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Rarely, allergies can present with angioedema alone, but more typically they are associated with other symptoms like hives, wheezing, runny nose, etc. Also, most people with allergic reaction 4 or 5 times a year typically recognize what triggers their allergies (either food or environmental exposure). So while not impossible, your case doesn’t sound typical for allergies.
Is it true that someone who does NOT have HAE can have an attack of angioedema if he has low enough serum levels of Alpha-1 antitrypsin (AAT)?
It does not seem like low levels of alpha-1-antitrypsin are a risk factor for angioedema. HAE is not the only cause of angioedema which can be caused by allergies, use of certain medicines or come on for no reason (idiopathic). I could find one case report of a patient with angioedema, normal C1-inhibitor (ruling out HAE) and low AAT function but that is it and the report didn’t demonstrate that the low AAT function was what caused his episodes of swelling.
My daughter age 16 was diagnosed with HAE approximately 2 years ago. She has suffered from abdominal attacks, leg swellings and esophageal swellings. She is currently on C1-INH IV infusions every 4 days. The medication as at most part kept her attacks under control. However, tonight she has been complaining of severe back pain with swelling along her lower back. Could this be an HAE attack. Thank you.
Yes, skin (cutaneous) swelling in HAE can involve any part of the body and it sounds like she is having swelling of the back.
I am wondering what happens to my blood after I receive the c1 inhibitor from the hospital? Is the c1 inhibitor like a temporary jump start to my immune system to relieve the attack? Does the protein count in my blood differ before and after I receive the c1 inhibitor?
The C1 inhibitor just helps replace some of the C1-inhibitor your body lacks (or that doesn’t work). People with HAE usually have 1 gene that codes for normal C1-inhibitor and one that doesn’t so they have low levels of C1-inhibitor already in their blood. It turns out that C1-inhibitor is only a small contributor to the total level of protein in the blood so an infusion doesn’t make a big difference in total blood protein levels.
Hi, I have been experiencing HAE episodes since 2008. At first they diagnosed me with SLE Lupus. The most debilitating part of my symptoms are the abdomen attacks!! They are extremely painful and they involve nausea and vomiting and I vomit to the point that I'm dehydrated and throwing up bile. I'm in and out of the hospital constantly. The scans show swelling in the lining of my stomach and some fluid. My lips swell to a point of deformation and it is embarrassing!! The swelling tends to go away on its own and it takes about 24-48 hrs., but I still have to call in because of the embarrassment on how I look when I'm having an episode. I also notice that when I'm on my period, the episodes get worse!!! I also brake out in hives, like rash on my arms and torso, but it’s never itchy! Benadryl does not seem to help at all. My question is, if I can somehow stop my period could this minimize or possibly eliminate my symptoms?? Thanks.
It is not unusual for menstruation to be associated with attacks in women with HAE. Unfortunately, in the one study I’m aware of, menopause actually led to an increase in symptoms more frequently than it decreased them. One thing to consider is that progesterone only contraceptive pills can improve symptoms in many women with HAE.
Yes, the increase in HAE attacks is almost certainly due to stopping the danazol. While danzaol is effective in preventing attacks in many HAE patients it cannot be used in women who are pregnant or might become pregnant because it can cause damage to the developing fetus. There are other more recently FDA approved medicines your wife could use to prevent and/or treat HAE attacks and she could discuss these with her doctor. If your wife’s doctor is not aware of the new HAE medications, please contact www.HAEA.org to connect with an HAE specialist in your area or to learn more about available HAE treatments.
For type I and II HAE (those with decreased C1-INH levels) patients generally have less attacks during childhood then the number and severity of attacks increases during adolescence. The pattern of attacks established in adolescence typically continues into adulthood. However, it is common for the HAE to get better or worse for a period of time without any clear reason. Specific medicines like estrogens and ACE-inhibitors along with stress can worsen HAE. Less is known about HAE with normal C1-INH (type III HAE) but it does seem like symptoms can change without a clear reason why. 2. This would not be a typical symptom of low blood volume (hypovolemia). The amount of fluid lost from the plasma during an HAE attack is not usually enough to cause this. If you are anxious or nervous about an attack (certainly a normal reaction!) you may be breathing faster and deeper and hyperventilating. This can cause tingling of the extremities but not usually inability to move. Immobility is not typically a symptom of HAE except for avoiding movement because of the pain, so I would definitely discuss with your doctor.
