Death by Cystic Fibrosis

I had been caring for 19 year old Ms. Samson for several days now and she was not getting better. On the first day she had been able to remove the Bipap mask long enough to eat her meals. Now we removed the mask only for her to take pills and even from this short pause her blood oxygen levels would drop to the low 80’s and return to the minimally acceptable 90’s very slowly after several minutes.

Ms. Samson’s room in the medicine intensive care unit (MICU) showed signs of a family accustomed to hospital stays. Bags of clothes, half full cups of sodas, half eaten sandwiches and untouched cups of Jello (saved just in case they might be wanted), open purses and extra sheets and blankets lay about the more than usually cluttered tables, vinyl chairs and floor. The lights were dim and the TV, at low volume, was turned to a morning show. Ms. Samson’s mother lay asleep on an armchair, wrapped in a couple of white hospital blankets. She did not stir as I performed my nursing assessment and administered the medications. I left the curtains by the entrance to the room drawn and slid the glass door closed as I exited to let them rest.

When the doctors rounded and discussed Ms. Samson’s case my suspicions were confirmed. Ms Samson was on the edge of requiring intubation, (being placed on mechanical ventilation through a plastic tube which is inserted directly into the lungs). Since Ms. Samson had Cystic Fibrosis this would be a death sentence. The thick sticky mucous that coated her lungs would be pressed further and further into her airways and, although she would appear better at first, after a few weeks to a few months of gradually increasing the ventilator pressures there would be nothing more to be done and she would die. In my short nursing career I had already witnessed this a few times. The hospital where I was working specialized in treating Cystic Fibrosis and since those suffering from the genetic disorder rarely live to their twenties and almost never to their thirties, we in the MICU had the unhappy task of caring for the otherwise normal teenagers and young adults as they succumbed.

The attending physician was equivocal – if she was intubated she would never be extubated (removed from mechanical ventilation). An intern raised the possibility of a lung transplant but the attending explained that the most important predictor for success in organ transplantation is the patient’s ability to strictly adhere to a complicated medical regimen. Since Ms. Samson had a history of noncompliance with medical regimens, she was not a candidate for a lung transplant. The doctors and pharmacists discussed which antibiotics would have the best hope of working, made a plan, wrote some orders and moved on to the next patient.

After the doctors had gone I entered Ms. Samson’s room, her mother was awake now, sitting quietly in the chair staring at the wall. I squatted by her side. “Mrs. Samson,” I said gently, “do you understand how serious your daughter’s condition is right now?” “I think so, kind of,” she replied seeming glad that I was asking, “but tell me.” I told her that her daughter would soon be intubated and that the doctors did not think she would ever be able to get off the vent again. She replied, calmly, that she had not realized that. I explained to her that her daughter would die slowly over weeks or months and I told her that there was the option of deciding not to put her on a ventilator.

I know what I think I would want for myself. I have seen what these patients go through and I do not feel it is very merciful to just keep them alive in miserable conditions for as long as possible. I encouraged Mrs. Samson’s mother to think about the choice that needed to be made. What would her daughter want?

She went to her daughter’s bedside. “Do you want help with breathing?” Her daughter nodded yes, but the mother was still uncomfortable. “I do not think she realizes she would never get off of the vent. I do not think she would want to live like that.” Ms. Samson’s tattoos and piercings seemed to indicate a free spirit. Maybe it would be better just to let her go.

What must it be like to know that you will not make it to age 30? Ms. Samson had missed her 16th birthday party and her high school graduation – she had been in the hospital with pneumonia both times. She had discussed with her therapist that she was tired of being controlled by her disease and so she had delayed going to the hospital this time with hopes of being able to celebrate 19 years of life. The therapist did not visit her in the hospital. “Where is that therapist now huh?” I thought to myself, unsure if I had a right to the feelings of indignation I was experiencing.

When a mother comes to know that she is going to have to make a life and death decision about her daughter, one day is not a whole lot of time to do it in. I brought the doctors to talk with her. They confirmed the situation. I helped Mrs. Samson interview them. I introduced her to the family of another cystic fibrosis patient who was also on the unit at the time. This girl was 15 years old and had already had a lung transplant. The transplant had gone bad, and she was in the MICU on a ventilator, too sick to get another transplant, wasting away. Her parents vainly clung to the hope that she would recover enough to get the transplant. They coaxed her and prodded her to walk up and down the hallway once a day. During the journey of about 50 paces all could see her emaciation. Her knees were twice as big around as her thighs. The pride of any anorexic would be crushed. “All we are doing is rearranging deck chairs on the Titanic!,” complained one of the residents referring to her care.

Mrs. Samson looked at the girl and talked with her family. The girl seemed comfortable with her head propped up on a pillow. She looked at you when you came into the room. It was a good day for her. I worried a little that Mrs. Samson might get a too rosy impression of her life, but she came back from the room shaking her head. “They seem happy with her life, but I do not know. She is so drugged out. I do not know what kind of life that is.”

At the end of the day, Mrs. Samson was still undecided, but was leaning towards not intubating. She had talked with the doctors, and they were aware of and seemed to agree with her sentiment. I let the night nurse know where things stood and left for the night.

When I returned the next day, the room was empty. The Samsons were gone. I asked what had happened.
A blood gas had been taken at the beginning of the night shift and it came back critically acidic. The nurse had informed Mrs. Samson that the time to make the decision had come. Mrs. Samson left to think and smoke a cigarette. If it had been me, I would have told her that there was not time for that, but the night nurse apparently did not feel that was her job. While her mother was out, Ms Samson began to code. The doctors, not wanting to go against the mother’s wishes, tried to buy time by ventilating Ms. Samson by hand with an ambu bag. She vomited and was choking on her vomit when her mother returned. “What is all this! I don’t want any of this!” exclaimed Mrs. Samson. The resuscitation was promptly ended and Ms. Samson’s heart stopped almost immediately. Mrs. Samson had then broken down.

The body had stayed in the room for longer than usual. The 17 year old sister, who also had cystic fibrosis and had avoided visiting her older sister, came to offer last respects. The night nurses criticized Mrs. Samson for her smoke break. “What kind of mother with a daughter who has cystic fibrosis doesn’t talk with her about intubation and her wishes?” In younger days I would have felt outrage. Why had these nurses just stood by and watched when they should have stopped Mrs. Samson and told her what they knew? But I remained peaceful with only some sadness. One has to realize what cannot be changed.

What would Mrs. Samson say to me if I met her on the street today? Would she thank me for helping her to spare her daughter from pain and suffering? Or, would she tell me that any moment of her daughter’s life was precious and that she would have wanted to give her that time ventilator or not? I do not know. When I die, perhaps there will be someone there who can tell me whether or not I did the right thing by Ms. Samson and her family.