PICC o Midline
nella fibrosi cistica?
M. Ricciuti, S. Di Matteo
U.O.C. Hospice e Cure Palliative
Epidemiology of Cystic Fibrosis
• Cystic fibrosis is a heterogeneous recessive genetic
disorder with features that reflect mutations in the
cystic fibrosis transmembrane conductance regulator
(CFTR) gene.
• Prevalence
• 30,000 in the US
• 70,000 worldwide
• Change in patient demographics
Cystic Fibrosis Mutations
•
Class I:- No synthesis of CFTR protein:
These are mis-sense,
nonsense or frame shift mutations making the cell devoid
of CFTR protein. The phenotypic effects tend to be more
severe.
•
Class II:- Defective processing of CFTR protein:
Common to this
class is ∆F508: This class of mutation is characterized by
faulty processing and lack of transport of CFTR protein to
the apical membrane.
•
Class III:- Defective Regulation:
It is a type of missense
mutation leading to the formation of nonfunctional CFTR
at the apical membrane.
•
Class IV:- Defective Conductance:
It targets the function of
CFTR, reducing the conductance of the chloride ion
channel.
•
Class V:- Reduced production of CFTR:
Characterized by reduced
Diagnosis of CF
Genotyping
• Over 1,500 mutations on the CF gene à
malfunctioning CF transmembrane conductance
regulator (CFTR)
• Absent CFTR or defective functioning of CFTR
is possible
• CFTR maintains periciliary fluid level to allow for
mucociliary clearance
Diagnosis of CF
Sweat chloride test
• Considered the “gold standard” for diagnosing
CF
• Measures the amount of chloride in sweat
• Results
– < 30 mmol/L = negative result
– 30-59 mmol/L = borderline result if < 6 months old
– 40-59 mmol/L = borderline result if > 6 months old
– ≥ 60 mmol/L = consistent with CF
Clinical Characteristics
• Classic cystic fibrosis is characterized by chronic
bacterial infection of the airways and sinuses, fat
maldigestion due to pancreatic exocrine insufficiency,
infertility in males due to obstructive azoospermia, and
elevated concentrations of chloride in sweat.
Pathophysiology of CF:
respiratory system
Abnormal CFTR
Ø Reduced airway surface liquid Ø Impaired mucociliary clearance
Objectives for planning CF venous access
•
Plan and book the line prior to admission: Elective /
semi elective lines are ideally planned a week ahead
•
Avoid emergency list.Elective / semi elective tune up
lines are better not done on an emergency list, as
these are associated with prolonged fasting,
unknown timing of access and out of hours
insertions. This causes undue stress and uncertainty
around the admission (and when the antibiotics can
start) for the child and parents.
Objectives for line choice for CF tune-ups
•
Appropriate line choice: Line is carefully chosen to
ensure it lasts the duration of the tune up, is suitable
for patient’s age and weight, thus reducing
complications such as thrombosis and infections.
•
Aim for first line to be the definitive line: Aim for the
definitive IV access which will last the duration of the
tune up to be inserted within hours of admission by an
experienced inserter.
•
Experienced line inserters only: CF patients are
recurrent patients requiring multiple IV access over a
lifetime.
Line choice considerations (1)
•
Duration of the treatment(10-14 days): Usually any IV therapy
lasting >7 days would indicate a CVAD (CVC or PICC) as the
preferred device for antibiotic therapy <30 days.
•
With the increasinguse of midline worldwide for antibiotictherapy
<30 days, mideline catheterrepresent an alternative to PICC if some
criteria are met
•
Infusate: Most CF antibioticscan be dilutedto strength suitable for
peripheral IV infusion ifrequired, however10-14 days of antibiotics
can be veno-irritant, and therefore central venousaccess, or access
in the high flow basilicor brachial veinsof the upperarm is
preferred to ensure vein preservationfor life
Line choice considerations (2)
•
Vein status: if arm veins are small, thrombosed, or have
extensive collateral development, a plan for a permanent
tunnelled access such as a port may be appropriate.
•
Frequency of tune ups: If respiratory disease is more
advanced, requiring tune ups every month or so,
permanent access such as a port may be appropriate.
•
Age: PICCS in children <2yo are difficult and a
percutaneous CVC is often a more practical solution. If a
child requires a general anaesthetic for access, a secure line
such as a PICC is preferred. Once <8yo the child will look
after the line better, a midline may suffice.
PICC or midline in CF?
•
There has been no research to date that has
compared rates of adverse events for PICCS
and midlines in CF patients.
•
Limited research has examined the devices
individually but focus on PICCs in paediatric
populations, making generalising to adult
populations problematic
PICC or midline in CF?
•
No direct comparison but only one study was identified
that examined adverse events in adult CF patients with
PICCs
This study centred on deep vein thrombosis (DVT) rates
and found a relatively high rate of symptomatic DVT (8%).
