Pediatr Blood Cancer 2005;44:669–672

Cathlink11 20: A Subcutaneous Implanted Central VenousAccess Device Used in Children With Sickle Cell Disease

on Long-Term Erythrocytapheresis—A Report of Low Complication Rates

Ashok Raj, MD,1 Salvatore Bertolone, MD,1* Sheldon Bond, MD,2 Diane Burnett, ARNP,1

and Audria Denker, RN3

INTRODUCTION

Erythrocytapheresis procedures are increasingly usedin the treatment of severe acute complications of sicklecell disease (SCD). These include stroke, acute chestsyndrome, priapism, primary stroke prevention in patientswith abnormal blood flow velocity in their internal carotidand middle cerebral arteries as measured by transcranialdoppler ultrasonography, in some patients with frequentsevere pain episodes, and in preparation for surgery withthe aim of reducing hemoglobin S to less that 30% [1,2].Central venous access devices (CVAD) are valuableinstruments for long-term intravenous treatment inpediatric patients, but implantation and use of thesedevices are each associated with complications. Experi-ence with the use of CVAD in patients with SCD on long-term transfusion is limited. The published studies alsoreport wide variability in the rates of CAVD-associatedcomplications.

Jeng et al. observed a higher percentage of CVAD-associated infection in 19 SCD patients aged 1.5–30 yearsthan patients with cancer in their institution [3]. Anotherstudy that evaluated five adult patients with SCDconcluded that these patients have a greater incidence ofCVAD-associated complications and an increased rate ofpremature removal of catheters than patients with cancer[4]. However, one study found that the rates of CVAD-

associated complications or of premature removal for25 patients with SCD aged 8 months to 23 years did notdiffer from those reported for patients with cancer,HIV infection, or cystic fibrosis [5]. Moreover, the studypopulations in these studieswere not uniformwith the typeof CVAD used as some patients had external Broviac linesand others had implantable ports.

A preferable CVAD for outpatient automated red cellexchange transfusions would combine the flexibility ofsilicone with the relative non-thrombogenicity of poly-urethane. It would permit adequate whole blood flows,would be acceptable to the apheresis staff, and would not

Background. Experience with the use ofcentral venous access device (CVAD) in chil-dren with sickle cell disease (SCD) on hyper-transfusion is limited and published studiesreport wide variability in the rates of CVAD-associated complications. Procedure. In thisstudy, a total of 18 Cathlink1 20 ports (BardAccess systems, Salt Lake City, UT) wereimplanted in 15 patients aged 7–20 years withSCD for 19, 230 catheter patient days. Results.No peri-operative complications were observed.Three episodes of catheter occlusion requiringreplacement occurred in two patients for anobserved rate of 0.16 per 1,000 catheter patientdays for thrombotic occlusion. One patient

required port replacement and another patientrequired replacement twice. In 13 out of 15patients, the median duration of port use was45 months. No episode of catheter-associatedbacteremia or catheter tunnel infections wasobserved. Conclusions. Comparing our resultswith other reported studies of CVAD-associatedcomplications in patients with SCD, we ob-served a lower rate of complications with theuse of Cathlink 20 ports. These findings may bepartly related to the design of the port and partlyto a dedicated group of nurses accessing theports in a controlled environment in an aphe-resis unit. Pediatr Blood Cancer 2005;44:669–672. � 2005 Wiley-Liss, Inc.

Key words: central venous access device; erythrocytapheresis; sickle cell disease

——————1Department of Pediatrics, Division of Pediatric Hematology/

Oncology, School of Medicine, University of Louisville, Louisville,

Kentucky

2Department of Surgery, School of Medicine, University of Louisville,

Louisville, Kentucky

3Apheresis Unit, Kosair Children’s Hospital, Louisville, Kentucky

Grant sponsor: National Institutes of Health Comprehensive Sickle

Cell Center Program Project; Grant number: G020252.

