Hemophilia secondary prevention
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]
Overview
Effective measures for the secondary prevention of hemophilia include avoidance of invasive fetal monitoring of a hemophilic fetus, avoidance of operative vaginal delivery, administration of vitamin K injection with care, availability of the factor concentrate at the time of delivery, infusion of factor VIII or IX concentrate at least once weekly for ≥ 45 weeks per year, and the use of e-Diaries to improve record keeping of hemophilia patients' home treatment and bleeding episodes.
Secondary Prevention
Effective measures for the secondary prevention of hemophilia include:
- Avoidance of invasive fetal monitoring of a hemophilic fetus[1]
- Operative vaginal delivery should be avoided due to the risk of intracranial and extracranial hemorrhage.[1][2]
- Vitamin K injection, via the intramuscular or subcutaneous route, should be given with a 27 gauge (or smaller) needle, and followed by the application of direct pressure for approximately 5 minutes[1][3]
- The World Federation of Hemophilia recommends that vaccines be administered subcutaneously to patients with hemophilia[1][4]
- An appropriately sized vial of the chosen factor concentrate should be available at the time of delivery and the staff present should be well skilled in the reconstitution and administration of the concentrate[1]
- Cryoprecipitate, and fresh frozen plasma should not be used in newborns unless the appropriate factor concentrate is available[1]
- Desmopressin should also be not used in newborns due to the risk of developing hyponatremia[5][6]
- Infusion of factor VIII or IX concentrate at least once weekly for ≥ 45 weeks per year[7][8][9][10]
- Emicizumab, a bispecific monoclonal antibody bridging factor IXa and Xa, can be used to replace the function of missing factor VIIIa[11]
- Invasive orthopedic interventions to control recurrent hemarthrosis and to prevent the loss of joint function[12][13]
- Recombinant factor VIII Fc fusion protein is associated with improved bleeding rates and lower weekly factor consumption compared to the conventional recombinant factor VIII [14]
- The use of e-Diaries improves record keeping of hemophilia patients' home treatment and bleeding episodes[15]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 Moorehead PC, Chan A, Lemyre B, Winikoff R, Scott H, Hawes SA, Shroff M, Thomas A, Price VE (October 2018). "A Practical Guide to the Management of the Fetus and Newborn With Hemophilia". Clin. Appl. Thromb. Hemost.: 1076029618807583. doi:10.1177/1076029618807583. PMID 30373387. Vancouver style error: initials (help)
- ↑ Kulkarni R, Soucie JM, Lusher J, Presley R, Shapiro A, Gill J, Manco-Johnson M, Koerper M, Mathew P, Abshire T, Dimichele D, Hoots K, Janco R, Nugent D, Geraghty S, Evatt B (November 2009). "Sites of initial bleeding episodes, mode of delivery and age of diagnosis in babies with haemophilia diagnosed before the age of 2 years: a report from The Centers for Disease Control and Prevention's (CDC) Universal Data Collection (UDC) project". Haemophilia. 15 (6): 1281–90. doi:10.1111/j.1365-2516.2009.02074.x. PMID 19637999.
- ↑ Busfield A, McNinch A, Tripp J (September 2007). "Neonatal vitamin K prophylaxis in Great Britain and Ireland: the impact of perceived risk and product licensing on effectiveness". Arch. Dis. Child. 92 (9): 754–8. doi:10.1136/adc.2006.105304. PMC 2084039. PMID 17537760.
- ↑ Srivastava A, Brewer AK, Mauser-Bunschoten EP, Key NS, Kitchen S, Llinas A, Ludlam CA, Mahlangu JN, Mulder K, Poon MC, Street A (January 2013). "Guidelines for the management of hemophilia". Haemophilia. 19 (1): e1–47. doi:10.1111/j.1365-2516.2012.02909.x. PMID 22776238.
- ↑ Das P, Carcao M, Hitzler J (June 2005). "DDAVP-induced hyponatremia in young children". J. Pediatr. Hematol. Oncol. 27 (6): 330–2. PMID 15956888.
