Vaso-occlusion and VOCs can lead to end-organ damage and multiorgan failure1–4
Sickle cell disease (SCD) is a multicellular disorder that can affect nearly every organ in the body, including the heart, eyes and bones5–8
Every VOC matters1–4
VOCs are unpredictable, extremely painful events that can last an average of 10 days, but the VOC burden can last a lifetime9–13*
One severe VOC alone can increase the risk of death and decrease quality of life14
One or more severe VOCs per year have been associated with an increased risk of death at an earlier age14†
Severe VOCs in adults may cause irreversible organ damage15†‡
In a 40-year observational study of 1,056 patients with SCD, VOCs leading to hospitalisation in adults were significantly associated with an increased risk of irreversible organ damage15
VOCs are the leading cause of hospitalisations and accident & emergency (A&E) visits in patients with SCD16–19
- 74% (n=470/632§) of hospital admissions in England for patients with SCD may be due to VOCs20**
- 32% (n=~96/299) of people in the UK experiencing a VOC in the past year had to be hospitalised21††
ADAKVEO® is subject to additional monitoring. This will allow quick identification of new safety information. Healthcare professionals are asked to report any suspected adverse reactions. Reporting suspected adverse reactions after authorisation of ADAKVEO® is important to understand the safety profile of the treatment for SCD patients.
Indication: ADAKVEO® (crizanlizumab) is indicated for the prevention of recurrent VOCs in patients with SCD who are aged 16 years and over. It can be given as an add-on therapy to hydroxyurea/hydroxycarbamide (HU/HC) or as monotherapy in patients for whom HU/HC is inappropriate or inadequate.22
ADAKVEO® has a conditional marketing authorisation and further evidence is awaited.
*Data accessed via literature review accomplished by PubMed search of ‘sickle cell disease’ in combination with other key terms: pain, vision, hearing, education, cognition, school performance, language, employment, depression, mobility, emotional problems, or behavioural problems. No restriction was placed on year of publication, no articles older than 1986 were used.13
†VOCs were defined as a sickle cell acute painful episode in people that were hospitalised.14
‡Irreversible organ damage defined as comorbid clinical conditions that do not spontaneously improve with time.15
§A spell is comprised of one or more continuous finished consultant episodes.20
**Results of an analysis of admissions between January 2008 and July 2010 in London.20
††Results from the 299 UK patients that contributed to the International Sickle Cell World Assessment Survey (SWAY).21
Abbreviations: A&E, accident and emergency; HC, hydroxycarbamide; HU, hydroxyurea; SCD, sickle cell disease; SWAY, Sickle Cell World Assessment Survey; VOC, vaso-occlusive crisis.
- Steinberg MH. Sickle cell disease and associated hemoglobinopathies. In: Goldman L, Ausiello D, eds, Cecil Medicine, 23rd ed. Philadelphia, PA; Saunders Elsevier; 2008:1217–1226.
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- Ballas SK, et al. Hemoglobin. 1995;19(6):323–333.
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- Darbari DS, et al. PLoS One. 2013;8(11):e79923.
- Powars DR, et al. Medicine (Baltimore). 2005;84(6):363–376.
- American Society of Hematology. State of Sickle Cell Disease: 2016 Report. Washington, DC: 2016. Available at: http://www.scdcoalition.org/report.html. Date accessed: March 2021.
- Lentz MB & Kautz DD. Nursing. 2017;41(1):67–68.
- Wilson BH & Nelson J. Pain Management Nursing. 2015;16(2):146–151.
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- Green SA, et al. J R Soc Med Sci Sh Rep. 2012;3(37):1–7.
- Inusa B, et al. UK Patient Perceptions on the Symptomatic and Emotional Burden of Vaso-Occlusive Crises Resulting From Sickle Cell Disease. Poster presented at the Annual Scientific Meeting of the British Society for Haematology 2020; Nov 09–14: Virtual Event.
- ADAKVEO® Summary of Product Characteristics.