Wall Street Journal on dealing with dying parent

Thank you, Wall Street Journal, for giving us this free article on dealing with a dying family member, focusing on children who live out of town.

The best point: "Just go." You won't regret it.

Hiatus (Over, I hope)

I haven't been blogging - I've been lobbying and working, instead. Whether in Austin or at work, my access to the blog is spotty. And I worried that anything I wrote might get in the way of some bills we were fighting for.

Unfortunately, the Texas legislature is self-destructing and virtually none of the pro-life, pro-family bills made it through.

One of the bills I was lobbying for contained amendments to the Texas Advance Directive Act (TADA) that would have increased protection for patients, prevented the removal of artificial hydration and nutrition, and more than doubled the time that doctors had to give medical treatment that they and others deemed "inappropriate." An improved process for communicating with families and a liaison between the family and the doctor was in the bill, which would have also added funding for facilities that offered complex medical treatments, such as dialysis for comatose patients, which simply don't exist in Texas. For two more years, we have the same law and the same arguments.

I do expect some of the recommendations, such as a dedicated liaison and improved communications to be adopted voluntarily in hospitals, as the good ideas that they are.

The Bill to limit embryonic stem cell research also failed, but we did get a brochure to explain the options available for donating cord blood and held the line on expanding unethical research, since several "clone and kill" bills were blocked.

Texas "Futile care debate: Prolonging life or suffering?"

The Houston Chronicle has an unusually good and balanced article on one case in the on-going debate in Texas on the end of life care, originally published May 6th.

The article uses the example of 91 year-old Mrs.Edith Pereira, and the way that her daughter, Zee Klein, made sure that she got the care that Mrs. Klein believed she should have:

Taking action on her own

Zee Klein wasn't about to just let her mother die, no matter what some hospital committee decided. But instead of waging a high-profile fight against the hospital, she decided to get her mother out on her own.

It wasn't going to be easy. For one, Medicare wouldn't cover Pereira's care if she were transferred to Christus St. Joseph, the downtown hospital where a doctor had agreed to take the case. Her coverage for her particular diagnosis already had been exhausted at Memorial Hermann.

Further complicating matters, Pereira's condition was deteriorating fast — by the time the hospital's futility committee ruled, she was in respiratory distress and her kidneys were failing. Doctors wrote in her chart that the discharge was against their advice.

"The patient was unstable," Castriotta said. "Given how sick she was, doctors felt her release would be dangerous."

The moment wasn't lost on Klein.
"She looked like she was in the throes of dying," said Klein, 68, who had previously cared for her late husband when he suffered a stroke and numerous heart attacks. "We didn't know how long she had."

Still, Klein had a plan. She would have her mother transferred back to St. Dominic nursing home for several hours, then taken to St. Joseph's emergency room, where federal law would require she be admitted.

But would she make it? Pereira's condition was so precarious that paramedics gave her oxygen through a respirator and stood ready to take her to a closer emergency room if it looked like she wouldn't survive the drive to St. Joseph.

On the afternoon of June 26, Pereira was discharged from Memorial Hermann and started the journey.

***
`Extremely poor' prognosis

Pereira made it to St. Joseph Hospital, but doctors summed up her prognosis in two words: "extremely poor."

It was understandable. On the day she was admitted, Pereira's problems included pneumonia; sepsis, a potentially fatal blood infection; dangerously high blood sugar; severe dehydration; a urinary-tract infection; kidney failure; and respiratory distress.

Doctors worked diligently over the next 72 hours to stabilize Pereira, giving her antibiotics, putting her on intravenous fluids, balancing out-of-whack electrolytes that were causing the kidney failure.

Pereira improved significantly, and St. Joseph scheduled an ethics committee hearing to consider all the options, which included inserting a gastric feeding tube. Many doctors, like those at Memorial Hermann, thought that the case seemed futile.

But Klein had one thing in her favor. St. Joseph, which since has been sold to Hospital Partners of America, then was a Catholic hospital.

"We follow the U.S. bishops' directive that the presumption be in favor of nutrition and hydration as long as the benefit outweighs the burdens on the patient," said Mike Sullivan, an administrator at Christus Health Gulf Coast, St. Joseph's corporate headquarters before the sale. "At a Catholic hospital, food and water are considered comfort care."

The ethics committee persuaded Klein to put a "do not resuscitate" order on her mother in the event of a cardiac arrest. A week after the meeting, a St. Joseph gastroenterologist inserted the feeding tube.

On Aug. 12 — a month later, finally free of all infections — Pereira returned to St. Dominic nursing home.

On the one hand, there's a lot to be said for the fact that Mrs. Pereira lived another 8 months and was stable for a while. Mrs. Klein spoke about her mother "firing" a nurse when she told her story to the House Public Health Committee meeting last August 9th, while Mrs. Pereira was still in the hospital. (RealPlay Video at about 8:00/12:38 to about 8:20/12:38 on the timeline) On the other hand, Mrs. Pereira was put through a lot of invasive interventions in the hospital, on a ventilator, dialysis, and other treatment for multiple organ failure, with several readmissions to the hospital over the next 8 months for infections. The last was for treatment for respiratory failure, ending with her death in a long term acute care hospital.

I especially like one of the comments from a reader, "KISDteacher," speaking about the care of her own 100 year old grandmother:

When it takes more than God to keep her alive, she made it clear that she doesn't want to keep going.

As I approach the first Mother's Day after my mother's death, I'm glad that she was spared the debates over feeding tubes and resuscitation. And I am so grateful that I was with her when she died and that I can remember rubbing her back and using a straw and spoon to feed her the afternoon of her death.

AMA on Texas Advance Directive (Futile Treatment)

The AMANews magazine, a weekly print newspaper for the members of the American Medical Association, has an article in the May 14 edition, available on line now. The excerpt is free here, but full content is only available to members and paid subscribers. Since LifeEthics readers have been following the progress of the legislation and know about the most recent action in the Senate Health and Human Services Committee, here's the portion that's not available for free:

Texas hospitals have used their state's advance directives law 27 times to withdraw treatment over family objections, said Robert L. Fine, MD, one of the 1999 law's architects.

Supporters of the status quo say the process normally extends far beyond 10 days.

"This law is usually invoked after days, weeks or even months of negotiation with families," said Tom Mayo, a health law professor at Southern Methodist University in Dallas who has helped evaluate more than a dozen medical futility cases on various hospital ethics committees.

