Alexandra Mănăilă
Jurnalism

Patient stories: A girl “with weight” in rare diseases: Oana Dan

Gratie Oanei Dan in Romania exista Asociatia Prader Willi Romania (APWR), care ii sprijina pe pacientii cu aceasta boala rara.
Gratie Oanei, alti romani cu Prader Willi pot fi diagnosticati mult mai usor decat in trecut, pot fi indrumati spre cei mai buni specialisti si tratati pentru a duce o viata normala. Gratie Oanei, medicii din Romania au aflat mai multe despre aceasta boala rara si au putut fi in rand cu ceilalti specialisti europeni. Oana este un caz fericit si un exemplu de urmat de toti pacientii cu aceeasi problema! O fata vesela, mandra de asociatie, mandra chiar si de Prader Willi, o boala rara, genetica, neuro-comportamentala, care rezulta din anomalia cromozomului 15.
Povestea ei actualizata o puteti citi mai jos in limba engleza.
Varianta initiala a  povestii, in limba romana, a fost publicata pe siteul clubulgrasilor.ro

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Patient stories: A girl “with weight” in rare diseases: Oana Dan, by Alexandra Manaila, journalist

Oana is a nice Transylvanian girl from Zalau, always with a smile on her face and with a wide-open heart, willing to help anyone. Although she weighs almost 100 kg, you wouldn’t guess she is 35, the age she has now, because she is a childish person, always up for a laugh. If someone doesn’t tell you about Oana that she suffers from Prader Willi syndrome, you don’t suspect what challenges she had to overcome. She had 145 kg but she lost a lot of weight and that’s because of her perseverance and determination.

Thanks to Oana Dan we have in Romania Prader Willi Association which this year celebrates 17 years since its establishment. Thanks to Oana, other Romanians with Prader Willi can be diagnosed much more easily than in the past, they can be directed to the best specialists and treated in order to lead normal lives. Thanks to Oana, doctors in Romania found out more about this rare disease and were able to be in line with other European specialists. Incredible, but true, and Oana is a happy case and an example to follow for all patients with the same problem! A cheerful girl, proud of the association, proud even of Prader Willi, a rare, genetic, neuro-behavioral disease, which results from an anomaly in the chromosome 15.

Uncontrolled desire to eat in Prader Willi Syndrome (PWS)

              The disease occurs in one in 12,000 or 15,000 people, affects both sexes equally and has been identified in all races. People with Prader-Willi Syndrome have a problem with the hypothalamus, the part of the brain that normally controls feelings of fullness or hunger. As a result, they do not feel full and have a constant desire to eat that they cannot control. This is how obesity occurs in children and can be life threatening. Besides the chronic sensation of hunger and a slow metabolism, there are also: muscular hypotonia, short stature if not treated with growth hormone, cognitive deficiencies, incomplete sexual development, behavioral disorders. At birth the baby has low weight compared to the duration of gestation, hypotonia, difficulty sucking due to weak muscles („failure in proper development”). In the second stage („exaggerated development”), beginning between the ages of 2-3 years, there is increased appetite and weight control disorders, delayed motor development and behavioral disorders. Other factors that can cause problems can be: adverse drug reactions, high pain tolerance, gastrointestinal and respiratory disorders, lack of vomiting and unstable temperature.

              Prader Willi Association, founded when Oana turned 18

              The birth of the Prader Willi Association is Oana’s merit in the first place, and her parent’s Dorica and Tiberiu Dan in the second place. 17 years of activity have passed. During this time, Oana, the first member of the association, was joined by other patients with Prader Willi and other rare diseases.

It took her and the Dan family ten years to obtain a definite diagnosis. “I always knew that something was wrong with Oana from birth; immediately after birth she was a hypotonic child, very well behaved, exaggeratedly so. She walked late, sat up late, held her head late… She started to walk at two years and started rehabilitation programs since she was six weeks, which was a good thing, but at the time she was wrongly diagnosed with chronic childhood encephalopathy. When Oana was 8 years old I participated in a Montessori training course and by chance the girl was with me. A teacher from Sweden told me that Oana is misdiagnosed. “I am not a doctor but I am convinced that she has Prader Willi Syndrome”, the teacher told me. At that time, I did not have access to the internet, I could not find information. I searched, but I didn’t find it, people didn’t know about this Prader Willi Syndrome. Ten years later I met an occupational therapist in Australia and asked her if she knew anything about this Prader Willi and she said yes and that she has children in therapy. I asked her to come and see Oana.

The moment she saw it, she confirmed it to me „, says Dorica Dan, Oana’s mother and president of the Romanian Prader Willi Association.

It took 18 years for Oana and her parents to know what she had. During all this time, Oana’s condition did not worsen. She gained weight but she started to walk, join kindergarten, school, didn’t have problems with her heart, her breathing, her diabetes… We were lucky… “Sure, if she had been diagnosed earlier, or if in Romania there was access to growth hormones earlier, maybe that muscle tone would be better, maybe she wouldn’t have gained so much weight. But Oana recovered all the time „, confessed Dorica Dan. And now Oana does sports, goes to the gym, runs on the treadmill, exercises for at least an hour a day. Twice a week she does massage, does not get away from the bike and exercises on the mattress. She is active and weighs frequently. She monitors her weight and takes care of it as much as possible.

