Advances in medical science and technology in the second half of the 20th century contributed to prolongation of life of many critically ill patients with respiratory failure (both children and adults) and enabled them to return home and continue therapy (e.g. using ventilators), hence reduced the treatment-related costs. This is one of the basic goals of the World Health Organization [1].

Home mechanical lung ventilation, like the beginnings of intensive therapy, dates back to the 50-ties of the 20th century, when widespread occurrence of Heine-Medina disease was observed in many European and North American towns. In 1952, a severe epidemic struck Copenhagen, where 2722 people were affected within 19 months (27.07-3.12) and 866 developed paralysis of the respiratory muscles or pharynx (or both groups of muscles) and 70 required mechanical lung ventilation [2]. Ibsen administered IPPV to those patients using a rubber bag with the Waters container through the tube placed in the tracheostomy opening. This novel method reduced the mortality rates from 80% to 45% [3].

In the successive decades, home mechanical ventilation (HMV) was applied in patients with chronic respiratory failure (hypoventilative, hypercapnic) and thanks to it, patients unable to breathe spontaneously survived without hospitalization. In 1977, HMV was first used in children.

The aim of the present study is to evaluate retrospectively the 7-year experience with home mechanical ventilation managed by the Lódz Hospice for Children.

METHODS

The Lódz Hospice for Children is a non-profit organization administering home palliative care and home mechanical ventilation under the Non-Public Health Care Unit. Home care involves charge-free, holistic assistance and treatment (medical, psychological, social, and spiritual), 24 h availability of a physician and nurse within 120 km from Lódz (hospice ambulance services). The accepted model is based on cooperation with medical and non-medical institutions (family doctor, emergency medical care, hospitals, social welfare, schools), the Teaching Children Hospital No. 4 in Lódz , in particular, which admits children due to co-existing diseases and complications and refers them for hospice care.

Analysis involved the usefulness of hospice home treatment of patients with respiratory failure, duration of lung ventilation and individual numbers of hours of 24 h ventilation. The quality of care provided, causes and number of deaths as well as abandoned home mechanical ventilations were assessed.

RESULTS

In the years 1999-2006, home care and therapy was administered to 112 individuals (both children and adults), including 34 (30.4%) untreatable patients with chronic, progressive respiratory failure. The characteristics of patients are presented in Table 1. The biggest group provided with mechanical lung ventilation consisted of 19 children, aged 8-18 years (55.9%); the youngest group included 4 (11.8%) newborns (6-12 months of age). As for the etiology of respiratory failure, neuromuscular diseases prevailed – 29 (85%) patients, including Duchenne muscular dystrophy – 15 (52%) (Table 2).

Indications for home therapy were defined on the basis of the underlying disease confirmed by lack or limited respiratory autonomy (e.g. during long-term ITU treatment), clinical status tests, pulsoxymetry, spirometry, acid-base balance examinations before or after hospitalization, and frequency of ITU admissions.
In the group of 18 (52.9%) cooperative patients, spirometry was performed which showed diverse values of forced vital capacity (FVC), depending on the type of disease (Table 3). According to the guidelines of the American Thoracic Society and Polish Pneumological Society, 15 patients were diagnosed with extremely severe restrictive disorders, including 4 with FVC below 10%. Twenty-six (76.5%) out of 34 patients required ITU treatment, including 24 – three times, 1 – 4 times and 1 – more than 5 times.

The ventilator therapy was administered both to children with preserved and limited consciousness following the informed consent of parents and patients. Families were provided with instructions concerning nursing care, cardiopulmonary resuscitation and operation of medical devices.

The following devices were used:

– ventilators,
– cough-supporting devices,
– pulsoxymeters,
– anti-bedsore mattresses,
– self-expanding bags,
– plug-in and battery operated suctions,
– oxygen concentrators,
– oxygen in cylinders,
– adjustable electric beds,
– power-generating sets.

If the health status permitted, non-invasive methods of lung ventilation were preferred (through silicon and gel mouthpieces or nasal masks).

