Life with Usher Syndrome (part 1)

Exclusive interview with Afroditi

Run

 

During the last and a half year that I have moved in Rotterdam I met many Greek newcomers who left the fatherland to come in the Netherlands to study or to find a job. One of the most interesting Greeks that I met when I visited the Greek community here was Afroditi. In the beginning I didn’t pay a lot of attention to her as I was looking around me all these completely new and unknown faces, but soon she talked to me and her smile was so warm that I sat on a chair next to her and we started a general conversation.

I cannot remember if we talked about it the second  pr the third time that we met but I am sure that soon after our first meeting she told me that she is diagnosed with Usher Syndrome. I remember when I heard these two new words I reacted in a spontaneous way by answering “Oh really?”. I knew that I have heard it before (probably during my bachelor studies) but I couldn’t be completely sure. So I asked her to remind me what the exact symptoms of Usher Syndrome were. The truth was that no, I didn’t remember a lot about it, for example that it was a syndrome with such serious symptoms. Moreover, I experienced the biggest surprise when in the next seconds Afroditi told me with completely natural voice and without any hesitation: “Now that we are talking I am wearing hearing aids. Also I don’t have a peripheral vision, so I need to look at you to be sure that I see you”. After these words she was acting like she had talked about the simplest thing in the world. She stood up from the chair and without any difficulty or hesitation she walked full in confidence to the bar of the Greek community to buy a bottle of soda. I was absolutely speechless. How is it possible this person to be diagnosed with this syndrome and has the problems she says?

The same thing I kept wondering all the next months that I was getting to know her better and better. If I wasn’t seeing from time to time her hearing aids on her ears and her cane – a special walking stick – I would have thought that she is fooling us. My disbelief reached its’ highest level when I saw her celebrating Greek Easter in the offices of Greek community and dancing passionately Cretan traditional dances. Honestly I couldn’t find a moment of difficulty or difference in her moves compared to the other people that they were dancing at the same time. Many friends were absolutely surprised when they were informed that Afroditi has a problem with her vision and hearing and can still dance like that. It was that night when I completely realized that there was a really special person in front of me who I wanted to “discover” little by little in the best possible way.

“Will you give me an interview?” I asked her one day when we went out for a coffee and then we ended up eating fruit cake in her place. “Interview?” she asked me with a puzzled face. “Yes, an interview” I said again with more excitement than before. “We will talk about Usher, you will tell me your experience and how is your life with it” I explained to her. “Then we will publish your interview in my blog in order to be read by people who are diagnosed with Usher Syndrome or people with various problems of their vision and/or hearing. Moreover, it is equally important to inform healthy people about what exactly is Usher Syndrome and how a person who is diagnosed with it could live his/her life decently. You could give a very strong message of life and hope if you just speak for your life and if you show to others that you are able to manage the whole situation” I ended up and then I remained silent and I was impatiently waiting to hear her response. Very soon she seemed to make a decision and without much hesitation she answered: “I don’t mind giving an interview. In the past I was embarrassed to talk about me and the syndrome but I don’t feel like that anymore”.

Some days went by and I began to read about Usher Syndrome while simultaneously I was trying to find some good forms of interviews – especially of people who describe diseases –and in this way I started beating the questionnaire that I would use during in our meeting to get a decent and comprehensive interview. I am not a doctor, no matter how much I read about this syndrome I couldn’t read all the studies that have been conducted during last years. Additionally, I didn’t have the experience of taking an interview from a person that has been diagnosed with a serious disease. In the end, I don’t know if I managed to take a really “useful” , comprehensive and complete interview. On the other hand, I am sure that I got a real and spontaneous interview by a determined woman who wanted to speak and share her personal story, how she “sees” and “hears” Usher Syndrome in her body all these years.

I changed this interview as less as possible since the biggest advantage of it is its’ authenticity. In the following document you will read how is the life of a person who has a genetic syndrome, who lived in three different countries, who visited many doctors and who has experience from different health systems. Afroditi is a young, bold woman who managed to live her life in the best way she can as she simply chose to “have the disease, but not to be the disease”. That’s why it is so important her story to be read and heard.

About Usher Syndrome

One of the best articles that I found regarding Usher Syndrome when I googled the words “Usher Syndrome” was published by the National Institute of Deafness and Other Communication Disorders (NIDCD). I will paste here some of the information that the Institute provides in its’ website in order to be better understood to all of you what is Usher Syndrome is all about. You can read the full article regarding Usher Syndrome here.

What is Usher Syndrome?

Usher Syndrome is the most common condition that affects both hearing and vision. A syndrome is a disease or disorder that has more than one feature or symptom. The major symptoms of Usher Syndrome are hearing loss and an eye disorder called retinitis pigmentosa, or RP. RP causes night-blindness and a loss of peripheral vision (side vision) through the progressive degeneration of the retina. The retina is a light-sensitive tissue at the back of the eye and is crucial for vision. As RP progresses, the field of vision narrows—a condition known as “tunnel vision”—until only central vision (the ability to see straight ahead) remains. Many people with Usher Syndrome also have severe balance problems.

There are three clinical types of Usher Syndrome: type 1, type 2, and type 3. In the United States, types 1 and 2 are the most common types. Together, they account for approximately 90 to 95 percent of all cases of children who have Usher Syndrome.

What causes Usher Syndrome?

Usher Syndrome is inherited, which means that it is passed from parents to their children through genes. Genes are located in almost every cell of the body. Genes contain instructions that tell cells what to do. Every person inherits two copies of each gene, one from each parent. Sometimes genes are altered, or mutated. Mutated genes may cause cells to act differently than expected.

What are the characteristics of the three types of Usher Syndrome?

Type 1

Children with type 1 Usher Syndrome are profoundly deaf at birth and have severe balance problems. Many of these children obtain little or no benefit from hearing aids. Parents should consult their doctor and other hearing health professionals as early as possible to determine the best communication method for their child. Intervention should be introduced early, during the first few years of life, so that the child can take advantage of the unique window of time during which the brain is most receptive to learning language, whether spoken or signed. If a child is diagnosed with type 1 Usher Syndrome early on, before he or she loses the ability to see, that child is more likely to benefit from the full spectrum of intervention strategies that can help him or her participate more fully in life’s activities.

Because of the balance problems associated with type 1 Usher Syndrome, children with this disorder are slow to sit without support and typically don’t walk independently before they are 18 months old. These children usually begin to develop vision problems in early childhood, almost always by the time they reach age 10. Vision problems most often begin with difficulty seeing at night, but tend to progress rapidly until the person is completely blind.

Type 2

Children with type 2 Usher Syndrome are born with moderate to severe hearing loss and normal balance. Although the severity of hearing loss varies, most of these children can benefit from hearing aids and can communicate orally. The vision problems in type 2 Usher Syndrome tend to progress more slowly than those in type 1, with the onset of RP often not apparent until the teens.

Type 3

Children with type 3 Usher Syndrome have normal hearing at birth. Although most children with the disorder have normal to near-normal balance, some may develop balance problems later on. Hearing and vision worsen over time, but the rate at which they decline can vary from person to person, even within the same family. A person with type 3 Usher Syndrome may develop hearing loss by the teens, and he or she will usually require hearing aids by mid- to late adulthood. Night blindness usually begins sometime during puberty. Blind spots appear by the late teens to early adulthood, and, by mid-adulthood, the person is usually legally blind.

How is Usher Syndrome diagnosed?

Because Usher Syndrome affects hearing, balance, and vision, diagnosis of the disorder usually includes the evaluation of all three senses. Evaluation of the eyes may include a visual field test to measure a person’s peripheral vision, an electroretinogram (ERG) to measure the electrical response of the eye’s light-sensitive cells, and a retinal examination to observe the retina and other structures in the back of the eye. A hearing (audiologic) evaluation measures how loud sounds at a range of frequencies need to be before a person can hear them. An electronystagmogram (ENG) measures involuntary eye movements that could signify a balance problem.

