The Aftermath of Phil’s Mumps

Today, Phil is a successful software engineer, father and husband, remarkably articulate and smart. It wasn’t smooth sailing getting there, though.

In 1964, Phil was 8, in third grade. He thinks he got the mumps from school: at that time, pre vaccine, it was going around. He had a “fairly standard course of mumps, with the swelling and so on. And then several days into that I was feeling better and was in my PJs on the floor playing with whatever it was I was playing with at the time, and I heard a ringing in my left ear and then nothing.” He could no longer hear in that ear. “And then shortly thereafter I started feeling very dizzy and the vertigo started. What happened was the world started spinning counter-clock-wise around an axis somewhere up and to the left. And it took my brain about a week to sort out and the vertigo to subside.”

Phil’s family were members of an early HMO, and the head of pediatrics there examined him and confirmed that he had encephalitis as a complication of mumps and that the “encephalitis severely damaged the auditory and vestibular nerves on my left side. There was deafness on the one side.” The vestibular nerve does a number of things, but most importantly for this story, helps maintain balance.

All his life, Phil lived with the after-effects. The encephalitis “pretty much knocked out the vestibular nerve. Many years later as an adult my intern heard the story and he made me a bet he knew he could win: he said ‘I bet you can’t walk a straight line with your eyes closed.’ And of course he was right, because I have learned to use vision to compensate for the loss of the vestibular function. Sometime it’s a little comical. When my son was young he loved vestibular stimulation. And there is a theme park up in New Hampshire geared towards little kids and they had this lovely antique gravity driven roller-coaster.  As roller coasters go, it’s very gentle, designed for little kids and their parents. I took my son on it once; he loved it, but I was white-knuckled and ready to heave everything in my stomach because I couldn’t deal with the motion of the roller coaster car.”

What was left of his vestibular function collapsed during final exams week in the spring quarter of his sophomore year in college. “What happened was I got this series of vertigo attacks and ultimately it landed me in the university’s teaching hospital. And they did a work up and pretty much concluded that it was the last gasp of the vestibular nerve on the left side. They followed me from then until I graduated, and when I graduated they gave me a referral to somebody at Mass. Eye and Ear. When I got to Mass. Eye and Ear this particular provider was no longer there and they had me see somebody else who apparently did not get a good grade in Bedside Manner 101, because when he was examining me the first thing he said to me was, ‘by all rights this should have been bilateral’.”

“The other thing that was damaged of course was my hearing. When they did neurological testing back after I recovered from the mumps encephalitis I had some bone conduction because it was my right ear picking up sound transmitted through my skull but nothing from my left ear. And so the other sort of comical thing is, if you make me try and rely on directionality of sound – I’m completely lost. If you hide in the bushes and call my name I’m going to have to use vision -- I’m going to have to turn in a 360 degree circle looking for signs of motion visually.” Games like Marco Polo were not for Phil.

Phil considers himself lucky, on many levels. He points out that the encephalitis could have been much worse: “I think I was very lucky, given that I’d contracted mumps encephalitis. There was a child two years younger than me in my elementary school who died of chicken pox encephalitis.” And “if this guy that had flunked Bedside Manner 101 had been right, I would be deaf. Fortunately I have the hearing on my right side.”

He says: “The other thing I was lucky about – I gather mumps can result in sterility. But I am the proud father of two wonderful grown children. “

No, he does not think any of the childhood diseases are benign. “There was no measles vaccine, so I had measles. I think that’s when I hit my record for body temperature, I ran 105° (Fahrenheit) fever for a little while, so that wasn’t particularly fun. I hope I don’t get shingles because I had varicella. Might as well mention rubella too. Rubella was part of the picture too - I know a woman my age who is on the autism spectrum, but her autism had a known cause: her mother contracted rubella when she was pregnant with her.  She was a rubella baby.”

Lucky as he was, he’d rather not have had the disease to start with. “Needless to say that is something I would rather have not happen. This is one of the reasons I think the whole anti-vax movement is particularly irksome. I think it’s crazy to let these childhood diseases take their natural course.

