'Jigsaw Kid' Defies Grim Prognosis

[Sasha]

Whoa....

'Jigsaw Kid' Defies Grim Prognosis
Full Article Here: http://www.aolhealth.com/health/chi.../bbdp/jigsaw-kid-defies-grim-prognosis/495253

(May 22) -- Bethany Jordan was born with five spleens, a hole in her heart, a backward liver, a malformed right lung and her stomach on the wrong side of her body. Doctors warned her parents that she wouldn't live much past birth, but she's now 6 years old and is "doing very well despite all of her problems," according to a report in London's Daily Mail.

Although Bethany has five spleens, she is more vulnerable to infections than other people because the spleens are undersized and don't clean her blood effectively. During strenuous exercise, her heart can be seen pounding through her back.

The girl has faced a host of medical complications since birth, including heart failure, liver surgery and a collapsed lung. One of her doctors said she will eventually need a liver transplant. But he noted that treatment for Ivemark Syndrome "is getting better and better."

How would you treat someone like this who goes into cardiac arrest? Is CPR even possible on since their heart is at their back?

 

[nibejeebies]

All i can say to this is... Wow......

 

[VentMedic]

What choice would you have?

Often the family has been trained for emergencies and will also have some form of written instructions to give to EMS or an ED.

When assessing a patient that has reversed or malpositioned organs, for the heart, you can first find the PMI (Point of Maximum Impulse). From there you can listen to heart sounds and get a fairly good idea about the way the heart is positioned.

For breath sounds, not all the alveoli may be available for aeration. Lung Hypoplasia may be present due the the malpositioning or from another anomaly like a Diaphragmatic Hernia.

Chances are she was diagnosed by sonograms long before she was born so there were few surprises in L&D.

Having malpositioned organs is rare but still happens. Some adults can be found with Situs Inversus or Situs Inversus Totalis and Dextrocardia. They should have a medical alert and may carry specific instructions in their wallet.

 

[Kookaburra]

Huh. I suppose you would have to figure out how to position the defib pads so that they were in the correct position. Wouldn't the reason for the cardiac arrest determine where you give compressions? I mean, if she's down because of a foreign body obstruction, you'd just give them normally until the cork popped like any other kid, right?

(I'm asking seriously, please correct me if I'm wrong)

Though this does remind me of a steer my vet had. It was born with his heart in his /neck/. You could see it beating and everything. My vet was convinced he would die when one of the other calves kicked him or butted him there or something, but he seemed healthy and gained weight just as fast as the other steers.

 

[VentMedic]

Huh. I suppose you would have to figure out how to position the defib pads so that they were in the correct position.

Since this will be a known condition, the family should have been trained to do CPR and use an AED. Often, if there is a chance of the child having a cardiac arrest, an AED will be provided for them along with the other home care equipment they may need.

This is one time you do not argue with the family or shove them aside. They will have valuable information for you.

 

[Kookaburra]

This is one time you do not argue with the family or shove them aside. They will have valuable information for you.

What about the chest compressions? I guess the family would only need to call EMS for foreign body obstruction if the marble or whatever was REALLY far down there, this is their kid, so they've probably practiced abd thrusts more than we have.

 

[VentMedic]

What about the chest compressions? I guess the family would only need to call EMS for foreign body obstruction if the marble or whatever was REALLY far down there, this is their kid, so they've probably practiced abd thrusts more than we have.

The trachea will probably be in the same location. The same concepts of FBAO may apply.

Of course, they will call EMS for emergencies. However, it should be understood that they will probably know a lot more then the EMS providers about their child's condition(s). If Sasha had not posted this news article, most of you would never have heard of anything like this or other similar anomalies.

The family will undoubtedly be distraught but you must give enough support to allow them to give you the information you need. Even though they have probably had hundreds of hours of training, this is still their child and they must also be allowed to be parents. However, most will respond to do what they have been trained for.

 

[DrankTheKoolaid]

re

Interesting case and certainly a treatment nightmare. one of the xray techs that works locally has what is called Situs Inversus (sp) basically all of her intrnal organs are opposite of how they should be lying inside her. Not all that rare im told, at least to some degree.