Understanding sickle cell disease in clinical practice

Sickle cell disease is one of the most common autosomal recessive genetic diseases associated with production of abnormal haemoglobin. The main clinical features are haemolytic anaemia and vascular occlusion. Chekroun et al., 2019 points out that acute complications are frequent and variable and include chest syndrome, stroke, infection mainly due to abnormal spleen function and bone pain. Oral lesions are common.

Clinical application: Oral lesions include aseptic pulp necrosis, mucosal damage due to anaemia, fungal infections due to numerous antibiotic therapies, dental eruption delays, bone pain and oral neuropathies including the mental nerve of the chin caused by infarction of the vascularisation of the lower mental nerve or its branches. Bone lesions may also be visible radiologically throughout the skeleton and at the level of the maxilla. Altered pallor of the oral mucosa is the most frequent oral manifestation observed. Atrophy of the tongue is often seen.

Practical implication: Adult sickle cell patients should be seen annually. Mild sickle cell disease does not require particular management. Severe or moderate sickle cell disease requires precautions for dental management:
1. Antibiotic prophylaxis for invasive treatment is needed (endodontics, oral surgery, periodontal surgery, root surface scaling).
2. Clinical and radiological examination to detect oral infectious foci
3. Steroidal anti-inflammatory drugs are contra-indicated.
4. Deep local anaesthesia is suggested and the amount of vasoconstrictor is reduced. Use nitrous oxide inhalation for sedation followed by an inhalation of 100% oxygen for 4-5 minutes at the end of treatment. Use anxiolytics.
5. Controlled prescription of analgesics. Paracetamol and codeine are used together.

Reference: Chekroun, M., Chérifi, H., Fournier, B., Gaultier, F., Sitbon, I.Y., Ferré, F.C. and Gogly, B., 2019. Oral manifestations of sickle cell disease. British dental journal, 226(1), p.27.