The main character of this interview is 31 years old; she is a jazz singer and vocal teacher.
Atea is a patient who tested positive for an anaemia blood test. She regularly had CBC test also known as a Complete Blood exam and was therefore treated for sickle cell disease during childhood and adolescence at the Hospital Sant Joan de Déu in Barcelona. Interview with Athena, a patient with sickle cell anaemia (drepanocytosis), who was examined and treated during childhood and adolescence at the Hospital Sant de Déu Barcelona.
When Athena was a child, she developed a high fever. Her mother took her to the Sant Déu Hospital in Barcelona, where, after numerous tests, the girl was diagnosed with sickle cell anaemia, drepanocytosis, a rare haematological disease. This pathology, also called drepanocytosis or sickle cell anaemia, is one of the world’s most common genetic blood diseases and affects red blood cells, which are round and designed to transport oxygen.
In people with sickle cell disease, some of these cells are sickle-shaped and become “stuck” in the blood vessels because they become stiff, impeding blood flow. When this occurs, taking a CBC Complete Blood Count test is important. It is called a vaso-occlusive crisis or hemolysis. These crises cause pain and can damage a person’s organs and tissues. Athenea, as a survivor of the disease, spoke about her experience at a meeting with pediatric patients and their families, and we took the opportunity to interview her.
What is it like meeting children who have anaemia as adults?
It’s very touching. I hugged Susanna Reeves, my doctor, and meeting other children and families was helpful because, often, these patients feel alone. For me, the contradiction between pediatric care. Anaemia testing combined with the transition to an adult hospital was that, in many cases, there is not enough knowledge about the consequences. Any risks of a crisis are, so you have to insist on treatment and explain the characteristics of the disease to a specialist yourself (even at the moment of crisis and in severe pain). People with this disease usually need to see a haematologist in adulthood and be warned about the risks of improper treatment.
How are you connected to the haematology team at Hospital Sant Joan de Déu?
I was diagnosed with an anaemia test kit here when I was two years old. From then until I was twenty, I was treated in this hospital. My mother says that many years ago, Dr Estella (then the head of the haematology department) gave her his phone number so that we would have someone to advise on relieving pain and crises when they arose. When I was about eight years old, the hospital medical team developed a protocol for caring for patients who had anaemia blood Tests, often also with sickle cell disease, in the emergency department that included pain management and immediate action.
Anaemia Testing of blood is not yet as widespread in adult hospitals. Over time, I concluded that it was treated equally to other pathologies. One of the specialists from Sant Joan told in training for patients that this is a “time-dependent” disease. They are different since hemolysis or vaso-occlusive crisis is similar to a thrombus.
The blood does not clot, but the red blood cells change form and accumulate. The effect is similar, and time cannot be allowed to pass. If this happens, the crisis can sometimes have serious consequences, such as affecting the bones and causing necrosis, or if it affects the cardiovascular system, it can lead to a stroke. We must act quickly, and spreading knowledge about the disease is key.
Does This sound alarming?
Because that’s how it is, a lot of work is left, and some associations are already working on it. Not all hospitals work like this one. Often, in the emergency room, in triage, until the haematologist shows up, no one knows what’s going on with you, and there isn’t (in adult hospitals) enough knowledge or protocols. I was often prescribed treatment that I did not need for my pathology. All this adds to the feeling of misunderstanding. It is a non-native disease that particularly affects groups of African descent and, in particular, many migrant families.
In these cases, people face not only language and bureaucratic barriers but also difficulties in a healthcare system that does not know enough about sickle cell disease despite it being one of the most common genetic diseases. I think there is a racial bias that shouldn’t exist, but unfortunately, it extends into the healthcare system, and I feel it every time I go for a CBC Test. I have always had to explain to my family and friends how to act in an emergency.
What should we do if such a situation arises?
These are painful crises, so first, you need to remain calm and ask the person affected what they need. In the case of temperature contrasts, there is a certain risk, and if it is very hot, we need to cool off in any shade, in an air-conditioned supermarket or elsewhere. We should not be thrown into the water suddenly or act abruptly. There is also a risk of crises due to exhaustion, anxiety or pressure changes. Suppose a person suffering from hemolysis complains of pain, which, as I said, is severe.
Then, you should ask him if he is taking medications to relieve it (morphic). If you don’t have these, it’s advisable to call 112 and explain what’s happening to the person so they can tell us what to do: whether to go to A&E, call an ambulance urgently, or how to act as quickly as possible. Suppose the person accompanying the patient in crisis is informed they have a positive anaemia Test result. In that case, they will better explain what is happening to the emergency team. I went through a period of crisis.
The people who helped me the most with their knowledge were my mother and my partner. As I said, you often have to insist that you be treated quickly in the emergency room for anaemia. This is a common experience of people who attend sessions at the association if they can afford to go private for their CBC Tests. Those who have experienced pain and crisis and seen white coats pass them by in the waiting room. However, time is against such patients, who need to be helped quickly.
Were your experiences different as a child and teenager than as an adult?
Unfortunately, yes. In paediatrics, it is usually treated adequately. Still, as people get older, the situation becomes more hostile. There is a need to raise awareness about the existence of this disease. It is often confused with iron deficiency anaemia. Every person with anaemia or sickle cell disease must know what to do, how to tell others about it, and how to help prevent crises. When you have a job, it’s frustrating when you have to leave because you’re in pain and feel like your coworkers don’t understand what’s going on with you until they see you with morphine.
We must continue raising anaemia blood test awareness about the benefits of blood donation for treating such diseases. We must also speed up emergency care for adults. And prioritise those with sickle cell disease. And encourage families to come together and get to know each other to make progress. For my part, I am trying to raise awareness of my initiative, but this is just a grain of sand to which many other actions need to be added.
What else is needed besides improving knowledge about sickle cell disease?
There are quite a lot of issues to discuss. The above should be added attention to the consequences. These can be more or less serious depending on the combination of sickle cell anaemia (from one or both parents). Some people who have had an anaemia test suffer pain during attacks. And damage to certain areas of the body.
In some cases, the disease also leads to reproductive problems. In the case of sickle cell disease, research is key, as is providing patients with tools from childhood. Taking care of yourself with regular monitoring with a CBC Count Test is extremely important. And you know how to recognise it in a serious crisis. Hydroxyurea is a drug that helps reduce the production of red blood cells. They become deformed from disease, so preventing a crisis is best.
As Susanna Rives once very well explained to me, the fewer red blood cells that are prone to deformation, the less not only are crises. But also, there is less need for blood transfusions. Finally, in our case, there is little talk about mental health. Being a teenager or young adult, Anaemia tests are common. This condition is difficult to find unless an anaemia blood Test is done.
You want to keep up because you feel like you are the problem. And there is social pressure to integrate into the group. If you don’t give yourself the tools, it’s easy to get hurt physically and psychologically. Trying to keep up with other people ultimately leads to illness. This is where raising awareness would help a lot: you don’t have to explain your illness constantly. If you suffer from pain similar to pain from a broken bone, don’t fight it.