Early diagnosis of Usher Syndrome is very important. The earlier that parents know if their child has Usher Syndrome, the sooner that child can begin special educational training programs to manage the loss of hearing and vision.

Is genetic testing for Usher Syndrome available?

So far, 11 genetic loci (a segment of chromosome on which a certain gene is located) have been found to cause Usher Syndrome, and nine genes have been pinpointed that cause the disorder. They are:

  • Type 1 Usher Syndrome: MY07AUSH1CCDH23PCDH15SANS
  • Type 2 Usher Syndrome: USH2AVLGR1, WHRN
  • Type 3 Usher Syndrome: USH3A

With so many possible genes involved in Usher Syndrome, genetic tests for the disorder are not conducted on a widespread basis. Diagnosis of Usher Syndrome is usually performed through hearing, balance, and vision tests. Genetic testing for a few of the identified genes is clinically available. To learn about laboratories that conduct clinical testing, visit the Web site www.GeneTests.org and search the laboratory directory by typing in the term “Usher Syndrome.” Genetic testing for additional Usher Syndrome genes may be available through clinical research studies. To learn about clinical trials that include genetic testing for Usher Syndrome, visit the Web site www.clinicaltrials.gov and type in the search term “Usher Syndrome” or “Usher genetic testing.”

How is Usher Syndrome treated?

Currently, there is no cure for Usher Syndrome. The best treatment involves early identification so that educational programs can begin as soon as possible. The exact nature of these programs will depend on the severity of the hearing and vision loss as well as the age and abilities of the person. Typically, treatment will include hearing aids, assistive listening devices, cochlear implants, or other communication methods such as American Sign Language; orientation and mobility training; and communication services and independent-living training that may include Braille instruction, low-vision services, or auditory training.

Some ophthalmologists believe that a high dose of vitamin A palmitate may slow, but not halt, the progression of retinitis pigmentosa. This belief stems from the results of a long-term clinical trial supported by the National Eye Institute and the Foundation for Fighting Blindness. Based on these findings, the researchers recommend that most adult patients with the common forms of RP take a daily supplement of 15,000 IU (international units) of vitamin A in the palmitate form under the supervision of their eye care professional. (Because people with type 1 Usher Syndrome did not take part in the study, high-dose vitamin A is not recommended for these patients.) People who are considering taking vitamin A should discuss this treatment option with their health care provider before proceeding. Other guidelines regarding this treatment option include:

  • Do not substitute vitamin A palmitate with a beta-carotene supplement.
  • Do not take vitamin A supplements greater than the recommended dose of 15,000 IU or modify your diet to select foods with high levels of vitamin A.
  • Women who are considering pregnancy should stop taking the high-dose supplement of vitamin A three months before trying to conceive due to the increased risk of birth defects.
  • Women who are pregnant should stop taking the high-dose supplement of vitamin A due to the increased risk of birth defects.

In addition, according to the same study, people with RP should avoid using supplements of more than 400 IU of vitamin E per day.

What matters most is how you see yourself

Afroditi’s interview

Fani: Afroditi, we may begin. Please give us some information about you.

Afroditi: I am Afroditi. I am 36 years old. I was born in Greece. I studied Business Administration in Greece (bachelor studies). Then I moved to England for my Master study in Finance and Accounting. I stayed and worked in England for about ten years. During the last years I was there I fell in love with a Dutch man (she is smiling) and I moved to the Netherlands where I live for the last four years.

F: Very well. You gave us some basic information about your life. Which facts or situations do you consider as the most important in your life? The place that you were born? The way you were raised? The time you moved abroad? The moving from country to country? What has made you the person you are today? Pick anything from the day you were born until today.

A: (She is thinking a bit) The change of the countries.

F: Why? Because a moving to another country is a holistic change?

A: It is a way to realize life in a better way. I remember myself in Greece and I have the sense that back then I was on a cloud and I was just going somewhere. I was following life. I was following culture, rules, my family. When I moved abroad I had a lot of freedom so I discovered myself and my limits.

F: Have you ever given an interview which will be published to the internet?

A: (She is smiling). No.

F: How do you feel that your words are going to be read by unknown people?

A: I often read interviews by unknown people too. I think that every kind of experience can move some people (Note: Until the publication of this interview, Afroditi was called to speak in a conference about deaf-blind people vision which took place in North Ireland. A short interview of Afroditi in which she was talking about Usher Syndrome was uploaded on the internet).

F: Very nice. Today we will talk about Usher Syndrome. You have been diagnosed with this syndrome. Do you want to start by telling me what is Usher Syndrome in general and what is it for you?

A: Usher Syndrome in general affects vision, hearing and sometimes physical balance. There are three types of it. For example you could be born deaf and you will get blind very soon or you could be born hard of hearing and you will get blind later in your life. Furthermore some people have balance problems.

I am diagnosed with type 2A of Usher Syndrome. This type is the least serious. Someone is born with a serious hearing impairment and vision starts getting worse after his/her twenties, peripheral vision will be lost in the future and only the central vision will be left in the end. There is always a possibility someone to lose his/her vision totally. In general that happens after his/her 50s which means that until that age most people have almost lost completely their vision.

F: Okay. However hearing doesn’t get lost and it can be supported by hearing aids, right?

A: Yes, that can be true but sometimes even hearing gets lost – just think that even hearing of regular people gets worst as they are getting older. All of us get old and all of us have suffered from some noises which increase the possibility of hearing decrease or loss.

F: But if you wear your hearing aids you cannot totally lose your hearing, right?

A: No, unfortunately some people will end up blind and deaf and they will not be able to communicate with none of the regular ways.

F: My next question was about the three types of Usher Syndrome. I was about to read the three types and find your type but you already know that.

A: Yes.

F: From what I read yes, your occasion is Type 2. Now regarding balance it is mentioned in many articles that it is an indirect symptom of Usher Syndrome. For example someone is not born without balance, it is just a symptom which comes up from loss of hearing. Could we consider that gradual loss of hearing could affect someone’s balance?

A: If I remember correctly in Type 2a there is not a balance problem.

F: Right, you don’t have a balance problem.

A: But I think that in Type 1 and Type 3 there is a balance problem from the beginning.

F: Yes, and this balance problem is related to the dysfunction of auditory system.  

A: Right and that was a part of the diagnostic tests that I had. There is a specific test: they put water inside my ear and I felt nausea. In case that nausea is absent, there is a balance problem.

F: Ok, that’s interesting!

A: Yes, there are some strange tests out there.

F: It would be nice if we will talk about these tests a little later. I will ask you to tell me your personal experience about hospitals, doctors etc. Right now let’s talk about the period that you understood that something is wrong regarding your senses. When and how that happened? What were the exacts symptoms to you?

A: The first symptom was the bad hearing. Maybe I was born with it or it came up later. Nobody knows. However, during the previous years some doctors told me that it is a genetic disability. Scientific evidence also supports this possibility.

F: Okay.

A: So I had a hearing impairment since I was a kid. I know that in Type 2 of Usher Syndrome I did and still have a 60% loss of hearing in high frequencies. So I am considered as a hard of hearing.

F: Right.

A: Speech usually is in high frequencies. It is hard to  hear people with high pitched voices, most of them are women. It is difficult for me. I don’t remember myself as a kid having difficulty with people that had low voices.

F: I want to get it better. We have a man with a deep voice and a woman with high voice. Whom you can hear better?

A: The man. Because in high frequencies I have 60% loss of hearing. In medium frequencies my loss of hearing is decreased and in low frequencies it is normal.

F: Okay, so if a person talks to you in a normal frequencies are you able to hear him/her?

A: You know it is very important that someone speaks clearly and not loud.

