Taking another look at the onset of vaso-occlusive pain crises

As both a physician and someone living with sickle cell disease (SCD), I spend a great deal of time reading SCD-related medical literature. Recently, while reviewing several publications, both old and recent, on vaso-occlusive crises, I noticed recurring statements that made me pause: Acute sickle cell pain and vaso-occlusive crises are usually described as having a sudden onset.

While sudden onset is undoubtedly a common presentation of acute pain crisis, it is not the only presentation. Adult sickle cell patients in different support groups commonly discuss how their crises sometimes give them cues and warning signs. Based on patient experiences, vaso-occlusive crises can be both sudden and gradual in onset.

Over the years, I’ve learned that my own pain crises don’t all begin the same way, either. Some arrive abruptly, without the slightest warning. Others develop gradually, giving me subtle clues that a crisis is approaching.

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Understanding the diverse nature of sickle cell pain crises

Pain crises triggered by sudden fear or panic, for example, often start without warning for me. The same is true for crises precipitated by exposure to cold temperatures. My menstrual-related pain crises also tend to begin suddenly. Although I know they are likely to occur before or during my menstrual cycle, I never know the exact day or time they will start, and they rarely provide warning signs before the pain becomes severe.

In contrast, stress-induced crises often tell me they are coming. I may develop unusual fatigue, generalized muscle aches, or simply feel that my body is becoming overwhelmed. Those early symptoms prompt me to stop what I’m doing, rest, increase hydration, and follow my hematologist’s pain-management plan. Sometimes those simple measures reduce the severity of the crisis or even prevent it from progressing.

I’ve also noticed that pain crises associated with infections or increased hemolysis (the destruction of red blood cells) often announce themselves differently. Deepening jaundice is frequently my earliest clue that something is wrong, long before severe pain develops. These experiences have convinced me that, for many patients, some vaso-occlusive crises have a warning phase that deserves greater recognition.

This is why I find it difficult to accept the broad statement that vaso-occlusive crises always have a sudden onset. It made me wonder why this description has become so firmly established in medical literature.

Updating the research

One possible explanation lies in the history of SCD research. Many of the landmark studies that shaped our current clinical definitions were conducted decades ago, when life expectancy for people with SCD was much shorter than it is today. At that time, many patients died in early childhood, meaning pediatric populations made up a large proportion of research participants.

Young children, especially infants and toddlers, cannot describe how their pain develops. Even older children may struggle to recognize and communicate subtle warning signs that signal a crisis is approaching. So all that is left is their reactions when the pain hits hard.

Due to these factors, clinicians and researchers often relied on caregiver observations, such as crying, irritability, or refusal to move, which show sudden severe pain. These observations remain extremely valuable. However, while this approach was appropriate given the available population, it may have unintentionally overlooked an important aspect of the patient experience.

Fortunately, advances in medical care and disease-modifying therapies have transformed the outlook for people living with SCD. Today, many patients survive well into adulthood, building careers, raising families, and becoming experts in their own disease.

This presents an opportunity to expand our understanding of vaso-occlusive crises by listening more closely to adults who can describe how their pain begins and progresses. Their experiences should not replace existing clinical knowledge, but they should complement it or correct it if need be.

Patient-reported experiences have become an increasingly important part of modern medicine, and SCD should be no exception. If a significant number of patients consistently describe a gradual onset for some pain crises, those experiences deserve careful study rather than dismissal.

Refining our clinical descriptions is about far more than correcting terminology. Understanding that some vaso-occlusive crises may have an identifiable warning phase could improve patient education, encourage earlier intervention, strengthen communication between patients and healthcare providers, and potentially reduce the severity of some pain episodes.

Sometimes the people living with a disease every day are the ones who help us see what the textbooks or medical literature have yet to fully capture.

Note: Sickle Cell Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Sickle Cell Disease News or its parent company, Bionews, and are intended to spark discussion about issues about sickle cell disease.