14 September 2023

Living with Prader-Willi Syndrome

This is Rowan. Rowan is smiley, sweet, cuddly, and so, so incredible. Rowan also lives with a lifelong genetic condition that we will have to manage for the rest of our lives. This condition does not define her, but is something she has. She will always be completely perfect to us.

This is a very long post about her birth and her diagnosis, and it’s a lot of trauma dumping, so sorry in advance, or maybe you’re welcome, if you’re nosey like me and like knowing everything about everyone. I feel like writing things out is the best way to explain feelings that I don’t fully know how to explain, so consider this a peek into a journal entry I probably should’ve kept private.

When I was pregnant with my daughter, I felt this overwhelming sense of something being…off. I couldn’t explain it, and when I tried to, I sounded like a paranoid wreck, but in my head, I knew that my girl was going to be different. People can try and tell me now that I am projecting it onto my memories, now that we know for sure, but I think I knew from the very beginning that she wouldn’t be like our son. 

Throughout the pregnancy, my anxiety hit an all-time high. I would spend hours obsessing over every detail the doctors told us, panicking over her movements, worrying about everything I did, worrying that I was harming her, but I think this anxiety was my mind’s way of telling me that, for once, my fear that everything wasn’t as it should be, was finally right. Call it a mother’s intuition, call it a lightening strike of the anxiety finally hitting a point, but I knew something was not as it should be. There seemed to be event after event that would tell me that I was right, and that I needed to say, but everything seemed to be fine to everyone else, so I assumed I was just being an overthinker, because that is one of my great skills in life. First she measured small, then she was fine, then there were extra checks just to make sure everything was alright, there was diabetes, then there was a chest infection that tore my stomach muscles open coughing, there were checks to make sure she was moving, again and again, there was always something, and all the while the voice in my head was screaming louder and louder to pay attention. 

When I was expecting my son, he would roll and move about like it was nobody’s business. He would swoop, and swim, and kick, and let me know he was there, and as much as it might’ve hurt sometimes, I loved every movement. My daughter was not the same. Her swooping and swimming was more gentle, less frequent, her kicks rarely paralysed my bladder like her brother’s did, and she was a much quieter baby. Initially I had hoped that I was going to be blessed with the rarest of babies; one who sleeps through the night, but that voice in my head kept telling me it was more than that. I would lie awake holding my belly, praying for a movement, bargaining  with God that if I felt her move, I could sleep, and tomorrow i would do better. Every night. Every night I eventually felt her move, but nothing could assuage my fears, no reassurances, no number of appointments, nothing could soothe that voice in my head. 

Her birth was not what I had hoped for, but after a bad experience with my son, I didn’t have particularly high hopes. I had asked not to be induced, so, of course, I was induced, and from my previous experience, I knew I wasn’t in for a good time. The days dragged by waiting for something to happen, for a delivery room to open up, and then at midnight, I was moved to the room to get ready to meet her. I rang my partner at an ungodly hour, and told him to take his time because it would be a while. Prophetic words, unfortunately. 

We were in there for hours, and our girl had decided now was the time to swim. She kept moving away from the monitor, so they kept losing her heartbeat. Birth isn’t the most comfortable of experiences, but having to contort yourself into shapes so a nurse can try hold a monitor on you is truly not the peaceful vibe a person needs in a situation like this. After a few more emergencies than I would have cared for, a team full of doctors and nurses seeing a whole lot more of me than I ever wanted, and more people having their hands places they shouldn’t be, I was done. I was so exhausted I couldn’t even catch my breath. They had tried breaking my waters twice, with no success, our girl kept moving away from the monitor, and it was getting increasingly difficult to keep an eye on her, I didn’t know how much more I could take. A doctor came in and said there was concern that the cord was too short, and that if we carried on, there could be a risk to her, if they were successful in breaking my waters. It was decided to go for an emergency section, which was absolutely nowhere on the birth plan, but I needed her out and safe. I walked to the operating room and could feel my heartbeat so loud, it was reverberating in my ears, drowned out only by the sound of my heavy breathing as I desperately tried not to have a panic attack in the middle of the operating room. My partner was told to wait, and I could hear the blood rushing in my ears as I sat and watched people rush around me, and the tears I was trying so hard to hold back began to overflow. The doctors and nurses were so kind and patient with me, but the whole time all I could think was “ I wish I’d worn better pyjamas for this”. Queen of flashing everything at the medical professionals. After that, everything seemed to move so quickly, I was prepped and ready, they started working away, just as my partner was brought in, and I’ve never seen a man look more traumatised, but at that point I just kept thinking how thirsty I was and how dry my lips were. I think I asked for a lip balm about 12 times, to no avail, unfortunately. 

