The San Francisco Municipality, the second largest in Zulia State, has been the home for more than two centuries of many people suffering from Huntington’s in the populous neighborhood of San Luis. The region has the largest number of patients with this condition in the world. An implacable, deadly pathology that still has no cure.
By La Patilla
Jul 20, 2022
Huntington’s Disease (HD) is hereditary with fatal prognosis due to its neurodegenerative voracity. It is transmitted from generation to generation, since it is caused by a mutation of the gene that is located in an arm on chromosome 4, which codes for a protein called Hungtingtin.
Its Origins in Zulia
Doctor Roberto Weiser established more than twenty years ago that Huntington’s disease, as it is also known, came to Zulia through a man of European origin, who settled on the shores of Lake Maracaibo and had several children in that area now called San Luis, which is a fishing village.
Since it was a closed community, it became a place that favored the congenital spread of the disease in the area. At the time, Dr. Weiser asserted that as the gene is passed from parent to child, the number of repeats in the DNA of the disease-producing protein tends to get larger. The higher the number of repeats, the greater the chance that a person will develop symptoms at a younger age.
An urban myth that gains strength in the streets of San Luis is that the transmission of this disease is on the rise due to inbreeding and consanguinity between members of the same group. Therefore, as the disease is passed from parent to child, symptoms develop at younger and younger ages.
Nutritionist Miriam Villalobos said that she treated patients with Huntington’s more than two decades ago. The genetic probability of inheriting this disease, described as “rare”, is 50% if a father or mother suffers from it.
Left to Their Fate
The San Luis neighborhood is where there is the largest number of patients with Huntington’s in the world
In the streets of the San Luis neighborhood it is common to see people walking around with the disease. Some residents of the area have come to think that they dance while they walk, but it is when the disease that affects them begins to take over the body that involuntary movements begin.
When Huntington reaches phase three, the involuntary movements are every second. They lose the ability to fend for themselves and become bedridden. The trachea also atrophies and they cannot swallow easily.
Norma Gotera is the mother of three women with Huntington’s disease. Her husband and her eldest daughter died from the same pathology. She says that her daughters are orphans by their father and also by the Venezuelan State, which completely abandoned Huntington’s patients in Zulia State.
Ana María Soto is a nurse. She and her sister Alexandra suffer from Huntington’s disease
Alexandra, her second daughter, is in a critical condition: she does not coordinate her body movements, she no longer speaks, she does not walk, she eats little food, not only because of the illness, but also because her family lives in extreme poverty and cannot guarantee the balanced diet that Alexandra needs. She has suffered from the disease for 10 years. Her health deteriorates more and more.
“Every day I ask God to help us. I can’t work because I don’t have anyone to take care of Alexandra. Honestly when I married her late father, I didn’t think he could have inherited it. My father-in-law also had Huntington’s. When my eldest daughter started with the symptoms, I asked God to free them from that evil, but that’s not the way it happened,” says Norma, who cries out for help for her daughter.
Alexandra is in the phase where she can only ingest liquids, because her trachea is already closing. “It hurts me to see how the lives of my two daughters fade little by little. And the fear grows, because my youngest daughter has two sons, and it is likely that they will inherit this evil,” said a moved Norma.
More Understanding
A woman with San Vito disease in Zulia
With tears in her eyes, she told the “La Patilla” team how they are discriminated against, not only in her neighborhood, but when they have to move to other places. “They look at us badly, they avoid us completely and call us ‘Sanviteros’ (from “Mal de San Vito”, common name for the affliction). We almost didn’t go out for fear of being mocked.”
Gotera, as her mother, said that they did not choose to be like that, it is something that came in her genes, from her father. She asked the authorities to educate society so that Huntington’s patients are not discriminated against.
In Alexandra the exaggerated involuntary movements do not stop. Nutritionist Miriam Villalobos explains that HD patients burn 7,000 calories a day. That’s why they look malnourished. She would need to eat at least seven times a day, but her family cannot cover this need. Sometimes, more and more frequently, she only eats once a day.
Those who suffer from Huntington’s may present changes in affection, hypersexuality and impulsiveness in addition to compromising some neurological functions such as memory, language and the loss of other mental activities, said specialist Villalobos.
“They did not choose to be born that way”
According to the information provided by residents of the San Luis neighborhood, around three thousand people live there. For everyone in the area it is part of their daily life to share with people who suffer from the disease and see how the symptoms change from one day to the next in those affected.
In San Luis there is a module of the Barrio Adentro Mission that cares for patients with this disease. They currently serve 130 patients with this health condition whose ages range from 20 to 60 years.
Rey Valera is a surgeon and psychiatrist. He considers it very important that society accept them and not discriminate against them. “They did not ask nor did they choose to be born with that disease. As a society, we must join efforts to support them and make this deadly disease more bearable,” he said.
The specialist indicated that a patient needs a multidisciplinary team in medical and psychological care, made up of a geneticist, neurologist, psychiatrist, physiatrist, psychologist and social worker. Currently, these 130 patients receive psychological and neurological care from doctors Rey Valera and Gabriela Ríos, who took on this mission out of vocation and because they believe that illness is a matter that requires everyone’s help.