Those seeking to abolish the time limit, however, allege that hospitals are most concerned about the estimated $10,000 a day it costs to provide intensive-care unit life support in these cases.

"The current statute, effectively allowing euthanasia with a polite and perfunctory 10-day notice, is misapplied and rips families away from the bedsides of their loved ones," said Bob Deuell, MD, author of a Senate treat-until-transfer bill, in an April letter to The Dallas Morning News. Dr. Deuell did not respond to AMNews' interview requests by deadline.

Bob Kafka, a Texas organizer for Not Dead Yet -- a disability rights group that opposes the advance directives law -- said in a statement that "the ability of a doctor to overrule both the patient and their surrogate in withdrawing life-sustaining treatment is in violation of the principle of patient autonomy."

But physicians argue that their obligation is principally to the terminally ill patient, not the family.

"It can be hard for patients' families to wrap their heads around the dying process," said Hanoch Patt, MD, an Austin, Texas, pediatric cardiologist who has served on hospital ethics committees and testified against the treat-until-transfer legislation. His patients often require invasive procedures if there is any hope for recovery, "but when a treatment can cause only more pain and suffering without any hope of benefit, then we're just prolonging the dying process, and I'm obligated to stop the treatment."
Compromise in the works

As this story went to press in late April, a compromise bill authored by House Public Health Committee Chair Dianne Delisi that would give families 21 days' notice to secure a transfer before the withdrawal of life support gained backing from the Texas Hospital Assn. and the Texas Medical Assn. The bill also would not apply to cases in which the only life support provided is artificial hydration and nutrition.

Such a compromise is not ideal, said Mark Casanova, MD, an internist at Baylor University Medical Center in Dallas.

"Physicians are going to live within the legal confines that we are forced to live within," he said, "but morally and based on medical ethical principles that are centuries old, we don't feel that it's necessarily appropriate. It's just 11 more days of suffering and pain for these patients that will not result in a single saved life."

AMA policy on futile care says hospitals should develop policies on how to handle such cases, refer them to ethics committees, involve families to the greatest extent possible and attempt to negotiate settlements. If the committee sides with the attending physician, the ethical opinion states, the hospital should seek a transfer, and if no transfer can be arranged, care should be withdrawn.

End of Life Compromise Texas

According to the Houston Chronicle, the Senate Health and Human Services passed a compromise bill to amend the Texas Advance Directive Act to extend the time lines for end of life care when the doctor believes that technological intervention is inappropriate.

The new committee substitute for Senator Dr. Duell's bill 439 was introduced by the Senator himself. It mirrors the current version of Representative Delisi's HB 3747. There will be 7 days notice before the ethics hearing and 21 days before care can be withdrawn or withheld against the surrogate's wishes. I believe that the House Bill will pass the Committee easily.

I'm sure that the Senator and Representative and their staffs have been working hard to come to this point. I know that those of us who have been advocates have put in many hours and much concern and prayer.

Bravo, 80th Texas Legislature!

Do No Harm 101 (Wesley Smith, Catholic Bishops and Futile Care)

Wesley Smith is covering Texas' legislature's debate over our Advance Directive Act. Yesterday, he accused the 24 Bishops of Texas of practicing "Futile Care Theory," which he defines as the decision to limit care by anyone other than a family member or patient. (In other words, here, he says that Terri Schiavo was not a "Futile Care Theory" case.)

Patient autonomy is not the first principle of medicine - that should be non maleficence guiding beneficence over-rides autonomy. "Heal when possible, but first, do no harm."

If you want to get to basics, the right to life means the right not to be killed, not the right to some one else's actions to maintain your life to the standard that you want and as long as you want. The doctor also has the right to life and the right to liberty.

Those rights intersect in medicine with/by a fiduciary duty of the doctor to place the patient's interests above his own and society has agreed that medicine is a good that we will provide for our community.

These interactions have to be guided by a respect for life and health and the medical knowledge and skills of the doctor. The medical knowledge and skills are the element that is measurable, licensed and verifiable by observers outside the patient-physician relationship. The practice of applying medical knowledge and skills requires medical judgment. Acquiring medical judgment - even if it's just the ability to understand statistics and not a growth of our actual wisdom - is how we learn to do surgery and chemotherapy when it's obvious that there's at least a short term harm.


I certainly don't practice medicine at what Wesley Smith has called "at the macro level." The closest I come to "macro level" thinking is that extra ankle xray to rule out fracture and ward off lawsuits. And I don't dare touch a pregnant woman after 20 weeks without all kinds of informed consent forms that ensures we understand that I'm taking care of the cold or sprained ankle (no xray here, without the Obstetrician's permission), because I'm not insured for OB care.

Nevertheless, money is a factor - society has a duty to the entire community, but the doctor's fiduciary duty has been to the patient in front of him. We've pretty much held that line over the last 20 years despite the push to make us "gatekeepers" and "managers." (read that, "bean counters." However, a doctor can't afford to financially bankrupt himself to devote care to one patient or to provide free or deeply discounted treatment for so many other patients that he can't provide for his family. (and pay his staff, taxes, rent, etc.)

Every day, our ability to offer increasing levels of technology and pharmaceutical interventions that can serve to keep a body alive longer and as more cells and organ systems fail. It was reported this week that new techniques of resuscitation after cardiac arrest that may benefit - and may harm - thousands of patients.

Each new ability to keep patients alive when they would have died "naturally" puts more stress on the medical judgment element. And puts the doctor in the position of increased likelyhood that he will have to determine the benefit to his patient for more and more invasive treatment. A side effect is that some patients and families may demand that he act against his judgment.

Carried to the extreme, the doctor could become the slave of any family - unable to withdraw from the care of a patient for months or years, forced to change IV's and IV settings, to maintain and adjust the ventilator and dialysis settings, or even to maintain the heart-lung assistance device that slows cell death, even after the heart has died.

When so many doctors agree that the demands to use medical technology on a patient is inappropriate, that's not "Futile care theory," no matter how finely some define it. That's the practice of medicine. For the Bishops to back the doctors in the practice of medicine is not "futile care theory," either.

Where are the doctors stepping up to cast doubt on specific decisions and doctors in the cases that have become so celebrated in Texas? We hear rumors, but I don't see action.

