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Here is the story of a very brave little boy, our son, Médéric 6 1/2 years old.
The first year of Médéric’s life was very eventful. At 6 weeks, he was hospitalized for breathing difficulties. He was then diagnosed with renal malformation, renal reflux, infection of the respiratory and urinary tract, plus a gastro. The breathing difficulties and pneumonia diagnosis were numerous during the first 3 years of his short life.
At the age of only 13 months, Médéric falls and seriously injures the inner corner of an eye. A visit to the ER is needed. Within a few minutes his mouth swells, but without bruises or redness. The doctor believes that he must have also fallen on the mouth to have such swelling.
We visit the emergency department a few times for stomach aches. At every visit, after palpation of the abdomen, the emergency physician tells us that all children have a stomachache, there is nothing alarming. Everything is supposedly normal. During these periods of stomach pain, Mederic eats very little or not at all. He closes himself, lying flat on his stomach. There is nothing to do. On several occasions, we believe it is gastro because besides stomach pains, there is vomiting. Vomiting has led to hospitalizations twice for severe dehydration. Stomachache periods were so repeted that quickly the growth curve of Médéric dropped. Stomachaches were very frequent, sometimes twice a week.
Mouth swelling was common. We made a chart of everything he ate suspecting a food allergy, we took many pictures of his hands and his swollen mouth, with all the dates. We were trying to understand if there was a constant, a trigger. Too often, we were told that our child had nothing! We couldn’t believe it.
At the age 2, we had the chance to follow up with a pediatrician who, at the sight of the pictures and the analysis of the history, pronounced for the first time the possibility of hereditary angioedema. The tests however came out ambiguous. He referred us to an allergist, but no allergies. Mederic was put on antihistamine continuously for a long period of time. No improvement of his symptoms. Then, after more than two years of testing, we are transferred to the hematologist. The tests are still so nebulous, always with a high hemoglobin level. Crises are always as frequent. We notify that with each injury and stress, Mederic swells from the mouth, hands or stomach.
We finally have the diagnosis of hereditary angioedema type 3 at 5 years of age. He begins is treatment with tranexamic acid. We notice a very slight decrease in symptoms, but they do not stop or change their frequency. We switch to a Berinert IV treatment. We finally see the light at the end of the tunnel! Since then, Médéric has returned to a more normal life. We have a bran new child, filled with life and energy. Its growth curve has resumed its course! Mederic is still having attacks on occasions, but nothing compared to the past years. We can recognize triggers and we can now act quickly, even prevent.
Despite a diagnosis and medication, the disease affects the afflicted person and all those around him. We had to make major changes in our family life. Médéric is in his first year of home school. He can go at his own pace and according to his condition.
Keep hoping because things are getting better.
A mother filled with gratitude.
I will always remember my parents’ reaction, when I was six and I had my first swelling.
I saw anxiety, helplessness and especially incomprehension regarding this strange phenomenon that I learned to name later: hereditary angioedema. What could that be? I could not answer this question for several years, although I knew all too well the impacts on my life that I will try to transmit to you through my experience.
Swelling at the extremities of my body (hands and feet) and the abdomen occurred first once a year, then 2 and exponentially in high school. In an insidious and silent way, my life revolved around this disease: I could no longer play sports, walk or stay up for a long time. The intense fatigue, helplessness, loneliness, frustration, suffering and especially anxiety and stress were present. This big gap between my life and adolescence that I wanted to have led me to question my future. How to travel, go to prom, go to college or work when I have to stay in bed and miss half of my school week?
C’est avec une chance énorme que j’ai pu répondre à cette question à l’âge de 15 ans : le Bérinert en prophylaxie.
It was with enormous luck that I was able to answer this question by age of 15: the Bérinert prophylaxis. Learning intravenous bites, though difficult and time consuming, has literally changed my future. The weekly visits to my medical clinic marked in the following years my new daily. Quietly, I was able to achieve what I had always wanted: freedom and autonomy! My swelling was now better controlled.
I could not ignore my journey with this disease by stressing the importance of the many actors who accompany us at every step: a dedicated and empathetic medical team, comforting family members and an association giving hope.
Each person living with angio-edema has a unique life course, but we join in our sufferings, difficulties lived, impotence and especially our desire to offer a better (less alone) course for the following generations. That is why the mission of the AOHQ is as important and up-to-date as improving the health and quality of life of those suffering from this disease.
