Rare Disease Column: Guillain-Barre

Posted on July 5, 2008 by arizela

Guillain-Barre may sound like a pub with a bit of a French flare, but this nasty little syndrome strikes seemingly at random and causes progressive muscle weakness, loss of sensation, paralysis, and in about 5% of cases, even death.

Guillain-Barre belongs to a class of poorly understood syndromes and disorders known as “autoimmune disease” – which basically means that your body’s immune system has got its wire’s crossed and is attacking you! A bit of friendly fire later and just about anything can go wrong with a body. In the case of Guillain-Barre, the immune system has set its sights on a very important part of the peripheral nervous system – myelin.

Myelin (see photo) is a layer of cells that cover the long branching fibers of nerve tissue, acting as both insulation and more importantly, speed enhancer for nerve signals. Nerves with myelin coverings transmit signals many times faster than nerves without myelin. That’s why Guillain-Barre causes such devastating weakness and even paralysis – with the myelin damaged and out of commission, the signals from the brain to the body and back get crossed and move slower than your average arthritic turtle.

A neuron with its branching nerve fibers and myelin sheath.

All this leads to a rapid progression of weakness, generally starting in the feet and progressing up the body. The weakness can progress all the way to full paralysis. Weakness generally peaks by 2-3 weeks after onset of symptoms and can take a very long time to resolve completely. 30% of patients still experience weakness three years after their initial onset, and a small percentage of patients may have recurrence or relapse of symptoms many years after recovery.

So what causes Guillain-Barre? We’d like to know, too. The truth is, while we know it isn’t contagious and it is caused by an auto-immune process, how it occurs is poorly understood. In most cases, symptoms begin within a few weeks of a respiratory or GI infection (cold, flu, stomach bug, vomitting, diarrhea). It makes sense, because after the immune system fights off an infection, it is vulnerable to other problems. Symptoms can also be triggered by surgery or immunizations – two more things that mess with your immune function.

The effects of this syndrome are temporary for most sufferers, but during the worst part of the weakness or paralysis, the person is totally dependant on others for daily needs. Even chewing food or breathing may be too demanding for sufferers of Guillain-Barre and it is not uncommon for artificial feedings, IV nutrition, and even respirators/ventillators to be employed. Guillain-Barre can be mild enough to require a great deal of assistance for a home recovery, but in most cases an admission to a hospital and often an intensive-care unit (ICU) is required.

Side effects of the syndrome – poor nutrition, loss of muscle tone, risk of falls, risk of pressure sores (bed sores), and the risk of failing respiratory effort – can lead to further complications. Recovery requires a great deal of physical therapy, and during the high point of the symptoms and thereafter, sufferers have a significant risk for being unable to emotionally cope with their sudden loss of function and dependence on others for basic needs. Emotional support and counseling are highly recommended.

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If a character gets an overdose of pain meds, what will modern medicine do for him?

Posted on July 1, 2008 by arizela

Pain medications are classified as narcotic or non-narcotic. There are numerous drugs in each class. Non-narcotic pain meds are often used for purposes other than just pain relief. There are also quite a few drugs out there used to help pain that are known as “adjunct therapies” – helpers, basically that work with narcotics to control severe pain. I’ll cover a few of the most common drugs to cause overdose and their effects and treatment.

Narcotics

Opiates – this class is composed of drugs like morphine, demerol, codeine, laudinum, opium, and vicodin. It also contains drugs that are mixed with non-narcotic medications, like percocet, tylenol 3, tylenol with codeine, and darvocet. Opiate overdose can cause respiratory depression to the point that someone just stops breathing altogether and then, well… you know what comes next. To counteract that, a hospital would initiate hand-bagging (see photo at left) by putting a mask with a bulb and special valve on the end to force air into the person’s lungs. They would then do one of the meanest things you can do to someone – “slam the Narcan”. Narcan is an IV drug that completely counteracts opiates, almost instantaneously. What that means is, the person who took opiates for pain relief is going to be in a hell of a lot of pain, real fast. And because opiates mimic the body’s own endorphins (brain chemicals that increase pain tolerance), those don’t work anymore either. The person who took opiates to get mellow is going to wake up hurting, too, and severely pissed off. It’s best to have several muscular coworkers standing by when slamming Narcan into someone.