F: That is what I don’t get. I think that it is impossible to not hear someone that speaks loudly. I am surprised that the lower the frequency, the better you hear it.

A: I see that on movies or on television. Someone yells to someone else in order to be heard. And the person with the hearing impairment responds: “Stop yelling, I am not deaf”. The truth is that no matter how much someone yells, he/she will not be beter heard by a hearing/impaired person. It is more important that someone speaks clearly. If you speak in a clear way a hard of hearing can hear you and understand you. If you speak fast he/she won’t hear anything and his/her brain won’t be able to process any signals.

F: How you consider a person that speaks clearly? He/she should speak slowly and in normal tempo?

A: Right, normal tempo. One should not speak (suddenly she starts speaking very slowly) veeeeeryyyyyyy veeeeeryyyyyy slooooowly (and now she is talking fast) or very fast! One should speak normally and it depends how hard of hearing someone is because probably he/she probably uses lip-reading technique to follow a conversation. That’s why the right use of the mouth and articulation is so important to hearing impaired people.

F: Okay. Nice. So to sum up: The first problem that you figured out had to with your hearing sense and probably it was present since you were born. What about your vision in the first years of your life? Did you have a vision problem at the beginning or it started later?

A: When I was a kid I remember – probably it was the period that I was in primary school – that I put glasses for astigmatism. It wasn’t something really serious as anyone can wear glasses because of astigmatism. Years later, when I was in England, at the age of 26-27 years old, I thought I needed a new prescription to change glasses. It was the first time that I followed this procedure outside of Greece. So, back then an optician checked my eyes and suggested that I visit a specialist because he thought that something was not quite right.

Yin Yan

F: These facts happened when you were 26-27 years old. So, until then you were only informed about your hearing impairment. You knew that your sense of hearing was decreased, right?

A: Yes, I knew that but because of the way I was raised – they were always telling me that I don’t have a problem! – I had never worn hearing aids till then. For sure I was raised in a really peaceful world (she is laughing).

F: Well there is a really positive side in what you describe (we are laughing). You were not hearing all the negative and unpleasant things that were happening and being said around you!

A: And all the bullshit too!

F: Right! So you told me that regarding your vision problem you knew from the optician that something was going wrong and you had to check it.

A: Yes, I went to a specialist from whom I received my first diagnosis.

F: So the first time you heard the words “Usher Syndrome” was when you were 26-27 years old, right?

A: Yes.

F: In what age did you put hearing aids and what difference did you notice to yourself compared with your hearing in the past years?

A: My hearing now that I wear hearing aids has almost nothing to do with my hearing previously. The first time that I tried to wear hearing aids I was 21 years old and I was in England. I was then a student and I was experiencing my new life abroad. The woman that fitted me the hearing aids for the first time was Greek but she did it in a wrong way.

F: What was wrong in her way?

A: When a doctor fits hearing aids to someone for the first time he/she should be very careful with the volume. In the beginning, the volume should be set lower and gradually adjust it to compensate for the full hearing loss.

F: So it was too loud for you.

A: Yes, the volume was set to compensate for the full hearing loss. She thought that I miss 60 decibel so she just added them in one go. And you know this volume has a greater impact when you walk in the busy streets of London.

F: I understand.

A: The doctor told me “Wear the hearing aids and get out, go to your place and don’t take them off”. The hospital was 15 minutes´ walk from my place. In the first 5 minutes of walking I thought I was losing my mind!

F: And later?

A: I wore them once more in the computers facility of my University . I felt the same unbelievable madness! I was able to here every button that I was pressing on the keyboard for the first time in my life! (She is laughing). Imagine yourself hearing the noise of a typewriter for the first time, you know this loud sound of the buttons!

F: Yeah, yeah.

A: So after these previous experiences I didn’t wear hearing aids. I visited the doctor once more and I said to her: “I can’t wear them, they drive me crazy, maybe we can do something”. She answered to me that I should get used to it and there was nothing we could do. Her response “helped” me a lot to take them off and stop concerning myself with them.

F: I see.

A: Later, when I was 26-27 and my visual impairment was diagnosed, I realized that from now on I couldn’t use my eyes so much and that I should instead find a balance between the use of my eyes and ears. During this period I started thinking again the possibility to wear hearing aids, I went to a public hospital in London but their hearing aids were not really useful to me. When they fitted me with a pair of hearing aids (this pair was provided for free) I was actually able to hear the interference from an electric plug!

F: So you were hearing again every little sound…

A: (She interrupts me) My complaint regarding the hearing aids was that doctors should have followed a different procedure with the volume and everything. Nowadays there are so many different kinds of hearing aids with various settings but the sad thing is that there are not enough specialists to set the settings properly. That’s why a small difference in the volume can drive someone completely mad when he/she will go out on the streets.

F: I see.

A: ( She remains silent for a bit). I searched so much for proper hearing aids when I was in London. I was testing different hearing aids for one year because this is how it goes: First you try different hearing aids until you find what are the ideal for you and then you buy them. I didn’t have real help from the hospital, I started having problems with my job. Then I decided to come in the Netherlands so I thought I would wait to buy hearing aids here. This is how it happened indeed, I bought them when I moved here. I had found the best from all I had tried. I had a  device like a mobile phone and the sound was coming directly in my hearing aids e.g. I was connecting my hearing aids with my computer and I was hearing everything directly, so no one else could hear what I was listening to.

F: This sounds actually nice.

A: Yes, I started learning things here… (she stays quiet for a bit)

F: You finally started seeing options…

A: Yes and simultaneously I realized how much I missed out in my life.

F: So we can say that the only experience you have from wearing hearing aids is during the last 4-5 years.

A: Yes.

F: So now you are ok with the idea of wearing hearing aids. It is not something annoying since you can control and regulate it in the way you like, right?

A: Yes, exactly.

F: Okay. I wanted to ask you something regarding what you previously said. I understand that by wearing hearing aids for the first time people with hearing impairment could experience a strong shock. But why does this happen? Is it because one suddenly hears a lot of sounds and so will will think “How many sounds  couldn’t hear until now!” or it is actually so annoying and intense experience that you feel a holistic unpleasant feeling? I am asking you because I saw a video on the internet with a doctor who put implants to a patient with Usher Syndrome. In brief this woman hears sounds in normal frequencies for the first time in her life and from the first seconds she starts crying while she says to her doctor that sounds are too loud. Her doctor responds that it is normal to hear everything too loud since her brain tries to “understand” this new situation so volume will go lower and in the future it will feel normal. Probably your experience is similar with this woman’s experience. For you it was a negative and annoying experience, this woman was happy because she accepted the temporary too loud sound. So the shock you experienced had to do with the sudden sounds in your head or with the disturbing noise? What was your basic feeling back then?

A: There is a big difference between hearing some sounds and not hearing at all. I suppose that this woman started preparing psychologically herself months ago before she put hearing aids. In some way she knew what she is going to experience. She managed the first “panic” because she obviously really wanted to put hearing aids. My situation is different because I was able to hear but not all the frequencies. So my experience with hearing aids was like someone added some millions more sounds in what I could already hear. It was a double shock for me: Firstly I was shocked because I was able to hear every single sound inside the doctor’s room e.g. the pen on the paper, the sound of the computer’s mouse. Another fact that I remember had to do with my previous house in London. I was renting an apartment really close to a dock of Thames and therefore around my building there was water and a lot of seagulls and ducks. One night some friends came by and slept over. The next morning they kept telling me: “We couldn’t sleep, the birds make a lot of noise”. So when I came back on foot that day from the hospital with the hearing aids and I heard everything I thought: Well, now I know why they couldn’t sleep that night. I honestly felt like I was going mad if I had to keep hearing them all day long. So when I was in my apartment I  used to take off my hearing aids only because I couldn’t stand the birds. I didn’t want to hear the noises of ducks and sea-birds anymore. Of course, later I started realizing that I felt angry because I had lost so many facts and information all these years. That was the second shock regarding what you asked me.