Soon our girl was earthside, and while her brother shot out of me with the force of a bullet, she was gently lifted from me, shown over the draping all around me, and then taken away. I felt so many emotions; pure joy that she was here, panic that something was wrong, guilt and anger that my body couldn’t do it the way I wanted. I felt completely overwhelmed, and if I didn’t see her in the next 10 seconds, I was going to send my partner over to grab her. Finally she was brought to us, and as soon as I saw her perfect face, I knew no matter what, I would go to the ends of the earth for her. The instant, fierce need to protect her and keep her safe overtook everything else, my arms ached to hold her and I just marvelled at this tiny, beautiful angel.

As we went to recovery, I tried latching her onto me, but she wasn’t having any of it, but people had told us that section babies are sleepier, it can take longer for them to feed, don’t worry about it, so I tried to keep calm. It didn’t occur to me that there would be an issue with breastfeeding, it had come so naturally with my son, I assumed it would be the same with her. We found out she did have a short cord, so the section was inevitable to keep us both safe, which helped ease my guilt temporarily. We moved onto the ward, and I tried feeding her again, but she still wasn’t having it, but she seemed so content that we thought nothing of it. Then after more time passed, I knew that something wasn’t right. She had been put in an incubator because her temperature was low, and I could barely move after the section, so I felt trapped in my bed, just stuck looking at her. I was too scared to touch her, but too worried to not try. A nurse came over and asked about the feeding, and I said we hadn’t been able to latch her on, and asked if something could be wrong. She took her and syringed some formula in, and i tried my best to hand express, but only managing to cry tears of frustration. Then the nurse said that she felt quite “floppy”, and she wanted the doctor to have a look, and see if there was maybe a reason she wasn’t feeding. This was the start of what felt like a nightmare. A doctor came and wheeled her away for tests, as I lay in bed feeling completely useless—unable to move, unable to feed my baby, unable to do anything but cry. The doctor brought her back after a few preliminary checks, and agreed something wasn’t right. They decided to put a cannula on her tiny hands to help pump antibiotics through her system, in the hopes that it was an infection, and I prayed harder than I ever had that this would be it. But, of course, it wasn’t. I heard a nurse walking away and talking about us saying “i’ve got one that won’t feed, it’s not looking good”, as if the curtains that she never closed provided some kind of sound barrier. The next day she was taken away for more checks, and brought back with word that the consultant paediatrician would come and see us soon, so we knew something was wrong. The curtains around my bed that had remained open for nurses and people to say hello, now remained closed. She came and brought students and other doctors with her to discuss what was happening, and it felt like the words were flying around the room and out the window, because not a single one told me why my baby couldn’t wake up, couldn’t feed, and couldn’t move. Thank god Stefan was there, because otherwise I would never have known what was happening. We were moved to a private ward, and nice as it was to have our own space, this was what fully solidified it for me that everything wasn’t going to be okay. There were talks of MRI’s, infections, genetic testing, lists of conditions that it could be, but “very likely it isn’t this one, this one is very rare”. I googled the first condition and immediately broke down at what it could mean for her, and vowed to not google again. I let Stefan research everything because I couldn’t stand to read any of it, because every time I did, the only conclusion that my brain could come to, was that my beautiful girl was going to die.The genetic testing was done, and she was taken for an MRI to see if an injury during birth was causing the issues, and seeing her tiny little body in that machine is something that will stay with me forever. It felt very odd to hope she had been injured during birth, but that was the scenario that had the best outcome, so we banked all our hopes on it, even though I think we knew it was pointless. 