Last year, a good man, a skilled and compassionate doctor, ended up treating Andrea Clark's family while doing his best to treat the patient, herself, but his actions were definitely futile, proved to be of no medical benefit. By treating the family's concerns, he enabled them to agree to stop increasing Mrs. Clarke's level of intervention. He showed wisdom in addition to knowledge, skills and judgment. However, my medical skills and judgment wouldn't have allowed me to go so far as to drain the gallbladder as he did.

End of Life Debate (Emilio, Texas Law)

The debate continues on the end of life decisions and care of the poor child, Emilio Gonzales, at Wesley Smith's blog.

Please take a look at the misunderstandings in the conversation and the fantastic review by "woundedpig," whose comments begin at about the 27th post.

It does appear that the lung collapses were due to atelectasis, rather than pneumothoraces and there were no chest tubes. That doesn't change the facts that no one "refused" Emilio a tracheostomy. It means that he was unstable and not a candidate for the procedure.

There's also links there to the House State Affairs committee hearing of April 25th, if you want to see some of the debate at the Texas Legislature - and some of the people involved. The hearing began in the morning, when Representative Delisi laid out her bill. After a recess for the regular House session, the Committee resumed late that afternoon and ran to 5 AM. I couldn't stay because I had a 6:30 AM meeting in Dallas the next day. But the other testimony is worth some time -- after you read "woundedpig's" comments, though!

Judge rules to restrain hospital on Emilio Gonzales

I'm very glad that the judge in Austin has ruled that Children's Hospital can't remove him at this time from the ventilator and that he has named a guardian ad litum, to look at the evidence from the baby's best interests.

The Austin News 8 TV news also tells us that Emilio's mother and her lawyers have another doctor who will step in to evaluate his condition and whether he is a candidate for a tracheotomy.

Hopefully, some new voices will bring the two sides together for Emilio. I'm convinced that the doctors and nurses caring for Emilio have been doing their best, saving Emilio's life daily. And I'm sure that his mother is getting through the whole ordeal the best way she knows how.

For more information, you can read the Ethics Committee Report at the North Country Gazette, published in a same March 18th post by June Maxam.

End of life on the internet

LifeNews and Channel 8 TV in Austin, as well as several bloggers are reporting on the denial of the temporary restraining order for Emilio Gonzales in Austin, Texas.

Unfortunately, there are quite a few errors in the story.

I'm disturbed that the false information is spread and that there seems to be no problem in accusing the doctors of wanting to cause Emilio to die. In fact, the disease is causing Emilio to die, and his mother has said as much. The nurses and doctors have fought to prevent Emilio from having a "natural" death.

The doctors have worked with Emilio's mother, and have kept him alive in spite of repeat episodes where the baby's lungs developed a leak - or a "pneumothorax" - caused by the very high ventilator pressures required to keep Emilio's oxygen levels up.

Somehow, the North Country Gazette obtained the Ethics Committee Report and they have posted it on the Internet. I have copied part of the report at this post.

A pneumothorax allows air to escape into the chest around the lung, the pressure outside the lung grows each time the ventilator pushes air into the lungs. The lungs will collapse unless a chest tube is placed, the air is sucked out of the chest, and the lung reinflates. The ventilator pressures, high concentrations of oxygen and the collapsing of the lungs each cause further damage to the lungs.

The same high pressures explain why the doctors did not put in a tracheotomy earlier. The "trach" would have leaked if placed while the lungs needed such a high pressure.

There's no question of the diagnosis and the vitamin IV was not one of the "life sustaining treatments" and probably was not needed at all.

There's no doubt that the child has Leigh's syndrome. The syndrome is diagnosed by a group of symptoms, findings on the exam and sometimes by labs. In Emilio's case, there are also MRI's that show areas of the brain are dead and there are EEG's that show he has seizures one third to one half of the time. There are some tests for finding whether and how many or how severely the cells are affected with a genetic defect that makes the mitochondria - the energy factories of the cells - damaged. In the worst form, the type that causes "necrosis," or cell death, of the brain cells, children die very early. An alternate name is "necrotizing encephalomyopathy," meaning that the nerve and muscle cells are affected.

What is not known is the exact nature of the genetic defect that is causing the child's cells to die. There are at least several dozen and from my reading, I'll bet there are a hundred or so genetic defects that can be inherited or may be a new mutation. Some are "X-linked," meaning they are passed through the mother. Some - especially the X-linked forms - disturb the metabolism of the pyruvate dehydrogenase enzyme within the mitochondria.

In the past, some children with the form of the syndrome that changed the metabolism of pyruvate dehydrogenase got better after given high doses of IV thiamine. Now we know that that only works for certain types of defects that cause the syndrome. Other people get better with Co-Q 10 or some other diet change. (The article that the lawyers used to "prove" that the doctors were denying needed therapy was from 1974. There is much more known about the Sydrome than was known then - for one thing, more is known about DNA and certain mutations. However, these types are usually much more slow in developing and it's much more likely that the docs have been able to order tests for this specific defect - the DNA is evaluated.

While the hospital ethics committee did vote behind closed doors, they heard the testimony of in a hearing that included the lawyers, Emilio's mother and at least one employee of Texas Right to Life. No one is disputing the facts of the report.

Doctors understandably can debate and discuss medicine in a closed conversation, without lawyers and non-medical family members present. How else could they freely discuss the case, without fear of more lawsuits and/or while using the exact medical terminology that doesn't always mean the same thing to lay people that it does to doctors?

The difference is between those who want to keep Emilio alive no matter what pain he is caused by his treatments, no matter how many times his body must be invaded by tubes and needles while his brain tissue - and probably his muscles and intestinal lining as well - dies as the ventilator forces air into his stiffened lungs.

How many needles will Emilio have before his mother changes her mind and lets him go in peace? How many times will he be snatched back from the edge of death by the doctors before he is allowed to live - even a short time - without the pain and irritation of chest tubes, ventilators that push air into him, etc.?

There's no mention of removing the food and water because Emilio's brain does not cause him to breathe without the ventilator. If the ventilator is removed before the damaged areas of the brain heal -- a very unlikely scenario in children without Leigh's disease -- Emilio will die within minutes. The disease will kill him, not the doctors. Removing the ventilator will allow - not cause - him to die.

One of the lawyers has been posting about Emilio at Wesley Smith's blog since mid February.

