Prevention is the best option

Hereditary angioedema: recommendations

Angioedema hereditari

It is essential for patients to actively participate in the monitoring and treatment of their disease to increase their personal satisfaction and autonomy. Having reliable, verifiable information is also of great help in managing the disease.

Hereditary angioedema is such a rare disease that it is little-known even among healthcare workers. This means that in this case the patients themselves particularly need to know how to act in the event of an emergency, especially when they are not in their usual environment or are far away from their medical team.

Description

What do you need to bear in mind if you have hereditary angioedema?

It is advisable, as far as possible, to avoid possible triggers or aggravators of attacks:

  • Pregnancy and hyperoestrogenic periods: if you are trying to conceive, either naturally or with in vitro fertilisation, you should consult your doctor with a view to adjusting your medication. In general, pregnancy does not affect the evolution of the disease, but it is advisable to undergo regular check-ups throughout this period. You should also inform your gynaecologist about your disease and provide them with an up-to-date clinical report. During pregnancy, angioedema attacks can be treated with C1-INH plasma concentrate, specifically Berinert® or Cinryze®. Prophylaxis can be provided with C1-INH plasma concentrate.
  • Oral contraceptives with oestrogens: in general, treatments with oestrogens should be avoided. Progestogens can be used. In an emergency situation (morning-after pill) it is only advisable to use drugs with progestogens: levonorgestrel (Norlevo®, Postinor®).
  • Stress or states of anxiety: in certain cases these may trigger an attack. You should ask your doctor or specialist whether you need to take measures or medication to control your anxiety.
  • Injuries: although the recommendation is to lead a normal life, some patients suffer inflammation in certain parts of the body as a result of knocks and bangs or playing contact sports. It is therefore a good idea to consult your doctor about physical activity and, where applicable, adapt your background treatment.
  • Dental visits and oral surgery: it is advisable to inform your dentist about your disease. Simple procedures such as cleaning do not require any additional precautions, but extractions and other procedures should only be performed after treatment with C1-INH plasma concentrate administered one to six hours in advance or with an increase in the intake of attenuated androgens for a few days before and after the appointment. It is advisable to have a full dose of emergency treatment with you at all times.
  • Infections: infections should be treated quickly in order to prevent possible angioedema attacks, especially those that affect the mouth and pharynx. Your doctor will indicate the most suitable treatment in each case.
  • High blood pressure medication: ACE inhibitors (angiotensin-converting-enzyme inhibitors), such as enalapril, captopril, lisinopril and imidapril, are formally contraindicated. Due to the introduction of new drugs with new brand names, you should ask your doctor to determine whether a particular medication is included in one of these groups.
  • Oral diabetes medication: it is not advisable to take DPP-4 (or DPP-IV) inhibitors (inhibitors of dipeptidyl peptidase 4): vildagliptin, sitagliptin, saxagliptin, linagliptin and alogliptin. These drugs are used to treat type 2 diabetes mellitus.

 

How can you quickly recognise an abdominal attack?

The following symptoms indicate a suspected case:

  • Recurrent colicky abdominal pain
  • Abdominal distension with or without nausea, vomiting, constipation or diarrhoea
  • Arterial hypotension (low blood pressure) with dehydration
  • An increase in normal waist circumference

The doctor must make a correct differential diagnosis in order to rule out other causes, such as appendicitis.

 

How can you quickly recognise a throat or larynx attack?

The following symptoms indicate a suspected case:

  • Tightness or a foreign body sensation in the throat or larynx.
  • Evident inflammation of the tongue
  • Voice changes, such as hoarseness or partial loss of voice
  • Difficulty swallowing
  • Strange respiratory sounds, such as a strident sound or an acute, rough sound

In this case it is important to remain calm and follow the doctor’s instructions. Here is some general advice:

 

What should you do in the case of a major attack (affecting the throat or larynx or difficulty breathing)?

1. Ask someone to help you explain what is happening to you.

2. Have the clinical report issued to you by your doctor at the ready.

3. If you have rescue or emergency medication (intravenous Berinert® or Cinryze®, or subcutaneous Firazyr®):

a) If you have been taught how, self-administer it in accordance with the instructions.

b) If you cannot administer it yourself, take it with you to the health centre.

4. Go to your nearest health centre for emergency treatment.

5. Make an appointment with your specialist once the immediate crisis has been dealt with.

In the case of a significant symptom burden in type I and II angioedemas, C1-INH may be administered as a prophylaxis.

 

Other recommendations and possible doubts

 

TRANSFUSIONS – CAN I GIVE BLOOD?

It is not advisable for patients with hereditary angioedema of any type or acquired C1-inhibitor deficiency to donate blood.

 

LONG OR FOREIGN TRIPS

We recommend you take an up-to-date copy of the clinical report issued by your doctor with you. It is a good idea to have the report translated into the language of your destination or English.

Find out where the nearest healthcare centre is.

Always carry rescue or emergency medication with you and make sure it has not expired. Have your medical report to hand at security controls at airports or railway stations to avoid problems.

 

DIET

You do not have a follow a special diet because it is not an allergic oedema and it is not caused or triggered by a food allergy.

Diet does not have any impact on the evolution of the disease. You should, of course, follow the healthy diet recommendations issued to everyone.

    
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