My story with HAE
The day I was shopping for my prom dress with my mother, trying on the dress, I saw in front of my breast, odd red shapes that were not prominent and that did not sting. However, they were present and in intense numbers. My mother was stunned when she saw those, then asked me to turn around so she could take to look at my back. I had just as much on my back! We were worried and thought that I was having a form of reaction, or that I was stressed because of the upcoming prom night, end of classes with all the exams. I also had little energy and was often dizzy with low blood pressure. I often had to go to bed. It frustrated me to be so tired all the time.
After a while, everything disappeared without us doing anything. On the other hand, the next day, my right hand was swollen and became 3 times its usual size. We did not make any connection between the red forms and swelling.
As my hand was very swollen, I went to the clinic with my parents. The doctor gave me Benadryl. Also, before prescribing anything, he took my parents out of the office, they did not understand why (and me neither). He then asked me if I had any sexual relations lately. I never had one yet and I really did not see any connection between the swelling of my hand and sexual intercourse. He requested blood tests that turned out to be normal and he concluded of an allergy attack.
He gave me Benadryl and it only made me sleep, but my hand remained swollen for at least 4 days.
I began to find that disturbing. Subsequently, I noticed that the red marks came back every month, before my periods and that my hand became swollen quite regularly during my periods and that it lasted 4 to 5 days. I made a connection between the two without understanding the reason why.
I swollen when going canoe camping for the physical education class and playing volleyball. I had to stop playing, being swollen at the end of every practice.
One day, while my mother was working, I got really scared when my hand swelled again. She immediately called my pediatrician she had talked to, without ever having seen my hand swollen. He told her for me to leave the house and come see him at his office immediately.
I left, with fear in my stomach, wondering what I could have. In his office, he examined my hand and gave me a prescription for a appointment with an emergency allergist. At that time, requests to see a specialist went faster and worked well … luckily for me!
When I went to meet the allergist, he asked for a simple blood test. I had the tests done and a week or two later, he called me at home, telling me to come and visit him quickly and in the meantime, not to go to the dentist! I must admit that at the time, I saw no connection between the two… But, I told him that I was leaving the dentist’s office barely 2 days ago and I came to have my wisdom teeth removed !!!! There was a long silence at the other end of the line… He then told me to come and consult him as soon as I could.
On the day of my appointment, my boyfriend and now husband accompanied me to the specialist’s office. The doctor was very uncomfortable and gave me the news. I was then 16 years old and I did not understand anything. He explained me, poorly, that I will have to take a medicine for the rest of my life and that it was very virilising, that I could not have children and that I would gain weight, but that I was lucky to have survived the dentist because my throat could have swollen and even die there. I did not understand anything. He asked me if people in my family had swellings, but I was the only one. My father and mother were tested and they had normal C1. At the time, the doctor did not specify an illness type (I think it did not exist), but he told me that I was a form of mutation … and that my only way out of a crisis was to have a plasma transfusion. He admitted that there were risks to that, but that I had no choice.
So I took Danazol high dosage at first. I gained a lot of weight, lost my diminished breasts, and my physique changed completely. I was lucky to have a boyfriend who stayed by my side and who accepted all this while continuing to love me.
Luckily for me, I was able to reduce the doses of Danazol and I tried to keep them at the lowest … I was at the limit of reasonable, but I tried to minimize the side effects. I did not have C1 at all in the body. I’m type 1. Today, I still think that when I had my teeth removed at the dentist, my time had not arrived … by the way, that’s what the allergist told me said. He did not understand anything.
Later, when it came time to get married, I asked my future husband several times to make sure he knew what he was doing by marrying a woman who would not give him children and who would need to take medication for life. This soul mate was kind enough to say that he preferred to be with a woman he loved more than anything in the world than to change for someone he would not like and have children. He is still with me today. He was put on my way, that I am sure.
Then it was the joys of the side effects that lasted more than 30 years, always staying on the limit for my C1. I always had crises from time to time, but especially in the periphery. Later, a doctor was looking at my file told me that I must had made in the past abdominal attacks, around the age of 9, when I came to the hospital for stomachaches and another at 15 when I had intense vomiting with rubella and that I had lost 11 pounds. But nobody knew what I had at the time. I suffered in silence.