Dizziness is not a typical side effect of Danazol and the normal MRI is reassuring. Are you sure Danazol is the cause of the dizziness? You might discuss with your doctor stopping it to make sure the Danazol is the cause. There are other medicines that can help decrease HAE attacks you could use instead.
It would be unusual, but not impossible for HAE to first present at this age. The fact that Benadryl helped would be more in favor of an allergic reaction than HAE. Patients can have allergic reactions to both azithromycin and ibuprofen (advil) so that would be something to discuss with your doctor. These reactions can linger so you may just need time for things to return to normal.
This is a confusing history. Patients with type I and type II HAE typically have very low C1-Inhibitor function, not high. I’m not aware of any condition associated with high C1-INH activity, in fact. While patients with HAE can have abdominal attacks, they aren’t typically triggered by eating, especially all foods. Reactions to gluten, including celiac disease and non-celiac gluten sensitivity can cause GI symptoms but typically related to gluten ingestion. Wheat allergy is distinct from gluten sensitivity and celiac disease but typically only causes symptoms when wheat is ingested. Hope that helps.
It sounds like an unusual presentation for an HAE attack. Being unable to move a body part, as opposed to it being just very painful to move it, would suggest a neurological (i.e. brain or other part of the nervous system) cause. I’d discuss with your primary care doctor.
While not typical, some patients do tend to have attacks at night for reasons that are not understood.
My 17 year old daughter is suspected to have HAE Type III. However, her milder abdominal attacks occur frequently over a 2 week period until they resolve on their own. Of all the HAE information I have read, HAE attacks typically last 3-5 days. Have you heard of attacks lasting the course of 2 weeks when left untreated? Is there a recommended diet for HAE Type III patients? We have recently received HAE medication which will be administered for future attacks.
It would be unusual but not impossible for an HAE attack to last 2 weeks. Of course, with abdominal attacks it is possible that she could be having several attacks that come on so close together they make one long attack. Also, HAE with normal C1-INH (the “new” name for type III HAE) has a wider variety of attack patterns. There is no special diet for HAE patients.
My dentist says I need a root canal - should I put it off? I'm afraid it will trigger an HAE attack?
A lot of HAE patients put off surgery or dental treatments because they're afraid it may trigger an attack. Talk with your doctor - there are treatments available that you can take short-term, before a procedure, to help prevent an attack.
During an internal abdominal swelling, does full distension of the abdomen occur or can it be just slightly bloated to be considered as a HAE attack?
The degree of abdominal distention seen in HAE attacks is be quite variable and can include mild or even no visible distension. Abdominal attacks of Hereditary Angioedema are due to edema (swelling) of the walls on the gastrointestinal tract, especially the intestines. This leads to the characteristic pain seen with these attacks and can also lead to vomiting and diarrhea. Depending on how much of the intestine is swollen, the abdomen will be visibly distended to different degrees. Distension may be more obvious in thinner patients as well.
Unfortunately, the signs and symptoms of an abdominal HAE attack can mimic those of illnesses such as appendicitis or other problems within the abdomen and in the past has led to unnecessary surgery for HAE patients. It is important for HAE patients to let health care providers know about their diagnosis so this doesn't happen. Also, newer ways of imaging the contents of the abdomen such as CT scans and ultrasound can help distinguish HAE attacks from other problems while avoiding surgery.
My name is Danielle and I have been having symptoms for the past 7 years. I have weekly outbreaks of swelling on feet, hands, lip, eyes, arms and legs. I have had my throat almost close 6 times now. I live in Hawaii so it's hard to see anybody with knowledge. My C4 complement was 26 and my C1 Esterase inhibitor protein was 24. I am waiting on the lab in Salt Lake to process my C1 esterase inhibitor activity, but in 2007 my test showed 111. I am thinking I am type II or III depending on when the lab comes back. What should I do next? I went to a specialist on Oahu but she has never seen it in her office. My GP is an allergist is on board to help in any way. I feel like I am in limbo here and not sure what the next move should be. Antihistamines and prednisone is not the answer! I have had 3 allergy tests in my life that came up with nothing. Please help Aloha, Danielle.
Typically in the past we’ve referred patients looking for specific providers to the HAEA.org. They offer physician referrals who specifically treat HAE patients.