*Correspondence to: Salvatore Bertolone, Professor of Pediatrics,

Division of Pediatric Hematology/Oncology, School of Medicine,

University of Louisville, 571 South Floyd Street, Suite 445, Louisville,

KY 40202. E-mail: sjbert01@louisville.edu

Received 19 April 2004; Accepted 27 September 2004

� 2005 Wiley-Liss, Inc.DOI 10.1002/pbc.20252

impose on the lifestyle of the patient [6]. With thesecriteria in mind, we investigated the Cathlink 20 (BardAccess Systems, Salt Lake City, UT), a subcutaneouslyimplantable CVAD, for long-term erythrocytapheresis inpatients with SCD.

MATERIALS AND METHODS

A study to define the efficacy and complications relatedto the Cathlink 20 as a CVAD for automated red cellexchange transfusions in patients with homozygous SCDwas performed at Kosair Children’s Hospital, Louisville,KY after approval from the Institutional Review Board.This was a non-randomized study since Cathlink 20 wasthe only CVAD used for erythrocytapheresis in our center.

Cathlink 20

Cathlink 20 ports were surgically implanted in thepatients under general anesthesia. All procedures wereperformed under fluoroscopic guidance with chest X-rayconfirmation. The Cathlink 20 port is placed in a smallsubcutaneous pocket on the anterolateral chest wall withthe catheter tip in the superior vena cava in a similarfashion to an infusaport placement. They were accessedby a group of four trained nurses in a controlled environ-ment in an apheresis unit using an 18 or 20 gauge BDAngiocathTM AutoguardTM IV Catheter (BD MedicalSystems, Sandy, UT). The port was flushed with 10 cc ofnormal saline initially and then drawn back to check forblood return. After completion of erythrocytapheresis, theport was again flushed with 10 cc of normal saline. Thiswas followed by a slow push of 2,000 U of heparin mixedwith 8 cc of normal saline while gently pulling the IVcatheter out. Cathlink 20 was accessed for blood culturesand antibiotic administration when patients presentedwith febrile episodes only when the trained nurses were

available.Otherwise peripheral blood cultureswere drawnand antibiotics administered through a peripheral vesseluntil the CVAD was accessed.

Erythrocytapheresis

Transfusion of all patients consisted of sickle negative,leukocyte-reduced, and phenotypically matched blood forred cell antigens C, E, K, Fya, and JKb. Erythrocytapher-esis was performed using a COBE (Lakewood, CO)Spectra continuous-flow system thatmaintains isovolemicconditions, as previously described [7]. Patients receivederythrocytapheresis therapy on a monthly basis, with theaim of maintaining HbS levels below 30%.

Complications

We assessed the rates of significant peri-operativecomplications, infections and catheter occlusion requiringcatheter removal. We used broader criteria for infectionsthan that described by Jeng et al. [3], to include positiveblood cultures drawn through a peripheral vessel orCVAD. We also looked for findings of catheter tunnelinfection or exit site infection on physical examination ofthe patients. We searched the radiologic records toevaluate the results of dye and ultrasonographic studies.Only radiologically identified thrombi was accepted asevidence of catheter-associated thrombosis as in the studyby Jeng et al. Minor episodes of catheter occlusionsuccessfully treated with thrombolytic agents were notrecorded. Complication rates were calculated per 1,000catheter days.

RESULTS

Characteristics of the study population are presented inTable I. All patients with homozygous SCD weremaintained on continuous erythrocytapheresis on amonthly basis. Patients were on long-term erythrocyta-pheresis for cerebrovascular accidents and for preventionof recurrent stroke (eight patients), abnormal blood flowvelocity on transcranial doppler ultrasound (two patients),recurrent acute chest syndrome (four patients), and forfrequent severe painful episodes following discontinua-tion of transfusion and failure of hydroxyurea therapy (onepatient). A total of 18 Cathlink 20 ports were implanted in15 patients with SCD for 19, 230 catheter patient days.No peri-operative complications were observed. Threeepisodes of catheter occlusion requiring replacementoccurred in two patients. One patient required portreplacement once after 23 months of continuous use.Another patient required port replacement twice. Thelatter had Cathlink 20 continuously used for 16 monthsprior to the first thrombotic occlusion and for 21 monthsbefore the second occlusion. The observed rate forthrombotic occlusion was 0.16 per 1,000 catheter patient