We were finally allowed home because she had started being able to drink from a bottle, and so began the exhausting journey of exclusively pumping for me. In my head, all I could think was, this is at least one thing I can do for her, so I very stubbornly stuck to my guns on this. We went home to wait for results, and try to adjust to our new life as a family of four. Her brother adores every hair on her head, to him, she is the most perfect little angel in the whole world, and we are so incredibly lucky to have been blessed with such a kind, sweet boy. Finally we got a call (after much pestering from me), the MRI was clear. It was a genetic issue we were looking at. Even though I think I had tried to prepare myself for this, it felt like the edges of our little world had begun to crumble, and I was just waiting for the inevitable collapse. We had an appointment for her to have more bloods taken coming up, and when we went in, they said our consultant would be with us soon. We hadn’t been meant to see her that day, so we knew something was back. I tried so hard to hope for the best possible outcome, as she brought us into a consultation room. I could hear us making inane small talk, all the while wanting to scream at her to tell me what was wrong. We sat down, and she very gently told us that the “very rare, unlikely to be condition” was, in fact, what she had. So rare, that I had never heard of it. Prader-Willi Syndrome. A genetic disorder that causes behavioural, emotional, and physical development issues, a disorder that means that, from around the age of 4, our daughter’s brain will no longer recognise she is full. She will continue to eat anything and everything, because to her, her brain will tell her that she is starving. That she needs to eat to survive. She needs a growth hormone to help regulate normal bodily function, and she will not experience puberty or grow up like other girls. She is unlikely to ever have kids of her own, and she may never develop enough mentally to be able to live on her own. Often people with PWS are unable to live independently, because if left to live freely, they will eat themselves to death. As the consultant told us about the condition, I could feel my brain begin to fog over, and the walls around our little family bubble that I had created in my head, began to crash down. This was a lifelong condition, with no cure, something we would have to live with every day. I could feel the hot tears falling, as I nodded, trying to pretend like i could hear what was happening, and could feel the tears soaking into my mask, the sympathetic eyes of the consultant burning into me. Stefan stayed so strong the whole way through, as I crumbled beside him. We went home with leaflets about the condition and advice on facebook groups for support, and promises of appointments in the new year. Our beautiful, tiny Christmas baby, brand new to the world, and already the promise of a life that would be so much work for her. 

Our son is a textbook example of a baby who overachieved. He could hold his head up from he was out of the womb, happily waving his head about to look around him, sitting up and crawling in no time, walking before he was a year old. He would babble and laugh and talk all day long if he was allowed, he would fight sleep because there was just so much to do and see, and so much babbling to do. He was, and still is, exceptional. I’m not saying that to blow smoke up his hole, he is a remarkably bright kid, even if he hates doing homework, loathes being forced into reading, and cries over practicing writing. He’s more than capable, and he has a logical mind that escapes me, but he would much rather run about like a mad hallion than sit inside with work. Having a son that was so incredibly capable, made watching Rowan even harder. We kept sitting, and waiting and watching her, begging for something like what Elijah could do. Where he had been awake, alert, strong, she was lethargic, sleepy, and floppy. She had no strength in her legs and arms, her neck couldn’t hold her head up, she wasn’t able to wake herself up for a feed, or hold herself to the breast. Often babies with PWS are tube fed because they aren’t able to get enough food or nutrients due to the lethargy and hypotonia, we were very lucky that we were able to avoid this. She had just enough strength —and stubbornness—to persevere with the bottle, and I am eternally grateful for that. Everyday I would try latch her on, in the hopes that I could give her the same feeding journey I was able to give her brother, but every time would end in tears—either hers, or mine. It’s incredibly difficult to all of a sudden not be able to do something that you know your body can do, and it’s very hard not to feel rejected when your baby just can’t latch on. Being at home with her, just watching her, knowing that she had a lifetime ahead of her that I had no way of preparing for was incredibly overwhelming. That feeling has never disappeared. Every day I hope it’ll ease, the panic, the sudden feeling of a lump in my throat, trying not to cry, the feeling that I have somehow failed her, that I did something wrong when I was growing her. A litany of irrational thoughts that run through my head, and every day I hope that today will be the day that I wake up and don’t feel guilty, that today i’ll have done enough for her, exercised her enough, read to her enough, given her every advantage that I can. I know mum guilt is something we all experience with our kids; I always worry that I haven’t paid enough attention to Eli, haven’t played with him enough, that I was too short tempered, but with Rowan, there’s an extra level of fear. Everything that I feel like I haven’t done with her, is another thing that is going to hold her back, that I am hindering her, not helping her. It’s exhausting being a parent, it’s even more exhausting when you second guess and question everything you are doing, when you spend hours of the day worrying about a future that is impossible to predict. There are so many conditions linked to PWS, it’s like a really shitty lottery situation trying to imagine which one will she end up with. Will it be mild, will it be severe, will she be able to go to school with her brother, will we have to fight for a place in a school that doesn’t have enough places for kids with additional needs, the list of questions goes on and on. I have spent more time on the phone chasing hospitals and doctors for her than I ever have for myself. We have gone to appointment after appointment, and still I feel like I will never be prepared for her future. I look at her tiny, perfect, beautiful face, and a wave of fear hits me so suddenly that I feel like can’t move. I know what it is like it live in a larger body in this society, and the cruelty of people knows no bounds, and I am so scared that she will experience the same treatment that I received for so long. In a cruel twist, whilst Rowan’s brain believes she is starving, her body will stop being able to metabolise food at the same rate as other people, and she will actually require less calories. If she is not on a strict calorie controlled diet, she will gain weight at an exponential rate, and her lack of muscle tone makes it much harder for her to do exercise in the future. I don’t fear her being overweight, I fear how other people will treat her. It does feel like a horrible karma to me though, to have spent so long obsessing over being bigger, of worrying about everything I ate, and now I have to do the same thing for my daughter. This is not a future I ever wanted for her, but it is a future we will face together.

