| Abstract | Cistična fibroza nasljedna je, autosomno recesivna bolest koja zahvaća brojne organe i
organske sustave.
Osnovno obilježje cistične fibroze jest stvaranje gustog, ljepljivog sekreta na svim mjestima
gdje ima žlijezda s vanjskim izlučivanjem, a to je najviše izraženo u dišnim putovima,
gušterači, crijevu, žučnom sustavu, reproduktivnom sustavu i znojnim žlijezdama.
Bolest je progresivnog tijeka i zahvaća praktički sve organe u tijelu, a može dovesti i do
značajnog skraćenja životnog vijeka. Danas bolesnici doživljavaju drugo i treće desetljeće, a
životni vijek određen je kroničnom plućnom bolesti. Zahvaljujući uvođenju cijelog niza
intervencija u samom tijeku bolesti, posljednjih desetljeća postignut je veliki napredak u
produljenju životnog vijeka bolesnika tako da je danas prosječni život vijek bolesnika 41
godina prema podacima europskog i američkog registra. Prije uvođenja metoda suvremenog
liječenja glavna klinička manifestacija cistične fibroze očitovala se preko poremećaja u
probavnom traktu. Razvijanjem različitih terapijskih pristupa navedeni problemi koji se tiču
probavnog puta s vremenom su postali kontrolirani, a mjesto glavnog vodećeg kliničkog
problema preuzima progresivni gubitak plućne funkcije, pogoršanje nutritivnog statusa uz
problem adherencije prema lijekovima. Dugoročnim sustavnim praćenjem bolesnika
primjećene su visoke korelacije stupnja pothranjenosti s mršavljenjem i padom plućne
funkcije. Mršavljenje i pothranjenost značajni su prediktori dužine života neovisno o plućnoj
funkciji.
U ovom radu prikazana je sva kompleksnost skrbi bolesnika sa cističnom fibrozom sa
naglaskom sa nutritivni status bolesnika i njegov utjecaj na plućne manifestacije bolesti, kao i
njihov utjecaj na anksioznost, depresiju i adherenciju kod bolesnika s CF. Zbrinjavanje
bolesnika nije moguće bez multidisciplinarnoga tima u kojem medicinska sestra ima izuzetno
važnu ulogu. |
| Abstract (english) | Cystic fibrosis is a hereditary, autosomal recessive disease that affects many organs and
organic systems.
The main feature of cystic fibrosis is the formation of a thick, sticky secretion in all sites
where the gland has external excretion, most notably in the airways, the pancreas, the
intestine, the gall bladder, the reproductive system, and the sweat glands.
Disease is a progressive course and affects virtually all organs in the body, and can lead to
significant shortening of life span. Today, patients experience the second and third decades,
and life expectancy is determined by chronic lung disease. Today, patients experience the
second and third decades, and life expectancy is determined by chronic lung disease. Thanks
to the introduction of a whole series of interventions in the course of the disease, significant
progress has been made in the last decades in prolonging the lifespan of patients, so that the
average life expectancy for patients is 41 years, according to European and American records.
Prior to the introduction of modern treatment methods, the main clinical manifestation of
cystic fibrosis manifested through disorders in the digestive tract. By developing different
therapeutic approaches, the problems related to the digestive tract over time have become
controlled, and the site of the leading clinical problem takes over the progressive loss of lung
function, deteriorating nutritional status with the drug adherence problem. Long-term
systemic monitoring of patients showed high correlations in the degree of malnutrition with
weight loss and pulmonary function decrease. Weight loss and malnutrition are significant
lifelong predictors independent of pulmonary function.
This paper presents all the complexity of the care of patients with cystic fibrosis with
emphasis on the nutritional status of the patient and its effect on lung manifestations of the
disease as well as their influence on anxiety, depression and adhesion in patients with CF.
Disposal of a patient is not possible without a multidisciplinary team where a nurse has an
extremely important role. |