At my first HAE conference, I met people with the same illness. Right away, they became my family. I was no longer alone … I was on a cloud and I had so many questions to ask … and it was also there that I finally began to understand the nature of my illness that prevented me from living normally. This meeting was a real revelation for me. I felt the affection of people around me who were living the same things. For some, it was worse than for me. I understood how lucky I was. I kept in touch with many of these people who are a bit like a family to me. Then this association dissolved. Then another one came and now I am really a part of a family with people who get the same treatments. We can discuss our choices. Doctors are more present and the disease is more and more known. I now know what I have to do before going to the dentist. I have friends within the association who are very important to me. Doctors are also human and dedicated people. It motivates us so much … and all the new treatments coming out every year allow me to live an even more beautiful life. My only regret is having it all too old to have a child. Today, I would not be afraid to give birth. I see hope and light at the end of the tunnel.
I remember another immunologist at the time who told me that I should try to have a child, because the statistics said that pregnant women had fewer attacks and after childbirth, it was possible that I did not have any more crises. I told her that since this disease was hereditary, I did not want to pass it on to my child and take the chance to be in those statistics of dead women in diapers with angio…. I really thought he wanted to see the result and if the statistics were true. I never agreed to try, due to fear of dying. Poorly monitored as I was, I think I would be dead, actually.
One day, I met Dr. Laramée in a convention and he decided that I had suffered enough. He took me as a patient. I followed the training for the Berinert treatment with my spouse at Notre-Dame Hospital. We learned to inject despite the few veins that I had. After getting the instructions, the first time at home, my husband worked so hard to succeed, but since I had very few veins, it was very difficult. After trying 3 veins, he finally succeeded … As the stress fell after finally succeeding, he made a vagal shock, finding his head between the two legs and I continued to inject the Berinert. I was telling him to stay, that way I could go on alone … hi hi hi … Today, we laugh, but at the time, it was quite stressful. The next time, I injected myself (we did it in turns), I also missed my shot 3 times and I finally succeeded. Then, I had to look, because I also made a pressure drop, being too stressed. Having veins to prick easily is a challenge… and know that I have no other options, not to get crisis is constant stress … My spouse has continued to inject me and I had a washcloth on my forehead, with my head bowed … .I told myself, what a pair of idiots we are being… I was afraid of what was waiting for me for the next time. Luckily, we have grown accustomed to each other and for several years, we always succeeded and my life got better. I was able to travel and live fully without the fear of dying.
There came a day when my husband could not inject me… we came to understanding why he was not succeeding every time. It was a great stress with every injection. Are we going to find a vein or not? For a time, I managed (both with my left hand and my right hand) to inject on top of the hands (they are small and my veins are almost non-existent). Then, I had to resign myself to going to a medical clinic to have it done by a nurse twice a week, because I was not able to do it by myself any more. Then, Dr. Laramée, who I consider my savior, helped me to have Berinert subcutaneously. My life is so changed and stress is finally gone…
I had 2 major attacks before getting to the subcutaneous injections and the Firazyr (which I drag around with me) was my ally. In 10 minutes and without any side effects, the crisis has disappeared and I can now breathe in peace. This medicine allows me to go everywhere, to travel without being afraid. It is my little security. I’m never afraid again. With the association, I am no longer alone. I feel supported and helped. I’m still testing the subcutaneous injections because it’s different from the IV, but it saved me because I would not have done it. I hurt myself with a good sprain. This required additional treatments. However, subcutaneously, I can have more without using my veins and I can go to work.
Thanks to my doctor and the Association … to CSL Behring and One Path.
I now have a life without fear, I found a lot of energy, lost weight and my physical strength in sport (hi hi hi) … I know can beat everyone in a bike race, I found myself good at last !!! I am no longer an extraterrestrial!!!!! I am me!!!! And I’m not alone anymore …
My experience with the health system
- First memory of HAE symptoms: “6 years old on the bus, stomachache, crying.
- All my childhood: stomachache, according to our family doctor: fragile intestines.
- 12 years old: 1989- Beginning of a hell that will last 6 years,from grade 1 in high school until my second College year”.
1st intubation: severe laryngitis, returned home.
Continuous emergency visits for severe stomach pain: morphine, bladder-kidney-intestine tests.
Respiratory difficulties: repeated intubations.
From 12 to 13 years old: 5 intubations of which, one tore my trachea…
Every time, same pattern:
- Breathing difficulties
- Intensive care, Cortisone
- Sent home, no follow up from medical professional’s…
Intubation, again… back home and a few days later permanent cough that looks like a dog barking. Continuous impression of looking for air. It becomes like a reflex cough.