And yet, neither of the lawyers mind saying things like,

People who could profit from an innocent person’s death should not get to decide when it occurs. Whatever the hospital’s motives are for pushing to end Emilio’s treatment, a child's life outweighs all other concerns--whether it’s to cut costs, or for convenience, or something else,” said Carden. “And furthermore, the twisted state law that allows hospitals to exterminate disabled children over their parents’ wishes needs to be changed.”

There is no profit for the doctors - the child has Medicaid and Medicare, according to what the lawyers and Texas Right to Life staffers have told me.

It is wrong to falsely accuse. It will not help Emilio. I sincerely doubt that it will help Emilio's mother get ready to let him go. And many people are being made angry and sad about the doctor's, hospital employees, nurses and even the Bishop of the Diocese of Austin's "murder" of Emilio. (The post at ProLife Blogs that had that last has been "snipped" to remove it, but the note of protest about accusing the bishop is still there. The quotes from Melanie Childers are still online at the North Country Gazette.)

Video on Emilio Gonzales

Austin Texas' KVUE has several video news stories available on their website covering the story about Emilio Gonzales and the Texas Advance Directive Act.

The report is in error because it says that the hospital makes these decisions, when the law allows the doctor to do so, and the ethics committee only determines whether the doctor's decision is correct about "medically inappropriate care."

These may not be available for long:
"Medical Ethics vs. Futile Care" is available on this page. (I can't link to this one directly.) This video covers the Ethics Committee and has part of an interview with Catarina Gonzalez.

There's more video, here. One is from the day the lawsuit was filed and one, "Life Support Decisions" is from Thursday the 22nd, with a long interview, "I live breath to breath," with a mother who faced a similar decision in December.

News stories from the last few days are linked to that page, too.





3/21/07 Mother gets more time to find new hospital for dying son

3/20/07 Dying toddler brings futile care bill to forefront

"Living Life's End"

The title is from a 2005 essay by Gilbert Meilaender in First Things. Not surprisingly, Dr. Meilaender speaks with much more clarity than I ever could in discussing the sort of dilemma that we face when considering the baby, Emilio Gonzales, and the treatment vs. the care he is to receive from his doctors, his mother, and the State and the distinction between letting "die those who were clearly dying," and “letting patients die who are in fact not dying.”

We are not abandoning the care of Emilio by saying, "This much and no more." If he requires constant painful intervention and technology while his condition continues to deteriorate, we do not cease to care for him when we cease to intervene and use technology.

As Dr. Meilaender points out,

On the one hand, we should not aim at their death (whether by action or omission). We shouldn’t do whatever we do so that they will die. On the other hand, because we do not think that continued life is the only good, or necessarily the greatest good, in every circumstance, we are not obligated to do everything that might be done to keep someone alive. If a possible treatment seems useless or (even if useful) quite burdensome for the patient, we are under no obligation to try it or continue it. And in withholding or withdrawing such a treatment, we do not aim at death. We simply aim at another good: the good of life (even if a shorter life) free of the burdens of the proposed treatment.(emphasis mine)

The reports at the Capitol are conflicting with what is being told at the North Country Gazette, Prolife Blogs, and Second Hand Smoke. And with one another.

No one that I know is arguing that Emilio is not dying - or that he could have died from any number of past events if the doctors had not intervened. It is time to stop intervening.

There is also a difference between causing another to do his duty and forcing another to act against his conscience and what he believes is his duty because you very much want him to do what you want him to do. How often do you want your doctor and your ICU nurse to practice going against her or his conscience? How often should the State force an action - a repeated action that causes other people to be forced to act - against a professional's conscience?

The Texas Right to Life lobbyists told me that they are offended because the Ethics Committee mentioned Emilio's "dignity." If the report published by the North Country Gazette is the actual Committee report, the members affirm Emilio's human dignity - but say that the aggressive treatment is an assault against his human dignity:

• The current aggressive treatment plan for Emilio amounts to a nearly constant assault on Emilio’s fundamental human dignity, and with little, if any, corresponding benefit to Emilio. Thus the burdens associated with such care clearly outweigh its benefits.

A trial of care including the ventilator, the feedings, and the chest tubes for a pneumothorax were appropriate. When Emilio continues to require more invasive care, when we can not keep him the same, much less make him better, it is time to stop hurting him, at least.

To force the doctors, nurses, phlebotomists and all the other people who are caring for Emilio to put aside their concern for his best interests, to subjugate their consciences and duty to Emilio for his mother's wishes is simply wrong.

To accuse them and even the Bishop of the diocese of Austin, of acting with the intention to "murder" Emilio (as June Maxam of the North Country Gazette quotes Melanie Childers as doing here and as "plb" quotes NCG here), to state that the hospital Ethics Committee is willing to speed Emilio's death in order "to free up a bed" (as Jerri Lynn Ward is quoted as saying in this news article: “You have a treating doctor who makes the initial decision, then you have an ethics committee at that same hospital with, frankly, a very clear conflict of interest,” Gonzales' attorney Jerri Lynn Ward said. “They have something, a bed they can free up basically, if their decision goes unchallenged."
, is simply evil.

(Edit 8:30 AM 3/21/07 addressed the "dilemma" of spelling)

More on Texas end of life case

A reader, unfortunately, shows us the other extreme of the end of life debate. However, she missed the point entirely. Our discussion about the end of Emilio's life is a debate about conscience, laws, and whether because doctors are licensed by the State, they abdicate their duty to act in the patient's best interest.

It is also a demonstration that the first principle of medical ethics should be non-maleficense, not autonomy. In the name of "autonomy," the mother is demanding that the doctors carry out acts that they believe are not in Emilio's best interests. The lawyer for the mother questions what the doctors are telling other hospitals, and patient's rights advocates accuse the doctors of abusing an "imbalance" of power.

The doctors - and the Ethics Committee, in their report as published by The North Country Gazette - have shown the evidence that leads them to believe that this child is experiencing pain and being harmed due to the actions of the doctors.

Here's the case they made, with a little explanation and guess work from me:

The lungs cannot tolerate the ventilator. We know this because of the repeated pneumothoraces (or "leaks"). Any one of these instances would have lead to a "natural death" by the compression of the lung tissue - and even the heart - if the doctors had not intervened on the mother's request.

There are large areas of the child's brain that are dead, and more are dying due to the seizures (and probably, the build up of lactic acid and other toxins). We know this because it shows on the MRI and the EEG.

