Our friends’ close encounter with this elusive, aggressive disease awoke us to the difficulty of diagnosis, the danger of death or lifelong damage, and the need for wider awareness
by Randy Hartnell with Craig Weatherby
It’s been said, “Knowledge is power”, and a nightmarish experience that recently befell some friends served to support the veracity of this old saw. To protect their privacy, we’ll call our friends “Bill and Debra,” and we’ll refer to their daughter as “Anna.”
Bill and Debra live near us, about two hours north of Seattle, and Anna is a bright, lively 20-year-old who’s been attending college in Manhattan. Had those around Anna, including her physician, possessed the knowledge we are about to share with you, they might have spared her and her parents a traumatic brush with tragedy.
Bill and Debra asked us to share their story with you, in the hope that it might save another child from months of rehabilitation—or worse—and spare other parents agonizing hours of heartbreak and anguish. We hope you’ll agree that it is vital information worthy of our forum.
Meningococcal meningitis—the bacterial disease that nearly killed Anna—is a menacing one because, although death or permanent injury can occur within hours of onset, its symptoms mimic those of the common flu: fever, headache, sore throat, achy joints, nausea, and diarrhea. Consequently, appropriate treatment is often delayed.
When Anna called her parents at their home in Washington State the morning of June 14th to tell them she had a fever of 104 degrees, Debra and Bill ordered her to get to the doctor. She said that she was too weak and tired to go but they insisted, so Anna pulled herself together and made her way into a clinic, where she arrived weak, ashen, and dehydrated.
By this time, her fever had dropped to 101, but she remained there for four hours. A blood test was taken (but not reviewed) and she was administered two units of saline solution. She was then sent home to recover from her apparent flu bug. She was so weak that clinic personnel had to help her into the cab.
Concerned that her temperature might rise again, Debra called every two hours throughout the night to insist that she check it. While the fever hadn’t spiked, in the morning Anna was suffering nausea, diarrhea, a terrible headache and had begun to notice small painful bruises appearing on her arms and legs.
Over the phone, Bill and Debra told her she had to get to the hospital. Anna said she was too weak to get out of bed, let alone leave her apartment, so her increasingly alarmed parents contacted a friend in the area, who summoned an ambulance.
They decided that Debra would take the next flight out from Seattle to be with her. But while Bill was rushing her to the airport, they received a phone call from the emergency room physician in New York where Anna had just been admitted. She said the words that every parent dreads, “Mr. Robbins, your daughter is in critical condition… you and your wife need to get to the hospital as soon as possible.”
Still not able to grasp the seriousness of the situation, Bill and Debra advised the doctor that they were 3,000 miles away and that her mother was on the way. Bill asked “Do I need to come too?” The urgency in the doctor’s sobering reply cleared up any lingering confusion: “Sir, we believe your daughter has bacterial meningitis. It’s a very serious disease. She is in critical condition. You and your wife need to come to the hospital as soon as possible.”
A few hours later, Bill and Debra streaked across the country on separate aircraft, stricken by the horrifying possibility suddenly facing them. The doctor’s words played over repeatedly in their minds, triggering waves of disbelief, “this can’t be happening…this can’t be happening…” But it was.
It is now nearly six weeks later and Anna remains hospitalized. She spent eight days in a coma before waking up, and didn’t speak for a month. The infection is long since defeated but the damage left in its wake will likely require months of rehabilitation. Her goal of graduating from college will have to wait.
Why have we chosen to share this story with you? Because among the many things we’ve learned since this ordeal began is that meningitis happens, often to children, and a large number of them do not survive.
And while the most common strains—-including the meningococcal bacteria that nearly took Anna's life—are extremely vulnerable to the most basic antibiotics, many young victims die, because, by the time this stealthy microbe is identified, the damage is done. Help comes too late.
In other words, many victims of meningitis die because neither they nor their physicians were aware of the recently identified early symptoms that can prompt people to seek treatment much sooner.
As we report below, the results of a new study from Britain document, for the first time, the existence of life-saving early signs of meningitis.
We hope you’ll take a few minutes to review this information. It may provide you with the knowledge to save a life. And if you’re one of our many readers in the healthcare field, the new findings we’re reporting here could ultimately save many lives, by alerting your own staff and by passing the information along to parents and others in your field.
