Help at the End - the Reality

At the University Hospital in Madison Wisconsin in 1938, a patient was dying from a very painful bone cancer which had produced fractures. The young interns knew that more morphine injections were needed but they feared they might be blamed for giving a lethal dose. So a tacit agreement was reached. Every hour or so,one of them would come into the patient's room and give a shot of morphine. This was rotated between four or five of doctors so no one really knew who gave the final dose. My father told me this story before he died. He was one of the interns - and he felt strongly that they had taken the right action in a moral sense.

In 1980, I saw a patient I'll call Sam with a malignancy of the lining of his lungs. It was related to working around asbestos in the shipyards during World War II. The asbestos would come in bags which they dumped into barrels, added water, then applied the paste to steam pipes in the holds of ships for insulation. They were covered with asbestos dust by the end of the day - dust similar to the spray used for fire protection on the steel girders in the high rises of downtown Seattle where the white clouds of asbestos settled onto the city streets during construction. Several of Sam's co-workers had already died of the asbestos related cancer - mesothelioma.

Surgery was unsuccessful in that all Sam's tumor couldn't be removed. Fluid kept building up in his chest and he was progressively short of breath to the point of gasping and not being able to lie flat. I could tap some fluid giving temporary relief and even tried to scar down the area using a talc solution without much success. Sam would come in with his wife Helen feeling more discouraged and depressed. Over time he began asking for frequent refills on his sleeping pills along with more narcotics. I felt he was likely planning to take a lethal overdose of these medications I was prescribing which, of course, was against Washington State law at the time - and continues to be against the law in 47 other states.

"Sam, are you planning to take a large dose at the end?"

"I don't know doctor, but I want to be able to decide when things are no longer tolerable."

Since I was a member of the hospital ethics committee, I decided to present Sam's case to the group. These discussions are helpful and advisory. The recommendations are not the feared "Death Panels" being referred to in political debate. Ethics Committees have the benefit of broad representation: nurses, doctors, social workers, clergy, consumers, pharmacists, and many others. We had an intense and lively discussion. This was in the days before Dr. Kevorkian, before Hospice, and before any state had a "Death with Dignity" law. After much debate, the consensus of the committee was that ethically Sam had the right to self-determination and to end his life when the suffering was too great (assuming that he was mentally clear and competent). The committee acknowledged, however, that physician assisted suicide is against the law.

I saw Sam several more times, each time he was worsening. Two weeks later, I got a call that he had died at home. The death certificate was delivered to my office the next day. Cause of death assigned by me: Mesothelioma - his cancer.

I called Helen a few weeks later with my condolences and asked her if she'd be willing to come in and discuss Sam's dying with the Ethics Committee. She rather hurriedly replied, "No way," an answer that I kind of expected.

There are now more options are available at the time of death to help patients avoid or ameliorate severe pain or severe shortness of breath - conditions that are not at all uncommon at the end of life.

Modern Hospice care is now widely available in the USA and is a covered Medicare benefit. The benefit commonly adds increased coverage of medications, equipment, nursing visits, spiritual care, etc. during the last six months of life. Some health plans offer Hospice for terminally ill patients under 65 as well. Hospice, for unclear reasons tends to be underutilized with patients being referred there often only a few days before death.

Although Hospice is constantly improving, three states (Oregon, Washington, and Montana) allow "Death with Dignity" - basically allowing physicians to prescribe lethal doses of a barbiturate in specific situations. The Wall Street Journal, discusses Washington's I-1000 Initiative which passed with a 59% voter approval rate in November of 2008.

Oregon's similar "Death with Dignity Law" has been in effect for over a decade. In an Oregon State report, "During 2009, 95 prescriptions for lethal medications were written under the provisions of the DWDA compared to 88 during 2008. Of these, 53 patients took the medications, 30 died of their underlying illness, and 12 were alive at the end of 2009. In addition, six patients with earlier prescriptions died from taking the medications, resulting in a total of 59 DWDA deaths during 2009. This corresponds to an estimated 19.3 DWDA deaths per 10,000 total deaths." So for every 500 natural deaths, there is one DWD death.

So far, many of the worrisome concerns about the law have not come to pass: such as abuse of the weakened elderly; abuse of the handicapped or mentally ill; expanding the criteria for qualifying patients; substituted judgment; etc. I still do read and hear worrisome comments though like the following: "It's too bad we can't just give the medication through his feeding tube; he's a vegetable costing society and his family so much of their resources; she can't speak for herself now, but I know she would want to receive the drugs - just like her beloved dog did; although he does have more than six months to live he should have the right to go anytime he wants."

There are likely going to be organized attempts to broaden the criteria for assisted suicide (DWD) in coming years by election initiatives and court rulings. the issue is far from settled. It is unknown whether the ratio of 1 DWD death to 500 natural deaths will remain stable.

There's a controversial organization called Dignitas in Switzerland promoting Suicide Tourism, see March 2010 Atlantic Monthly. Also Frontline, a documentary show on PBS has a well done video of an American traveling to Switzerland specifically to commit suicide. Switzerland allows non-residents to receive physician assisted suicide. Other European countries like the Netherlands, Belgium and Luxembourg allow physician assisted suicide only for residents.

The unfortunate patient, Craig Ewert, had severe ALS, was losing muscle function and needed the support of a breathing machine. He wanted to be in control and did not want to suffer at the end. It was clear he would prefer to live but saw no hope for the future.

In my practice in Pulmonary and Critical Care Medicine I cared for many patients with ALS at the end. With the aid of hospice and aggressive palliative care with the intent of relieving pain and terrible shortness of breath, a patient like Mr. Ewert can receive care at home with the loved ones nearby. This type of death does not apply the "Death With Dignity" Washington law of a self-administered intentional lethal drug. It would mean giving small doses of morphine and/or midazolam (a sedative), removing the ventilator support, and giving enough medication to relieve symptoms. The intent is to relieve suffering and to adhere to the patient's wishes. Yes, the life ends but it is not a suicide, the loved ones are nearby, and the patient's wishes are met.

With the advent of care like this, we are seeing more terminal patients being referred to hospice, fewer hospital deaths, and more comfort for the patient and loved ones.

The DWD law in Washington, Oregon, and Montana does remain an option. But with good hospice care and good communication, the use of terminal lethal overdoses will remain at a minimum. The law has actually made the medical and nursing profession step up and provide more knowledge, more research, and more care focused on the end of life.

One More Trip to Guam

Marie is too young to have a fatal diagnosis. She has Primary Pulmonary Hypertension. This condition is called "Primary" because we have no idea of the cause. Medical lingo has a variety of ways of labeling unknown causes such as "Idiopathic" or "Essential", but in Marie's case, it's "Primary" - as if that label somehow shows some understanding.

Actually, I do understand that this is a devastating diagnosis which tends to affect adult women about three times as often as men.

Her family physician sends her to me for puzzling shortness of breath. Marie is a delightful soft spoken woman of Asian descent, born in Guam but moving to the USA many years ago. She has raised three children, been healthy, and helps out in her husbands business as a bookkeeper. She has a beautiful smile and tends to downplay her symptoms.

A non-smoker, her lung functions are normal, but she has some swelling of her ankles. Her heart tones are normal except for a split second heart sound, a sign of delayed closure of the valve leading from the heart to the lungs - the pulmonic valve. The EKG shows strain in the right ventricle. All this points to possible pulmonary hypertension which is confirmed later by heart catheterization and cardiac ultrasound.

We rule out things like fen-phen or blood clots and begin some drug treatment to try to dilate the vessels going to her lungs. Essentially all the blood returning from the body needs to be pumped out to the lungs where it gives off carbon dioxide and picks up oxygen. It's normally a low pressure system, but not in Marie's case.

Over the next few years she begins to fail. Other drugs are tried even a battery powered continuous IV. Oxygen is now needed. Marie somehow arranges an annual trip to Guam, her birthplace where many relatives still reside. Between office visits, she hardly ever calls and we have to reach out to see what's going on.

"Marie, how are you doing?"

"Oh, just fine. A little limited but I can't complain."

I have her come in. There's no doubt things are worsening and that more drastic measures need to be considered.

"Marie, we need to have a talk about next steps. Your cardiac echo is showing very high pulmonary artery pressure. This is becoming life threatening. The drugs are no longer working well. I'd like to refer you to the University Hospital to consider the possibility of lung transplantation."

"Oh, doctor. I'd never want anything like that."

"Why not?"

"Well you see, I'm at peace with God. What will be will be. I don't expect any miracles. Surgery seems so risky to me. I think I'd rather just go on."