- ↑ Sharma R, Stein D (August 2014). "Hyponatremia after desmopressin (DDAVP) use in pediatric patients with bleeding disorders undergoing surgeries". J. Pediatr. Hematol. Oncol. 36 (6): e371–5. doi:10.1097/MPH.0000000000000185. PMID 24942016.
- ↑ Biss TT, Chan AK, Blanchette VS, Iwenofu LN, McLimont M, Carcao MD (September 2008). "The use of prophylaxis in 2663 children and adults with haemophilia: results of the 2006 Canadian national haemophilia prophylaxis survey". Haemophilia. 14 (5): 923–30. doi:10.1111/j.1365-2516.2008.01810.x. PMID 18637844.
- ↑ Feldman BM, Rivard GE, Babyn P, Wu J, Steele M, Poon MC, Card RT, Israels SJ, Laferriere N, Gill K, Chan AK, Carcao M, Klaassen RJ, Cloutier S, Price VE, Dover S, Blanchette VS (June 2018). "Tailored frequency-escalated primary prophylaxis for severe haemophilia A: results of the 16-year Canadian Hemophilia Prophylaxis Study longitudinal cohort". Lancet Haematol. 5 (6): e252–e260. doi:10.1016/S2352-3026(18)30048-6. PMID 29731369. Vancouver style error: initials (help)
- ↑ Herbert RD, Broderick CR, Barnes C, Billot L, Zhou A, Latimer J (2018). "Optimization of prophylaxis for hemophilia A". PLoS ONE. 13 (2): e0192783. doi:10.1371/journal.pone.0192783. PMC 5813962. PMID 29447219.
- ↑ Fischer K, Ljung R (September 2017). "Primary prophylaxis in haemophilia care: Guideline update 2016". Blood Cells Mol. Dis. 67: 81–85. doi:10.1016/j.bcmd.2017.02.004. PMID 28302350.
- ↑ Mahlangu J, Oldenburg J, Paz-Priel I, Negrier C, Niggli M, Mancuso ME, Schmitt C, Jiménez-Yuste V, Kempton C, Dhalluin C, Callaghan MU, Bujan W, Shima M, Adamkewicz JI, Asikanius E, Levy GG, Kruse-Jarres R (August 2018). "Emicizumab Prophylaxis in Patients Who Have Hemophilia A without Inhibitors". N. Engl. J. Med. 379 (9): 811–822. doi:10.1056/NEJMoa1803550. PMID 30157389.
- ↑ Tobase P, Lane H, Siddiqi AE, Soucie JM, Ingram-Rich R, Ward S, Gill JC (November 2018). "Risk factors associated with invasive orthopaedic interventions in males with haemophilia enrolled in the Universal Data Collection program from 2000 to 2010". Haemophilia. 24 (6): 964–970. doi:10.1111/hae.13511. PMID 29957840.
- ↑ Serban M, Poenaru D, Patrascu J, Ursu E, Savescu D, Ionita H, Jinca C, Pop L, Talpos-Niculescu S, Ritli L, Arghirescu S, Mihailov D, Schramm W (2014). "Risks and challenges of orthopaedic invasive interventions in haemophilia in a low-resource country. A single-center experience". Hamostaseologie. 34 Suppl 1: S30–5. doi:10.5482/HAMO-14-01-0007. PMID 25382767.
- ↑ Iorio A, Krishnan S, Myrén KJ, Lethagen S, McCormick N, Yermakov S, Karner P (May 2017). "Indirect comparisons of efficacy and weekly factor consumption during continuous prophylaxis with recombinant factor VIII Fc fusion protein and conventional recombinant factor VIII products". Haemophilia. 23 (3): 408–416. doi:10.1111/hae.13160. PMID 28233383.
- ↑ Banchev A, Goldmann G, Marquardt N, Klein C, Horneff S, Langenkamp R, Frankenberger T, Oldenburg J (January 2019). "Impact of Telemedicine Tools on Record Keeping and Compliance in Haemophilia Care". Hamostaseologie. doi:10.1055/s-0038-1676128. PMID 30654393.