Waldemar Iwańczuk, Grzegorz Saładajczyk
Long-term artificial ventilation at home: report on three cases
Department of Anaesthesiology and Intensive Therapy,
Head: G. Saładajczyk, M.D. L.Perzyna Memorial Hospital, Kalisz, Poland
We present three cases of prolonged home mechanical ventilation. A 17-yr-old boy with Duchenne muscular dystrophy has been ventilated at home the Bennet MA1 ventilator since 1997. His condition is stable.
A 30-yr-old women had been mechanically ventilated two years at the hospital, and subsequently four years at home with Bennet Companion ventilator, because of kyphoscoliosis and pulmonary hypertension. She died at age of 36.
A 31-yr-old woman has been ventilated at home since 1985, first with the Dräger Assistor, than with the Warwick CBVS ventilator, because of the Pompe disease (glycogen storage disease). Her condition has been stable since 1978, she remains in the sitting position in bed and she is fully intellectually active.
The indications for articial ventilation in chronic respiratory insufficiency are generally those, in which alveolar hypoventilation occurs. This can be observed in some myopathic diseases (Duchenne disease, myotonies, glycogenoses), nervous system diseases (spinal muscular atrophy, myasthenia, Guillain-Barre syndrome, atrophic lateral sclerosis, anterior medullary inflammation) and postural deformities involving the thorax. Many cases of long-term artificial ventilation in hospital settings have been described [1,2,3,4]. The longest lasted for 21 years  in a patient who suffered from respiratory failure resulting from epidemics of anterior medullary horns inflammation in Copenhagen in 1953. In selected patients, requiring periodic ventilatory support with atmospheric air, the treatment at home is possible. 3 such cases are described.
1. A 17-year old male, suffering from Duchenne disease, was admitted to the hospital after cardiac arrest resulting from respiratory failure. After successful resuscitation he was transferred to the Intensive Therapy Unit and artificially ventilated (at the beginning - controlled, then - supported ventilation). A right-sided pneumothorax required introduction of the pleural suction drain for a period of 4 days. On the 6th day after admission tracheostomy was performed. The parents were instructed in the ventilator operation and tracheostomy tube care. The patient was discharged home with a ventilator Bennett MA1 (Puritan Bennett, USA). Since December 1997, the patient has not been hospitalised again.
2. A 30-year old female was admitted for the first time to the ITU in January 1992, with a diagnosis of kyphoscoliotic cor pulmonale. In 1984, in the postpartum period an episode of pulmonary thromboembolism took place, resulting in pulmonary hypertension, confirmed by heart catheterization. In 1991 she was treated pharmacologically in the Internal Medicine Department for circulatory insufficiency. Several months later she was admitted again with symptoms of decompensated respiratory and circulatory failure. The lose of consciousness, resulting from hypercapnia was the cause of her transfer to the ITU. Artificial ventilation was maintained for 3 days, than tracheostomy was performed. The patient returned several times to the ITU for short-term artificial ventilation. From February 1992 she spent 305 days in the ITU, ventilated artificially for 4-16 hours a day. Pharmacological treatment consisted of digitalis alkaloids, verapamil and diuretics. In January 1993 she was discharged home with a Companion ventilator (Puritan Bennett, USA). During the following four years the patient stayed at home. In May 1997 she was admitted again to the ITU for respiratory failure caused by bilateral pneumonia, which proved fatal.
3. A 17-year old female with diabetes type IIb, diagnosed earlier in another centre, was admitted to the hospital in January 1985. During her previous hospitalisation she underwent tracheostomy. She presented with bilateral pneumonia, treated successfully. Seven weeks later pneumonia recurred and was the cause of readmission. Apart from pharmacological treatment she required periodic support with artificial ventilation. The patient returned several times to the ITU due to respiratory insufficiency (recurrent infections) and need for respiratory support. In 1987 an Assistor 640 ventilator (Dräger, Germany) was leased for of home ventilation. Unfortunately, it was not efficient for periodic ventilation, because of a high level of malfunctions. Another ventilator used at home - a volume - cycled CBVS (Warwick, Great Britain) fulfilled her needs until 1997, when it broke down. Then it was changed for a Puritan Bennett Companion ventilator (USA). Between 1987 and 1997, the patient was hospitalised once for pneumonia and several times for servicing of the ventilator. The patient remains in bed in a half-sitting position and, in spite of thoracic deformity, generalised muscle atrophy, shows the great will of life, and is fully intellectually active.
Home treatment with a ventilator seems to reduce the scale of some problems:
- therapeutic - diminishing the risk of dangerous hospital infections
- economic - reducing costs of ITU stay
- psychological - improvement of quality of life of the patients.
Introduction of mechanical ventilation in patients with respiratory muscles fatigue seems fully justified, but remains controversial in patients with cor pulmonale and restrictive hypodynamic changes. It is well known that positive pressure ventilation is connected with many adverse effects . Our observations, however (case 2) produce some positive arguments in this discussion. The treatment of the patient, begun where she was in a critical condition, enabled her to survive 6 years at her own home, with a relatively good quality of life.
1. Chronic respiratory insufficiency, resulting from a hypodynamic mechanism may be successfully treated with home-based artificial ventilation with cooperation from patients and their families.
2. It would be much desirable to create the "bank" of ventilators to rent.
Originally published in Anestezjologia Intensywna Terapia 31; (2), 109-110, 1999.
1. Skolimowski J., Grzybowska J., Skarb., Sir J.: 645 osób sztucznej wentylacji płuc u pacjentki ze stwardnieniem zanikowym bocznym. Anestezjologia Intensywna Terapia 1988, 20, 136-140.
2. Orzechowski Z., Dąbrowski W.: Przewlekła wentylacja zastępcza u chorej z rozmięknieniem rdzenia. Anestezjologia Intensywna Terapia 1990, 22, 138-140.
3. Domarecki J., Drozdowski W., Malinowska L.: Wielokrotne leczenie oddechem zastępczym niewydolności oddechowej powikłanej zakażeniem układu oddechowego w przebiegu miastenii u chorej po usunięciu grasicy. Anestezjologia Intensywna Terapia 1984, 16, 389-391.
4. Skorupa A., Karpel E., Szczechowski L., Dyaczyńska - Herman A.: Zespół Guillain-Barre - problemy diagnostyczno-terapeutyczne. Anestezjologia Intensywna Terapia 1984, 16, 289-294.
5. Siedlecki J.: IV Europejski Kongres Anestezjologów w Madrycie - sprawozdanie. Anestezja, Reanimacja, Intensywna Terapia 1975, 7, 341-346.
6. Zieliński J.: Przewlekłe serce płucne. PZWL, Warszawa 1985.