HOW THE SPEECH-LANGUAGE PATHOLOGIST INTERACTS WITH CYSTIC FIBROSIS PATIENTS? A SCOPING REVIEW

Objective: The review describes the changes related to sleep-breathing disorders, communication and feeding processes in subjects with CF, through a scoping review. Meth-ods: A scoping review based on the PRISMA protocol was carried out in Pubmed, Lilacs and Scopus databases, to investigate the possibility of including SLP in the treatment of patients with CF. The inclusion criteria included studies that addressed communication and eating processes and disorders related to CF. Studies with a sample that presented other comorbidities that would justify the worsening of the condition, or secondary studies, were excluded. There was no time or language delimitation. Results: The search found 1566 works, 27 Lilacs, 1009 Pubmed and 530 Scopus, by analysis of titles, abstract and reading in full. A total of 30 articles were selected for inclusion, 2 Lilacs, 22 Pubmed and 6 Scopus, all related to Speech-Language Pathology. Conclusion: It was observed that there is a poor sleep quality due to nocturnal desaturation, mild and moderate


INTRODUCTION
Cystic Fibrosis (CF) is a genetic autosomal recessive progressive disorder of the Transmembrane Conductance Regulator (CFTR) gene due to the deficiency, dysfunction or absence of its protein (Morales-Múnera, Rosero-Ascuntar et al., 2020).There are numerous genetic alterations related to CF, but the most common significantly reduces the excretion of chlorine in the human body, so there is an increase in intracellular electronegativity.To try to control this, the body sends a greater flow of Na (Sodium) and water to the cell.This process causes fibrosis resulting from the dehydration of mucous secretions and obstruction of the ducts (Ribeiro, Ribeiro et al., 2002).
In Brazil, the incidence rate of the disease is 1:7 in 576 live births (Athanazio, Silva et al., 2017).Symptoms depend on the affected area, lung disease occurs in more than 90% of the cases, with respiratory symptoms being the most common, such as chronic cough, pneumonia and recurrent bronchitis (Spicuzza, Sciuto et al., 2012).
The treatment of pulmonary disease in CF includes anti-inflammatory drugs, inhaled corticosteroids, antibiotics, mucolytics, hypertonic saline solution and physical therapy (Furlan, Ribeiro et al., 2017).Given the variety of alterations that CF can cause, multidisciplinary treatment is of paramount importance, aiming at a better quality of life and better performance in the physical and psychological aspects (Athanazio, Silva et al., 2017).
Regarding the interface with Speech-Language Pathology (SLP), it can be highlighted that CF basic condition brings interesting correlations, although still little explored.Firstly, the relation with the hearing area is highlighted, considering that the middle ear is directly linked to the respiratory tract through the Eustachian tube, as the buildup of secretions and respiratory infections can cause acute suppurative otitis media (Forman-Franco, Abramson et al., 1979).Recurrent sinusitis and tonsillitis can also occur, presenting anatomical and functional factors that justify the condition of deviated septum, respiratory difficulty, tubal dysfunction, tonsil hypertrophy, adenoids and allergies (Balbani, Sanchez et al., 1998).
Considering these respiratory changes, these patients can acquire mouth-breathing pattern, which can also be related to dental malocclusion and changes in masticatory function (Blacharsh, 1977).In addition to SLP in the adequacy of mastication, SLP focused on breathing has shown significant results, readjusting and raising awareness about respiratory training (Marson, Tessitore et al., 2012).
Still on breathing, Sleep Therapy is an area of expertise recognized by the Brazilian Sleep Association.Therefore, in addition to changes in daytime breathing, the possibility of changing conditions during sleep is highlighted, such as reduced sleep quality due to factors such as: intermittent hypoxemia and transient nocturnal hypercapnia, increased nocturnal respiratory rate and obstructive apnea of sleep (Shakkottai, O'brien et al., 2018).
Breathing is the fuel for vocal production, which can be related to CF due to reduced vocal quality, vocal loudness regardless of gender, and HNR, using acoustic analysis as a parameter to represent the signal-to-noise ratio, increases in the variation of vocal amplitude and frequencies, such as changes in jitter and shimmer, which are normally related to irregularity of vocal fold vibration and, generally, are also related to vocal harshness and the presence of noise in the vocal signal.Turbulence such as partial upper airway obstruction can cause mucus buildup and chronic cough, which are standard symptoms of respiratory pathology in CF patients (Lourenço, Costa et al., 2014).
As exposed above, the CF patient suffers affections in several areas covered by the SLP interaction.A better understanding of what is known, and which are the best therapy approaches for hearing, breathing, mastication and speech in a CF patient will improve their quality of life, reminding that the new medication protocols improved a lot their life expectancy.Thus, a scoping review was carried out to systematically map all the alterations in the cited studies.SLP is an important area in the multidisciplinary work developed for patients with CF, enabling and rehabilitating functions related to communication and nutrition, providing a better quality of life.
The objective of this study was to describe what is known regarding the alterations related to the breathing, communication and feeding processes in subjects with CF, investigating the possibility of SLP therapy, through a scoping review.

