A good decision is based on knowledge and not on numbers. Plato
A good decision is based on knowledge and not on numbers. Plato
In her 1972 testimony before Congress, Dr. Elizabeth Kubler-Ross, author of the 1969 best-selling book On Death and Dying, stated that “We live in a very particular death-denying society. We isolate both the dying and the old, and it serves a purpose. They are reminders of our own mortality.” What she wanted was recognition on the part of the government that families could be helped more with home care and visiting nurses at the end of a loved one’s life than with institutional and aggressive medical care. Her testimony was a description of a philosophy of medical care known in England as hospice – a medieval word for the traveler’s hostels run by monks in the Alps.
The hospice movement begins in the USA
The hospice movement in the US had begun in the 1960s when the nursing school dean at Yale University invited Dame Cicely Saunders, the mother of the hospice movement in England, to teach for several months. Hospice growth was stuttering over the next few decades, with growing pains coming not only from wrangling over Congressional allocations of money, but also from the process of trying to identify the suitability of patients for hospice care. The requirement for a prediction that a patient entering hospice care would live less than 6 months proved extremely difficult, particularly when the patients did not have cancer.
Initially, hospice care was viewed negatively by many as either giving up on life or as a form of euthanasia or doctor-assisted suicide. It is none of these. Hospice is a shift away from attempting to cure medical problems and toward care of the whole patient by a multidisciplinary team with the patient and family at the center. From demonstration hospice projects launched in 1979 to the current care of more than 1.65 million Americans a year, the philosophy of caring rather than curing has proven itself good. In 2007, a paper in the Journal of Pain and Symptom Management reported that patients who had hospice care lived slightly longer than similarly ill patients who were treated conventionally. This surprising conclusion was followed two years later by a New England Journal of Medicine report that patients with non-small cell lung cancer may live longer with hospice care than with other therapies.
Shifting the focus
Hospice is medical care, but care with an aim different from the curative focus of conventional medical care. There is no fighting imagery used in hospice – no war on the cancer, no battle to be bravely fought. The care in hospice is palliative, emphasizing comfort and acceptance, with the meeting of physical needs in an environment as close to home as possible. The patient and family are the unit of care, and the team consists of the patient’s doctor, a hospice doctor, nurses, nurses’ aides, social workers, physical therapists, spiritual counselors, bereavement counselors and volunteers. The focus of patient care is pain and symptom control, as well as emotional and spiritual support for all involved.
Hospice care begins with a doctor’s referral when a patient and his family realize they are ready to turn away from the aggressive attempts to cure a problem which will eventually result in death. The Medicare Guidelines for entering a hospice program require that a patient have a terminal illness with less than six months to live. (Medicare is the payment source for most hospice care). But that six-month prognosis should not be confused with length of care in hospice – care is provided for however long it is necessary. Over 12% of hospice patients live past the initial 6 months of care.
Once a hospice referral is made, a team member, usually a nurse, begins an assessment of physical and emotional needs and crafts a team to meet those needs. Hospice provides the home equipment, medications and support for family as they learn to provide physical care. Volunteers help with respite care to allow family members time to themselves. Social workers evaluate economic needs and pastoral care members address spiritual and emotional needs. Short term hospitalizations are arranged if necessary for symptom control. While most hospice care takes place in the home, similar teams operate in institutions like hospitals, nursing homes and fee-standing hospice facilities, depending upon the availability and competence of family members.
A longer period of comfort
Sadly, over a third of hospice enrollees live less than a week. The time to begin thinking about hospice care is early in the course of a potentially lethal illness since preparation may help a patient live a longer period of a terminal illness in more physical and emotional comfort.
It is helpful to have time to see what hospice organizations are available locally, to check certifications, and to talk with people who provide hospice services. The National Hospice and Palliative Care Association is an invaluable source of information.* Hospitals are committed to helping arrange hospice care and a direct appeal to the hospital’s hospice coordinator is possible if the patient’s doctor does not make a referral. If a patient is not ready for hospice care, but is also unwilling to continue aggressive curative attempts, palliative care is also available – care aimed at comfort and symptom control alone rather than cure. An example is quitting or refusing chemotherapy for cancers which respond poorly.
Hospice care is paid for by most insurance policies in the US (but not in other countries) and under the Medicare Hospice benefit. Medicaid is also a payer. Surveys report that 94% of families feel their experience with hospice care was very good or excellent. The US Department of Health and Human Services is now behind expanding the availability of hospice care because it “holds enormous potential benefits for those nearing the end of life…” So as medicine moves into the brave new age of genetics, with new, individualized treatments for cancer, and more and more procedures to rewire, replumb and reconstruct the body, hospice care also moves forward, bringing the elderly and the dying out of isolation and educating the people who love them about the universal and necessary process of dying.
We are now 100 years into an epidemic of avoidable, tobacco-induced health problems and over 50 years into the attempt to stop it, with more knowledge accumulating every year about the toll tobacco smoking takes on every part of the human body. Each year, smoking costs US society $130-170,000,000 in medical care, $150,000,000 in lost productivity and 400,000 lives lost prematurely. Over 160 million people live with serious, smoking related illnesses. Still, 20% of adults smoke regularly, and young people continue to join their ranks. If you never picked up the habit, be glad. If you have picked it up and managed to kick it, congratulations. You are part of a slow public health success story, and, by understanding the smoking habit, you may be able to help someone else quit.
The evolution of cigarettes
Tobacco was the first crop sold for profit by the American colonists, who introduced Europeans to pipe-smoking and tobacco chewing in the 1600s. However, the smoking habit did not begin in earnest until the invention of a cigarette rolling machine in 1883. By the 1940s smokers in the United States lit up 300 billion cigarettes per year and during WWII, soldiers’ ration kits included cigarettes. By the 1950s, 44% of US adults smoked regularly. Psychiatry texts in the 1960s urged doctors to light up with their patients and, by then, cigarette consumption topped 500 billion per year. Older adults today recall being raised in smoke-filled houses, driven around (without seatbelts) in smoke-filled cars, and sent to the corner store to buy cigarettes for their elders.
