Rare Disesae: Happy rare disease day!

It might seem weird to wish someone a happy rare disease day but I am chronically ill with a rare disease. And that is why I know how important it is to create awareness for all kinds of (rare) diseases and chronic illnesses.

 

I have been chronically ill with a rare disease for over 10 years, I am 1 in a 1.000.000 and I have CAPS disease. 

 

In the past recent months I have started to share more about myself, about my rare disease but never wrote down the words I have CAPS and in honour of rare disease day 2021, I felt it was time to just do so. CAPS is short for Cryopyrin-associated periodic syndrome which is an overarching name for multiple diseases. I'll probably write another blog about what CAPS is and all soon but for now, it is enough. I just know that it can be very difficult when you have are rare disease to find like-minded people and therefore I wanted to just put it out there and to let you, someone know that if you are that person with a rare disease; 

 

You are not alone, together we are many.

x Poppy

 

 

Rare disease: it has been one heck of a year in quarantine

 

It has been a year, a little over a year even and the world has not been the same, I am not the same. I never thought that at the beginning of this pandemic I would feel the way I do today. It was not that I thought it would be all over in a year or that it would have blown over or anything. I actually thought I would have been able to handle it better. An in the beginning I did, although with a few more tears than normal that is but as you may know I am chronically ill with a rare disease and therefore I am no stranger to being in isolation. I have had periods before where I didn’t leave the house for days, weeks, months and where even a visit to the doctor would be a breath of fresh air. I knew how it felt not being able to do all the things I enjoyed before I got sick, I knew how it felt not being able to plan ahead or just being able to be spontaneous and just live my life. I hoped I would have been able to not let this pandemic get to me but it has been hard very hard. The weird thing is just before the pandemic hit us all I started seeing progress form my new treatment and I was able to even go to the store alone, I was able to go visit my friends by myself, I even started taking Pilates lessons. And sure this sounds a lot more rose coloured than it was cause still I had loads of precautions to think about and there was nothing spontaneous about these activities and sure if I planned one thing I needed to recover the rest of the week. But I had a little of my freedom back. Freedom meaning in my case a glimpse of the life I had before I got sick. I think that is why it has been more difficult because I was getting into a better place than I was in years. Before this treatment, that I am now on, I didn’t think it would ever be possible to regain even the slightest piece of independence. I spoke about this in a previous blog but the first time, since I got sick, that I took a bus all by myself, I actually cried. It was such a feeling of freedom to be able to do something on my own. Today I haven't left the house in over a year, I haven't seen friends, haven't been to Pilates not even to the store. And not just me because my family has been cooped up inside the house, just the three of us, yeeeh for all the damaged genes, with the only exception that my brother is a teacher so he had classes to teach before we went in lockdown. But all joking about bad genes aside we are all high-risk meaning we could and most likely would get very ill if we would get the coronavirus. Yesterday our government let us know some restrictions our about to be lifted like (most of) the (high)schools are going to start again with less zoom meeting and more real-life classroom teaching. Contact profession has been giving some green light which means things like hairdressers and masseuses can open up their salons again. It scares me. And I mean don't get me wrong I am not against opening up again that is not what I mean. It scares me because vaccination seems to be going slow, infections are rising so it is a scary time to loosen restrictions. But trust me when I say I get it that children, students want to go back to school and going to the salon is also about the inside, people are getting lonely, sad even depressed, struggling to pass this year struggling to keep their shop open. I just hope it is enough, I hope that there won't be a dip in following guidelines but more of a collective let's do it together like it was at the beginning of the pandemic. So we can get out of it together instead of groups of people saying we should split society into those who are vulnerable and those who are not. I don't want to waste too many words on that but I think someone like that doesn't know that people who are vulnerable already are in so many cases on the outside and already take so many precautions nowadays, that this is a scenario that is just inhumane and complete and utter unspeakable of. But I want to end on a more positive note: I hope that these loosening of restrictions will lead to a chance for everyone to catch their breath whether it is financially, mentally, emotionally, physically I hope spirits will be lifted again and we create a we-culture like at the beginning of this pandemic. Because it is not over yet but together we can make it more bearable and together we can get out of this situation. As for me, I hope I can get myself out of this rud I am in. I want to look forward but there is so little to look forward to right now which makes doing that so difficult. I am trying to make myself do a thing I enjoy but it is so hard to even put on make-up or brushing my hair some days or getting into something else then sweats let alone picking up a pencil to draw or a crochet hook to crochet. But I am trying and I am really proud of the fact I made two posts this month, so focussing on the positive I hope will help me. I don't think I will be leaving the house anytime soon and surely (knocks on wood nothing happens) I will not be leaving the house before I get the vaccine. But it is taking its toll on me and it is hard not to burst into tears, it's difficult not to cry myself to sleep every night but I am really thankful I am not alone, that I do not live alone, that I live with people who love me, who understand me and vice versa. So that was it for ending more positive I guess anyway, if you ever need someone to talk to I am always up for a friendly chat, you can find me on Instagram and Twitter just send me a dm. Wishing you a lovely day and stay safe!