My 4 year old daughter has had a history of unexplained stomach pain. About a month ago she had painful swollen hands that lasted a day or two. She is now battling severe itching that she describes as needles ripping through her skin all over her body. No rashes but gets red cheeks. Antihistamines or prednisone have not worked. Labs showed low C4 with C1 normal. Her allergist believes it may be type III HAE but says she is too young for any of the medications. Does this diagnosis fit? Is there anything that can be done for this type of itching? I live in CT, who can treat children? Thank you.
It would be very unusual for a girl of this age to have type III HAE, which is now called “HAE with normal C1-INH”. Itching is not typically a sign of HAE, although 4 year olds sometimes have trouble describing what they are feeling/experiencing. Antihistamines are typically used for itching but unfortunately are not always effective. We refer patients looking for specific providers to the HAEA.org.
HAE is not typically associated with hives (also called welts or urticarial). There are 2 kinds of steroids- anabolic steroids and corticosteroids. Anabolic steroids (examples are stanzalol and danazol) help patients with HAE, probably by increasing the amount of C1-inhibitor protein produced by the liver and are also the steroids used by athletes to increase muscle size. They have lots of side effects like liver disease, elevated blood lipids, severe skin problems like acne and virilization in women (facial hair, balding, deepening voice). Corticosteroids work to suppress inflammation and are utilized in diseases like asthma and rheumatoid arthritis. Corticosteroids are effective for hives but not for HAE, so if the “steroids” you’ve received and had help from are corticosteroids (examples are prednisone, dexamethasone, and methylprednisolone) it would make HAE unlikely but chronic urticarial and angioedema more likely.
I am glad the episodes are getting less frequent! I agree this would not be typical for food allergy. It is possible this could be HAE although the hives going along with swelling would be unusual. That said, HAE often gets worse around onset of menses in women and about 1 out of 4 cases has no family history. I think your current allergist is likely on the right track with the diagnosis of chronic urticarial and angioedema (which often go together in patients without HAE) and dermatographism.
Hello, I suffered from hives and swelling from the age of 21-29. I have never heard of HAE and I guess my doctors did not either, because it was never mentioned. The episodes stopped and I thought I was cured, but here I am 44 years old and the hives and swelling are back and worse than they were 15 years ago. I was just admitted to hospital a couple days ago because of throat swelling. I spoke to my brother and he is having the same symptoms. Could I have HAE? I also had some blood tests done and my estrogen level is low. Does pre menopause make the HAE symptoms worse?
You could have HAE, but typically patients with HAE don’t have hives that go along with the swelling, making it unlikely. The fact your brother has similar swelling would make a genetic disease like HAE more likely, particularly if one of your parents had angioedema as well. HAE can get worse around menopause with one study showing about 1/3 of patients getting worse, ½ staying the same and the rest getting better. Estrogen replacement therapy is a known trigger for HAE so if that is started at the time of a menopause in a woman with HAE, we’d expect symptoms to get worse.
I would slightly rephrase how you put things above. Angioedema just means swelling and is seen both as part of HAE and in association with hives/urticaria. When there is no allergic cause we often call the angioedema “idiopathic” which means there is no identified cause. Confusingly, about 10% of patients with idiopathic urticaria and angioedema don’t have any urticaria, only angioedema and this can be hard to differentiate from HAE. Most patients with idiopathic angioedema (with or without hives/urticaria) respond to medicines like antihistamines
and steroids (which don’t help with HAE). You are right that HAE often causes dramatic swelling making your symptoms less of a fit. While HAE can be accompanied by a rash, typically it does not involve flushing. There are a number of other causes of flushing as well, so that might be something to discuss with your doctors.
HAE typically presents by age 20 so presentation at age of menopause would be unusual. There is something called acquired angioedema that is often associated with and underlying illness and can present late in life. Testing reveals decreased C1-inh level and activity as in HAE.
It sounds like the angioedema is really causing a lot of trouble for you. It sounds like antihistamines and steroids have been effective in preventing and/or treating the attacks but for some reason they’ve gotten worse recently. Your observation that being run down or stressed can worsen attacks is consistent with what is often seen. On the other hand there is not good evidence that yeast/mold exposure is a trigger or that diet is a big player in angioedema, although some patients seem to notice a connection. We really try to avoid regular use of steroids for angioedema because of the myriad side effects, although sometimes they are necessary. A first step to discuss with your doctor would be to increase the dose of antihistamines (doses of up to 4x normal are sometimes effective). The medicine omalizumab has recently been approved by the FDA for treatment of chronic urticaria and might be an alternative. Some patients who are not helped by antihistamines and require frequent use of steroids can benefit from using immunosuppressive medications like cyclosporine which have their own risks.
