Sickle Cell and acute chest syndrome

July 20, 2019

Over the years, I have had a sickle cell condition, which started from some parts of my body and lingered. It has gone to my chest; this is called acute chest syndrome. When one has a crisis, the pain is not only acute but also pretty severe at the best of times. I don’t know how to describe this particular chest pain. But one thing I remember is that my rib cage would hurt so much and in the process, I would find it very difficult to breathe.

Normally, red blood cells are smooth, round and flexible. Sickle cell disease turns some of them into crescent shapes that look like sickles. Because they are unhealthy, the sickle cells die quickly. This leaves behind fewer healthy red blood cells. Sickle cells can block blood flow. One of the problems this can cause is trouble with breathing, which can lead to acute chest syndrome. In patients with sickle cell disease, ACS occurs most frequently in patients with haemoglobin genotype SS less so in those with HbSß0 thalassemia or HbSC.

Acute chest syndrome is a term used to cover conditions characterised by chest pain, cough, fever, hypoxia (low oxygen level) and lung infiltrates. Acute chest syndrome may be the result of the sickling process in the small blood vessels in the lungs causing a pulmonary infarction/emboli or viral or bacterial pneumonia. The severity of ACS events is categorised as mild, moderate to severe; this definition and its severity apply both to children and adults. ACS can be a severe life-threatening condition. Early recognition of progression to acute respiratory failure is vital.

Although there is no one condition that causes ACS, another illness can trigger it. It could be a lung infection, such as pneumonia, fluid overload, opiate narcosis, hypoventilation or pulmonary embolism. Fatal cases generally develop into rapid pulmonary failure and a third of them were associated with bacteraemia. Age has an impact on the clinical picture of ACS. In children, ACS is milder and more likely due to infection; whereas in adults, ACS is severe, associated with pain and has a higher mortality rate.

ACS resembles pneumonia, e.g. the patient has signs and symptoms of lower respiratory tract disease and a new pulmonary infiltrate on chest radiograph. ACS can develop suddenly or insidiously. Patients may present with ACS, or it may occur during hospitalisation for a vaso-occlusive crisis or after a surgical procedure, especially one involving the abdomen. Therefore, vigilance should be maintained.

Individuals with sickle cell anaemia have an increased risk of severe bacterial infection. The risk continues throughout childhood and to a lesser extent in adults. Fever may herald many acute and sometimes life-threatening conditions, such as acute chest syndrome or osteomyelitis.

A key difference in the clinical presentation of ACS between children and adults with SCD is the greater disease severity and a higher mortality rate in adults, largely due to a higher incidence of bone marrow and fat emboli in adults. In adults with SCD, up to 77 per cent of ACS episodes are the result of bone marrow or fat emboli; in children, the proportion is much lower. In adults, this manifests with complaints of shortness of breath, chills, and severe chest pains and breathing problems.

Frequency of presenting symptoms in ACS appears to be age-specific. In young children (two to four years old), fever and cough are typical; pain is rare, and upper lobe disease is more common. Adults tend to present with shortness of breath, chills, severe pain, and no fever; multi-lobe and lower lobe disease are more frequent. Seasonal variation is seen, with more cases reported in the winter.

Acute chest syndrome is a leading cause of death for patients with sickle cell disease. It comes about from fever, respiratory symptoms, pneumonia, or a SCD crisis. ACS in adults with SCD requires prompt management to prevent clinical deterioration and death.

Treatment in the hospital will usually involve simple transfusion administered early; this may halt progressive respiratory deterioration and prevent complications such as increasing tachypnoea and need for supplemental oxygen. If necessary, an exchange transfusion is performed.

As you know, I am not a health worker and as such, I have had to research ACS, in order to write about it in a way that hopefully makes sense to us all. See links below, if you want to know more.

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If you would like to get in touch with me, please do so on: t.dehinde@yahoo.com and do visit my website: www.howtolivewithsicklecell.co.uk.

Sources: ncbi.nlm.gov; uptodate.com; webmd.com; bloodjournal.com; sickkids.ca; medscape.com; ststn.couk and healthline.com.