Recognition of the problem
Though the 1964 Surgeon General’s report confirmed what many people by then knew – that tobacco was bad for health, wrinkled skin prematurely and caused lung cancer, other lung problems and vascular disease – cigarette consumption rates continued to climb well into the 1980s. But then decades of educational, political, legal and economic pressures on smokers began to work. By 2012, public places were largely free of tobacco smoke, ex-smokers outnumbered active smokers, and cigarette consumption rates had fallen back to the 1940’s levels. Anti-smoking advocates are rightly proud of their efforts, but the credit must also go, in great measure, to the individuals who did battle with the smoking habit and succeeded. Breaking the smoking habit is difficult, often requiring many attempts and relapses before the goal is achieved.
Nicotine is addictive
Smoking becomes a habit because tobacco contains nicotine, which changes the chemistry of the brain in a way that makes the tobacco user uncomfortable when the nicotine level falls. Tobacco companies have exploited the addictive qualities of nicotine by manufacturing their products to diminish negative physical effects and enhance addictive ones. Menthol soothes the smoke-irritated throat. Nicotine is added in just the right dose – not enough to provoke toxic symptoms like nausea, vomiting dizziness and diarrhea, but just enough to ensure the desire for more.
In addition to physical addiction, smokers develop psychological addiction, a learned desire or craving for smoking that arises from the association of smoking’s pleasure with certain situations such as social gatherings, meals, stressful or anxiety provoking circumstances or boredom. Sophisticated advertising techniques add to the social cachet of smoking and subtly enhance these psychological cravings. The combination of physical and psychological addiction means a two-pronged attack is often necessary to help a smoker who wants to quit.
Two-part attack on a two-part addiction
Physical addiction to any substance produces withdrawal symptoms when the substance is no longer available to the body. In the case of tobacco, irritability, anxiety, insomnia, abdominal cramps and depression occur within hours of smoking cessation. These symptoms peak and begin to diminish within several days and will stop in a predictable period of time – about 2-4 weeks – after the last does of nicotine.
Since nicotine can be delivered to the brain without the many carcinogenic chemicals in cigarette smoke, nicotine replacement therapy (NRT) is helpful to someone who is trying to stop a smoking habit, allowing time to deal with the psycho Nicotine is available in non-prescription gums, lozenges and skin patches. Nicotine inhalers and nasal sprays require a prescription. Eventually, though, the physical withdrawal symptoms will have to be suffered when the ex-smoker decides to give up the nicotine.
E-cigarettes also deliver smoke-free nicotine, by vaporizing it in water. They are highly engineered products containing plastics, ceramics and metals and their long term risks are as yet unknown. Unlike the other nicotine delivery systems, e-cigarettes involve regular smoking behaviors and cannot be expected to help diminish the psychological cravings involved in the habit.
Psychological cravings that prompt smoking are often far longer lasting than physical withdrawal symptoms – and more responsible for relapse. In most studies of smoking cessation, behavioral therapy is key to long term cessation. Such intervention can take many forms, including one-on-one counseling, supportive group therapy and even online group participation. Many online resources are available to help smokers cope with this aspect of tobacco addiction. (see list below).
Two drugs are also commonly prescribed to help smokers quit. One, varenicline (Chantrix), attaches itself to nicotine receptors partially stimulating them and relieving withdrawal symptoms and at the same time blocking a sense of reward from inhaled nicotine. Another drug, Bupropion (Wellbutrin), is an antidepressant. These drugs improve smoking cessation rates slightly, and are more effective if used in conjunction with NRT. Both, however, are associated with some troublesome reports of behavioral changes, now noted in black box warnings on their packaging.
The cold turkey method- just as effective
While public health measures have contributed significantly to decreasing smoking rates, breaking the smoking habit remains an individual project and the single most effective measure a smoker can take to improve health. One curious smoking cessation statistic confirms what many doctors have long observed – that cold-turkey quitting is as effective as any of the assisted methods. It appears that the whole-hearted decision to stop, once made without any reservations, could be the most important factor in long term success. This method has no unwanted side effects or risks and does not require withdrawal of nicotine replacements once the psychological smoking habit is tamed. Non-smokers can play a large role in aiding people whom they care about to make this final decision.
Resources for Smokers Who Want to Quit
At my Grandmother’s house, there was little for children to do after dinner but play games and eavesdrop on the grownups who sat around the table for hours after the end of the meal. Back then, the adults seldom talked about “diseases,” but they did seem to think that bodies, like cars, had parts that could be relied upon to malfunction with age and abuse. Dining room table wisdom held that “heartburn” was the expected result of overindulgence in rich food, alcohol, coffee and cigarettes, and that Alka Seltzer was the best remedy. I can still recall the fizz of the tablets dissolving in water.
Renaming a symptom
Heartburn is a vivid word image for the most common gastric complaint – distressing, burning pain just beneath the breastbone. With time, technology, and advertising, heartburn has been replaced by the anatomically correct term gastro-esophageal reflux (GER), which accurately describes the source of the symptom: acid splashing up into the esophagus from the stomach. However, renaming a symptom, heartburn, with its cause, reflux, does not make it a disease, no matter how many TV commercials advertising drugs for GERD (gastro-esophageal reflux disease) say otherwise. Diseases are sometimes associated with GER, but they are diseases that promote reflux, diseases treated with medications that promote reflux, or diseases that result from stomach acid eating away the sensitive lining of the esophagus.
How the esophagus works
Stomach acid is potent stuff, necessary to break down food and to kill off the bacteria that come along with it. Acid also helps with the absorption of vital substances like calcium, iron and Vitamin B12. The stomach lining produces acid and is remarkably resistant to its corrosive effects, unlike the lining of the esophagus above. The job of the esophagus, a muscular tube, is to assist gravity in getting food from the mouth to the stomach via a series of coordinated contractions. In its lowest portion, the esophageal muscle works like a valve. It opens to let food into the stomach and closes to hold it there as digestion begins. The esophagus can also reverse its normal action, opening the valve and emptying the stomach in a hurry, propelling its contents back out the mouth. This very unpleasant action, called vomiting, is perceived mainly as a squeezing, muscular sensation rather than a burning pain, because the exposure of the esophagus to the upward rushing acid is short-lived.