I have been diagnosed with Angioedema...I'm not quite sure of what degree I have it although my allergist/immunologist I saw, after my second attack going to the hospital, feels it's not hereditary. My attacks started [occurring] every 3 weeks and are getting closer together. To date all attacks have involved my face...the latest just my tongue. He told me the next time I feel it coming on, which I'm now able to detect, to take a combination of 5 pills 3-prednisone...1-singulair and 1-cetirizine followed by the same pills 12 hours later. So far that has proven effective. One of my outbreaks occurred after a severe attack in my stomach...With a history of H pylori and bad acid reflux, which my mother, grandfather and grandmother have also had, I'm wondering if I should look into this with another doctor. I did have blood work and according to my doctor all my tests came back fine...just looking for some more answers.
HAE of all types can cause severe stomach attacks. It is good that the combination of prednisone (a steroid) along with Singulair and cetirizine (an anti-histamine) help. Unfortunately the prednisone could make reflux worse. You might talk to either your allergist or PCP about treatments for reflux or seeing a GI specialist
My daughter started experiencing bouts of angioedema suddenly at age 12. We saw an allergist, had allergy testing and we've been treating outbreaks as they occur. She is now in her mid 20's and recently her attacks are becoming more frequent and she now has lymph node swelling. Can angioedema cause lymph node swelling? Her attacks usually involve mouth, tongue and eyes and the swelling can be severe. She has not been diagnosed with HAE, but I'm becoming more and more concerned with the severity of her attacks and increasing frequency. I'm wondering what steps we should now take to determine the cause and diagnosis.
Lymph node swelling is not a typical symptom of HAE and I agree that the increase in frequency of her attacks is concerning. She should definitely see an HAE specialist (the HAE association can help locate one near where she lives) to evaluate her and see if any triggers for her worsening symptoms can be identified.
I am a 54 year old male with a 37 year history of angioedema without urticaria (hands, feet, lips, face, gastrointestinal tract, genitals, and throat). Antihistamines have little if any effect. Generally I'm unable to find a trigger but sometimes it is outgassing from a new carpet, adhesive, washing the car with bare feet (maybe pressure). Lately, I've developed arthritis in both hips and suspect there is a link as I've had swelling in my knee joints. Do you have any ideas what this might be…does it fit HAE ?
As far as I know, arthritis is not typically associated with HAE, except that use of androgens can sometimes. The swelling in HAE is mostly in the skin, not inside the joint so I’m not sure the HAE attacks would predispose to arthritis. You are not unusual in not being able to find a trigger for many of your attacks.
I think so. Many episodes of swelling in HAE patients are less dramatic than those you may have seen. The swelling is typically quite painful which sounds similar to what you are experiencing. I would definitely discuss testing with your doctor.
It is entirely possible for patients with HAE to have primarily or exclusively HAE symptoms [in one area] and diagnosis is often delayed in these patients. Typically, HAE attacks are a bit slower in onset, but they can definitely last only a few hours or a few days. Surgery can trigger HAE attacks as well. I definitely think HAE should be considered in this case, although it is probably not the underlying cause.
I don’t know of any connection between telogen effluvium (which is when scalp hairs stop growing early) and HAE. However both illness and stress can trigger telogen effluvium and it sounds like your daughter has had both. Hopefully she’ll make a full recovery.
Typically HAE attacks come and go over 2-4 days or so; swelling that lasted this long would be unusual for HAE, and the normal labs rule out type I or II HAE. HAE with normal C1-INH (previously called Type III HAE) could possibly be the cause, but a single attack lasting this long would be unusual. If the zyrtec and zantac haven’t helped you should talk to your doctor about other therapies but a course of steroids like prednisone might be considered first to see if it helped.
Antihistamines will not affect testing for HAE. There are many types of angioedema. There are 3 types of HAE. Type I is due to low levels of C1-inhibitor protein. Type II has normal levels but decreased function of C1-inhibitor. HAE with normal C1-inhibitor (aka Type III HAE) has normal testing but a family history. It is hard to diagnose with certainty. Angioedema can also occur on its own (not hereditary) and can be idiopathic (no cause known), due to a blood disorder (acquired angioedema-c1 inhibitor is low in these patients), due to allergies (allergic angioedema) or due to medicines (usually ACE inhibitors).
