Hereditary Angioedema

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Hereditary Angioedema (HAE)


Antibacterial — Allergy Clinic in Idaho Falls, ID

What is Immunodeficiency?

Immunodeficiency is a disorder that affects your body's immune system. It prevents your body from being able to fight diseases and infections. Immunodeficiency also makes it easier for infections. This disorder is either acquired or congenital.

Immunology Specialist

Dr. Dave Petty uses top of the line technology to diagnose your immunodeficiency disorder. He will be able to properly diagnose you to ensure that you receive the best treatment for 
your condition.

Hereditary Angioedema

Have you suffered from abdominal pain that was quick and severe? Does the pain last a few days before it is resolved? You may have hereditary angioedema. Often times the pain is triggered randomly by menstrual cycles, injuries or by an injury. Dr. David Petty at Idaho Allergy And Asthma Clinic has the knowledge and experience to diagnose and treat this condition. Random episodes of severe face or body swelling can also be possible signs of hereditary angioedema.

If you suspect that you may have an immunodeficiency disorder or you are suffering from hereditary angioedema, contact our doctors at 208-529-9292 to make an appointment.
Angioedema or (an area of swelling) can have many causes. Allergic reactions on the skin can frequently have hives and swelling. Sometimes swelling can occur with no hives and can be localized to just one small part on your body. One hand, or one lip, or even your gut may swell, and this episode can last for a few days. It can be very painful. These types of episodes may be unrelated to an allergic reaction and may be something called Hereditary angioedema.
 
Hereditary Angioedema is a genetic condition where people do not have a break mechanism to stop swelling. They can have severe swelling episodes of different places on their body including extremeties, face, their gut, or even their airway. Frequently there will be patterns to their swelling episodes ie always having their left hand swell up when an attack comes on. However, even if you have a very consistent pattern of swelling of just your left hand, your next swelling episode could be swelling of your airway, which can be fatal. 
 
HAE has a specific defect that causes this condition. Usually a protein is not made, or a defective protein is made and unable to do its job. This protein puts the "breaks" on a swelling episode and so patients with HAE can have severe swelling that can last for days. 
 
Usually the onset of this condition is before or during the teenage years, but can develop later in life. The early onset is usually due to genetic mutations, and the later onset can be due to an underlying condition an therefore can be labled an accquired angioedema.
 
Because this condition has a very specific cause it has very specific treatments. HAE usually does not respond to allergy medicaitons like benadryl. They have special categories of medicine that are used. For many years fresh frozen plasma infusions, or anti-estrogen medications. Starting in the 2000's new medications for HAE started getting approval.

HAE.ORG has created the following list of FDA approved treatments:

  • BERINERT®

    Is an FDA-approved C1-inhibitor concentrate for treating acute HAE attacks in adults and pediatric patients. Berinert is delivered intravenously and is approved for on-demand treatment through self-administration. The medicine is usually administered when a patient feels an attack coming on. For more information go to: www.berinert.com.

  • CINRYZE®

    Is an FDA-approved C1-inhibitor concentrate for preventing HAE attacks in teenagers and adults. Cinryze is delivered intravenously and is approved for home infusion to prevent HAE attacks. For more information go to: www.cinryze.com.

  • FIRAZYR®

    Is an FDA-approved B2 bradykinin receptor antagonist for treating acute HAE attacks in patients 18 years and older. Firazyr is delivered by subcutaneous injection and is approved for self-administration. The medicine is usually administered when a patient feels an attack coming on. For more information go to: www.firazyr.com.

  • HAEGARDA®

    Is a self-administered, plasma-derived concentrate of C1-esterase inhibitor and the only subcutaneous therapy approved in the United States for routine prophylaxis to prevent HAE attacks in adolescent and adult patients. HAEGARDA will be available to U.S. patients with HAE in the near future. For more information go to: www.haegarda.com.

  • KALBITOR®

    Is an FDA-approved kallikrein inhibitor for treating acute HAE attacks in patients 12 years of age and older. Kalbitor is delivered by subcutaneous injection and must be administered by a healthcare professional. For more information go to: www.kalbitor.com.

  • RUCONEST®

    Is an FDA-approved plasma free recombinant C1-inhibitor concentrate for treating acute HAE attacks in adults and adolescents. Ruconest is delivered intravenously and is approved for self-administration. The medicine is usually administered when a patient feels an attack coming on. For more information go to: www.ruconest.com.

https://www.haea.org/ApprovedTreatments.php

HAE like attacks can occur from blood pressure medications. The "Angiotensin Converting Enzyme Inhibitor" or ACE - inhibitor family of medications can result in dry cough, or in swelling episodes. Lisinopril is one of the most common drugs from this family. This side effect can occur despite tolerating this medication for years. And swelling attacks can occur for months after stopping the medication. Swelling attacks from this medication can be life threatening.
 
If you or your loved one are concerned about Hereditary Angioedema, please call our office for an evaluation.

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