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Central Venous Lines in Haemophilia. Ljung, Rolf Central venous lines in haemophilia. Ljung, Rolf Published in: Haemophilia DOI: 10.1046/j.1365-2516.9.s1.7.x 2003 Link to publication Citation for published version (APA): Ljung, R. (2003). Central venous lines in haemophilia. Haemophilia, 9(Suppl 1), 88-92. https://doi.org/10.1046/j.1365-2516.9.s1.7.x Total number of authors: 1 General rights Unless other specific re-use rights are stated the following general rights apply: Copyright and moral rights for the publications made accessible in the public portal are retained by the authors and/or other copyright owners and it is a condition of accessing publications that users recognise and abide by the legal requirements associated with these rights. • Users may download and print one copy of any publication from the public portal for the purpose of private study or research. • You may not further distribute the material or use it for any profit-making activity or commercial gain • You may freely distribute the URL identifying the publication in the public portal Read more about Creative commons licenses: https://creativecommons.org/licenses/ Take down policy If you believe that this document breaches copyright please contact us providing details, and we will remove access to the work immediately and investigate your claim. LUND UNIVERSITY PO Box 117 221 00 Lund +46 46-222 00 00 Haemophilia (2003), 9, (Suppl. 1), 88–93 Central venous lines in haemophilia R. LJUNG Departments of Paediatrics and Coagulation Disorders, Lund University, University Hospital, Malmoo,€ Sweden Summary. Infections and technical problems are the been seen in some but not in others. The final most frequent complications when using implantable decision to use a central line has to take into account central venous access devices in patients with hae- the medical goal, the patient’s bleeding tendency, the mophilia. There are two major experiences reported social situation and the expected risk of complica- concerning infections in noninhibitor patients: one is tions at the particular haemophilia centre. Some of approximately 0.2 infections per 1000 days and the the complications may be reduced by adequate other approximately 1.0 (0.7–1.6) per 1000 days. aseptic measures both during implantation and in Infections are more frequent in inhibitor patients and subsequent use, and by clear basic routines for approximately one infection per 6–12 months of use surveillance of the systems and repeated education can be expected. The figures are low for clinically of the users. apparent thrombosis in the larger series on record, but routine venograms were not carried out in most Keywords: haemophilia A, haemophilia B, factor of these series. In studies where this has been done, a VIII, factor IX, catheter high frequency of abnormalities on venograms has venous catheters. Some clinicians are in favour while Introduction others are more critical due to the frequency of Treatment of haemophilia A or B with, respectively, complications. The aim of this paper is to give an factor VIII (FVIII) or factor IX (FIX) concentrates, overview of the experiences so far with the use of irrespective of whether this is done in the event of a central venous lines in patients with haemophilia. bleed or as a prophylactic infusion, requires uncom- plicated venous access. If the patient is treated on a Evaluating the studies on record prophylactic regimen, infusions are usually given three times per week or every other day in haemo- When evaluating previous studies on central venous philia A and twice per week or every third day in lines for treatment in haemophilia, several aspects haemophilia B. Ideally, primary prophylaxis should need to be considered. First, some series include both be started at an early age, and it should be possible patients with external catheters and those with for the parents to administer the concentrate at home implanted subcutaneous ports [6]. It has been clearly [1]. This may be very difficult using a peripheral vein shown in a large series of non-haemophilia children in a small boy. If the child is treated on demand, the that implantable systems have a much lower risk of administration of concentrate for a bleed should infection compared with external catheters: 0.7 preferably be done immediately by the parents at infections per 1000 patient days for subcutaneous home, a situation in which safe and easy access to a ports vs. 4.3 for external catheters (P < 0.0001) [14]. vein is necessary. Several reports have been published Secondly, many series include haemophilia describing varying experiences with the use of central patients both with and without inhibitors. It has venous catheters in patients with haemophilia [2–13]. been shown in many series that the frequency of There is a diversity of opinions among clinicians infection is higher in patients with inhibitors concerning the benefits and adverse effects of central [3,6,11]. In one series with a median follow-up time of 30 months, 23% (nine of 39) of noninhibitor patients had complications, corresponding to 0.23 Correspondence: Rolf Ljung, MD, Paediatric Clinic, University complications per 1000 patient days. In comparison, Hospital, SE-205 02 Malmoo,€ Sweden. Tel.: +46 40 331286; fax: +46 40 336226; 64% (seven of 11) of inhibitor patients manifested e-mail: [email protected] complications [9]. 