Effects of a comprehensive, inpatient pulmonary rehabilitation programme in a cachectic patient with very severe COPD and chronic respiratory failure

Medical history

• 1990: right-sided pneumothorax, treated with chest tube and drainage

• 2010: left-sided pneumothorax, treated by video-assisted thoracoscopy with pleural rubbing and bullectomy

• 2010: COPD

• 2014: pulmonary hypertension, mean pulmonary artery pressure 55 mmHg, normal left ventricular function

The man had been hospitalised for exacerbations of COPD five times in the previous year. Each time, he suffered from acute-on-chronic respiratory failure, for which he was treated with noninvasive ventilation (NIV) three times. When admitted to CIRO, he experienced shortness of breath at rest and had trouble reaching the restroom on his own. He had chronic cough and sputum with purulence. Until admission, he continued to smoke six cigarettes per day and had acquired 40 pack-years. His past was characterised by extensive substance and alcohol abuse, but this was not recent. He was using long-term oxygen therapy, 2 L·min⁻¹. Besides intermittent oedema, he had no cardiac symptoms. Over the past year, he was fed by a percutaneous endoscopic gastrostomy feeding tube and he had gained approximately 10 kg in weight. His appetite was very variable. He lived alone, had a very limited social network and was deemed unfit for work. The man hardly derived any pleasures in his life.

Medication when admitted

Prednisolone 1×30 mg as maintenance therapy, azithromycin 250 mg three times per week, formoterol/budesonide 9/320 dry powder inhaler two times per day, salbutamol/ipratropium 2.5 mg four times per day, and as needed, bumetanide 1×5 mg, enalapril 1×5 mg, pantoprazole 1×40 mg.

Physical examination

A badly attended, cachectic, dyspnoeic and tachypnoeic man. Resting oxygen saturation of 86% with 2 L of oxygen therapy. Central venous pressure was not raised. Over his lungs he had very soft breathing sounds with prolonged expiration. No abnormal heart sounds. Slight pitting oedema of the lower extremities.

Pre-rehabilitation assessment

Patient characteristics before and after PR are presented in tables 1 and 2. At baseline, the man was underweight with low fat-free mass (FFM) and osteoporosis. The degree of airflow limitation was very severe, as was the degree of lung hyperinflation. Blood gas analysis on oxygen supplementation revealed severe hypercapnia and hypoxaemia. Modified Medical Research Council (mMRC) dyspnoea score was high. Exercise tolerance was very limited and he had severe muscle weakness. Health status was poor and the man had depressive symptoms.

Case summary

A relatively young man with very severe COPD with frequent exacerbations and hospitalisations and a high burden of disease (indicated by mMRC, COPD Assessment Test (CAT), Clinical COPD Questionnaire (CCQ) and St. George's Respiratory Questionnaire (SGRQ)). According to the GOLD classification he was in GOLD grade 4D. In addition, he had severe chronic hypercapnia, severe lung hyperinflation and persistent cigarette use. Also, he had class III pulmonary hypertension, very severe cachexia characterised by low body weight, low FFM and osteoporosis. Functional exercise capacity and peripheral muscle strength were minimal and he suffered from significant psychological symptoms. Finally, there was a complete lack of a social support.