Yes, it is possible. The disease is hereditary and since you have family history, even though you have not had any symptoms, you might have HAE as well, and could have passed it on to your daughter. There are rare people who have HAE, but never have attacks (I think approximately <5% of people with the disease). So you should get tested and have your daughter tested as well.
Great questions: 1. Type II HAE is due to low C1 function but it should be low all the time, not just during attacks. 2. There is no hard and fast rule on this, but it sounds like it is not working for you. 3. Not often but they can occur.
It is impossible to say how long swelling will last, but to diagnose HAE specific tests (of C1-inhibitor level and function) must be run. One other consideration is that head and neck swelling can often be caused by a kind of medicine called ACE-inhibitors (ACE stands for Angiotensisn converting enzyme) which are often taken to lower blood pressure or for kidney or heart disease. They can cause the swelling even after having been taken without problems for months. Allergies can cause swelling but often the allergy is obvious (e.g. a person swells each time they eat a specific food).
Yes, your history is consistent with “Hereditary Angioedema with normal C1-inhibitor” which is the new name for Type III HAE. You have episodes of swelling that don’t respond to typical treatments like steroids and antihistamines and a family history of similar swelling. Unfortunately, we don’t have a definitive test for HAE with nl C1-INH so it is a clinical diagnosis, usually made in those with a consistent history of symptoms, no response to antihistamines/steroids and a family history. Please talk with your treating physician about possibly getting tested for HAE Type I and Type II to eliminate those possibilities.
It is typically thought that HAE is not associated with hives (urticarial) and it definitely doesn't respond to antihistamines like Benadryl. However, hives are common and there is no reason a patient could not have hives AND HAE. With this history testing for HAE would definitely be reasonable, although it is unlikely to explain her stomach issues. She should probably follow up with both a gastroenterologist for the GI symptoms and an allergist (if she hasn't already) for the idiopathic anaphylaxis and hives.
Unfortunately, HAE type III (now called “HAE with normal C1-inhibitor) does not have any abnormal lab findings associated with it. C1-Inhibitor level and function are normal as is C4 and other blood tests. Some patients in Germany with HAE with normal C1-Inhibitor were found to have a mutation in the gene encoding a protein called Factor XII which works in the same pathway as C1-inhibitor but this does not appear to be common elsewhere. At this point, the only recommended testing is to rule out C1-inhibitor deficiency.
HAE runs in my family, and I've had swelling in my hands recently. Is there a way to know for sure if I have the disease?
Any doctor can perform a test to check your C4 level. C4 is a protein that is part of the body's complement system, a group of proteins that move through your bloodstream. They work with your immune system and play a role in the development of inflammation. If your C4 level is normal during an attack, that basically rules out HAE. If your C4 level is abnormal, you should see an HAE specialist.
Is it possible to have HAE even though the blood tests come back negative for it? My daughter has tested negative but has all the symptoms.
There are many reasons why people have angioedema (swelling) only one of which is Hereditary Angioedema (HAE). Some people may have symptoms that are typical for HAE but not have a defect in the C1-esterase inhibitor, the enzyme responsible for HAE Types 1 and 2. In cases where there is also a family history, some physicians with expertise in HAE may make a diagnosis of HAE Type 3, although, in most cases, there is not definitive test for this type of HAE. I’d recommend your daughter see an allergist with expertise in angioedema, including HAE, to discuss the different possibilities.
There is a 50% change that a child of a person with HAE will inherit the illness, so it is not certain but definitely possible. The range of severity ranges greatly in patients with HAE, from infrequent mild attacks to frequent severe attacks. The good news is that there are now effective treatments that can both treat attacks before they get so bad as well as prevent them.
This is a good question. Use of androgens such as danazol should be avoided in women who are pregnant or trying to get pregnant. They can interfere with fertility, but by 3-5 months after stopping there should be no more effect. HAE itself does not seem to be associated with decreased fertility, either so I don't think this is related to either the HAE or the danazol.
My son has HAE, and I have read that the brain can swell. My question is, could brain swelling cause major depression and/or schizophrenia symptoms? Thank you.
Not that I am aware of. While rarely HAE may cause swelling of the brain I don’t think it would cause chronic symptoms of depression or schizophrenia.