I look at other babies around her age or younger, and I see everything they can do, how they overtake her, and it’s so incredibly hard not to feel jealous, not to feel overwhelmed by grief for a life that she should’ve had. A life without hospital appointments, without fear and worry, a life where her own body wasn’t trying to hinder her. I have to skip past stories on instagram, or videos on tiktok of babies sitting up, and crawling and moving around, because they make 

me so jealous that I can’t see straight. I get so angry that this isn’t our life, that everything will be so much harder for her. Then I look at her, and I let the grief wave over me, I try and let the anger and guilt go, and I look at this beautiful, remarkable little warrior. Her life will be harder, that is true, and we don’t know how much harder yet, but this girl is a fighter, I know that in my bones. She is the smiliest, happiest baby, and she has gotten so strong from the day we came home from the hospital. She tries every day to do what must feel impossible to her. She rolls around, kicks her legs, and is now a firm believer that nap times are for the weak. She is as curious and as sweet as her brother, and just like him, she is completely magical in every way. I wish everyone could see just how phenomenal she is. We might not have imagined this life for her, and it might fill me with more panic than I can ever fully articulate, but everyday I am thankful for her. I am thankful that I get to be her mum, that I get to be the one by her side as she grows, and continues to be exceptional, that I get to protect her. I am thankful that her dad is who he is, that he adores her more every day, and that her brother will be an even more fierce protector than we could ever be. I will remind her that, no matter how tough it gets, or how cruel people can be, she is made of magic and stardust. She was made for us, and she is perfect, and I will tell her that every day of her life. 

If you've gotten all the way down here, congratulations, you deserve a Blue Peter badge, and possibly a large drink. Thank you for taking the time to read my ramblings.

I hope you're all well.



04 April 2020

#3--Surviving a Pandemic when you have some Seriously Bitchin’ Anxiety.

Look, I’ll be honest, this could be the shortest post ever, because honestly, I don’t have a fuckin’ clue. 
I, along with literally everyone else in the world, have been having a hard time with the fact that everything seems to have gone to hell in a very large, rickety basket, that may or may not, have been built by a blind, inept toddler, who only has control over one arm.
It’s all just a bit mad, isn’t it?

Sunshine before the Social Distancing 
 My partner and myself are both classed as Key Workers, so we still have to go to work. Obviously, we’re both incredibly lucky to still a) have a job, and b) be well enough to get to work, but it’s still very difficult to get out of bed every morning, knowing that you’re putting yourself and your family at risk. As I’ve mentioned EXTENSIVELY before, I have some pretty sweet anxiety (try to not be too jealous), and anxiety in the time of Covid-19--much like love in the time of cholera--is really not the one. Or maybe it is, I never finished the book. Every time I get on the train for work, I worry that this will be the time I’ll get it, or every time he goes to work, I worry he’ll catch it from a customer who doesn’t feel like telling him that they’re quarantining. I worry about my family, his family, my friends, their friends; I am a constant ball of worry and stress. I try my best to keep my panic to a minimum, or at least keep it internal, because I’m worried if I told people how much I actually worry about literally everything, they might actually think I am insane. I mean, I'm even worrying about my worry. Everyday in work we sit and chat about what’s happening, and I can feel the panic rising inside, but I have to pretend that I’m not completely freaking out, so I have become the champion of Changing The Subject. 

‘Cold today, isn’t it?’ ‘Aye, a drink would be lovely.’ ‘Isn’t starting a new job in a the middle of a pandemic a terrible idea?’ Any number of inane subject changes, all to avoid talking about our spiralling into what I imagine an apocalypse feels like. It’s like 28 Days Later out there, except everyone’s fighting over toilet roll and battering old people out of the grocery aisles, rather than worrying about zombies.

