It is June 29th Sunday as I pen this. I want to discuss about the issues which arise in the care of a terminally ill ALS patient. ALS as you know is a progressive neurodegenerative disorder characterized by the loss of anterior horn cell neurons in the spinal cord as well as in the brain stem (for more information read my earlier posts on ALS at http://braindiseases.info). As more and more anterior horn cells die, patients become progressively more incapaciated. They become weak losing first the ability to ambulate on their own, then wheel chair dependent and finally bed bound too weak to even feed themselves or take care of other activities of daily living. Throughout this their cognitive abilities remain relatively intact and they do not have memory issues. However as bulbar (brain stem) dysfunction occurs they are unable to speak and even unable to swallow or manage their own salivary secretions. Bladder and bowel function as well as eye movements are preserved till late in the disease course. No one quite knows why these functions are selectively preserved while the others are so affected. They also start having difficulty with breathing as they are unable to manage their salivary secretions and unable to cough if something enters their trachea.
As you can imagine the family as well as the patient have to deal with many issues. Thankfully there is much out there which can make life easier for these patients and their caregivers. Motorized wheel chairs are available which help in ambulation. Some of them are quite advanced and can even be operated by voice commands. Devices which serve as finger/ hand extensions are available and these can be of immense use to these patients if they want to pick something out like from the cupboard. Artificial voice devices are also available and hence patients are able to communicate their needs. Patients feel their independence is not lost and that is extremely important.
As bulbar functions deteriorate and secretion control becomes difficult it is important that something be done. Most of these patients need a tracheostomy finally but we can avoid it at least for a while with the aid of devices which produce an artifical cough. Mechanical insufflation-exsufflation devices (also known as In-Exsufflator, Cofflator, or cough machine) alternate positive and negative airway pressure to stimulate cough. These devices clear secretions by gradually applying a positive pressure to the airway and then rapidly shifting to a negative pressure thus stimulating a natural cough. Their use has been shown to decrease the occurence of respiratory infections in patients with ALS. Moreover they are non-invasive and easy to use.
Feeding becomes an issue and so is the maintainence of nurtitional status. While initially nasogastric tube feeding can be used, most of these patients finally need what we call PEG feeds. This is a tube placed by the surgeon into the stomach (the procedure is quite safe and simple actually). Once the PEG tube is in place, all the medications as well as feeds can be given via it.
I would advise patients and caregivers to discuss end of life issues at some point, even though these discussions are unpleasant and hard on everyone involved. Get in touch with the ALS association which shall be able to advise you further about devices out there which make life easier.
There is always hope and one must never lose it!!!
Nitin Sethi, MD