Great question. There are rare people who have HAE based on testing but rarely if ever have attacks. I think these are <5% of people with the disease. You are right that any child of a patient with HAE has a 50% chance of also having it. It does not matter if it is a boy or girl. It is possible that the child would inherit the gene but never have an attack but that is rare.
It is thought that testing is most reliable after 1 year of age so I typically test then.
Idiopathic angioedema/urticarial can be very difficult to treat. The first step is antihistamines (such as cetirizine, loratadine or fexofenadine). Often high doses, up to 4x the usual dose, are needed to control symptoms. If that doesn’t work, montelukast (Singulair) is effective for around ½ of patients. If symptoms are still not controlled, there are a variety of other options, but they start to have more side effects. Sedating antihistamines (hydroxyzine, diphenhydramine/Benadryl) work for some but cause sedations. Immunosuppressive medicines such as cyclosporine can be very effective but have side effects that need to be watched for. Finally, newer evidence shows that omalizumab (Xolair) and antibody typically used to treat asthma is effective for some patients. Some patients may choose to just live with some swelling and hives. A major children’s hospital near where you live might be a good place to get a 2nd opinion.
This is a very good question without a clear answer. Since your attacks started while you were pregnant I’d be concerned about a possibility that a future pregnancy could have the same effect on your attacks. However, very little is known about pregnancy and idiopathic angioedema, other than increases in estrogen seem to worsen symptoms or many patients. Unfortunately, it can’t really be predicted what would happen if you got pregnant again. I would highly recommend speaking with your treating physician before making a decision on whether to get pregnant again.
I have HAE and I am 32 years old. I want to know if it is possible to conduct a gene test about our disease. I want to have a healthy baby and I read about pre implantation genetic diagnosis. Is this possible? Thank you very much!
Decisions about family are very difficult for patients with HAE. HAE is a genetic disease and 50% of children of HAE patients will have HAE. A new option that you mention is pre-implantation genetic diagnosis. In this procedure, in vitro fertilization is used and then the developing embryo is checked for the genetic mutation that causes the disease of interest. The embryo is only implanted in the mother if it is free of the genetic mutation causing the disease.
I would recommend setting up an appointment with a genetic counselor to discuss the issues related to inheritance and possible options. If prenatal diagnosis is an option, this could be discussed with a fertility specialist.
I have HAE and recently got married. My husband and I would like to have children; will our children have the disease?
Not necessarily. If one parent has HAE, there is a 50% likelihood that his or her child will have hereditary angioedema, too. Most diseases are recessive - that means that you need 2 "bad" copies of the gene for the disease, one from each parent, for the child to develop that disease. But HAE is what's known as an autosomal dominant disease — you only need one "good" and one "bad" gene for that child to have the disease.
My mother has a problem with Quincke's edema/Angioedema so she can’t take medicines which include aspirin. Every time she has high or low blood pressure she can’t take any pill, because of edema. Which medicines can she use in this situation?
While it is impossible for me to give specific advice, the class of medicines for high-blood pressure that typically cause angioedema is angiotensin converting enzyme inhibitors (ACE-inhibitors for short). Other classes of medicines for high blood pressure are usually well-tolerated although a class related to ACE-inhibitors called angiotensin receptor blockers (ARBs for short) may also cause angioedema.
Hi. I am 46 years old and have been suffering with facial pain and swelling for a few years now. I have been to several doctors and have no answers. I have a sibling who also has symptoms. What kind of doctor would be the best to see to have a C Workup done?
An allergist/immunologist would be the best person to see. Not all are experts in HAE but they should be able to do the workup for HAE.
First, 16 is the lower limit of normal for C1-INH in many labs and typically patients with HAE have levels well below 16 as well as low C4 levels, so your values don’t necessarily indicate that you would have HAE. It is hard to say for sure without actually
seing the labs, of course. It would also be unusual to present with swelling for the first time at this age with HAE, although there is something called Acquired Angioedema (AAE) which can develop at this age and is often associated with underlying illnesses. I don’t think the steroid shot would explain abnormal labs (if they are indeed abnormal). The good news is that while patients sometimes dies of HAE attacks in the past, this should not occur anymore as there are safe and effective therapies to treat attacks.
It is unfortunately common for patients to get infections from ports (and other forms of ongoing vascular access). C1-INH may also increase the risk of blood clots forming, making ports for HAE patients particularly problematic. We generally try to avoid them as much as possible in patients with HAE. Some patients can use subcutaneous medicines to treat acute attacks but right now there is no treatment that prevents attack that can be given subcutaneously. Luckily several approaches that will work subc for prevention of attacks are being tested.
