‘They began to compare me with Fiona … ‘
Stories of people with a rare disease ‘acromegaly’ whom the Velikan Foundation, Russia, is helping.
What do the prototype of Shrek, the great kings and Nikolai Valuev have in common? On the evening of the World Day of Distribution of Information about Acromegaly, which is celebrated on November 1st, we tell the stories of those people who have encountered this mysterious disease. About what a difficult path they went through, how they were able to accept and “tame” the disease, including thanks to the support of the unique charitable foundation “Velikan” (The Giant), established in St. Petersburg, Russia.
“Puffy face, arms, legs and the general condition of the “inflated, puffy ball”: every week my reflection in the mirror disgusted not only me, but also others. Yes! Some couldn’t even hide it. The nose – it became just ugly, like from Soviet cartoons about Baba Yaga, and the lips!!! The last drop before a nervous breakdown was comparing me to Fiona from the cartoon Shrek. Weeping … There was an ocean of tears. The vicious circle of “poor condition – fatigue – sweating – change in appearance – enlargement and deformity of facial features – change in the size of arms and legs” began to be replenished with psychological problems – hysterics and terrible depression started.” 43-year-old Anna from Surgut city describes her disease … acromegaly.
This is a rare severe disease caused by the formation of a tumor in the pituitary gland. It provokes an increased release of growth hormone, so patients’ facial features change, become rougher, nose and lips increase, hands and feet grow wider, internal organs increase. By the way, Anna compared herself to Shrek’s fiancee for a reason. It is believed that the prototype of the famous cartoon ogre was the French fighter in the ring Maurice Tillet, who suffered from acromegaly.
Photo: from open sources, Creative Commons
It is also called the disease of athletes and kings. Judging by the remaining portraits, King Charles II of Spain and King Philip IV of Spain suffered from acromegaly. The disease was especially difficult for women. For example, the English nurse Mary Ann Bevan (1874-1933) at the beginning of the twentieth century was recognized as “the ugliest woman in the world.” The delicate, elegant features of a beautiful woman had got coarsened under the influence of the disease, the appearance became of a repulsive kind.
English nurse Mary Ann Bevan
Photo: provided by the Velikan Foundation
But a lot has changed today. And timely diagnosis of the disease, treatment with newest drugs allow a person to live a full happy life. The deputy of the State Duma, and in the past a professional boxer, world heavyweight champion Nikolai Valuev also faced this disease:
‘I have never hidden that such a disease has befallen me. When I first mentioned this disease, a huge number of people, my friends and family, called me on the phone with such a mood that could be described as if I have some kind of inoperable brain tumor. In fact, acromegaly, although not very common, is not so much an out-of-the-ordinary disease that people are exposed to. And it just happened so that it has befallen me.
Photo: provided by the Velikan Foundation
Soviet giant basketball player Alexander Sizonenko was ill with one of the varieties of acromegaly, gigantism. He continued to grow until the end of his life, clothes and shoes were made to order for him. In 1990, the basketball player was recognized as the tallest person in the world.
The unique Velikan Foundation has been helping such huge, but very vulnerable people, for almost 10 years already. Created in St. Petersburg, it took patients from different parts of Russia under its wing. Today it unites 1.5 thousand patients from St. Petersburg, Yekaterinburg, Novosibirsk, Moscow, Krasnodar, Rostov-on-Don, assistance for residents of other regions of Russia is provided remotely. And in 2014, “Giant” established and registered the World Organization “WAPO” (World Alliance of Pituitary Organizations) in the Netherlands, which includes communities of patients with acromegaly from 35 countries of the world!
On the eve of the World Day of Distribution of Information about Acromegaly, which is celebrated on November 1st, we tell stories of people who have encountered this rare disease. About what a difficult path they went through, how they were able to accept and “tame” the disease, including thanks to support of the “Velikan”.
Anna, 43 years old: “The doctor said that he had seen this in University textbooks”
Now Anna, the heroine of the beginning of the article, has taken the disease under control. But for a very long time visits to the doctors did not bring any results. The tests were more or less normal. Her complaints about poor health doctors have been connecting with the “severe climatic zone” in which Anna lived. But no one offered to be examined further.
At the next visit, when I had already lost all my hope, I was asked to show my passport, Anna recalls. Passport! At a doctor’s appointment! The endocrinologist was checking me and the passport photo for a pretty long time, as if I was going through passport control at the airport. The verdict was no less shocking to me: “You know, I’ve seen this in textbooks, but I’m not completely sure. Here are the doctor’s contacts for you – find her, she recently arrived from training.”