x Poppy

Rare disease: 180 days of being in (Corona) isolation

Yep you read that right it has been 180 days, that is indeed half a year. And even though I am no stranger to being isolated it is kind of driving me up the wall right now. So why not go outside you say, go for a walk, get some groceries, enjoy some company etcetera. Well for someone that is chronically ill with a rare disease it is just not that simple. I cannot take the risk of getting infected with the Coronavirus and because I am at high risk it is just better to eliminate as much risk as I can.

Anyways I have been seeing more and more articles, stories etc. about why should we be careful around the vulnerable, the chronically ill and the elderly?! To put it in a very blunt way; Why should we save them because they were going to die soon anyway. Well, I wasn’t planning on dying anytime soon, I want to make something of my life. I am 32 years old and have been chronically ill for over the past 12 years and have been living in isolation for long periods at a time during these twelve years, meaning not going outside at all, not seeing anyone else besides my family or maybe only at those time go outside for the necessary doctor's appointment. About 2 years ago I finally got to test a new medicine and they make my life more bearable more of a life again. It’s not that I am not ill anymore it hasn’t taken away my rare the disease but in a nutshell, the flare-ups are less frequent which gives me more time. Now Corona has come along and I am (back) in complete isolation since the end of February. I haven’t left my house at all. At first, there was so much understanding and so much appreciation for all medical staff for people who already live in isolation etc. I saw so many started initiatives to bring people “together” to help out those in need. I do understand that the rules can and are confusing and ever contradicting at some times. I get that everyone wants to get back to normal because it would also be devastating for the economy if it didn’t. And if they are so worried about the economy regarding these rules and not following them anyway what do they think will happen to the economy if another lockdown will happen god forbid even a longer one or worse one. Isn't it far easier to adjust to the guidelines for a while and I am talking about those who really ignore all rules and even endanger others by doing so. Even if that someone doesn't get sick they could infect someone else. Someone vulnerable, someone who works in healthcare, someone with a vital profession. Don't they understand that even though, over here in the Netherlands, that the death toll and the infections aren't as high as they where that it doesn't mean we are out of the woods? We do know now how to treat people better and there are more bed in the hospital open to take care of those who are sick but there still isn't a vaccine or medication for this so unless we are all careful and take our responsibility we can keep on going to "control" the virus. Or are all the people who feel that way, who think they won't get sick, who don't care about the butterfly effect they could set in motion are they such tough guys they just don’t care a "little" (chronically) ill person less right?!

I don't understand why not just hold to at least those few rules and guidelines that are there, like keeping distance, no shaking hands and hugging (those outside your bubble), wearing a mask in places where it is asked of you, washing your hands frequently, avoid crowded places and not thinking well they are going... Things like that. 

What concerns me more is that I hear/read/see more and that those who are vulnerable should keep their distance, but how would this work in a supermarket for instance. Would someone write it on their face, wear a tag, announce themselves every few minutes just to be sure others keep away, please enlighten me. And in a country like the Netherlands where I am from, we have about 17 million people. Statistics say that on January first 2018 we had about 9,9 million people known who have any type of form of chronic disease. Meaning highly likely no form of recovery is in sight. That was about 58% of the population at the time. Just for those who still think it is just about a few people who we need to be careful around. 

I am someone’s daughter.

I am someone’s sister.

I am a person of flesh and blood.

I am a person with feelings.

I am not my rare disease.

I am someone!

So why are so many saying let the vulnerable keep distance, you wouldn’t be able to tell if you saw me that I am very ill and am at high risk. Why is it so difficult to hold to those few rules for just a little while longer, why is it that my life has no value compared to someone who isn’t ill, why can’t we do this together, all these people didn’t have to die yet, it is not only nature, besides isn’t it only humane to take care of one another. So today it has been exactly 180 days of being in isolation and I am starting to see my future getting grimmer by the minute. I had hoped people would become more understanding but alas I hear more and more that people like me shouldn’t be here anyway and I am only a waste of space. Great! I can only hope that 180 is a symbolic number to change and a change of mindset and caring for one another.

And I have said this before but I will post this here again:

Just please follow the guidelines of your government and health department, like:

• wash your hands regularly 
• don't shake hands, avoid close contact with others
• avoid touching your eyes, nose or mouth with (unwashed) hands
• cough and sneeze in your elbow
• avoid going to crowded places
• stay at home if you are sick
• avoid contact with people who are sick
• work from home if you can
• don’t visit those who are at high risk like your elderly or (chronically) ill relatives/friends
• check-in on one another (family, friends, neighbours) call or text them
• help if you can
• 
  

Regarding that last note even if it is just calling your grandparents, parents, siblings, friends or other relatives who might feel or be alone or feel or be frightened. Maybe hang a note in you apartment complex asking if someone needs help or share some more positivity on social media.