Fig. 1. Cathlink1 20 port system with a funnel shaped entrance that

guides the over the needle IV catheter assembly into the angled access

pathway to the needle stop.

670 Raj et al.

days. In 13 out of 15 patients, the median length ofcontinuous port usewas 45months (range 30–64months).There were six febrile episodes in the patients but noepisode of bacteremia or catheter tunnel infections wasobserved.

DISCUSSION

Limited experience exists in the literature regarding thelong-term use of CVAD in children with SCD onhypertransfusion. Our experience with the use of Cathlink20 port in 15 patients on continuous erythrocytapheresison a monthly basis andmonitored over an extended lengthof time has been associated with a low rate of complica-tions.

Although the potential risk of peri-operative mechan-ical complications of CVAD in these children is same asfor other groups [8], none was observed. Likewise noadverse anesthetic related problemswere encountered.Weattribute this to careful preoperative preparation andimproved surgical and anesthetic techniques.

None of our patients developed invasive infectionsduring the period of hypertransfusion. All our patients hadreceived pneumococcal immunizations and were main-tained on penicillin prophylaxis, which could haveprevented invasive pneumococcal infections. A recentstudy byAdamkiewicz et al. [10] has shown a reduction inthe rates of invasive infections in childrenwith SCD for theperiod 1997–2000, an era of penicillin prophylaxis andthe 23-valent pneumococcal vaccine (PPV23), to at leasthalf those of the preceding decade and an even greaterreduction compared to the rates reported in the 1970s,before the use of pneumococcal vaccines and penicillinprophylaxis. However, the organisms (Gram positive andGram negative) commonly detected from blood culturesdrawn from CVAD [3] are not penicillin susceptible andtherefore not influenced by penicillin prophylaxis.

Previously reported studies on hypertransfusion in SCDshow discordant rates of infection in patients with SCD asshown in Table II. The patients studied by Jeng et al.included both children and adults. Adult patients had asignificantly higher rate of infection (7.0 per 1,000 CVAD

TABLE I. Characteristics of the Patients With Sickle Cell Disease (SCD) UndergoingLong-Term Erythrocytapheresis

Patient

number

Age

(years)

Age at start

of ECP

(years)

Indication

for ECP

Duration of

CVAD (until

report), months Catheter complications

1 13 9 Recurrent ACS 45 None

2 20 16 CVA 57 None

3 13 9 Recurrent ACS 44 None

4 11 9 " TCD 30 None

5 15 13 CVA 30 None

6 10 6 CVA 45 Catheter thrombosis w/occlusion�2

7 11 7 CVA 37 None

8 7 3 Recurrent ACS 47 None

9 18 14 MPC 47 None

10 18 15 Recurrent ACS 37 Catheter thrombosis w/occlusion�1

11 15 11 CVA 37 None

12 10 8 " TCD 30 None

13 17 12 CVA 60 None

14 15 10 CVA 64 None

15 17 13 CVA 54 None

ECP, erythrocytapheresis; ACS, acute chest syndrome; " TCD, increased transcranial doppler; CVA,

cerebrovascular accident; MPC, multiple pain crisis.

TABLE II. Central Venous Access Device (CVAD)-Associated Complications From Literature

Study Patient demographics

Number of

patients

with SCD

Rate of

infection

Rate of thrombotic

events (per 100

CVAD days)

Present study Pediatric 15 0.00 0.16

Jeng et al. [3] Adult and pediatric 19 5.50 0.99

Abdul-Rauf et al. [5] Pediatric 25 0.86 0.29

McReady et al. [4] Adult 5 4.00 N/A

Phillips et al. [9] Adult 10 0.46a 0.46a

aIncidence not published, but calculated in the study by Jeng et al.