Acknowledgements: I am very grateful to Phil for sharing his story and helping with the draft and to Alice Warning Wasney for comments on the draft.

Four and a Half Years is not Enough: Neisseria Meningitides Takes a Child

It’s been years, but Nona (name altered to protect privacy) still remembers it vividly. It was 2005. The clinic in Israel served mostly Ultra-Orthodox Jews, families with multiple children living in very close quarters and hence very susceptible for infections transferring from one to another. The child in question was four and a half, and has been to the clinic only once before. “A cute kid,” Nona says. “Pale face, I think on the tall and skinny side for a girl her age, dark long hair in  a ponytail.” That day, however, she was not doing well.

It was in the second half of June 2005. Nona was seeing a patient, and her secretary came in and told her there was a very sick looking little girl outside. Nona and her patient understood the urgency and finished their session, and the child’s mother wheeled her in in a stroller.

Nona says: “You know when a girl past the toddler stage is being brought in a stroller, it's serious. The mother told me her daughter was fine until last night, when she developed a high fever and vomited a few times. Then she seemed to settle down and sleep, but this morning was hard to wake up. She put the little girl on the examining table and we undressed her. The girl was in a semicomatose state, she responded a bit to pain stimulus but not much else, her neck was stiff as a board, and I noticed some odd markings on her legs. It wasn't the classic bleeding under the skin you see in more established cases. Still, I knew immediately what it was.”

The markings, said Nona, looked like this or like this.

At a later stage, it probably developed into something like this:

Picture taken from the Public Health Image Library, Courtesy of CDC/Mr. Gust

Nona had seen a full-blown case as a student. It was very, very memorable. Nona whispered to the nurse that they had a little girl with meningitis, probably meningococcemia, and asked her to bring an IV set. She also told the secretary to call and ask for a mobile ICU unit and to write down the names of all the patients in the waiting room. Nona knew they may have to put all the patients on antibiotics. In the event, the instruction from the public health authority was that given the short duration of the exposure, only the nurse, Nona herself and the little girl’s family – including her mother, pregnant at the time -  needed the antibiotics.

She went back to her patient, and put in an IV. The little girl was “fully comatose by then and made no protest at all to the painful stimulus.” The rash turned more purple on the legs climbed up to chest level – although it had only been ten minutes or less. Nona says: “I'd never seen ANY infectious disease progress that fast.”

Nona went searching for the appropriate antibiotics, ceftriaxone. She explains that it’s a “very broad-spectrum antibiotic. A good thing to give if you don't know what you're dealing with for sure.” She couldn’t get them: on Tuesday, the pharmacy was only open in the evening, and this was morning… and only the pharmacist had the key. The Mobile ICU showed up; Nona showed the doctor the rash and he understood immediately what was this was. Nona said “I asked if he had any antibiotics for the way, I seemed to think he did but ultimately found out the ambulances don't carry it (or didn't at the time).” Since that episode, the doctors at the clinic bought privately their own supply of cefrtriaxone and kept it in the resuscitation cart. “Luckily,” says Nona, “we never had to use it again.”

The Mobile ICU took the little girl away. Later, Nona called the hospital’s PICU and talked to the chief. The Chief told her that the little girl arrived at the hospital intubated. She’d lost consciousness before the arrival of the Mobile ICU, before she was taken away, but Nona explains that this suggests that her situation had continued to deteriorate in the ambulance, or that they put the tube in a s a precautionary measure, something often done for unconscious patients expected to be on life support. The ambulance called ahead, and a full team was waiting for the child as she arrived. She was given antibiotics immediately upon arrival. Since she was in such a bad condition, the diagnosis was based on her clinical signs: they did not wait for a lab culture.

The little girl “hung on in the PICU for 9 days without ever regaining consciousness.”

Then she died.

Meningococcal is a horrible disease, with a high rate of deaths and a high rate of complications; see also here.

