Every VOC matters1–4
Sickle cell disease (SCD) is a multicellular disorder that can affect nearly every organ in the body, including the heart, eyes and bones5–8
VOCs are unpredictable, extremely painful events that can last an average of 10 days, but the VOC burden can last a lifetime9–13*
Vaso-occlusion and VOCs can lead to end-organ damage and multiorgan failure1–5
Severe VOCs in adults may cause irreversible organ damage31†‡
In a 40-year observational study of 1,056 patients with SCD, hospitalisation following a VOC, was a marker for the increased likelihood of the presence of irreversible organ damage, including:31
One severe VOC alone can increase the risk of death32
During a longitudinal study, 264 adult patients with sickle cell anaemia (HbSS) were tracked for a median of ~5 years in the United States (The Bethesda Sickle Cell Cohort Study, 2001–2007) to determine associations between VOCs and death32
One or more severe VOCs per year have been associated with an increased risk of death at an earlier age32†
- VOCs were associated with death at a younger age: 55.8 years for patients with 1 severe VOC in the past year vs. 66.2 years for patients with 0 VOCs in the past year (P=0.04)32
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.33
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.31
‡Irreversible organ damage defined as comorbid clinical conditions that do not spontaneously improve with time.31
Abbreviations: ACS, acute chest syndrome; A&E, accident and emergency; HbSS, sickle cell anaemia; HC, hydroxycarbamide; HU, hydroxyurea; SCD, sickle cell disease; VOCs, vaso-occlusive crises.
- 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.
- Conran N, et al. Hemoglobin. 2009;33(1):1–16.
- Steinberg MH. N Engl J Med. 1999;340(13):1021–1030.
- Madigan C & Malik P. Expert Rev Mol Med. 2006;8(9):1–23.
- Piel FB, et al. N Engl J Med. 2017;376(16):1561–1573.
- Serjeant GR. J R Coll Physicians Lond. 1996;30:37–41.
- Rees DC, et al. Lancet. 2010;376:2018–2031.
- Vichinsky EP, et al. N Engl J Med. 2000;342(25):1855–1865.
- Ballas SK, et al. Blood. 2012;120(18):3647–3656.
- Ballas SK, et al. Hemoglobin. 1995;19(6):323–333.
- Hasan S, et al. J Natl Med Assoc. 2003;95(5):533–538.
- Brandow AM, et al. Br J Haematol. 2009;144(5):782–788.
- Swanson ME, et al. Am J Prev Med. 2011;41(6 Suppl 4):S390–S397.
- Thust SC, et al. Br J Radiol. 2014;87(1040):20130699.
- Lonergan GJ, et al. Radiographics. 2001;21(4):971–994.
- Shah R, et al. World J Gastrointest Pathophysiol. 2017;8(3):108–116.
- Diggs, LW. Am J Clin Pathol. 1956;26(10):1109–1118.
- Schubert TT. Gastroenterology. 1986;90(6),2013–2021.
- Koskinas J, et al. Scand J Gastroenterol. 2007;42(4):499–507.
- Crane GM & Bennet NE. Anemia. 2011;2011:297364.
- Bennett N & Mulhall J. J Sex Med. 2008;5(5):1244–1250.
- Adeyoju AB, et al. BJU international. 2002;90(9):898–902.
- Smith Whitley K. Blood. 2014;124(24):3538–3542.
- Samuels-Reid J & Scott RB. South Med J. 1985;78(4):384–385.
- Westerman MP, et al. Am J Hematol. 1997;54(3):183–188.
- Yoong WC & Tuck SM. J Obstet Gynaecol. 2002;22(4):399–401.
- Gladwin MT, et al. N Engl J Med. 2004;350(9):886–895.
- Nath KA & Hebbel RP. Nat Rev Nephrol. 2015;11(3):161–171.
- Platt OS, et al. N Engl J Med. 1994;330(23):1639–1644.
- Hamideh D & Alvarez O. Pediatric blood & cancer. 2013;60(9):1482–1486.
- Powars DR, et al. Medicine (Baltimore). 2005;84(6):363–376.
- Darbari DS, et al. PLoS One. 2013;8(11):e79923.
- ADAKVEO® Summary of Product Characteristics.