Report of Low Complication Rates 671

days) than pediatric patients (4.5 per 1,000 CVAD days)(P¼ 0.05). Infection rates were higher with Mediports(5.9 per 1,000 CVAD days) than external Broviac lines(1.9 per 1,000 CVAD days) (P< 0.05). In their study,Mediports were the CVAD most commonly used inpatients undergoing chronic transfusion therapy whileexternal Broviac catheters were used in patients under-going bone marrow transplantation (BMT). The authorsalso state that the Mediports were frequently accessed butdo not state if only highly trained nurses were allowed toaccess theCVAD.Most of their patientswithBroviac lineswere undergoing BMT, which requires skilled nursingcare in the hospital. Jeng et al. state that the likely reasonfor a lower incidence of infection in their patients withBroviac lines was a heightened awareness of infectioncontrol practices in the nurses.A pediatric study byAbdul-Rauf et al. [5] included 25 patients. Their infection ratewas 0.29 per 1,000CVADdays. Twenty-eight of 31CVADwere implanted ports and three were Broviac catheters.Indications for CVAD placement included long-termtransfusion, venous access for recurrent medical compli-cations requiring intravenous fluids, antibiotics, andanalgesics in association with poor peripheral access orlong-term antibiotic administration to treat osteomyelitis.All the infections in the series by Abdul-Rauf et al. [5]occurred in patients with implanted ports. However, theauthors do not state if there was restricted access for theports to only highly trained nurses. On the other hand, thedesign of Cathlink 20, particularly the polymer materialused to make the catheter may also be contributing factor.Indorf et al. [11] have shown that silicone cathetermaterials may have unique properties that increase therisk of infection after implantation than polyurethanecatheters. The mechanism of this effect is localizedexcessive complement activation by silicone, which caninterferewith neutrophil chemotaxis as well as deplete theability to opsonize microorganisms [11].

The lack of infections in our patient population may betherefore related to a combination of factors. The absenceof catheter infectionsmay be partly related to the design ofCathlink 20 and to restricted access by only a dedicatedgroup of specialty trained nurses in a controlled environ-ment in an apheresis unit.

Our rate of catheter thrombosis is lower than otherreported studies (Table II). Our criteria for definingcatheter thrombosis relied strictly on radiographic doc-umentation as used in the study by Jeng et al. [3]. However,criteria used as evidence of catheter thrombosis in otherstudies included episodes of difficult catheter flushing ordifficult blood withdrawal [4,5]. Because line positioncould have contributed to these difficulties without thepresence of a thrombus, criteria used in these studies mayhave resulted in overestimation of the incidence ofthrombotic events.

The conclusions drawn from the published literatureregarding the thrombotic potential of a CVAD in sicklecell patients is also tenuous because the patients were alsonot uniformly fitted with the same type of device. Becauseour sample size was small, further studies on a largernumber of patients with Cathlink 20 are required to ensurea low rate of catheter thrombosis with this type of CVAD.The fact that numerous etiologies are being uncovered topromote thrombotic potential heightens this problem ofneeding high numbers to reveal thrombotic events. Therewere no reported problems in removal of Cathlink 20 fromthe patients. However, our experiencewith removal of thisdevice in two patients is too little to assure a low rate ofcomplications with catheter removal.

Although our experience with 15 children with SCDindicates that Cathlink 20 represents an effective, reliable,and safe means of establishing and maintaining venousaccess for patients requiring long-term erythrocytapher-esis, it must be emphasized that the longest follow up forany of our patients is less than 6 years. Therefore, long-term risks of infection, thrombosis, problems withremoval of this device, and other unforeseen complica-tions are unknown.

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