Seven (20.6%) patients required 24 h invasive ventilation through the tracheostomy tube. The remaining 27 (79.4%) had moderate to extremely severe respiratory insufficiency – 3 required invasive ventilation (in total – 10 patients – 29.4%). Time-dependent, pressure-regulated ventilation was used in 18 patients and volume-regulated – in 16. In 3 newborns with type 1 spinal muscular atrophy, the nasal non-invasive method of bilevel inspiratory positive airway pressure (BiPAP) was used.

In the majority of cases, duration of artificial lung ventilation ranged from 8 h to 12 h (Table 4). The average duration of non-invasive ventilation was about 12 h a day; it always covered the night and day sleep periods. In one patient who did not consent for tracheostomy, despite the baseline FVC of 4%, 24 h non-invasive ventilation was provided (through a mouthpiece at daytime and nasal mask at night).

The total duration of home ventilation therapy in individual patients was 7-3089 days; with the invasive method – 3089 days and with the non-invasive one – 1833 days (in the youngest child – 1713 days).

Eight out of 34 patients died, including 4 with invasive ventilation administered. The cause of death of 2 children with type 1 spinal muscular atrophy was the respiratory accident. The remaining patients died due to progression of the disease or respiratory infections (myopathy – 2, atrophic lateral sclerosis – 1, type 2 spinal muscular atrophy – 1, Duchenne muscular dystrophy – 1, vegetative state – 1). Out of 4 children in whom home lung ventilation was discontinued, three died in the ITU setting. Ventilation was abandoned in 3 patients with 24 h ventilation shorter than 3 h (despite indications, they did not accept longer respiratory support); in one case, ventilation was discontinued due to return of efficient respiration.

Six families whose children died had various social problems; 4 – single-parent families, 4 – alcohol abuse. The care providers of 4 children who died had elementary education (Table 1).

Patients and their families presented various attitudes towards home therapy; some accepted it (highly motivated children and parents) whereas some were against it. In one case, lack of adequate care and involvement of untrained caregivers (grand parents) led to accidental decannulation of the trachea and death.

DISCUSSION

Chronic hypoventilation of the alveoli may affect all age groups of patients [2]. In children, it develops in the diseases of airways and pulmonary parenchyma, in central nervous system dysfunctions, peripheral disturbances such as thoracic deformities or neuromuscular diseases [2, 4, 5].

The goal of home mechanical ventilation is to prolong life, improve the quality of life of patients and their families, provide suitable conditions to increase the patient`s individual potential, reduce morbidity, improve physical and psychological parameters, and reduce treatment costs.

HMV prolongs life of patients with chronic hypercapnic respiratory failure, with nocturnal hypoventilation, after recovery with high risk of further life-threatening episodes of acute respiratory failure or without earlier respiratory problems yet with high risk of acute respiratory failure.

Home therapy improves the quality of life of patients with respiratory failure during sleep, with the chronic cor pulmonale syndrome, or daytime respiratory disorders.
The method enables to maintain the optimal level of lung function; in children below 8 years of age, it stimulates the lungs for development enabling further life. Moreover, it decreases the incidence of infections within the respiratory system in children with muscular diseases receiving non-invasive respiratory support compared to those without such a therapy [6].

The choice of patients, who are likely to benefit from home mechanical ventilation, should consider: risk factors of developing respiratory failure, past episodes of acute respiratory failure, present respiratory efficiency, incidence of episodes of respiratory deterioration, probable effects of supportive ventilation on survival and on the respiratory symptoms, skills and requests of patients, and family support [7, 8, 9, 10, 11, 12].

The earlier study carried out in the Lódz Hospice for Children in the group of 30 children showed that both the patients and their families expected mainly the provision of medical needs (equipment, therapy, nurse, physiotherapy) and safety (help, nursing care). The study findings confirmed the clarity and understanding of instructions concerning the management and therapy as well as the operation of medical devices. Social support was provided for 66.6% of patients, although initially only 10 % expected it. Over 76% of families confirmed that their needs for emotional support were met and 46.6% confirmed the hospice assistance to initiate or continue individual education of the affected children [13].