I ask Marie to bring in her husband Gerald who is hardly ever in the exam room with her. I want to make sure he and the children understand what the situation is. She smiles at him when I bring up lung transplantation. Gerald smiles back at her and doesn't show frustration, "Look doctor, we've already talked about this. She doesn't want surgery. She knows her time is short, and we're flying back to Guam next week for a month's visit."

After more conversation, I begin to understand that they are further along in accepting her impending death than I am. They are Catholic, having a strong faith along with a kind of fatalism that I don't often see in my practice. They have accepted her destiny, while I'm still ready to fight on!

I OK the oxygen use on the airline and they have arranged for oxygen in Guam. During that month I receive a postcard basically saying, "Don't worry, I'm OK."

But on return to Seattle, Marie is worse. She can no longer walk across the room, has episodes of severe shortness of breath, and is becoming bed-bound. Marie and Gerald agree to a hospice referral. Hospice steps in with their usual grace and provides amazing support to Marie and the family.

From that point on I receive mainly electronic progress reports from the hospice nurses and an occasional call for medications. Small doses of palliative medications are keeping her comfortable.

Then the hospice nurse calls me, "Doctor deMaine, we're wondering if you could visit Marie at home. She and the family would really like to see you."

I don't know what to expect when I leave the office at 6PM to stop by their Bellevue home. The house is in a pleasant well groomed neighborhood. A child shyly answers the door and calls for her Grandpa, Gerald. The scene surprisingly seems festive to me. Food abounds on the large dining room table, music is playing, and kids are running around playing games. The living room has been transformed into a bed room. There in the middle of it all is Marie. Her hospital bed's head is propped up a bit so she can see. A sleepy smile appears on her face when she sees me.

"Why don't you get something to eat doctor, you must be tired and hungry."

I sit with her for awhile, listen to her chest with my stethoscope and hold her hand. The warmth and love surrounding her and her family is palpable. I feel tremendous respect for emotional strength.

Within a week Marie is gone from this life. She has left a beautiful legacy of love and acceptance of death - a blessing for her family, and me too. I come away with the hope that I can some day face my own death with such equanimity, and that I can have my loved ones close by.

A Call to Jerusalem

I'm not at all sure what is going on and that's not a very comfortable spot for a doctor to be in. The call from the ER doctor tells me that he's admitting a very sick Boeing engineer to the intensive care unit. Abdul Yasu (not his real name) has been in normal health, until he had sudden onset of stabbing left chest pain and violent coughing this morning. This was followed by sweats, a fever to 104, and trouble breathing. His blood pressure is thready and he is wheezing.

The strange thing is that I know Abdul. He's a neighbor living just a few houses away and our kids go have gone to school together. A brilliant aeronautical engineer with an equally brilliant wife mathematician Maryam, they immigrated from northern Galilee about 30 years ago. They were Christian Palestinians looking for a better life for their children. Boeing and Seattle have been their secular savior. Abdul has explained to me that his name in Arabic means "servant of Jesus" - a name he is proud of. He last visited his native land three years ago.

Abdul gives me a weak smile when he sees me walk into his ICU room. The cardiac and oxygen monitors are already in place and he's receiving an aerosol treatment for his wheezing: "Praise God that you're here to see me Dr. deMaine. I'm very sick."

That is not news to me! Maryam is at the bedside, very worried and their son who works at nearby Microsoft is on the way over.

Trying to be methodical and not miss anything obvious, I try to get more history. Any recent colds? Any unusual exposure to fumes, sprays or toxins? Any allergy history? Any history of heart or lung disease or smoking? Any history of HIV exposure (this important question is always hard to ask in front of family)? Tuberculosis history? Any friends or family ill? Any new medications or change in medications. Any new health food or alternative medications?

The answers were all to the negative. His list of chronic medications is very short. A diuretic for blood pressure, a multivitamin, and a baby aspirin. That is it. He's not taking any over-the-counter or herbal medications.

About this time the chest X-Ray arrives and I'm very concerned. There's a huge 10 cm cavity in the middle of the left lung which has very strange characteristics. Usually a cancer will be thick walled and irregular. Usually TB is also thick walled with a dense area of infection around it in the upper part of the lung. Abdul's large cavity (basically a hole) in the left lung is very thin walled and has some fluid gathered in the bottom - much like a cup holding water.

Looking further there is a possible second spot in the other lung, about 2 cm in diameter - something we would call a "coin lesion" in medical descriptive talk. This adds to my worry. An old X-Ray is located from two years ago that's entirely normal.

The exam doesn't add much to my knowledge base. There are no enlarged lymph nodes or skin lesions. The throat is clear. The lungs reveal diffuse raspy wheezing. He's not coughing up anything for testing, specifically no blood or off colored sputum. The heart is regular but fast at 120. The abdomen is benign. Orders are written for cytology (cancer check), and for multiple cultures - routine, TB, and fungus.

The blood work begins to return. The main remarkable feature is in the white blood count. This is very elevated with lots of eosinophils. These are the kinds of cells containing red-stained granules that we commonly associate with an allergic reaction. His count is 24% when it would normally be 0-2%. Thankfully the kidney function, liver function, and blood sugar are all normal.

All this was very puzzling. What could cause an sudden allergic-type reaction and a new lung cavity? I begin to think I might have an answer but I need a quick trip upstairs to the medical library. There are a number of syndromes involving eosinophils and the lungs, but in Abdul's case the acute dramatic onset of symptoms suggest a rare diagnosis - a pulmonary hydatid cyst which has acutely ruptured and produced his critical status.

I haven't seen a patient like this for 30 years. My current knowledge of what's called tropical medicine is very rusty. Fortunately in the 1960's I had a great teacher named Harold Brown. He was a Professor of Tropical Medicine at Columbia's School of Medicine and the author of a definitive medical textbook about parasites. The good doctor taught me about intestinal parasites at the CDC in my preparation to care for Peace Corps Volunteers in Afghanistan. Affectionately known as "Stoolie Brown", he made the parasitic intestinal diseases come alive.

Hydatid disase starts as a worm in a domestic or wild dog's intestine. The eggs are shed into the dog feces which often litter the pastures where sheep graze. The unwitting sheep (or an occasion human) ingests contaminated vegetation. The eggs hatch in the new host and penetrate into the liver (or lungs) where they form cysts. The cycle is completed when other uninfected dogs feed on the liver or lung cysts and develop intestinal worms. Humans in sheep raising countries are "innocent bystanders" becoming infected when the salads they eat are contaminated.

But does Abdul really have Hydatid Cysts in his lungs? We're taught as students to first look for uncommon presentations of common diseases, so I still need to rule out cancer, tuberculosis, etc. Abdul agrees to a bronchoscopy, CT scan of the chest and abdomen, and more blood work - specifically a serology for echinococcus (the medical names for hydatid cysts). The CT scan shows no cysts in the liver and demonstrates the large air and fluid filled cavity within the left lung. This is a 2.4 cm small low density (water density) rounded lesion at the base of the right lung.

He is so sick, I did the bronchoscopy at the bedside. With mild sedation and lidocaine aerosols, I slide the bronchoscope through the left nostril and follow its course on the video screen. The vocal cords are healthy and move well, The trachea and bronchial passages look normal, much like one is gazing up a hollow tree trunk. I do a saline lavage of 100cc's in order to get fluid from the affected area of the left lung. Then I do a biopsy of the mucous membranes nearby. I decide not to try to get into the cavity of the lesion which might induce more havoc.

By the next day, he's feeling much better. The biopsy and lavage are all negative except for the presence of eosinophils. I walk over the the radiology department to review the CT scan with my favorite radiologist. She notices that both on the chest x-ray and CT scan that there isn't just fluid at the bottom of the cyst - there's what she calls the "Water Lilly sign" - crumpled cyst wall at the bottom. She pulls out the CT text books which confirm the suspicions.

All this is beginning to finally make sense. The thin walled cyst acutely ruptured on the day of admission, spilling it's liquid product into the patients lung airways. He had an anaphylactic-type reaction, which can be fatal to some persons - so Abdul is fortunate to survive.

The following day the blood serology confirms a very high level of antibodies to echinococcus, the causative organism. False positives can occur but given the clinical presentation I'm sure I finally have a diagnosis.

But what should be done? I have no experience here, despite doing an Infectious Disease Fellowship years ago. So I pick up the phone and make several calls; to the CDC, and several medical schools with expertise in tropical medicine. The consensus was to begin treatment with a drug called Albendazole, but the big downside was that it provided a cure in only 30% of the patients. Surgery was an added option.

I discussed this with Abdul and Maryam. Their immediate response was, "You must call Maryam's brother is Jerusalem." He is a thoracic surgeon at Hadassah Hospital and must see a lot of this disease.