Fentanyl is a drug used to treat pain in the hospital setting. The trouble with this drug is that if it is administered to quickly via IV, it can cause your muscles to lock solid. Including your diaphragm. Even hand-bagging won’t save you, as your chest is too rigid to force air into or let air out. The only way to manage this type of situation is to administer a paralytic – a drug that literally causes temporary muscle paralysis (Vecuronium, called “Vec” by health care workers, is a common one) but not unconsciousness or pain relief. The paralyzed person would then be hand-bagged or more likely have a tube put into his/her airway and be hooked up to a ventillator until the paralytic and the fentanyl wore off. Meanwhile, the person would be awake to experience the whole thing but completely helpless, unable to move or talk, unless the dose of fentanyl was big enough to put them out.

Non-Narcotics

Tylenol (acetaminophen) – overdosing on this might not sound like a horrible thing. It’s over the counter, right? So taking a couple extra shouldn’t be a problem? WRONG. Tylenol overdose is one of the worst things you can do to your body with over-the-counter meds. Tylenol is safe in the dosages recommended by the manufacturer, but in large doses or prolonged cases of taking just a little more than recommended for several days, it can be extremely toxic to the liver. What this means is that while most people who overdose on tylenol are ok afterwards, there’s a chance that you’ve just managed to kill your liver. Liver failure, let me tell you, is a horrible way to die. A person overdosed on tylenol will usually start feeling ill pretty quickly – nausea, vomiting, looking pale. The bigger the dose, the more likely the symptoms. They may go into a latent (silent) phase for the next two days with no symptoms. Some people never progress to the third stage, which involves necrosis (death) of the liver. While most people who overdose on tylenol eventually resolve without dying, the third stage can progress to death, even if treated. Symptoms includes right upper abdominal pain, prolonged bleeding times, low blood sugar, bleeding, and brain damage. 3-4% of the people who experience large or prolonged small overdoses of tylenol die from complications associated with necrotic liver. Treatment consists of inducing vomiting, stomach pumping (removing stomach contents through a tube), giving activated charcoal through a tube (see photo at right. Yes, it’s as gross as it looks, and it’s probably going to come back up the hard way), and in cases of high doses or prolonged moderate doses, giving the antidote drug acetylcysteine, which is effective in most but not all cases.

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How premature does a baby have to be to have trouble breathing?

Posted on July 1, 2008 by arizela

Any baby can have trouble breathing after birth, regardless of prematurity or size, but breathing problems are much more common in babies who are very early, very small and underdeveloped, or very large. Some common reasons for respiratory distress syndrome (breathing trouble requiring oxygen or other support) in newborns include:

  • Prematurity – lungs are underdeveloped. Typical pregnancies last 38-42 weeks gestation (how long the baby is cooking from the mother’s last menstrual period). Respiratory distress syndrome is generally mild to moderate from 34-37 weeks in healthy babies and becomes increasingly severe if the baby comes earlier. Babies less than 28 weeks very often require special medication delivered right into their lungs call Surfactant, to help keep their lungs from collapsing between breaths.
  • Infection – infection in the mother can be passed to the baby. Babies who have respiratory distress that lasts more than a few hours or is severe at or shortly after birth are generally screened for infection (also known as sepsis) and given 48 hour courses of Gentamicin and Ampicillin (antibiotics) even if no infection is identified. Because of immature immune systems, even if the baby has no outward signs of infection other than respiratory distress and the cultures come back negative, the baby could still have a hidden infection.
  • Heart and circulatory defects – there are many kinds of heart defects and circulatory problems that can result in poor perfusion to the lungs or to the body, resulting eventually in breathing problems. Some heart problems don’t cause noticeable problems until the baby’s body starts to accommodate to life outside the womb by closing down special circulatory pathways that the fetus needs, but a breathing baby doesn’t. This can take several hours or even days.
  • Diabetic mothers – these babies are usually very large – >9lb at birth and can be very large even if born early. Because of mother’s chronic high blood sugar, baby doesn’t develop in quite the pattern expected and can suffer respiratory distress, low blood sugars soon after birth, heart problems, and multiple birth defects, particularly if the diabetes was poorly controlled during the pregnancy. Gestational diabetes – a form that goes away after the baby’s birth, can cause the same problems.
  • Anatomical defects – certain birth defects which are rare but can cause severe respiratory distress include anything that prevents the lungs from fully forming or expanding, anything that impairs circulation, brain or brain stem malformations, and anything that causes severe pain or nervous system irritability.


