Amyotrophic lateral sclerosis (ALS) is an incurable disease of the central nervous system in which motor neurons are damaged. The patient gradually develops paralysis and atrophy of all muscles, including those that are responsible for breathing. In this case, the person remains conscious until the last.
The causes of ALS are still unknown. Only in 5% of cases the disease is inherited. At the same time, patients are quite able to conceive, bear and give birth to a healthy baby: modern perinatal technologies allow all this.
Although ALS is on the list of medical indications for abortion, the decision to maintain a pregnancy is, by law, made by a woman. But in practice, many Russian doctors are afraid to take responsibility for complex patients, so they try to put pressure on future parents: they refuse to register in the antenatal clinic or endlessly direct them from the hospital to the hospital. RT talks about three families who were able to overcome all these barriers for the sake of the child.
"The thirteenth case"
Muscovites Pavel and Svetlana Golovinsky are familiar from the first class. Married when both were 23 years old. When Svetlana was already waiting for her third child, her arm began to work poorly.
“I thought that Sveta's nerve pinched. We decided that we would give birth and go to the doctor to adjust the spine, ”recalls Pavel Golovinsky.
By the time of birth, Svetlana had already weakened both hands. When the son was one and a half months old, the Golovinsky learned a terrible diagnosis, says Pavel: “The doctor took me aside and said that Sveta had 30 months left from his practice, then she would stop breathing. We have not accepted his statistics and are not accepting them so far - for 11 years now. ”
After giving birth, Svetlana’s condition began to worsen. And on the 30th month, which, in fact, the doctor “unsubscribed” to her, the couple found out that they would have a fourth child.
That was almost six years ago. By that time, Svetlana could only move a little with the help of her husband. Hands completely refused, the gait was constrained and unstable.
In the antenatal clinic, the head rude to Pavel: "She said that I was irresponsible, I want to destroy my wife."
Pregnancy was eventually conducted at the Center for Traditional Obstetrics. The tests throughout the pregnancy were in order, the fetus developed normally. The question arose where to give birth. Doctors said that it is possible to have a cesarean section if there is a resuscitation in the hospital, because there may be a respiratory distress in the woman in labor. But they did not want to take responsibility for the woman in any of the clinics where Pavel had been contacting for ten weeks: as soon as they found out the diagnosis, they stated that “you are not our profile” or presented other excuses.
“I see on the Internet a few days before 40 weeks that the maternity hospital was opened at the 7th city hospital, with intensive care,” continues Pavel Golovinsky. - I’m calling, they tell me that they accept everyone. Sveta was barely walking when we arrived there. But on the spot, the doctors again began to try to get rid of us, they were indignant at who allowed such a woman in labor. And who banned? Or, for example, a pediatrician comes and asks: "And what, will give birth?". As if there are more options! ”
As a result, Svetlana was given two days to prepare and had a cesarean section. According to Pavel, most of the work was with anesthesiologists: it was necessary to choose anesthesia so that his wife then did not “hang” on mechanical ventilation. The operation was successful, Svetlana woke up and began to breathe herself. But the pressure of the doctors did not end there.
“She was urged to bandage the pipes so that the“ maniac ”, that is, I,“ did nothing more with her, ”Pavel lists. - Refused, of course. They offered to give an injection to stop lactation - after all, the load on the body. She also refused. Sveta fed Danyu for nine months. ”
It is noteworthy that from the fourth birth the state of Svetlana remains stable.
“Health has not deteriorated, maybe only the neck muscles have weakened a little,” says Pavel. “Depression is gone, and the symptoms diminished when Sveta believed.”
Now his wife is breathing, eating, talking with children - and the husband left work to devote himself to his family.
“When Sveta gave birth to Daniel, we began to call ourselves“ The Thirteenth Case, ”because at that time only about a dozen cases of birth with ALS in the world were known,” added Pavel Golovinsky.
“They scared that both the mother and the child would die”
The first signs of illness in a resident of Cheboksary Tatyana Polkova appeared in the winter of 2016: she fell several times out of the blue. Doctors made various diagnoses and had surgery. In October 2017, Tatyana fell and lost consciousness: their son informed Maxim Kartaev about this to her husband. Soon not only legs but also arms began to weaken. In August 2018, Tatyana was diagnosed with ALS. And the next year, she found out that she was pregnant with her second child.
- © Photo from the personal archive
“When we arrived at the local perinatal center, the doctors first spoke in a raised voice with Tanya, and then with me. Like, who allowed you to give birth with such a disease? They frightened that the child would be born with a pathology, that either the mother, or the child, or both, would surely die, and that I would be to blame. In the end, they recalled that Tanya was registered in Nizhny Novgorod, which means that in Cheboksary she allegedly cannot be registered, ”recalls Kartaev.
For almost seven months, the future parents rushed between three hospitals in two cities: at first Tatyana and Maxim were offered to come, and then refused to accept. In Nizhny Novgorod they said they were afraid for the condition of the woman in labor and the child, they tried to find the pathology of the fetus and were sent to the perinatal center in Cheboksary. And there they again recalled the lack of local registration and sent back to Nizhny. Maxim went to Moscow to arrange with one of the local maternity hospitals, but Tatyana started giving birth from worries and relocations - two months before the expected date - and she was sent to the nearest hospital in Cheboksary at that time.