Dr. Nassir is just back from additional training at the University of Pennsylvania and was most helpful. After hearing the story, he explains that Hydatid Cysts are endemic in northern Galilee and a relative common reason for lung surgery there. He is definite, "It must come out."

Fortunately, my thoracic surgery consultant has worked abroad and is fearless in tackling a case like this. Abdul and the family feel comfortable going ahead and removing the cyst, which means about a third of the left lung. The operation itself doesn't take place for about two months, waiting for the Albendazole drug treatment to kick in and hopefully sterilize the infection so surgery wouldn't spread it to new sites.

The surgery goes well and Abdul makes an uneventful recovery. The Albendazole is stopped after about 18 months. Over the next five years, we follow his chest x-rays and with an occasional CT scan. The remaining right lung spot doesn't disappear, so it is removed with thoracoscopic surgery. It indeed is a small hydatid cyst but sterile and would not have caused problems.

His "near death" experience no doubt affects him and perhaps makes him even closer to his religious faith than before. Beyond that, he's very proud to be an American and considers himself most fortunate - and I think we'd agree.

Comment: A case such as this never leaves me. I feel fortunate to have spent two years in Afghanistan and a year in Saudi Arabia practicing medicine. With modern travel, we can see diseases such as malaria, worms, etc arriving back here where we are very unfamiliar with the diagnosis and treatment. With travel medicine clinics and referral areas of expertise like the CDC we can get the backup we need - if we ask the right questions and make the right calls. This one to Jerusalem certainly helped my patient - and me.

I did present this case at a local chest conference as an unknown - and of course didn't mention the patient's name. The Pulmonary Fellow really struggled with the presentation and couldn't come up with a diagnosis: the reason being that he forgot to ask about "Travel History". Once it was clear that the patient had lived and traveled in the middle east, the diagnosis wasn't so hard. It reminds me that our lives and medical care are really accidents of zip codes - a lottery over which we have limited control. But Abdul managed to win the lottery. His father had told him the streets of America are paved with gold. Abdul tells me this and smiles, "And you know they really are!"

Dying Wishes at Lunch

Larry surprises me during a visit to my pulmonary clinic in 1983. “Doc, I want to take you out to lunch. There’s something I want to discuss with you.”

I am a little nervous about the invitation coming from my patient, because he is a super salesman, very nice but a take-charge type of guy. I’m concerned he might to try to sell me something - and in a way he is. He's a favorite of mine coming across as a bit crusty but a straight shooter.

Larry has severe COPD (Chronic Obstructive Pulmonary Disease) and is in a downward spiral. He always appears well groomed and has a piercing gaze which acquires a twinkle when he cracks one of his frequent jokes. But Larry was serious this time. He’s just been discharged from the hospital after another of several flare-ups with severe wheezing, gasping and coughing due to infection.

His waterfront home on Vashon Island where he lives alone at age 77 is becoming a prison to him: “Doc, I can’t handle the stairs, go crabbing, or even lean over to dig clams. This is the pits.”

“Larry, how about hiring someone in or moving over to Seattle to be nearer your family and medical care”

“No way, I don’t want to move and bother my sons or have some stranger in my home!”

On examining Larry, I can see the distended neck veins and the barrel shaped chest. It is apparent that the lungs were over expanded and the diaphragms flattened and moving poorly. There is a trace of swelling in his legs. The blood gases now show an elevation in his carbon dioxide. He can’t breathe sufficiently to either maintain oxygen or get rid of the CO2. Chronic respiratory failure due to chronic tobacco abuse is his long standing diagnosis. He finally kicked the habit 5 years earlier which helped some but not enough. He hates the oxygen tubes and prednisone side effects. He now has the “moon face”, bruising of the arms, muscle wasting, and weakness – all the scourge of chronic prednisone use.

A few weeks after his request I arrange to meet Larry at a restaurant nearby the hospital. After some pleasantries, Larry lets he know that he wants to talk about dying: “Look, I’ve lived a long time and what I’m doing now isn’t really living. These flare-ups are torture, I feel like a fish out of water and I don’t want to die that way. My biggest fear is suffocating to death.”

We talk about ventilators to support breathing, “No”; ICU care with aggressive support, “No”. Larry is clear; he wants to be in control. “Look Doc, all I want you to do is promise me that you’ll help me at the end”.

I continue to listen as he explained his feelings and fears. He doesn’t appear significantly depressed but I need to check. “Larry, do you feel sad or depressed?” He replied, “No, just mad as hell that I can’t get better.” Actually, I guess that Larry had very natural “situational depression” from his illness and I suggest a low dose antidepressant, but he refuses - perhaps avoiding some unpleasant medication side effects.

So we talk options for what would now be called aggressive palliative care. “Larry, look I’m not Dr. Kevorkian but there are options. Other than the meds and oxygen you are using to help breathing, the best drug at the end is morphine. This drug takes away the suffocating feeling, treats any pain, sedates you and produces euphoria.”

“Well doc, that’s what I want”. I reply, “Larry there’s a significant catch, it would likely speed up your death by several hours or even a few days. If the intent is to relieve suffering in a terminal patient, drugs like morphine are acceptable even it might hasten death.” Larry responded, “Look, I just want to die comfortably. I know I’m dying so what’s a few hours or days in my condition.”

Later I discuss this with a few of my colleagues and got mixed responses. One says, “There are situations that you just can’t jump into. This is too close to assisted suicide.” Another replies, “The patient should have the right to ask for help from suffering. After all, since you can’t cure him you are at least obligated to relieve his intolerable symptoms.” A Swedenborgian scholar friend of mine has a broad view, “Everything in the Lord’s Providence has a reason and the Lord is always trying to bend bad toward good. Your patient is suffering and you have tools to help him. I don’t think it wise to abandon him in his need. He will be transitioning from this life to the next soon. Why needless suffering?”

Larry and I make a tentative agreement to comply with his wishes at a future date when another inevitable severe flare-up would occur, but I tell Larry that he needs to discuss his wishes and hopefully get buy-in from his two sons, one an attorney and the other a veterinarian. A week later I confer with Larry and his sons who are in agreement with their dad, and then we wait.

In the dark Seattle Christmas season, my pulmonary practice always seems busiest. Pneumonias are at a peak, new lung cancers are being discovered, and the sad stories seem relentless. My anger at the tobacco companies flares. It seems like a losing battle seeing the continuing ravages of tobacco abuse. But my spirits are buoyed by a recent significant skirmish that we won. (Smoking was finally banned at my hospital in 1983. Prior to that, doctors smoked while making rounds and families smoked in patient rooms. When I was chair of the Smoking Advisory Committee of the American Lung Association of Washington, we surveyed all of the hospitals in the state and found that only a few had a policy and none had completely banned smoking. Now, of course, smoking in any public place is banned.)

At dinner two days before Christmas, I am called by the ER doctor. Larry is there. Shortly thereafter at the bedside I see that Larry looks awful, but is able to give me that piercing look and says in a rasping voice, “Remember what you promised.” So I once more review the plan and he nodded. Normally he would go to the intensive care unit, but tonight we bypass the ICU and admit him directly to medical unit with a do-not-resuscitate order.

I pull the nurses aside and explain the situation. Fortunately, they are experienced senior RN’s, a model of their profession’s caring and competency. They tell me that they had seen too many cases where CPR was used on terminal patients. “Doctor, I’m glad you don’t view death as a failure in this situation.” Bless the nurses, they were looking after me!

The morphine drip is started, with small supplemental boluses. His sons are there. Larry seems peaceful and I say prayers for him silently on the drive home. I sleep well until the phone rings. Larry passes from his world at 3:14 AM.

Comment: The ethics in Larry’s death revolve around my intent (Arch Intern Med 1996;156:1785–1786.). In 1983 when I was caring for Larry, there were no clear guidelines so I had to function from my own training and personal comfort zone. In recent years, specialty societies have published guidelines (American Journal of Respiratory and Critical Care Medicine 2008;177: 912-927) but they are still not totally clear – hence the recommendation to use ethics committees in difficult cases. The type of sedation we used for Larry has more recently been referred to as "palliative sedation," which avoids the implication that it is the sedation that is causing the patient's death.

The intent with palliative sedation differs from euthanasia or assisted suicide. Palliation is used to relieve severe suffering, whereas the intent of euthanasia is to cause the patient's death. Some have derided this “dual intent” as splitting hairs. However, there is a unanimous 1997 U.S. Supreme Court ruling noting these distinctions, which may give significant legal support for this type of sedation (13Vacco v Quill. 521 U.S. 793 (1997). The change in thinking in bioethics continues to rapidly evolve in this area (Beauchamp, The Right to Die as the Triumph of Autonomy, J Med Philosophy, Dec 1, 2006).

