Pohju, Anne, University of Helsinki, Faculty of Medicine, Doctoral Program in Clinical Research, Helsingin yliopisto, lääketieteellinen tiedekunta, Kliininen tohtoriohjelma, Helsingfors universitet, medicinska fakulteten, Doktorandprogrammet i klinisk forskning, Pihlajamäki, Jussi, Sipponen, Taina, and Pakarinen, Mikko
Severe reduction of bowel function, such that long-term parenteral support (PS) is necessary to maintain health, defines chronic intestinal failure (CIF). This rare gastroenterological condition covers a wide range of underlying diseases; thus, its onset as well as course vary greatly. The multidisciplinary treatment of CIF includes providing PS, dietary therapy, and medical and surgical interventions. Despite specialised multidisciplinary team taking care of CIF patients, difficult complications may arise from CIF and long-term PS. Overall, the CIF treatment is burdensome and expensive, both to the patient and the health care system. Data regarding CIF in Finnish adults has so far been limited. This study, Treatment of Adult Patients with Intestinal Failure in Finland (TAPIFF), aimed to fill this gap in knowledge. The specific objectives of the TAPIFF study were I) to evaluate current management of long-term PS across Finland; II a) to investigate the intestinal failure (IF) prevalence among Finnish adults; II b) to describe clinical details of Finnish adults with IF; and III) to investigate catheter-related bloodstream infection (CRBSI) rate, longitudinal changes in biochemical liver and kidney tests, and their explanatory factors. As part of the TAPIFF study, we also gathered an institutional cohort of adult IF patients in Helsinki University Hospital to especially investigate liver status with non-invasive methods. In September 2017, all Finnish health care units with the potential of longterm PS provision to adult IF patients received an electronic survey investigating the local practices of PS management. The survey also inquired whether the unit had provided long-term PS to any adult(s) during the preceding year. All units that responded they had managed at least one such patient, as well as those units which did not respond to the survey, were recontacted. Patient records of the enrolled patients were obtained. The inclusion criteria were age at least 18 years, PS duration at least 120 consecutive days, IF as the indication for PS, and the availability of patient records. Clinical data was manually collected from the hospital patient records from the start of PS (baseline) up to the latest record entry in 2017 (data collection). The statistical software was IBM SPSS statistics versions 24, 25 and 27 (IBM Corp., Armonk, NY). In study I, 71 health care professionals from 52 different units responded to the survey, resulting in an overall response rate of 47% of the invited units. The responses revealed that three out of four units had some experience in managing long-term PS. This experience was, however, very limited, because most units managed currently only 0–2 patients. Hospital-at-home services had primary responsibility for the practical administration of PS, as well as for the supply of PS equipment and admixtures. In most units, assigned teams managing patients on PS, and written protocols on the practical PS management were lacking. Study II identified 52 adult patients who fulfilled the inclusion criteria. The calculated IF prevalence in adults in 2017 was 11.7 per million (95% confidence interval 8.9–15.3). Most patients in this national cross-sectional cohort were women (69%), and the median age was 62 years (interquartile range, IQR 45– 72). Short bowel syndrome was the most frequent (73%) pathophysiological mechanism. The most frequent underlying conditions, in order of prevalence, were surgical complications, Crohn’s disease, and mesenteric ischaemia. The median duration of PS was 27.5 (IQR 11.3–57.3) months. Patients received a median of 7 (IQR 3.5–7) parenteral infusions per week