The authors surmised that the risk for DVTs in the CF
population is heightened due to an inflammatory
response involved with lung infection as well as an
increased risk of an inherited thrombotic abnormality
Incidence of Deep Vein Thrombosis Associated with
Peripherally Inserted Central Catheters in Adults with
Cystic Fibrosis
•
Twelve of the 147 subjects (8.2%) developed
symptomatic PICC-related DVT proven by
duplex US. Five additional subjects were
diagnosed with asymptomatic PICC-related
DVT diagnosed by contrast venography at
subsequent PICC insertion
•
A significantly greater proportion
of BCC-positive (Burkolderia Cepacia) subjects
developed PICC-related DVT (20.9%) compared
with BCC-negative subjects (7.7%; P .02).
•
The median time to diagnosis of PICC-related
DVT was 16 days (range, 1–65 d). There was no
significant difference between the number of
PICCs inserted in subjects who developed
PICC-related DVT
PICC or midline in CF?
•
Conversely, research in midlines in the adult CF
population demonstrated low rates of adverse
events including DVT
This study, which was based in the same hospital as
the present study, found no examples of either DVT
or catheter-related blood stream infection (CR-BSI)
during the study period and a rate of 2% and 1%,
respectively in the following 6 months
The safety and efficacy of midlinescompared to peripherally inserted
central catheters for adult cysticfibrosis patients: A retrospective,
observational study
Published studies regarding peripherally-inserted
central venous catheters in children.
Quality Improvement Initiative to Reduce Deep Vein
Thrombosis Associated with Peripherally Inserted Central
Catheters in Adults with Cystic Fibrosis
Mermis et al. 2014
Objectives: To assessed potential risk factors for symptomatic
PICC-associated DVT with subsequent implementation of a quality
improvement (QI) initiative to reduce PICC-associated DVT in
Results
1. Patients with CF are atincreased risk for development of PICCassociated DVT, and
suggest that use of QI efforts focused on judicious PICC use and insertion of
smaller-diameter catheters can reduce PICC-associated DVT. The authors found that PICC size is
a significant risk factor for DVT is consistent with studies examining risk in general
medical patients.
2. To reduce risk of PICC-associated DVT in patients with CF, QI strategies should focus on
insertion of smallerdiameter 4F PICCs and reduction in PICC use in high-risk patients
when possible.
3. The QI initiative resulted in an absolute risk reduction in DVT per PICC placed of 6.1% (P
= 0.055).
MIDLINE IN FIBROSI CISTICA
Esperienza dell’Ospedale San Carlo Potenza
•
Convergenza tra l’intuizione del Responsabile Medico del Centro per la Fibrosi
cistica dell’U.O. di Pediatria dell’ A.O.R. e la disponibilità del team che si occupa
degli impianti venosi, dell’U.O. di Terapia antalgica e cure palliative
•
Dopo le prime esperienze, la decisione di impiantare Midline ai pazienti del
Centro, ricoverati o in ambulatorio o DH, è diventata routine, peraltro richiesta dai
pazienti
•
La scelta del Midline rispetto al PICC è scaturita dai dati di letteratura e dalla
maggiore facilità e rapidità di impianto bedside, dal minore impatto emotivo per i
pazienti già provati, dalla congruità con i trattamenti endovenosi comunemente
utilizzati.
MIDLINE IN FIBROSI CISTICA
Esperienza dell’Ospedale San Carlo Potenza
MODELLO ORGANIZZATIVO
2. RICHIESTA AL
VASCULAR
TEAM
3. PRESA IN CARICO DELLA RICHIESTA E
POSIZIONAMENTO A LETTO DEL PAZIENTE
IN COLLABORAZIONE CON GLI
INFERMIERI DELLA PEDIATRIA,
NELLE ORE SUCCESSIVE AL RICOVERO
4. GESTIONE DEL DEVICE DA PARTE
DEL PERSONALE INFERMIERISTICO,
ADDESTRATO, FINO ALLA DIMISSIONE
1. PROPOSTA AL PAZIENTE
(accoglienza 100%)