Comprehensive pulmonary rehabilitation

The results of the integrated assessment of his physical and psychological condition were discussed with the patient. He received information about his prognosis and possible disease trajectory. Severity and complexity of his pulmonary condition, smoking behaviour, the systemic manifestations and comorbidities were emphasised. Motivation and urgency of behaviour change were addressed. Although the multidisciplinary team of the centre had serious doubts regarding the possibility of comprehensive PR, resulting from both the severity of disease as well as the patient's motivation, he was given the benefit of the doubt and PR was initiated. Since his exercise tolerance was minimal in combination with severe chronic respiratory failure, he was not a candidate for traditional progressive endurance or interval exercise training on a treadmill or bicycle [9]. Instead, NMES was initiated (two times per day for 18 min, 75 Hz, intensity adjusted to individual toleration [10]) and combined with resistance exercise training, consisting of bilateral leg extension and leg press, chest press and upper back training (three times per week, three sets of ten repetitions, initially at 65% of maximal strength and increasing by 5% every 2 weeks). Nocturnal NIV was initiated to achieve control of nocturnal hypoventilation (Philips Respironics Inc., Murrysville, PA, USA). The protocol and settings for NIV were auto-titrated average volume-assured pressure support (AVAPS-AE), maximum pressure: 26 cmH₂O, pressure support maximum: 18 cmH₂O, pressure support minimum: 10 cmH₂O, expiratory positive airway pressure maximum: 8 cmH₂O, expiratory positive airway pressure minimum: 6 cmH₂O, rise time: 200 ms, AVAPS rate: 5 cmH₂O·min⁻¹. A long-acting muscarinic antagonist was added to his pulmonary medications [11] and the maintenance dose of oral glucocorticosteroids (OCS) was carefully tapered, as OCS have no role in the chronic daily treatment in COPD because of a lack of benefit balanced against a high rate of systemic complications [12]. Pharmacotherapy for osteoporosis was started [13]. Nicotine replacement therapy (14 mg·day⁻¹) and smoking cessation counselling were initiated. This counselling was provided by his coaching respiratory nurse and in weekly group sessions. Based on recent smoking, severe hypercapnia and very poor exercise tolerance, he was considered not to be a candidate for lung volume reduction [14]. The patient participated in educational sessions about COPD, medications and healthy living. One of the educational sessions included information about life-sustaining treatments. Also, his goals of care, preferences regarding life-sustaining treatments and end-of-life care were discussed. Self-management interventions were performed. High-caloric and high-protein nutritional supplementation (Nutrison Energy Multi Fibre, 1.53 kcal·mL⁻¹ tube feed enriched with 1.5 g/100 mL of Multi Fibre, 500 mL per night) was given by the feeding tube and anabolic steroids were given (a deep i.m. injection of 50 mg of nandrolone decanoate once every 2 weeks [15]). Training in skills to overcome problematic activities of daily living and functional capacity was provided by an occupational therapist. In addition, the patient received individualised psychological support at a frequency of 2–3 times per week, focusing on problem-solving therapy related to his passive coping and depressive symptoms. Finally, a social worker was involved in order to improve the home environment of the patient.

Course of the intervention

Initially, the patient experienced great difficulties complying to the programme, especially with giving up smoking and sleeping with NIV. After ∼2 weeks, he started to feel a bit better and he did not smoke anymore. He was less dyspnoeic, his mobility increased and he became less gloomy. During his 8-week inpatient stay, he experienced one exacerbation of COPD, during which Haemophilus influenzae was cultured in his sputum and based on sensitivity analysis, he was treated with amoxicillin 3×500 mg and prednisone 1×30 mg for 7 days. The exacerbation did not result in acute worsening of his chronic respiratory failure. The patient completed his inpatient 5 days a week programme of 8 weeks.

Outcomes of treatment

As presented in table 1, the patient gained more than 14 kg in body weight, of which 10 kg consisted of FFM. The degree of dyspnoea and hypercapnia improved, although both remained prominently present. Functional exercise performance and peripheral muscle strength improved to a clinically significant degree (table 2). Also, symptoms of anxiety and depression decreased (table 2). The patient stated that he was satisfied with the results of the treatment and that he had reached his goals (quitting smoking, improving mobility and increasing in body weight). This allowed him to perform important activities of daily living and resulted in strongly improved health-related quality of life as was shown by the results of various questionnaires. To conclude, the PR programme was very successful in the short term. Care for the patient was transferred to his local respiratory physician and general practitioner. In addition, the continuity of care in the home environment was ensured for the various disciplines involved in the treatment. Following discharge from CIRO, the patient was able to reside in his home environment for almost 6 months, a sharp contrast to his frequent hospitalisations in the year prior to PR. Eventually, he was readmitted to the hospital with acute-on-chronic respiratory failure resulting from an exacerbation and died during this event.