Marcie transitioned to my care when she turned sixteen. She felt too grown up now to be in the same waiting room with “kids” at the children’s clinic. But she wasn’t doing well.
Her pediatrician had suspected the problem shortly after birth. She wasn’t growing normally, had nasal congestion, and more respiratory infections than usual. A sweat chloride test was ordered which was positive. The diagnosis was cystic fibrosis (CF). The diagnosis was devastating to Rich and Sally, her mom and dad. Both of them were free of the disease, yet they were “carriers” of an abnormal gene which meant that statistically 25% of their children would have both abnormal genes and thus have CF. Marcie was the unlucky one. Her sister was free of the disease but a “carrier” by genetic testing.
Sally was very committed to learning and focusing on what could be done for Marcie. Rich was angry about the whole thing and distancing himself. By the time Marcie transferred to my practice, Rich and Sally had been divorced several years earlier. Sally, as many parents with CF children, had become very well versed in CF care, advocacy, and support. She had a very close bond with Marcie. Much of their education came from support of the Cystic Fibrosis Foundation and their national registry for CF.
She learned that CF problems were related to very thick mucous secretions in the lungs and that a variety of aerosols were very beneficial. Early treatment of lung infections was very important as was watching the levels of fat soluble vitamins in the blood (D,E,A and K). The pancreas did not secrete enzymes normally so enzyme pills were part of the daily regimen along with vitamins. Calories were counted and weight closely monitored because normal digestion was impaired.
At least once or twice a year there would be a severe flare up with respiratory infections requiring hospitalization. Much to her chagrin, the hospital would insist on putting Marcie on the pediatric unit where the nurses were much more versed in CF care. But this way she did get a private room which pleased her.
Marcie was excelling in school and growing into a lovely young lady. She hoped to train as a nurse and, in early high school years, volunteered at a local hospital. She had started dating and had a wide circle of friends.
During a hospitalization at age 16 she complained of “peeing all the time”. I asked the nurses and resident if they had done a urinalysis or checked a blood sugar. The latter turned out to be 310 (normal less than 100). So Marcie’s pancreas was failing and she was now diabetic complicating her care further. Additionally her liver was becoming involved and she’d had some bleeding from veins in her lower esophagus which ultimately required more intervention.
Despite this Marcie tried to set goals for herself. Her friends and Mom were her lifeline. She knew her time was limited and the goals became more near term.
Partly to reassure Marcie, the family (and me) that all was being done that could be done medically, I referred her to the University Adult CF clinic which was actively involved in research and patient care. As pediatric care has improved, the average lifespan of a CF patient has expanded from early childhood now to age 35. The consultation was supportive but didn’t offer new avenues of care. Marcie had so many complications that her outlook was looking progressively poor and the hospitalizations more frequent. At that time she did not meet the criteria for lung/liver/pancreas transplant unfortunately.
So I was faced with the difficult situation of discussing end of life issues with a young woman who was just turning 18 and about to graduate from high school. All along, Sally encouraged Marcie to “go for it” and live life to the fullest. But time was running out. Marcie had seen CF friends die and found discussing her own mortality very difficult and depressing. She did manage to say that if there was really no hope that she wouldn’t want to keep going on machines.
As Marcie neared the end of her senior year the deterioration in lung function continued, but she had her heart set on going to the senior prom. Her boyfriend said “it was no big deal”. Marcie though pictured this as an all important once in a lifetime event. She new it was risky in terms of her health as the day approached so she and Sally, her very supportive mom, had a heart to heart discussion. Sally managed to find the inner strength to support Marcie’s wish. So Marcie, delighted, went out for the big night – and had a wonderful time.
But within a week she had acute respiratory failure, presented to a nearby emergency room where was intubated and placed on a respirator. She was then transferred to my care in the ICU. There was no improvement over the next three days. The very poor lung function at baseline showed no improvement. Heavy sedation was required to keep Marcie comfortable and from pulling out her tubes.
There were daily conferences in the ICU. Sally, as a mom, was reluctant to accept that her daughter wasn’t going to get better. I discussed the situation with my colleague at the University Hospital and was very delighted that he offered to come across the lake to help with the family discussion and decision making. He affirmed that Marcie could not make it off the ventilator and that keeping her alive this way was only prolonging the dying process.
At the end, it was agreed to wait until Sunday since her birthday was on Saturday. Sally, the boyfriend, sister and pastor were all at the bedside. I turned off all the monitors in the room gave them all time to say their goodbyes and shed tears. It was a sad scene for all the nurses who had given her such excellent care. After asking the family to briefly step outside, we gave enough sedation for comfort and removed the endotracheal tube. I had prepared the family to expect shallow respirations. They held her and talked to her at the end.
Her mom, Sally, said to me though tears, “When she was a little girl I dreamed of so many things for Marcie. She was stronger than I and helped me through my sadness. The one bright spot is that she achieved a special goal – going to the prom.”
Comment: In the modern Intensive Care Units, there are many patients on ventilators to support their breathing while trying to heal and recover from a variety of diseases and injuries. Some do heal and return to reasonable function. But some worsen, develop complications, and have multiple organs fail. Surprisingly about 70% of deaths in the ICU’s are related to withdrawal of the ventilator – the only thing that is prolonging the dying process. The decision to withdraw the ventilator, even when expected and supported, is never easy but it does show respect for the wishes of patient as reflected in family discussions. There are times when the ventilator is continued for days, weeks, or even months when the surrogate decision makers disagree – but fortunately the futile continuation of care is unusual. Ethics consultations and recommendations are invaluable in such situations.
– Jim deMaine is a pulmonary physician who blogs at End of Life – thoughts from an MD