Managing acute chest syndrome and sickle cell disease

Last week, every deep breath brought excruciating chest pain that felt as though my ribs were crashing into one another. The symptoms reminded me of acute chest syndrome, a serious sickle cell disease (SCD) complication I have rarely discussed on my platform. As I recovered, I found myself thinking about my childhood, when respiratory complications frequently interrupted my life, and how decades of living with a chronic condition can make familiar symptoms seem less alarming than they really are.

Acute chest syndrome is a medical emergency that can present with chest pain, difficulty breathing, fever, cough, or some combination of these symptoms. It can also show a characteristic infiltration on a chest X-ray and is better managed in the hospital.

My first experience with acute chest syndrome was as a child, and it happened to me frequently. Every time rain fell or the weather became cold, I developed chest pain and difficulty breathing. Sometimes I also had a fever or a cough, although most of my episodes happened without a cough.

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While acute chest syndrome closely resembles chest infections like pneumonia, their underlying causes can differ. Pneumonia stems primarily from infectious organisms like bacteria or viruses. Conversely, acute chest syndrome can result from an infection, blood vessel blockage caused by sickled red cells, or both.

These symptoms occurred frequently in cold weather or when I drank cold water. Exposure to cold can cause vasoconstriction, the sudden narrowing of blood vessels. This worsened the vascular occlusion already caused by sickled cells, promptly triggering a pain crisis or acute chest syndrome.

Fortunately, my childhood episodes never progressed to severe hypoxia or acute anemia, allowing me to recover within a few hours or days.

As I grew older and entered my teenage years, the acute chest syndrome episodes completely stopped. My body no longer reacted to cold drinks, rain, or mild chills, and I could finally tolerate electric fans. Although extreme cold and harsh air conditioning could still trigger general pain crises, my severe lung complications seemed to disappear in adulthood.

That long period of relief ended in my mid-30s when I contracted a severe COVID-19 infection. It was also complicated by pleural effusion, and I had low oxygen saturation that required oxygen therapy for a while. I spent a month in the hospital before I recovered.

A concerning shift

Over the past two years, however, I have noticed a concerning shift. The acute chest syndrome symptoms that disappeared during my youth have gradually returned whenever I face severe cold or infections. I experienced it once or twice last year, and I just recovered from another episode this year.

The associated chest pain is brutal. The best description I can give is that it feels like my ribs are crashing into one another whenever I breathe deeply. For me, that chest pain, along with difficulty breathing, is always the most challenging part of the experience.

To people living with sickle cell disease, acute chest syndrome is a life-threatening emergency that requires urgent medical attention. If you experience concerning symptoms, go to the hospital and follow your sickle cell care plan.

Staying up to date with recommended vaccinations can also help reduce the risk of some respiratory complications associated with SCD.

Hospital management depends entirely on the severity of the illness. Over the years, my treatment plans have included intravenous hydration, oral or IV antibiotics, targeted pain management, and oxygen therapy. Some people with severe complications may also need a blood transfusion or an exchange transfusion to restore healthy blood flow and protect organ function.

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 pertaining to sickle cell disease.