METHODS
This study is a scoping review to investigate the possibility of including SLP in the treatment of patients with CF, by searching the literature in the following databases: Pubmed, Lilacs and Scopus.The scoping review was performed using the PRISMA checklist (Tricco, Lillie et al., 2018), with the studies included in the review, and meeting the inclusion criteria and the review behavior.In this study there were no funding sources.

Inclusion and Exclusion Criteria
The inclusion criteria included studies that addressed communication and eating processes and disorders related to CF.Studies with a sample that presented other comorbidities that would justify the worsening of the condition, or secondary studies, were excluded.There was no time or language delimitation.

Data Collection Procedure
After the searches, the selection was made by reading the titles, and those that were not related to SLP were excluded.Duplicate titles were also excluded and kept only in one of the databases, in total there were 4 duplicates.The others were selected for abstract reading.Research that has not been published in article format, that were not related to SLP, did not have relevant data and literature review were excluded.
Data collection was carried out through a table containing 7 columns, with the topics: "Author, year, country", "Type of study", "Sample", "Instruments applied to measure Speech-Language alterations", "Instrument applied to diagnose CF", "Main results" and "Conclusion".

RESULTS
After the inclusion and exclusion criteria, 30 articles were selected, showing a variability of published articles on CF, but few studies related to speech-language disorders.The Decs/Mesh terms used in each base and the initial location in the bases are detailed in Table 1.
The study shows that the PSQI can be useful in detecting CF patients with poor sleep quality, and that stable CF patients have sleep disruption and this may be in part related to the severity of the lung disease.Hayes, 2014 (Hayes, Daniels et  Arquivos de Ciências da Saúde da UNIPAR, Umuarama, v.27, n.9, p. 4916-4943, 2023. ISSN 1982-114X reflux were detected by ).Doumit, 2015 (Doumit, Belessis et al., 2016) Australia Quantitative study 39 Individuals < 4 years -No report of speech therapy procedures.
-Neonatal screening -Sweat test or genetic mutation analysis.
We used 65 paired samples from 39 children evaluated, 18 of which were sampled on one occasion, 16 were sampled on two occasions, and 5 were sampled on three occasions.Oropharyngeal suction sample.
The study shows that the level of distress during oropharyngeal suction (OPS) was generally high, and assessing this distress during OPS collection in children with CF is important to reduce the feeling of distress.The study measures the treatment performed by the drug ivacaftor, and the questionnaire applied shows significant speechlanguage disorders.The study concludes that the use of the drug offers benefits to the CF population.Table caption:  There was a greater concentration of articles related to audiology and sleep, the others were from different areas, but with a small number of results.The areas found show symptoms that occur due to functional and structural changes caused by CF and medication use.
Questionnaires showed difficulty falling asleep, difficulty staying asleep, and reduced sleep efficiency.

Voice
To assess the voice quality of individuals with CF, a study used vocal assessments: Maximum phonation time, the Praat software and the GRBAS scale, showing that there is a relationship between CF and vocal alterations such as reduced voice amplitude (Lourenço, Costa et al., 2014).

Dysphagia
One study used spirometry, which made it possible to correlate dysphagia as a consequence of gastroesophageal reflux, showing the importance of a speech-language assessment for a better conduct (Sabati, Kempainen et al., 2010).
The other study used, as an evaluation method, the visualization of the mucosa with an otoscope to identify possible aspirations, in which it was possible to observe aspirations (Nelson, Karempelis et al.).

Orofacial Mobility
No test with speech-language data was used, but since suction is one of the functions of the stomatognathic system in which the SLP is responsible for enabling and rehabilitating, referral should be made (Doumit, Belessis et al., 2016).

Articles Reporting Changes Related to More than One Area of SLP
One article was found measuring the quality of life of patients with CF, in which the SNOT-20 questionnaire was used and showed results such as facial pain, ear fullness and difficulty sleeping (Mccormick, Cho et al.).
Another article relates quality of life to the incidence of gastroesophageal reflux in individuals with CF (Dziekiewicz, Banaszkiewicz et al., 2015).