So please don’t be that person who says I’m not scared of shaking someone’s hand or hugging someone, well I don’t want you to be scared at all! I want you to be sensible and responsible for yourself, your loved ones and others. I get we all process this in different ways and I don't want to be pointing fingers or telling people what to do and don't do. But I do want to say that don't yell at someone who hoards you don't know their reason, don't diminish someone who panics they could have already sick relatives/friends, please take your responsibilities, please use your common sense, please don’t panic, please don’t downgrade the seriousness, please if you can do help out someone, please don't judge others, please stay safe.

I hope everyone stays as safe and healthy as they can be and try to do everything in their power to prevent it from spreading further. 

x Poppy

Rare disease: (in)visible illness and Covid-19

Hi, I am 32 and chronically ill. If you would see me you probably wouldn’t be able to tell. Because there are no visible signs per se and also because if I leave the house that probably means I have to or feel well enough... Due to the global pandemic, I have been self-isolating since the end of February and haven’t left the house, not at all, yup that is almost 6 months that I haven’t left the house. But I know there are so many others just like me in similar situations and I am not just talking about the elderly we are all been warned to be careful around I am talking about small children, young adults, adults who are dealing with a visible and invisible illness, which makes us extra vulnerable to infections, diseases, viruses such as COVID-19. I know a lot of people, including doctors, have told the public about the health hazards for the elderly and vulnerable. But you are not always able to tell or know if someone is (chronically) ill and/or has (other) underlying health issues. Vulnerable people are not just the elderly. It can be your best friend, co-worker, aunt/uncle, sibling, neighbour, cousin... And therefore I wanted to create a little more awareness of why it is important to follow guidelines. I ask you to consider keeping your bubble small with those in your household and keep your distance from those who don’t belong in your household. Because even if you are not coming in contact with someone who would be at high risk you could pass it on to someone who will be in contact with someone who would be at high risk. Please wear a mask when asked. (*not speaking about those who physically/medically are not able to!) Even if you are not visiting your elderly (grand)parents because we are advised not to do so or with great caution (if you do so, thank you for that, that must be difficult!) but you still can come across others who are also very vulnerable without you knowing. This virus isn’t only dangerous for the elderly it is also dangerous for the vulnerable and that means any age. This virus does not discriminate it doesn’t care where you are from, where you have been, what you have done good or bad in your life and it does not care how old you are. So my main point I want to make is this virus could affect anyone, someone you may consider looking young, healthy and vital. But let’s imagine you are that person you consider looking young, healthy and vital but actually you are at high risk but no one would know because you can’t tell from just looking at you. When you are being careful following guidelines and such and someone else isn’t just because that person might not think anything of it and doesn’t visit their (grand)parents so that someone doesn’t feel the need to follow other guidelines because you do not qualify to them as being high risk. You can not judge a book by its cover, you can carry it with you, maybe and hopefully you do not get (very) sick from it but still pass it on to someone who will get very sick and might die. And with small gestures like keeping a distance from those outside your bubble, no shaking hands or hugging and wearing a mask in places where it is asked, we will be able to control this virus. Which is so important until there will be a vaccine or medicine. And some might say well they should stay inside than, I want my freedom. Or whatever bullocks I’ve over the past couple of weeks. I get you want your freedom and do whatever you want but aren’t we all human and isn’t it a humane thing to do to this together and help control this global pandemic. If this doesn’t help you see from a different perspective and you are someone who thinks are vulnerable people should stay inside or such. Have you realized while saying things like that; like staying inside that it isn’t always an option people might need to get there medicine, food, supplies, go to work, have doctors appointments etc. They might not be able to get or ask help from others, to have their prescriptions picked up or have their groceries delivered or work from home. Just like you need to be somewhere or go somewhere. So that in my mind is not a valid point. All I ask is for those who didn’t understand read and share this to help create awareness because even though we can’t always be together we need to do this together.

Rare Disease: the adrenaline injection

Have you ever heard of an adrenaline injection like an EpiPen or a Jext-pen? These are injections given to a person when they are having an allergic reaction. There are over 1.000.000 different types of allergies in which you could end up using an adrenaline injection in case of anaphylaxis, which could lead to shock or even death. Anaphylaxis is a potentially life-threatening, severe allergic reaction and should always be treated as a medical emergency. Going into anaphylaxis can be quite scary, it is heavier than a "regular" allergic reaction. I used hyphens here because there isn't really anything normal about an allergic reaction but what I mean is your normal. Your body is letting go of a chemical called histamine which causes your throat to swell up, which makes it difficult to breathe, your heart feels like it is going a million beats a minute, your lips and tongue swell up, you experience a severe skin rash and it even affects multiple organs. This all could lead to the person to faint and even die. That is why it is always very important that when anaphylaxis is suspected you give the person or if the person is you, yourself an adrenaline injection. Because not giving the adrenaline injection can be way more harmful than not. Even when in hindsight it may not have been necessary. 

