Blog 3: Sickle Cell Crisis by Debbie Omodele
What words come to mind when you hear the words ‘sickle cell’? I can almost guarantee that many of you thought of the word pain amongst many. Pain is one of the most common complications of Sickle Cell Disorder (SCD). As mentioned in blog 1 and 2, SCD affects the red blood cells therefore a painful crisis could be triggered anywhere in the body. The most common areas are the:
- Upper and lower limbs
A sickle cell crisis is unpredictable and as there are various triggers for a painful crisis, it is important for patients, parents, and healthcare professionals to be aware of common triggers to educate and ensure they are avoided or at most limit exposure to them. Common triggers include (not exhaustive):
- A sudden change in temperature
One of the many activities that has been often reported to trigger a crisis is swimming where parents frequently request swimming advice. Although we can agree that swimming is an essential life skill however, it has to be considered if it is worth the frequent painful sickle cell episodes that occur soon after every session? Every child is different, and there should be a joint discussion between parent(s)/carers and the child (if old enough to understand risks) where the benefits vs risks are weighed up.
The main trigger in this situation is usually moving from warm air to cold water (sudden change of temperature) and then back into warm air. Having a warm shower before and after swimming to help the body adjust to the atmospheric temperature and using a pool ideally between 26-28 degrees Celsius could be a beneficial precaution to incorporate into the child’s routine.
Did you know there are also non-painful crisis? Yes, you absolutely read that correctly. These are usually referred to as anaemic crisis (a sudden profound episode of anaemia) and require the same level of urgent attention and treatment as a vaso-occlusive crisis (sickle cells blocking blood flow in small blood vessels). The 4 major types of sickle cell crisis include:
- Vaso-Occlusive crisis (VOC)- manage with pain relief and if severe, treatment at your local hospital is required.
- Acute Chest Crisis (primarily caused by hypoxia because of hypoventilation caused by VOC and accounts for 25% of deaths)- managed in a hospital and could start off with symptoms of a chest infection.
- Aplastic crisis (bone marrow stops producing red blood cells usually caused by parvovirus leading to severe anaemia)- usually requires a blood transfusion to gradually raise the haemoglobin.
- Hyper-haemolytic crisis (acute drop in haemoglobin common in patients with G6PD deficiency)- usually requires a blood transfusion to gradually raise the haemoglobin.
Psychological and psycho-social effects are often overlooked yet essential aspects of managing SCD. It is important to offer services that provide a safe and confidential space for patients with SCD to express their feelings and thoughts about having a long-term condition and how to manage low moments by developing practical steps towards self-care and mental wellness. Below are some resources that could support with achieving and maintaining a positive mindset and mental well-being.
Remember, SCD does not define an individual and having a good supportive network is key towards building a positive mindset. With support from family, friends, and healthcare professionals, individuals with SCD can live a fulfilled life and should be encouraged to go after their dreams and aspirations.
https://www.nhs.uk/apps-library/catch-it/ Catch it Learn ow to manage anxiety and depression
https://thrive.uk.com/ Helps you to manage stress and anxiety
https://www.calm.com/ The #1App for meditation and sleep
- Borhade, M.B. and Kondamudi, N.P. Sickle Cell Crisis. [Updated 2022 Aug 29]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK526064/
- Anie, K.A. (2005) Psychological complications in sickle cell disease. British Journal of Haematology. 129(6), pp. 723-9. doi: 10.1111/j.1365-2141.2005.05500.x. PMID: 15952997.
- Pecker, L.H. and Darbari, D.S. (2019) Psychosocial and affective comorbidities in sickle cell disease. Neurosci Lett. 13;705:1-6. doi: 10.1016/j.neulet.2019.04.011. Epub 2019 Apr 6. PMID: 30965108.
- Pool Temperatures | PWTAG Technical Notes 2021- Pool water Treatment Advisory Group
- (https://www.danthermgroup.com/en-gb/set/swimming-pool-temperature-and-other-factors-to-determine-operating-conditions#:~:text=Water%20temperature,-What%20temperature%20is&text=The%20following%20benchmarks%20are%20a,%C2%B0C%2D30%C2%B0C )
- Sickle Cell Disease in Childhood: Standards and Recommendations for Clinical Care (2019) https://www.sicklecellsociety.org/resource/paediatricstandardsresource/
- Simon, E., Long. B. and Koyfman, A. (2016) Emergency Medicine Management of Sickle Cell Disease Complications: An Evidence-Based Update. Journal of Emergency Medicine. 51(4), pp. 370-381.