35 Years after Baby Doe Continued: The Simon Crosier and Charlie Gard Cases

Last week, I wrote about Baby Doe and my daughter Karen and how both were medically discriminated against 35 years ago because they were born with Down Syndrome. The ethical rationale in both cases help set the stage for the legalized assisted suicide/euthanasia we struggle against today.

Unfortunately, the legal right to live for children with disabilities and their parents continue to be under attack today with the cases of Simon Crosier and Charlie Gard.

SIMON CROSIER

Last August, I wrote a blog titled “Parent Power” about the efforts by some legislators in Missouri and Kansas to block the passage of Simon’s Law, a bill that exposed and sought to change the secret futility policies in even some Catholic hospitals that led to the death of Simon Crosier, a baby born with Trisomy 18.

Since that blog, Kansas finally did pass Simon’s Law but in Simon’s home state of Missouri, the bill is still stuck in committee even though new information about the life expectancy and prognosis for such children led to an opinion editorial in the prestigious Journal of the American Medical Association (JAMA) admitting that withholding life-sustaining treatment from babies with Trisomy 13 and 18 was really a value judgment rather than a medical judgment.

The fight for Missouri’s Simon’s Law will continue.

CHARLIE GARD

On June 30, 2017, the parents of 11 month old Charlie Gard lost their appeal to European Court of Human Rights to keep their son with a rare genetic disorder on life support and take him to the US to try an experimental treatment. The appeal was filed after Britain’s Supreme Court decided to let the hospital remove Baby Charlie’s life support, finding that prolonging Charlie’s life was “not in his best interests.”

Baby Charlie has a rare genetic disorder affecting his muscles and brain that has caused  brain damage, seizures and prevents him from breathing on his own.  A neurologist in the US has suggested an experimental nucleoside treatment that might, in theory, offer some benefit although the treatment has not been tried before in a situation like Baby Charlie’s.

On Facebook, Charlie’s parents said that they were “heartbroken” and aghast that the hospital would not even allow them to take their son home to die.

Charlie’s parents have been fighting for months for the right to take their son to the US try this experimental treatment. By the time of the court decision, $1.7 million had been raise through crowdfunding media to cover expenses.

Unlike the Simon Crosier case, the withdrawal of treatment decision by the hospital was not secret.

Apparently under British law, parents do have the right to make decisions about the treatment of their children unless the treatment is not in a child’s “best interests” and usually it is parents who refuse treatment for their children on moral of religious grounds who end up in court.

The Charlie Gard case has caused enormous conflict among ethicists, lawyers and even religious leaders across the world as well as the general public.

However, in the last few days, Baby Charlie and his parents gained support from two important people: Pope Francis and US President Donald J. Trump.

At first, a Vatican official issued a statement on Baby Charlie that “We must do what advances the health of the patient, but we must also accept the limits of medicine and, as stated in paragraph 65 of the Encyclical Evangelium Vitae, avoid aggressive medical procedures that are disproportionate to any expected results or excessively burdensome to the patient or the family.” But a few days later and after much criticism of the first statement, Pope Francis expressed hope that the desire of 10-month-old Charlie Gard’s parents “to accompany and care for their own child to the end” will be respected. (Emphasis added)

Then, after a July 3, 2017 offer by President Trump to help, an unnamed US hospital came forward to offer free treatment to Baby Charlie.

CONCLUSION

On their GoFundMe page , Baby Charlie’s parents wrote:

If Charlie receives this treatment and it does work like the Dr in America thinks, it won’t be just Charlie’s life that has been saved, it will be many more children in the future, who are born with this horrible disease and it will open up other trials on other mitochondrial depletion syndrome’s.

We need to change things and show how determined parents can forge a path for other families encountering similar obstacles. We need to find treatments for incurable diseases. We need to give other people hope. We need to start saving lives.. hopefully starting with Charlie xxx (Emphasis in original)

Over my decades of nursing experience, I have seen people decide to try experimental treatments because they hoped for a cure, improvement or at least to help doctors learn more that may help someone else later.

Sometimes the treatments worked and sometimes they didn’t but after talking with these patients (or parents), I could not help but admire their love, courage, faith and determination.

May God bless Baby Charlie, Baby Simon and their parents!

