Paula Baez was a cheerful woman with Down's Syndrome who was always accompanied by her neice Sonia during her visits to the office. During the last year of her life, she was hospitalized on several occasions with aspiration pneumonia. Studies revealed oropharyngeal swallowing dysfunction, not unlike that seen with with dementia. Alzheimer's disease is common in those Down's patient of this age, and this is presumably what she was developing. During one hospital stay she was diagnosed with hypothyroidism and we hoped replacement would bring about some improvment, but it did not help.
Her family did what they could to be with her and comfort her in the hospital, but they could not always be there, particularly during tests and procedures. At these times, she sometimes needed mechanical restraints. It was difficult to reassure and soothe here because her retardation and language barrier made it impossible for her to fully understand why strangers were doing these things to her.
During her last hospitalization, her breathing deteriorated and she was given oxygen by mask and BIPAP (non-invasive respiratory support) was attempted but the mask on her face frightened her. Her family understood she had lived longer than many Down's patients and requested that she receive only comfort measures. While palliative care was absolutely the best treatment for her, it would mean forgoing mechanical ventilation. While there was no doubt in any of our minds that this was right, we knew there was no legal basis for this in a never-competent patient. This was confirmed by hospital counsel and she was intubated. Mercifully, she died within a day anyway, but in the cold inhospitable ICU environment.
Jack P. Freer, M.D.
Medical Director
Hertel Elmwood Internal Medicine Center
Buffalo, NY