What is meningitis?
The term “meningitis” means an inflammation of the meninges: the membranes that cover the brain and spinal cord. Meningitis is usually caused by a viral or bacterial infection.
Bacterial meningitis is much less common than viral meningitis, but far more dangerous. And it moves so fast that its victims often die or suffer serious injury within 24 hours of beginning to feeling ill.
Before the 1990s, Haemophilus influenzae type b (Hib) was the leading cause of meningitis in children in the United States. But the widespread use of the Hib vaccine as a routine childhood immunization has dramatically decreased the frequency of meningitis caused by Hib.
Many different types of bacteria can cause meningitis: Group B Streptococcus, E. coli, and Listeria monocytogenes are the most common causes of meningitis in newborns, while Streptococcus pneumoniae (pneumococcus) and Neisseria meningitidis (meningococcus) are more frequent in children older than two months of age.
Enemy number one: Meningococcal meningitis
Among the kinds of bacteria that can cause meningitis, Neisseria meningitidis (meningococcus) stands out for its toxicity and lethality.
The five main serogroups (or types) of meningococcus bacteria in the U.S. are A, B, C, Y and W-135, which cause more than 95 percent of meningococcal disease worldwide. Researchers are unsure why the bacteria attacks some people while most of the population are not affected.
According to the National Meningitis Association (NMA), almost one-third of the 2,000 to 3,000 cases of meningococcal meningitis reported in the United States annually result in fatalities or severe disabilities, such as amputations and organ damage.
And surveys indicate there’s been a recent increase in the number of adolescent cases and deaths in the 1990's. Adolescents and young adults now account for nearly 30 percent of all cases in the U.S.
But one sobering set of statistics should make all parents and teens sit up and take notice: one in four adolescents infected with meningococcal meningitis die, and up to 20 percent of the survivors experience permanent disability.
Every year, about 18,000 people in the U.S.—mostly young children and teenagers or young adults—get some form of bacterial meningitis. Many suffer some form of lasting injury to their brains or nervous systems, and about 400 die. Those who survive often suffer brain damage or severe scarring. In extreme cases, limbs have to be amputated.
According to the Centers for Disease Control and Prevention, young adults living in close quarters—such as in college dorms and military barracks—are at higher risk than most, because they breathe the same air and tend to share food, drink, lipstick and the like.
While the injuries caused by the most common bacterial cause of meningitis—meningococcus—typically occur in the brain and nervous system, this germ can also cause a system-wide “toxic-shock” syndrome called meningococcal septicemia, which causes blood to rush to vital organs. The resulting lack of blood in the rest of the body makes patients very pale, while their hands and feet become very cold.
The rapid advancement of meningococcal meningitis and septicemia can be accompanied by an “auto-amputating” necrosis that may manifest as the small, painful “bruises” that Anna experienced. Unless it’s caught early, doctors may have to amputate any affected extremities.
Recognizing an aggressive crippler and killer
Needless to say, early diagnosis of bacterial meningitis—either by recognition of its clinical signs or by analysis of spinal fluid—is critical to a patient’s survival and prospects for full recovery.
The first symptoms of meningitis may surface several days after a child, teenager, or immune-compromised adult—has had a cold and runny nose, diarrhea and vomiting, or other signs of a bacterial or viral infection.
Newborns and infants with meningitis may lack the classical signs described below or simply be extremely irritable or lethargic. Normally, infants who are not feeling well will be comforted when their mothers pick them up. However, a baby who has meningitis may become more distressed when picked up and rocked, because this further irritates their inflamed meninges.
“Classic” (late) Symptom Triad
For many decades, doctors suspected meningitis if the patient displayed a cluster of three classic symptoms called the “triad”:
- Stiff neck
- Mental confusion, lethargy and/or headache
Secondary “Classic” (late) Symptoms
- Eye-sensitivity to light
- Pinprick (hemorrhagic) rash that does not fade under pressure
- Rash featuring purple spots
- Nausea and vomiting
- Pale or off-color skin
- Sore joints
- Jaundice (yellowish skin),
- Lower-than-normal temperature
- Poor feeding, a weak suck, or high-pitched crying (infants)
- Bulging fontanelles on a baby's head (Fontanelles are the soft spot at the top/front of the baby's skull, where the bones of the skull join and are still open)
While patients can suffer severe injury or death within 24 hours of the first hints of illness, the “classic” symptoms listed above—including the “meningitis triad” of fever, stiff neck, and lethargy/confusion—typically appear only after 13 to 22 hours have elapsed since the first signs of anything being amiss.