Reflux is backwards, upward flow of stomach contents to the esophagus
Reflux is a more leisurely affair. The muscular valve at the junction of the esophagus and the stomach is not a tight one, and it is subject to the effects of foods and drugs, and diseases that limit its responsiveness (see sidebar). Reflux is more common in people with hiatal hernias – upward slippage of the stomach top into the chest through a weak spot in the diaphragm (muscle between the abdomen and chest). With a stomach full of actively digesting food, reflux can occur simply with lying down or bending over, positions that limit gravity’s help in holding stomach contents down. And held down they must be, for at least awhile, because the stomach is a reservoir where the initial breakdown of food occurs.
The abdomen is a crowded space
The reservoir sits like a pool behind a dam, awaiting opening of the pyloric valve between the stomach and small intestine. The stomach shares close quarters with the liver, spleen, pancreas, small intestine and colon – and with fat tucked around all these organs. The more fat -and the tighter belts and pants are – the less the space available. The upper valve, between the esophagus and the stomach, is the weak link when pressure rises; it gives way while the pyloric valve holds fast, and stomach contents flow into the esophagus. The upper valve also yields in pure overeating (e.g. hot-dog eating contests), when the stomach fills faster than digestion can proceed.
Acid in the wrong location causes trouble
Chronic exposure to stomach acid inflames the lining of the esophagus, and then diseases appear – shallow erosions, deeper ulcers, and scars that interfere with swallowing. Risk of esophageal cancer rises. Acid reflux can go as high as the mouth and erode the enamel of the teeth, add to gum disease, and produce mysterious sore throats and hoarseness. Lung problems and asthma are more frequent in people with chronic reflux.
Reducing stomach acid works, but may cause other problems
Heartburn sometimes requires medical evaluation (see sidebar), but is often responsive to simple antacids like Maalox that temporarily lower stomach acidity. Powerful, safe and effective drugs called proton-pump inhibitors (Prilosec, Nexium, etc.) block the last step of acid production in the stomach. Reflux goes on, but stomach contents are no longer corrosive. But no drugs are free of unintended consequences. Without acid, food digestion is slower. Risks of pneumonia and gastrointestinal infections in long-term users rise, suggesting that in an acid-free environment, bacteria survive in the gut and spill upward into the respiratory tract. Some researchers believe that proton-pump inhibitors also turn acid production off in osteoclasts, cells that build bone, resulting in an increased rate of hip fractures in long-term users. One very worrisome statistic in the age of altering stomach acidity is a rise in rates of esophageal cancer, though the cause of the rise is not fully understood. Lack of stomach acid, however, has long been known to be a risk factor for the development of stomach cancer.
New generations have replaced my grandparents and TV and social media outdraw after-dinner talk, but heartburn, though renamed, is still just a symptom of a mechanical problem – acid reflux. Blunting the effects of acid with over-the-counter or prescription drugs is a temporary solution that does nothing for the inciting problem – the reflux. Weight loss, smoking cessation, elevation of the head of the bed (on 6-9” blocks), avoidance of large meals and offending foods and drugs, and allowing several hours to elapse between meals and bedtime are the keys to taming reflux and keeping GER from becoming GERD.
Common Factors in Patients with Heartburn:
• Obesity, the most common factor.
• Diabetes (delays stomach emptying)
• Pregnancy (space shortage + hormone effects)
• Medications: antihistamines, antidepressants, narcotics, calcium channel blockers for high blood pressure and heart disease, progesterone, anticholinergics (bladder control drugs), some sedatives and tranquilizers
• Foods: fried foods, chocolate, alcohol, caffeine and others.
• Smoking(stimulates stomach acid)
• Asthma and anti-asthmatic medications
• Hiatal hernia
• Stomach outlet obstruction by ulcer or tumor
Seek a Medical Evaluation for Heartburn that:
• Wakes you up at night
• Happens regularly, more than once a week
• Is unresponsive to simple antacids like Maalox or Rolaids
• Recurs promptly after antacids or drugs wear off
• Has associated symptoms: nausea, vomiting, abdominal pain, difficulty swallowing, increasing abdominal girth, or blood in the stool
In the early 1920s, John Romulus Brinkley, an entrepreneurial Kansas physician with dubious professional credentials, ran a lucrative business implanting goat testicles under the skin or in the scrotum or abdomen of patients who sought his help for any one of twenty-seven ailments, including flatulence. He was one of many medical hucksters who took advantage of an unregulated medical world in the United States at that time and made fantastic claims for their medical treatments, potions, and elixirs. Eventually, Brinkley’s medical license was revoked by the state of Kansas, and he moved south to Texas, where he advertised his services via a folksy radio station broadcasting from just over the border in Mexico. His station’s powerful antenna beamed his ads and promises all the way to the Canadian border, drawing patients south for his “treatments.” In the end, Brinkley is said to have made millions of dollars before a federal grand jury indicted him in 1941 for postal fraud. He died, bankrupt, in 1942, before he ever stood trial.
Fast-forward to a well-regulated medical world in December 2014. A famous ex-hockey player suffers a stroke and travels to Mexico for stem cell treatments. His family claims great results, and his therapists corroborate the reports, leaving many who read the story to wonder whether their brain-damaged relatives could also benefit from this therapy and why they would have to travel to Mexico to get it. Is this the same type of treatment being offered, without FDA approval, at the new stem cell clinics popping up in many American cities? And why is stem cell treatment not FDA approved? The answers require some understanding of stem cell research, clinical trials of new therapies, and the reasons why the FDA exercises caution when approving novel therapies.
What are stem cells?
Stem cells begin in a fertilized human egg, which splits into two cells, then again into four, and again into eight, and on and on. The first few dozen cells have the potential to become any type of cell in the body and are called embryonic stem cells. As the fetus develops, cells begin committing themselves to different roles, shaping a human body with all its complex organs. Some cells in each organ, however, retain enough of their primitive character to serve as a reservoir of cells for the repair and growth of the organ. These cells are called adult stem cells. The term “stem cell treatment” might refer to procedures involving either type of stem cell, but the stem cell therapies being offered commercially are adult cells.