“You have a macroadenoma of the pituitary gland, hormone-active .. acromegaly”, a diagnosis was made in the District Hospital that turned Anna’s life upside down. The woman was sent for an operation (under compulsory medical insurance) at the NMC of Endocrinology of the Ministry of Health of Russia. It went well, Anna began to recover quite quickly, the swelling and puffiness went away, Fiona was turning into an ordinary person again. Life became easier psychologically.
Photo: a frame from the movie
“Rehabilitation was normal, but I got back to the “active” life too fast”, says Anna. And six months later, I again felt symptoms that could not be confused with anything else. Swelling, headaches and excess weight returned. It became clear that the disease had not completely receded. In this disease, surgery is the first line of treatment, but it does not always mean complete healing. It depends on the specific location of the tumor. Now I receive a monthly medication that keeps me in a normal state. The lifestyle at the moment dictates the physical condition.
Anna officially has a disability, without it, the patient will not be included into the regional list of drug provision. In addition, disability was associated with partial disablement. The documents for the Disability Examination Commission were prepared by the physician.
“Once faced with such a disease, I read a lot, searched for information on the Internet and came across a group of “Velikan”, this community was like a breath of fresh air”, admits Anna. ‘The understanding itself that you are not alone, that there are people with the same disease, that they can help you – and not only in a word, gives you the aspiration to fight, not to give up.’
Ekaterina, 37 years old: “I was preparing for surgery, but I found out that I was expecting a baby”
Katya found out about her illness at the age of 23, before that, she had been going to the doctors since she was 18, when problems with her periods began. Gynecologists threw their hands up, but a competent endocrinologist still made the correct diagnosis.
I was very scared, some doctors said that this tumor could lead to complete loss of vision, Ekaterina recalls. At first, I was not suggest to do a surgery, doctors prescribed drug therapy. But when the microadenoma became a macroadenoma, I started collecting documents for the operation. But found out that I was expecting a baby.
It was a miracle! Katya tried to get pregnant for 8 years. The doctors selected the correct protocol for pregnancy management, taking into account the disease, and in 2016, Ekaterina’s first daughter was born. And three years later she became a mother for the second time.
Both daughters were born absolutely healthy. The children are now almost 5 years old and 1,8 years old. However, after the second caesarean, my eyesight has gone down, now I wear glasses. But in general everything is fine, Ekaterina admits.
Like many others, Katya was greatly supported in the fight against the disease by the patient support group “Velikan”, she found out about the foundation quite by accident on Instagram.
I periodically check the group, read the posts of those who have just been diagnosed with a serious diagnosis, those who have been struggling with the disease for more than one year, I send them words of support in a personal account, people write back to me, ask questions, ask for advice on how to cope with the disease and more, says Ekaterina.
Irina, 49 years old: “I remember hearing a girl say to her father: “Dad, look at how big that lady’s nose is!”
By that time, I had already read about acromegaly on the internet, and the thought of the possibility of such a disease terrified me. Therefore, I was glad that the tests were good, and the cycle had normalized by that time” Irina recalls. But fatigue did not go away, I again passed the analysis in another laboratory and growth hormone was exceeded 4.5 times. A MRI revealed a large pituitary tumor.
In 2006, Irina underwent surgery.
At discharge, the surgeon said that I was healthy, advised me to visit the regional endocrinologist regularly. I was very happy, I felt well and for many years I tried not to remember about the disease.
It seemed that the disease really receded: from the moment of the operation, the feet stopped growing (initially it increased from 37.5 to 40-41 sizes), the roughness in the facial features disappeared, and in order to return the former beautiful nose, Irina had rhinoplasty. But over the years, fatigue and weakness began to appear, Irina blamed it on high blood pressure, from which she still could not find the right pills. So, I turned to an endocrinologist, as the surgeon recommended, and I did not take any medications. In July 2019, on the way to work, she suddenly lost consciousness.
I woke up in the ambulance, they told me that I had an epilepsy attack. At the hospital, I had a CT scan, which revealed a large formation in the pituitary gland. Since that time, I realized that the disease is for life, hiding from it is not the best strategy, the woman admits.
It turned out that over the years of ignoring the disease, the informational environment had changed, and many new methods of treating acromegaly have appeared. I got examined at the Almazov Clinic, Saint-Peterburg.
Painting by the artist Adolf Richard Hoelzel “House of Prayers”
It depicts a woman with acromegaly: the coarsening of facial features, enlarged nose and lips are visible.