A photo of acrocyanotic feet. Acrocyanotic means a bluish tinge on the periphery of the body – the hands and feet. Notice the purplish heels especially

And since this is for writing and I’m assuming you’ll be describing the scene and the problems, here’s a quick rundown on how a normal respiratory effort looks and some of the trouble signs:

  • Respiratory rate 30-60 breaths per minute – too fast or too slow can be bad. As a nurse, I’d rather see too fast than to slow, though. Breathing too slow (or not at all) is an ominous sign of trouble.
  • No grunting noises – babies in distress often make a little grunting sound at the end of each breath. The reason for this is they are trying to keep a little pressure in their lungs at the end of the breath, to keep their lungs from collapsing. It’s imperative that lungs stay slightly open after a breath, because lungs that are completely closed require many times as much effort to open back up.
  • No nasal flaring – The sides of their noses will flare out if they are trying hard to get more oxygen. Think of someone you’ve seen about to launch into a tirade and how their noses widen a bit as they get their breath to blow like Mount Vesuvius.
  • No head bobbing – Babies in distress will bob their heads with each breath in an effort to open the airways wider to breath in and narrow them down a bit when breathing out.
  • Breath sounds clear and equal – breath sounds may sound a little “wet” or crackly in some babies, particularly if born by C-section as there isn’t a lot of squeezing to get that excess water out of the lungs like a vaginal birth. If the breath sounds are decreased or absent on one side, this could indicate that the baby has a collapsed lung, or is having a problem called pnuemothorax, which means baby has developed a hole in the lung which allows air to collect in the cavity between the lung and the chest wall, compressing the lung more and more with each breath and not allowing it to re-expand. This can lead to a life-threatening condition known as “tension pneumo” in which so much air has built up in the chest cavity that it is pushing everything over – the lungs and eventually the heart – and pressing so hard it’s decreasing circulation. Pneumothorax and tension pneumos are treated by inserting a “chest tube” into the chest cavity that allows the air to be slowly pulled back out and gives room for the lung to re-expand and heal
  • No central cyanosis (blue tinge around face, lips, or the center of the body). It’s ok for baby’s hands and feet to be blue or purplish for the first 24-48 hours, but an overall pink color (pink as in a healthy coloring, not as in salmon or caucasian) is preferred. Babies with very dark skin can be assessed by looking at lips and tongue – if those are pink, no worries

Treatment for respiratory distress in the newborn is pretty organized. If breathing, baby would first be given oxygen either by nasal cannula (see photo at right), by “blow-by” which means blowing oxygen across the baby’s face, or by face mask. If that doesn’t work, hand-bagging would start, which means using a mask and bag with a special valve to force air into the baby’s lungs. A baby being hand-bagged will quickly be intubated (have a small plastic tube inserted past the vocal cords and into the lungs) and would either continue to be bagged through the tube or be hooked up to a ventillator. Deep suctioning through the breathing tube is likely to occur and Surfactant may be given to help the baby keep the lungs open between breaths. Unless the lungs are seriously malformed, this is usually sufficient. In some very rare cases, if the baby was born at a fetal care center or a center that was prepared for an unusual defect, a baby unable to be helped by hand-bagging or ventillator might be put on ECMO which is a long-term (up to 3 weeks, sometimes) heart-lung bi-pass machine. This has MANY risks and about a 50% chance of survival, depending on the reason it is used.