The hope of stem cell therapy
The promise of adult stem cell therapy is based on the hope that stem cells can be harnessed to perform on demand and develop into mature cells to replace aging or damaged cells. In an ideal world stem cells could be coerced to act like the stem cells residing in bone marrow do naturally and regularly. Marrow stem cells make new blood components continuously – red blood cells, white blood cells, and platelets. They must do so because blood cells have life spans of only a few weeks.
Bone marrow transplants were the first stem cell treatments. They were used successfully in treating blood cancers and worked by replacing the patient’s bone marrow with that of a healthy donor. Bone marrow contains many types of blood and immune cells, but it is the stem cell component that repopulates the patient’s diseased marrow after it has been wiped out by chemotherapy. Originally, drugs were needed to keep the body of a recipient of a bone marrow transplant from rejecting the foreign cells, but advances in doctors’ ability to separate healthy stem cells from all the other bone marrow components now make it possible for physicians to give the patient his or her own cells, making antirejection drugs unnecessary.
The discovery that stem cells existed in many different tissues widened research horizons and raised hopes that stem cells could be used to repair and regenerate more tissues than just bone marrow. A wide variety of stem cell experiments aimed at treating a number of diseases, including multiple sclerosis and heart disease, are now being conducted. Ongoing clinical trials at academic medical centers are enrolling patients under strict protocols. Such trials are the only objective way to establish the safety and efficacy of stem cell treatments compared to standard care and to placebos. But so far, these trials are still ongoing, and the FDA has only approved marrow and umbilical cord blood stem cell use for the treatment of blood diseases like leukemia and lymphoma.
The hype: what are stem cell clinics selling?
But what about the stem cell clinics in the United States that offer treatments for a wide range of diseases related to aging, including diseases such as Parkinson’s disease, Alzheimer’s disease, chronic lung disease, and cardiac disease? These clinics are entrepreneurial ventures that operate without FDA approval. Stem cells are not considered drugs because they are harvested from the patients own body for re-injection into it; therefore, the FDA has no jurisdiction over the hundreds of stem cell clinics operating in the United States. If trouble occurs in these clinics (and it has, including death) then state medical boards are responsible for policing the people providing services until some kind of FDA regulation is put in place.
Fat is the usual stem cell source
Today’s stem cell clinics appeared after the discovery that fat was one of the richest sources of adult stem cells. With the purchase of a cell separator and participation in a weekend seminar, any doctor can open a stem cell clinic. During stem cell treatments, fat is harvested from each patient through a version of the liposuction process. The stem cells that reside deep in the fat, near blood vessels, are concentrated in the cell separator and mixed with the patient’s plasma to cause the stem cells to begin to divide and grow. Some clinics also obtain marrow stem cells through a large needle inserted into the bone of the hip, and, in some clinics, lasers are used to activate the stem cells and raise them from a dormant state to a reproductive one. Treated cells are then injected back into the patient, into the bloodstream, under the skin, into a joint, or into the spinal fluid. In theory, these cells find their way to the targeted, ailing tissues, turn into the desired cell type – such as knee cartilage – and heal the problem. Some practitioners, however, believe that the stem cells may not propagate themselves but only stimulate the body’s natural repair processes.
Stem cell clinic literature often gives the impression that these procedures are risk free, but, in fact, all invasive medical procedures carry some risks, like infection and adverse effects such as dizziness. Recently several patients became blind after stem cell injections aimed at curing threating the macular degeneration in their retinas. Risks and side effects should be disclosed to every patient, even if their rates of occurrence are low.
No long-term follow-up studies available
Stem cell treatment is so new that there have been no long-term follow-up studies done on large numbers of patients, the type of studies that are used to uncover unexpected trouble with new procedures. Since stem cells are meant to reproduce and grow and since manipulating them outside their natural environments and injecting them into patients presents a risk of changing the programming that keeps their reproduction under control, long-term follow up studies over many years are essential. While some stem cell clinics purport to do studies on patient outcomes, they demand that their patients pay for their treatments, and no legitimate study would require payment for the patients to be involved. Furthermore, the studies done in these clinics do not include control cases to assess placebo response or control groups to compare results with standard treatment.
Over-regulation or protection against charlatans?
With advertising claims being made that stem cell therapy rejuvenates aging body parts, with many people suffering from painful joints and other ravages of life, and with the FDA restricting stem cell treatment approval to specific blood conditions, what is the interested potential patient to think? That in the last one hundred years we have overreacted to charlatans and are using regulatory agencies to unnecessarily restrict useful medical therapies? Or that the injection of stem cells into the body with the expectation that these cells will reach the correct destination and perform just the repairs needed is a bit like trusting the promises John Brinkley once made about his goat testicle therapy? Desperation may drive some people to try what is easily available (though very expensive), but the safest course at this time is to stick to FDA sponsored stem cell studies for any attempted treatment. And to remember that placebo effect of any medical treatment, even antibiotics, accounts for 30% or more of the positive responses in studies of efficacy. Glowing reports from friends and relatives don’t necessarily mean that demonstrable changes occurred in their bodies. Research on stem cell treatments continues, but there is still a very long way to go.
Readers wanting to know more about some topics pose very good questions. My original magazine column about immunization to prevent shingles (September 2011) generated enough reader mail to warrant another column sharing some of the answers.
Recognizing recurrent shingles episodes:
One reader had suffered through an eruption of ophthalmic shingles, which involves the nerve that carries sensation from the eye, including the cornea, from the skin around the eye, and also from the forehead. The reader wanted to know “What are the signs and symptoms for a re-occurrence of the zoster virus in the eye so I would know what to look for if I am getting an attack?” As in other areas of the body, symptoms that come before the rash erupts in the eye and the face are sensory – tingling, burning, itching and pain. Warning sensations in ophthalmic shingles might also include irritating dryness and a sense something lodged in the eye. Our reader understood that taking an antiviral drug early in the course of an eruption might lessen the likelihood of scarring of the cornea, so paying attention to early symptoms has therapeutic consequences. If an abnormal sensation persists for several hours without explanation or response to simple measures like rinsing the eye out, then the symptom is worth bringing to the attention of the doctor. That said, the use of antiviral drugs early in the course of a shingles outbreak does not prevent the eruption from progressing, but it may shorten the duration and lessen its intensity. When the surface of the eye is involved, anything that can be done to prevent corneal scarring is of some value.
