Other symptoms, such as psychiatric manifestations, cerebellar ataxia, involuntary muscle contraction, neuropathy, heart disease, short stature and diabetes mellitus have also been observed in patients with this syndrome 2. The disease is rare and has a prevalence of 0. It presents rapid and aggressive progression 3 and the average life expectancy observed is around During the course of the disease, muscular and neurological alterations can impact the functions of the stomatognathic system, in particular, swallowing. Speech-Language Therapy SLT responsibility lies in the management of swallowing, and in these cases, the importance of an approach based on palliative care principles is emphasized 5 , since this function is not only a means of physical or biological maintenance, but also an important form of socialization and pleasure.
According to the World Health Organization WHO , palliative care encompasses the promotion of the quality of life of patients and family members with life-threatening diseases. Therefore, the promotion of quality of life mainly includes prevention and relief of the individual's suffering 5. Being a disease of progressive character with an unfavorable diagnosis, it is imperative to undertake a palliative care approach from diagnosis, in order to provide a better quality of life and relief from suffering.
To date, there are few published studies on swallowing, dysphagia, and the decision-making process on the feeding pathway in patients with MELAS syndrome. Therefore, this article aimed to describe the progression of dysphagia and the feeding pathway decision in a case of MELAS syndrome, under the perspective of palliative care. The legal guardian for the patient signed the Terms of Free and Informed Consent.
Wallenberg Syndrome: Stroke Affects Swallowing
We describe the case of a female patient with MELAS syndrome, 34 years old, Brazilian, single and without children, who at age 26 was diagnosed with progressive mitochondrial encephalopathy, MELAS syndrome. Eight years after the onset of symptoms, the patient had motor impairment, non-walking and showing generalized weakness in the skeletal muscles, in addition to daytime sleepiness, flaccid dysarthria and difficulty in swallowing. At age 34, the patient was referred by the neurologist to evaluate swallowing and SLT follow-up.
SLT follow-up was initiated based on a swallowing safety assessment protocol 6 , the use of compensatory maneuvers to protect the airways and cleanse of stasis after swallowing, changes in diet consistency and control volume by swallowing. The visits were given at the outpatient level with weekly and monthly frequency. Figure 1.
- Stop It!!
- What Can I Play on Sunday?, Book 4: July & August Services: 10 Easily Prepared Piano Arrangements (Sacred Performer Collections).
- ARTICLE NAVIGATION!
- Children – congenital or inherited conditions.
- Muscle disorders affecting oral and pharyngeal swallowing : GI Motility online.
- Peaches n Cream [Loving in Silver 1] (Siren Publishing Menage Amour)!
At age 32, the patient no longer fed on solid consistency. Feeding was spontaneously reduced to pasty, due to the difficulty of chewing and oral preparation of solid foods. At 34 years of age, the patient had gagged saliva and had been referred for SLT follow-up after the introduction of a nasoenteral tube NET for alternative feeding during a hospital stay.
The patient attended the first outpatient SLT evaluation, moving in a wheelchair, drowsy, communicating in isolated words and responding only to simple verbal commands. According to the caregiver, the patient had removed the NET on her own accord the previous day and was brought to the medical service to re-establish the alternative feeding pathway.
When asked about the patient's previous history, the caregiver first referred primarily to diarrhea after the introduction of the enteral diet and the need to keep it contained in the bed, so as not to withdraw the NET. The patient then complained of the discomfort caused by the tube.
At this time, the patient was at level 1 of FOIS, which represents the exclusive tube feeding. During the evaluation, cervical control, nasal breathing and efficient lip seal were observed. Absence of salivary sialorrhea or stasis in oral cavity was observed. At rest, the patient presented a contained jaw, non-protruding tongue posture and negative cervical auscultation. The absence of voluntary swallowing of saliva was observed. The patient's communication was restricted to word vocalizations and small phrases in low intensity, with flaccid dysarthria, characterized by hypernasal, imprecise and pasty speech articulation.
- Leading Beyond Your Own Style.
- Stephen Spender: A Life in Modernism!
- Search A-Z.
- The Upside of the Down Low, a Pastor’s Wife’s Memoir.
- SAT High Score Grammar Guide (2013) - 19 Grammar Rules You Must Know To Get A High Score On The SAT Test!.
- What is Pediatric Dysphagia (Swallowing Disorder)?.
In the structural evaluation, complete dentition and absence of occlusal alteration were observed. The isolated movements of the lips, tongue, cheeks and jaw were minimal and showed imprecision, low amplitude and reduced muscle strength. The results obtained are summarized in Figure 2. After the evaluation, it was concluded that the patient had a functional diagnosis of moderate oropharyngeal dysphagia and FOIS level 5.
Moderate oropharyngeal dysphagia predicts the risk of aspiration. At FOIS level 5, oral feeding is recommended in more than one consistency; however, there is a need for special compensation or preparation. Based on the principles of palliative care 5 , the suggestion was to withdraw the NET, as well as to guide oral feeding in the honey, nectar and liquid consistencies. The caregiver was instructed to offer oral feeding, with volume and speed control, namely, offering small sips, with a longer time between them, especially for liquid.
It was advised to avoid pudding consistency due to extremely slow oral transit. The patient was kept under ambulatory monitoring of swallowing. Due to cognitive difficulty, weakness and muscle fatigue, it was decided not to perform exercises or techniques that required greater effort than swallowing itself.