I was deeply touched by the letter of thanks from his two sons that came a few weeks after Larry’s death, “Dad would love to shake your hand if he could; he died the way he wanted to. Thanks and God bless.”

A Cat on the Bed

There's a new book out about a special somewhat clairvoyant cat named Oscar. Dr. David Dosa works caring for Alzheimer's patients in a Boston nursing home. The uncanny cat, Oscar, is noticed by the staff to have an unfailing accuracy in being able to identify a patient in their last day of life. Although Oscar is amazing in his talents, no one really knows how he knows when death is imminent.

Dr. Dosa's story is about more than Oscar. He delves deeply into the family/patient/staff interactions with pre-terminal and terminal Alzheimer's patients and families. It was very hard for many families to accept that their loved one was dying and that hospice care was the best option at the end. There are sad poignant stories of families wanting to "do everything" including intensive care, ventilators, and CPR.

This brings to mind a patient of mine I'll call Frank. He had very severe chronic lung disease and was on oxygen. Every time I brought up end-of-life care he'd get mad at me, "Listen doctor, I'll decide that when the time comes. I don't want to think about it. And if I'm unconscious then my kids can decide what needs to be done."

"Frank, have you talked about your wishes with your children?"

"No, but they love me and will know what to do."

A few months later, Frank severely gasping called 911 when he couldn't breathe. The medics found him blue with shallow breathing, so they put a tube down his throat into his trachea. He struggled so much they had to heavily drug him. At the hospital he was placed under my care in the ICU. He had pneumonia which was very slow to clear. After about four weeks on a ventilator, it was clear that this was going to be a much longer trial for Frank. A tracheotomy was done along with attempts at nutrition and muscle strengthening. Attempts to wean him off the ventilator were unsuccessful.

During all this, the family was struggling. What would Frank really want? Was he in pain? Communication was difficult because he was so agitated when awake. Finally he was sent to a chronic ventilator facility where they specialized in cases like Frank's - trying all the means to keep him going.

Indeed, Frank spent two more months there before succumbing to a stroke. His end of life wishes were unknown. It was very hard for the family to limit care. So by making no decision, Frank had really decided on the default choice - do everything.

Comment: Now there's some hope for the benefit of end-of-life discussions in the research field. In a 2008 article in the Journal of the American Medical Association the authors report the following: "The researchers found that such discussions were not associated with higher rates of major depressive disorder or more worry, but these patients received significantly fewer aggressive medical interventions near death: lower rates of ventilation (1.6 percent vs. 11.0 percent), resuscitation (0.8 percent vs. 6.7 percent), and ICU admission (4.1 percent vs. 12.4 percent). Patients who had end-of-life discussions had earlier hospice enrollment (65.6 percent vs. 44.5 percent), and longer hospice stays were associated with better patient quality of life, while more aggressive medical care was associated with worse patient quality of life.

Patients who reported engaging in these conversations were significantly more likely to accept that their illness was terminal, prefer medical treatment focused on relieving pain and discomfort over life-extending therapies, and have complete a do-not-resuscitate order.

Caregivers of patients who received any aggressive care were at higher risk for developing a major depressive disorder, experiencing regret and feeling unprepared for the patient’s death, compared with caregivers of patients who did not receive aggressive care. They also had worse quality of life outcomes, including overall quality of life, self-reported health and increased role limitations. Better patient quality of life was associated with better caregiver quality of life at follow-up.

'Our results suggest that end-of-life discussions may have cascading benefits for patients and their caregivers. Despite physicians’ concerns that patients may experience psychological harm due to end-of-life discussions, we found no evidence that they were significantly associated with increased emotional distress or psychiatric disorders. Instead, the worst outcomes were seen in patients who did not report having these conversations." the authors write.

"Given the adverse outcomes associated with not having end-of-life discussions, there appears to be a need to increase the frequency of these conversations. By acknowledging that death is near, patients, caregivers, and physicians can focus on clarifying patients’ priorities and improving pain and symptom management.'"
(JAMA. 2008;300[14]:1665-1673.

Although the research, as above, is showing that end-of-live discussions can benefit both patients and caregivers, the medical profession has a long way to go. We are taught to diagnose and treat. Now prevention of illness, life styles, etc. are being rightly promoted to enhance quality and quantity of life.

However, death, the "D word", is still not effectively dealt with in our medical system. Death is universal yet achingly difficult emotionally to bring up and discuss. The time though to have these discussions with our loved ones begin is when we're healthy and in our right minds.

When Oscar eventually approaches our bed, we need to be ready. Can we say, "I've lived a full life, told everyone that I love them, apologized to those I needed to, and come to peace with my maker?"

Let's hope we can smile when Oscar hops on our bed and snuggles up close.

Pull the Plug - Now!

The voice on the phone is demanding, quavering, pleading; "Dr. deMaine you are going to be the attending Critical Care doctor when our mother is transferred to your hospital today. She needs to be taken off the respirator and allowed to die. We have been pleading with the doctors at the burn center, but they keep doing more procedures. We want her off life support as soon as she gets there."

I am a bit taken aback since I'd never heard of the patient, let alone that I am destined to be her doctor in the ICU. I tell the family to try to come to the hospital to meet with us today when the patient arrives.

After hanging up the phone, I receive the call from the transfer coordinator, "We have a patient in the burn unit who is 87 years old. She has 50% third degree burns and 20% second degree burns. There have been multiple grafts. The complications have been pneumonia, stroke, heart failure and adult respiratory distress syndrome. The house staff will fill you in. They think she should stay there but the family is very unhappy that the patient is suffering through all these treatments."

In thinking about this, I first reflect that it's not too uncommon that the family feels left out in critical care decision making. They sometimes don't understand that a sick relative may look awful, but still have a good chance of recovering. Lots of communication is needed, but the doctors often don't have the best skills in this area. It can be very hard to switch from a "curative mindset" to a "palliative mindset" for all involved.

I'm paged to the ICU when she arrives by a life-support transfer Medic One team. This unfortunate woman has now been on life support for one month. There are multiple burn grafts in place. She is unresponsive to verbal or physical stimulus. The blood pressure is marginal, kidney function poor, liver functions elevated, and chest x-ray diffusely abnormal. If this were a young patient, there might be some hope but this woman is going progressively downhill. It's a tough call. If the patient could talk to us, what would she want?

I meet with the family, the new RN team, and our social worker in the conference room. After introductions, I give them an outline of the process we'll use over the next half hour to go over their Mom's care. First we review all the medical facts and time-line of hospital care so far. Then I outline the options for further care including discontinuing care - and the benefits and burdens of each possibility. We discuss her daily life prior to the the fire. Her son and daughter, Jerome and Ada, describe her as a happy person yet very limited over the past few years due to arthritis. She was feeling isolated because all her contemporaries had died.

We then discuss her wishes for aggressive life support care. She has never signed a living will or executed a durable power of attorney for health care. Her husband is deceased, so under Washington State Law, the decision making authority rests with her two adult children who must agree. Both are adamant that Mom would never have wanted this kind of aggressive care. She has been ready to "pass on" for some time.

Jerome says, "It's bad enough to die once in a fire, but dying every day in the ICU is just too much!"

After nearly an hour of discussion, which includes the nurses and social worker, all agree that the most reasonable course of action is to remove the ventilator. I ask the family to step out of the room. Then I turn off the monitors in the patient's room, make sure the patient is still unconscious, turn off the ventilator and remove the endotracheal tube.

Jerome and Ada are at the bedside holding mom's hands as she exits from this world. There are tears of both sadness and joy. The nurses and social worker continue to be supportive and help the family with the arrangements that need to be made.

Comment: About 70% of all deaths in American ICU's occur by removing life support. Heroic attempts have been tried, but ultimately failed. Medicine is slowly facing the issue of futile and expensive care at the end of life - a time when patients, families and medical care givers often prolong care far beyond a reasonable limit.

The day after the patient's death, I got a call from the surgical resident in the burn unit, wanting to know about the patient's grafts and general status. I explained as gently as I could that we removed life support at the request of the family. The surgical resident wasn't happy.

The Unasked Question

A large heavy package arrives at my office. I am expecting these charts after my conversation with the attorney. He says, "Doctor deMaine I'm representing a prestigious teaching hospital related to the death of a firefighter." He briefly explains the issues involved and I agree to review the case.

The large cardboard box contains hundreds of xeroxed pages from treating doctors outside the hospital as well as the complete hospital records and the autopsy report. I lug all this home and spend hours that evening sifting and sorting the time-line of events. This 34 year old female fire fighter begins to notice being easily winded with exercise over the past few months. She sees her internist who checks her heart and lungs including a chest x-ray and EKG. Her heart rate is 90 which is within a normal range but higher than her usual rate of 70. This isn't enough to raise red flags at this point, but there's no diagnosis for her symptoms.