Drugs that make the virus awaken
The same reader also wanted to know what drugs might predispose her to another eruption, and how to avoid them. The drugs that put people at most risk for a herpes zoster outbreak are the ones that suppress the function of immune cells in the body. The most common offenders belong to the steroid class on anti-inflammatory drugs, and have names like prednisone, dexamethasone, decadron, and prednisolone. They are used to treat conditions like multiple sclerosis and lupus and rheumatoid arthritis and when used for periods longer than a week, they begin to impair immune response. Sometimes they are part of a chemotherapy regimen for cancer. Other chemotherapy drugs and radiation also impair immune cell function, so shingles eruptions are not surprising in patients undergoing cancer treatment. Paradoxically, steroids are part of the treatment for shingles – but they are used for only a short time, to decrease inflammation.
Another reader wrote:” My husband never had Chicken Pox and yet he did have a severe case of shingles and he was in his 40’s when they occurred. At that time we were told the opposite of the article…we were told he got shingles because he had never had Chicken Pox. This was over 20 years ago so perhaps research has changed that. Does the fact he had shingles mean he cannot get the vaccine?” There are many people whose childhood chicken pox was so mild that they have no memory of the disease. Blood testing will show whether or not there is any trace of immunity to the virus in people who think they did not have the disease. An adult who contracts chicken pox for the first time has a rash that involves much more of his body than the shingles rash does. He is also extremely sick, much more so than a child with the disease. So if an adult develops what is a typical shingles rash, it is considered proof that he has had chicken pox in the past.
You probably did have chicken pox
Age 40 is on the young side for shingles, but there are many idiosyncrasies in the immune system, with some people have worse immune “memory” for specific viruses than others do. Having had a shingles eruption does not prevent this reader’s husband from getting the vaccine, and given that it is now over 20 years since the last time the virus stimulated his immune system, immunization might be a very good idea. Guidelines for vaccine administration also do not exclude people who think they did not have chicken pox as a child, even though, in theory, a vaccine made from a live, weakened virus could cause a full blown case of chicken pox in a chicken pox virgin (more on different vaccine constructions below). It is estimated that 99% of people in the US have had chickenpox, whether or not they are aware of it.
Being refused the vaccine
Getting an immunization proved difficult for another reader. He went to his county health department seeking a shingles immunization, but he was turned down because he has non-Hodgkin’s lymphoma, a form of lymphatic system cancer. While his disease is in remission and his blood work indicates good immune cell function, there is a theoretical risk that the vaccine, which contains a live, weakened version of the virus, will reactivate the line of white cells that caused his lymphoma. Many people face this type of risk-balancing problem in choosing whether or not to get a vaccine, and each individual case requires weighing risks versus benefits. In some cases, for example someone with AIDS who has good white blood cell tests and is not sick, the patient’s doctor may advise getting the vaccine because the risk of the effect of a shingles outbreak is greater than the risk that the virus in the vaccine will cause trouble. In the case of people with history of cancers that arise directly from immune system cells, however, no one wants to take a chance of triggering cells to become cancerous by the introduction of a live virus in the form of a vaccine. In addition, no one wants to introduce an infection that the immune system cannot control. These problems are the reason that researchers have pushed to develop a new vaccine, just becoming widely available in in 2017-18, which does not contain any live virus.
The old and the new vaccines
Lastly, several readers inquired about the frequency of the zoster vaccination. Immunizing for shingles is relatively new, and recommendations may change, but right now, Zostavax, the old vaccine, is recommended for all people over age 60, as a one time shot. Zostavax cuts the rate of shingles by 51% and the development of post-herpetic shingles pain by 65%. The new vaccine, Shingrix, is recommended beginning at age fifty and in tests improves these prevention rates to 98% and 85% respectively. Shingrix requires two separate doses. The effectiveness of the vaccines does wane over time, and there is more experience with the old one. Currently there are not any guidelines about repeat administration, but there are no contraindications to getting the new vaccine for people who have already had the old one.
Where to get immunized
Immunizations are available at pharmacies, grocery stores, county health offices, and walk in clinics and all of these facilities have guidelines which will exclude some people. Anyone excluded by general criteria should review the reason with a doctor who cares for the problem that caused the exclusion.
Each of us begins life as a single cell. The chromosomes within, formed out of long chains of DNA called genes, carry all the information necessary for the formation of a human being. The entire complement of DNA, including long segments that have no known function, must replicate itself billions of times over the course of development of the baby and the life of the individual who emerges from the womb. If there were never any errors in the original DNA, if there were never any errors in replication of DNA, and if genes did not turn on and off in response to environmental factors, aging would not occur and human misery would be confined to infectious diseases and trauma. Now that the human genome has been decoded, can we look forward to a disease free world and to extension of the seemingly fixed lifespan of 110-120 years? Not likely, but the focus on the genetic contribution to disease will change medicine, for better and for worse, in the years to come.
Visible genetics: the family history
Doctors have always taken a family history as part of the initial evaluation of a patient. Physical features, health and ailments that run in families are visible genetic information. Height and body shape, nose shape, bunions, baldness, premature white hair all “run in” families. So do a variety of illnesses. Family history tells the story of genes that have expressed themselves already. Laboratory analysis of an individual’s genes attempts to predict what may happen in the future.
In a few rare illnesses such as cystic fibrosis, sickle cell disease, and some devastating neurological degenerative disorders resulting from errors in very small parts of the genetic code, the genetic information is clear cut – the individual has an abnormal gene, and they have or will eventually show signs of the disease. These diseases, along with problems of chromosome breakage or duplication such as Downs syndrome, are the object of prenatal screening tests. For the most part, however, genetic results are statistics – the odds that a problem will eventually appear. Potentially damaging treatments applied to healthy people, based only on future odds, is the “for worse” change that may come with the addition of genetic information in the practice of everyday medicine.
