Friday, July 26, 2019

PAM is a very bad thing. Primary amoebic meningoencephalitis.

A swimmer has died after contracting brain-eating amoeba at a water park in North Carolina, according to reports.

The swimmer was exposed to Naegleria fowleri, an amoeba commonly found in warm freshwater, at Fantasy Lake Water Park in Cumberland County on July 12, Fox 46 Charlotte reported.
The news outlet added that were just 145 known cases of infected Americans from 1962 through 2018.

North Carolina had five cases during that time period. 

"Our sympathies are with the family and loved ones," Dr. Zack Moore, the state’s epidemiologist, told Fox 46. "People should be aware that this organism is present in warm freshwater lakes, rivers and hot springs across North Carolina, so be mindful as you swim or enjoy water sports."

PAM is a very bad thing. Not PAM the cooking spray or Pam from The Office but PAM as in primary amoebic meningoencephalitis.

This condition can occur when Naegleria fowleri, a type of amoeba, goes up your nose, makes it to your brain, and starts eating your brain. Getting your brain eaten by an amoeba is rare but bad, since your brain is pretty important and helps you text and do other stuff like live. In fact, nearly everyone who gets such PAM ends up dying.

The most recent reported case and death just occurred in North Carolina when a person became infected with the brain-eating amoeba after swimming in the Fantasy Lake Water Park, as reported by Stephanie Towers reported for WYFF News 4.  Naegleria fowleri tend to live in warmer fresh water, such as lakes, rivers, hot springs, poorly chlorinated swimming pools, water in water heaters, or water from industrial plants.

You can't get infected by Naegleria fowleri from simply drinking contaminated water. In this case, the best way to your brain is not through a book or a TED Talk but up your nose. The amoeba has to go up your nose to get to your brain. And since N. fowleri don't ride e-scooters, water containing the amoeba has to be forced up your nose. This can occur when you dive into or dunk your head into contaminated water. It can also happen when you irrigate your nose with non-sterile water for religious or other reasons like using a neti pot. As I have written before for Forbes, tap water and french fries are two things that shouldn't go up your nose. N. fowleri can also be found in soil. However, unless you are an aardvark or literally a brown nose, you probably don't tend to shove soil up your nose.  

After getting infected, you may start to notice symptoms in one to nine days (an average of five days) such as a fever, a headache, nausea, or vomiting. This can seem like the flu, but soon other symptoms related to inflammation of the brain and the membranes around the brain occur, such as getting stiff neck, becoming confused, having hallucinations, losing your sense of balance, suffering seizures, and lapsing into a coma. The disease progresses pretty quickly and many times death results within 5 days. All of this, of course, sounds terrible unless you are a brain-eating amoeba.

Fortunately, N. fowleri infections of the brain are very rare with only 145 documented cases in the U.S. from 1962 to 2018, according to the Centers for Disease Control and Prevention (CDC). Unfortunately, though, only 4 of these cases managed to survive. As seen in this ABC News segment, Kali Hardig, 12-years-old at the time, became the third person in the U.S. to survive in 2013:


In early July of 2015, a group of friends and family gathered at a home in Orlando, Florida, to celebrate 11-year-old Jordan Cole Smelski, a fun-loving thrill seeker who excelled at sports, academics and making people laugh.

“We decided to have a party, I think we had 75 people over to the house,” Jordan’s father, Steve Smelski, told AccuWeather. “We cooked Jordan’s favorite, which was a special type of hamburger that he liked, and we had burgers for everybody.” It’s the kind of gathering you’d typically expect for special occasions, like birthdays.

Except this occasion was quite different.

Steve and his wife, Shelly, spent time with family and friends until around 6 p.m. that evening. “Then, it kind of became overwhelming, and we snuck off to our room,” Smelski said. “It’s a very emotional day.”

It was not Jordan’s birthday that those who knew him were recognizing. He would have turned 13 later that year on Nov. 9, 2015.

Instead, loved ones had gathered that July in remembrance of a young life cut short just a year prior. On July 2, 2014, Jordan lost his battle with primary amebic meningoencephalitis (PAM), an illness he contracted during a swim in a freshwater pool in Costa Rica, where he was infected with Naegleria fowleri, commonly known as the brain-eating amoeba.

