I agonized over this post before I hit the publish button. I talk about a lot of things, but I rarely talk about the risks that I take on a regular basis. If you’re easily shocked, know me or don’t like the gory details, stop reading now. However, I can’t gloss over the really hard days. The snatches of life I try to capture here are varied, skewed and incomplete, but very real. Bleeding is part of that reality.
“There’s blood, a taste I remember. It tastes of orange popsicles, penny gumballs, red licorice, gnawed hair, dirty ice.”
— Margaret Atwood (Cat’s Eye)
EDS is a motley medley of heritable connective tissue diseases that all have one thing in common – the genetic defect affects collagen synthesis. Although there are multiple different types of EDS, it’s common to have a bit of a hybrid form with characteristics of more than one type. The genetic quirk that runs in my family has features of hypermobility, classical and vascular EDS. I have been diagnosed with EDS, hypermobility type with overlapping classical and vascular features. I try not to dwell on the latter.
It does mean that I am used to bleeding. My skin is thin and fragile and have a tendency to split open with little or no encouragement. The layer of tissue underneath the skin isn’t much stronger and cuts easily penetrate deeply whilst bruising can be rather spectacular from the smallest bump. Capillary arteries have a tendency to rupture. It’s odd when you can use your finger to rearrange the blood vessels underneath your skin but it’s something entirely different when one of them ruptures. I really need to carry a camera within easier reach. Nobody ever takes pictures and someone should. Relocations of dislocated joints go wrong about once a month and blood pools underneath my skin, it’s not pretty either. But it’s the things that nobody talks about that are a little more serious and deserve a mention.
It’s worrisome that I know how to distinguish whether I’m vomiting blood, coughing up blood or having a bad nosebleed. It sounds like an easy task, but it isn’t. It may be any or all of the above. Usually, if it’s your lungs, the blood is pink and frothy, almost milkshake like or bright red. It’s unmistakeable. If you’re vomiting blood form intestinal bleeding, it’s usually much darker or has the texture of coffee grains. Nosebleeds, even posterior ones, are pretty obvious in source. The problem comes when all three collides.
I have not had a good day. I woke up with an asthma attack, still coughing up blood, but not enough to make me worry. Again, I would live at my doctor’s surgery if I made an appointment for minor stuff like this. I’ve had all the tests and examinations, it’s a lifelong condition, I know when it’s just my collagen tearing under additional strain and when it’s something more. It’s not something more, it’s nothing treatable, it’s just part of life as I know it. Coughing for an hour did have the very negative effect of rupturing a blood vessel in the back of my nose.
I get posterior nosebleeds and have gotten them since childhood. They can be freaky, they can get very scary, but most of the time, they follow a predictable course and everything is fine. With allergy season starting, a mid-april nosebleed is right on scedule and expected. The thunderstorm brewing at five a.m. didn’t help much and I knew what was coming before it happened. I was prepared. Or I thought I was. I was perched on a little stool with my head leaning over the sink coughing my lungs out when the bleeding started. There’s something eerie about blood gushing out both nostrils and into your mouth when you lean forward. There is a short moment of panic when you realise that it’s a posterior nosebleed, obviously a serious one and some of it you’re going to have to swallow. Please don’t choke.
I’ve been down this road before. I vividly recall the emergency room trip nightmares where stupid doctors insist that applying pressure is the way to go. Lets inflate that little balloon in the back of your nose, oh wait, hang on, that makes it bleed more as blood vessel walls burst under the additional pressure. Oops. I’ve been there when the last resort was to cauterize the bleeding vessels without anaesthetic as locals don’t work on me at all. I also vividly recall the storming out of the hospital with a still bleeding nose after refusing the emergency surgery I really should have. I sat in my car for an hour bleeding onto the rubber floor mat and went back in when I knew surgery would no longer be required. Surgery with my connective tissue is a bad idea unless it’s absolutely vital. I didn’t think it was and I trust my judgment.
I don’t recommend following my example . In fact, I am fully aware that I am playing a very dangerous game when I take my health into my own hands, particularly when it starts to hang on the thread of steadily lowering blood pressure I am both stupid and arrogant enough to keep doing it anyway. I make my medical decisions and I will decide when medical intervention will help more than hurt. It was with this thought in hand that I was prepared for my first spring nosebleed. I called Chris just before/as the bleeding starts, just in case. When the gushing starts, I put the plug in the sink. These are the facts:
- A class I Haemorrhage involves blood loss of about 15% of blood volume. When you donate blood, they take about 10%. This type of loss generally produces no symptoms, no change in vital signs, capillary refill is <2 seconds and it require no intervention. Your body will replace the lost fluid pretty quickly. For me, 15% is about 750ml approximately. In someone with low BP and mild hypovolemia, symptoms start here. My pulse top 110 before we reach 10%, my BP drops a little and I start to feel dizzy. It’s one of the reasons I’m not allowed to donate blood.