DISCUSSION
In this scoping review, professionals are described in the literature as fundamental to compose the care team for patients with CF, including: pediatricians, pulmonologists, gastroenterologists, physiotherapists, nutritionists, nurses, psychologists, pharmacists and social workers (Athanazio, Silva et al., 2017).The discreet inclusion of the speechlanguage therapist in the interdisciplinary teams of care for patients with CF was observed.
SLP is responsible for the functions and structures of the stomatognathic system, so the SLP contribution in the multidisciplinary treatment of CF is important for the quality of life of these patients through the improvement of orofacial structures and functions, as well as quality of sleep and hearing.
Considering the 30 articles selected, 46.66% were from the USA (Mccormick, Cho et al.;Nelson, Karempelis et al.;Forcucci e Stark, 1972;Jankelowitz, Reid et al., 2005;Naqvi, Sotelo et al., 2008;Cheng, Johnston et al., 2009;Sabati, Kempainen et al., 2010;Hayes, Daniels et al., 2014;Lourenço, Costa et al., 2014;Garinis, Cross et al., 2017;Handelsman, Nasr et al., 2017;Kreicher, Bauschard et al., 2018;Isaiah, Daher et al., 2019)  Several types of studies were found, such as: descriptive studies to determine the distribution of diseases or conditions related to the health of the patients involved; crosssectional study to relate the time of evaluation and manifestation of the disease and its risk factors; longitudinal and prospective study to assess the cause and effects caused in patients; randomized and controlled study to collect information and test the effectiveness of therapeutic approaches in patients; non-probabilistic sequential sampling studies, in which the collection is based on previously defined criteria and quantitative and qualitative studies to gather data and measure the quality of life of patients with CF.
A total of 2,471 individuals participated in this scoping review, and only 49 of them were part of the healthy control, representing 1.98% of the total.The participating individuals were children, adolescents, adults and elderly males and females in the age Studies related to sleep cite desaturation/hypoxemia, with breathing being directly linked to sleep, showing that patients with CF have frequent complaints about the quality of their sleep due to desaturation during the night (Ramos, Salles et al., 2011).Another study carried out using Actigraphy and Polysomnography observed that patients with CF have a higher frequency of nocturnal awakenings and longer time to fall asleep, resulting in a lower quality of sleep than the group of patients without CF (Shakkottai, O'brien et al., 2018).Studies point to the need for more routine monitoring, as sleep is a factor that directly influences the quality of life.Some articles have identified Obstructive Sleep Apnea (OSA) in some patients with CF (Ramos, Salles et al., 2009;Perin, Fagondes et al., 2012;Spicuzza, Sciuto et al., 2012).Rosa (2010) (Spicuzza, Sciuto et al., 2012) shows the relationship between SLP and Obstructive Sleep Apnea.According to the author, the speech-language therapist can be inserted as early as in the diagnosis, guidelines and treatment, helping to improve the functions and structures involved in breathing during sleep.
Other articles have shown that there is a relation between hearing loss and patients with CF, which can be explained mostly by the ototoxicity caused by the frequent use of some ototoxic drugs (Forcucci e Stark, 1972;Stavroulaki, Vossinakis et al., 2002;Piltcher, Teixeira et al., 2003;Mulheran, Hyman-Taylor et al., 2006;Cheng, Johnston et al., 2009;Martins, Camargos et al., 2010;Geyer, Barreto et al., 2015;Garinis, Cross et al., 2017;Handelsman, Nasr et al., 2017;Garinis, Keefe et al., 2018;Kreicher, Bauschard et al., 2018;Vijayasingam, Frost et al., 2020).According to a study published in 2005, ototoxicity can be defined as disorders caused by drugs that compromise the auditory function and/or the peripheral vestibular system and that is characterized by a sensorineural hearing loss of more than 25 db in one or more frequencies in the range of 250 to 8000hz (Hyppolito e Oliveira, 2005).
Regarding hearing (Forcucci e Stark, 1972;Stavroulaki, Vossinakis et al., 2002;Piltcher, Teixeira et al., 2003;Mulheran, Hyman-Taylor et al., 2006;Cheng, Johnston et al., 2009;Martins, Camargos et al., 2010;Geyer, Barreto et al., 2015;Garinis, Cross et al., 2017;Handelsman, Nasr et al., 2017;Garinis, Keefe et al., 2018;Kreicher, Bauschard et al., 2018;Vijayasingam, Frost et al., 2020), the research shows that all articles present patients with some type and degree of hearing loss or some alteration in the middle ear (Forcucci e Stark, 1972).According to a study published in 2017, CF patients without hearing complaints may have some type of loss at high frequencies.This finding can be justified by the fact that these patients are subject to ototoxicity, assuming that audiometry at high frequencies and otoacoustic emissions can be an adequate method for diagnosis and follow-up of CF patients at risk of ototoxicity (Caumo, Geyer et al., 2017).The results of the studies showed that there are significant alterations in the field of SLP in patients with CF.Studies on hearing evidence the need for a more rigorous audiological followup in patients with CF (Forcucci e Stark, 1972;Stavroulaki, Vossinakis et al., 2002;Piltcher, Teixeira et al., 2003;Mulheran, Hyman-Taylor et al., 2006;Cheng, Johnston et al., 2009;Martins, Camargos et al., 2010;Geyer, Barreto et al., 2015;Garinis, Cross et al., 2017;Handelsman, Nasr et al., 2017;Garinis, Keefe et al., 2018;Kreicher, Bauschard et al., 2018;Vijayasingam, Frost et al., 2020).A study published in 2006 focuses on the importance of audiological monitoring for patients who use ototoxic drugs.Technological advances make this monitoring possible more quickly and effectively, being able to identify hearing loss early, reducing or interrupting the damage.(Jacob, Aguiar et al., 2006).
CF is a disease that requires constant treatment and monitoring.Therefore, these patients undergo daily examinations, use of antibiotics, therapies and hospitalizations.
Most of these treatments are considered vital, as without them lifespan decreases.
However, when it comes to research, family members and guardians are reluctant to agree to the participation of these patients, as submitting them to more exams and more therapies in addition to routine ones can become stressful, which may justify the difficulty in collecting data and conducting research in several areas, especially in the speechlanguage field.Even today, SLP treatment is seen by many people as non-vital, because the lack of a SLP intervention will not generate immediate death.However, some studies confirm that by improving the quality of life of CF patients, their longevity is extended.
Based on this observation, it is important to jeopardize more randomized clinical trials, deepening the speech therapy therapeutic actions, being this the most important limitation (HELRIGLE, PEREIRA, LEMOS, 2014).
As it is a new and little explored area of activity, CF is not a pathology commonly addressed in the undergraduate courses, so many professionals do not have theoretical and practical knowledge for the best management of these patients.There is a need for the academy to build protocols of SLP evaluation, including all areas of interface, based on high rated research studies as randomized clinical trials.The speech-language therapist must be included in the multidisciplinary team for the treatment of CF, regarding a longer life expectancy with improved quality of life.

