Saturday, September 13, 2008

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.


BreathinSteven said...

Cystic fibrosis sucks. I cannot think of something more miserable than watching a young person's lungs fail -- watching them slowly suffocate and drown in their own secretions...

Thank you for taking the time to describe this scene -- for taking the time to describe it from the viewpoint of a non-family caregiver. And, for considering what the feelings of loved ones watching must be like...

I don't know what Mrs. Sampson might say would she meet you on the street today, or years from now... Her feelings about how precious a moment of her daughter's life is, or the desire to reduce or end her pain and suffering may very well change day to day, or moment to moment... Love is funny that way -- I want you here with me forever, yet I cannot bear the thought of you suffering, knowing that it will not end well... Is it selfish to want you here for just one more moment, or one more day? Might it be one more moment or day that you will feel my love?

I don't think there is a right thing or a wrong thing in this situation.

Thank you for being there. Thank you for being compassionate with her Mother... I'm sorry the end came like it did -- but I also understand why and how it happened the way it did -- and even her Mother's reluctance or indecisiveness at the very end, until the decision was more or less made for her...

I spent a fair amount of time in hospital rooms and even the MICU. I remember knowing that I would not make it to my 30s... Then knowing that I would not make it much past my 30s. I got lucky -- very lucky... I'm 48 1/2 now and I've been breathing with beautiful, "new" lungs for the past 8 years -- lungs from a precious 17-year-old girl from Iowa named Kari. She's on my thoughts throughout the day, every day -- I can see her smile when I close my eyes.

Kari and her family are my heroes -- Kari saved my life when I needed her most -- but there have been so many doctors, nurses and medical professional in my life who are my heroes too, and who are on my mind quite often. Kari saved my life, but they helped carry me to her door, and carry her gift to me...

You take care... Thanks for sharing this story...


Steve Ferkau
Chicago, IL

Makarios said...

Glad to see you back to active posting, Leo. You may not realize it, but this is a very important blog indeed. Your narratives of what occurs in ICU's, which is both straightforward and compassionate, can be a revelation to those who haven't "been and done." Keep up the good work. Please.

Leo Levy said...

Thank you for the encouragement. I am trying to get at something deep and meaningful, but I am not always sure if I am succeeding. I have many more stories to tell. Thank you for hearing these. I will try to tell them all.

Sharon said...


My name is Sharon Ray and I am the assistant editor of I am contacting you today in hopes of developing a relationship with your website; we have seen your site and think your content is great. offer a free informational resource to both the general and professional public on this terrible disease.

I hope you show some interest in building relationship, please contact me at

Nanny said...

I ran across this blog while searching for other info. I want to thank you for a well read article. Our daughter died at the age of 14 at Children's Hospital in L.A. in 2001. She had gone through resp failure and shortly thereafter was intubated. Within a week, she was gone. Thank you for your heartfelt recap. It brought back sad memories, but I know Erin is in a much better place.

N. Hutchison
Ventura CA

Natasha smith 13 said...

Hi my name is Natasha Smith and im 12 years old i have Cystic fibrosis and some tips to be as healthy as i am is tip 1.All ways carry hand sanitizer tip 2.Take ur medicon even if u dont like it the littelst amount could save your life.......Some people have cystic fibrosis in varryis parts of there body and orgens i have fullblown cystic fibrosis itsa not the easyst thing to handel when ur a pre teen but i got diagnost with it at 2 years old i almost died because i've had it longer but they didnt know i am 100lbs and im 5'4"it took me 11 years to get this heavy and tall and i am so proud of my self and i hope every body who has cystic fibrosis and reads this takes this advice:If the docters ever tell u u have tuill the age of 10 tolive dont give up dont think ur gonna die because if u do tht i bet u god will help u live longer tht healped me i was supposed to be gone but im gnna be 13 on oct.13 i was born in 1998 and they didnt have any of the tecnolegy tht they have now so i have 1 last word b4 i stop typing i belive in every infit,kid,teen,and young adult tht they can survive if they take care of there self and good luck if u have any qustions feel free to email me i will answerany qustions u have thank you -Natasha Smith <3

Leo said...