Subsequently, at a house fire, she needs to scale a ladder carrying a hose. Becoming very winded, she collapses and has a brief "seizure-like" episode. Medic 1 responds and she is taken to the hospital where she is evaluated in the Emergency Room. Her blood pressure is fine, the heart rate is 110, and the neurological exam is normal. The neurology service admits her for observation and a "seizure workup".

After two days in the hospital, no diagnosis is established. The shortness of breath history is noted. The pulse rate never drops below 110. She is discharged from the hospital with an appointment made for the Pulmonary Clinic in two weeks. Three days later, she collapses in the shower. Her roommate calls 911 and she's brought to the hospital where the diagnosis of a pulmonary embolus is established. She has sudden cardiac arrest in the x-ray department and dies undergoing CPR.

I notice from her outpatient record buried in her history that she has been taking birth control pills for the past six months. Also there is no history of asthma, allergies, or any lung disease.

The attorney is not happy when I tell him that I can't testify for the defense. "Settle this one and try to stay away from a jury", is the best advice I can give him. "Look," I said, "if you framed the case with the appropriate information, any emergency physician would come to the diagnosis. You have a young woman on birth control pills who is becoming short of breath. This is a red flag for blood clots beginning to clog up the lungs. They start in the upper legs and pelvis and have no local symptoms - but can be deadly as they break loose and are pumped through the right side of the heart into the arteries of the lung. The heart rate goes up and I'll bet you anything that her neck veins were distended (from the obstructed blood flow). The 'seizure' was a red herring. The patient shouldn't have been discharged without a diagnosis. Basically no one put two and two together. The right questions weren't asked, so the right diagnosis was never considered. A simple lung scan would have made the diagnosis and anti-coagulation would have saved her life."

The attorney angrily told me that I wasn't being helpful to the medical profession and that I was prohibited in testifying for the plaintiff (which I very much dislike to do anyway).

Comment: This was a very sad death and very preventable if only the diagnosis of pulmonary embolism had been considered. There aren't many things that cause a young woman on birth control pills to have severe shortness of breath, but this patient was felt to have a brief "seizure" and admitted to the neurology service. Each specialty tends to focus in its area and we naturally try to fit the patient into a learned pattern. The smartest doctors I know (please count me out) have a sixth sense in a case like this; an ability to step back and ask the right questions.

All this said, I don't think the doctors sued were bad. Dragging them through the courts when they all felt guilty and terrible doesn't help anyone. I hope that in tort reform that we can develop expert review panels who can review a case like this, bring about a fair settlement, and not clog up the legal system for years with expensive "expert witnesses", discovery, rulings, appeals, etc.

Talking to God?

"We have a patient on a ventilator here who is stable enough to transfer to your ICU, if that's OK with you."

This call, coming from the transfer coordinator, is emblematic of an insurance generated "medical care" coverage issue. I am salaried under the patient's insurance carrier so the insurer wants this patient back under its wing as soon as possible.

The community hospital has been keeping Stella Norris (not her real name), an 89 year old woman, as long as possible. She is incapacitated from a massive stroke suffered five years ago. There is a feeding tube inserted through the stomach wall and she has needed total body care. 911 was called when she stopped breathing and she was taken to the closest hospital

The daughter, Janice, seldom visits because there is no ability to communicate. The home care has been given over to George, a step grandson. George has devoted his life to care for Stella, day and night in his small apartment. He believes in miracles, he says, and spends his days reading about alternative medicine and watching televangelists on TV. He says God speaks to him.

The precipitating event at the community hospital, I learned later was this. George is found in the ICU briefly disconnecting the endotracheal tube from Stella's ventilator and inserting herbal medications down the tube. The alarms go off, and the nurses are so horrified they now designate the patient stable enough to transfer. Yes, loss of income for the hospital, but a real problem patient out of their hair. The are delighted to "turf" (medical slang for passing your problem patient to someone else) to another institution!

I admit the patient, get the scoop from the referral medical team, and examine this very debilitated ill woman. She does not respond to any verbal or physical stimuli, has shrunken limbs on the right side of her body, has bed sores, infected urine and a pneumonia on chest X-Ray. I confirm with the family that she has not been responsive or out of bed for five years. They are, however, delighted with the care that George has provided.

At our first family conference the daughter, Janice, seems reasonable and understanding when I probed the issue of Stella's wishes for invasive care. Stella had no known Advance Directives. Janice says, "I'm really not sure Mom would have wanted any of this. I'm certain I wouldn't want any extended care like this for myself, but..."

I explain in detail the poor outlook and the process of prolonging death that we are providing with a ventilator, tube feeding, antibiotics, etc. There is a dynamic that I can't figure out. George has sat there quietly with a scowl on his face, never nodding in agreement.

"George, what are your thoughts?", I ask.

"I know she's not ready to die. God and spoken to me and I know."

Janice and other family members subsequently back off, don't want to attend family conferences and defer to George. They seem intimidated to challenge him, even though I say he may not be acting in Stella's best interest.

The next week George posts a notice in the ICU for Stella's memorial service - more than three months hence! I try to confront George and the family to no avail. We discus the case at our Ethics Committee of which I'm a member and they designate the Hospital Chaplain to try to intervene. He's unable to connect in a rational way with George. The Ethics Committee reminds the daughter Janice that under Washington State law, that she has legal decision making authority. Janice again defers to George. Our legal department is contacted and says we could try for a court appointed guardian, but that the guardian would be very unlikely to go against the wishes of the daughter.

After eight weeks in the ICU, Stella begins to worsen. The circulation begins to fail and her entire left arm becomes cold and gangrenous. The orthopedic surgeon does not offer amputation because she wouldn't survive surgery.

About this time, I finally tell George that I'm unwilling to do CPR or electric shocks to restart to heart when it stops. He gives me a vacant look devoid of visible emotion. It gives me the creeps, and the nurses begin to freak out wondering what George will do at Stella's death.

The end is weirdly peaceful. She dies with George there. The heart has flat-lined but the ventilator continued to puff fruitlessly. I receive this call in the evening at home. I'm beat and really don't want to drive back in but I need an experienced forceful doctor at the bedside.

Then I remembered my buddy Don, the burly no-nonsense ER doctor was in house. "Don can you do me a huge a favor?"

So after asking the nurses to call Security to have them present in the ICU, Don agrees to pronounce Stella dead, and to tell George (I think I still owe Don for that one).

George receives the news, leaves the hospital, and we never hear from him or the family again.

Comment: Futility, is medically hard to define, except in extreme cases. This one qualified in my book, but we felt impotent because of hidden family dynamics and mental illness. Was George really crazy? Was a social security check or other financial benefit a motivator? Did George control the inheritance?

A few years back, Dr. John Luce wrote an article in the medical journal "Chest" where he compared the care of this kind of patient in France and in the USA. The French doctor said, "No problem. In France, we have limited visiting hours and the families look to the doctor to make decisions. When the family is gone and we've had discussions, we simply remove the ventilator and let the patient die peacefully." In contrast, the American doctor said she "tried for consensus and shared decision making giving autonomy the highest value. In some situations consensus was difficult or impossible to achieve".

The problem in this case was that the patient's wishes were never known, the care was futile, and everyone lost out.

My Afghan Introduction to Medicine

Najiba is ill with weeping infected blistering lesions around her mouth which should be easily managed with an antibiotic. I can only hope that the Afghan medic knows what to do. Her child looks even more ill. They have been waiting with forlorn patience for hours sitting on the ground outside the clinic in Laskar Gah in the desert heat of the Helmand Valley . As a medical student in 1962 working for the summer in Afghanistan, this is my introduction to medical deprivation.

The Afghan medic briefly looks at her and the child, writes a prescription on a piece of paper in Farsi and says, "next." As an observer collecting research data on infant mortality (continues at 30% within the first year of life), I feel so helpless.

Another mother on the way out begs me to look at her child's ear. An otoscope is produced, and I see a protruding black object from the child's left ear canal. Somehow with a hemostat I manage to remove a wriggling cockroach. One small thing accomplished but such a feeling of overwhelming impotence.

The child drinks water is from an open ditch. She has round worm (Ascaris) infestation in her bowels, a nearly universal finding in Afghan children who have no access to clean water. Immunizations are rare with smallpox common. In the late 1960's Peace Corps female health workers travel from village to village with an eradication campaign. Small pox disappears from Afghanistan but water born diseases remain.