MIDLINE IN FIBROSI CISTICA
Esperienza dell’Ospedale San Carlo Potenza
MIDLINE IN FIBROSI CISTICA
Esperienza dell’Ospedale San Carlo Potenza
TESTIMONIANZE
1. COSA EVETE PENSATO QUANDO VI HANNO PROPOSTO PER LA PRIMA VOLTA LA POSSIBILITA’ DI POTER POSIZIONARE UN MIDLINE ?
SINCERAMENTE QUANDO I MEDICI DEL MIO CENTRO MI HANNO PROPOSTO PER LA PRIMA VOLTA DI
POSIZIONARE IL MIDLINE, L’IDEA MI SPAVENTAVA, SOPRATTUTTO PERCHE’ ERA UNA PROCEDURA CHE NON CONOSCEVO. AVEVO CAPITO SOLO CHE SI TRATTAVA DI UN AGO “PARTICOLARE” POSIZIONATO PIU’ IN PROFONDITA’ E CHE, A DETTA DEI MIEI MEDICI, AVREBBE RESO PIU’ AGEVOLI LE MIE TERAPIE
ENDOVENOSE. COSI’ UN PO’ PERPLESSA E
IMPAURITA, HO COMUNQUE DECISO DI FARMELO POSIZIONARE
HO CONSIDERATO SIN DA SUBITO LA
PROPOSTA DI METTERE IL MIDLINE COME UN’OTTIMA ALTERNATIVA ALLE TANTE AGO CANNULE CHE DURANTE LA DEGENZA DOBBIAMO SOSTITUIRE , CON LA PAURA DI NON RIUSCIRE A “BECCARE” LA VENA GIUSTA. OVVIAMENTE AVEVO UN PO’ DI PAURA, NON SAPENDO IN CHE MODO SI POSIZIONASSE. PERO’ METTERLO NE E’ VALSA DAVVERO LA PENA .
MIDLINE IN FIBROSI CISTICA
Esperienza dell’Ospedale San Carlo Potenza
TESTIMONIANZE
2. AVER POSIZIONATO UN MIDLINE VI HA FATTO SENTIRE PIU’ AMMALATE?
IL POSIZIONAMENTO NON MI HA FATTO SENTIRE ASSOLUTAMENTE PIU’ MALATA ANZI, GRAZIE AL MIDLINE, HO POTUTO VIVERE LE MIE TERAPIE ENDOVENOSE CON PIU’ NATURALEZZA E
TRANQUILLITA’ SENZA PREOCCUPARMI DEI
MOVIMENTI DEL BRACCIO E DELLA POSSIBILITA’ DI FAR USCIRE L’AGO FUORI VENA VIVENDO LA MIA QUOTIDIANITA’ SENZA DOVER RINUNCIARE A NULLA
OVVIAMENTE NON FA ALCUNA DIFFERENZA DA UNA SEMPLICE
AGOCANNULA. C’E’ IL VANTAGGIO DI POTERLO TENERE PIU’ TEMPO PER TERAPIE PIU’ PROLUNGATE, SENZA
ALCUN DISAGIO FISICO NE’ PSICOLOGICO
DANIELA
MIDLINE IN FIBROSI CISTICA
Esperienza dell’Ospedale San Carlo Potenza
TESTIMONIANZE
3. AVETE AVUTO DISAGIO FISICO O AVETE VISSUTO CON ANSIA LA PROCEDURA DI POSIZIONAMENTO?
QUANDO ME LO HANNO POSIZIONATO PER LA PRIMA VOLTA, SONO ARRIVATA CON UNA CERTA DOSE
D’ANSIA PERCHE’ NON CONOSCEVO LA PROCEDURA ( E SI SA, LE COSE CHE NON SI CONOSCONO BENE CREANO UN PO’ DI ANSIA), MA LA BRAVURA DEI MEDICI NEL TRASMETTERMI TRANQULLITA’ , OLTRE CHE LA LORO COMPETENZA NEL POSIZIONARLO, MI HANNO FATTO SUPERARE OGNI COSA E
SINCERAMENTE HO PROVATO SOLO UN PO’ DI INIZIALE FASTIDIO MA NESSUN DOLORE FISICO
L’ANSIA MAGGIORE E’ STATA
SICURAMENTE LA PREPARAZIONE DI TUTTO “IL PIANO OPERATORIO” E IN MINIMA PARTE LA PAURA DEL DOLORE PER L’INSERIMENTO DI UN TUBICINO IN UNA VENA
DANIELA
MIDLINE IN FIBROSI CISTICA
Esperienza dell’Ospedale San Carlo Potenza
TESTIMONIANZE
4. QUAL’E’ IL VOSTRO GIUDIZIO FINALE SULLA POSSIBILITA’ DI POSIZIONARE E UTILIZZARE UN MIDLINE NEL CORSO DEL RICOVERO ?
ALLA LUCE DI TUTTO CIO’, RITENGO CHE IL MIDLINE SIA UNA TROVATA OTTIMA PER TUTTI NOI PAZIENTI COSTRETTI A FARE TERAPIE ENDOVENA RICORRENTI, PERCHE’ PERMETTE DI CONTINUARE A SVOLGERE LA PROPRIA VITA QUOTIDIANA, I PROPRI MOVIMENTI, SENZA PREOCCUPARSI ASSOLUTAMENTE DI AVERE “UN AGO IN VENA”. INSOMMA IL MIDLINE PER ME E’ UN OTTIMO DISPOSITIVO CHE RIESCE A FAR SUPERARE IN PARTE LA DIFFICOLTA’ DELLE TERAPIE ENDOVENA
GIUDICO POSITIVO L’USO DEL MIDLINE. CONSIGLIO VIVAMENTE A TUTTI I RAGAZZI DI CONSIDERARE QUESTA ALTERNATIVA CHE SICURAMENTE “SALVA” UN PO’ LE NOSTRE “VENUZZE”
