Immunodeficiency Disorder

Immunodeficiency Disorder

Immunodeficiency disorders prevent your body from being able to fight infections and diseases the way it should. An immunodeficiency disorder makes you considerably more susceptible to catching viruses and bacterial infections.

Immune disorders are oftentimes categorized as either congenital or acquired. When you’re born with a disorder, it’s sometimes called a congenital or primary disorder. Acquired disorders are sometimes called secondary disorders. Secondary disorders are more common than primary.

How Does The Immune System Work

To understand immune disorders, you need to understand the basics of how your immune system works. The immune system is made up of several organs including your spleen, tonsils, bone marrow, and lymph nodes. These organs make and release lymphocytes, which are white blood cells classified as B cells and T cells. B and T cells fight invaders called antigens. Examples of antigens are bacteria, viruses, cancer cells, or bodily fluids from a sick person. B cells release antibodies specific to the disease your body detects. T cells kill off the cells in your body that are being attacked by disease.

Types Of Immune Deficiency Disorders

Primary immunodeficiency disorders are immune disorders you are born with. Primary disorders include:

• X-linked agammaglobulinemia (XLA)
• severe combined immunodeficiency (SCID disorders)
• common variable immunodeficiency
• Alymphocytosis (“boy in a bubble” disease)

Secondary disorders happen when your body is attacked by an outside source, such as a toxic chemical or an infection. Severe burns and radiation also can cause secondary disorders. Secondary disorders include:

• AIDS
• cancers of the immune system, such as leukemia
• immune complex diseases, such as viral hepatitis
• multiple myeloma

Who Is At Risk

People who have a family history of primary disorders are at a higher-than-normal risk for them. 

Anything that weakens your immune system can lead to a secondary immunodeficiency disorder. For example, the exposure to bodily fluids such as blood and sperm from someone infected with HIV can cause AIDS. 

Removing the spleen can weaken your immune system. Spleen removal may be required in response to one of any number of diseases or injuries, including cirrhosis of the liver, sickle cell anemia, or trauma to the spleen. Aging also weakens your immune system. As you age, some of the organs that produce white blood cells shrink and produce fewer of them. 

Proteins are important to your immunity. An insufficient amount of protein in the diet can reduce the strength of your immune system. Additionally, when you sleep, your body produces proteins that help your immune system fight infection. For this reason, lack of sleep reduces your immune defenses. 

Cancers and the chemotherapy drugs used to treat them can reduce your immunity.

The following diseases and conditions are also linked to immunodeficiency disorders:

• ataxia-telangiectasia
• Chediak-Higashi syndrome
• combined immunodeficiency disease
• complement deficiencies
• DiGeorge syndrome
• hypogammaglobulinemia
• Job syndrome
• leukocyte adhesion defects
• panhypogammaglobulinemia
• Bruton’s disease
• congenital agammaglobulinemia
• selective deficiency of IgA
• Wiskott-Aldrich syndrome

How To Recognise Symptoms 

Each disorder has unique symptoms. The first indicator that something is wrong with your immune system is becoming repeatedly and chronically sick, even with minor illnesses, including pinkeye, sinus infections, or diarrhea. If these problems don’t respond to treatment or don’t completely get better over time, your doctor might test you for a disorder. Recurrent pneumonia and yeast infections could also suggest you have a disorder.

How Do You Get Diagnosed

If your doctor thinks you might have an immunodeficiency disorder, he or she will want to do a thorough medical exam including:

• medical history
• physical exam
• T cell count
• white blood cell count

Vaccines can be used to test your immune system response in what is called an antibody test. Your doctor will give you a vaccine and make an appointment in a few days or weeks to test your blood for its response to the vaccine. If you don’t have an immunodeficiency disorder, your immune system will produce antibodies to fight the organisms in the vaccine. You might have a disorder if your blood test doesn’t show antibodies.

How To Treat 

Each disorder will be treated according to the specific conditions it causes. For example, AIDS causes several different infections, including Kaposi’s sarcoma, which is treated with doxorubicin lipid complex, and cryptococcosis, which is treated with fluconazole.

Treatment for immunodeficiency disorders commonly includes antibiotics and antibody replacement. A drug called interferon is often used to treat the viral infections caused by a disorder. 

If your bone marrow is not producing enough lymphocytes, your doctor might order a bone marrow transplant.

What Is The Outlook

Most doctors agree that people with immunodeficiency disorders can lead full and productive lives. Early identification and treatment of the disorder and the problems it causes are very important.