Hi Natasha, Thank you so much for posting! It is really nice to hear from you!. It sounds like you are working really hard at staying healthy and that you doing a great job. It must be so hard sometimes. Hang in there! Wishing you the best of everything. Leo.

Anonymous said...

Thank you Leo for your story. CF is a vile thing. My 5yr old son does not have CF but he does have panc issues from a successful BMT but the GVHD is roaming around now killing off different systems... My heart says some day I'll have to make the choice. It helps to know that the medical pros at his bedside might feel like you and view quality and not quantity as most important. Some people don't understand how a parent could walk away when there was a stone yet unturned, time that could be gained but when it comes time for my baby, I will choose quality for him because I love him that much. Thank you for being out there educating the patients and their parents of their rights.

Anonymous said...

I am 26 and living with cystic fibrosis. As a youngster I was in and out of the hospital more often, likely because it was my parents' decision back then. Those hospital visits have traumatized me, to say the least. My worst memory is of a particular hospitalization when I was 13 during which I was given my first pic line. This "tune up", as everyone insensitively referred to it, went terribly wrong. For the four days I was in the hospital, every hour or two brought in a team of nurses and doctors who held me down to a table, against my screaming and crying and flailing, to try again at inserting a pic line. Finally after days of failure, I was fully sedated for the procedure. I woke up with a tube in one of my two dark black and blue arms and a new understanding of what my life would look like. I've never been the same.

Not only have I (indefinitely) put off hospital check ups, but just the thought of doing meds makes my blood boil and sets off a mini-panic attack. I'm severely depressed and have been since I was in grade school. I have developed generalized anxiety, from which I never get a moment of relief. The thought of my shortened future has kept me from looking forward to establishing my own family and career. I've buried myself in graduate school, studying applied mathematics, so that I don't have to face the reality of it all. I keep busy and barely stay afloat, knowing that the day is coming soon when I will suffocate to death in a hospital bed.

I know very well how to delay this day's arrival; being vigilant about my meds. But after a life of pain and anguish, it hardly seems worth prolonging. I've contemplated leaving my girlfriend of 3 years to spare her the pain that awaits her when I finally slowly die.

Now I'm thinking of quitting school, so as to enjoy the last leg of my life more completely (or at all). I've given up on making new friends for the sake of not having to explain what a wreck I am. The closer it gets, the more I feel like a terminal animal that wants the wander off and be alone for the end.

And the worst part is that it's all invisible. No one, including those who know me best, would ever think this was a description of my life. By all outward appearances I'm just like everyone else.

Please understand what a life-ruiner this disease is. This is how I really feel, all optimism and pessimism aside. These are my struggles. I just wish more people with cf could relate, or if they can that they would. I'm always told that talking with others in my situation could help, but it just makes me feel more disconnected. All I ever hear from others with cf is how "you gotta stay positive and stay healthy". There are those of us ( probably most of us) with cf who are neither. What do we do?

Leo Levy said...

Thank you so much for your comment. Sometimes our bodies are like cages from which there is no escape. With severe illness there is trauma that is inescapable. too often the lines blur between the trauma that comes from the illness and the trauma that is inflicted on us during the course of our medical care. Those with good intentions can still be callous and removed. Some may focus on the disease and forget the person. Some may simply be lacking in humanity. You are supposed to receive it all with gratitude. There is no room in our culture for an ungrateful patient, neither is there generally any appreciation or sympathy for the depth and severity of the trauma that may be experienced during hospitalization. I hear you expressing that you feel isolated both by the disease itself and by the experiences during treatment. One of those is not in anyone's power to change, the other very much the opposite. You are entitled to your feelings. None of us knows when the end of our lives will arrive, but when we know our time is short, it becomes all the more valuable. I hope you can find a way out from under it all. As someone who has worked in the kinds of places where you had those experiences, I would like to say that I am truly sorry that you were treated that way, that it did not have to be that way and that I wish it had been different for you. I wish I could offer more. Sometimes a small gesture can make a big difference. Even 13 years later. I hope so anyhow. Please take care. Leo.

Anonymous said...

Thank you Leo. I can't tell you how nice it is to read such heart-felt, judgement-free response to my comment. Your small gesture is not small at all to me. If nothing else, today is a good day thanks to it. Please keep up the good work, it makes all the difference.