Should Ethics Committees be Death Panels?

I volunteered to serve on a newly started hospital ethics committees in the 1990s. While I wanted to help analyze difficult cases and serve as a resource for hospital personnel with ethical concerns, I became increasingly alarmed when I saw cost containment and judgments based on “quality of life” brought up as decisive factors by others on the committee.

I understood more when I researched the beginnings of ethics committees.

BACKGROUND

After numerous failed attempts to legalize euthanasia, the Euthanasia Society of America invented the “living will” in 1967 as a first step in gaining public acceptance of euthanasia by promoting a so-called “right to die”. The group achieved much success by publicly promoting “living wills” as a patient rights document that would give people and/or their families  choice and control at the end of life or, especially after the 1976 Karen Quinlan case, if their quality of life was considered too poor.

Karen Quinlan’s case (where despite predictions,  she continued to live for years after her ventilator was removed) effectively extended the “right to die” to non-terminally ill people said to be in a “persistent vegetative state”, a term invented in 1972 to describe brain-injured people who were awake but assumed unaware.

The later cases of Nancy Cruzan and Terri Schiavo  further extended the “right to die” to withdrawal of feeding tubes and other basic medical care.

Eventually the Euthanasia Society of American became The Society for the Right to Die and finally evolved into Compassion and Choices and the “right to die” became “death with dignity” by lethal overdose.

ETHICS COMMITTEES

Ethics committees in hospitals began to emerge after the Quinlan case as an alternative to controversial court cases and became more prevalent in the 1980s, especially after the Baby Doe case in 1982. That case involved a newborn boy with Down Syndrome and an easily correctable defect that prevented him from eating safely. The parents refused the surgery and were upheld by a judge. An appeal was started but the baby died of starvation and dehydration before his appeal could be heard. Disability, pro-life and other groups and individuals were outraged.

Concerns about lethal medical discrimination against infants with disabilities resulted in the Baby Doe Regulations mandating maximal care to any impaired infant, unless certain exceptions are met.

However, there was much medical and legal opposition to these rules and:

“By the end of 1984,  the American Academy of Pediatrics and the American Hospital Association issued statements supporting the use of interdisciplinary ethics committees as an alternative to governmental investigation in such cases.” (Emphasis added)

AN UNEXPECTED PROBLEM

But a surprising development happened on the way to privatizing life and death decisions through ethics committees.

Some people and families resisted and insisted that medical treatment be continued for themselves or their loved ones despite a “hopeless” prognosis and the recommendations of doctors and/or ethicists to stop treatment. Many doctors and ethicists were appalled that their expertise would be challenged and they theorized that such families or patients were unrealistic, “in denial” about the prognosis or were mired in guilt or dysfunctional family relationships.

However, these doctors and ethicists were shocked when in 1991, a court ruled in favor of the husband of Helga Wanglie when he insisted that treatment be continued for his wife despite a “persistent vegetative state” diagnosis.

That court decision was widely criticized in ethics circles and in 1999, Texas enacted a medical futility law.

THE TEXAS ADVANCE DIRECTIVES ACT

In 1999, Texas became the first state to expressly permit doctors to stop life sustaining treatment without consent after a review process by an ethics committee. According to the Texas Advance Directives Act, when care is deemed “futile” or “inappropriate” and the patient or family disagrees,  the patient or family is given 10 days to find another health facility for the patient and pay for “any costs incurred” in the transfer. “If a provider cannot be found willing to give the requested treatment within 10 days, life-sustaining treatment may be withdrawn unless a court of law has granted an extension. “ (Emphasis added)

Thus an ethics committee becomes the equivalent of a death panel.

This kind of ethics committee overreach has now spread far beyond Texas. For example in 2002, I was involved in a similar a case in Missouri involving a brain-injured man despite the state not having a Texas-style law.  It was difficult to find another health care facility to accept the man within the 2 week deadline given by the hospital but ultimately the man not only survived but recovered after the transfer.

The Texas law is now being challenged in the case of Evelyn Kelly, individually, and on behalf of the estate of David Christopher Dunn v Harris Methodist Hospital, a case where a terminally ill, conscious man’s mother fought the ethics committee’s decision to remove his ventilator.

Although Mr. Dunn has since died, the case continues on the question of whether the Texas law is constitutional or not.