Back to the hospital: no intubation, but I am hospitalized. Beginning of a stay in the pediatric unit that will last 2 months. Every day, I get codeine drugs to inhibit my cough, but my saturation stays around 85-88%, so I am constantly in the oxygen tent.
Still drugged, always out of breath, I am 13 years old…
After 1 ½ months, the Baie-Comeau hospital decided to transfer me to Quebec City because they do not know what to do with me anymore.
Psychiatrist, Social Worker and much more specialists are part of my daily routine.
Are you being abused?
Are your parents bickering?
Why do you need so much attention?
After 2 weeks of hospitalization: I am sent back home, giving my parents the following conclusion:
- She simulates breathing stops to seek attention.
- She needs psychiatric help.
- All of this is happening in her head.
My mother explains that the intubations are very difficult, that they have already torn my trachea, which she has twice signed discharges for tracheal, because I was so swollen that they did not think they could succeed intubation.
Doctors answer: Your daughter has unsuspected manipulative power over her body. It’s a mental illness.
I put myself in my parents’ shoes, who must have wondered if it was not the case…
Adolescence continues to pace with emergency room visits, excruciating stomachaches and intubations.
In April 1994, with my boyfriend in Forestville, about 1 hour from Baie-Comeau
- once again: not able to breathe, transfered to Baie-Comeau by ambulance
- loss of consciousness
- IV intubation.
Remembering floating above the room, I see my mother crying, I see people I love, I see a light, I hear the medical staff screaming and the machines beeping and, suddenly, I regain consciousness…
I spent 2 weeks intubated in intensive care. Whenever we tried to remove the tube, my throat closed and I was re-intubated urgently.
In a fog of morphine, because the belly pain was unbearable…
During this hospitalization: I met a young and freshly graduated internal medicine specialist who had vaguely heard about HAE during his classes. He makes me take tests, which consist of a dosage of the inhibitor of C1, C2 and C4.
The results will come during the Cégep de Chicoutimi fall mid-term.
- C1-lowest value of normal
- C2-low limit of normal
- C4-10 point below normal
He therefore concludes that I have HAE, but not in its classical form. But until now, very little information is available. According to him, I would probably be the tenth person with this condition in Québec.
So I leave with a prescription of Danazol, which is, it seems, the only available treatment … Next checkup, Christmas Eve.
My second living hell starts.
My second living hell starts.
In 4 months:
- I gain over 50 lbs
- My periods stop.
- Loss of my breasts
- Hair on the chin and on the chest. Uncontrollable level of aggressiveness (I am normally a very calm person). I am always sick … no throat attacks, but stomach pain constantly makes me miss College regularly.
After one year: I am unrecognizable, suicidal and always so sick.
I tell the doctor if I do not change medication, I will kill myself, that I already chose the day, the time and place. I was in deep psychological distress. He accepts without much enthusiasm to stop Danazol and start Cyclokapron. MIRACLE, it works, I find back my femininity and, myself again.
In 2000, I moved to the Montreal South Shore.
I am referred to Ste-Justine hospital. I then consider a pregnancy; they make me try a blood product: the Berinert 500U / week in IV. It controls the disease pretty well. I become pregnant and the more the pregnancy progresses, the more the injections increase, due to an increase of the symptoms. I will be followed in High Risk Pregnancy Clinic. Labor will be induced at 36 weeks 6 days because I am constantly in crisis, and fear for the baby. At this moment, I receive 2-3 injections a day.
I experience a natural delivery.
Year after delivery: I have a lot of attacks. They put me back to Cyklokapron on a regular basis, and Berinert 1x week to treat the crisis.
Over the years, the crisis and the injections increase. At the end of 2009, I have 7 injections / week of 1000U.
The pain exhausts me and doctors do not know what to do anymore. I had more than 90 attacks in the last year, mostly to the abdomen. In the meantime, since my C1 blood values are still normal, I am classified as AOH, type 3.
- Therefore, in January 2010 I start plasma transfusions at a frequency of 3x / week, 2x / week, 1x / week. It will last 6 years …
My third living hell…
Transfusions had better attack control; I go from 90 to 7 attacks the first year of transfusions.
After 2 years, in 2012, I begin to have transfusion reactions, we integrate a premedication, that is effective at the beginning. In 2013, the transfusion reactions were so intense that I thought I would die at each transfusion.
I redid my will over again, making sure that my daughter would be taken care of in case something happened to me.
I begin receiving OCTOPLASMA, which is a standardized solvent-detergent plasma that ensures uniformity, transfusion after transfusion.
The side effects disappear.