This time delay between the first hints of illness and the appearance of the “classic” symptoms can make the difference between full recovery and the worst-case scenarios: permanent injury or death.
The guidance on the CDC’s meningitis Web page fails to mention that many adults with bacterial meningitis do not display the classic symptoms. Doctors at the Banner Good Samaritan Medical Center in Phoenix, Arizona examined the records of 38 cases of bacterial meningitis in adults (Pizon AF 2006), with these results:
- The classic meningitis “triad” (fever, neck stiffness, mental status change or headache) was present in only eight out of 38 cases
- Seven of the 38 patients died
And Swiss researchers issued this sober assessment in 2006: “The mortality of bacterial meningitis can reach 30%, and up to 50% of survivors suffer from persisting neurological deficits [damage] as a consequence of the disease. The incidence of neurological sequelae [consequences] of bacterial meningitis has not improved over the last decade.”
But these grim outcomes relate to the lack of early warning signs that would permit administration of antibiotics soon enough to prevent death or serious damage.
Once a patient arrives at a hospital, doctors can confirm a clinical (symptom-based) diagnosis of bacterial meningitis by performing a lumbar puncture to test spinal fluid for signs of infection. Physicians may also perform a chest x-ray to look for other sites of infection, and a head CT scan to look for hydrocephalus (fluid on the brain), an abscess, or deep swelling.
But to prevent serious consequences, doctors may need to administer antibiotics before the results of these technical tests come back. And to ensure that antibiotic treatment is given soon enough to prevent brain damage or worse, patients must get to a hospital before the “classic” symptoms appear.
Fortunately, British researchers working with the records of pediatric meningitis patients recently published their discovery of meningitis-specific symptoms that occur within eight hours of any signs of illness: much sooner than the “classic” symptoms manifest.
Landmark study identifies early signs of meningitis
Thanks to a team of British researchers, there’s new hope for improving the discouraging statistics associated with bacterial meningitis. We hope that you will share their findings with family and friends, since anyone—especially children and teens—can fall victim to this uncommon, but uncommonly dangerous, disease.
The results of the groundbreaking study—published in the famed medical journal Lancet in January of 2006—demonstrate that the “classic” symptoms of bacterial meningitis are, in fact, the symptoms of advanced meningitis (Thompson MJ 2006).
Their research was prompted by the astonishing fact that, at the time they initiated their study, no one had researched the occurrence and nature of symptoms before meningitis patients were admitted to a hospital.
As the authors said in the introduction to their report, “…many children who are admitted to hospital with meningococcal disease had been initially misdiagnosed by a doctor before admission. Since infection can progress from initial symptoms to death within hours, individuals must be diagnosed as early as possible.
“…many children will be first examined by a clinician who has never before seen a case outside hospital. Identification of the disease will therefore depend on clinicians' experience in hospital and on textbook descriptions of classic features such as haemorrhagic rash, meningism, and impaired consciousness that occur late in the illness.”
The British team—led by Dr. Matthew J. Thomson of Oxford University—analyzed two sources of data regarding 448 children aged 16 years or younger who had been hospitalized with bacterial meningitis:
- Primary-care physicians’ records of the course of illness before admission to hospital
- Questionnaires answered by parents
Their findings showed that most children had only non-specific symptoms—signs that could be attributed to the flu or other relatively benign infections—in the first 4 to 6 hours, but were close to death within 24 hours of any sign of illness.
And the classic symptoms—pinprick rash, stiff neck, headache, and impaired consciousness—did not appear until 13 to 22 hours had passed since the first signs of anything being amiss.
Their key discovery was that on average (point underlined for emphasis), 72 percent of the children had leg pains, cold hands and feet, and abnormal skin color within 8 hours of any sign of illness. This is eleven hours earlier than the average of 19 hours that elapsed before the children in the study had been admitted to a hospital.
As Dr. Matthews’ team reported, “Classic clinical features of meningococcal disease appear late in the illness. Recognizing early symptoms of sepsis [infection] could increase the proportion of children identified by primary-care clinicians and shorten the time to hospital admission.”