The problems with statistical medicine
Statistical predictions apply to large numbers of people, not to individuals. Not all people who have a gene associated with Alzheimer’s dementia will get the disease, but since more people born with that particular gene will develop Alzheimer’s than those born without it, the gene is said to increase the risk for the Alzheimer’s disease. The prediction of a strongly hereditary trait – one associated with an 85% likelihood that some kind of illness or cancer will appear by a given age – gives the impression that the illness is almost inevitable for every member of the family. But from the standpoint of an individual within the family, the risk is always 50%. Either they will or will not be affected.
The importance of treatment availability when disease risk is high
What does knowing that there is an increased risk of suffering a given disease do for you? If there is a treatment that prevents the evolution of the disease, and it has no adverse effects, then it would be reasonable to gamble on undergoing treatment based on worry that the disease will appear, rather than waiting to see if one is in the lucky percentage that escapes. Women who undergo removal of their breasts and or ovaries because they have a genetic trait that greatly increases risk for cancer in these organs have made this judgment. (They also must accept the minuscule risk that breast or ovarian cancer can still occur even after surgical removal of the organs.)
In the case of the devastating brain degeneration known as Huntington’s disease (HD), people usually know they are at risk because one parent has developed the characteristic dementia and movement disorder in midlife. Because of the so called dominant inheritance pattern of HD, half of the offspring of the parent, statistically, will also be affected. But no one knows which children carry the gene unless testing is done. Now available and definitive, the genetic test for this disease is a double-edged sword. Life is normal for the carriers of the gene until middle age. The knowledge of what is coming might convince the bearer of the gene not to reproduce, but also make living a full and happy life seem out of reach.
The role of genetics in common diseases
What about the prediction of increased susceptibility to more common diseases, such as heart disease and some cancers? This aspect of genetic testing may help people with their motivation to lose weight, exercise, eat well and get their routine colonoscopies. Genetic analysis of cancers that have already developed is already proving helpful in the design of specific treatments for specific tumors. All tumors of lung or liver or brain are not alike and “one size fits all” treatments, the only type available in the past, will give way to individualized plans and drugs. An individual’s responses to drugs for conditions such as high blood pressure and heart disease, including both desired and to adverse effects, is also based on genetic makeup. Personalized treatments are already increasing for these problems.
Genetics and aging
Genetic studies are also teasing out at least some of the pathways involved in aging. Will advances someday lead to immortality? Very unlikely, and a bad social idea anyway. But understanding the way the genes gradually fail in their the mission of cellular repair may well lead to better old age. The key will be to use the knowledge early in life. So far it looks like preventing the errors in DNA replication that contribute to the diseases of old age depends on the same old things that your grandmother might have advised – eating modest amounts real food, preferably from fresh sources in all food groups, avoiding sugar and other refined carbohydrates, drinking little alcohol, avoiding sunburn, keeping in motion, sleeping enough, and most importantly, avoiding the biggest DNA error trigger of all – cigarette smoke. Of equal importance is recognizing that no matter how much we know and try to prevent, life will eventually wind down. Living one’s individual allotment well and fully is as important as avoiding cancer – and may actually help genes replicate correctly.
(For readers especially interested in how the genetic code works, Francis Collins’s The Language of Life, and Matt Ridley’s Genome: The Autobiography of a Species in 23 Chapters are good starting points.)
Large scale studies of survival after cardiac arrest have produced dismal statistics, with survival to hospital discharge of 17.6% when the patient is already in the hospital at the time of the arrest and only 6.1% when the arrest occurs outside the hospital. The development and widespread deployment of portable automatic external defibrillators (AEDS) in public places has increased the number of people who make it to the hospital after cardiac arrest. However, the survivor’s longer term outcome depends in large part on how much brain damage occurs during the arrest and whether or not the restoration of circulation damages the brain further, a phenomenon called reperfusion injury. Because the odds of initial survival have improved, and because lowering the core temperature of the body appears to lessen reperfusion injury, the subject of hypothermia has emerged as a vibrant area of research and therapeutics.
Therapeutic hypothermia is an old idea
Hippocrates (460-370B.C.) recognized the value of cold temperatures in the outcome of soldiers with head injuries and in people suffering from tetanus. In the 1800s, Napoleon’s surgeon used ice to prepare limbs for amputation because it numbed pain and reduced bleeding. Over much of history, miraculous recoveries were reported in victims of cold water submersion. But not until the late 1950s did therapeutic hypothermia become a routine part of some surgical care, when experiments in animals demonstrated its value in protecting the brain during the open-heart surgery. Despite some attempts to cool patients for other problems such as cardiac arrest, strokes and head injuries, the number of problems encountered in during cooling and in the re-warming phase put a damper on the use of the technique. Now, however, we are in the middle of a revival of interest in therapeutic hypothermia.
How does cold help?
Cold protects the brain because the biochemical reactions that sustain life are influenced by temperature. If the heart stops, the brain runs out of fresh supplies for energy production in two minutes. A downward spiral toward brain cell death begins unless blood flow is restored within the next two minutes. When blood flow is restored (reperfusion), a cascade of potentially damaging chemical reactions begins in cells that have been deprived of oxygen. The longer the period of arrested circulation was, the more damaging these reactions are.
The body at different temperatures
Changing the body’s temperature changes the speed and efficiency of its chemical reactions. At temperatures over 105 many processes fail completely. As body temperature falls below normal, chemical reactions slow down. Between 92 and 89.6 the damaging chemical responses that come after blood flow returns are blunted enough to improve outcomes significantly. By 90, pulse and respirations slow and peripheral circulation shuts down. By 86 the patient may still be alive, but looks dead. This level of deep hypothermia is used for some long, difficult cardiac and neurosurgical procedures.
Lowering temperature is now routine, sometimes
Since 2005, the American Heart Association has recommended therapeutic hypothermia as a routine part of patient care after a cardiac arrest in circumstances that depend on the reason for the cardiac arrest, the speed of the resuscitation, and the state of the patient after circulation is restored. Some medical centers are also experimenting with the technique in the treatment of certain types of strokes and head injuries.