From July 2013 until December 2013, I have unbearable pain under the ribs, on the right side. I have many tests; the pain seems to come from the gallbladder, even if nothing appears to be conclusive on the tests.
During this period, I receive 2 transfusions / week in addition to the 500 units of Berinert per day. In December, I go through surgery to remove the gallbladder with laparoscopic ally. What should have been a fairly regular intervention turned into a nightmare.
Initially, following the surgery, I had to be sent to the intensive care unit to intervene quickly if symptoms of edema occurred in the postoperative period.
The doctor on duty in the recovery room judged that my condition was far too good to admit me to the intensive care unit. During the night, I felt the swelling start in my belly…
I asked to see the doctor on duty. He sent the resident. I explained him the urgency of the situation, he refused that I would receive Bérinert or a plasma transfusion. He gave me Benadryl and cortisone.
There was however a protocol to be followed clearly dictated by the doctor in the file. I called my husband asking him to come to administer my medicine, he came but was denied access by security.
Meanwhile : I was swelling more and more.
My bladder and my urethra were so swollen that I had to get a urinary catheter. My face, my neck, all my limbs, my abdomen, everything was swollen, not to mention the excruciating pain in the stomach. When my spouse was allowed to come in around 7 am, he found me in a terrible condition. My doctor came, prescribed 2 plasma transfusions on the spot and left.
The medication to be induced took 3 hours to get from the hospital basement to the 6th floor !!!!
This crisis, was my biggest ever, lasted 2 full days. I finally returned home and went back to transfusions every week.
February 2014: return of transfusion reactions … return to pre-med.
I must always be accompanied for my visits to the hospital because I can not drive, too much side effects due to pre-med.
- My life is built around my transfusions. Consequences: Anxiety starting the day before, pre-med side effects, physical and mental exhaustion.
- In March 2015, I asked my doctor for another alternative. According to him, no other possibilities … I asked him to prescribe a new drug recently available: Firazyr, which stops HAE attacks quickly. He refuses, because I’m not a classic case, so it will not work on me, according to him.
In September 2015,
I went at the AOHQ’s (HAE) educational day, I heard Dr. Hébert’s conference and I approached him at the coffee break to explain my distress and asked him for help.
His answer was positive.
I got an appointment with him on November 9, 2015 and since then my life has changed.
- He offered me a treatment with Berinert, but in high doses. That is 3x / week 1500U.
- He prescribed me the famous Firazyr!
- Since then, I have not had a transfusion. It’s the 307th day today.
- I do 3 injections a week that take 15 minutes, at home vs. 4-5 hours in the hospital, I have no side effects.
- When I have attacks, I take FIRAZYR and immediate response, in 15 minutes or less, pain and swelling are things of the past.
My daughter Juliette is unfortunately also affected. She had a few symptom episodes that led to the diagnosis when she was 8 years old. It was relatively quiet during the last years. 2-3 minor crises per year.
Last May, she had a throat crisis.
We rushed her to the ER, we had a medical certificate and the medication on hand. More than 2 hours of procrastination before they decided on the treatment to follow. I had to “shake them up badly” to make things happen with the emergency physician, for him to understand the urgency of the situation and explained him my personal experience with intubations.
The next morning, he came back to me and said that often this disease is psychosomatic!!!!!! That Berinert had a placebo effect according to him.
I left the hospital with my daughter with an incredible frustration feeling… hoping not to come back soon…
The following week, another throat crisis.
Back to the hospital. This time :
We stayed 2 hours in all.
Crisis treated quickly, crisis that does not persist. Other team = other treatment quality.
3 weeks ago, uvula surgery, complications…
The uvula has swollen 3 times the volume, touches the tonsils. The pediatrician on duty has called Juliette’s doctor at Ste-Justine, who is supposedly an HAE specialist…
He refused to border Berinert, prescribed Cyklokapron IV and Solucortef???
There is a treatment and, still today, we have to fight to get it!
To live with a rare disease is to experience isolation, doubt, suffering, incomprehension. As the disease is not visible, judgments about the intensity of it are very common. “It must not be so bad, maybe you’re too attentive to your body?” etc.
It is also living in financial precariousness. I have not worked for 7 years
If my testimony can help even one patient get a correct diagnosis and be treated optimally quickly, my goal will be achieved.
I thank you for your presence and your openness to our special condition.
Better diagnosis = better treatment = less suffering, both physically and emotionally, and above, all a BETTER QUALITY OF LIFE!