Photo: provided by the Velikan Foundation
The second operation was successful. The tumor was able to be removed by 70 percent, and lifelong drug therapy was prescribed.
Irina believes that a great success on the way of her treatment was the acquaintance with the “Velikan” Foundation, which unites patients with acromegaly.
When you read the stories of people who find themselves in a similar situation, you understand that you are not alone and there is hope for remission, there is strength to fight. In addition, the “Velikan” gives the opportunity to do the blood test for IPFR-1 (insulin-like growth factor) for free, and it was its team that made it possible for the uninterrupted availability of the necessary medicine in pharmacies.
And the foundation, in turn, was helped by Nikolai Valuev:
From the ” Velikan” I learned that there is a new foreign drug of broader action, let’s say, cleaner, which has fewer side effects, it is certified in all advanced countries of the world, but not certified in Russia, as this is associated with great difficulties.
Therefore, difficulties were created in prescribing this drug by doctors of leading endocrinological centers. In 2019, a conference was held at which I told what difficulties patients with acromegaly face in Russia. That many of them require treatment with expensive drugs that are available abroad, that such a drug should be put into effect and certified in Russia as soon as possible in order to be able to treat patients with acromegaly. As a result, the State Duma added the drug to the list of life-saving medication (LSM), the newest foreign drug, which made it available to people with severe endocrine disease acromegaly. Those patients who seek help after surgery or are prescribed to drug therapy can receive any medications for the treatment of acromegaly free of charge as prescribed by a doctor.
Acromegaly is one of those diseases that changes the appearance of a person. In Russia, it’s hard when you look different, it stigmatizes,” says Irina. I remember walking down the street and I heard a little girl say to her father: “Dad, look at how big that lady’s nose is!” It hurts to be ugly, to be different from everyone else. But it’s not just about appearance, the disease slowly destroys the body from the inside, if you do not take any measures. Therefore, at the slightest signs of hormonal abnormalities, you need to consult a doctor, do not let the treatment course “to go on it’s own way”.
It is necessary to consult an endocrinologist if you notice the following symptoms:
- change of appearance;
- increasing the size of the hands, feet;
- excessive sweating;
- muscle weakness;
- numbness of fingers and toes;
- reduced mobility and joint pain;
- deterioration (narrowing of the fields) of vision;
Ekaterina Andrusova, Chairman of the Board of the “Velikan” Foundation:
You will be surprised, but world statistics shows that pituitary adenoma occurs in every fourth human being. But in rare cases – 1 per 10,000 population – the adenoma begins to produce excess growth hormone and becomes a benign somatotropinoma, which with its hormonal activity begins to affect the entire body. The pituitary gland, as the conductor of the whole organism, can unnecessarily produce other hormones: prolactin, cortisol, adrenocorticotropic hormone ACTH, and there are also gonadotropinomas (excess hormones regulating the activity of the sex glands). A systematic study of patients who died from a variety of diseases shows the presence of pituitary tumors in 6.8-22.6% of cases. In fact, neither doctors nor patients in many cases even suspect their existence.
Acromegaly at the initial stage proceeds imperceptibly and it may take 5-8 years before the diagnosis. The first symptoms can be attributed to various neurological diseases: frequent headaches, swelling of the face, and the condition in the morning as after a heavy hangover. There may be a narrowing of the visual fields. Excessive irritability, short temper, fatigue and a constant desire to sleep, poor memory, as well as excessive sweating during sleep, snoring and apnea. Here’s another symptom: children, when they grow up, fly in their sleep, but adults fly in their sleep if they produce excessive growth hormone.
When the disease has been causing irreparable harm to the body for 5-8 years and irreversible consequences occur, then we see how the feet has increased by 1-2 sizes for no apparent reason, the wedding ring, gloves, fingers on the hands have become like sausages. The appearance has changed and the facial features have hardened. The bite changed, a gap appeared between the front teeth and the lower jaw moved forward. Your brow ridges have enlarged and your nose has changed. Depression, general weakness, constant desire to sleep, nervous tension, constant breakdowns. Wrist syndrome.
Thanks to the “Velikan” Foundation in Russia, you can get complete informational support on the course of the disease and its treatment. This is truly priceless, a great chance for a healthy full life and complete remission.
You can ask for help on the Velikan’s website in the section “About us” >> Get help. (link: https://velikan.info/about/help/)
Source and translation by ‘Velikan’, RussiaLeave a reply