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Rare Diseases: Necrotizing Fasciitis

Posted on June 26, 2008 by arizela

Necrotizing Fasciitis, or flesh-eating disease as the media likes to call it, is a rapidly spreading infection along the fascia or the layer of tissue that binds skin to muscle. This deadly disease is caused by a virulent strain of the same bacteria also responsible for strep throat, rheumatoid fever, scarlet fever, impetigo, childbed fever, and toxic shock syndrome – Group A Streptococcus pyogenes.

This particular form of strep infection usually starts at an area of trivial skin injury – a bruise, scrape, or small cut. The area becomes reddened and hot to the touch as the infection begins, but over 2-3 days the infected tissue begins to die (necrotize). The skin will gradually darken to purplish or black, and large, blood-filled blisters called bullae will begin to form. The below photo from UCSD med school illustrates both the appearance of skin infected with necrotizing fasciitis and the blood-filled bullae.

Left untreated, this infection can quickly march over enormous areas of the body and lead to death in a matter of a couple of days. Treatment involves surgically removing all dead and infected tissue in a process called debridement (de-breed-ment), which usually leaves the muscle layer intact and simply removes all the superficial tissue and skin in the affected region and for a clear margin around the lesion. If the infection continues to spread over limbs after attempts to debride, amputation may be life saving.

Both the infection itself and the treatment are horrendously painful. The lady in the photo above would likely have all the skin stripped off her leg from below the knee to the crotch if she wants to live, after which she’ll look like she’s been partially dissected until after skin grafts are applied. Meanwhile, not having skin is a huge risk for other types of infections. Recovery is likely to be a long process, involving physical therapy and plastic surgery and lots and lots of medications.

While very rare, the Centers for Disease Control reports that there are fewer than 1000 cases a year in the US. Even with quick treatment, death occurs in many cases. Some names you may recognize of individuals who have been infected or died from Group A Strep necrotizing fasciitis include Jim Henson of muppet fame, and Melvin Franklin of The Tempations.

For more information, see these articles from:
— The Centers for Disease Control though this article discusses all invasive forms of Group A Strep together, not just Necrotizing Fasciitis.
— WebMD

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Infected Cuts

Posted on June 26, 2008 by arizela

Q: What do infected cuts look like?

A: First off, there is likely to be an angry red color around the margins that extends anywhere from a few millimeters to several inches (reasonable size for a smallish cut 1-5cm). The area will be swollen, tender to the touch, and warmer than the surrounding skin.

The wound may drain blood, clear fluid, or pus or a combination of these. Pus colors range based on what the infectious organism is, and can include yellow, yellow-green, green, white, or tan/beige, but can also be tinted pink by blood getting into it. Blood from a wound that is infected will often be very dark or have clumps of pus floating in it.

The wound may form a moist-looking yellow scab or a dark, hard scab as it attempts to heal – these are both composed of dead tissue and the wound will heal faster and cleaner if they are removed and the wound bed is kept moist but not sopping wet. That said, constantly picking at a wound is a bad idea, too.

The best treatment for a small cut is to wash with soap and water, and cover with a clean dry bandage (and an ointment like Polysporin if modern era). Try to keep it clean and don’t do what my son does and leave the bandage on for two weeks or until it disintegrates in the shower.

Large or deep cuts may require sutures or some sort of adhesive glue meant for surgical application (no, please don’t use SuperGlue. It works like a charm, but non-toxic really isn’t meant to read: safe to pour into open wounds). Again, washing the wound, keeping it clean, and keeping the wound-bed moist or the sutured cut dry will aid healing.

Infected minor cuts rarely turn into systemic problems, but if the red margin of the wound starts to look streaky or the red starts to follow veins in streaks, this is a sign of serious, potentially fatal infection of the bloodstream and needs emergent treatment. Likewise, a sudden, serious increase in pain, or severe swelling of the area can indicate serious infections.

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