Congenital Insensitivy to Pain: A Real Medical Phenomenon

/ Jordyn Redwood / Leave a comment

Recently, I went to a nursing conference and part of the two day adventure was a discussion of ethics. There are always plenty of ethical issues going on in medicine these days (the Jahi McMath Case and the case of the Texas woman kept on life support for her unborn child to name two recent news headliners.)

The nursing researcher was discussing how to identify ethical issues early on and had developed a research based tool to aid medical staff in, perhaps, not letting these things get out of proportion– say before the news media gets involved.

As part of her talk we answered questions about ethical scenarios. One scenario she presented was of a family whose child was admitted for pain of unknown origin and had frequent trips between the floor and the pediatric ICU for pain management. One question around this scenario was, “Unnecessary pain in children is the worst thing.”

I disagree with this statement. Pain is necessary. It signals us to stop doing something when it is harmful to our bodies– like touching a hot iron. Pain tells us, in differing degrees of severity, when we should seek medical attention. So, I think pain is good. What I would say is that “Uncontrolled pain in children is the worst thing.”

Yes, that I would agree with.

Parents with children who have congenital insensitivity to pain wish pain upon their children. Yes, you read that correctly. They understand the purpose of pain and how it ultimately protects the human organism. In fact, children with this disorder often harm themselves because they can’t feel pain such as gouging at their eyes to the point of injury. In this child’s story below– the parents talk about how, when she was a baby, they took her to an eye doctor and she had a large corneal abrasion and the infant never cried. That’s how they diagnosed her. I can attest– corneal abrasions (a scratch to the eyeball) are some of the most painful injuries there are and are one of the leading causes a crying, fussy infant is evaluated in the ER.

It is a rare, inherited autosomal recessive disease which means both parents have to be carriers and their offspring would have a 25% chance of fully expressing the disease. If you’re a carrier, you can pass it on but are not symptomatic.

Here’s one story about a family with a child with congenital insensitivity to pain.

https://youtube.googleapis.com/v/n6iOUW523BE&source=uds

So, no, pain is not always a bad thing.

Next post, I’ll review a novel where an author used this congenital disorder. 

Up and Coming

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Hello Redwood’s Fans!

Everyone still with us after celebrating St. Patrick’s Day? Anyone do anything FUN?!?

This week it’s all about celebrating good books and weird medical conditions. I’ll be highlighting Lisa Gardner’s Fear Nothing and the medical condition she highlights in the novel– congenital insensitivity to pain.

I’m curious to know what you think– would it be a blessing or a curse to never feel physical pain?

Hope you all have a fantastic week!

Jordyn

Author Question: Drowning

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Sally joins Redwood’s Medical Edge again with an author question. She had visited previously with a question about food allergies that you can find here.

Sally asks:

I have a character that someone is trying to drown. They hold her under water, and she runs out of air, but someone else pulls her up. I don’t want a scenario where she needs CPR or mouth-to-mouth, maybe just some coughing and choking, spitting out water. Does that work for her to be under water only a couple seconds and suck in water?

Jordyn says:

Yes, it works.

I like these types of injuries when writing medical scenarios because it offers you a wide range of things you could do to your character– from nothing at all to significant injury.

Not all people react the same when their head is pushed under water. There is what’s called a diver’s reflex where you will instinctively hold your breath and drop your HR when your face hits cooler water– which most bodies of water are less than your body temperature.

That being said, what a person does after those first few seconds is up in the air which is good because it gives you writing leeway. So the amount of water they inhale and at what point they would do that is for you as the writer to decide.

Possible patient outcomes for this particular scenario:


1. She is just fine (once she gets over her initial coughing and spitting up of water) and doesn’t need any medical intervention.

2. She inhales a little water where it might be good for her to be medically observed for a while but she suffers no ill effects and doesn’t need any medical intervention– just observation on a monitor. This may be only a few hours to twelve hours depending on the physician.

3. She inhales some water and she is symptomatic (low blood oxygen levels, increased respiratory rate, increased work of breathing like using accessory muscles) but doesn’t need any dramatic intervention– maybe just some oxygen to tide her over and medical observation until this clears up. Her shortest observation time would likely be 12-16 hours to watch for further developing lung injury.

4. She inhales a lot of water and develops respiratory distress to the point she would need to be intubated– or go on a breathing machine.

Every patient is different. Sometimes we in the medical field feel that the mechanism isn’t that impressive but the patient does suffer ill effects– so again, a lot of room for the author.

And remember– drowning doesn’t always look like what television portrays it as.

If interested in further medical posts are drowning they can be found here and here.