We owe these researchers a debt of gratitude for looking more deeply into a deadly disease whose relative rarity has left its diagnostic signs poorly defined.
Limiting the neurological damage
Meningitis often leaves its victims with some degree of neurological damage, all or some of which may be reversible with rehabilitative therapy.
However, there are ways to limit the damage caused by bacterial meningitis. The results of a large-scale study published in the New England Journal of Medicine (de Gans J 2002) showed that a synthetic corticosteroid drug called dexamethasone can reduce brain and nerve damage in adults with bacterial meningitis, when it is administered immediately, either before or with antibiotics.
And this year, researchers from Switzerland reported that dexamethasone can reduce brain and nerve damage in children with the pneumococcal form of bacterial meningitis. They also reported that in rodents with bacterial meningitis, the natural bodily antioxidant melatonin can reduces brain and nerve damage: a finding that should be tested in humans (Grandgirard D 2006).
Who is susceptible?
Anyone of any age can develop bacterial meningitis, but the risk of contracting meningococcal disease is highest in children under the age of five, while there is a second, smaller rise in the rate of infections among those 15 to 24 years of age. Most cases occur between December and April.
A person is susceptible to meningococcal disease if he or she has no immunity against the strain infecting the back of the nose and throat. By 20 years of age, more than four out of five people have developed immunity to the meningococci bacteria—Neisseria meningitidis—and are no longer at risk of becoming ill after becoming a carrier.
Only one or two out of every 100,000 carriers develop meningitis or meningococcal septicemia every year, but those few are in great danger unless they recognize the early warning signs and get treatment within a very few hours.
The people at greatest risk for all types of meningitis—for whom routine vaccination is recommended—include those with weakened immune systems, the very young, and people who spend time among large groups of people routinely (e.g., day-care centers, dormitories, and military bases).
Military recruits are routinely vaccinated against meningococcal meningitis, and The American Academy of Pediatrics encourages college students—particularly freshmen living in dormitories—to get the meningococcal vaccine.
In May of 2005, the U.S. Centers for Disease Control and Prevention officially recommended routine immunization at the preadolescent doctor’s visit (11-12 years old), and routine vaccination of preadolescents, teenagers, college-bound students, and college freshmen living in dormitories. CDC also recommends that adolescents who were not previously immunized receive the vaccine at high school entry (at about age 15 years).
Vaccination is often recommended for all children over two years of age—especially kids in day care and college dorms—and all persons over 65 years of age (for details, see “What are the vaccines?” below).
In addition, vaccination is recommended for the following groups:
- Microbiologists who work with meningococcal bacteria
- Anyone who has a damaged spleen or removed spleen
- Anyone who has an immune system disorder called terminal complement component deficiency
- Anyone who is traveling to countries experiencing an outbreak of meningococcal disease, and people who may have been exposed to meningitis during an outbreak (Information on areas for which meningococcal vaccine is recommended can be obtained by calling the Centers for Disease Control and Prevention at 404-332-4565)
How does bacterial meningitis spread?
Meningococcal bacteria are common, and they live in the back of the nose and throat. Some 15 percent of adolescents and adults carry them without suffering any harm. Once a person becomes a carrier, the “silent” infection may last for up to six months.
Fortunately, meningococcal bacteria do not survive long outside of the body and are not very contagious. Infection is spread by direct contact with secretions from the nose and throat, so it requires close, direct physical contact, combined with activities as kissing, coughing, and sneezing. It can also spread through saliva (spit) when sharing items such as cigarettes, lipstick, and food or drinks.
The most common source of infection is the respiratory tract, but an infection can start in the skin, gastrointestinal tract, or urinary system. From there the microorganisms can enter the bloodstream, travel through the body, and enter the central nervous system. In some cases of bacterial meningitis, the germs spread directly to the meninges from a serious ear or nasal sinus infection. The bacteria can even invade the central nervous system after a severe head trauma or head surgery.
A person who is carrying meningitis can spread the disease for up to seven days before becoming ill, and can spread the disease for up to one day after treatment with antibiotics begins.
What are the vaccines?
There are vaccines against Hib, some types of N. meningitides (meningococcal meningitis), and many types of S. pneumoniae (pneumococcal meningitis).