Fear of Rejection
Yoelvis Soto is 33 years old, and from the age of 26 he began to manifest symptoms. Today, involuntary movements have taken over his body, although fortunately for him, he can still walk. He has two children, and the ten-year-old boy has had symptoms for two months, such as nervous tics in his eyes and shoulders.
Yoelvis, in a conversation with La Patilla, said that the strongest part of this disease is rejection and ridicule. “Sometimes when I went out to do errands and could work, they told me blessed San Vitero, witch, demon-possessed, get out of here.” He said that they pushed him around and harassed him.
“As a result of that I did not leave this area where we are all accepted and loved. The disease of San Vito is true and it is active here in San Luis.”
Argenis Gotera is 29 years old and is a young man with a vocation for community service. His father died of Huntington’s disease. His brother is now 17 years old and also has HD.
He says that his life has a before and an after. Everything changed for Argenis in 2017. “Since I can remember, my father, two aunts and an uncle suffer from Huntington’s. That is not easy. I denied my reality, I didn’t accept it because at high school they made fun of me. I didn’t tell people that my family had it, although here in the neighborhood it’s normal. Every family has at least one member with this illness,” he said.
Argenis recounts that six years ago his younger brother, Angervis, began to blink his eyes, his shoulders trembled. His relatives and even himself told Angervis that it will be gone by the morning, to stay put. After a few months, his brother began to have difficulty walking, he fell playing soccer and many children made fun of the minor.
Argenis as the older brother told him to rest, that he should not play. One afternoon, thoughtful, he looked at his brother and told his mother that his brother had Huntington’s.
Light in the Darkness
Since then, Argenis has been doing social work in the community of San Luis. He said that as a son of that neighborhood, he is hurt by the harsh reality that many families live because they don’t have the resources to pay for proper treatment of a disease that requires attention, care and food.
In the company of other young people they do social work, seek help from countrymen, companies, public entities, NGOs and foundations to collaborate with Huntington’s patients.
Gabriela Ríos, neurologist and pediatrician, is a volunteer of the “Habitat Luz” foundation. She recounted her experience caring for Huntington’s patients since 2019, when she joined this work team.
Within the foundation, she coordinates the “Brazo” program (Arm program) that cares for the children of parents with Huntington’s disease. She said that from the institution they provide comprehensive, medical, psychological and educational care.
The neurologist said that they currently serve 75 children at risk in the town of San Luis, and 164 in Barranquitas, an area located between the municipalities of “La Cañada de Urdaneta” and “Villa del Rosario”, through “Habitat Luz”.
These infants do not yet have symptoms, but are under medical supervision because they could develop the disease.
To know if the children of parents with San Vito’s disease can suffer from it there are specific genetic studies. Some samples can be processed in Zulia State, and others must be sent to Caracas. It should also be followed up frequently by a geneticist to determine if the gene is active in each person.
A Door That Was Closed
Assembly for the collection of signatures that seeks the reopening of the Casa Hogar
In the San Francisco municipality, in the San Luis neighborhood, there was the “Casa Hogar Corea de Huntington, Amor y Fe”, a place created by international organizations. It also received the support of the government of Zulia since its opening in 1999. According to neighbors, nothing is currently known about this institution, which is no longer providing aid services.
It has been closed for more than 10 years and nobody knows the reason for its closure. There, the sick and their families received medical and social care. In the opinion of the residents and relatives of the area, since the closure of the home a real ordeal began, the most terrible nightmare for those who already suffered from Huntington’s and the new cases that have arisen, since they were left unprotected, without anyone’s support or protection.
They denounced that the outgoing former mayor of the regime in the San Francisco Municipality, Dirwings Arrieta, changed all the locks of the home and never reopened the care center. The whereabouts of the keys are also unknown. This event described as “a crime” occurred in mid-2018.
The neighbors told that they received quality care there. The foster home was also looted by the “friends of the other’s (stuff)”.
In view of this situation, the relatives of the Huntington’s patients went to the municipal and legislative government instances to seek a solution,to try to reopen this medical care center.
An assembly was held in front of the “Casa Hogar Corea de Huntington, Amor y Fe”, which was attended by the new Mayor Gustavo Fernández, legislators Alejandra Barrientos, Yover Sánchez, as well as Argenis Gotera, psychiatrist Ray Valero and others involved in this issue. At the meeting, signatures were collected to request the State Attorney’s Office to demand the reopening of the center.
Legislator Alejandra Barrientos reported that they are in the investigation phase to find out the legal status of the home. The case is handled by the Síndico (Ombudsman Office) of the San Francisco Municipality and the Zulia State Attorney’s Office.
“After the legal owners are located or we know who the legal representatives are, the conversations will begin for the reopening of the center and determine who will be responsible for the administration of the home and start in this place the care services that have been provided in the Barrio Adentro module lately.”
For those who extend a helping hand to the families and patients of Huntington’s, social work does not stop. They continue to search for help to care for those affected by this deadly disease that is so stigmatized by society.
The Chavista healthcare authorities have not issued any public statement on the situation of the patients with this disease. The relatives cry out for the help of the central government, they pray that through apathy they do not leave them in abandonment and destitute.
…
Read More: La Patilla – For Huntington’s patients, life is extinguished between hunger, discrimination and abandonment of the Chavista regime
…