You may have already read in a previous posts that I am chronically ill with a rare disease. Part of that disease is that I can get severe allergic reactions, that means I always have to carry an adrenaline injection with me.

I have been carrying an adrenaline injection with me wherever I go for over the past 12 years. And I have had to use it a couple of times, which can be quite scary. It is not really difficult to use the adrenaline injection and anyone could do this which is a good thing because like in my case I could end up in shock and there is a possibility then that I can't do it myself. It is always important that when you get an adrenaline injection to carry with you, your direct surroundings do know you are carrying such an injection, in case they would have to use it on you and even more important is it that they know the correct way of using the adrenaline injection.

I have had a friend of mine once asked me about it (this is years and years ago when I first started to carry an EpiPen) and she wanted to know where I kept it and when she should use it on me. So as we were thankfully talking about the subject she asked me; so basically it comes to taking the injection and sticking it into your heart. PLEASE DON'T! This is a totally different procedure and has nothing to do with inserting an EpiPen.

And it isn't that crazy she would think something like that maybe you even thought about that being the right thing to do. I mean it is what John Travolta does to Uma Thurman in Pulp fiction. Well, actually such a thing doesn't even exist nowadays. There is no form of an adrenaline injection, that you would carry around with you in case of an allergic reaction, where you would stick a needle through someone's heart. So that has been a widely known misconception about adrenaline injections.

And yes of course, some adrenaline injections may work differently from one another. I only had the experiences myself with an EpiPen and a Jext pen which work quite the same. But as far as I know there is really no such adrenaline pen which you would carry that would work like that.

I never like the term allergies because some people don't understand, they just wave it away like it is nothing or even make fun of a person with an allergy saying things like well you shouldn't eat that or you shouldn't have done that and they just don't fully grasp how dangerous allergies can be. That is hurtful and I hope if you were unaware of what lies behind the word allergy that what I wrote helps you understand and be more understanding. And when it comes to allergies or intolerances I have always believed that you cannot know or judge something if you don't know how it feels or how harmful it can be. And all allergies are different even the same one allergy can be more severe for one person than another. So if you ever come across someone who tells you about their allergy please take time to listen and take them seriously. I hope you can understand it can be difficult for someone to share that with you so just try and be as understanding as you can even though you might not (fully) understand their situation be open to what they are sharing with you. And sometimes someone just needs a listening ear or even a shoulder to cry on.

If you would like some more information about an EpiPen or a Jext-pen I have put the links of a few of the official websites down below. And there are even official EpiPen and Jext-pen apps available for Apple and Android phones. These apps are quite similar to what they offer. They both seem to have an alert option as to when you'll be needing to replace your adrenaline injection and instructions on how to use the adrenaline injection. I say seem to cause I am not a fan of the apps because they aren't available to anyone who downloads it. Because for the EpiPen app you'll need a password from your doctor which I think isn't even for every country only for the UK or China and when it comes to the Jext app you'll need to enter a number that is on your Jext-pen. I think for both apps it is a missed opportunity to make them only available for the patient, I think it could come in handy in a lot of situations like when you are a teacher and one of your students is carrying an adrenaline injection or maybe one of your coworkers. Like I said before it is always a good idea to let your direct surroundings now you are carrying an adrenaline injection, for what purpose you are carrying it and how one can help and handle if something would happen. Because sometimes you are not able to do it alone or at all. So when I sum it all up I won't be using any of the apps because it has no extra value to me but I do understand the value for some and therefore it might for you. That why I did want to share the existence of the apps. If like me you are not going to use the apps, what I do is set a reminder in my phone for when I should get a new adrenaline injection every time I get a new one and don't forget that once you used an adrenaline injection you should always replace it with a new one. 

x Poppy

EpiPen app Logo

Jext app Logo

Other than using my own experiences I have also used a couple of sources to write this to the best of my abilities. 

If you suspect you have an allergy please console your doctor.

informative websites:
https://www.epipen.com/en 
https://jext.co.uk/

sources:
https://www.epipen.com/en
https://jext.co.uk/
https://www.allergy.org.au/patients/allergy-treatment/adrenaline-for-severe-allergies
https://www.leef.nl/kennisbank/de-adrenaline-injectiepen-tegen-anafylaxie-in-voorbereiding-op-vakantie/