No lock on the fridge, but supervised    

Oana is 1.54 m, quite tall, considering that in this disease children have deficiencies of growth hormones and are small in stature. They take growth hormones and follow this treatment for life. They try to eat less, but with variation. „I can’t say that she has a special diet, but she eats without fat, without sugar, without a lot of flour. Rather eats vegetables, fruits, meat. We take care of what she eats. She is still a child with Prader Willi and if she has the opportunity to get her hands on the food she will eat. Clearly! But in principle you can refrain, unlike other cases with Oana you can go to the restaurant without problems. She knows she has to order a salad and that’s enough.”, says Dorica Dan. Oana is an educated, well-mannered young lady, with good common sense and who can control herself. Although, believe me it’s not easy at all when you’re greedy…You know all too well how hard it is to refrain from opening the fridge knowing that it contains your favorite snack! All the more difficult because in Prader Willi Syndrome there are no foods you like and foods you don’t like. You like all foods. And Oana likes all foods, of course! „She used to go to the Dezna Rehabilitation Hospital and say that she doesn’t like poppy seeds. But that doesn’t mean she doesn’t eat them. But she claims that she doesn’t like them „, says Oana’s mother laughing. It is true that there are children with Prader Willi, who cannot refrain, and internationally the family’s attitude is to turn off the food. „The problem is that closing access to the food solves the problem at the moment, at home. If the child lives alone in the house, it is a solution. But it is not a long-term solution, in my opinion. The child must be integrated into a community. Therefore, the child must somehow learn to abstain; because no one will ever close all the food just because there is a child with PWS around „, says the president of the Prader Willi Association.

In the case of their daughter, they did not cut the access to the food. They thought it’s best to keep an eye on it while the refrigerator was not closed. According to the Dan family, people who do not have access to certain things are even more frustrated, and mentally it does not help them. „Psychically, Oana would not be the same if we closed the access to the food. Oana has no complexes, she is always positive, we do not know how to find the balance, it is a difficult disease. And it is very difficult to find a balance on how to behave in this situation and still ensure integration in the community. We are glad that Oana is a child integrated in society, that she has friends, that she knows how to do a lot of things, she knows how to help others, she is a very good child, she has no evil, no matter who says what, she has no malice towards the respective person; she could be an example for those around her „, say Oana’s parents proudly.

Daddy’s daughter, as Tiberiu Dan calls her, has always been among children her age, from kindergarten to school and now she is at the NoRo Center (the only center in Romania dedicated to rare diseases, in Zalau). The integration helped Oana to be like others. She set out to be like everyone else and she is! She finished school and was employed. She works at the ARCrare and tries to do her best to solve the tasks she has, sometimes even exaggerating. And speaking of food, if there is anyone who supervises a child with Prader Willi in terms of nutrition, they supervise each other very well.

Realizing that she should lose weight

Oana realizes that she weighs a lot more and that she should lose weight, she knows very well that she has always been told this. But she doesn’t complain. Oana generally does not complain about anything. In recent years, her weight has been maintained, not increased. On holidays, unlike others, Oana does not gain weight because then she goes out more with her parents and her brother Alex and does even more exercise. „A child with Prader Willi can put on 1-2 kilograms a day. It is very easy to put on weight and very difficult to lose it. But the fact that she has been wearing the same clothes for 9 years is gratifying. Now, she is under 100 kg and we are very proud of her”, her mother confessed to me. However, Oana likes to be chic and obviously like any lady she loves shopping. „Unfortunately, there are ugly clothes for people with obesity in general, and in the case of Prader Willi, due to muscular hypotonia the shape of the belly is a little different, in the sense that it is looser, so they need special tailors. It is difficult to find clothes, but in recent years it is getting better, even in Romania „, says Dorica. Oana prefers pants. It fits very well in blue, red and does not hesitate to match beads, bracelets, jewelry to her outfit. „The first thing when we go to a store is to buy her something because she looks at everything. She asks “Who is this for?” So, this problem must always be solved „, the parents confess.

A real joy in packing Kinder eggs

Oana has good computer skills, she is happy to search and provide others with information about certain diseases. She is happy to be with the children from the NoRo center and to support them in their socio-educational activity. She is very caring with her peers and if she also meets children with Prader Willi she tells them that she has it too, that it is a mild illness, she encourages them… A job that could be boring and traumatic for most of us, for a person with Prader Willi isn’t. For example, it is a real happiness for Oana to pack Kinder eggs. Then, the children from the Salaj center make certain handicrafts, meticulous things, drawings, many of them being displayed in the window of the NoRo center.

Oana’s story was not written with a positive flavor, it is a positive story in Romania. Yes, it sounds strange, but it is true and encouraging. What should parents of children with PWS learn from the story of Oana and the Dan family? It is important to show the child that you love them, this will certainly be told by any parent, any psychologist, any Christian. „It is important to give them confidence that they can lead their lives, in the sense that they could take care of themselves, including food, provided you supervise them. But they must have the impression that they are independent, that they can have a normal life, go out as much as possible, be integrated in school, do an activity that they master and that they know how to do „, this is what Tiberiu and Dorica Dan say. What would Oana want from her family: her parents and younger brother, Alex? „Maybe I would like more understanding, sometimes… Certainly the most important thing is that I want us to stay together for as long as possible.” What was the biggest challenge for Oana (except loosing weight)? To become truly independent. Oana really wants a house in which to live, to take care of herself. So far, she has shown that she can do it, she has a job. Oana is an independent child. The only thing she is not independent of is food.

If in Oana’s story, nutrition remains the key word, in the story of the Prader Willi Association, the involvement of both parents and specialists, when it comes to patient counseling, remains the key to success.

 

 

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