Infant diarrhea and dehydration leading to death is all too common. Two ex-US Army nurses enlist as Peace Corps pediatric nurses in a Kabul Hospital. I watch them with disbelief. The child arrives sick, dehydrated and with a weak cry. He needs intravenous hydration but no IV fluids are available. Also not available are sterile needles, saline, or gloves. The nurses boil up a saline IV formula along with some reusable needles and 50 cc syringes. Bare handed they place their finger over the end of the syringe, pour in saline, attach a needle, and inject the saline into the infants left flank. They then repeat the procedure with the same needle, into the right flank. The infant is gently handed back to the mother who left with a glint of hope in her eyes, saying "Tashakor." (thank you).

A woman from a village 50 miles away is pregnant for the 8th time, having lost four of children to illness. But this time she is very ill, at term pregnancy, in labor yet having severe abdominal pain. The Afghan doctor says, "It's likely a ruptured uterus." The visiting American OB-GYN says, "No one could walk in with a ruptured uterus, it must be an ectopic pregnancy." At surgery, the uterus has indeed ruptured and the child amazingly survived. They explain to me that Afghan women lack Vitamin D, have no sun exposure, and lose calcium from breast feeding and multiple pregnancies. The bones soften and the birth canal narrows, leading to the obstetrical emergency and uterine rupture.

The young man lies on a charpoi bed in a remote village near Maimana in the north. His father pleads with me to come to his house and examine him. The young man is thin, a teenager, embarrassed and uncomfortable - possibly in pain. The father gently lowers the bed cover and I'm shocked. The left testicle is hugely swollen about five times the normal size. It is rock hard with similar hard enlarged lymph nodes in the groin. The cancer is obvious, even treatable. Again I'm helpless as a medical student. I write out the name of an excellent surgeon at Kabul's Avicenna Hospital and advise the father that some help may be available there. I told him I hope that his son does well. The father nods, and adds accepting his fate, "Enshallah." (Allah willing).


Comment: I returned to Afghanistan in 1965, a relatively peaceful time under King Zahir Shah. In the US Public Health Service assigned to the Peace Corps, I provided health care for the volunteers and did programming work for the volunteer health care workers. The Afghans were eager to learn English and improve their skills in many fields. Yet they were proud and naturally mistrustful of foreigners given their history. We were "non-believers". The stories of the British invasion and occupation in the mid 1800's were passed from generation to generation. They are a proud people, tribal, and independent. Yet we found them respectful and friendly with a quick smile.

I have great respect for the young Americans serving in the Peace Corps. These were the early days of the Peace Corps when Sargent Shriver was still the director. I still remember my interview with him and the huge picture of the Kennedy family hanging on his office wall - quite daunting.

Dysentery was a way of life for the Peace Corps there and bowels became the frequent subject of complaint and humor. There were amoeba, giardia, salmonella and, of course, worms.

An urgent call comes from a volunteer, "I've got worms, I've got worms."

"OK, it's OK. We've got really good medication to get rid of them. Come on in."

She replies, "But you don't understand!"

"What don't I understand?"

"It's only half way out!"

These round worms are often not a major issue and pretty easily treated. But they can enter the appendix or bile duct and cause acute obstruction - or even at times bowel obstruction.

We hear a lot about war injuries, but lack of basic medical care continues to be a huge problem. An Afghan doctor is trying to open a large outpatient urgent care center in Kabul, built with funds from the US military. But the local university medical graduates have very marginal training. 80% of them failed a very basic skills test and none of them had ever sewn up a laceration. There's a long road ahead, even after the violence ends.

Doctor for the Family?

In retirement I get a lot of inquiries from family and friends about aspects of their disease or care. I try not to be intrusive and to mostly provide reassurance, but sometimes I just can't stand by.

My sister in rural Pennsylvania had severe shortness of breath and also a known leaky heart valve (stable since childhood). A local cardiologist told her she needed three heart valves replaced! This was so over the top that I "interfered" by advising her to head to the relatively nearby Cleveland Clinic (her records didn't arrive so it took two trips there). The description of the consultant she saw there was a disinterested "cold fish." He told her no surgery was indicated and that all she needed to do was lose weight. He saw no need for further tests or referrals.

So I entered the picture again and contacted a Pulmonologist at the University of Pittsburgh Medical Center who was very gracious. Doctor's don't necessarily look forward to being the third consultant - basically a referee. He did pulmonary functions, CT scans and cardiac ultrasounds to evaluate her pulmonary hypertension. My sister subsequently went all the way through a thoracoscopic lung biopsy which unfortunately showed a serious irreversible lung disease.

I check in by phone with my sister every few days. She is now on Hospice care, but the Hospice base is 40 miles away and several different RN's have been to see her. In treating her shortness of breath she got pretty goofy and unstable on the sedative/narcotic regimen so they put her in the inpatient Hospice Unit for a week. She's pretty much bed ridden, on oxygen and at peace with her Swedenborgian-based belief that she will pass into a vibrant spiritual world in a much more perfect disease free spiritual body. Although, she's reluctant to leave her beloved husband and extended family behind, she's actually looking forward to the day when all of the family can be together once more.

After coming home from the Hospice Unit, she casually mentioned that she really felt hyper, shaky and jittery. After reviewing her meds, we discovered that she was on both long acting plus short acting asthma inhaler meds. So I said, "check with your medical team, but for now stop the short acting albuterol and use it only as needed." The jitters resolved. I told her to send my apologies to her team and that I'm sure they would have given the same advice.

In general, doctor's are advised not to provide direct care for their families. That certainly didn't apply to our family when we were kids. My father was an "old time" family doctor. We got our ears checked, penicillin shots, etc. by going into dad's office after school.

I stitched up my son's laceration at a Boy Scout Camp when I was the doctor there. It would have been silly to send him back to Seattle. Of course my son, now a lawyer, still points to the scar on his knee and says, "boy, you can sure tell that wasn't done by a plastic surgeon."

The label of nurse or doctor carries over to the stage of retirement. I hope I have been of some help to my sister, but it's a balancing act. In general, being supportive with bits of education and reassurance is probably the best medicine we have to offer for our own families.

You Killed My Mother

Martha's death was difficult but not unexpected. She had been in and out of the hospital three times in the past six weeks. Her husband was there at the end.

About an hour later I got a call from the son, John, who hadn't been around much. He was upset, angry and aggressive: "Doctor, you killed my mother. I want to meet with you and my lawyer there today!"

I think I must have surprised him by saying, "OK, how about 2PM today." I had just had a patient cancel and had a free spot. I arranged for a conference room since I didn't know how many people would be there. Medically, I didn't think I had done anything wrong and was reluctant to generate unnecessary tension. I was pretty certain that if I called the hospital's legal department that they would want to meet with me first and then perhaps be at the meeting. I had been in enough contentious situations over the years and I didn't want this one to escalate. Also, I had enough gray hairs, that I'd been in similar situations before.

So I ordered up all Martha's records and took them to the third floor conference room next to the library. Four family members arrived: John the son, Ed the husband, Mike the brother-in-law and Sue the patient's sister.

I asked whether their attorney was coming, and Mike said he was a tax attorney in Seattle. So John's initial challenge that he would bring "his attorney", turned out to be a family member who wasn't a litigator.

After introductions, I asked John what the concerns about his Mom's care were. He said, "I think she got too much morphine and it killed her."

I said, "Let's review the care and medications she received. Here's the chart so we can all review it and look at the medication record and the nurses notes. First, I think we all know that Martha had very severe emphysema. At age 81 she was weakening and her lung function worsening despite aggressive use of prednisone and inhalers. Infectious were vigorously treated with antibiotics. But she was slowly suffocating even with oxygen."

Mike said, "Well, where does the morphine come in."

"Good question. Martha had decided that she didn't want to be put on a ventilator because her quality of life was so poor, yet she felt panicked often being unable to catch her breath. She said she felt like a fish out of water. Indeed her carbon dioxide was climbing and her oxygen levels falling in her blood gas samples. The end wasn't far away."

"OK, but what about the morphine, John interjected."

"Well, your Mom was becoming agitated, not making sense and beginning to cry out. At that point I talked to your dad, John, and told him morphine or a sedative would help your mom be more comfortable. So we began small doses to try to titrate care for comfort."

Mike said, "Is that right dad?"

"Yes, Martha was so uncomfortable and so confused. I felt we needed to do something to make her more peaceful. I felt she was dying."

"Let's look at the medication sheet. The nurses gave her 1 or 2 mg of morphine at each of these times over a three hour period. The total dose was 10 mg. Although it could be argued that the morphine speeded up her death by a matter of minutes, the morphine did several positive things for her. It releaved her anxiety, decreased her sense of shortness of breath, and basically relaxed her as she drifted into sleep and ultimately death. Without the morphine she would have had a miserable death."