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Sally Bradley has worked for two publishers, writing sales and marketing materials, sorting through the slush pile, and proofreading and editing fiction. She has a BA in English and a love for perfecting novels, whether it’s her work or the work of others. A judge in fiction contests, Sally is a member of ACFW, The Christian PEN, and the Christian Editor Network. She runs Bradley Writing and Editing Services from her home outside Kansas City. A mother of three, Sally is married to a pastor who moonlights as a small-town cop.

Nosebleeds: Hollywood– Please Stop.

/ Jordyn Redwood / Leave a comment

There are a few things that parents freak out about that are of minor concern to us in the pediatric emergency department.

One of those is the nosebleed. Particularly living in a dry state like Colorado– nosebleeds happen. Your nasal tissue is very vascular so if it becomes dry and irritated, it won’t take much to get a nosebleed started. Generally, all that is needed is a little extra moisture. A humidifier in the room. Some saline nose drops and perhaps some Vaseline applied to the inside lower portion of the nostril to resolve.

In my twenty plus years of nursing, I’ve never seen a nosebleed be a sign of any horrifying diagnosis. I’m not saying that it can’t be (and this is what likely sends most parents to the ER) but it would be an uber-rare event.

But Hollywood seems to have a fascination with nosebleeds. Anytime a character is using any increased mental prowess or mental super power– this is signified by a nosebleed.

In fact, I found some support of this ridiculousness with this blog post on 7 Most Ridiculous Psychic Nosebleeds in Movies and TV. It’s genius. 

And it has become an annoyance of mine.

Evidently, the medical assumption is that there is soooo much pressure in the brain from all this mental sommuersaulting that it has caused the nose to start bleeding.

If that were true, then we would see medical correlation for this. I’ve worked in intensive care where patients have had measured increased intracranial pressure to the point that they herniated (or shifted) their brain to places it shouldn’t go.

And still– no nosebleed.

Your nasal tissue isn’t in direct communication with your brain (it’s not part of that cavity) so it doesn’t make sense for a nosebleed to be evidence of increased brain pressure.

The only instance this might be medically reasonable is when there is a basilar skull fracture where the bones that line the bottom of your skull break. Then there does become a correlation between your brain and your sinus cavity and drainage from the nose can happen in that instance.

But otherwise– Hollywood– let’s let the nosebleed go.

Up and Coming

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Hello Redwood’s Fans!

What’s new on your side of the world?

Here? Finally the snow is melting and spring might be peaking around the corner. I am hopeful we have seen the last of the snow but I do live in Colorado and we had snow into May last year.

For you this week:

Tuesday: A blog post about Hollywood’s fascination with the nosebleed. Is it medically accurate to portray nosebleeds as a sign of increased pressure in your head?

Thursday: Sally Bradley returns to the Medical Edge with a question about drowning.

Hope you have a fabulous week! And if you’re celebrating St. Patrick’s Day– remember– responsible drinking peeps.

Author Question: Does a Stroke Signal a Monitor Alarm?

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Holly Asks:

When a patient is in the ICU being monitored, are there warning signals (ie beeping noises from machines to alert, etc) right before or after a person has a stroke? In other words, can it come on suddenly and how does the nurse know it’s happening or has happened?

Jordyn Says:

This is a great question. What exactly can a monitor do and not do for a nurse?

When I was still in nursing school, I did an internship in an adult ICU. At first, I would go running to every alarm and, often times, the nurses would stay at the station and analyze what was happening. Then, I never went into the patient’s room and one day– all the nurses went running to help a patient suffering a lethal rhythm. Discerning what is and is not a patient emergency is a learning curve for every healthcare provider and the monitor should be a tool in the toolbox and not the ultimate decision maker.

Generally, when a patient is admitted to the ICU, they are placed on cardiac monitoring. This generally includes an ECG (the heart rhythm), the placement of the chest leads will provide a rough count of breathing (through movement of the chest wall as detected by the leads) and oxygen level (which is the lighted probe placed to a finger.)

These are the basics.

All ICU monitoring systems have a tiered alarm system. Meaning, each heart arrhythmia (and other things) are not treated with the same severity. For instance, a heart rate that falls outside the preset parameters may cause the monitor to signal a repetitive single beep or other tone. A good example of this in pediatrics might be a kid whose heart rate increases due to fever and speeds up outside the preset zone.

When a patient goes into a lethal rhythm, like v-fib, v-tach or asystole, the monitor will triple tone.