There’s a vaccine available against meningococcal, though it does not cover all the common strains. It is recommended for teen-agers and college students, though it is not routinely recommended for infants in the United States  (it is in Australia). The National Meningitis Association summarizes:

“Meningococcal disease, sometimes called bacterial meningitis, is a potentially fatal bacterial infection that may cause death or disability within hours. However, it can be potentially prevented through vaccination.” 

Would the vaccine have helped this little girl? We don’t know; it would depend which strain she had (and there’s always a slight risk of vaccine failure). But it can prevent other children from having the disease, with its dangers and complications. 

For a long, long time, Nona felt guilty, on top of the pain and distress at losing the child. To some degree she still does, though she had since realized that she and her colleagues did the best they could in the circumstances. She blamed herself for not immediately giving antibiotics, not having the necessary antibiotic available, though eventually she realized the antibiotics in question was not standard issue for clinics, not something usually available, because it was so rarely needed. The child’s family did not blame or criticize them: they  “thanked us profusely”, and are still with the clinic. But she remembers, vividly, the little girl lost to the disease. 

http://beforevaccines.blogspot.com/2013/09/four-and-half-years-is-not-enough.html

Acknowledgements: I'm grateful to Karen Ernst and Alice Warning Wasney for their help with the draft, and to Nona for sharing her story. 

Too Close for Comfort: Rahel’s Experience with Chicken Pox

It was a hard time for Pamela as it was. Her husband had just died, and she found herself alone with four young children, three boys and a girl. Her oldest, at six, had just started first grade. Her second son was in Kinder Garten, her third was three, and her daughter was still a breastfed baby, at seven months. The youngest two were home with her, and the focus on them helped, though she was lost and upset, trying to find some normality in a world turned upside down.

Baby Rahel was already sitting on her own and had just started pulling herself to a standing position.

And then it started. Says Pamela: “One day my oldest came home acting a bit 'off'. Later that evening, when I took his clothes off, I noticed a blister under his arm and at first thought that his clothes had rubbed against his skin. I have to add that my oldest is handicapped and has extremely sensitive skin, so that was also a possibility. Anyway, the next morning he was covered in blisters.”

The child’s fever shot up, “quite high”, Pamela said. During that second day, her second son started to get sick, followed by her three-year-old the following day.

All of them suffered substantially, says Pamela: “[the eldest] had blisters mostly on his torso and his fever went away after a few days, once the blisters started to dry up. [her second son, five years old] had it bad, the back of his knees was so covered he couldn´t even bend them and he also had blisters on the inside of his eyelids. [her third son], three, had a very high fever for several days and was covered from head to toe."

At first Pamela thought the disease spared baby Rahel – “we got lucky with her.” Pamela herself knew that a few years earlier, when her titers were measured, she had high levels of antibodies; she believed breastfeeding Rahel protected her against the chicken pox.

She was wrong. A few days after the boys were sick, as they were healing, “I woke up in the middle of the night to her whimpering. When I turned the light on, I was in shock: she was covered with blisters and had a really high fever.”

“During the course of the day, she got more and more spots and her fever got higher so we called our pediatrician . He came to the house (as he usually did in very serious cases) to check on her and prescribed some calamine lotion and something to bring the fever down a bit.”

The pediatrician was shocked. He was an experienced pediatrician – who took care of Pamela herself as a baby. He said that in his many years practicing, this was one of the worst cases he had ever seen.

Rahel was not getting better. After a little while, says Pamela, “there were more blisters on her than normal skin. It was awful, she had a raging fever, was so weak she couldn't even feed anymore and could only whimper. Her eyes were red and light-sensitive, horrible.” The pediatrician, asked to visit again, took one look at the baby and called an ambulance. Pamela and Rahel were taken to the hospital; Rahel arrived with a fever of 41 degrees Celsius, 105.8 Fahrenheit. They was immediately admitted to the isolation unit in ICU, where Rahel was attached to monitored and an IV inserted into her arm. She was given fluids and anti-viral medications and medication to reduce her fever. Pamela was very, very frightened.