88 Ó 2003 Blackwell Publishing Ltd HAEMOPHILIA – CENTRAL VENOUS LINES 89 Thirdly, some series include patients with HIV Infections infection who may be immunocompromised and thus naturally have a higher risk of infection than the Infection is the most frequent complication when HIV-negative patients [6]. Series that include many using a central venous line. Table 1 shows some of HIV-infected persons do not give an accurate risk the larger studies from recent years. Unfortunately, it figure for the otherwise healthy haemophilia patient is usually not possible to study exclusively a cohort considering a venous device. Another relevant con- of haemophilia children who are HIV negative and to sideration in assessing risk is that many series also separate inhibitor from noninhibitor patients. Some include patients with other coagulation disorders series distinguish local infections around the port [4,8], and we do not know if experiences from from proved bacteraemia/septicaemia. When discuss- other coagulation disorders are transferable to ing infections or other complications it should be haemophilia. emphasized that many systems have been used for Most series with implantable catheters have been prolonged periods before the complication occurred. using the Port-A-Cath system, and almost all reports It is obvious that the frequency of infections is of larger series in haemophilia patients are with this higher in patients with inhibitors. In the series by system. However, some series also report peripheral Ljung et al. [9], 64% of the inhibitor patients (seven intravenous access devices (P.A.S. PortsTM, Slim- of 11) manifested infections after 1–47 months of use PortsTM) [8]. These seem to be well accepted by the (mean, 15 months; median, 15 months). In the series children and parents. In young children it is less by McMahon et al. [11], 23 infections occurred in 18 threatening to insert a needle in the periphery of the central venous lines in patients with inhibitors, a rate body, and they can avoid the visible profile of the of one infection per 8.5 patient months or 4.3 port on the chest. However, peripheral ports have infections per 1000 patient days, which is a consid- been associated with a higher frequency of thrombo- erable higher frequency than in the other study. In a phlebitis and thrombosis, and the average time that review, van den Berg [16] found that in various the patient may benefit from the device is probably studies 50%)83% of patients with inhibitors can be shorter [8]. The PercusealÒ device (Percuseal Med- expected to get an infection. Collins et al. calculated ical, Huskvarna, Sweden) is implanted into the that children with inhibitors develop one infection subcutaneous tissue, with the top portion protruding per 8.3 months compared with one per 50 months from the surface of the skin. The advantage is for noninhibitor patients [17]. In the Spanish registry painless clotting factor application without skin of central venous lines, 54% (19 of 35) of the puncture [15]. Although interesting, since it is based patients with inhibitors had infections, compared on a new technique, its usefulness needs to be with 30% (six of 20) of the patients without demonstrated in larger series. inhibitors (Tusell et al., personal communication This overview will be mainly focused on the Port- and World Federation of Haemophilia (WFH) Con- A-Cath system, since most data from larger series are gress, Seville, 2002). One reason for the higher based on this device. The most frequent compli- frequency of infections in inhibitor patients may be cations with implantable ports are infections and that these patients have small haemorrhages around technical problems. Thrombosis is a serious potential the port after an injection that will stimulate bacter- adverse effect. ial growth in the subcutaneous tissue. Another Table 1. The rate of infection in recent series with haemophilia patients using central venous lines [6,8–11]. Number of Rate of infection Study patients (n) per 1000 patient days Comment Blanchette et al. 1996 [6] 19 0.7 3 patients with inhibitors, 3 HIV+ Perkins et al. 1997 [8] 35 1.2 (central) 7/32 inhibitors, 2/32 vWD 0.7 (peripheral device) Ljung et al. 1998 [3] 53 0.19 11 patients with inhibitors Sanagostino et al. 1998 [13] 15 0.3 2 inhibitor patients, 13 on prophylaxis Miller et al. 1998 [10] 41 0.14 Includes external McMahon et al. 2000 [11] 58 1.6 (without inhibitor) 77/86 devices Port-A-Cath; 4.3 (with inhibitor) 37/58 patients haemophilia Tusell (personal 20 0.28 (prophylaxis) Port-A-Caths used for communication, 2002) 35 0.68 (ITI) prophylaxis/on demand or ITI Ó 2003 Blackwell Publishing Ltd Haemophilia (2003), 9, (Suppl.
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