More than likely, feeding by IV, a feeding tube placed in the gut or even by mouth harms this child. We know that the cells lining the intestines grow and replace themselves faster than any other cells in the body. Probably, the lining of the gut is mostly dead because the cells require energy from the mitochondria to replace themselves and to fight off infection. Where the lining does remain, food in the form of fats, proteins and many nutrients must be moved into the system from the gut by actions of cells that require energy, rather than passive absorption. Everything must be moved through the gut by the actions of muscles that require energy or they will sit, build up, and decay.

Even in a child who has a healthy liver and functional mitochondria, long-term "total parenteral nutrition" damages the liver. In this case, the cells of the liver aren't likely to be functioning well due to the mitochondrial defect and to the build up of lactic acid and other poisons, including those IV feedings. Virtually all food is processed in the liver by cells that use energy to function. Most waste products are also processed by the liver, again, in cells that use energy.

Very soon, if not already, Emilio will need kidney dialysis. Due to the brain damage and the acid build up in his blood, his blood vessels will not be able to contract and hold his blood pressure up and his heart will beat irregularly.

The doctors laid this out for the Committee. The Committee's report laid it out for us. From what I've read in the press and other blogs, the doctors have tried to explain this to Emilio's mother.

And yet, we read that the doctors, the hospital, the Catholic Church and the State of Texas are all conspiring to kill Emilio against his mother's wishes.

Leigh's Disease (Long post on end of life and baby Emilio Gonzales)

The mitochondria are the power plants of the cells. They take the sugar and turn it into the power that runs all the processes of the body. Leigh's disease is a defect in the genes of these mitochondria or of the body’s ability to make a protein or an enzyme that is used in the mitochondria, like pyruvate dehydrogenase.

The prognosis for individuals with Leigh's disease is poor. Individuals who lack mitochondrial complex IV activity and those with pyruvate dehydrogenase deficiency tend to have the worst prognosis and die within a few years. Those with partial deficiencies have a better prognosis, and may live to be 6 or 7 years of age. Some have survived to their mid-teenage years.

The defect can be nearly complete or it can be very mild depending on which of the many genes that can be involved are involved. The cells may be able to make energy but only very slowly - or very, very slowly. Or some cells may be able to make some power, but others can't. Some functions of the body need constant energy, others do not.

Pro-Life Blogs and other blogs carry posts about the very sad case of little Emilio Gonzales, who has been diagnosed with "Leigh's Disease." Unfortunately, they are full of implications that doctors (and even the Bishop of the diocese of Austin) are planning to "murder" the baby boy.

We do not have good tests to determine where the genes went wrong and they can go wrong in several places causing different symptoms and even different symptoms in the same child at different times. So this is a “syndrome” or a diagnosis given when we see a pattern of symptoms. AIDS is a syndrome that has only one cause -- infection with a virus instead of many different gene defects -- but it still develops differently in different people and can mimic different diseases in the early stages.

Sometimes Leigh’s disease does respond to thiamine – but only if the defect is in certain genes. Not in the vast majority of cases.

Another name for the syndrome is “Subacute Necrotising Encephalitis,” because in the late stages, areas of the brain die and break down. This little boy's nerve cells all over his body, including large areas in his brain, have died. This can be seen on his MRI.

It’s also supported by the findings on his EEG – that shows that he is having seizures one third to one half of the time. The seizures cause more cells to die.


However, the pain nerves still live: the report says he still reacts to painful stimuli as though he is in pain. He isn't able to process the pain.

Other organ systems fail as the lactic acid builds in his blood and tissues – cooking the proteins that make up the muscles, enzymes, and most structures of the body. If you’ve ever been sore the day after exercise, you know what lactic acid build up in one spot feels like and how long it takes to go away.

And, of course, growing and healing takes energy. The fastest growing cells for most of us line the intestines and make up the liver where food is processed - they die first, but can grow back sometimes - so the symptoms seem to come and go. When they are dead, it hurts the patient to give him tube feedings that must be absorbed by the gut or to give IV feedings that contain anything that must be processed by the liver.

Even the kidneys use energy. And the sphincters of the bowel and bladder do, too. If there is an imbalance between nerves, the sphincters spasm shut - so Emilio needs a catheter in his bladder.

The lungs stiffen with prolonged ventilator use. The stiff lungs and muscles mean that the pressure from the ventilator needs to be so high in order to give him enough oxygen that he is having leaks appear in his lungs as some of the airways break. The baby's lungs are repeatedly collapsing and having to be re-inflated. This means that over and over, he has a “pneumothorax.”The air goes into the sac around his lungs, squeezing the lung tissue itself down. The pressure outside the lungs is even higher than the pressure the ventilator is making inside the lungs -- each push from the ventilator pushes more air through the leak.

The docs then have to place a chest tube or chest tubes – possibly in a baby, they would use the temporary insertion of large bore needles - to release the air around the lungs, and allow the lung itself to inflate.

Some of the air moves between the tissues of his body, coming to the surface in little pockets – causing “subcutaneous emphysema.” Patients tell me that the pneumothorax and the subcutaneous emphysema hurt. The chest tube does not take away the air in the tissues – that takes days or weeks to be reabsorbed.

What would I do? I would suggest that the mother and the docs adopt a strategy of "this much and no more." Give Emilio droppers of fluids by mouth, to keep his mouth moist. Hold him as much as possible. Continue the ventilator, but stop placing the chest tubes and stop changing the ventilator settings. Do not add new medicines and do not resuscitate when the heart stops.

The Ethics Committee Report has been published at the North Country Gazette.

Medical Update Since Last Ethics Committee Meeting on 2/19/07:

Dr. Alexandra Wilson, the patient’s current attending physician, states that Emilio is unable to move his arms and legs and only has abnormal posturing movements with stimulation. He rarely opens his eyes, does not gag, cannot cough and cannot breathe without use of the ventilator; his pupils are not normally responsive to light. In addition, he can not empty his bladder and has to have a catheter. He does appear to experience pain, as he grimaces in response to deep stimulation, and is now receiving pain medications. He also bites his tongue, which causes bleeding, making it necessary to place a device in his mouth to prevent further injury to his tongue. In addition, he has experienced repeated full and partial collapses of his lungs, and his physicians and the treatment team are having great difficulty keeping his lungs inflated, even with the assistance of the mechanical ventilator. Finally, he is now having seizures, some of which produce visible physical symptoms, and scans (MRIs) of his head show progressive loss of brain tissue.

