The reality of life with Sickle Cell
“People don't understand how severe it can become,” the 23-year-old tells This Is Medtech. On the other hand, day-to-day life is just like everyone else’s, for the most part. “When I’m pain free, no-one would ever know I had an underlying condition because I can do what everyone else can do.”
Sickle Cell disease (SCD) is a group of disorders that affects haemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with SCD have atypical haemoglobin, which can distort red blood cells from their usual donut shape into a sickle or crescent shape. The misshapen cells lack flexibility and can block small blood vessels, impairing blood flow.
Poor blood oxygen levels and blood vessel blockages in people with SCD can lead to chronic acute pain syndromes, severe bacterial infections and necrosis (tissue death). Chantelle knows all about these so-called crises. “Since my first crisis a few years ago, I’ve been hospitalised once a month on average,” she says. Once she’s had morphine, she has blood tests that check her haemoglobin and infection levels, and x-rays to see if there is any inflammation around the painful area.
“It would be useful to have small, portable machines that are easily accessible that can detect veins when I am having blood tests or getting a cannula put in. Because my veins have been used so often, it is now harder for nurses, doctors and anaesthetists to find them, so a machine such as this would prevent me from becoming a pin cushion,” Chantelle remarks. “I believe there are similar machines that are quite bulky that exist already but hospital staff seem reluctant to use them for some reason,” she adds.
Chantelle’s main trigger is the cold, but stress can be a factor, too. She feels lucky that she didn’t experience a crisis until she reached adulthood, which she believes is quite uncommon. “I didn’t suffer from crises until I was in my second year at university,” she says. “I can’t remember the details but I had just returned to uni after the Christmas holidays. I had deadlines, one of which was a group project. The group was due to meet but instead I ended up in hospital. I couldn’t help but think the group thought I was faking and didn't want to contribute because at that point, no one at uni knew I had sickle cell,” Chantelle notes.
“I remember being scared because I had never felt that level of pain before and I remember thinking: ‘Is this what a crisis feels like?’ ‒ I was in hospital for six days.” Chantelle describes the crises as variable. Sometimes the pain comes on gradually and gets worse; other times it hits her instantly. “When the pain is gradual, I tend to get tingly sensations to start with where the pain is. I take this as a warning sign that I have limited time to take painkillers before the pain gets excruciating. If those painkillers don’t work however, the pain starts to become unbearable which means I have to go to hospital,” she says.
Moreover, the pain can be anywhere. “I’ve had it in all of my joints (wrists, elbows, knees and ankles), head, shoulders, neck, lower arms, chest, back, hips, thighs, and shins,” she explains. “Crisis pain can differ a lot, for example if it is in my chest, it can sometimes feel as though an elephant is sitting on me, whereas other times it feels as though someone is taking a heavy hammer at me. If the pain is in my arms or legs, they can feel dull and heavy. When this happens, I cannot move that limb and I literally have to carry it with me. For example, I have to hold my painful arm with my good arm as if it were in a sling and if it’s my leg(s) I cannot walk ‒I have to crawl or shuffle.”
Even when she’s feeling fine, Chantelle has to take various tablets daily and she has to make sure she’s dressed for the weather. “On a chilly day, everyone else may be ok wearing just a coat, but I have to ensure I have my hat, scarf and gloves too,” she explains. Carrying painkillers at all times is essential in case a crisis strikes.
It’s not only the physical aspects that people with SCD face. “It affects my work-life balance, because when I have to take time off work because I’m hospital, I always feel guilty about the amount of time I miss, and how my managers really feel about me being off once again,” comments Chantelle. She admits that in the midst of a bad crisis, she can get seriously down.
Raising awareness & dispelling myths
Chantelle believes raising public awareness is of the utmost importance in order to help Sickle Cell sufferers to be more successful in school, at work, in hospital and in life. “Only by spreading the word will we dispel the myths surrounding SCD,” she says. “One is that it only affects black people. This is not true. It can affect people of Asian and Mediterranean descent also and I think that as the world becomes more multicultural, it is possible that white people could also suffer from it. Once people see that it is not just a ‘black thing’, I believe it would be taken more seriously.”
Another myth involves the pain levels. “I’ve read, heard and experienced myself that SC sufferers think people don’t believe the level of pain they’re in,” she points out. “The amount of strong painkillers we have to take can be frowned upon because people sometimes assume we are addicted to them when in fact the reality is, we would rather not be taking drugs such as codeine and morphine or stronger ones at all.”
Chantelle feels especially strongly that kids and teens growing up with SCD should educate themselves and she advises them not to suffer in silence. “Even if your pain is not visible, tell your friends, tell your teachers. Heck, tell everyone if you want.”