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Hereditary Angioedema: Treatment, Cost and Side Effects

Last Updated: Nov 20, 2024

What is the Hereditary Angioedema?

Hereditary angioedema or HAE is a very rare genetic disorder that causes swelling underneath the skin at different parts of the body. If left without any treatment, the condition may also even cause death in some cases. Even though HAR is incurable, it can be managed through medication and treatment.

The first symptoms appear during early childhood and grow worse during the teenage years. However, since its rarity, most people are unaware that HAE causes the swelling until they are well into adulthood. The swelling is caused due to fluid deposits under the skin, which is a result of an imbalance in certain proteins.

The intensity and frequency of the swelling can differ from one patient to the next. Often, the swelling may move from one part of the body to the other during the same bout. The most dangerous form of swelling occurs in the throat region, which may cause the air passages to get blocked. This is an emergency situation and can lead to asphyxiation.

Since it is a hereditary condition, the most common form of HAE may be caused when one of the parents suffered from the same disease. A child born from such parents may have a 50 percent chance of contracting the condition.

How is the Hereditary Angioedema treatment done?

The only symptom for the disease is swelling. There is no itching or irritation of the skin, which often accompanies the swelling related to allergies. Each bout of swelling can last from 2 to 5 days. Swelling in certain regions of the body can be difficult to withstand and painful. For instance, swelling in the hands and feet can be both painful and may also be difficult to manage and conduct day to day work with.

However, throat swelling is the most dangerous symptom related to the condition. This is why, the best way to treat the symptoms is for the patient to be aware of the triggers that may cause bring on the swelling. In case of HAE, patients need to keep an eye out for stress, common cold or flu, medications for high blood pressure and heart disorders, injury and surgery, all of which can cause a bout of swelling. Furthermore, certain physical activities may also cause the symptoms to worsen.

Medication such as Cinryze, Berinert, Ecallantide and Icatibant are generally prescribed to patients. In certain cases, IV fluids and oxygen may also be needed. However, patients need to be aware of the triggers for the condition, so that they can limit the swelling by ingesting medications beforehand.

Who is eligible for the treatment? (When is the treatment done?)

People suffering from HAE should seek treatment, as without any form of remedy, the condition is liable to get worse. Furthermore, if the swelling occurs at the throat, proper medication and treatment is needed to save the patient’s life.

Who is not eligible for the treatment?

People who have swelling along with other symptoms should first consult their physicians. In most cases of hereditary angioedema, the swelling is the only primary symptom. So if the swelling is accompanied with something else, it may an allergy and not HAE.

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Are there any side effects?

All of the medications used to treat HAE comes with minor side effects. For instance, both berinert and cinryze may cause headache, nausea, stomach pain and dizziness. However, since the medication needs to be taken constantly throughout the entire life of the patient, it is always better to find a medicine that does not cause as many side effects and suits the physiology of the patient. Report any side effects to your primary physician and he may decide to change it with an alternate medication.

What are the post-treatment guidelines?

The treatment is continuous and patients will need to keep taking medicines for the rest of their lives. Patients need to keep know the triggers for the condition and should always carry medication with them. Furthermore, it is better to carry the medical information when patients step out of their homes.

How long does it take to recover?

The disease lasts for a lifetime. However, the bouts of swelling can be managed through medications. These medicines usually act fast and limit the swelling that patients experience.

What is the price of the treatment in India?

The price of treatment is generally quite high. The medications alone are quite pricy and may result in a lifetime cost of Rs. 10 Lakhs or more for the treatment.

Are the results of the treatment permanent?

Results are not permanent and another bout of swelling is bound to happen sometime in the future. The medication only offers temporary relief from the symptoms of the disease. Patients need to be aware of the trigger factors for the swelling in order to get medicated on time and limit the swelling.

What are the alternatives to the treatment?

People can try out natural remedies as an alternative form of treatment for the condition. For instance, Alfalfa herb, parsley, dandelion and Uva Ursi are some of the things that can help bring the edema under control. Corriander seed tea is also known to be a helpful natural remedy against swelling.

References

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Written ByDr. Rlv Phani Kumar Diploma in Diabetes,MD,MBBSInternal Medicine
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