A stroke is a brain event. Either bleeding, a clot or ischemia causes the patient to lose certain neurological functions that may include speech, and function of a limb. ONLY if these symptoms were precipitated by other vital sign changes (perhaps a drop in oxygen level due to poor breathing) would the monitor alarm. A stroke may not present with a lethal heart arrhythmia. I think a patient would have to signal a nurse that they are experiencing these symptoms or the nurse may discover the patient has suffered a stroke at a scheduled assessment.

However, if the patient is seizing as a result of the stroke, this could cause the monitor to alarm. The seizure motion shakes the leads and it can resemble v-fib on the monitor even though the patient may still be in a normal rhythm but the monitor doesn’t know the difference so it will alarm. 

So, I would say it is possible for a patient to suffer a stroke without the monitor alarming.

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 Holly Michael has been published in various national magazines, local newspapers, and in Guideposts books. She also worked as a journalist and features writer. Recently, she signed a contract with Harvest House for a devotional book she’s writing with her son, a type one diabetic in the NFL. Holly lives in Kansas City with her husband, who is an Anglican Bishop. She blogs at http://www.writingstraight.com/ Visit her author page at https://www.facebook.com/AuthorHollyMichael

Forensic Topic: DNA Analysis

/ Jordyn Redwood / Leave a comment

I’m so pleased to have forensic expert Amryn Cross back with a question from a reader.

Welcome back, Amryn!


S.H. posed the following question about DNA analysis:
In my book, an investigator has a DNA test done on some samples and two profiles are found. The profiles are for half siblings, a man and a woman, who share the same father but different mothers.
What I need to know is this: Could they tell from just the brother and sister’s DNA that they are half siblings? I know they could probably tell that they’re related, but how clear would the match be? Would it be possible to say they share the same father? Or would they need to take a sample from dad to indicate that both were likely to be his children?
This is a great question about what can and can’t be gained from DNA testing. The tests performed in most crime labs will look at a set of 13 markers plus an additional marker to determine sex (amelogenin). For each of these 13 markers, a person will have two numbers. For instance, at marker number one, person A might have the numbers 10 and 13. We would say that their profile at that marker is a 10,13.
Using basic genetics, we know that a person inherits one of these numbers from the mother and one from the dad. In the above example, person A’s mother might have been 10,11 and the father might have been 11, 13. In that case, person A inherited the 10 from the mother and the 13 from the father. As you can imagine, this gets quite complicated when you have to look at several sets of these numbers which make up a DNA profile.
If you compared the profiles of a mother and her son or daughter, they would have at least one number in common at each of these 13 locations. If you also had the profile of the father, you could see that the child would also share at least one number with him as well. But for a brother and sister, things get more complicated. Look at this example:
Mother: 10, 11
Father: 13, 14
Son: 10, 13
Daughter: 11, 14
As you can see, each child received one number from their parents, but the brother and sister don’t share any numbers. Therefore, it is possible that we wouldn’t suspect they were related based on their DNA profiles. If you throw half-siblings in the mix, it becomes even less likely that the connection would be recognized if you didn’t have a reason to suspect it in the first place. It is possible that half-siblings would share no markers or at least not any more than unrelated people.
If you had the father’s DNA profile you would likely be able to say that he’s possibly the father of both, but not definitely. There are statistical calculations that can be done to help determine the degree of relatedness. Special testing of the Y chromosome would be able to tell that the father and son are definitively related but wouldn’t be helpful with the daughter.
As a side note, many crime labs won’t do paternity testing, and if your investigator didn’t already suspect these two people to be siblings, he would have a hard time getting a warrant for the father’s DNA profile. Of course, if the father willingly provided it, a comparison could potentially be made.
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Amryn Cross is a full-time forensic scientist and author of romantic suspense novels. Her first novel, Learning to Die, will be released in September. In her spare time, she enjoys college football, reading, watching movies, and researching her next novel. You can connect with Amryn via her website, Twitter and Facebook.

Up and Coming

/ Jordyn Redwood / Leave a comment

Hello Redwood’s Fans!

How has your week been?

Nothing too new here on the Redwood front. Busy saving lives, writing book proposals, blog posts and learning all about databases. I know– I can see you’re full of jealousy.

For you this week:

Tuesday: Amory Cannon is back answering a really fascinating forensics question about DNA testing.

Thursday: What can a monitoring system for a nurse? Can it detect a stroke? I’ll take on this question for Holly this week.

Hope you all are well.

Jordyn

Author Question: TB and Lung Surgery

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What happens when a surgeon takes out the wrong lung?

This writer’s question came from Lana and actually brings up several interesting points of discussion for her novel. First of all, the question stems from a family incident in 1954 which would really be considered historical as far as medicine is concerned.

Let’s dive into Lana’s question.