Daughter Sonya was born healthy, but since she was premature, she was placed in the intensive care unit of the center. Tatyana was transferred to the intensive care unit for pregnant women, and then to the usual one. The daughter was not even shown to her mother immediately after she was born, arguing that Tatyana had "weak hands, she would not hold the baby."
Mom and daughter spent two weeks apart. Maxim visited both of them all the time: “In the intensive care unit, Tanya picked up bronchitis, but as soon as she was allowed to go out, I immediately took my wife and drove me to another building - to Sonya.”
The daughter of Tatyana and Maxim is now actively gaining weight, the discharge is scheduled in two to three weeks. Tatyana’s condition also returned to normal, says Kartaev: “She can already stand, in a few days, I hope, she will go by herself. Hands, of course, are weak. Swearing with doctors took a lot of strength. Tanya lost weight, became just like a match. Now is slowly recovering. ”
Maxim does not want to punish doctors who actually contributed to the situation without providing timely support.
“But I would like at least an apology. Doctors actually behaved ugly. We knew that we were taking a big risk, and were ready for this. Sonya is our long-awaited child. I have a feeling that the disease is not with Tanya, but with both of us, and together we go through all the difficulties, ”the man says.
The family tries not to get hung up on negative emotions, Maxim emphasizes: “They say that with ALS you have to live for today. But we live today, tomorrow and the day after tomorrow. ”
"A more active life than before the disease"
Muscovites Tatyana and Pavel Shestimerov have two children: daughter Lyuba and son Cyril. In the spring of 1999, Tatyana, pregnant with a younger one, began to limp. Then the couple thought it was a sprain. Soon the second leg began to obey worse, her hands began to weaken.
“They gave birth themselves, the son was born healthy, Tatusya fed him for ten months. But then obvious deterioration began, it was necessary to stop feeding and go to hospital. They found out the diagnosis there, ”says Pavel.
The family did not encounter any opposition from doctors during pregnancy and childbirth, because the diagnosis was not made at that time: you never know why the expectant mother is limping, he adds.
20 years ago there was almost no information about ALS in Russia, Pavel recalls: “Only a film about musician Vladimir Migul (died from ALS in 1996. - RT ) and another friend got a brochure with terrible predictions. But we decided that we should not concentrate on this, but we should raise children and give them as much as possible. ”
- © Photo from the personal archive
A lot of doctors told Pavel that they had to prepare to raise their children alone, after two or three years Tatiana would have to switch to the ventilator, and ten years ago, taking her to the hospital with pneumonia, the doctors immediately gave her husband an engagement ring and said, “in a couple of days come for the rest. "
“Now we have a more active lifestyle than before the disease,” says Pavel. - One of the incentives - many people with ALS are equal to us. We have a longer and less rapid deterioration, so the symptoms were by and large not a shock. But all the same, Tatyusha can’t do anything now, just speak, but breathes through the tracheostomy. ”
“No one can force an abortion”
The list of medical indications for artificial termination of pregnancy, approved by the Ministry of Health in 2007, includes ALS in severe cases. There are also diseases of the expectant mother, such as tuberculosis (some forms), malignant neoplasms requiring chemotherapy or radiation therapy in the pelvic area, hereditary mental disorders, severe epilepsy, a number of cases of heart disease, aortic aneurysm, congenital absence of limbs, and also "the state of physiological immaturity of a pregnant woman before reaching the age of 15 years."
“Any of these grounds is nothing more than the conclusion of a commission that gives its recommendation on the artificial termination of pregnancy. The decision on this is made only by the woman, this is her right. Doctors have no right to pressure her, force her to have an abortion, not to register her in a antenatal clinic or refuse to accept her in a maternity hospital, ”Polina Gabay, founder of the Faculty of Medical Law, explained to RT.
If doctors refuse to register a pregnant woman in a antenatal clinic, then parents need to write an appropriate appeal and demand an official written refusal, and you can already file a complaint with the Ministry of Health or go to court, the expert added.
The recommendation does not deprive patients of the right to become pregnant and bear a child, says Natalia Lugovaya, director general of the charity foundation for helping people with BAS “Live Now”. But such women scare doctors, they don’t understand what to do even with patients without pregnancy - unless they just send them home to die.
“In the world, pregnancy with ALS is a very real story, they conduct conversations with the family, explain all the consequences and risks. And I haven’t heard of a single case when a woman abroad would be denied pregnancy if she has ALS, ”she explained to RT.
At the same time, taking into account modern perinatal technologies, it is quite possible to maintain a pregnancy and successfully take birth: the baby is developing normally.
“UAS is inherited extremely rarely,” continues Natalya Lugovaya. - Yes, a woman usually cannot give birth herself, but there is a cesarean section. The only thing worth paying special attention to is how to organize anesthesia so that the patient then returns to independent breathing. ”
The problem is that in Russia there is still not a single prescribed protocol for the care of such patients, states the chapter "Live now."
“With the support of our foundation’s experts, clinical recommendations for ALS were written and they underwent an initial discussion, but were stuck on signatures at the Ministry of Health. Since 2017, we cannot achieve that the department finally signed them. Therefore, most doctors, especially regional ones, unanimously say that nothing can be done for patients with ALS. This is not true. It is possible to make life easier for people and, in a sense, extend it by proper care and improvement of its quality, ”summed up Lugovaya.