Dr. Brendle Glomb, pediatric pulmonologist, then discussed Emilio’s pulmonary status, which as noted above has continued to deteriorate. He explained the current functioning and support provided by the ventilator, and then described why Emilio would no longer benefit from a tracheostomy. He also noted that the repeated collapse and reinflation of Emilio’s lungs is damaging to the lungs, and increases the risk that they will tear or even burst during attempts at re-inflation.

Dr. Jeffrey Kane, pediatric neurologist, then discussed Emilio’s current neurological status, which has continued to deteriorate since the last consultation. Overall Emilio shows no purposeful response or movement, which is evidence that the deeper functioning of the brain is absent. In addition, Emilio’s EEG suggests that Emilio is experiencing many more seizures than those at the bedside can see. Based on his review of Emilio’s most recent EEG, Dr. Kane believes Emilio’s brain may be experiencing seizures between 1/3 and ½ of the time. Dr. Kane believes that the seizure activity will continue to increase, and that continued seizures will accelerate the death of Emilio’s remaining brain tissue. In response to a question, Dr. Kane noted that the current brain damage is not reversible, even if the seizure activity for some reason should slow or stop. Instead, he believes that Emilio will continue to experience the relentless and progressive loss of his brain tissue and brain function, and that no therapy or other intervention has been identified which could stop or reverse this process.

Dr. David Anglin, a pediatric intensivist involved in Emilio’s care, then discussed Emilio’s care-setting. He noted that during the Ethics Consultation on February 19, 2007, there was some hope that Emilio might be able to be transferred to a lower-acuity care setting or discharged home, and particularly if he were to undergo a tracheostomy and receive a feeding tube. However, given Emilio’s continued deterioration, he is too ill to survive anywhere but an intensive care setting, and he certainly is not a candidate to be discharged to home.

Edit 03/19/07 at 21:30 - cleaning up grammar.

Debate On Ethics

After several days of discussion about a baby that Texas lawyer Jerri Ward asked Wesley Smith to blog about on Secondhand Smoke, I have been asked "How can you be a doctor and not know this about what passes for ethics nowadays?"

Because I have a different understanding "about what passes for ethics nowadays."

I do not agree that euthanasia is practiced in Texas medicine or that utilitarian arguments prevail in medical ethics, especially in Texas. I am a pro-life family doctor who has been studying and practicing medicine, and now, bioethics in Texas. My activism and biggest motivator has been focused on the manipulation and dehumanizing of humans at the beginning of life, because of the advocacy for abortion and destructive embryonic research. I am repeatedly reassured that our Texas physicians do not support euthanasia at the end of life by what I know of them and by what I witness at our medical association meetings and at the meetings where we have been debating the amendment of the Texas Advance Directive Act.

I know what is said in the literature, in the media, in the blogs, and what is said between doctors. I've experienced being the patient, the daughter, wife and mother of a patient, and the doctor in some tough ethical situations.

The elite "ethicists" across the world voice and publish all sorts of utilitarian ideas, including the feminist bioethicists at the American Society of Bioethics and Humanities who discounted conscience as a legitimate guide for physician's actions. I oppose this sort of "ethics" every chance I get.

However, the doctors in Texas do not advocate or encourage such drivel. The very rare doc who is unwise enough to voice the opinion that some lives are not worth living is immediately countered and out-numbered and out-reasoned by his or her professional and compassionate colleagues.

In contrast to my own experiences and education, in the one-sided reports on the blogs and in the media, I hear a story that never quite fits what I know about medical facts, much less about the way I see practical clinical ethics being practiced and taught in Texas. Unlike the other posters at Secondhand Smoke, it is not at all "obvious" to me what happened in this case.

Concerning the bit we know about the case in question, it's not at all "obvious" to me that any pediatrician would have argued to an ethics committee that there are not enough resources to go around or that a child would be better off dead than to have a safe tracheostomy and feeding tube placement in order to continue the current level of technology and in anticipation of transfer to the proper step down care.

On the other hand, if, as I suspected, there had been concerns about imminent death or about a crisis due to the mitochondrial defect flaring after stress of surgery, then it would have been appropriate to object to treatment that could hasten death while causing pain and (surgically) separating the child from her/his mother.

I spent several hours over the last two days watching and listening to the hearings last August 9 on the TADA. I couldn't attend them because my mother was in the hospital - she died August 14. I was reassured by the testimony of the doctors and hospital representatives.

As I said, part of what the lawyer who reported this case experiences is most likely ("obviously") the result of her previously publicized comments in the media that doctors and hospitals kill patients and bury their mistakes.

There's (Still) No "Futile Care Act" in Texas

I keep running across news articles like this one in the Dallas Morning News (free subscription required) which claim that Texas has some sort of "futile care" law. There is no such thing as a "Futile Care Law" in Texas and never has been. (Previous LifeEthics posts include several in April, 2006.)

There is a law called the Texas Advance Directive Act (TADA). (Here at the Texas Statutes website in pdf, and an excerpt with my comments, here.) The law was written by a coalition of doctors, lawyers, patient advocates, disability rights groups, and pro-life groups such as Texas Right to Life, National Right to Life, and Texas Alliance for Life.

In that DMN article above, please note that Dr. Fine was interviewed without knowledge that he would be quoted in an article about a very sad case, one which had not come to the point of being subject to TADA at the time of the interview.

I am concerned that Texas Right to Life is not speaking up about why they found the law acceptible in 1999, but feel that they can condemn it - and all doctors and hospitals - now.

We never stop treating the patient. The TADA has never been invoked in order to withhold food or water from patients or from a patient who only needs artificial food and water. The Texas Advance Directive Act does, however, allow doctors to refuse to use medicine and technology that is not in our patient's best interest, treatment that is harmful or futile.

There are no futile patients, and no "futile care law," there is only futile medicine and technology.

If my patient suffers organ failure after organ failure, some medicines and technology can become harmful -- sometimes by causing side effects and more organ failure, often by prolonging the patient's dying.

In fact the TADA coalition has been trying to come to a consensus on making sure that the Act cannot be invoked if the only intervention needed is food and water. It has also been agreed that the Coalition will support a longer period of notice before the ethics committee meeting is held, that medical records need to be offered in a timely manner and that the family should have two weeks in order to find another doctor or facility.