Vaccines for S. pneumoniae (pneumococcal meningitis)
The pneumococcal polysaccharide vaccine is recommended for all persons over 65 years of age and younger persons at least 2 years old with certain chronic medical problems. There is a newly licensed vaccine (pneumococcal conjugate vaccine) that appears to be effective in infants for the prevention of pneumococcal infections and is routinely recommended for all children over two years of age.
Vaccines for N. meningitides (meningococcal meningitis)
Both of the two vaccines against Neisseria meningitidis—which causes meningococcal-type meningitis—can prevent four types of meningococcal disease, including two of the three types most common in the U.S.:
- Meningococcal polysaccharide vaccine (MPSV4 or Menomune®) should be used for children 2 to 10 years of age and for adults over 55 who are at special risk.
- Meningococcal conjugate vaccine (MCV4 or MenactraT) is recommended for all children at 11 to 12 years of age. For those who have never gotten MCV4 previously, a dose is recommended at high school entry. Other adolescents who want to decrease their risk of meningococcal disease can also get the vaccine.
Unfortunately, there is a current shortage of Menactra, and many doctors are limiting it to susceptible adults and to teens entering college. Even these groups are being turned away, or are being charged exorbitant prices.
We urge you to send this article along to friends and family, especially to households with children who will be entering middle school, high school or college. Wider awareness of this deadly, speedy threat could save lives, limbs, and a great deal of heartache. (Just click the “send me to a friend” links placed at the top and bottom of this newsletter.)
Information and resources
- Meningitis Foundation of America at http://www.musa.org/
- National Meningitis Association (NMA) at http://www.nmaus.org/
- Meningitis Research Foundation at http://www.meningitis.org/
- U.S. Centers for Disease Control and Prevention at http://www.cdc.gov/ncidod/dbmd/diseaseinfo/meningococcal_g.htm
- Meningococcal Disease. U.S. Centers for Disease Control and Prevention. Revised October 12, 2005. Accessed online July 20, 2006 at http://www.cdc.gov/ncidod/dbmd/diseaseinfo/meningococcal_g.htm.
- Thompson MJ, Ninis N, Perera R, Mayon-White R, Phillips C, Bailey L, Harnden A, Mant D, Levin M. Clinical recognition of meningococcal disease in children and adolescents. Lancet. 2006 Feb 4;367(9508):397-403.
- Meningitis Foundation of America. Accessed online July 20, 2006 at http://www.musa.org/
- National Meningitis Association. Accessed online July 20, 2006 at http://www.nmaus.org/
- Meningitis Research Foundation. Accessed online July 20, 2006 at http://www.meningitis.org/
- Pizon AF, Bonner MR, Wang HE, Kaplan RM. Ten years of clinical experience with adult meningitis at an urban academic medical center. J Emerg Med. 2006 May;30(4):367-70.
- Weisfelt M, de Gans J, van der Poll T, van de Beek D. Pneumococcal meningitis in adults: new approaches to management and prevention. Lancet Neurol. 2006 Apr;5(4):332-42. Review.
- Ostergaard C, Hoiby N, Konradsen HB, Samuelsson S. Prehospital diagnostic and therapeutic management of otogenic Streptococcus pneumoniae meningitis. Scand J Infect Dis. 2006;38(3):172-80.
- Grandgirard D, Leib SL. Strategies to prevent neuronal damage in paediatric bacterial meningitis. Curr Opin Pediatr. 2006 Apr;18(2):112-8.
- van de Beek D, de Gans J. Dexamethasone in adults with community-acquired bacterial meningitis. Drugs. 2006;66(4):415-27.
- de Gans J, van de Beek D; European Dexamethasone in Adulthood Bacterial Meningitis Study Investigators. Dexamethasone in adults with bacterial meningitis. N Engl J Med. 2002 Nov 14;347(20):1549-56.
- Nadel S, Britto J, Booy R, Maconochie I, Habibi P, Levin M. Avoidable deficiencies in the delivery of health care to children with meningococcal disease. J Accid Emerg Med. 1998 Sep;15(5):298-303.
- Granier S, Owen P, Pill R, Jacobson L. Recognising meningococcal disease in primary care: qualitative study of how general practitioners process clinical and contextual information. BMJ. 1998 Jan 24;316(7127):276-9.