F: So after that did you feel determined to live all those you couldn’t live until then? Or you thought that it was a useless feeling? Could the anger you were feeling lead you somewhere?

A: Anger is always useless. However, we feel it and we have to deal with it.

F: Yes.

A: It is not possible to avoid anger. I was angry with the others and myself. Anger is a phase you should experience and then you will start finding your balance. Time passes and your brain starts getting used to the new situation of hearing – that’s why you should increase volume gradually and later will reach the maximum level of hearing. Your brain needs about 6 months to filter all the sounds and actually learn every single one from the beginning. Later it will get rid of the unnecessary ones e.g. you stop hearing the noise of the fridge – which I heard some years ago for the first time in my life. You need time to discover all the sounds that come from every object around you.

F: What you are saying is actually really interesting. You were hearing sounds for the first time and you didn’t know where they come from, which objects does what sound.

A: I was really scared especially when I was walking outside. I was hearing cars and I had the impression that they were next to me and then I was realising that they were actually far from me. Or I was hearing horns from distant cars and I was thinking that they were from cars that were next to me.

F: I see. You need a lot of time to get used to all the sounds inside your head. Later I guess you can recognize sounds. You know for example what is the normal sound of the fridge and how it will sounds when it will be broken. In other words you start understanding different frequencies and change of sounds.

A: Yes, sure. However, I usually don’t wear hearing aids when I am at home.

F: Why?

A: Well, being at home it’s a break for me. When I remove my hearing aids from my ears, I feel that I remove something that is not part of my body.

F: Not part of your body. Why? Because you wear them? Or do you want so much to lower the frequency? And why do you need lower frequencies?

A: Because that is what I knew for 30 years. I feel calm when I don’t wear my hearing aids. When I am at my place I want to be in the way I am used to all these years. I feel more free. It is still tiring having to wear hearing aids the whole day long. Wherever I am, even at work, where I wear them for 10 hours, at some point I feel the need to take them off for 5 minutes.

F: Right now we are talking and you don’t wear them. However you can hear me well. Why? Is it because I speak slowly and clearly? Or because I don’t talk too much loud? And by the way you know how difficult that is for me!

A: (She is laughing). Well, comparing to other times, now that you are interviewing me you are calm and your voice frequency is medium to low.

F: (I am laughing) Perfect. So, me and my voice will stay the same!

A: The thing is that we speak in Greek right now so it is easier for me. It would be a problem if this discussion was in English or Dutch. In this case hearing aids would be absolutely necessary. On the other hand, when the other person is someone that I know well and I am used to his/her voice it will also be easier.

F: Right, because I guess it’s not only a matter of language. You should be familiar with his/her accent in order to understands what he/she says.

A: Yes because our language is full of vowels. English and Dutch contain more consonants and these are in the higher frequencies. So I need to make a lot of effort to hear properly.

F: Nice information to learn!

A: Every hard of hearing will tell you that! It is much easier to hear and communicate in your mother tongue than any other one.

F: Sure. And I suspect that your difficulty has also to do with lip-reading. It is much more easier to read lips from people that talk your mother tongue that any other one.

A: Definitely. Especially lip reading most Dutch people is very difficult for me.

F: I see. After all Dutch is not such a simple and easy language.

A: It’s not only that. It’s the way they speak. Every word is inside their mouth. That’s the way they speak Dutch.

F: Right, “mouths are closed” as I say. And I can also tell you that it’s not only a matter of hearing. Even a healthy person finds Dutch pronunciation difficult. You understand words but sometimes not all of them. I guess if someone is hard of hearing, things can be much more difficult.

A: Exactly.

F: Afroditi, I want to take you back now, to your childhood.

A: (She is smiling) Should I lie down?

F: (I am laughing). Well if you want, of course you can. Let’s start by telling me how important is the family’s role in the diagnosis ofUsher Syndrome? Is it simple for parents to see the symptoms when a child is still too young? What they can do when they will figure out that their child’s senses do not function as they should?

A:  When I was a child I knew – they had told me – that I had a hearing problem. It was not hidden from me. But they kept telling me “It’s not important. Everything is fine. You are normal”.

F: So when you were 7-8 years old you were aware of your hearing impairment.

A: Yes I knew it. “You have a problem with your hearing, it’s ok. Keep it to yourself”.

F: “Keep it to yourself”. Why?

A: Because my family was one of these traditional Greek families that considers disability   as something embarrassing and not only that. In general anything that was not perfect was embarrassing. Any weakness that has to do with body, character, mind, anything.

F: Your childhood was in Athens or somewhere else in Greece?

A: It  was in Athens.

F: But your parents come from Crete, right?

A: Yes, they moved to Athens when they got married. I was born and raised in Athens.

F: Nice. So your parents knew about your hearing impairment. They told you that everything is ok, so I guess it was not discussed. Did they investigate further? Did they want to learn more details?

A: I do remember as a child that we have visited some hospitals and I had some tests which I found years later.

F: So your parents did want to know more.

A: Yes, I had been tested. They knew that I had a hearing impairment. They told me that they sent my tests abroad because there was a dilemma if I should wear hearing aids or not. The final decision was not to wear hearing aids.

F: The doctors abroad made that decision?

A: That is what I was told. But let’s be honest, we are talking about facts that happened 30 years ago. I doubt that Usher Syndrome was well-known in Greece.

F: Yes, but even 30 years ago I guess you had the right to wear hearing aids since they existed and since you were diagnosed with a hearing impairment.

A: Well I maybe had the right. However, when you are a child your parents decide what is good or not for you. My parents thought back then that if I wear hearing aids all the time (30 years ago hearing aids were regulated with a “wheel”) my ears would be more damaged and not helped.

F: Yes, I can imagine that they were thinking that too high sounds would decrease your existing hearing.

A: Yes that’s what they were thinking. They told me that tests from abroad – which I never found – recommended to avoid wearing hearing aids. In other words my sense of hearing was not in such a tragic level and hearing aids were not necessary.

F: I see.

A: The only formal paper that I have today is from an institution for deaf and mute people in Athens in which it was recommended that I do speech therapy.

F: Why?

A: Because of the way that I speak.

F: That you used to speak or that you speak now?

A: The way that I speak in general. I have a problem with my accent. Right now I speak in a clear way but it is not always like that. In addition, I did not have a rich vocabulary that I should be using compared to peers of my age. Moreover, the biggest problem with my speech was that I spoke in low voice. I don’t know if it has to do with who I am talking to and how comfortable I feel with him/her but I often  talked in low voice and others couldn’t hear me.

F: Well I suppose it is related to your lack of hearing, right? I mean you speak in low voice because you hear everything in lower frequencies.

A: Yes, exactly that. Speech therapy can help with this. One should articulate correctly but you should also be able to hear and be heard. You can learn various techniques. Speech therapy not only helps you with speech but also with hearing. I know the difference because a month before I started my intensive Dutch lessons, I had some speech therapy sessions. That’s how I learned the Dutch pronunciation. They showed me how to use my mouth.

F: And that’s why you have a very nice pronounciation in Dutch!

A: That’s what I also wanted to tell you. I pronounce Dutch better than English. When I speak English you definitely know that I am not English! (Laughs). Fortunately, my Dutch accent is much better. Not perfect, but better.

F: Very interesting discussion we are having about speech therapy. We will add more information later, if necessary.

A: (She is smiling). I started talking about different subjects than what you asked me, huh?

F: No worries. We will continue from the point we previously were. The last thing we said was that despite of your hearing impairment, the final decision was that you wouldn’t wear hearing aids.

