People living with systemic mastocytosis (SM) are at risk of developing anaphylaxis as part of an allergic reaction. Severe or fatal anaphylaxis can occur when mast cells are activated by symptom triggers such as insect stings and certain medications. If epinephrine is not urgently administered via an EpiPen, the consequences can be life-threatening.
What is an EpiPen?
EpiPen® is the commercial name of an epinephrine autoinjector. It is a medical device used in emergency situations when someone is having a severe anaphylactic allergic reaction. People diagnosed with SM are advised to carry two doses with them at all times, and to go to the hospital for follow-up care after using an EpiPen.
Each autoinjector holds one measured dose of adrenaline. It is effective in reversing the effects of a severe allergic reaction. It works by increasing blood pressure and opening the airways.
There are two different doses available in the United States:
- 0.15 mg, for patients weighing 33 to 66 lbs (15 to 30 kg)
- 0.3 mg, for patients over 66 lbs (30 kg)
The EpiPen needs to be injected into the outer thigh at a right angle. In children, it is advised to hold the leg tight to avoid injury. It can be injected through clothing in an emergency.
When should an EpiPen be used?
Symptoms of anaphylaxis can progress quickly and the proactive use of your EpiPen is strongly advised to avoid a life-threatening event. With the onset of anaphylaxis, blood pressure quickly drops, and the airways start to close over. Associated symptoms may include:
- Difficulty breathing
- Wheezing
- Dizziness or fainting
- Rapid heartbeat or pulse
- Tightness in the chest
- Swelling of the tongue, lips and eyes
- Difficulty swallowing
- Tingling around the mouth
- Slurred speech
- Confusion
- Fatigue
- Hives
- Red, itchy skin rash
If you’re not sure of the seriousness of your reaction, it is better to inject the epinephrine instead of waiting to be sure. Deaths from anaphylaxis are often due to a delay in administering epinephrine.
Read more about SM signs and symptoms
Tips for using an EpiPen
Your healthcare team will advise you on how to store and correctly use your EpiPen, but keep in mind the following tips for best use:
- Keep two doses of epinephrine with you at all times in your emergency medical kit, and keep your medical history and healthcare team’s contact details close.
- Check the expiry dates of your EpiPens regularly and replace them as needed.
- When administering the EpiPen, insert the needle firmly into the thigh and hold for three seconds before removing it.
- Call 911 for an ambulance and tell them you are experiencing anaphylaxis.
- Lie down with your feet raised while you wait for the ambulance.
- If you become breathless, sit up until you feel better. Then lie down again.
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