The pediatrician said it was the worst case he had seen in his many years practicing. He asked permission to give Rahel a new drug that has just come out, explaining that without something to help she would die anyway, and that way, she at least had a chance. Rahel was not really conscious – but Pamela was both scared and distressed. She says: “It was horror. After losing my husband a month earlier, I thought, now I am losing my only daughter as well.”

Pamela and Rahel spent four days in ICU, and then several more days in the hospital. Recovery at home was long. Rahel, previously active and already pulling herself to a standing position, was so weak she could hardly lift her head. She had lost 15% of her body weight – a lot, for such a young baby. The blisters left on her body covered her all over, including her diaper area, kept getting infected and oozing, and had to be covered with antibiotic ointment. It took many weeks for her to get anywhere near normal.

Pamela feels very strongly about vaccination. Having been through such a traumatic experience, she cannot understand why a parent would not vaccinate their child against chicken pox. The disease might be mild in many cases, but it can kill (See, for example, here and here) or lead to seriouscomplications and suffering. The vaccine, on the other hand, is extremely safe (see also here), and although a child can still get chicken pox after it, it is almost always a much milder version:

Meme provided courtesy of the Facebook page Refutations to Anti-Vaccine Memes: https://www.facebook.com/photo.php?fbid=505923146144366&set=pb.414643305272351.-2207520000.1379638360.&type=3&theater  

Rates of shingles among vaccinated children are also substantially lower than among unvaccinated children.

Pamela is clear: if she could at the time, she would have vaccinated her children against the disease. Her main reason for sharing her story is so that other parents realize how dangerous chicken pox can be, and take precautions to prevent their kids suffering through something like this. 

Acknowledgements: I'm grateful to Pamela for sharing her story and to Alice Warning Wasney and Clara Obscura for reading and commenting on my draft. All errors are, of course, my own. 

A March of Diseases

Peter was  born in 1945. He has one older sister and one younger. He remembers his miserable experience with both chicken pox and measles. He was lucky, he knows, not to have any complications from either disease, but young as he was, he remembers his experience with both as quite unpleasant.

Peter was not yet six when he had the chicken pox.  His older sister got it, and he got it a few days after. His parents feared that the youngest, his two-year-old sister, would also get it. Luckily, she did not. Peter says: “I think we were both had the running sores at the same time. That was pretty miserable.”

Peter remembers being  “slathered with calamine lotion several times a day and having these big pink splotches everywhere. Although it was standard treatment at the time, it didn’t seem to help much.” He doesn’t remember how long he was sick for, but “the temptation to scratch was so intense that for a few days I know I was wearing my kiddie-size boxing gloves that I had gotten for Christmas the year before.”  It was his parents’ idea, and “they definitely kept me from scratching.”

His measles experience, at around ten years of age, was also memorable, though he seemed to have had a mild case, since he does not remember feeling particularly ill. He does vividly remember having to stay in a darkened room and not being allowed to do anything at all. Any mental stimulation or excitement was thought to be bad for a child with measles at that time. It was summer, and he missed the Fourth of July fireworks, and playing with sparklers, and was upset that he could hear his sisters enjoying themselves outside. An avid reader, he was not allowed to read, and felt that deprivation keenly.

Two summers in a row, a little past his tenth birthday, there were polio epidemics. They were not allowed to go to crowded public places, including beaches, amusement parks, municipal swimming pools, even to the movies. “It was basically stay-close-to-home time. We were worried to death. At one point we were in a doctor’s waiting room and a kid came through who had been exposed to someone who had active polio, and I had a few days of worrying about that, though I now realize the risk was very small. That’s how strong and pervasive the fear was before the Salk vaccine.”

He remembers seeing and reading about iron lungs, the iconic symbol of polio. They were tubular metal tanks in which one lay, with only one’s head sticking out. A tight seal around the neck isolated the tank from room air pressure, and a piston decreased the air pressure inside, drawing air into the paralyzed person’s lungs.

Picture taken from: http://en.wikipedia.org/wiki/File:Iron_Lung_ward-Rancho_Los_Amigos_Hospital.gif.  

