ISTANBUL- The healing of Havin Ömür Aydar, who has been struggling with SMA1 disease for the last two years, depends on her Zolgensma treatment. As the family does not have the means to pay for medical care and nurse for their daughter, it is her mother, Mürüvvet Aydar, who takes care of the six medical devices and provides her little girl with daily care at home.  

 

We want to attractyour attention on the fact that SMA (Spinal Muscular Atrophy) patients in Turkey number around one per six thousand. This disease had often took place in news in Turkey those last days. What is this SMA disease? Is it possible to cure?  First of all, concerning SMA patients,as the genes SMN1 and SMN2 do not produce enough proteins, the cell of the motor nerves cannot recieve enough, this causes a dysfunction of the muscles which can no longer funstion correctly.  The SMA symptoms are divided into four types depending of the age of onset, the speed of spreading and the mobility of the individual. If SMA occurs at an early age, the course of the disease becomes more severe.

 

First treatment found in 2016

 

SMA is fatal because of the gene disorders and drug treatment was found for the first time in 2016.  The drug called nusinersen(Spinraza®), accepted by the American Food and Drug Administration (FDA), is injected into the cerebrospinal fluid to increase SMN synthesis. This drug also aims to delay the death of motor neurons. While clinical studies continue, tree different treatment methods are currently used for SMA, with the Spinraza, Zolgensma and Risdiplam.

 

Zolgensma gave hope to families

 

The Zolgensma treatment, which was found in 2019, applied at once and performed by replacing the SMN1 gene in patients with a working gene version, is currently known as the most expensive treatment method in the world. Although this treatment is hoped for by many families, its price, about $ 2 million has caused disappointment in families. At the beginning of 2020, the Roche medicine company started to sell the drug Risdiplam with the brand name Evrysdi. Risdiplam is the first and only oral drug produced for SMA disease.

 

For now, the Turkish Social Security Institution (SGK)  is only advancing the funds and only for the Spinraz, on condition of repaying later.  But people need to fulfill some criterias of this treatment. The USA applies the Zolgensma treatment for children younger than the age of two, while European countries accept children under 21 kilograms.

 

Havin awaits for treatment

 

Families of children who struggle with SMA in Turkey, they try to make their voice heard by the public. These families require the Zolgensma treatment. Many of them, after receiving the funds from money campaigns, choose to go abroad for their children to receive the best treatment available. Havin is only 2 years and a half old and awaits for treatment.

 

Havin's mother, Mürüvvet Aydar, has become like a nurse for her daughter. She did a lot of researches on the disease and learned nursing by her own means. Now she can't leave her daughter for a minute. The family has even convert a room for the medical equipment of their daughter in their home. Havin's breathing and feeding strictly depends of her medical devices.  Mürüvvet says the family get to know about their daughter's disease when she was only 50 days old. She also said that the SMA disease has not been foun in Havin's mother and father families untill now. From the family doctor to the specialists, passing by the pediatrician, and the neurologist, the family had to struggle hard to determine what kind of deasease Havin was suffering of, as to get appointments at the hospital. As the family didn't have enough money to take their daughter to a private hospital, they took a gene test at the state hospital, which resulted, after two months, that Havin had SMA. Pretty soon, the little girl stopped feeding at her mother breast as she lost her swallowing and sucking reflexes, and was sent straight to the hospital to receive Spinraza treamtment.’’

 

The 5th dose could be given after six months.

 

Mürüvvet stated that early diagnosises and treatments are of a vital importance in this disease. Havin has got at first positive results from the treatment.  Mürrüvet emphasizes that Havin was not able to receive the fifth dose due to the fact that the doctors didn't accept more critical patients at the hospital during the covid pandemic wave, and had to wait the 6th month to finally get it.  She emphasizes that due to the lack of money and means, they could not go to the private clinic and had to go to the state hospital. There, Havin was not able to receive her 5th dose and had to wait untill the end of her 6th month to finally receive her treatment again. She explained ''My child also lost the function she had gained until then. She became addicted to intubation. Later, it was decided for Havin to have a general surgery for her reflux problems. But she couldn't have it as she had a too big liver. That said, the children of our friends who had general surgery were able to be discharged after receiving their fifth dose. Havin had to stay in the hospital for a year and a half because of a leakage in her lungs. I could not see my daughter for months due to the pandemic. My child experienced the lack of her parents, her sister and love also due to the pandemic. As a result, she started to encounter psychological difficulties.