How Can Immune Deficiency Disorders Be Prevented 

Primary disorders can be controlled and treated, but not prevented. Secondary disorders can be prevented in a number of ways. For example, it is possible to avoid AIDS by not having unprotected sex with someone who carries the virus. 

Sleep is very important to a healthy immune system. According to the Mayo Clinic, adults need about eight hours of sleep per night.

If your immune system is not working properly, it is important that you stay away from people who are sick. 

If you have a contagious immune disorder like AIDS, you can keep others healthy by practicing safe sex and not sharing bodily fluids with people who don’t have the condition.

Gammaguard Liquid (pros & cons)

GAMMAGARD LIQUID is used to treat patients with primary immunodeficiency diseases (PI). There are many forms of PI. The most common types of PI result in an inability to make a very important type of protein called antibodies, which help the body fight off infections from bacteria or viruses. GAMMAGARD LIQUID is made from human plasma that is donated by healthy people. GAMMAGARD LIQUID contains antibodies collected from these healthy people that replace the missing antibodies in PI patients.

Tell your healthcare provider if you have a history of poor kidney function or kidney failure. If so, GAMMAGARD LIQUID should be administered at the slowest intravenous infusion rate as determined by your healthcare provider.

Contact your healthcare provider or call emergency services immediately if you have reduced urination, sudden weight gain, or swelling in your legs. These could be signs of a kidney problem.

Do not use GAMMAGARD LIQUID if you have a known history of a severe allergic reaction to immune globulin or other blood products. If you have such a history, discuss this with your healthcare provider to determine if GAMMAGARD LIQUID can be given to you. Tell your healthcare provider if you have a condition called selective (or severe) immunoglobulin A (IgA) deficiency.

If any of the following problems occur after starting treatment with GAMMAGARD LIQUID, stop the infusion immediately and contact your healthcare provider or call emergency services. These could be signs of a serious problem.

• Hives, swelling in the mouth or throat, itching, trouble breathing, wheezing, fainting or dizziness. These could be signs of a serious allergic reaction.
• Bad headache with nausea, vomiting, stiff neck, fever, and sensitivity to light. These could be signs of irritation of the lining around your brain.
• Reduced urination, sudden weight gain, or swelling in your legs. These could be signs of a kidney problem.
• Pain, swelling, warmth, redness, or a lump in your legs or arms. These could be signs of a blood clot. 
• Brown or red urine, fast heart rate, yellow skin or eyes. These could be signs of a liver problem or a blood problem.
• Chest pain or trouble breathing, blue lips or extremities. These could be signs of a serious heart or lung problem.
• Fever over 100°F. This could be a sign of an infection. 

These are not all of the possible side effects with GAMMAGARD LIQUID. You can ask your healthcare provider for physician’s information leaflet.

If you or someone you love is living with primary immunodeficiency  (PI) disease, you are probably already aware that regular medical treatments are necessary to help prevent getting frequent infections and secondary complications. But there are also many non-medical activities you can do on a daily basis to help maintain good physical and mental health. Below are common-sense tips to guide everyday living for PI patients. 

• Nutrition—Eat a balanced diet to help fight off diseases and infection. This is especially important for children, who are growing and developing.
• Hygiene—Wash your hands frequently, especially before meals and after using the bathroom. Viruses, including the common cold, are most often spread by unwashed hands.
• Wound care—Clean cuts or scrapes with antiseptic to help avoid infection.
• Dental care—Brush your teeth and floss daily, and visit your dentist on a regular basis. People with primary immunodeficiency are more likely to experience tooth decay and gum disease.
• Regular exercise—Exercise regularly to keep your body strong and your mind active.
• Stress—Avoid stress as much as possible, as stress can negatively affect how the immune system operates.
• Sleep—Get consistent and sufficient sleep every day. Not getting enough sleep can make you feel “run down” and can affect your immune system.
• Illness—Avoid people who are ill as much as possible, as they can spread infections, to which PI patients are particularly vulnerable.
• Interacting with others—Talk with your spouse, friends, family members, and healthcare team about anything you’re going through. Many people find joining a support group to be extremely helpful and reassuring. Ask your healthcare team if there are support groups in your area.
• Educating yourself—Learn all you can about your condition. Knowing the facts can go a long way in alleviating any uncertainty or fear you might be feeling.

Taking good care of yourself can also mean treating the signs and symptoms of infections and illnesses as they occur. Talk with your doctor before taking any medications. Besides antibiotics as prescribed by your doctor, this could include over-the-counter medications, such as:

• Aspirin or ibuprofen for pain and fever relief
• Decongestants for sinus congestion relief
• Expectorants to clear airways