The patient returned, maintaining the clinical profile previously described and oral feeding. The patient's caregiver reported a few incidents of choking with water throughout the week. The consistencies oriented to oral feeding after the first evaluation were re-evaluated and the results obtained are presented in Figure 3. After this re-evaluation, the same levels of dysphagia severity and oral ingestion levels were attributed.
The same orientations as the previous week were maintained. In addition, it was advised that the caregiver would allow dry swallows before offering a new supply of liquid or food.
The patient was referred for evaluation and nutritional management since there was no nutritional follow-up initiated at the time of the enteral diet introduction. After a month, the patient returned, maintaining the initial clinical profile and the caregiver reported gagging only with macerated medications.
Pediatric Dysphagia (Swallowing Disorder)
Regarding diet, there was no reduction in the level of oral intake, or complaints of gagging. Based on the principles of palliative care 5 , it was advised to offer a soft and kneaded solid, specifically rice and beans, according to the patient's request. Because it was an unsafe consistency, due to the difficulty in the preparatory and oral stages of swallowing, the caregiver was instructed to offer the food in small quantity, only when the patient wished, to be fully alert and seated at 90 degrees.
The liquid consistency was maintained under the aforementioned conditions and directed to the ingestion of macerated tablets with the aid of honey consistency. After this re-evaluation, the levels of dysphagia severity and oral ingestion levels were also maintained. Over the next two months, the patient returned for functional reassessment and swallowing management and was visibly more debilitated, still in wheelchair, drowsy, with longer latency for responses to simple verbal commands and less communicative, as well as remaining with the eyes closed throughout all of the service.
The consistency with lower risk of aspiration, according to the clinical evaluation, was the honey consistency. In addition, it was observed that the patient performed several incomplete swallows until was able to swallow the bolus. As a course of treatment, it was directed to the caregiver to offer only the honey consistency. Lastly, moderate to severe oropharyngeal dysphagia and FOIS level 4 were found.
Indications for a study
In moderate to severe oropharyngeal dysphagia, there is tolerance of only one consistency, with maximum assistance for the use of strategies. There are signs of aspiration of two or more consistencies, requiring multiple clearing requests, absence of reflex cough, weak and ineffective voluntary cough. If the patient's pulmonary status is impaired, it is necessary to suspend oral feeding 8. At FOIS level 4, oral feeding is complete but recommended in only one consistency. It is noteworthy that pulmonary signs and symptoms were monitored throughout the SLT follow-up and there were no episodes of pneumonia or other pulmonary complications during this period.
It was decided not to submit the patient to videofluoroscopic swallowing study, due to the risk of barium bronchoaspiration, clearly observed during the clinical evaluation. In addition, confirmation of aspiration, or the hypothesis of pathophysiological changes evidenced in the clinical evaluation, would not change the approach adopted. A conversation was made with the patient and the family about the feeding pathways, their benefits and harms, and then performed a new counter-reference to the neurologist. Continuing to be based on the principles of palliative care 5 , the patient remained without reintroduction of NET, with an oral diet oriented towards honey consistency, for two months 4 th and 5 th months of management , when at the 6th month of follow-up, gastrostomy surgery was performed.
The option for gastrostomy was made by the family, since the patient was considered legally incapable. After gastrostomy, the patient did not return to dysphagia outpatient and contact was lost with the patient's network of care. The present article proposed the discussion of the evolution of dysphagia and the feeding decision in the case of a year-old female patient with MELAS syndrome, during six months of SLT follow-up. No publications of longitudinal studies were found that described the evolution of dysphagia in the disease, or that objectively investigated participants' swallowing.
A case study of an year-old female patient with MELAS syndrome described the loss of functional swallowing clinically evaluated since the age of 7 years. With initial impact to the solid consistency and pasty feeding since 7 years of age, it evolved with feeding restricted to liquid and liquid thickened. Four years after the onset of swallowing changes, there was difficulty in opening the mouth, absence of suction and rotational movements of the mandible. As in the present case, the reduction of the volume of the bolus by swallowing was an efficient compensatory maneuver to reduce the clinical signs of laryngotracheal aspiration.
There was significant weight loss for the period of one year The initial swallowing difficulty of the present case was related to the solid consistency. Similar to solid, the ejection of the pasty bolus also demands greater muscle strength than the less thick consistencies. As in the case under discussion, slowed oral transit was also observed in the previously published case study, which may be explained by weakness in the lips, tongue and mandible musculature for the preparation of the bolus, which consequently impairs the conduction of the bolus to the esophagus, raising the risk of pharyngeal residue and laryngotracheal aspiration Electromyography and video fluoroscopy test results show no abnormalities.
What is phagophobia? The case of Mr. C brings to light the condition known as phagophobia —a sensation of not being able to swallow. Phagophobia mimics oral apraxia; pharyngoesophageal and neurologic functions as well as the ability to speak remain intact, however. A number of psychiatric conditions, including panic disorder, obsessive-compulsive disorder, social phobia, anorexia nervosa, globus hystericus, hypersensitive gag reflex, and posttraumatic stress disorder can simulate this condition. When Barofsky and Fontaine 4 compared phagophobia patients with other subjects—healthy controls, anorexia nervosa restrictors, dysphagic patients with esophageal obstruction, dysphagic patients with motility disturbance, and patients with non-motility non-obstructive dysphagia—they found that patients with psychogenic dysphagia did not appear to have an eating disorder.
However, they did have a clinically significant level of psychological distress, particularly anxiety. Non-invasive assessment tools along with educational modalities usually are tried alone or together with psychopharmacological intervention. It is, however, imperative that you have an empathetic and understanding approach to such patients.