C1-inhibitor level and function can be tested to make the diagnosis of HAE in most cases (there is a type of HAE in which C1-inh is normal but this is relatively uncommon in men). I don’t know of any connection between psoriasis and HAE.
This is a great question. Typically swelling of HAE involves a larger part of the body such as an entire hand or at least several fingers. Swelling from arthritis is often, but not always more chronic (longer lasting) with some pain and discomfort in the area even when swelling resolves, whereas HAE is typically severe for one to a few days and then completely resolves
I don’t think so. HAE attacks are typically severe and limited in time not chronic. So the neck and shoulder pain and stiffness you are experiencing are probably distinct from your HAE.
In the largest series I’m aware of (Bork et al. 2006. Hereditary Angioedema: New Findings Concerning Symptoms, Affected Organs, and Course. American Journal of Medicine 119:267-274) is a series of 221 patients for Germany with 131 thousand total HAE attacks. 54% of patients had one or more laryngeal attacks at some point in their life, but the larynx was involved in <1% of all attacks. For any individual patient, then, the risk of a laryngeal attack over a short time is very low. However, the risk over the lifetime is much higher. I don’t think the risk would warrant keeping a person in prison, but of course a plan for prompt treatment of life-threatening attacks should be in place for all patients with HAE.
My husband has been diagnosed with HAE III and also has Gerd, Hiatal Hernia, IBS, Asthma, allergies to foods and sulfites...lots of things that trigger his HAE attacks. He takes on-demand treatment for his frequent attacks and misses a lot of work as an IT contractor because of his conditions. We are hoping to get him on SSDI and wondered if others have been successful getting approved for SSDI with HAE and other contributing factors? We are also looking for resources and information that can help us get started with the process, if it is worth applying for.
I don’t have any experience with SSDI but don’t see why HAE should be different from any other chronic illness. Of course most HAE patients are able to lead near normal lives, either with on-demand treatment of attacks or preventative therapy. Your husband might speak to his HAE doctor about possible preventive therapies if he is using on-demand treatment frequently and it is affecting his life significantly, as it sounds like it is.
Hereditary Angioedema (HAE) is typically due to loss of C1-inhibitor (also called C1 esterase-inhibitor) function and does not usually involve B-cells or antibodies directly. Patients with type II HAE make C1-INH but it doesn’t work correctly, which sounds consistent with your picture. Rare patients have B cells that make antibodies against their own C1-INH and develop symptoms similar to HAE. This is termed acquired angioedema (AAE). Myasthenia Gravis (MG) is a disorder where antibodies are made against receptors on nerve cells, leading to weakness. I don’t know of any association between either HAE or AAE and MG.
This would be a very unusual presentation of allergies. Angioedema by itself does not usually present alone but rather is typically accompanied by hives and often wheezing, runny nose, etc. You are getting abdominal pain without an obvious trigger (other than going to California). While possible this seems unlikely to be caused by angioedema due to allergies.
People with HAE can definitely have adverse effects from medication but I don’t know of any data that they are more common than in people without HAE. The one exception might be that any form of stress can trigger an HAE attack so that form of reaction might be more common in someone with HAE.
HAE is not necessarily associated with low bone mass. However all teenagers and especially teenage girls should be sure to get enough calcium and vitamin D in their diet as bone density peeks during the teenage years. The American Academy of Pediatrics recommends a minimum of 600IU of vitamin D per day for children >1 year of age. This can be obtained both from food sources and supplement.
Alavert is loratadine, an antihistamine which typically doesn’t help with HAE. Danazol does help prevent attacks but does have many side effects. There are no over-the counter medicines that would help but several new medicines available to prevent and treat attacks. I’d recommend seeing a doctor who takes care of HAE patients to discuss your options. If you need help connecting to an HAE specialist in your area, please contact the US HAE Association (www.haea.org).
I certainly sympathize with all the symptoms you’ve had all this time. At this point, we tend to divide angioedema into symptoms caused by histamine (often due to allergies/IgE) and that caused by bradykinin. There is at this point no good test to distinguish between histamine and bradykinin mediated angioedema when test results for C1-inhibitor is normal (it is abnormal in types I and II HAE), unless there is a family history. We mostly rely on response (or lack of response) to medicine like antihistamines and steroids, which are effective only for histamine mediated angioedema. This is unsatisfying to say the least. There are no large trials, but anecdotal evidence suggests that newer HAE medicines are effective for some patients with normal C1-inhibitor and a trial of one of these for your attacks might be warranted. Hope that helps.