 

A tracheostomy was performed

 

Underlining that Havin's could not get her 6th dose of medicine for eight months due to the bureaucracy, Mürüvvet points out that her child has completely lost the abilities she has gained by the past. Mürüvvet said that Havin, while she was intubated, had a cardiac arrest, and that they were required by the medical staff to go quick at the bedside of their daughter. She continued as follows: “Our child was put to coma for about ten days. I phoned the hospital twice a day to learn about my child's condition. Sometimes, those who were on the phone were mad at me, asking me why was I always calling. I am her mother so I have the right to ask about my child's situation.

 

At the end of ten days, they woke her up. There was no damage in the brain. Later on, on the advice of the medical staff, it was hastly decided to perform a tracheostomy on our child. At that time, going to the hospital, I had 8 bus changes for being able of seeing my Havin for only 15 minutes a day.  And for that, I had to leave my other daughter at my mother's home. Finally, after my daughter received her 6th dose, they discharged her and we took her back home.

 

'A fungal infection was detected in her respiratory tract'

 

Mürüvvet said that they had to take Havin to the emergency department 4 times in a month after she was discharged from the hospital. She stated that after Havin has passed some tests, the medical staff discovered that the little girl had contracted a fungal infection in her respiratory tract, due to the fact that she was connected to her breathing devices for too long. When we went to the hospital that followed our daughter, they said that they could not accept her into the intensive care services anymore as the hospital has been turned into a pandemic hospital.. Even there was still an empty room, they did not accept our daughter no more. So when her father took her to the hospital for a breathing test, she got worse and he had to take her to the rooms of the intensive care unit.

 

'My child has been fighting death for 30 months'

 

 Mürüvvet said that Turkey should support the current Spinraza treatment for a lifetime. She stated that this treatment was injected into the spinal cord so that it was very painful for her daughter. She noted that Zolgensma treatment should be started as soon as possible. Mürüvvet added, “Children treated with Zongensma can sit, walk and disconnect from the medical  devices. Our wish is to have access to this treatment. My child has been fighting death for 30 months because we have to go from one side of Istanbul, which is a huge megalopolis of 15 millions people, to another all the time.. We shuttled between hospitals, being constantly redirected from one to another with various excuses. Most of them argued that there were no rooms left.

 

If it is  a specialized hospital in SMA, they have to provide all kinds of services for SMA patiens. Our state hospital can serve even foreign citizens as long as they got money, but they cannot serve their own citizens when they're poor. My child has been fighting death for 30 months. My husband gets the minimum wage (min. wage is 2324 TL in Turkey). We could not buy medicine for our child for 8 months because  there is no agreement  between the pharmaceutical company and the SGK. If we lived in a social state, it would provide this service to their citizens ”

 

‘Havin is constantly living connected to 6 medical devices’

 

Mürüvvet also explains that sometimes they cannot pay the invoices of the electricity company so the companies disconnect them, which leads Havin's eletrical devices to not workin' for a while. Citing an incident that they lived through as an example, Mürüvvet said “We brought Havin home after she was discharged. Then we realized that there were no electricity. Only our building in the neighborhood had a blackout Although they shouldn’t cut off the curent because of our little girl situation, and that they were aware of it, they cut it off anyway. My daughter is fighting with death for her life.

 

 For no less than 6 hours and a half I fought to keep my daughter alive with a procedure called ambu. " We did everything we could do to connect the devices cables of my daughter by pulling them from my neighbors's." 

 

'Human life should not depend on scoring and criteria'

 

Criticizing the criterias to be able to receive the treatment applied to SMA patients, Mürüvvet added 'Human life should not depend of scoring and some criterias". She said that they borrowed the coughing assist device for Havin because they could not afford it and that the SGK did not cover it. She said that they pay 1800 TL per  month for the medical supplies. Mürüvvet criticizes the health system arguing that “It is the right of my child to be cured and to live” and asked people to support Havin's money campaign on the social media. 

 

https://www.facebook.com/100014231259472/videos/606173946533642'Havin can't get support because of her name'

 

Mürüvvet pointed out that some people refuses to support Havin's cause because of her kurdish name.' And she said, “Children do not have religion, language or race.  Those who do not support Havin because of her kurdish name don't help and that's why we cannot advance in our struggle for Havin's life and cannot  make our voices heard. Our Berfins, Kardelens and Havins have the same right to live than the others kids. Our only goal is to collect the required amount for Havin to recieve a Zongensma treatment ”.

 

Those who want Havin's voice to be heard can reach the family at the following contact addresses and numbers:

                                                                  

https://taplink.cc/smahavinenefesol

 

https://twitter.com/SmaHavinnefesol

 

https://instagram.com/smahavinenefesol?igshid=18twwcs8wzdfg

 

Mürüvvet Aydar: 0 537 468 82 93  (90 537 468 82 93 )