By this time John had calmed down and began to realize that he needed to support dad. I relearned the lesson that all family members need to be involved at the end - with active outreach if they aren't there. There seems to be a direct correlation between anger and distance from care.

Comment: Over the years, I would come to insist that all family members be involved in the family conferences. We would arrange conference calls so "Aunt Minnie for Minneapolis" could be there and feel included. Allowing all concerns to be addressed, transparency of medical records, admitting our own shortcomings, and wishing that things were different were all "OK." In this case, I should have brought John, the son, in much earlier so he could understand the issue of palliative use of morphine in end stage lung disease.

Viva Puerto Villarta

Mike sat by his mother's bed saying to her, "Mom, hurry up and get well so we can go to Puerto Vallarta. The problem was there was no way to tell if anything was registering with Midge Jackson, his mom. She was on life support, heavily sedated and things didn't look good. Mike was a firefighter and an upbeat take charge kind of guy. He was with Mom most of the time he could spare and, as her doctor in the ICU, Mike and I were in frequent communication.

I first met Midge and the family in medical crisis - unfortunately not an unusual mode of initial contact in the ICU. The call came from my surgical colleague, "We're sending a patient to the ICU post triple A and her pressure is falling, could you see her?" The "triple A" was an abdominal aortic aneurysm replacement in this 76 year old woman. The large trunk of an artery in the lower abdomen was threatening to burst and she had just survived the urgent surgery which replaced the aneurysm with a sizable graft.

Initially Midge seemed to improve and we thought she was stabilizing, but then an intestinal bleeding occured. After transfusions, the GI specialists found stress ulcers in the stomach and got the bleeding stopped. She remained on life support and the lungs began to fill up with fluid. It became hard for the ventilator to give enough support. We were concerned about both giving too much oxygen or too little, too much pressure or too little ventilation. It was difficult but I advised the family that she had a reasonable chance of pulling through this new complication, adult respiratory distress syndrome.

At week three in the ICU Midge's lungs weren't much better, but they weren't worse either. Mike the firefighter kept saying to Midge, "Come on Mom, get better. Remember we're going to Mexico." Midge was minimally responsive to her environment but could open her eyes and more all her limbs, so the family knew she was still there.

At week five after surviving a bout of urinary infection, Midge's temperature spiked to 104 and the blood pressure and urine output fell drastically. We did the usual cultures and big gun antibiotics but in a day found out that a yeast type fungus was growing out of her blood. We began antifungal medications, but things didn't look good.

Some of the staff began to wonder if we were really doing Midge a favor keeping her alive this way. Mike, however, correctly stated "I don't think you can say she has a terminal diagnosis. Do you think so?"

We had a family conference frequently and were almost at the eighth week now. Some family members began to say that they weren't sure that Mom would want to keep going like this. But they, and Mike, all described Midge as a vital person who really loved life and "wasn't going to go easily."

I felt that the family was indeed acting in Midge's best interest but told them that if there were further complications like a stroke or heart attact or kidney failure that the outlook would be grim.

One day in the 10th week, like magic bowel function returned, the blood count normalized and then the lungs began to clear. We lightened sedation and she began to respond and have weaning trials off the ventilator. Mike kept saying, "Mom remember, it's Puerto Villarta for a cerveza."

After nearly three months in the ICU, we celbrated the day that Midge was able to transfer to the medical unit under the care of the Hospitalists there. It felt like a graduation, but the attempts at rehabilitation had just begun. She needed lots of strengthening and physical therapy. At that point I lost track of Midge except that I heard subsequently that she had gone to a skilled nursing facility for intensive physical therapy.

Comment: Unlike many elderly ICU patients Midge's case was gratifying and one that the nurses and I would long remember. It was humbling to think that we were becoming discouraged and wondering if it was wise to push on with life support. The huge advantage that Midge had is that she didn't have a terminally disabling medical condition. Her care could never be called futile.

My readers may not believe the following, but it is absolutely true! In the spring of the year Midge was discharged from the hospital, our family was at the airport boarding a flight to Puerto Vallarta. Out of nowhere and from across the lobby, I heard a shriek, "There's my doctor, you saved my life." I only saw a nice white haired well groomed elderly lady and wondered whom she was calling out to. Then I saw Mike, and began to recognize Midge with a smile. We were on the same flight to Puerto Vallarta and even managed to have a cerveza together there.

Going to the Prom

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.

Seeing Through Another's Eyes

I have tried to write a letter of thanks but don't know what to say or even how to begin. I don't know the persons I am writing to, but part of their loved one is literally now a part of me.

It began with a phone call from my brother. "Jim, what the hell is Fuchs' Dystrophy anyway - do you have it too?"

I racked my brain and tried to dust off distant learning from medical school, but I had to turn to the internet for answers. My brother had begun to have hazy vision and could no longer follow the flight of a golf ball, or even a hard line drive in baseball. He couldn't read clearly until early afternoon. The problem was that his cornea (the outer layer of the eye) was waterlogged. Blowing a hairdryer into his eyes helped some as did a strong solution of salt water - but these really didn't help enough and the problem was worsening.

His ophthalmologist explained to him that this was an inherited disease. Our parents had passed away and we knew that their eyesight wasn't the greatest in their 80's and 90's but they were never diagnosed with Fuchs. The treatment options were explained to him: no treatment (leading possibly to scarring and blindness), the traditional corneal transplant, or the relatively new Descemet's membrane transplant (called DSAEK which is basically a partial corneal transplant).

The cornea, I discovered, is an absolutely amazing part of our body. This window for our vision has five layers and, with its curvature, provides two-thirds of the refraction needed for clear vision. The innermost layer, the endothelium, produces Descemet's membrane. The function of this region is to pump water out of the cornea to keep it crystalline clear. So basically it's our own sump pump built into the cornea. In Fuchs, the endothelial cells start to die off prematurely and consequently the cornea starts to swell affecting vision.

So my brother underwent the DSAEK procedure in both eyes and had a stunning result. He's now back to golf (without a spotter) and has excellent vision.

But then it was my turn. I thought initially it was cataracts, but it turned out that I was, like my brother, in the unlucky 50% inheritance chain. The procedure felt strange. I was in my own clinic where I knew everyone, but they appropriately kept a professional demeanor asking my name and birth date three times as part of the safety controls. The corneal surgeon had extensive experience and my confidence was high. The procedure was under local with "conscious sedation". After an tiny incision and stripping a button of the ineffective endothelium from my eye, a similar sized button from a cadaver was inserted. An air bubble was then introduced to keep the graft in place while I laid on my back for an hour before going home and lying on my back some more. Amazingly the vision after 4 days cleared, the images were sharp and the halos gone.

So what about the cadaver? I hadn't given my donor much thought until I tried to write a letter of thanks to the loved ones. My transplant of course was pretty minor compared to a kidney or lung transplant, yet every time I open my eyes I'm most thankful that someone was so thoughtful in giving part of themselves to others.

I had so many questions. What happened to my donor? How old? Was it expected or sudden? How can I really express my thankfulness? I've always checked the box "organ donor" on my driver's license but never really given it much thought. But the fact is I now am literally seeing more clearly through another's eyes as I write this. An anonymous gift of one person to the person of another. I still must try to write the letter.

What Comes Next?

William would come to see me every three months for his COPD (Chronic Obstructive Pulmonary Disease). We would chat about his disease, his meds, and his grief. He had lost his wife a few years back, but after 52 years he found it very hard to be without her.

On one visit he said, "Doctor deMaine, can I tell you something - and promise you won't think I'm crazy?"

"Sure."

"Well, my wife and I always had a favorite restaurant where we'd go for our dinner on Sundays. Let me tell you why I still go there. I wear my old tie and coat and sit in our favorite booth. And I swear that I see her. Her image is perfect, younger, and smiling. She looks beautiful. She doesn't really communicate except with the smile - which seems to me to say that she's waiting for me."

Other patients related different views. A very sweet British lady told me on an office visit that she had been a widow now for six months.

"That's sad," I said. "Do you believe in an afterlife?"

"Of course", she replied.

"How about marriage in Heaven?"

"Yes."

Trying to tie this together I said, "That's wonderful. Perhaps you and your husband can spend eternity together."

She paused, tilted her head, smiled and said, "Well, I might play the field a bit first!"

There are many views of the afterlife that vary from none to quite fanciful. Harvard theologian Huston Smith gave his views in the Ingersoll Lecture called Intimations of Immortality. In his lecture he highlighted Swedenborg's expositions on the next life. Also, he discussed the near death experiences in Raymond Moody's book "Life After Life".