Later, as an adult, he got a close-up experience: “…I started working for a med school, and … we had a few old iron lungs tucked in the back of one hallway.  Before they were disposed of we actually got a chance to see what it was like to be in one. It was quite strange having the air go in and out with no effort on your part. They even had a cough setting which would actually force you to cough by a rapid motion of the piston.”

Asked how it felt, he said: “I’ve never been particularly claustrophobic (until the first time I had an MRI of my head) so I didn’t have that feeling, but the seal around the neck was not terribly pleasant—then again, if you were in one of those things you probably wouldn’t be able to scratch it anyway. But the knowledge that this machine is breathing for you, even when you could breathe on your own, that was a little strange. I don’t know if I can get my head around what it would be like to have to breathe with that machine.” 

In 2008, he mentions, the last person dependent on an ironlung in the United States died – not of her disease per se, but from a power failure.

There were almost always children in every school Peter attended that had legs in calipers (Americans call them braces). He vividly remembers a boy who sat next to him in a class whose right arm was nothing but skin and bone from polio. “I had a strange fascination with it; I’m sure he thought I was extremely rude.”

A memory from a later age also remains with him: “When I was in college, I went to a  mixer, …  and I saw this stunningly beautiful girl sitting at a table. I went over and made a little chit-chat, asked her if she wanted to dance and she said “No, I can’t.” I stupidly asked why, and she just kind of turned away and waved me off, making a “go away” gesture with her hand.  Later in the evening I saw her leaving the area where we had the mixer and she was walking with a pronounced limp. One of her legs was just skin and bone. ”

While Peter did not have other vaccine preventable diseases, they were in the background – whooping cough, mumps. He remembers mumps “was going around when I was in my teens and my mother I recall being concerned that I would get it because I’m the last male in my particular patriarchal line and I had ‘a responsibility to carry on the family name.’ ”

In high school, there was a classmate who died of meningitis. “It was early in the week, Monday or Tuesday, and in Latin class Miss Gardner started talking about a girl who was absent, whom I didn't know very well, and the way she was talking—"it was meningitis, and it was very quick"—and I was thinking "Wait, what? Someone died?" Yes, someone in our Latin class died of meningitis over a weekend. I'm told that because of the vaccine, most doctors these days have never seen a case of it.”

Peter also remembers having the flu as an adult. He says: “it’s not a bad cold. It feels like you’ve been hit by a truck. If the air is circulating in the room and your hair moves it hurts. Just incredible hypersensitivity to any sort of touch to the skin. Any effort at all would cause me to start sweating, and I could feel the droplets flowing across my scalp. They hurt."

More recently, in 2009, Peter lost a Facebook friend to H1N1 flu. He describes her as a “very colorful woman; absolutely unforgettable.” To protect her privacy, he asked not to disclose her name, but he said: “When she was first taken sick, she kept going on — posting on Facebook — about how bad she felt. Really, really bad. She was hospitalized and it was reported that they were still trying to figure out what she had, and a few days later some family was there and all of a sudden she sat bolt upright, stopped breathing, fell back and she was gone; they couldn’t being her back. And it turns out that it was H1N1 flu, verified by PCR testing.”

Peter says: “When the H1N1 flu vaccine first came out, one of my Facebook friends asked our circle if we thought she should get the vaccine. Despite the fact that that particular group leans strongly toward “alternative medicine,” the discussion was fairly polite. Some of the usual misinformation was shared, like “I got the flu from the vaccine; never again,”  and she said that she'd decided to take her chances because the "natural" immunity was "better,” and I thought, “To get the natural immunity you have to get sick as hell for two weeks; what is wrong with you.” I don’t remember whether I posted that or not; sometimes it’s best to just accept that people won’t make the best choice despite your efforts.”

“Pro-vaccination?” Peter concludes.  “I'm as pro-vaccination as it gets.” Knowing what he knows, seeing what he has seen, he does not understand how anyone could be otherwise.