How do you lower someone’s core temperature?
How do you cool a body that normally maintains a constant temperature that hovers within a few tenths of a degree of 98.6? Any environment with a temperature less than body temperature provides a gradient for heat loss. Deliberately making someone hypothermic means increasing that temperature gradient. In the presence of a gradient, heat radiates away from the body. Heat is also conducted away when the body is in contact with any colder substance; when the colder substance such as air or water is in motion, heat is lost even faster, by convection. Heat is also drawn away by evaporation of perspiration on the skin’s surface, where the sweat keeps the microclimate humidity at 70% even when you think you are dry. Heat also dissipates when warm moist air is exhaled from the lungs.
Several internal and external ways of changing the temperature gradient exist: ice packs applied to the head, neck, axillae, groin, where large blood vessels are close to the surface; cooling blankets that house cold water circuits; closed catheters through which cold saline circulates inserted into large blood vessels; ice water balloons in the bladder. These methods are directed at the core temperature of the body – the temperature of the internal organs and the brain. They do not cause problems like frostbite, seen commonly with accidental hypothermia, because the ambient temperature is not freezing and the skin is protected from direct exposure to ice packs being used for cooling.
The body resists lowering the temperature
Normally, we protect ourselves from falling temperatures by putting on more clothes and increasing activity, and by shaking and shivering, which produce heat. A patient who has suffered a cardiac arrest will not engage in the normal behavioral responses, but he will shiver and perhaps become agitated, both of which are counterproductive to getting the temperature down. Sedation and even muscle paralysis are therefore necessary for the period of cooling.
Despite problems, therapeutic hypothermia is here to stay
Current therapeutic hypothermia protocols call for maintenance of temperature between 32-34 (89.6-93.2) for 18-24 hours, followed by passive re-warming over the next 24 hours. Overshooting and undershooting of temperature are both common, as are difficulties maintaining electrolyte and sugar balance. Some complications like pneumonia and bleeding problems are more common than in similar patients not being treated with cold temperatures. Much work remains to determine the best timing for induction and maintenance of hypothermia after cardiac arrest, but it is clear that “the sooner the better” is the general rule and that the revival of interest in therapeutic hypothermia is here to stay.
True preventive medicine is an intervention that stops a disease from developing, not one which simply slows disease progress. The body’s immune system is the master of disease prevention and it is no accident that one of the first medical efforts at preventing disease stemmed from the observation in the late 1700s that suffering a mild infection like cowpox prevented a similar but more severe infection – smallpox. Immunization was born, and to this date is the single most effective form of prevention of lethal disease. In the current age of rejection of routine immunization by a significant number of people, the disease called tetanus and its prevention by immunization is a story worth reviewing.
What causes tetanus?
Tetanus a disease is caused by a type of bacteria called Clostridia tetani, a fragile little organism that can’t tolerate oxygen or high temperatures but which changes itself into a tough intermediate form called a spore to lie in wait for potential victims. C. tetani spores survive indefinitely, are common in soil, particularly manure rich soil, and are found in intestinal tracts of farm animal, cats, guinea pigs, rats and people. They can survive oxygen rich environments, the usual antiseptics and even the temperatures used to sterilize medical instruments. Once the spores gain entry into body tissues, they revert to fragile bacterial form, reproduce and begin to manufacture tetanospasmin, one of the most lethal toxins known to man and the substance responsible for the symptoms of the disease. Though farm animals and people are susceptible to tetanus infection, dogs and cats are not.
Development of symptoms
Tetanus infections are usually acquired when C. tetani spores enter the body through a deep wound in the skin that air does not reach. Contaminated batches of heroin are also sources of infection when the drug is injected under the skin or intravenously. In the first few days after C. tetani spores come to life inside the body, no symptoms or tests indicate anything amiss. As the toxin producing bacteria increase in number, and the toxin produced finds its way to the spaces between nerves and muscle and between motor nerve cells in the brain and spinal cord, profound muscle spasms begin. Tetanospasmin works by the blocking normal neurochemical signals that inhibit muscle tone and motor nerve excitability.
The time from infection to development of symptoms in any infection is known as the incubation period. In human tetanus, the closer the entrance point of the bacteria to the brain or spinal cord the shorter the incubation period. On average symptoms begin about a week after injury. Though localized forms of tetanus can occur, with muscle spasm limited to the area around the wound, most cases are general and symptoms begin in the muscles of the head and neck. Spasm of the powerful masseter muscles of the jaw is the origin of the term “lockjaw,” a commonly used name for tetanus infection. Vocal cord and respiratory muscle involvement can interfere with breathing. Abdominal, trunk and skeletal muscle involvement are extremely painful and spasms can be strong enough to fracture long bones and spinal vertebrae. Other complications arise from involvement of the central nervous system: fever, high blood pressure, heart rhythm abnormalities and seizures. Secondary complications like bladder infections, pneumonia and blood clots in the legs and lungs also contribute to the lethality of the disease. In the pre-immunization era, treatment was confined to supporting the patient through the four weeks it takes for the toxin’s effects to wane.
Making the immune system remember the disease
Unlike cow pox, in which the natural immune response directed against the cowpox virus prevents more cowpox episodes but also smallpox, a full-blown case of tetanus does not confer any immunity because the minute amounts of toxin that produce the symptoms are not sufficient to stimulate the immune system to make antibodies against it. Immunization to tetanus is accomplished by presenting the immune system with a much larger amount of a formaldehyde weakened version of the toxin, to which it will produce antibodies which will neutralize the real toxin should it ever appear. This process takes a few weeks and several doses are required over time to reach full potency of an antibody response.
Immunization programs have made tetanus rare enough for people to forget how terrible an illness it is. In the US, since routine, active immunization began in the 1940s, tetanus rates declined steadily and were at an all-time low of .01 cases per 100,000 people in 2009. In addition, with better supportive care, mortality rates declined from 30% in the mid-1900s to 10% in the first decade of the 21st C.