Helen Keller with her blindness and deafness was, in those senses, cut off from the world. Yet she was a great student of the Bible and eventually became very influenced by Swedenborg's writings. ... "she began to read much of Swedenborg's works as had been put into Braille:

Here was a faith [she says] that emphasized what I felt so keenly -- the separateness between soul and body, between a realm I could picture as a whole and the chaos of fragmentary things and limited physical senses met at every turn . . . As I realized the meaning of what I read, my soul seemed to expand and gain confidence amid the difficulties which beset me. ."

The 1998 movie, What Dreams May Come, gives a vivid and possibly realistic picture of what Heaven and Hell could be like. The film has many parallels with Swedenborg's book, "Heaven and Hell." We are alive, active, useful, on the go and finding a home with those we love.

Mark Twain's irreverent views on religion are highlighted in humorously poignant ways: "Go to Heaven for the climate; go to Hell for the company." Further, from Captain Stormfield's Visit to Heaven, .."Now you just remember this - heaven is as blissful and lovely as it can be; but it's just the busiest place you ever heard of. There ain't any idle people here after the first day. Singing hymns and waving palm branches through all eternity is pretty when you hear about it in the pulpit, but it's as poor a way to put in valuable time as a body could contrive. It would just make a heaven of warbling ignoramuses, don't you see?"

My education along these lines was when I attended Bryn Athyn College for two years. I was immersed in studies of Plato, Aristotle, and others right up though the age of enlightenment including Swedenborg. The emphasis was that there are two capabilities that make us uniquely human: freedom and rationality. We were charged to wisely apply learning to life - heady stuff for a 20 year old.

So does any of this carry over into medical practice? Since we are all in some sense spiritual beings as well as natural beings, it seems logical for a physician to deal with the whole person, particularly around the time of crisis or death. I'm not one who thinks Christians have the "inside path" or die easier than atheists. I had some confirmed non-believers in my care who died comfortably believing that "that was that." They appeared to be good people who had led good lives.

But others want to talk, are afraid, or have regrets. All hospitals now have chaplains. Many ministers, rabbis, imams and priests make frequent hospital and nursing home visits. Doctors of course, need to respect all beliefs and understand boundaries. Although I loved having philosophical and religious discussions with patients, I always tried to approach it from their interest and comfort zone.

One Pulmonologist in the area here would ask patients to pray with him before he did their procedure such as a bronchoscopy. Clearly this was inappropriate and boundaries were violated (his group let him go). Patients can, at times, cross boundaries but the "power differential" is much less. I had one very sweet lady who was so sad I wouldn't make it to Heaven because I hadn't been baptized in her faith!

If common sense and good will prevail, spiritual beliefs can be an important part of the conversation as we approach the end of life. I encouraged my students, interns and residents to not avoid exploring these areas. Healing occurs on many levels.

Where's the Best City to Die?

When I ask patients about their greatest hopes or fears at the end of life I often get a comment like this, "Well, my biggest fear is that no one will understand what I want and that I'll end up being a burden to my family. I'm just afraid that the people caring for me might put me through hopeless painful attempts to keep me alive and that I won't have any say."

As we go through the discussion, often people haven't even begun to have conversations with their families and loved ones about death. It often takes a scare or serious illness to raise the topic, but then we might not be mentally competent. Even people who've had the discussions might not have completed a Durable Power of Attorney for Health Care or a Living Will. Or if they have done these, they are at times filed away in a hard to access spot and may not have been reviewed for years. Electronic records and national living will registries have now made it possible for some medical systems and individuals to retrieve important information like Living Wills 24/7.

In general medical system defaults to treatment when it comes to saving lives. When 911 is called or you present to an emergency room, if there are no instructions to the contrary (such as a POLST form) and it's very urgent, the medics will understandably and appropriately initiate treatment. But this is similar to having surgery without understanding the risks or benefits. When we have a cancer diagnosis, are very elderly, or have a combination of serious chronic conditions, we may want to ask for limitations in "heroic care". Hence a program like Respecting Choices in La Crosse Wisconsin which is actively reaching out and helping people complete advance directives.

The Gunderson Clinic in La Crosse has shown us a powerful way to bring end of life discussions to a community in such an effective way that 96% of all deaths there have advance directives completed. The main reason for the directives is to respect a patient's wishes. The ethical principles involved are to respect a person's autonomy and to do no harm. There have been significant expenses incurred by the hospital to keep this program operating. Even so, they funded the program because it was the right thing to do.

"At Gundersen Lutheran, the cost of care for someone in the last two years of life is about $18,000. The national average is close to $26,000. At one hospital in New York City, it's more than $75,000."

This is not rationing. There aren't (and never were) proposals for "death panels". But there is a proposal —"it's in the health bill passed by the House of Representatives — that would pay for the kind of periodic and continued end-of-life discussions with patients that are routine in La Crosse. Gundersen Lutheran is pushing for it."

It's encouraging that the medical professions, the public, and now the politicians are seriously engaging in this area. La Crosse is a powerful model leading the way.

Who's in Control?

Vonnie was new to my practice. She came in, looked me in the eye and said, "I'm here because I have sarcoidosis. I've looked you up in the Directory of Medical Specialists, talked to friends at the University, and have decided that I want you to be my doctor. Vonnie, I quickly surmised, would be fun but maybe a challenge to care for.

She taught medical terminology to medical assistants so knew all about pulmonary functions, prednisone, rales, wheezes and all the organs which could be affected by her disease. I was just a young pulmonologist at that time and tried to assert some control or at least set some boundaries. We negotiated what tests needed to be done. She was fine with that, but treatment was another issue.

Her disease was serious and progressive. Sarcoidosis was initially named for its red lumpy skin lesions, the name literally meaning "flesh-like" (sarcasm on the other hand literally means "cutting through the flesh"). Small clusters of lymphocytes can multiply and congregate in literally any organ in the body: particularly lungs, liver, eyes, and skin. Researchers have spent a lifetime looking for the cause such as an infectious agent or some kind of environmental exposure. Lots of theories have led up blind alleys. Most patients with sarcoidosis have a benign course often with complete spontaneous resolution, but then there are some like Vonnie, where it is relentlessly progressing.

Vonnie hated the prednisone side effects. The common ones of weight gain, puffiness, bruising, etc. she could put up with, but the mental effects were disabling. She felt spacey, irritable, and just not herself. It was straining family relationships which were all important to her.

Over time, I learned to listen to Vonnie. She reminded me, "Dr. deMaine, you do remember Francis Peabody's dictum don't you."

I nodded.

"The secret of caring for the patient is to care for the patient."

In repartee I said, Vonnie do you remember what the root word of "doctor" means?

"Of course" she smiled "docere 'to teach'. You're my teacher aren't you?"

As things worsened Vonnie's family rallied. Her strong Catholic faith helped her to persevere. She smiled and said, "We Catholics understand suffering!" The end came quickly perhaps from a pulmonary embolism. The family described her wake to me. Vonnie had it all planned: the music, the poems, the silver, china and crystal - and made a wish that all would raise a glass (or two) and wish her well in her new adventure with God.

A few days after her death, a note from Vonnie arrived in my in-box: "Dear Doctor deMaine, Because it is the aim of the physician to heal and to extend life, it must have been difficult for you to let go and allow me to reject the steroids. Yet without the anxiety produced by the prednisone, my spirit is healed. I am 'myself' again. (I did not ask for a personal healing at Lourdes, only that the cause and cure of sarcoidosis be found.) As a result, I am able to embrace life and to live it with a great degree of peace. My children now know me as I am and can learn that death is a companion, not a tyrant. In your own inimitable way, you've give me the kindest of care - a mirror of love with which God tends me. Thanks and prayers, Vonnie."

Comment: One evening about 9 years ago, I was giving a talk to a patient group about sleep apnea. In the middle of the talk, I crossed my arms and felt a very large firm rubbery non-tender lymph node near my elbow. This alarmed me, but I was able to limp through the rest of the talk. Examining myself at home later, I found enlarged lymph nodes all over my body. Immediately I assumed the worst, non-hodgkins lymphoma (cancer of the lymph system). I called the surgeon on call the following day, a Friday, and he took out a lymph node for diagnosis that afternoon. I waited all weekend and then got the smiling message from the surgeon: "You dodged a bullet, it's sarcoidosis".

Mine was different than Vonnie's in that it involved the skin and eyes and not much the lungs. A newer use of the "steroid sparing" drug, methotrexate, worked beautifully for me making me disease free. I wonder if Vonnie is smiling down at me and thinking, "See, doctor and patient, there really isn't much difference. We're a team. Do you remember my prayers at Lourdes?"