What blood pressure medicine can I take with HAE?
Patients with HAE should not use medications from the class of drugs called ACE-inhibitors, where ACE stands for 'Angiotensin Converting Enzyme'. This is because ACE breaks down bradykinin, which is a chemical produced during HAE attacks and thought to cause the swelling and pain associated with HAE. Because ACE-inhibitor drugs block the activity of ACE in the body, higher levels of bradykinin might build up in the body. As a result, use of an ACE-inhibitor medication might lead to more frequent or severe HAE attacks. However, there are other types of blood pressure medications that do not have this effect. Talk with your doctor about what medications should be avoided and what medications could be used to treat high blood pressure as alternatives to ACE-inhibitors.
I have HAE type II. Can I live a normal life?
Patients with HAE can lead normal lives, including going to school, pursuing their chosen career and having a family. This has become much easier recently with approval of medicines for both prevention and treatment of HAE attacks. You should discuss with you doctor what your options are and which therapy or therapies are most appropriate for you.
What are the long-term effects on a female HAE Type I patient using Danocrine/Danazol since the age of 14?
Although the use of steroids, such as danazol, can be very helpful for patients with HAE, they are associated with a long list of side effects. Among the most common of these are virilization (or the development of typically male characteristics, like body and facial hair), weight gain, menstrual abnormalities, increased levels of liver enzymes, and increased cholesterol levels.
Patients on steroid medicines, but especially women, should consult with their doctor about safety concerns with the use of steroids. Patients should use the lowest possible dose as recommended by their doctor. Cholesterol levels and liver enzymes should be followed regularly and patients should get an ultrasound of the liver regularly to check for adenomas.
With the advent of newer HAE therapies to prevent or treat acute attacks, some patients may be able to decrease their steroid dose and use newer therapies to prevent or treat HAE attacks. Patients should speak with their doctors about the range of available therapies to decide what is right for them.
I am a patient with HAE since I was 5 years old and I am currently 25 years old. I want to take birth control pills however when I took birth control pills in the past I swelled a lot. From my understanding hormones increase the attacks of swelling. Is there any birth control pill you advice HAE patients to take? Is it known exactly which hormones trigger attacks? If so which ones are they? Thank you.
It is the estrogen that worsens HAE. There are birth control pills that do not contain estrogen (they are called “progesterone only”) and these are recommended for women with HAE who need to take oral contraceptives. Please discuss with your OBGYN if progesterone only birth control pills will work for you.
I have hereditary angioedema. I have been suffering with attacks for over 5 years and unable to work because of it for the past 2 1/2 years. I have been working with my doctor to get this under control with no success. Can I, or does anyone else, get disability because of hereditary angioedema? I am 52 years old.
Yes, some patients have gone on disability for this, although it is rarely necessary.
I am a college student, and was recently diagnosed with HAE after a year and a half with it effecting my academic life and social life. I always wanted to pursue medicine, [to become] a doctor specifically, but I feel like the attacks will be seen as a liability. Is that true? Or are there people with HAE who are successful in the medical world?
HAE can indeed affect all aspect of life. However, it should not cause you to give up on your goals! I know of 2 physicians who have HAE and others with HAE who have been successful in many other fields. With new, effective therapies, there is even less reason HAE should be an impediment to achieving your goals. Good luck.
I was diagnosed with HAE in 2007. I was told I need to have surgery for a perforated eardrum. Do I need medication before surgery to avoid an attack?
Depending on the surgery, it is often advisable to use a medicine beforehand to help prevent HAE attacks. Previously, prior to surgery, patients could receive fresh frozen plasma, which contains C1 inhibitor, the protein that is lacking in HAE. Now that purified C1 inhibitor is available, many HAE specialists are using this treatment prior to surgery to help prevent attacks. I recommend consulting with an HAE specialist to discuss preparation for your specific surgery, including prophylactic options.
If you need help connecting with an HAE specialist in your area, the US Hereditary Angioedema Association (www.haea.org) may be able to help. Please contact an HAEA Patient Support Services representative in your area by visitinghttp://www.haea.org/contact/patient-services-representatives/.