prevent long term complications.

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3 Causes

The cause of the immune system defects in primary immunodeficiency is problems in the DNA which is the genetic code that acts as a blueprint for producing cells. 

Many cases of primary immunodeficiency are hereditary that came from one or both parents.

The types of immunodeficiency disorders are classified into six groups based on the part of the immune system that is affected are:

  • B cell or antibody deficiencies;
  • T cell deficiencies;
  • Combination of B and T cell deficiencies;
  • Defective phagocytes;
  • Complement deficiencies;
  • unknown or idiopathic.

4 Making a Diagnosis

Making a diagnosis of primary immunodeficiency is done by performing several tests.

If you consult your doctor because of this, he may refer you to an immunologist who specializes in immune system disorders.

You can write down all your symptoms in a notebook. You can also write down the vitamins, supplements and vitamins that you are taking.

Bring your hospital records and medical test results and ask your family or relatives if anyone has been diagnosed with this condition.

Some of the questions that you can ask your doctor include:

  • What is causing my symptoms?
  • What are the other possible causes?
  • What tests do I need?
  • What is the prognosis?
  • What treatments are available?
  • What are the possible side effects of these treatments?
  • I have this other health condition, how can I manage them both?
  • Do I have any restrictions?

Your doctor will also ask you questions such as:

  • When did the symptoms begin?
  • Are they occasional or continuous?
  • How many infections have you had during the past year?
  • How long do they usually last? Do antibiotics help?
  • Does anyone in your family have primary immunodeficiency?

Your doctor will conduct a physical exam and will ask you about your medical history and the medical history of your family.

Some of the tests that diagnose primary immunodeficiency include:

  • blood tests – this can check if your immune system is responding properly and at the same time producing antibodies (which kills foreign invaders such as viruses) and also to check if you have normal level of immunoglobulin in your blood;
  • prenatal testing – you may want to be tested if your child has been diagnosed with primary immunodeficiency for future pregnancies and DNA testing can also be done to test for a genetic defect. Your doctor will get samples of cells from the tissue or blood or amniotic fluid to check for abnormalities.

5 Treatment

Preventing and treating infections, treating the underlying cause of the immune problem and boosting the immune system are the treatments for primary immunodeficiency.

Primary immune disorders are sometimes associated with serious illnesses such as cancer or autoimmune disorder. You can manage the infections by:

  • treating the infection with antibiotics;
  • preventing infections by taking long term antibiotics to prevent respiratory infections that can damage your lungs and ears, if your child has primary immunodeficiency he may not be able to have vaccines such as oral polio because it has live viruses;
  • treating symptoms such as ibuprofen for fever and pain, expectorants for thin mucus and decongestants for sinus congestion;
  • postural drainage for the relief of the discomfort or chronic respiratory infections.

The treatment to boost immune system include:

Immunoglobulin therapy

To fight infections because it consists of antibody proteins, this can be done by an IV line ort subcutaneous infusion;

Gamma interferon therapy

To fight viruses and stimulate immune system cells, this is used to treat chronic granulomatous disease;

Growth factors

To increase levels of immune-strengthening white blood cells. In stem cell transplantation, normal cell will be transferred so that he will have a normal function of the immune system. Stem cells can be obtained in a bone marrow or placenta at birth. Mostly a parent or a close relative is the donor of the stem cell because it must have a close biological match of the tissues but sometimes this does not work.

6 Prevention

There are no ways to prevent primary immunodeficiency because these are caused by genetic defects.

You can take precautions for infections if you have a weakened immune system such as:

  • practicing good hygiene by washing your hands with soap and water before eating and after using the toilet;
  • take care of your teeth by brushing it at least twice a day;
  • by eating a healthy, nutritious and balanced diet to prevent infections;
  • staying physically active;
  • getting enough sleep and rest everyday;
  • avoid people who have colds or other infections;
  • learn to manage your stress by doing activities such as meditating or yoga;
  • consult your doctor for vaccinations.

7 Alternative and Homeopathic Remedies

There are no homeopathic remedies for primary immunodeficiency.

8 Lifestyle and Coping

Even if you have this condition, you can still go to school or work but there is a constant threat of infections.

You may want to find support groups who have the same condition as you have.

Check for local group near your area so you can cope and at the same time ask for advices from them.

The Immune Deficiency Foundation has a peer support program.

9 Risk and Complications

The risk factor for having primary immunodeficiency is a family history of a primary immune deficiency disorder.

The complications of primary immunodeficiency vary on the type that you have and it includes:

  • recurrent infections;
  • slowed growth;
  • autoimmune disorders;
  • increased risk of cancer;
  • damage to the heart, nervous system, lungs and digestive tract;
  • death from serious infection.
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