In the popular Soviet film "Amphibian Man", the protagonist Ichthyander is a handsome, attractive young man, from whom you cannot take your eyes off. But a fairy tale is a fairy tale so as not to be a reality. In real life, there are also "ichthyandras" - this is the name of people suffering from a rare hereditary disease of harlequin ichthyosis. Unlike the character of Vladimir Korenev, you cannot look at these sufferers without tears.
All 20-year-old girls dream of love - this is an undeniable fact. But Hunter Steinitz from Pittsburgh, Pennsylvania, USA, has one thing only - to get well. Unfortunately, the girl's dreams are never destined to come true. From the first days, the American woman has been sick with a terrible and incurable disease called Harlequin-type ichthyosis.
This is a congenital disease. When a child is born, his skin is covered with gray-beige scales, the thickness of which reaches 1 cm. Deep cracks can be seen between them. The hard scales are tightly stuck together, with the result that the baby's mouth is either stretched or narrowed so that the feeding tube can hardly pass through the lips.
Due to skin abnormalities, the eyelids are twisted, the auricles are damaged, nails are often missing, there are bridges between the fingers and toes, in addition, skeletal defects are observed. Due to the high tissue density, the hair follicles are blocked, so there is not a single hair on the body.
The body of a child born with such a diagnosis is unable to regulate the water balance in the tissues and cannot fight against pathogenic microbes. In this regard, most children die immediately after birth from a lack of water in the body or from microbes. For this reason, doctors consider the disease incompatible with life.
If a baby with Harlequin's ichthyosis does not die in the first months after birth, his condition remains very difficult, and the probability of living to 12-13 years old is 2-3 percent. Only one of several hundred patients lives up to 18 years old. Hunter Steinitz is a rare specimen, she has been living for 21 years!
Infants with Harlequin Ichthyosis often die shortly after birth and look like characters in horror films
Promotional video:
LIKE A FISH IN WATER
Every day Hunter is a struggle to survive. The morning starts the same way - with a bath. The girl sits in warm water for a long time - two hours. This is a necessary measure, because the skin is sorely lacking in water. In order for the skin to absorb as much moisture as possible, the poor man rubs it with various lotions, oils and balms.
Rubbing has to be repeated several times a day, otherwise the body is threatened with dehydration. Hunter is a regular at the local pool. Other swimmers are used to a strange visitor with skin defects, but for beginners such a sight is a wonder. However, this is how they react to it not only in the pool - everywhere.
At first, many try to bypass the girl, and some have tantrums if she accidentally touches them. Most people unknowingly believe that the disease is contagious. Previously, Hunter was very worried when she saw that people bypassed her by the tenth road, but now she herself began to approach strangers and explain that she would not harm them.
Harlequin ichthyosis is not spread from person to person. “One of the most difficult problems in the life of patients with harlequin ichthyosis is the reaction of others. At the first meeting, most people experience a powerful shock: they do not know what to say or how to act,”says the persistent girl.
WILL LIVE
People can be understood too. Hunter Steinitz, bitterly to admit, looks like she's from a horror movie. It seems that the girl was badly damaged in the fire and all her skin was burned. Skin color is not the same as everyone else's - pale pink, but bright red like beets. The whole body is covered with a crust.
The skin is constantly cracking, which makes it seem as if the girl is covered with scales, like a fish. The fingers and toes do not bend well, the skin is so thick. The eyelids are turned inside out and Hunter cannot close his eyes. Moreover, the poor thing can't even blink.
When a girl sleeps, her eyes still remain open. There are no eyebrows and no hair on the head. Therefore, Hunter wears a wig all his life. No matter how hot it is, she never sweats.
However, a serious illness hardened the spirit of the girl: several years ago she successfully graduated from college, she enjoys communicating on Facebook pages. To the best of her strength and capabilities, the young American leads an active lifestyle. In recent years, she regularly gives lectures to schoolchildren and students, telling them about her illness and the difficulties that are full of her life and the life of her comrades in misfortune.
This girl's name is Brenna, she is 4 years old. Her mother uses organic coconut oil to soften the baby's skin.
This girl is called Mui Thomas, she is 22 years old and she is also one of the rare lucky ones who survived to this age.
Stephanie Turner, 23, lives in Arkansas. She is the mother of two children who luckily did not inherit her condition.
TO NOT GIVE UP
Englishwoman Nelly Shaheen (real name Nasrin), born in 1987, did not die in infancy either. Now Nelly is 32 years old and she is the absolute record holder for those with a similar disease. Despite her serious illness, Nelly leads an active lifestyle. He is fond of sports and goes shopping with great pleasure.
Of course, illness seriously complicates her life. What are the sidelong glances of others, and any trifle like a splinter from a street bench can cause an infectious disease in a girl and throw her into bed for several weeks.
But the Englishwoman does not give up. And this despite the fact that an insidious disease claimed the lives of her four brothers and sisters (the family had nine children). Shahin overcame the isolation usual for such patients and, as they say, went out to people.
Not only did she receive an education, she also constantly participates in television shows and actively speaks on behalf of the same sufferers. There are now 14 people with harlequin ichthyosis in the United Kingdom. Nelly actively communicates with other patients using Facebook, exchanging subtleties and techniques to help make life easier.
RARE DISEASE
Ichthyosis was first described in 1750 by a priest from South Carolina, USA, the Rev. Oliver Hart, “On Thursday, April 5, I went to see a unique specimen - a child born at night to a certain Mary Evans. Everyone who watched the baby was confused, and I still don't know how to describe what I saw. Its skin is dry and hard and resembles fish scales. The mouth is large and open.
The baby was born with two holes for a nose. The eyes are huge, bloodshot and bulging like a plum. The ears are also absent, with only small holes in their place. The arms and legs are swollen and hard as stone. The baby lived for about two days and died."
To date, scientists know about 30 varieties of ichthyosis - a severe congenital pathology, the causes of which have not yet been established.