With that all said, here are my top tips for not Completely Freaking the Fuck Out, I hope they help you, and if you have any other ideas, let me know! I am always open to more ways of not just being a ball of panic all the time. 

Find a place that makes you feel relaxed
1)  Change the subject! I am very open about this; I will just stop talking about something if it makes me uncomfortable. My go-to technique is to slowly stop replying, and then completely change the subject. I am not subtle about it, but I also have no shame in it. Don’t talk about things that make you feel shitty, or stressed if you don’t need to. 

2)  Avoid the news and social media! Keep up with developments when you can, or when you need to, but don’t submerse yourself in every piece of news that comes out, because you will drive yourself mad with worry. The media exists to inform, but also to sensationalise because they want you to keep clicking on their links, keep buying their papers, keep talking about their stories. Please remember that the media works as a business; not everything they say is with your best interests at heart, it’s with theirs. The more dramatic the headline, the more they sell. Take everything with a pinch of salt, and try not to just read every terrible piece of news emerging. Read what you need to be informed, and then click away. I’d take the same approach with social media, even though I am more than guilty of wasting hours on it. Facebook is littered with news that has never met a fact checker, Twitter is full of angry people and conspiracy theories, and Instagram is bursting with people who want to convince you their lives are better than yours. Get your fix, and then move on. 

3)  Find something Positive! Find something that makes you happy, something that makes you smile and forget what’s happening for a little while. I mentioned in my last post, I have started writing two good things that happen everyday. It’s not always easy, but it does force me to realise that not everything is terrible. So try finding something everyday that brings you a bit of joy, something that helps you see that not everything is awful, because if we only focus on the shitshow around us, we’d never want to leave the house anyway. With that being said, don’t feel like you have to be positive all the time to keep morale up, just keep on top of your feelings; if it all feels like it’s getting too much, talk to someone. You’re not under any obligation to do anything, other than take care of yourself. 

4)  Don’t put Pressure on Yourself! I’ve seen so many people setting mad goals for them to achieve during their self-isolation. Look, if you want to learn a new language, write the new War and Peace, or cure cancer, more power to you, but equally, if all you want to do is survive, then go on with your bad self. I’ve seen so many parents convincing themselves that they must educate their kids to the same level that their teachers would, and I mean what I am about to say in the nicest sense possible. Teachers are people who have trained how to teach your kid shit, stop trying to come up with a better curriculum than the trained professionals. Just do your best, make sure they don’t become illiterate, and enjoy an extra glass of gin to celebrate surviving spending another 24 hours enclosed with hyper children who are desperately trying to escape. All you can do during times like these, is look after yourself and the ones closest to you. 

5)  Keep Yourself Distracted! Listen to music, read a book, watch TikTok for 16 hours, find a new show to watch, find 10 new shows to watch, enjoy spending time with your family. I know that last part might be difficult when you’re stuck in isolation, and your kid has asked you for the 300thtime if they can have another biscuit, or if they can they go to the park, and you’ve watched your 75thepisode of Hey Duggee, but honestly, I’d love to be home with my kid, even if he is annoying the shit out of me, because at least we’d be safe. Just try and find something that makes it easier to see something good in each day. Maybe you get to watch a really sweet episode of Hey Duggee, or maybe that glass of wine at the end of the day tastes extra good. I’m not suggesting you take up alcoholism, but if a glass of wine helps get you through the days, you do you hun. Spend time in your garden, or go for a walk somewhere quiet. You are allowed to get fresh air, just don’t be a dick and go with 30 of your closest pals.  Break your day up with different activities, and hopefully it’ll help the time pass quicker.
A ray of sunshine.
I know these all seem very vapid, and when I’m feeling particularly anxious, the existentialism hits me hard, and I wonder what the point even is in any of it, because in the middle of all this; of life seeming to have ground to a stop for most of the world, of people dying, of people losing their jobs, their financial stability, it’s hard to see a point, it’s hard to find the hope. That’s the thing though; if we allow ourselves to just wallow in that despair, we will never come out the other side. To find the other side, we need to find a reason to look for it, and the only thing that gives reason to something like this, is hope. So even if these tips for helping you to Calm the Fuck Down, seem wildly obvious, hopefully seeing them written down will help keep them in your mind when it all feels like it’s getting too much. 

Keep safe, 


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