Imagine that a water pipe in your house is blocked by something. Then the water does not flow properly and gets stuck in one place, causing a big problem. That is what happens inside our bodies during a "Sickle Cell Crisis". This is a very painful experience. So let's talk about this clearly today.
What is this sickle cell disease ( SCD ) and the 'crisis'?
Simply put, our blood contains a type of cell called
red blood cells . In a healthy person, these cells are round and flexible. They are like little balls. This allows them to move through the very small blood vessels in the body without any obstruction and carry oxygen to all parts of the body. However, in a person with
Sickle Cell Disease (SCD) , some of the red blood cells are shaped differently. They are not round, but rather
curved into a C-shape . Also, these cells are very stiff and sticky. So, when these sickle-shaped cells move through those small blood vessels, they are more likely to get stuck in one place.
Just like the water in the pipe mentioned earlier gets stuck, these cells also join together and completely stop the flow of blood through the blood vessels.
When the blood flow stops in this way, the parts of the body that are nourished by that channel do not receive the necessary oxygen. This is called
(Hypoxia) in medical science. This lack of oxygen causes unbearable, very intense pain. That is what we call
"Sickle Cell Crisis" . This pain can last from a few hours to a few weeks. Especially due to the blockage of small blood vessels in the chest, a serious condition called
(Acute Chest Syndrome) can occur. This affects more than 50% of sickle cell patients.
What are the symptoms of a 'sickle cell crisis'?
In this situation, each person may show different symptoms. But there are some common symptoms. Let's look at them in a table.
| Symptom | A description about it |
|---|
| Unbearable pain | This is the main and most common symptom. The pain may come on suddenly and may be dull, stabbing, squeezing, or sharp. It is most commonly felt in the arms and legs, abdomen, chest, and lower back. In young children, the pain may be more intense in the palms of the hands and soles of the feet. |
| Difficulty breathing | Shortness of breath, accompanied by chest pain, may occur. This is a more serious symptom. |
| Extreme tiredness/fatigue | You may feel lifeless and extremely tired. |
| Yellowing of the skin (Jaundice) | The whites of the eyes and skin may turn yellow. |
| Other features | Dizziness, headache, difficulty moving some parts of the body, and painful erections in men may occur. |
Are there any specific reasons for this 'crisis'?
It is often difficult to pinpoint the exact cause of this condition, but several common 'triggers' have been identified that can contribute to it.
- Dehydration : Not drinking enough water.
- Temperature changes: Going to an environment with a sudden change in temperature . For example, going from a well-air-conditioned room to suddenly going outside into the hot sun.
- Infections: Even a minor infection like a cold can be a cause of this.
- Stress: Excessive stress is also a major cause.
- High altitudes: Going to very high places, such as mountain peaks, where there is less oxygen.
What should you do when a 'crisis' occurs? What are the treatments?
If you have this condition,
The most important thing is to talk to your doctor and come up with a treatment plan to manage a 'crisis' if it occurs. Things you can do at home
As soon as the pain starts, there are several things you can do at home.- Drink plenty of water: It is very important to keep your body hydrated.
- Painkillers: You can take a common painkiller like Paracetamol or Ibuprofen . But ask your doctor which medicine is best for you. For example, if you have kidney problems, Paracetamol may be more suitable.
- Other methods: Applying a hot water bottle, taking a hot bath, and gentle massage can help relieve pain.
- Mental health: Calm your mind through methods like meditation. Seek support from family and friends.
Medical treatment
There are several specific medications that doctors recommend to prevent and manage this 'crisis'.- Hydroxyurea : This medication reduces the formation of sickle-shaped red blood cells, which reduces the frequency of painful crises.
- L-glutamine oral powder (Endari): This also helps prevent 'crisis' and reduce the need for hospitalization.
- Crizanlizumab-tmca (Adakveo): This drug stops blood cells from sticking together and blocking blood vessels.
- Important information: A drug called Voxelotor (Oxbryta) was previously used for this purpose. However, due to safety concerns, it was removed from the market in September 2024. If you are currently taking this drug, it is essential to discuss it with your doctor immediately and switch to another appropriate treatment.
If home treatments do not control the pain, go to the emergency room (ETU) of a hospital without delay. There you will be given stronger painkillers. You may need to stay in the hospital for a few days until the pain is controlled.
Can a 'crisis' be prevented?
While it's impossible to stop it completely, there are many things you can do to reduce the risk of a 'crisis' . Lifestyle changes to make:
- Protect yourself from the cold:Avoid bathing or swimming in cold water. Wear warm clothes on cold days and when going to places with AC.
- Drink plenty of water: Drink plenty of water throughout the day.
- Limit alcohol: Reduce alcohol consumption as much as possible.
- Air travel: If traveling, only fly on commercial aircraft with a pressurized cabin.
- Manage stress: Find ways to keep your mind relaxed.
To stay healthy in general,
- Stay away from illness: Stay away from people who are sick as much as possible. Wash your hands often.
- Avoid smoking.
- Exercise: Light exercise is good, but avoid exercises that are too strenuous (like intense weight training). Drink plenty of water while exercising.
- Control other diseases: If you have other diseases, such as diabetes, control them well according to your doctor's instructions.
- Get vaccinated: Get all required vaccines on time.
- Pregnancy: If you are pregnant or planning to become pregnant, seek medical advice immediately.
Take-Home Message
- Sickle cell crisis is a very painful condition caused by sickle-shaped red blood cells blocking small blood vessels, leading to oxygen deprivation.
- The main symptoms are sudden onset of severe pain, difficulty breathing, and extreme fatigue.
- This condition can be caused by things like dehydration, infection, stress, and temperature changes.
- Always follow your doctor's treatment plan. If the pain cannot be controlled at home, go to a hospital's Emergency Department (ETU) immediately.
- The risk of a 'crisis' can be reduced by regularly drinking plenty of water, protecting yourself from the cold, and maintaining a healthy lifestyle.
Sickle Cell Crisis, Sickle Cell Disease, Red Blood Cells, Severe Pain, Blockage of Blood Vessels
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