A: Right. This was about my hearing and now I will tell you about my vision problem. I was alone in London and I remember that I called my parents to tell them. I honestly don’t remember much of those days. I think I told them that I have a problem with my eyes and that I would go  blind. The way I got diagnosed and how I was informed myself about it is another big story!

F: What did your doctor in London tell you?

A: It’s a really long story! Ok, I will tell you. I had been in an optician’s shop and he told me to go and see an eye specialist. It took me six months to get the appointment. I visited her and she put drops in my eyes, in order to dilate my pupils. I waited for 20 minutes. Then, she took a quick look in my eyes and she simply said to me: “You will go blind In five years. Nothing we can do. Go home”. The specialist just sent me home. I remember that I went home  on autopilot. I cannot remember how I reached my place, how I got inside. The only thing I remember is that when I got in my apartment I started crying. I also remember I was very depressed. Probably that is why I have a difficulty remembering details from those days, like how and when I called my parents. I guess one day I managed to call them. They couldn’t understand what I was telling them, they couldn’t understand what was going on. Even nowadays it is hard for them to realise what is happening because I live abroad and far from them. Moreover, I went through all those difficult phases mainly by myself, the did not go through everything with me and therefore cannot comprehend it.

F: Is it possible that they cannot believe that this is happening to you?

A: Yes, it is possible. After a lot of events, they tried the stimulation glasses I have and they were shocked (Note: Afroditi has a pair of plastic glasses that are covered with black tape and left “open” a small circle in the middle of both lenses. In this way Afroditi can show to anyone who doesn’t understand what having “tunnel vision” means and how she sees the world at the moment).

Stimulation glasses

 

F: Why do you think they were shocked?

A: They did not expect that I couldn’t see.

F: And when they figured out that you can’t see?What was their first reaction? What was the first thing they said?

A: Nothing. Just shock. Later they told me “You see almost nothing”.

F: Did you ever discuss it again?

A: No, we didn’t. Every time the discussion comes up, they are in denial.

F: I understand that it is difficult for them. Based on what you lived with your family do you want to give a piece of advice to parents that just realized that their kids have either problem with their hearing or vision?

A: First they should get an official diagnosis. Then they should get a second and a third opinion from different doctors. It would be good if they also get in touch with a specialised institution, or other specialists, or other parents who have children with similar health problems. Let’s not forget that this is also a big shock for the parents.. The way that  parents will cope with this issue will have a direct impact on the child. Parents should not hide the truth from their kid. They should however be very well-informed, in order to give           to their child all the necessary “weapons” to deal with this situation.

F: What is considered as wrong behavior from the family’s side? How should a child with health problems be treated from his/her family?

A: First and above all parents should be honest. About eveything. Honest about what is going on, what the facts are, what the emotions about this situation are- how the parents feel about the whole situation. Parents should deal with this situation together with the child. It was wrong what I was told that there is nothing wrong with me. There is something wrong and it is actually very serious! On the other hand,  “very serious does not mean “absolutely crucial” too. I did survive and “What a miracle!” as many specialists already said! I studied, I found a job, I danced. My brain found a way to adjust and survive. The point is that with a better management of my disability from the very beginning, many things in my life would have been easier and I would have been able to achieve much more.

F: Right.

A: My general conclusion is that parents should  choose the best for their kids but in order to do that they should put their ego aside.

F: What do you mean when you talk about “ego”? Are you talking about the thought “My child should not have had a disability or be sick”? Where could ego lead some parents in such situations?

A: Ego is expressed through many thoughts and beliefs such as: “What will people say”,  “What will the society say” “What will people close to us think?”, “I don’t accept that my child has a defect”. I believe that when you want to help your child you will not act in a  way that helps you. If you do things that only help you, then you don’t help your child and vice versa. That’s how I think of it at least. Of course, I do also realise that the rules and norms of the local  society, the culture that parents are raised in and the acquired knowledge are very crucial in the way that the parents will eventually raise their child. However, if parents want to support their kid, they should not  care about what others will say.

F: Now I will ask you something completely hypothetical. You have experienced this situation as a child. Let’s now assume that you are a mother and your child was just diagnosed with Usher Syndrome. What would your first thought be and what your first reaction?

A: Hypothetically… (She stops and thinks).

F: Yes, hypothetically. It could be a realisitic possibility. Of course I will say that it would be better if any future kids wouldn’t be diagnosed with Usher Syndrome – things would then be easier for everyone. But let’s say that it just happened. What do you think that you would do?

A: I am sure that I would be shocked at first. I would feel guilty. Since I have it I don’t want my kids to have it too.

F: Of course we must clarify that Usher Syndrome is passed to a child when both parents have a specific defective gene, right? How possible is it that you give birth to children with the same syndrome?

A: Well, doctors told me that 1 out of my 4 children can have Usher Syndrome.

F: Yes, but this is also something relevant, right? Maybe you can have four children and none of them will have it.

A: Yes, it depends. Personally, I have met many different families of people with Usher Syndrome.  I know a person whose three out of four kids were diagnosed with Usher Syndrome and I know another person who has three completely healthy children. You cannot predict anything accurately.

F: I suppose then that if you and your future husband will be tested and he doesn’t have a “defective” gene to pass it to his children, you have many possibilities to give birth to healthy children.

A: I guess so. The funny thing is that I still do not have a genetic diagnosis of the defective gene related to Usher Syndrome myself.

F: I see. So let’s go back to what we were discussing a few minutes ago. You told me that if your child was diagnosed with Usher Syndrome you would be shocked. After shock, what you could do?

A: If my child would have my type of Usher Syndrome I already know that the child would already have hearing-vision problems. After the first official diagnosis, I would have tried to learn at what age the child should wear hearing aids. Then I would be consider the possibility of speech therapy sessions. In general, I would ask various questions to many different doctors and specialists. I would try to understand which the right first steps are. It is really important to know when hearing aids should be fitted. Moreover, I would try to find out how I can help the child with my speech, which means I would do my best to articulate and speak clearly.

F: So after the first shock you would be completely energized.

A: (She is smiling). Yes. I hope so. And that is I want to emphasize. A parent should  quickly get over the phase “Shock – guilt – depression” (I haven’t experienced it but I have heard from parents that did experience it that you cannot escape it) and try to find a balance as quickly as possible. Yes, it’s difficult but they should be there to help their  children.

F: So direct actions by parents are important.

A: Usher can nowadays be diagnosed soon after birth. So, when it is still a baby. Unfortunately, I can imagine when a child is too young parents and specialists cannot do much. Later they are able to do a lot more, so parents can take advantage of this time period to research,  to get informed, to put things in order.

F: Regarding what we have been discussing, how important is that Usher Syndrome is diagnosed on time? What can parents do better if they take their child to a doctor earlier?

A: Nice question (She is thinking a bit). In my situation, when my visual impairment was diagnosed,  some doctors  – especially Greek ones but my parents also – told me that it was better I didn’t know before, because I was more carefree.

F: Did the doctors really tell you that?

A: Yes, they thought that since there is no therapy yet, it was better that I  had not discovered anything earlier. Of course the problem was there – I realized something was wrong but was not sure, could not imagine what it was- because I was clumsy and stumbling  into things and people around me. I did  wonder quite a few times what would my life be like if I knew it since my twenties. If I had for example tried to get a driving license and found out during the tests.

F: Apart from the tests, would you have not figured out during the driving lessons that something is not quite right?

A: No, because back then (when I was 20) I still had my peripheral vision. It was when I was around 25 that I think I lost most of my peripheral vision because this is when I remember  stumbling everywhere. I honestly cannot be sure when exactly and how quickly I lost my peripheral vision.

F: So you figured out  that something was not going well because you were stumbling around. But if we assume that your vision was good until your early twenties didn’t you realize that your tunnel vision started closing? In other very simple words didn’t you have the sense that your vision has gone a little darker or decreased?

