I wake up with an active volcano for a chest. When I breathe in, even lightly, I hear wheezing for miles. I don’t want to call the doctor but I call the doctor. Fifteen minutes later a receptionist leaves me a voicemail: If I can get to the surgery within the hour, I’ll be seen. I’m not sure how I make it down the stairs – and into the car and through the 20-minute journey and out of the car and across the courtyard and into the reception and down the corridor to knock on the doctor’s door – now that my limbs are made of lead. I collapse into a chair, sit feebly and at a nonsensical angle. This chair is too hard for patients, I think. Are the chairs always this hard?
The doctor takes my stats – pulse, blood pressure, and blood oxygen levels which are low at 82% (they should be, like yours, between 95% and 100%) – and apologises as she picks up her phone to call an ambulance. Two male paramedics just appear, chipper in starched, green uniforms. One lifts me into a wheelchair; the other pushes me the long way round the surgery, through the back door, and into the waiting ambulance. All the sick people in reception stare.
I have sickle cell anaemia and am in crisis. Sickle cell is a blood disorder caused by abnormal haemoglobin where red blood cells take on an irregular sickle shape, diminishing the level of oxygen in the body. The term "crisis" is used to describe the happening in a sickle cell sufferer’s body when the faulty cells are doing their worst. The most pronounced pain comes from the unstreamlined cells bumping and scraping along my vessel walls, producing an unbearable, rusty ache in my bones. Especially at the joints which sickle cells have trouble squeezing through, and so often they logjam and cause swelling. I’m at my worst, all puffy, stiff, and defeated.
What causes a crisis can be as individual as the sufferer. Last year a family friend was admitted to hospital 11 times; every cold, cough, and bit of tiredness upset her blood. By contrast, my triggers have been wide-ranging and infrequent: pneumonia, flu, white wine, holiday dehydration, stress, and pipe smoking. This time I am fizzy with a new mum’s lack of energy, run down from seven months of breastfeeding, undereating, and interrupted sleep. After a routine X-ray my consultant informs me I also have a chest infection.
For the longest time I never spoke about sickle cell and the bad blood that moves inside me. Discussing it meant acknowledging it plainly and somehow jinxing myself into more suffering. Even now as I write I hold my breath, worry, am wary of all my body’s clicks and clacks.
I’ve dealt with sickle cell in isolation because that’s what made me feel most normal, which is to say healthiest. When you are sick – when you have a sickness – you do whatever you can to seem well, even cheat your way to it. There are rare need-to-know bases on which I’ve shared my body woes. Once, on a midnight walk up the steepest hill with a boy who had the longest eyelashes I’d ever daydreamed about, I dropped to my knees; the air was too thin. I thought better to seem to this boy – whose calves I’d memorised and wrists I wanted to lick – unwell rather than unfit. On the advice of doctors I have confided in cabin crew on long-haul flights. And there is something about that hushed confessional at the front of the plane that's felt like an unburdening. But maybe that’s because it's led to free soft drinks, extra pillows, and attention.
I do have a tell, though. My mother and brothers and now my husband can read my eyes like tea leaves for signs of the jaundice that can mean an impending crisis. Strangers with medical knowledge have stared me down on the tube, stopped me in the street, and asked me about my eyes temporarily flashing yellow, if I know I might be very sick?
Some of the secrecy comes from the fact that sickle cell is an inherited disease predominantly suffered by black people. Prevalent among people with ancestry in malaria-stricken areas, it is thought the mutation in the haemoglobin occurred as evolved resistance to malaria. When both parents have the carrier gene there is a 1 in 4 chance that their children will have sickle cell. I am one of three, and the only one with sickle cell. My older brother is a carrier; my younger brother, true to the charmed nature of youngest siblings, got nothing. My 19-month-old daughter is a carrier, as any child I birth would be. In the first few years of their life, carriers are naturally protected against mosquito-borne infectious diseases, though they are still encouraged to take precautions as though they weren’t.
It is our disease, so it is our problem. I’ve only ever seen it discussed in avowedly black spaces – church, home, with family, on African news bulletins, in the pages of The Voice. And as black people, don’t we have enough trouble already finding acceptance without drawing attention to a disease that blights only us? And for me specifically, so often the only black girl in the room, suffering from anything first of all removes me from the perfection I’ve always thought I needed to attain to justify my presence. But also it pins me to a specific blackness without negotiation. This is a problem because I, like many first-generation black Brits, want to define what it means to be black for myself, not forced into an understanding by blood I can’t control.
After mentioning to my mum that I was finally going to write about sickle cell she sent me an email. She wanted to make sure I got her side of the story straight, to impress upon me the initial surreality of discovering my condition, of finding out (in England, of all places; she is from Gabon) that she had always carried the potential to create a disease. The email was 600 words long and for every question it answered it made me want to ask 10 more. So I call her.