Now, five years after Jordan’s tragic death, the unexpected loss remains difficult to cope with for his parents, who were devastated by the loss of their only child. The inseparable trio had been known as the “three amigos” during Jordan’s lifetime. 

“We went from having a child who was 11-and-a-half, graduating elementary school and getting ready to go to middle school, and now, we’re empty nesters,” Smelski said. “We’ve missed out on the high school years, college years, graduation, seeing him have a girlfriend or learning to drive.”

How the nightmare began

The life-altering moment for the Smelski family started out in the most unexpected of ways — following an enjoyable, leisurely six-hour swim in their Costa Rican resort’s pool in 2014. The family was wrapping up their nine-day vacation in late June. They normally planned fun getaways at least once each summer, and had previously visited Costa Rica and the same resort in 2011. Before the swim that rocked their worlds forever, the 2014 adventure started out as a “good vacation,” Smelski recalled. “We went horseback riding a couple of times. We did ziplining. Jordan loved to zipline. The scarier, longer and faster, the better for him.”

Near the end of the trip, Smelski and his son went for a dip in a pool with a water slide. It was filled with non-chlorinated hot spring water, and repeatedly going down the water slide had forced water up their noses each time they splashed into the pool.

Unbeknownst to them at the time, lurking within the freshwater was Naegleria fowleri. While Jordan’s dad had experienced no ill health effects following the swim, Jordan was not as fortunate.

His symptoms began with a headache the following day. His parents treated him with Motrin. Over next few days as they wrapped up their trip and traveled back home to Orlando, the throbbing headache persisted.

“We got up Saturday morning and he still had a headache, but he was playing video games all morning,” Smelski said. “We had him sit down, and by afternoon, he was lying down and wasn’t playing video games, so he definitely wasn’t feeling well.”

By 11:30 that evening, Jordan went to bed and began vomiting. It lasted through the night. His parents’ worries mounted as the fourth day approached and Jordan's headache remained constant. They wondered if he’d caught something while they were out of the country. They took him to the hospital on Sunday, June 29, 2014.

Doctors gave Jordan medication to treat the vomiting and headache, and decided that a lumbar puncture was needed to test his cerebral spinal fluid. Jordan had added a stiff neck to his growing list of ailments, and doctors assumed he might have some type of meningitis. Following a CT scan, it was revealed that Jordan was suffering from viral meningitis -- or so it appeared.

“The problem with PAM is it masks itself as something else,” Smelski said. Having known virtually nothing about the disease and Naegleria fowleri before his son’s death, he’s become an expert on the topic in the years since. “It can look like viral meningitis early in the infection. A day or two later, it can look like bacterial meningitis or encephalitis. There’s no initial test for it; you have to know what you’re looking for, and it’s very hard to find.”

Eleven-year-old Jordan Smelski ziplines through trees on a family vacation in Costa Rica in June 2014 — just days before falling ill with primary amebic meningoencephalitis (PAM). (Photo/Steve Smelski) 

2 hours too late

Jordan was then admitted to a children's hospital, and while Sunday night was rough for him, his condition seemed to improve a little as Monday began. However, around 8:45 p.m., Jordan’s headache began to explode once again. “[It was] like a nine on a scale of one to 10,” his father recalled.

Nurses gave the ailing child morphine, hoping to lessen his pain. Not long after the morphine entered his system, Jordan’s pupils began dilating and he started hallucinating. “He wasn’t making much sense,” Smelski said. “That went on for about four-and-a-half hours.”

It got to the point where Jordan’s parents would lie down on top of him to prevent his uncontrollable movements from popping out his IVs. “He had this look in his eye like he didn’t know where he was,” Smelski remembered. Then, Jordan had a seizure.

He was eventually moved to the intensive care unit, and after doctors noticed his sodium levels were low, they put Jordan on saline. He seemed better, more stable, Smelski told AccuWeather.

“Doctors came the next morning, which was Tuesday, a week after we swam,” he said. “They told us, ‘It’s going to be two or three weeks, but he’s going to be fine, you’ll be able to take him home.”