A: No and that is the problem. It was so gradual – it doesn’t happen in one go, like you wake up one day and you cannot see – so the loss of my peripheral vision and my visual field happened progressively. My brain had the time to adjust to this progressive change and I was therefore not capable to realize it. If you ask me now how my peripheral vision previously was, like 20 years ago, I cannot even remember how it was to see everything around me. Since my senses have adjusted to the new circumstances, there is no memory whatsoever.

F: I see.

A: In conclusion, even if I knew about it when I was 20, I don’t know if I would be able to do anything more. Apart from maybe visiting some doctors earlier.

F: Can you prevent something if you know it earlier?

A: The way things are now, no. Current scientific studies try to find a cure to prevent any further deterioration of the effects of the syndrome. In my case that means that my symptoms will stop getting worse and they will remain as they are now. I will not be able to have any of the lost vision and hearing restored but they will not further deteriorate.

F: That is really important, right?

A: Of course. Just think that if this therapy existed nowadays, then the health of newborn children with Usher Syndrome would never deteriorate. It would be amazing to stop the syndrome in its initial phase. I wish such therapy existed because all of us with Usher Syndrome would stop further deterioration. But since there is no therapy yet, I sort of understand why doctors told me “Beter you didn’t know it all these years” because frankly there is not much that can be done.

F: Yes, I understand what you say. It is a rational thought.

A: After the “wonderful” diagnosis “You will get blind, go home” that I received in London I want to say that in this kind of occasions a psychologist is absolutely necessary. When doctors provide the patient with a serious diagnosis, they should also provide with a psychologist. I really don’t know what they are being taught in medical schools but there were very few doctots thatI felt comfortable with . Most of them don’t want to give you further explanation than the absolutely basic. The procedure they follow more or less goes like this: This is what you have, take this medicine, and goodbye! (We are laughing). Especially in my case things were not exactly simple, I didn’t have a flu. I still remember that after my first diagnosis in London, I visited another doctor 6 months later to have some more tests and the first thing he told me was to quit smoking – ok, don’t write this! (We are laughing).

F: Hang on, why not? This is interesting! (We are laughing).

A: Anyway, the advice that I received was: “Stop smoking, stop drinking, eat healthy and exercise”. Basically he suggested a healthy life-style. When I asked him what should I do with my other health problems, like my hearing problem he answered: “Which problems? You don’t have a hearing impairment”. What happened there? The health system of England is really similar to the Dutch one. You go to the general practitioner and he/she will send you to a specialist. The specialist should receive the medical history form the general practitioner together with the referral. Apparently, the specialist didn’t have it or he couldn’t bother reading it – that happens even here in the Netherlands. They talk to you without having read your medical record! So he asked me: “Do you have a hearing impairment since you were very young?”. I gave a positive answer. And then he continued asking: “How can you understand me? Why don’t you wear hearing aids?”. I responded that I have never worn hearing aids in my life. He was absolutely shocked! And then he told me that with the symptoms I was describing I probably had Usher Syndrome. He didn’t suspect anything else, he directly told me that it was Usher Syndrome.

F: So he recognized the symptoms of the syndrome.

A: Yes and this was the first time that I ever heard the phrase “Usher Syndrome”. Of course, he then also send me home and just repeated to follow a healthy lifestyle.

F: But since he diagnosed the syndrome, didn’t he tell you anything else?

A: No. What happened after I went back home is that I started searching online about Usher Syndrome and I found a Clinic in London that was specialized in  of  Usher Syndrome. This was a scientific team that  provided with  a complete diagnosis of Usher Syndrome. I contacted them and I then found out that the doctor I had just visited was a part of this scientific team.

F: I see.

A: What I remember is that during this period of my life is that I had a lot of anger inside me, it felt like the maximum anger I ever felt. I was angry about everything and anyone. All the suppressed anger I had since my childhood regarding my disability came out as I started realizing what I have been missing out on. I remember getting angry with anyone around me, but especially with the doctors. I remembered again the first doctor  that she told me to go home. The second doctor was a member of this clinical team and even though he recognized the symptoms of Usher Syndrome,  he didn’t tell me “There is a specialized clinic for Usher Syndrome for further tests or a complete diagnosis”. Of course, my anger and the fact that I was blaming everyone else was a big problem but I just couldn’t accept that I had to do everything by myself in order to help myself because nobody else would help me. I simply couldn’t accept that doctors would behave this way. I considered that  they should at least have been more cautious.

F:The only thing that I can assume is that doctors have been taught to avoid providing with further advice and allow patients to do their own research – we are talking about an English doctor now, I am not sure how it would have been with a Greek doctor. Is it possible that he didn’t want to give you more information because he couldn’t know what your reaction would be?

A: I don’t think so.

F: I am asking you that because that is what psychologists and psychotherapists hear all the time. During our education we have been told that we should be good at our job but we should remember that our client’s problem is not our problem and we should not try too much to help or to change another person’s life especially if we don’t see such an intention like that from that person. So I am just wondering if there is a general scientific belief that except their duties, they are not obliged to do or to say anything more. If someone wants more information, he/she should ask for details. Could what I describe to you be a possible explanation of what you experienced or do you think that they are just unacceptable?Did you ever wonder why they had this attitude and not a different one?

A: I have thought about it a lot. I am now calmer that I used to be. When I was so angry I couldn’t think at all. When I was pissed off it was much easier to blame the others than myself. However, I do insist that I didn’t know anything about Usher Syndrome and I found out everything by myself. If someone doesn’t know what exactly a doctor is talking about, the doctor should be careful. When I received my first diagnosis regarding my eyes, I was completely depressed. It took me so long to start thinking again in a rational way and say: “Okay. I should learn something more about it”.

F: Yes, I see what you mean.

A: I started researching but doctors already knew what I didn’t know.

Shadiow

F: Do they? My next question is if you feel that specialists  have a good and right behavior towards people who have the same or similar syndromes like yours? Are there any ready doctors to give some kind of solutions to people with Usher Syndrome?

A: Look, it depends on which doctor you will visit and what your situation is. I know that the problem in retina can be really easily diagnosed! It is just so obvious! Regarding my type the diagnosis is “retinitis pigmentosa”. If the specialist puts the drops in someone’s eyes and checks the retina, it can immediately be seen!

F: So the problem in the retina is easily diagnosed.

A: Yes, no one had a problem with that. Everyone gave me the same diagnosis. The difficult part in my case was to combine all the symptoms. In other words if I haven’t asked the second doctor about my hearing impairment, I might have still been lost! I wouldn’t know anything about Usher Syndrome!

F: And all this happens because they don’t read the medical record and they don’t check eyes and ears at the same time, right?

A: It is usual that there are not doctors for both senses in the same center (eyes and ears). Imagine that when I visited the Usher clinic I got tested by four different specialists. One checked my eyes, another one checked my ears, a third one checked my balance and the last one was a geneticist. This team aims to test people from all the types of Usher Syndrome. Of course I understand that it’s too difficult (and probably unprofessional) eyes and ears to be checked by the same doctor. I cannot be sure if a general doctor could diagnose problems for both eyes and ears since my experience from general doctors in London and also here (The Netherlands) is really bad. You should explain analytically what is going on, they open their “bible”, they stare at you and you know that they are lost. They don’t even have a proper time for a diagnosis since their system doesn’t permit them to see you more than 10 minutes.

F: Like a race, huh? In 10 minutes you should tell everything you can and then you will hope that they will suspect something.

A: I don’t even know if they know anything about Usher Syndrome. Moreover, I was not sure how should I express myself to make them understand the whole situation. I didn’t know what to ask them and in which way.

F: Yes, but you were researching about it yourself. Did you know what you should say or not?