- A class II Haemorrhage involves blood loss of 15-30% (750ml – 1.5litres in my case). At this point, pulse may go up, BP is still normal, capillary refill is delayed, the body compensates through peripheral vasoconstriction and the skin start to look pale or mottled and feel cool to the touch. In someone with Raynaud’s, like me, the blue-white hands and feet tend to give it away. At this point, irritability, anxiety and a little aggression may start to set in.
- A class III Haemorrhage involves blood loss of 30 – 40 % of blood volume (For me: 1.5 – 2 litres). At this point, BP starts to drop, heart rate increases, capillary refill is prolonged, skin is significantly mottled, pale and cold, breathing starts to be shallow and fast, pulse becomes thready, pain response is diminished and mental state is irritable lethargy. Somewhere along this continuum, shock sets in. At that point, a transfusion is required.
- A class IV Haemorrhage involves blood loss of >40% of blood volume. It’s a life-threatening medical emergency. BP crashes, pulse is thready and rapid, significant acidosis set in, extremities are cold and pale with a blue tinge and lethargy progresses to coma.
The downstairs sink holds 3.5 litres. I’ve measured it shortly after we moved in. It’s important information. It means that the sink cannot be more than 50% full. The 50% mark is the ‘call an ambulance now’ mark. I also carefully placed the phone next to the sink just in case. The shocking bit here – I prefer being downstairs for a couple of reasons. The first is that I am a few metres away from the front door. The second is that there’s a beige carpet on the floor in the downstairs bathroom and I know that I will go to great lengths not to faint and splatter it with blood.
The bleeding starts and I can’t make it stop. I am aware that quite a lot of the blood is dribbling down the back of my throat and revise my 50% rule. If I’m swallowing about 30%, a litre or so is the line drawn in the sand. I spend quite some time bleeding into the sink, hoping that my platelets would clot. Clot! Stupid stretchy skin stop gaping. I pass out on the cream carpet. Do not tell Chris this. When the bleeding finally slows down, the nausea really sets in and the throwing up begins. Not out of the woods just yet. Patchy digestive tracts don’t like vomiting and it doesn’t take long for the intestinal bleeding to rear its ugly head. Fingers crossed that it’s just routine, to be expected, nothing seriously serious. One wish granted me thinks. My body doesn’t like coughing and vomiting. Enter splitting headache, ringing in the ears, slight deafness and watercolour painting vision. The myriad trips from bathroom to couch and back again wreak havoc on joints. Wrists are in and out, knees, fingers, shoulder, ankle. I feel shell shocked and battered. Obvious solution – I log into wow to attempt fishing despite a dislocated wrist I cannot get back in.
Chris gets home at five and finds me on the couch, still crying. I have been crying on and off all day. Sobbing really. I spit out blood inbetween sobs which fuels the tears. My throat is too raw to allow me to talk without whimpering. Chris puts my joints back in, serves ice-cream and makes me eat it whilst I continue my valiant attempt to fish in Dalaran. Mostly I just find it reassuring to have Lileya sat in her little corner in the sewers even if I can’t fish. I manage to swallow two spoons of ice cream before throwing up. The bleeding starts up again and so does the crying. Chris sits quietly next to me on the couch and strokes my arm, massages my neck, pops my shoulder back in that I didn’t notice was out. It’s going to take weeks before the bleeding really stops and heals and the next little thing that goes wrong is going to require significant medical intervention. It’s a depressing thought, but I try not to dwell on it. I try to find something good to take from today. A snatch of a happy conversation, the feeling of a cat curled up around me and my keyboard on the couch, the sensation of silk again my skin, the taste of chocolate ice-cream. I am back at the simple pleasures and I try desperately to hold on to what I do have:
“Now it’s full night, clear, moonless and filled with stars, which are not eternal as was once thought, which are not where we think they are. If they were sounds, they would be echoes, of something that happened millions of years ago; a word made of numbers. Echoes of light, shining out of the midst of nothing. It’s old light, and there’s not much of it, but it’s enough to see by” — Margaret Atwood (Cat’s Eye)