CONCLUSION
The scoping review showed the alterations related to SLP that impact the lives of Based on the data provided by this review, SLP is of paramount importance in the routine monitoring of these patients, as part of the multidisciplinary team for the diagnosis, guidance and treatment of the changes involved in hearing, sleep, swallowing, sucking, dysphagia, voice and quality of life.
F: Female, M: Male, CF: Cystic Fibrosis, PTA: Pure-Tone Audiometry, HFPTA: High Frequency Pure-Tone Audiometry, AGS: Aminoglycosides, DPOAE: Distortion Product Otoacoustic Emissions, OPS: Oropharyngeal Suction Distress, GER: Gastroesophageal reflux, TTS: Total sleep time, AI: obstructive apnea index, SDB: Sleep-disordered breathing, OSA: Obstructive sleep apnea, OSAS: Obstructive sleep apnea syndrome, AHI: Apnea-hypopnea index, PH: Pulmonary hypertension, PSQI: Pittsburgh Sleep Quality Index, ESS: Epworth Sleepiness Scale.Source: Prepared by the authors (2023) people with CF, among them, decreased sleep quality, sleep breathing disorders as obstructive sleep apnea, sensorineural hearing loss, reduced vocal range, dysphagia, difficulty in suction, facial pain and bronchoaspiration.Most of the studies were limited to the SLP evaluation, confirming an existence of worsed functions of breathing, mastication and deglutition, and on speech and voice, but unfortunately there are missing protocols for interventions.It is noteworthy that the publications in the area are still scarce, requiring further research, to enhance the strength of the effect of the intervention.

Table 1 -
Results of articles located and selected by database, using search strategies.

Table 2 -
Data on authorship, year, country, sample, evaluation method, results and conclusion.

Author, year, country Type of study Sample Instruments applied to measure speech-language alterations Instrument used to diagnose CF Main Results Conclusion
6 (32%) out of the 19 subjects with normal hearing had conductive alterations.7 (22%) out of the 31 subjects had low scores on the speech-language tests.standard PTA data sets before and after.During the research, 13 patients were removed from the study because they already had preexisting hearing loss.In the follow-up performed at 6 and 8 weeks, no audiological changes were observed.different dosages (once a day and 3 times a day) does not indicate cochleotoxicity, an effect that would cause hearing loss.However, the study does not provide a basis for the use of the drug in the long term or for repeated therapies, requiring a long-term study with routine monitoring of audiometry.
published in 1972, and from 2007 to 2019.This demonstrates a considerable incidence of CF, in addition to good investments in scientific research, which may explain the amount of research carried out in the country.According to the literature, 30,000 children and adults are affected, and this frequency is 1 in every 3,500 individuals in the USA, putting the country in 12th place according to data from the World Health Organization (De Cássia Firmida e Lopes, 2011).