A: The truth is that things are difficult if you don’t know what you need or what you should ask. The other side probably won’t know either, you only have 10 minutes, so communication is difficult. In general I don’t think it is weird that doctors have difficulty in understanding what exactly Usher Syndrome is. For many people it’s difficult, even for me it’s difficult to fully comprehend it and I am the one who lives with it! However, doctors have completed some studies, so if they hear the words and they are not completely unknown to them, you expect them to show some empathy and provide with some guidance. Of course, it is crazy that a doctor who is a member of the  Usher clinic team does not tell you during the appointment that this team exists – in which he is a member himself! – and that could help you.

F: So having the experience of this specialized team that was diagnosing Usher Syndrome in London, was it a better experience for you? Do you think that atmosphere was different/better there because they had previous experience with people with Usher Syndrome? Did things go better? Did you get the answers you needed?

A: Well the coordinator of this group was an otolaryngologist (ENT). She was a very intelligent lady and she knew what she was doing. She had organized the clinic and I was mainly talking with her. She gave me the complete diagnosis. She told me: “Bsed on your clinical symptoms I confirm that you have type 2A of Usher Syndrome. I suggest you that you wear hearing aids in order to stop using your eyes for lip reading. In this way you will not feel that tired. You will realise that you will gain more energy than you have now. It would be good if you have some genetic tests especially if you want to have your own family. These tests are really expensive in England. If you intend to move in the Netherlands, you should better have these tests done there once you make the decision to have a family. The possibilities are 1 out of your 4 children to have Usher Syndrome. They are just possibilities, no one can be sure or predict anything”. I didn’t have a further discussion with the eye specialist – he had already tested my eyes.

F: So from what I understand is that they spoke to you in a good and clear way.

A: Yes, they were clear with me. Of course, I still didn’t realise the full impact of Usher Syndrome, which I did when I moved to the Netherlands. Here I learnt more about my visual impairment. I went to a revalidation centre for blind and visually impaired people. There I realized for the first time in my life how light affects my eyes and consequently my energy. I remember when I was a kid I was called “lethargic”. Except the fact that world around me was so quiet, I was quite lethargic as a person. “You are not active and energetic. You don’t run around to get things done” I remember people telling me. I was doing everything in a slow tempo and in my own time.

F: Is lethargy related to light?

A: It does not only have to do with light. All day I was trying to hear what the hell was going on around me! I was always tired. What I learned from this revalidation is that I used to consume double energy than other people to get through the day. Just think that I had a double impairment: both vision and hearing and they both demanded extra energy consumption!

F: Did you feel that you saved energy by wearing hearing aids?

A: It took me two years to feel that. I started realizing that I did not have to read lips anymore. I was able to look at somewhere else. That’s why nowadays when somebody talks to me I sometimes avoid looking at him/her. Of course. I don’t avoid him/her. I just try not to read his/her lips. I have to keep reminding myself: You can hear them now, why do you read their lips?

F: It’s so beautiful that you realized what you thought as necessary in the past (lip-reading), is not necessary anymore!

A: Yes, I learned a lot of stuff in this program. Especially the ways that light influences my energy was a really useful knowledge. They were telling me for example “Sunglasses are not enough in sunny days. You also need a hat”. I remember how much light there was in the building. They were asking  me to reduce the light that I receive by wearing sunglasses, because other people needed more light to see better. That’s how I reached the point nowadays and I understand when light bothers me and when it doesn’t. Some days I may feel that I receive too much light and I want to intervene, to change it in some way.

F: So you need controllable light, not too much.

A:  I actually need just a little. The retina filters light. Light enters from one corner and exits from another. My retina cannot filter light correctly and so light spreads everywhere. It’s like the sun is shining directly into my eyes. It blinds me.

F: I see. Now if I ask you to tell me how many doctors you have visited in your life about Usher Syndrome, can you enumerate? Additionally, do you see differences between doctors and the way they behave from country to country?

A: I think I visited more or less 20 doctors in 3 countries (Greece, England, The Netherlands). In Greece doctors told me that I don’t need hearing aids, in England they were asking me how do I live without hearing aids. Or wait, no. I made a mistake. I remember that I visited a doctor in Greece and she told me to wear hearing aids. That happened before I left for my master studies in England. This doctor was left speechless during our meeting!

F: Why?

A: Because she couldn’t realize how I managed to finish school. Moreover, she couldn’t believe that I had a bachelor. She was calling me “The miracle of Greece”. “Do you have a bachelor?” she was asking me again and again.

F: Do you want to tell me how was school for you? Were you able to hear your teachers and professors?

A: I was always sitting in the front. I missed out a lot of information (She is laughing uncomfortable).

F: I see.

A: The negative image that I have from my whole life is that I was always considered as stupid or naive kid due to lack of understanding. That is what lack of knowledge and understanding of the syndrome does. There are a lot of kids with physical or mental disabilities who just need a chance to prove what they can do. If one is patient with them, they can help them achieve much more.

F: Sure. This applies to all the people who are somehow “disabled”.

A: Well if you think about it, every person is disabled in their own way.

 F: What you say is very nice. Even if we are used to say – as I just did– that disability is what the average people, who seem normal in every aspect of them, does not have.

A: Yes.

Kyknos

F: Let’s finish the whole medical subject with one last question. What is your opinion regarding different health systems in the countries you have been. You have visited Greek, English and Dutch doctors. Is there an ideal health system? Can you choose one as the best? What are disadvantages of each one of them? In which country doctors are better trained regarding Usher Syndrome? And in order to combine all the previous information with the next question, tell me if you visit one doctor today, what are you going to ask? Who can be considered as a good doctor for your case?

A: I haven’t visited a specialized medical centre in Greece. However, since I was a kid I remember all the Greek doctors telling me that I don’t need to wear hearing aids. Of course I should mention that those that I visited knew only about my hearing impairment, they had no idea about my visual impairment. In England, I cannot say that I had the most positive experiences. Neither in the Netherlands did I have any positive experiences. The health system is different here. I know that when someone wants to buy hearing aids, one should first visit a audiologic centre to get tested. Since I had a double problem of hearing and vision I should have been sent to an audiologic centre, in order to buy the right hearing aids with more settings and possibilities. At first, I was not aware of that, I didn’t have any previous experience, no one informed me that I should go to an audiologic centre before buying the hearing aids. I just bought a pair of hearing aids that did not have the right settings. So I do not consider that I was lucky in the Netherlands either.

F: So you are not satisfied with none of these three health systems (Greek, English, Dutch). Do you think that in general doctors did not treat you properly?

A: Yes indeed. On the other hand, I met more people with disabilities here in the Netherlands. During my revalidation I met a guy with Usher Syndrome. He was about 15 years older than me so his symptoms are more advanced than mine. His vision and hearing are more decreased. He has been wearing hearing aids since he was a child. I have seen so many differences between me and him. He could not realize how I had lived all those years of my life without wearing hearing aids. Of course he managed the problem in a much better way than I did. He is in his fifties and he is a member of a project about deaf-blind people and he told me that nowadays some rules and pieces of advice started existing for people with problems of hearing and vision. There is information about the specialized medical centers that someone can visit to have some tests, in order to buy the right hearing aids with the right settings. That is what I told you earlier: It is so important to know where is the car, in what distance from you and from which direction it is coming. Your hearing aids should be perfectly set for all that and if you have vision problems, it is absolutely necessary.

F: So this guy still develops his knowledge regarding Usher Syndrome. There are many stuff that we don’t know about Usher Syndrome and we will learn about them during next years.

A: That is the case indeed. It is even difficult for doctors to remember or to know very well all the syndromes that have to do with hearing or vision problems.

F:  No, no one excepts something like that from doctors. However, they should suspect that a problem in the sense of hearing or vision could be combined with another health problem since there are many different syndromes. Regarding Usher Syndrome I don’t know if it is nowadays considered as a really rare syndrome – so many doctors don’t know it – or it is quite common so many have started learning information about it. Moreover, there should be many people with Usher Syndrome who visited doctors who were able to diagnose quickly what was going on instead of your case. But in the end everything depends on various factors, right? In this interview I have only your opinion.