Borrowing someone else’s immunity
Nevertheless, tetanus infections still occur and may increase in frequency if immunization rates drop. Fortunately, another type of immunization helps when tetanus develops in people who have not been immunized – a passive immunization process that allows patients to borrow antibodies produced in the blood of other people who have been immunized against C.tetani. This “antitoxin” is a mixture of human gamma globulin from screened donors and antibodies in it that “recognize” tetanus toxin react with the toxin circulating in the tetanus victim’s body, neutralizing a lot of its potency.
Boosting weakened immunity
The antibody response to tetanus toxoid wanes over time, but a repeat injection brings it up to full speed quickly. Booster doses are recommended for all adults every 10 years and in the event of penetrating wounds, especially if immunization status is unknown. Awareness of the symptoms of tetanus and the status of immunization of anyone someone suffering from heroin addiction, a sad and growing problem, is crucial for anyone who cares for them. Tetanus is the poster child for preventive medicine and no one should have to suffer this disease. The earlier it is recognized, the better the outcome is likely to be.
As Supreme Court Justice Potter Stewart famously said, when confronted with a decision about what constituted pornography, the definition is hard, but “I know what it is when I see it.” An all-encompassing definition of fatigue is similarly difficult, but everyone knows what fatigue feels like. The profound lassitude that signals an oncoming flu is a gluey, mesmerizing state of mind and body that renders one incapable of remembering ever feeling good, of imagining ever feeling energetic again, or of conceiving of a desire to participate in any physical, social or mental activity beyond crawling beneath the bedcovers.
The perception of energy failure
Where there is life, there is fatigue. All plants and animals run on energy produced in little chemical factories (mitochondria) in every cell. The ultimate source of biologic energy is the sun’s nuclear energy, converted to usable form by plants and transferred to animals as food. The more complex the living thing, the more obvious the need for periods of rest and recovery to replenish energy. When the demand energy use outpaces the time needed for recovery, or when normal function is derailed by illness, drugs or toxins, fatigue is the name we give to what we feel, mentally and physically. To the research scientist, fatigue is a by-product of numerous little proteins (cytokines) produced by the immune system to protect us from outside invaders and internal disorders like cancer. How these proteins create the feeling of fatigue is a mystery, but there is admirable logic in a system that commandeers a patient’s energy, drive and ambition and sends him packing off to bed while an internal battle rages.
Less admirable is our ability to override the biology that produces tiredness, and to become passive, cranky and sleep-deprived. In fact, most complaints of fatigue reflect the deliberate choice to ignore the symptom and would and yield to simple lifestyle changes – if one were willing and able to sleep more, lose weight, eat regular, well-balanced meals, exercise enough, manage time wisely, avoid smoking, excess alcohol, and junk food, and engage in satisfying work. In our culture these are tall orders, and a background level of fatigue is often accepted as normal.
Evaluation of fatigue
New, unexpected and persistent tiredness, however, may signal underlying illness or environmental stress and warrants a serious evaluation, with clear communication about exactly what fatigue means to the patient. First, a description of the patient’s normal “background energy” is important. Some people are full of energy from the day they are born. Others are inveterate couch potatoes, happy to sit and watch life go by. The feeling of fatigue that prompts one to see a doctor is, by definition, different from the patient’s normal state, but the doctor sees only a snapshot in time. Patients and families should never be shy about volunteering information about what life used to be like.
Defining the symptom
Next, the language used by patients to describe fatigue needs to be clear. “I’m tired” sometimes means “I’m weak,” and “I’m weak” sometimes means “I’m tired,” but in the jargon of medicine, weakness means loss of muscle strength. Provided that they exert full effort, tired people can generate normal muscle power upon request, but people with strokes or nerve and muscle diseases cannot. Separating weakness from fatigue is the doctor’s first job – otherwise he may head off on the wrong diagnostic road. Description of the activities affected by tiredness and/or weakness, and characterization of changes fatigue brings to daily life are crucial to the process of diagnosis.
Finding the source
Once a doctor understands the way fatigue affects life for a patient, he moves on to a “review of systems” – a top to bottom list of questions ranging over all the body’s organs, looking for clues to the presence of heart, kidney or liver disease, diabetes, cancer, sleep apnea, restless leg syndrome, insomnia, degenerative neurologic diseases like Parkinson’s, autoimmune illnesses like lupus or MS, chronic infections, eating disorders and problems of the thyroid, adrenal and pituitary glands. A good doctor will then delve into the lifestyle and life events surrounding the appearance of fatigue. Tiredness is a complex, high level symptom that may also originate in the mind – it is one of the cardinal symptoms of depression.
Is it the drugs
Next comes a careful inventory of all medicines in use, prescription and non-prescription. New fatigue symptoms may parallel the addition of new drugs (even antibiotics can cause fatigue). An inventory of potential toxins and hazards in the environment may turn up a faulty furnace producing carbon monoxide or exposure to toxins such as volatile hydrocarbons that can damage the part of the brain called the cerebellum – a major player in energy balance.
Following the clues
Following a good, inquisitive medical history, a complete physical exam (the kind that requires undressing) may turn up other clues that suggest the need for more than “routine” tests. Fatigue is messenger bringing information about conditions ranging from minor to mortal. When not readily explained, fatigue warrants the best of our medical tools to ferret out the source of trouble. The first step though, is still a careful history and physical examination. Without these, advanced medical technological evaluation of fatigue is little better than a fishing expedition sent to sea with no information about where the fish hang out.
The Chronic Fatigue Syndrome
Profound, life-altering fatigue lasting more than 6 months.
May follow a viral infection, but no test abnormalities persist along with the fatigue.
Physical and mental activities both worsen symptoms.
Variety of accompanying symptoms: weakness, muscle and skeletal aches and pains, impaired memory, lack of drive, poor sleep.
No specific tests, other than exclusion of other illnesses that produce these symptoms, among others. CFS is a “diagnosis of exclusion.”
Conditions to be excluded:
Chronic infections, mononucleosis, autoimmune disorders (lupus, M.S.), hypothyroidism, low adrenal function, sleep apnea, cancer (particularly pancreatic), obesity, eating disorders, drug and alcohol abuse, major psychiatric disturbances: schizophrenia, depression.