with a median weekly volume of 7.3 (IQR 4.4–14) litres and a median weekly energy supply of 6100 (IQR 3900–9800) kcal. The daily volume of parenteral nutrition was 2 litres or less in 66% patients, and 15% received fluids and electrolytes only. Ten patients (19%) ceased PS treatment during 2017 after a median PS duration of 20.0 (IQR 9.0–40.3) months. Of these ten, eight were successfully weaned from PS, one lost venous access sites, and one died. The retrospective study III on the national cohort of 52 patients indicated a CRBSI rate of 1.35 per 1000 catheter days. In long-term catheters, CRBSI led to catheter removal in 73% of cases. A statistically significant median change occurred both in estimated glomerular filtration rate (eGFR; -8.5 ml/min/1.73 m2, IQR -30–7, p=0.005), and in alkaline phosphatase (26 U/l, IQR -11–95, p=0.019) during a median PS treatment time of 27.5 (IQR 11.3–57.3) months. In a multiple regression model for eGFR at data collection, strong explanatory variables were age and baseline eGFR. In conclusion, the prevalence of IF in the Finnish adult population, as well as their clinical characteristics, are in line with reports from other Western countries. Over time, as the PS continues, abnormal biochemical liver tests and decreased kidney function become more frequent findings. The incidence of CRBSI in the Finnish adult IF population well represents a rate described in nonspecialised units. The experience of clinicians managing Finnish patients on long-term PS appears limited, and, on national level, the management of these patients seems fragmented. Suolen vaikea vajaatoiminta (SVV) on harvinainen ryhmä sairauksia, joita yhdistää riittämätön nesteen ja ravinnon imeytyminen suolesta. Ravitsemustilan ja terveyden ylläpitämiseksi SVV:aa sairastava tarvitsee pysyvän keskuslaskimokatetrin kautta annettavaa suonensisäistä ravitsemushoitoa (SR). Tutkittu tieto SVV:sta suomalaisilla aikuisilla on vähäistä, mikä johti Suolen vaikeaa vajaatoimintaa sairastavien potilaiden hoito Suomessa -tutkimuksen käynnistämiseen. Terveydenhuollon yksiköille suunnatun kyselyn avulla löysimme 52 vähintään 18-vuotiasta suomalaista, jotka olivat saaneet SVV:n vuoksi SR:a vähintään 120 vrk ajan. Näin ollen SVV:n vallitsevuus Suomessa vuonna 2017 oli 11,7 tapausta miljoonaa aikuista kohti. Suurin osa näistä potilaista oli naisia, he olivat keskimäärin 62-vuotiaita ja saaneet SR:a päivittäin keskimäärin 28 kk ajan. Yleisimmät syyt SVV:n taustalla olivat kirurgiset komplikaatiot, Crohnin tauti ja suoliston verenkiertohäiriöt, ja mekanismina näiden pohjalta kehittynyt lyhytsuolioireyhtymä. Tässä kansallisessa aineistossa keskuslaskimokatetriperäisten veriviljelypositiivisten infektioiden ilmaantuvuus oli 1,35 infektiota 1000 katetripäivää kohti. Pysyvissä keskuslaskimokatetreissa todetuista infektioista 73 % johti katetrin poistoon. Pitkäaikaisen SR:n aikana potilaiden munuaistoiminta verikokein määritettynä heikkeni tilastollisesti merkitsevästi, kun taas maksa-arvoista alkalisen fosfataasin pitoisuus kasvoi. Kyselyymme pitkäaikaisen SR:n käytännöistä vastasi 71 terveydenhuollon ammattilaista 52 yksiköstä kattavasti ympäri Suomen. Kokemus pitkäaikaisen SR:n toteutuksesta oli rajallista, sillä suurin osa yksiköistä oli ajankohtaisesti vastuussa korkeintaan kahden SVV-potilaan hoidosta. Suurimmassa osassa yksiköitä ei vastausten perusteella ollut nimettyä ryhmää, joka vastaisi SVV-potilaiden hoidosta, eikä kirjallista ohjetta SR:n toteutuksesta. Tutkimuksen johtopäätökset ovat, että SVV:n vallitsevuus suomalaisessa aikuisväestössä, kuten myös potilasryhmän kliiniset piirteet, pitkittäismuutokset maksan tilaa ja munuaistoimintaa kuvaavissa verikokeissa sekä katetriperäisten infektioiden ilmaantuvuus ovat samankaltaisia kuin muista länsimaista raportoidut tulokset. Suomalaisten terveydenhuollon ammattilaisten kokemus pitkäaikaisesta SR:sta vaikuttaa vähäiseltä. Kansallisella tasolla SVV-potilaiden hoito kuvautuu hajanaisena.