A: What can I say? What I keep thinking over and over is that someone should be able to ask the right questions to doctors. This realization used to anger me a lot because I simply didn’t know what I should ask.

F: Were you mad with yourself or with others?

A: With others of course (She is laughing). There was a period that I was angry with everything and everyone. This changed later and I was angry with myself – because I could not ask the right questions. Then I realized that I should find a balance between the two in order to control my anger. Then I accepted that I didn’t know the right questions but I started asking from others to explain to me what they thought was going on. Fortunately nowadays things are different. The internet is unbelievably helpful! I don’t say that everything you find in the internet is true or right but on the other hand there is a lot of good information. You will definitely learn something more, so you will be able to ask to your doctor extra questions. However, if a doctor is not willing to help, there is nothing much you can do about it. It is always possible that he/she doesn’t have enough time (just some minutes) required to answer all the questions. The last advice that my friend with Usher Syndrome gave me was to start visiting doctors and having someone else with me. This is possible in the Netherlands and you can take someone with you.

F: Why is the presence of another person helpful?

A: Simply put, when someone doesn’t hear or see everything, one can miss out in the communication with the doctor. I can see it in myself: it depends on the day, on my mood, on my energy. It is easy to get confused or pissed off with something, especially when I feel that the doctor doesn’t want to help me. When someone else is with me, it helps to keep a balance (between a doctor and the patient). It is so important that someone is available to go with you because I can remember myself at times having just gone away from some doctors because I wanted to start breaking everything around me. Unfortunately all these negative experiences are accumulated inside me and I carry them with me in future visits to doctors. Every time I would visit a new doctor, I was thinking: What mistake are you going to make? What information will you keep from me? What will you not tell me? What will I not be able to ask you?

F: So you reached a point where you would visit a doctor and think that he/she will hide you the truth! Unbelievable, huh? I find it unthinkable because I suppose that people should visit doctors and feel calm and safe and hopeful that they will have a nice communication. You cannot to do that when you are angry…

A: Yes, you are supposed to go to a doctor calm and smiling. I recently had an annual eye check-up and I visited the eye hospital really happy and in a good mood. A friend of mine had told me that this doctor was really good, as she was specialized in Usher Syndrome too. So I get to the hospital full of positive expectations. I believed that I will learn something new about my vision problem or that I will just receive an evaluation of the current situation. I was hoping that my vision had not gotten worse. I had different tests on two different days. I went for the appointment with the specialist and to take my results and I was expecting something more than what I have already experienced. She put drops in my eyes again to check my retina – without previous notice – and that pissed me off because with those drops you are not able to see clearly for about 4 hours. She started asking me information that was already in my medical record. When I told her that this information is already in her computer she responded that if I didn’t mind , she prefers to hear it from me. I was already frustrated by then. I had been waiting for this fucking appointment for 4 months and of course the doctor had not even read my record before the meeting. I believe that she had not even glanced it! I remember that while we were talking I clarified that they haven’t found anything in my genes regarding Usher Syndrome. She asked me where my tests were and I told her that they should be in her computer. She said that they weren’t. Then my anger moved one more level up. I searched in my folder –I have by now realized that I should always carry it with me  not only for the eyes but for any health problem because I am almost sure that no one reads my medical record! I found the tests and gave them to her. I asked her: “What can we do from now on?”. She answered that my tests show that I don’t have Usher Syndrome. I asked her again: “Nice. What can we do now? I want an official diagnosis from you. It is important to me to know what I have and what I haven’t. I want to know so  that I can follow any scientific developments”. Then she told me that she will send me back to hospital to have some more tests! (She is laughing). Then I raised my hand and I showed her a piece of paper that I was holding. I told her that I wrote some questions that I want to ask her but with the drops in my eyes it was impossible for me to read them. I asked her if I was better or worse. She told me that maybe I was even a little better. After the conversation about my visual field I asked her if my cataract that was in a primary phase has gone worse. She answered “I don’t know” (We are laughing). You can imagine how angry I was by this moment because she didn’t look either my previous test regarding cataract. Finally, I told her that I need a medical referral for a geneticist to have some more tests about Usher Syndrome, I wanted a prescription for new glasses and a certificate of my visual impairment for everyone who will ask comfirmation of it. Then she told me “Ok, I will send them to you” and then I started calling and calling them for months and I finally got the two of the three papers I asked. The medical referral for the geneticist has never come to my hands and I have been waiting for 5 months! I cannot remember anymore how many times I called and went there and asked for it. I also had a big problem getting a prescription for glasses. She told me that she was not the right person to give me something like that! I answered her: “Excuse me, this is an eye hospital, yes or no? I have done so many tests here. Are you telling me that you are not the right person to print a page with the  prescription?”. Her answer was that I should visit another organization to get a prescription and in order to do that I would have to wait for another 5 months! So I responded: “You don’t get it, do you? This is an EYE HOSPITAL. You are an EYE SPECIALIST. I want the prescription from you”. Two months went by and I finally received a letter saying that she couldn’t provide me with a prescription. After a lot of fights and many-many visits she finally told me that the prescription I already had in my current glasses was good to buy new glasses, as it hadn’t changed. I didn’t need a new one. Imagine how difficult it was to say that!

F: It is unbelievable how they could behave to people with disabilities. They just don’t care.

A: I still try to understand how things work here in the Netherlands. Customer service is unacceptable! (negative tone). I can understand that there is a pressure of time to everything – e.g. in 10 minutes you should finish you appointment – but what drives me crazy is the fact that they claim all the time how busy they are! Ok, you are busy for one month. I don’t accept one day more! This is your job, you should send medical papers to your clients. You are a secretary and that is your  job, so send me my papers now!

F: One of your most negative experiences right?

A: I think that my most negative experience will always be that with the English doctor and her phrase: “You will get blind in 5 years. Go home”. However, this one with the Dutch doctor was absolutely negative! It is just insane how many good things my friend told me about her. I remember I was talking in Dutch with her but that day was not so good for my Dutch. She suggested to me to continue in English and I told her “It would be much better. Thank you”. But after that everything went bad. I don’t know what is wrong. Maybe that I am not Dutch. Is it possible that they don’t want to bother a lot just because I am a foreigner?

F: Well you live here, you work here, you pay Dutch taxes, you pay a Dutch health insurance. What you need more to be considered as a worthy citizen of this country?

A: I think that only the fact that I am paying a Dutch health insurance should be enough. However, sometimes it doesn’t seem enough in the Netherlands.

F: I think it is good to write this down so some people can read it. What else do you want to say?

A: In general we should be well-informed about a health system and maybe take someone with us – for example a social worker – who will have experience with the Dutch health system and he/she will  help us with the language. In general Dutch are needed everywhere. I cannot say that I have had the greatest communication with Dutch people when I was talking  to them in English.

F: Why? Dutch don’t speak very good English or if you speak only English you don’t receive proper services?

A: I believe that most Dutch people know good English in order to go on vacation. Doctors must know English because they always read scientific books and articles in English, but even then, talking and communicating is something different. I don’t know. There are some that speak English absolutely fluently and some others that speak English only when they go on holiday and they try to understand what I am trying to tell them.

F: What I have experienced so far regarding Dutch people is that we talk and at some point I feel that a Dutch did not understand what I just said. Most of the times they will nod with their heads and they will show that they understand you. It is so rare to hear someone telling you “Sorry, I didn’t get you. Say it again”.

A: Yes, this is ego. I have also noticed what you are describing. I can see it in their eyes but also in their body language – I am used to that since I was a kid – and I know when they don’t understand me but don’t say it.

End of the Part 1

          * The photos of the interview were chosen by Afroditi

 

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