In the summary judgement motion filed, Ms. Kelly and her lawyers state that the ethics committee’s action “is an alarming delegation of power by the state law” and “is a far cry from the due process intended to protect the first liberty mentioned in Article 1, Section 19 of the Texas Constitution and that of the Fourteenth Amendment”. (Emphasis added)

Mrs. Kelly and her lawyers make a good point. How can a life and death decision be automatically delegated by law to an unregulated, unaccountable group of individuals outside the legal system?

Even worse, how can the right to live have less legal protection than the “right” to be dead?

The answers to these questions are critically important.

Parent Power

During the last few months, I have been writing about efforts by some legislators in Missouri and now Kansas to block the passage of Simon’s Law, a bill that exposed and sought to change the secret futility polices in hospitals that led to the death of Simon Crosier, a baby with Trisomy 18. I even wrote my own testimony in support of the bill.

But now in a stunning development, Dr. John Lantos wrote an opinion editorial in the Journal of the American Medical Association (JAMA) admitting that withholding life-sustaining treatment from babies with Trisomy 13 and 18 was really a value judgment rather than a medical judgment:

Thirty years ago, pediatric residents were taught that trisomy 13 and 18 were lethal congenital anomalies. Parents were told that these conditions were incompatible with life. There was a tacit consensus that life-sustaining treatment was not medically indicated. Clinical experience usually was consistent with this self-fulfilling prophecy.

But with social media, this changed.  Parents share stories and videos, showing their happy 4 and 5 year old children with these conditions. Survival, it turns out, is not a rare as once thought.”

This survival is even more impressive in light of a related JAMA article titled “Outcomes of Surgical Interventions in Children with Trisomies 13 and 18”   which stated that although “The median age of survival was 13 and 9 days, respectively, in children with trisomies 13 and 18”:

Among children with trisomies 13 and 18 who lived to 6 months, survival at age 10 years was 51% and 60%, respectively.

Note that what is changing the former medical perception of “incompatible with life” is not any new medical advance but rather loving parents who refused to accept the predicted death sentences for their babies, insisted on treatment and then used Facebook and other social and regular media to show off their children’s real lives. I call this Parent Power and it is based on love.

That power may help bills like Simon’s Law to finally pass.

DOWN SYNDROME (Trisomy 21)  AND PARENT POWER

I personally  learned about parent power when my daughter Karen was born in 1982 with Down Syndrome and a severe heart defect. 20+ years before Karen’s birth,  children with Down Syndrome were routinely institutionalized as accepted medical practice. But by the time Karen was born, almost all newborns with Down Syndrome went home with their parents and eligible early childhood programs.

What caused this dramatic change?

Again, it was primarily parent power.

Parents like Kay and Marty McGee not only ignored the standard medical advice to institutionalize their daughter but also fought for support and help for their daughter and others like her. They eventually founded the National Association for Down Syndrome in 1960 (http://www.nads.org/about-us/history-of-nads/ )

By working with other parents and reaching out to willing educational, legal and medical professionals, they helped change medical attitudes, the educational system and public acceptance of people with Down Syndrome who are now achieving goals once thought impossible.

Although it continues to be a long-term effort to ensure non-discriminatory medical treatment for people with Down Syndrome or other disabilities, people with Down Syndrome who were once predicted to die at an early age now have a life expectancy of 60 years and are achieving goals once thought impossible.

THE FIGHT  IS STILL FAR FROM OVER

Unfortunately and on the heels of the welcome editorial comment on babies with Trisomy 13 and 18, comes the news about the tragic circumstances surrounding the death of little Israel Stinson.

Two-year old Israel died after a judge suddenly and unexpectedly rescinded a court order that prevented a California hospital from removing the ventilator from little Israel before his parents could get an opinion from another neurologist after conflicting medical opinions about whether or not Israel was brain dead.

In this case, parent power was overturned by a single judge after the family thought that Israel was protected by a court order.

Not only is this personally tragic for Israel and his parents but this development also serves to devastate the crucial trust needed in our medical and legal systems.

 

 

 

Can We Choose to Live?