The use of home mechanical ventilation, especially non-invasive, in newborns is of particular interest [2, 14, 15]; 90% of children with type 1 spinal muscular atrophy die within 2 years. The use of invasive ventilation prolonged their lives and facilitated their further development in the home setting. With the hospice help, the first of such patients is living for more than 13 years. Initially treated in ITU, since 1998 he has been living in his family home. Thanks to the introduction of non-invasive lung ventilation, children diagnosed with type 1 spinal muscular atrophy below 1 year of age may not only overcome death but even start to speak, which makes their development markedly richer.

CONCLUSIONS

1. Home mechanical lung ventilation enables therapy of patients with advanced respiratory failure prolonging their life and improving its quality.

2. Neuromuscular diseases are the commonest causes of respiratory failure in children treated at home.

3. Home mechanical ventilation is mainly based on non-invasive methods, even in the youngest age groups (newborns), and thus postpones tracheostomy.

4. The deaths of patients treated with home mechanical ventilation result most frequently from infections and progression of the disease. Failures are also affected by the structure and social conditions of families.

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REFERENCES

1.   Murkowski M, Koronkiewicz A, Nowacki W: Hospitalizacja domowa (medyczna opieka domowa). Centrum Organizacji iOchrony Zdrowia. Warszawa, 1999.

2.   Bach JR: Noninvasive mechanical ventilation. Hanley&Belfus, Philadelphia 2002.

3.   Gilbertson A: Before intensive therapy? J R Soc Med 1995; 88: 459-463.

4.   Fauroux B, Desguerre I: Home oxygen therapy equipment and mechanical ventilators for children. The European Respiratory Buyers 1995; 1: 13-19.

5.   Eigen H, Zander J: Home mechanical ventilation of pediatric patients. Am Rev Respir Dis 1990; 141: 258-259.

6.   Dohna-Schwake C, Podlewski P, Voit T, Mellies U: Non-invasive ventilation reduces respiratory tract infections in children with neuromuscular disorders. Pediatr Pulmonol 2008; 43: 67-71.

7.   Muir JF: Home mechanical ventilation. Eur Respir J 1995, 1: 7-12.

8.   Pierson DJ, Kacmarek RM: Home ventilator care; in: Principles and practice of pulmonary rehabilitation. (Eds.: Casaburi R, Petty TL), W.B. Saunders, Philadelphia, 1993.

9.   O’Donohue WJ, Giovannoni RM, Goldberg AI, Keens TG, Make BJ, Plummer AL: Long-term mechanical ventilation. Guidelines for management in the home and at alternate community sites. Report of the ad hoc committee, respiratory care section, ACCP. Chest 1986; 90 (Suppl.): 1-37.

10. Plummer AL, O’Donohue WJ, Petty TL: Consensus conference on problems in home mechanical ventilations. Am Rev Resp Dis 1989; 140: 555-560.

11. Make BJ, Gilmartin ME: Rehabilitation and home care for ventilator-assisted individuals. Clin Chest Med 1989, 7: 679-691.

12. Simonds AK: Home ventilation. Eur Resp J 2003, 22: 38-46.

13. Adamczyk J: Funkcje dydaktyczno-wychowawcze iLódzkiego Hospicjum dla Dzieci. Wyzwania dla opieki paliatywnej wwieku. Praca magisterska. Wyzsza Szkola Humanistyczno-Ekonomiczna wLodzi, Lódz, 2006.

14. Stengert PK, Siemiatkowska-Stengert W, Piotrowski A, Wodzynska E, Kania M: Juz 10 lat zyje – porównanie doswiadczen wza pomoca respiratora wichlopca chorujacego na rdzeniowy zanik miesni typu 1. Przeg Pediatr 2005; 35 (Suppl. 1): 46-47.

15. Stengert PK: Zapobieganie ostrej niewydolnosci oddechowej wdziecka wiotkiego. Przeg Pediatr 2007; 37 (Suppl. 1): 39-40.

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Address:

*Piotr K. Stengert
Adres do korespondencji:
Kliniczny Oddzial Intensywnej Terapii
Uniwersyteckiego Szpitala Klinicznego Nr 4 wLodzi
ul. Sporna 36/50, 91-738 Lódz
tel./fax: 0-4277 77, 0-603 872 283,
e-mail: pkstengerti@wp.pl

Received: 17.12.2008.
Accepted: 04.02.2009.