But Jordan would never return home. He’d never play soccer or video games, or ride roller coasters ever again.

By Tuesday night, the swelling inside of Jordan’s skull had become so bad, doctors worried that it would push down on his brain stem, which would kill him.

With his parents’ permission, the neurosurgeon drilled into the top of Jordan’s head in an effort to save his life. “That’s the cerebral spinal fluid sample they got out that showed the amoeba,” Smelski said. His parents initially thought finding the source of the problem was a good thing — until they learned that the fatality rate after infection with the amoeba is over 97 percent. Of 145 known infected individuals in the United States between 1962 and 2018, only four have survived, according to the Centers for Disease Control and Prevention (CDC). The amoeba normally kills its host with 12-14 days after symptoms surface.

The drug that could have saved Jordan, miltefosine, was on its way from the CDC in Atlanta. The plane carrying the drug landed in Orlando two hours after Jordan died. 

The ‘rare’ amoeba

Jordan had contracted the Naegleria fowleri amoeba in the only known way that humans can be infected: through contaminated water entering the nose. The amoeba travels up the nasal passage and to the brain, where it destroys tissue. “Exposure usually happens during recreational activities in freshwater, where people have their head underwater and water may be forced up the nose, mainly when they’re jumping in or they’re doing flips in the water,” Trisha Robinson, supervisor of the waterborne diseases unit of the Minnesota Department of Health, told AccuWeather.

Minnesota saw two fatal cases of amoeba infection in 2010 and 2012, both associated with exposure at the same lake. “The water temperature [in those cases] was 73 degrees Fahrenheit,” Smelski said. “If it can happen in Minnesota, it can happen in any state in the U.S., so when people say, ‘it’s just the warmest months,’ that’s not the case.”

Naegleria fowleri can be found in any body of freshwater. Drinking contaminated water, however, will not sicken a person.

The CDC states that the heat-loving organism grows best at higher temperatures up to and sometimes exceeding 115 F. “It can survive all the way down to 40 degrees, which is a lot lower than anybody thought it could survive,” Smelski said.

Florida and Texas have seen the most cases, although the amoeba can be found in freshwater anywhere in the U.S. It can’t survive in sea water.

“The fact that Jordan swam once [and became infected] lets you know that you play Russian Roulette every time you allow your kids in that water, because it’s in every fresh body of water,” Smelski said.

Raising life-saving awareness

Right now, PAM, the illness caused by Naegleria fowleri, is reported by health departments in only three states — Florida, Louisiana and Texas — but is tracked nationally by the CDC on an informal basis. “With 47 states not having to report it, nobody looks for it,” Smelski said.

Through the Jordan Smelski Foundation that was started months after Jordan’s passing, his parents and their board of directors made up of family friends and those who knew Jordan began working to raise global awareness of PAM, especially for medical professionals.

“We now know that by using miltefosine and other drugs, lowering body temperature and inducing coma, the patient can actually come out of it in pretty good shape, whereas before, it was almost always fatal,” Smelski said.

Each year, the foundation hosts the PAM Summit, which invites medical experts from across the country to learn and speak about the illness and better ways to catch it early enough to save lives. The summit is now in its fourth year, and the next event takes place in September 2019.

“We worked with doctors at the first PAM Summit to come up with a care pathway change for hospitals, so that if a patient comes in and is diagnosed with meningitis, it throws an alert into the system that says, ‘You need to check and see if the patient had freshwater exposure up the nose in the last 14 days,’” Smelski said. “It’s an alert [for ruling out PAM and Naegleria fowleri] before you move on and call this a meningitis case. We’ve been trying to share that with as many hospital systems as we can.”

The foundation has also worked to make miltefosine available in more medical facilities. “It needs to be local. It can’t be shipped on a plane because every minute counts, and when the drug is being flown to the patient, you lose many hours.”

In the hours, days and years that have passed since they lost Jordan, the one day that Steve Smelski would give anything to go back and change is June 24, 2014 — the fateful day that he and Jordan went swimming.

“We don’t want to see anyone else go through that, so that’s why we’re continuing to do what we’re doing,” he said.


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