Mum: When you were diagnosed it was a death sentence.
Sara: They told you I wouldn’t live long?
M: Twenty-one. They said you had 21 years in you, which I rejected.
S: That’s so scary to me now that I am much older than 21, like I’m on borrowed time.
M: I only had hyperemesis gravidarum [severe nausea in pregnancy, which can include dehydration, low blood pressure, and weight loss] when I was pregnant with you and I was so nervous. What was happening to my body and my baby? They tested me for all sorts and told me I was a carrier for sickle cell – they called it “trait” – and said it was a good thing because it meant resistance to malaria. They were sure you’d be born a carrier too.
S: But they didn’t test me when I was born? [They test as a matter of routine now.]
M: Not in the '80s. We found out you had sickle cell when you were 3. We took you to the doctor and he thought it might be asthma or a chest infection and he told us to go to the hospital. You were crying all the time and so weak. And eventually they ran blood tests and they came to me with sickle cell, full-blown, disease, anaemia.
S: And did you have any experience of it before then?
M: I knew the malaria side of things, I think because our family is full of carriers. I heard of people getting sick and swollen bones and all that but I didn’t know anyone specifically and I was so young. I didn’t know doctors or hospitals, especially not English hospitals. I couldn’t name any disease.
S: What was the worst thing about raising a child with sickle cell?
M: The trips to the hospital. The uncertainty. I had two other children who I didn’t want hanging around a children’s ward all day because it’s not the happiest or healthiest place to be. And no one to help me take care of them when you suddenly got ill – and it was always sudden. And there was no way of knowing how long you’d be sick. Sometimes it was a month. Sometimes a week. You’d bounce back or not. It’s hard to see your child in pain. It’s the wrong way round – as a parent you want to take that pain.
S: When [my daughter] has so much as a cold, I panic. I can’t imagine the stress of having a diseased child.
M: But you’re not a diseased child! I didn’t think of you as diseased, not now or then or ever. You got sick, sometimes very sick, but you always got better. And now I don’t worry because I know you can take care of yourself.
S: Most of the time…
M: Well, you still don’t drink enough water.
To think of your body as diseased is to look at death with unblinking eyes. To consider the mundanities of your funeral, the oak of your coffin, the chrysanthemums in your wreath, the taste of soil at the back of the attendees’ throats as you're lowered forever into the ground. It is to ponder out loud what humans endlessly distract themselves from thinking. We are not invincible.
I go on spontaneous splurges for positive information. In my lifetime the estimated mean survival age for female sickle-cell sufferers has moved from 47 to 58. I’ve heard of the rejuvenating powers of hydroxyurea. Bone marrow transplants have cured children. Gene therapy has successfully treated sickle cell in mice. The oldest person currently living with the disease is 86.
When I decided I was ready to bring another human being kicking, screaming, and sighing into our messy world I did an uncharacteristically responsible thing and arranged a bunch of health tests. I wanted to get checked out top-to-toe to make sure I could conceive, carry, and deliver. After my last test, an echocardiogram in a new-build hospital where I had a clear view of Warren Street, the doctor explained that my heart has a slightly leaky valve, an unalarming occurrence in sickle cell sufferers. My response must have been to look petrified because he went on to make heavy work of reassuring me. “Everyone’s body is imperfect in some way,” he said. “Anyone can be sick if they have enough tests, especially as many as you’ve had. At least you know how you are sick.” I was not comforted.
Once I’m processed through A&E I’m put on a temporary ward between two older ladies bickering about teabags. After a couple of hours a bed in a private room is found on the oncology/haematology ward. A nurse visits to explain that two pints of blood have been ordered to go through me over the course of the next 24 hours. She puts a mask of formality on her otherwise congenial face, has me identify myself and confirm my hospital number. A bag of O is then hooked up to begin its journey into my body.
When I was transfused as a child I imagined the people who gave me blood, invented them. A tall man who liked oranges. A ballet dancer. A businesswoman who looked like Melanie Griffith in Working Girl and spoke softly just like her but also really fast. They were always taller or louder or faster, somehow more alive than me. And when I got home from the hospital I would ham up these new qualities, exaggeratedly act out the ways in which their good blood had transformed me.
This blood is cold. It’s been in the blood bank for up to 42 days, it's chilly where it enters my body, chilly in the vessel up my arm, soothing and unsettling at the same time. It’s purple-red, a claret; I can’t be the first person to have noticed it looks like wine. This is the only decent part of being sick, the feeling of getting drastically better. The mechanics of repair. I lie supine on a squeaky hospital bed, itchy in borrowed pyjamas, willing the blood to travel through me faster, to fix me. And it feels like such a miracle when it begins to. From my toes up to my knees first, and then higher. I’m in and out of sleep. Hearts beat in my joints. Eventually my brain ticks clear.