The cases in Texas which have been cited to criticize the TADA include:

• A woman who had been in the ICU for 4 or 5 months after heart surgery, who had had a stroke and was on a ventilator. Her doctors had suggested to her family that perhaps they should not begin dialysis when her kidneys failed. The family rejected the suggestion and the patient had to have continuous, then intermittent dialysis. She then needed increasing doses of medicines to raise her blood pressure. After a while, the attending doctor again suggested stopping or withholding the addition of new treatments because of the risk of pain and complications that each treatment added on top of the previous complications. Again, the family refused. The doctor decided that he couldn't continue to escalate the interventions and asked for an ethics committee meeting under 166.046. The family members postponed several times, putting off the meeting. Somewhere in this time, the family threatened legal action, picketing, and raising as much bad publicity for the family as they could muster. Finally, the hospital set a meeting for a firm time and refused to budge. The ethics committee members agreed with the doctor and began the process to help the family find a new doctor or a new facility for the patient. The family began protests that became news nation-wide. A new doctor was found and additional treatments and invasive interventions were performed on the patient. Finally, the doctor reported that the patient had had a heart attack, and the ventilator was stopped without verifying brain death.
• In another case, the patient was brain dead, but the family members sued to prevent the doctor - who had been the woman's family doctor for years - from turning off the ventilator. A new doctor came in, did an additional test, and confirmed that the patient was brain dead.
• A third case involved a woman who had a stroke, was unconscious and required a ventilator and dialysis. The patient's daughter was an ER doctor in that hospital. There are no Texas long term facilities that offer dialysis to an unconscious patient on a ventilator. The patient was sent home with home dialysis and ventilator.
• A fourth case involved another woman who had had a stroke, was unconscious and required dialysis. There is still no facility in Texas that offers dialysis for a comatose patient on a ventilator. Her family objected to the hospital's "forcing" them to move her to another facility. Eventually, she was moved to another state, where there is a long term facility that could provide the treatment she needed.

Unfortunately, a repeating theme from some of the lawyers at the meetings is an objection that doctors are protected from malpractice if they follow the Act.

Two identical Bills before the Texas Legislature, SB 439 and HB 1094 not only remove the current protection from risk of malpractice for doctors who follow their consciences and would require doctors to use ever escalationg "life saving treatment" until (as in the case of Andrea Clark) the family decides to remove it or until transfer to another doctor or facility is arranged, without a time limit.


Do we really want to override doctors' consciences? Remember the NEJM article from last week.

Concerning abortion due to failure of contraception and prescribing contraceptives to 14 to 16 year olds when parents object, the Chicago Tribune reports that some believe this is the case:

That approach doesn't even give a patient the option to access other physicians," said R. Alta Charo, a professor of law and bioethics at the University of Wisconsin-Madison who was not involved in the study. "It's a raw imposition of your personal beliefs on all those who come to you for professional services."

Short course (long post) on medical ethics.

Are doctors killing patients or taking life when they withdraw or withhold care? Do families who don't insist that "everything be done" kill their loved one? Do patients who refuse ventilators, dialysis, etc., commit suicide? For that matter, does a ventilator equal dialysis equal a feeding tube?

Can the patient who refuses all attempts to resuscitate and the family who demands that every effort be made to keep the patient’s body alive even if there is no hope of awareness, both be right?

There's an old saying that pneumonia is the old man's friend. If the surrogate decision maker for a patient with Alzheimer's demands that she be allowed to die while suffering from an easily treatable condition such as pneumonia, but the doctor believes that it would be medically inappropriate to withhold nutrition and hydration, who is right? What if the patient is a child?

At what point does care become medically inappropriate? Who is best qualified to make that determination? Is there a place to say, “This much, and no more?” Is there a point where we say that the last step was where we went too far? And are we doing all this for us, or as care for the patient?

How many events must happen before we "Let go, and let God?"

These are some of the questions raised by the Texas House Committee on Public Health on August 9, 2006. You can watch the full 12 1/2 hour archived video at Texas Legislature Online. (Free RealPlayer necessary) I recommend moving the cursor to 2:20/12:38 and watching until 4:10/12:38. If you only have 30 minutes, Dr. Bob Fine, MD, is very informative (3:12 to 3:40) as a physician who helped develop the practice of hospital clinical ethics in the U.S.

The only question allowed under Section 166.046 of the Texas Advance Directive Act of the Health and Safety Code should be whether or not medical treatment is "inappropriate medical care" for the patient. Section 166.046 is an attempt to allow clinical judgment by doctors carrying out Advance Directives, for oversight of doctors by hospital ethics committees and for disagreements between doctors and the patient or surrogate by allowing time for transfer to another doctor who does not believe that the treatment is medically inappropriate.

Examples of medical procedures and technology that are not medically appropriate care are sometimes clear-cut, and sometimes professional judgment or conscience is needed to make the distinction. The same medicines and procedures used to relieve pain and symptoms carry known, but unintended side effects. Something as simple as oxygen by nasal canula or face mask can sometimes blunt a patient's drive to breathe and force a decision on whether or not to use a ventilator.

At the end of life, even “life sustaining” treatment such as pacemakers, ventilators, dialysis and tube feedings may not always be medically appropriate care. Doctors and family members are faced with decisions about whether a given technology or procedure is life saving or only prolongs dying in patients.

Today, we have the ability to keep the body alive for a few days, even after the brainstem is dead. This is not bad for the patient, because if the brainstem is dead, the part of the brain that could be aware of pain, is also dead. It is not good for the patient either, for the same reason.

If the patient is not brain dead, we can keep them alive much longer because signals from the brain help us maintain blood pressure and heart rate.

In order to keep the heart beating and the lungs breathing on the ventilator, we have to add IV feedings and do frequent blood draws, maintain arterial blood lines to follow the oxygenation, nutrition and blood pressures. Medicines to regulate blood pressure may actually decrease blood flow to the fingers and toes. The patient who is not brain dead or in a coma will require some level of sedation until we are able to create a tracheostomy. Sometimes, we have to paralyze patients and then completely sedate them to make the ventilator tube in the throat tolerable.

When dialysis is needed over months and years, we don't have the ability to prevent the side effects. Patients begin to have “pathological” fractures in their arms, legs, and ribs, simply when they are repositioned in their beds. If the patient only communicates with us to tell us that he or she is in pain, should we continue to hook the patient up to the dialysis machine three times a week because his surrogate insists that “everything” be done? What should we do when he has a heart attack, a stroke, or seizures and the family insists on chest compression, ventilator support or a surgical procedure to place a permanent feeding tube? How about when our efforts keep a patient in physical or mental pain that is uncontrollable – when the doctors cannot control pain or (as in the case of my mother)the patient can’t process stimulation as anything but excruciating pain?