In a 2016 study “What does “futility” mean? An empirical study of doctors’ perceptions” in the Medical Journal of Australia distilled this definition from the majority of responses:

Futile treatment is treatment that has only a very low chance of achieving meaningful benefit for the patient in terms of:

  • improving quality of life;

  • sufficiently prolonging life of acceptable quality; or

  • bringing benefits that outweigh the burdens of treatment

Alarmingly, the article also states: “Doctors may reach a view that treatment is futile, informed by their definition of futility and clinical indicators such as functional status, disease severity, and age.” (Emphasis added.)

Over 10 years ago, I wrote an article “Futility Policies and the Duty to Die” about little-known futility policies being promoted, even in Catholic hospitals. These policies allow doctors and ethics committees to overrule patients’ or families’ decisions to continue  care or treatment when a person’s prognosis or “quality of life” was considered too poor.

In February, I wrote about the still not passed Simon’s Law here in Missouri that exposed the secret futility policies that led to the death of Simon Crosier, a baby with Trisomy 18.

However, a couple of weeks ago, a horrified nurse friend showed me two health care directive she recently received as a patient. One was from a Catholic  health care facility and the other was a standard Missouri durable power of attorney directive . The wording in both made her question whether such futility policies were now being incorporated into such directives.

I understand her concern.

THE CATHOLIC  DURABLE POWER OF ATTORNEY FOR HEALTH CARE DIRECTIVE

A person signs such a directive in order to have a family member or other trusted person make health care decisions when they are incapacitated. An legally incapacitated person is  defined as  a “Person unable to make rational decisions or engage in responsible actions. Mental and/or physical deficiency, disability, illness, drug use causing temporary or permanent impairment.”

“Living wills” and other advance health care directives,  invented by so-called “right to die” groups, claimed to give people the power to choose at the end of life

Remembering the prolonged dehydration deaths of Nancy Cruzan and Terri Schiavo, two non-terminally ill but severely brain-injured women said to be in the so-called “persistent vegetative state”, a person might sign a directive but want to prevent such a terrible death for himself or herself.

However, while this Catholic directive has a section to make such a decision, it also an asterisked section attached to both withdrawal and refusal of withdrawal:

I DO NOT AUTHORIZE my Agent/Proxy to direct a health care provider to withhold or withdraw artificially supplied nutrition and hydration (including tube feeding of food and water) as  permitted by law.*

*(In a XXXXX health care facility, nutrition and hydration may be withheld or withdrawn if I have an irreversible condition which is end-state or terminal AND if the means of preserving my life have likely risks and burdens which outweigh the expected benefits or are disproportionate without a reasonable hope of benefit.) (Emphasis added)

Using such terms as “end-state or terminal”  could, for example, apply  not only to a “persistent vegetative state” but also to Alzheimer’s or other dementia. “Artificially supplied” could encompass a simple IV while the asterisked section inexplicably does not even include the words “artificially supplied” before the food and water.  Along with using terms like “disproportionate without a reasonable hope of benefit” without stating who makes that determination or what the criteria is for benefit, the average person could be understandably confused in a real life situation.

THE MISSOURI DURABLE POWER OF ATTORNEY DIRECTIVE

Many, if not most, Missouri hospitals have this directive.

This directive has a section stating:

If I am persistently unconscious or there is no reasonable expectation of my recovery from a seriously incapacitating or terminal illness or condition, I direct that all of the life-prolonging procedures that I have initialed below be withheld or withdrawn. (Emphasis added)

This list includes not only “artificially supplied nutrition and hydration” but also antibiotics, CPR and “all other life-prolonging medical or surgical procedures that are merely intended to keep me alive without reasonable hope of improving my condition or curing my illness or injury.” (Emphasis added) Note that, according to the directive, a person need not have a terminal illness or be in a coma to qualify for withdrawal.

The next section can seem reassuring if a person has qualms about a decision to withdraw treatment or care being made too quickly or influenced by age or disability.  However, the directive only states that such treatments or care may  be tried-at the doctor’s  discretion-for an undefined “reasonable”period of time before withdrawal. Unfortunately, this section also includes automatic consent to pain relief, even in dosages that can suppress breathing and appetite as in terminal sedation:

3. However, if my physician believes that any life-prolonging procedure may lead to a recovery significant to me as communicated by me or my Agent to my physician, then I direct my physician to try the treatment for a reasonable period of time.  If it does not cause my condition to improve, I direct the treatment to be withdrawn even if it shortens my life.  I also direct that I be given medical treatment to relieve pain or to provide comfort, even if such treatment might shorten or suppress my appetite or my breathing, or be habit-forming. (Emphasis added)

The Catholic health directive also includes this section, almost verbatim.