Lana asks:

I am a new writer and have some questions regarding a medical incident that occurred in my family in approximately 1954, but today the details are sketchy. Dr. Mabry (thanks Richard!) gave me your name.

The story: My uncle was told he had TB and must have his diseased lung removed. He had surgery, but the wrong lung was taken out.

Question #1: Would they have planned to remove the lung because of TB and would a doctor have actually taken out a whole lung or would it have been one lobe?

Question #2: Would the doctor have been able to see his mistake immediately after surgery? I’m not sure how the mistake was made or discovered.

Question #3: After removing the wrong lung (or lobe), how long would it have taken to reschedule another surgery?

Jordyn says:

One– I have to thank a physician coworker for her help on these– thanks, Liz!

Question #1: It depends on how diseased the lung was. Back then– there weren’t antibiotics to treat TB like there is now so this was considered treatment. However, since it didn’t cure the infection like antibiotics would– I’m not sure how beneficial it was for the patient. If on x-ray it looked like the whole lung was involved then they would have taken the whole thing out. If it looked like just part was involved– then perhaps just a lobe.

Question #2: The doctor would not have known about his mistake until the pathology report came back. The doctor I spoke to said on the outside– the lung might be very normal appearing (which perhaps played into the wrong lung being removed) but all removed biological things go to pathology to confirm a diagnosis. The wrong something being taken out or off is rare but does happen and lots of things play into these surgical errors. I’m going to provide some links below that talk about how these happen in some other situations.

Question #3: Reschedule surgery? Obviously– if they took out the whole lung he could not go back for another surgery to remove a whole other lung– because then he’d have nothing to do oxygen exchange and would therefore die. I guess they could remove part of the remaining lung but I’m not sure how much lung tissue you need to survive. This could be an area for you do some reading on. I couldn’t find a quick answer for you. It looks like the first successful lung transplant was in 1963 and it would have taken time for these procedures to become commonplace. If they did take him back– perhaps they’d wait for him to recover from the first surgery which might be a good 2-4 weeks I’m guessing.

Here are some links to this particular kind of surgery error:

http://www.nytimes.com/1995/09/17/us/doctor-who-cut-off-wrong-leg-is-defended-by-colleagues.html

http://www.cnn.com/2010/HEALTH/10/18/health.surgery.mixups.common/

http://www.lasvegassun.com/news/2011/jan/29/double-ouch-doctor-operates-wrong-knee/

Has anyone had this experience or known someone this has happened to? Did the hospital disclose why the error happened?

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I’m Lana Kruse—last name pronounced Kruzey, rhymes with doozie! I’m one of the original baby boomers—you know, before we became a whole generation. It’s been fun to have been well-known, watched and written about since birth! I’m a wife, mother of two, grandmother of five (aka Mimi), and friend. I hope you will join me in that last category via my blog. I love people, words, laughter and eating out. Put all of these things together, and I’m in heaven!

Author Question: Lung Injury

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Stacy Asks:

What would a lung contusion involve? Specifically, how would it be diagnosed (you said it could take up to six hours to show on x-ray), how would it be treated, etc? I Googled it, but still wasn’t sure how it would work in my story. Since she has broken ribs, I’m assuming the treatment would have to be altered a bit (I read something about using a spirometer and coughing …) She’s in a small-town med center rather than a big-city fully-equipped hospital.

Jordyn Says:

A lung contusion is a bruise. It really depends on how much of the lung is involved as to how sick the patient will be. From this link you can see how different a chest x-ray can change after a few hours with this injury. Whenever anything is injured, an inflammatory response happens which explains why there is a delay seeing it on chest film. Chest x-rays, in certain disease conditions, can lag behind patient exam so they are just one piece of the puzzle.

The problem becomes that whenever there is fluid from an inflammatory response, those areas of the lung can’t participate in oxygen exchange because the lining between the cells where gas exchange occurs is big, puffy and full of water. The more involved the lung is the sicker the patient will be. We can try to drive out the fluid by using ventilators, or positive-pressure ventilation, to force the water from the lungs. In absence of pending respiratory failure, the patient needs good pain control, chest physiotherapy and supplemental oxygen if saturation levels are below 90%.

I think the above link gives a nice overview of the injury complications and treatment.

I don’t see that trying spirometry would hurt– it’s just going to be painful but they can assist with her doing this exercise by giving her a narcotic pain reliever.

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Stacy Aannestad lives deep in the heart of the great state of Texas with a husband named Bjorn, just across town from where their son is studying linguistics at the University of Texas. She is currently writing the first of a series of novels involving a guy from New Zealand, a girl from Texas and a Christian rock band comprised of guys with accents from several parts of the English-speaking world. (Clearly her family has a love of languages!)