Next: what can we do?

Edited November 29, 2007, to add labels.

Texas Advance Directive Act - in its own words

§ 166.046. PROCEDURE IF NOT EFFECTUATING A DIRECTIVE OR TREATMENT DECISION.

My comments are interspersed and at the bottom.

(a) If an attending physician refuses to honor a patient's advance directive or a health care or treatment decision made by or on behalf of a patient, the physician's refusal shall be reviewed by an ethics or medical committee. The attending physician may not be a member of that committee. The patient shall be given life-sustaining treatment during the review.

Please note that the decision is the doctors' to make. The committee may affirm or not.

(b) The patient or the person responsible for the health care decisions of the individual who has made the decision regarding the directive or treatment decision:
(1) may be given a written description of the ethics or medical committee review process and any other policies and procedures related to this section adopted by the health care facility;
(2) shall be informed of the committee review process not less than 48 hours before the meeting called to discuss the patient's directive, unless the time period is waived by mutual agreement;
(3) at the time of being so informed, shall be provided:
(A) a copy of the appropriate statement set forth in Section 166.052; and
(B) a copy of the registry list of health care providers and referral groups that have volunteered their readiness to consider accepting transfer or to assist in locating a provider willing to accept transfer that is posted on the website maintained by the Texas Health Care Information Council under Section 166.053;
and
(4) is entitled to:
(A) attend the meeting; and
(B) receive a written explanation of the decision reached during the review process.

(c) The written explanation required by Subsection (b)(2)(B) must be included in the patient's medical record.

(d) If the attending physician, the patient, or the person responsible for the health care decisions of the individual does not agree with the decision reached during the review process under Subsection (b), the physician shall make a reasonable effort to transfer the patient to a physician who is willing to comply with the directive. If the patient is a patient in a health care facility, the facility's personnel shall assist the physician in arranging the patient's transfer to:
(1) another physician;
(2) an alternative care setting within that facility; or
(3) another facility.


Either the doctor or the patient or the person making decisions for her may object to the ethics committee conclusion.

The doctor who has decided that the treatment is inappropriate may transfer to another doctor in the hospital or another place in that hospital or another hospital.

(e) If the patient or the person responsible for the health care decisions of the patient is requesting life-sustaining treatment that the attending physician has decided and the review process has affirmed is inappropriate treatment, the patient shall be given available life-sustaining treatment pending transfer under Subsection (d). The patient is responsible for any costs incurred in transferring the patient to another facility. The physician and the health care facility are not obligated to provide life-sustaining treatment after the 10th day after the written decision required under Subsection (b) is provided to the patient
or the person responsible for the health care decisions of the patient unless ordered to do so under Subsection (g).

(e-1) If during a previous admission to a facility a patient's attending physician and the review process under Subsection (b) have determined that life-sustaining treatment is inappropriate, and the patient is readmitted to the same facility within six months from the date of the decision reached during the review process conducted upon the previous admission, Subsections (b) through (e) need not be followed if the patient's attending physician and a consulting physician who is a member of the ethics or medical committee of the facility document on the patient's readmission that the patient's condition either has not improved or has deteriorated since the review process was conducted.

(f) Life-sustaining treatment under this section may not be entered in the patient's medical record as medically unnecessary treatment until the time period provided under Subsection (e) has expired.

There is a distinction here between "inappropriate" treatment and "medically unnecessary" treatment.

(g) At the request of the patient or the person responsible for the health care decisions of the patient, the appropriate district or county court shall extend the time period provided under Subsection (e) only if the court finds, by a preponderance of the evidence, that there is a reasonable expectation that a physician or health care facility that will honor the patient's directive will be found if the time extension is granted.

(h) This section may not be construed to impose an obligation on a facility or a home and community support services agency licensed under Chapter 142 or similar organization that is
beyond the scope of the services or resources of the facility or agency. This section does not apply to hospice services provided by a home and community support services agency licensed under Chapter 142.


First, I probably would not remove the ventilator against the patient's or the patient's family's wishes. But I would cease hooking Mrs. Clark up to the dialysis and stop the tests and as many of the other invasive procedures as possible if I were convinced that I was hurting her more than I was helping her. As organ system after organ system failed, each added treatment requires more and more complicated coordination and has less and less chance of actually helping.

Unfortunately, I'm not sure that the TADA allows for refusal of some of the intensive treatment, while maintaining the rest.

There are still comments about what the hospital or the ethics committee is doing. While I suspect that there is some hostility between the family and some of the hospital personnel, the hospital cannot practice medicine.

I'm surprised that any facility that is capable of both ventilation and kidney dialysis cannot give any sort of medications that a patient would need. The hard part would be finding someplace that can monitor both the ventiliation and the dialysis. The only guess I have is the need for constant supervision of pressors - medicines that make the blood vessels tighten up so that the blood pressure can be maintained high enough. These medicines are very dependent on nurses and protocols, unless the doctor can stay at the bedside and turn the dials himself.

I'm surprised about the way people are talking and writing about the doctors, the ICU nurses and the hospital personnel in general. In your experience, are ICU nurses likely to be in on the conspiracy that is alleged?


On the contrary, the nurses are probably struggling to suppress their grief at the torture they are forced to inflict on Mrs. Clark.


For that matter, I haven't seen many conspiracies hold up as well as the rumored "aggreement" between the Houston hospitals that are said (by Wesley Smith and others) to refuse to accept Andrea because St. Luke's has declared her treatment futile.

More than likely the truth is that other doctors and hospitals understand the medical situation and agree that the care is prolonging death and actually adding complications that may lead to death.


As to pain and sedation:
75% of patients on dialysis have itching. I've had to practically knock them out with antihistamines and tranquilizers.


Now then, I've seen criticism about the doctor going on vacation this week. In most large facilities, the vacation times are planned a year in advance. And I know that I have to buy airplane tickets and make reservations in advance to save money. And I can't imagine telling my kids they can't go on vacation. They were used to the phone ringing in the night, but they expected me to be home on my vacations and other times I'd arranged for another doctor to cover for me. If doctors only went on vacation when none of their patients were sick, they'd never get to go.


It's likely that the doctor thought the family would be swayed by the ethics committee and/or their decision and that this would all be over before he left.