CONCLUSION

With the help of the media, mentally disabling conditions like Alzheimer’s are often portrayed to the public as a fate worse than death and a terrible burden on a family. Tragically, the “right to die” mentality has led many people to conclude that they should die if they develop such conditions or, if dying, that their death may be accelerated to spare their families.

As a nurse who has seen the problems with advance directives firsthand, I helped design my own durable power of attorney advance directive without exemptions or checkoffs that could be misused or misinterpreted. I also educated my husband and family about the medical ethics involved.

As I wrote in my blog Living with “Living Wills”, there are better alternatives available to the standard kinds of advance directives even though no directive is foolproof.

Adequately informed consent is required for legal consent to surgery. Shouldn’t advance directives that involve life or death be held to the same standard before signing?

 

 

My testimony for Simon’s Law

On February 16,  a hearing was held by the Health committee of the Missouri Legislature on Simon’s Law.

Here is my submitted testimony to Dr. Frederick and all the committee members:

I am a past member of the Down Syndrome Association in St. Louis, an RN and legal nurse consultant and most importantly, the mother of a daughter who had special needs.
I cannot be at the hearing tomorrow but please accept my testimony in favor of Simon’s Law:

In September 1982, I gave birth to a beautiful baby girl we named Karen. Karen was born with both Down Syndrome and a severe heart defect called a complete endocardial cushion defect. A pediatric cardiologist was called in and even before I left the recovery room, he gave me the bad news about our Karen’s heart defect and even said that it was inoperable. He said to take Karen home where she would die in 2 weeks to 2 months.

This doctor turned out to be wrong. Further testing revealed that Karen’s heart defect could be fixed with one open heart operation and she had a 90% chance of survival.

My husband (a doctor) and I (an ICU nurse) were determined that our daughter receive the best medical care possible for her heart condition and without bias because she had Down Syndrome. We knew about the recent Baby Doe case where the parents of baby boy with Down Syndrome and an easily correctable tracheoesophageal fistula refused surgery so that their baby would die. The case went to court and a judge ruled that the parents could make that lethal choice. As medical professionals, we were appalled by this case but at least we could make sure that our daughter would have her chance at life. Or so I thought.

The bias against children like Karen soon became apparent when the cardiologist said he would support us “100%” if we chose to let our Karen die without surgery. I had to insist that Karen be treated for her heart defect the same way any other child would be treated for the same heart defect. To do otherwise was medical discrimination and illegal.

Then, the surgeon recommended for Karen’s pre-op heart catheterization was overheard questioning the wisdom of even treating “all these little mongoloids”! Another doctor sympathetically told us that “people like you shouldn’t be saddled with a child like this.” We were stunned by this negative view of children with Down Syndrome.

Later on when Karen developed a pneumonia that was being successfully treated in the hospital, I found out that my trusted pediatrician had even made Karen a “Do Not Resuscitate” behind my back because I “was too emotionally involved with that retarded baby”. The DNR was rescinded and we took Karen home but I found it hard to trust any doctor after that.

Unfortunately, Karen developed another bout of pneumonia and died of complications just before her scheduled open-heart surgery. But even at the very end, when Karen was apparently dying, a young resident physician “offered” to pull all her tubes so that she would die as soon as possible. I reported this young man to the chief of pediatric cardiology who was furious with the resident. (This chief of cardiology later started a clinic for children with Down Syndrome to meet their special health needs.)

Although we lost Karen when she was just 5 ½ months old, I still treasure my time with her and because of her, I became an advocate and volunteer for people with disabilities.
I wish I could say that my story is unique but I have seen many similar situations over the last three decades involving people of all ages with disabilities.

Therefore, I beg you to approve Simon’s Law. It will potentially save lives as well as send a strong message that medical discrimination against the disabled based on subjective judgements of “medical futility” and/or predicted “poor quality of life” is wrong.

Sincerely,

Nancy Valko, RN ALNC