POTS Story

[ETOLY]

Today's WASHINGTON POST focused on a story of a child with POTS as part of their fundraising campaign for Children's Hospital. Thought it might be of interest....See:

http://www.washingtonpost.com/wp-dyn/artic...-2004Dec14.html

Etoly

[MomtoGiuliana]

Thanks for posting this! Here's the article, in case you don't want to go to the effort to register.

A Puzzle in the Shape of a Girl

By John Kelly

Wednesday, December 15, 2004; Page C12

Elise seemed to be just a particularly unlucky little girl, stricken by a variety of unrelated ailments. But what if they weren't unrelated at all? Here's a look at a medical mystery, solved at Children's Hospital.

The first symptoms: Columbia's Elise Smith is 14 now. When she was in kindergarten, she regularly complained of stomachaches. When doctors examined her, they found nothing abnormal. Eventually, though, Elise was unable to move her bowels. She grew distended and experienced abdominal pains. Taking regular doses of a laxative and a stool softener provided some relief but did not eliminate the problem.

Because Elise couldn't reliably clear out her system from the bottom end, she would reflux at the top end, vomiting after she experienced spasms in her esophagus. As long as she could keep her system relatively empty, with laxatives and enemas, she was fine.

The second symptoms: When she was a little older, Elise started having an alarming reaction to exercise. When running down a basketball court, she would turn beet red and get short of breath. Occasionally, she would faint. It was assumed that this was due to the asthma that Elise suffered from, but treating her with an inhaler did little to alleviate the symptoms.

Sometimes, for no apparent reason, her heart would race, a condition known as tachycardia. At the same time, her blood pressure would drop precipitously. Doctors could find nothing mechanically wrong with her heart.

The test: For years, Elise saw various pediatricians and specialists. Then, after her mother started working as a nurse at Children's Hospital, she turned to Children's and Jeffrey Moak, director of electrophysiology and pacing at the hospital.

Dr. Moak scheduled Elise for an unusual test. She was strapped in a supine position -- on her back -- onto a special table. An IV was inserted into her wrist to monitor arterial blood pressure; electrocardiogram leads were connected to her chest to monitor her heartbeat.

As long as she was on her back, Elise was fine, but as Dr. Moak tilted the table to about 80 degrees -- raising Elise's upper body and lowering her lower body -- her blood pressure plummeted to 70 over 30. Eventually, she fainted.

Dr. Moak quickly revived her with a liter of fluid. And then, said Elise's mother, Vannesia Smith, who had been watching the test, "he got all excited." Elise, he said, had classic symptoms of a little-understood ailment.

But even more encouraging: "He said, 'I know how to fix her.' "

The syndrome: Dr. Moak deduced that Elise had a problem with her autonomic nervous system. The autonomic system, also known as the involuntary nervous system, runs all the parts of your body that you don't need to think about. You don't need to command your heart to beat or your stomach to digest food. These things happen automatically, courtesy of the signals the autonomic system is continually sending out.

Elise's autonomic nervous system was out of whack. When she was sitting or standing, blood pooled in her lower extremities. A normal autonomic system makes adjustments, signaling the veins in the legs to constrict and force more blood up to the heart. Instead, Elise's heart was starved of blood and her blood pressure dropped. The official name for her condition is postural orthostatic tachycardia syndrome.

Elise's gastrointestinal problems were related. The autonomic system also regulates motility, the spontaneous contraction of the intestines that moves food through them.

The treatment: Doctors don't know what causes autonomic system disorders. It may be that the nervous system isn't sending messages to the veins in the lower extremities that tell the various blood vessels to constrict. It may be that the veins themselves are somehow defective. Research continues.

In the meantime, Dr. Moak and others have been determining what medicines in which doses provide Elise the most relief. They think they've helped her reach an equilibrium. Inderal helps regulate her heartbeat. ProAmatine raises her low blood pressure. She takes MiraLax, a laxative, and Zelnorm, a drug that treats constipation.

"For the most part, she's normal," Vannesia said. "As long as she stays on the [drug] regimen."

She owes it to Children's. "They don't just accept that this is the most that a child can get out of life," Vannesia said. "You go here and hit a wall, then you come back and try something else."

Putting It Together

"It truly was a mystery," Vannesia said of Elise's problems. The family had been on a roller coaster for years, treating various symptoms in ways that would then cause unforeseen problems. Dr. Moak and his colleagues at Children's Hospital put it all together.

"If you can tell the family that it's all part of the same disease entity, it provides a lot of reassurance," Dr. Moak said.

My e-mail: kellyj@washpost.com

[geneva]

Is it just me or does this seem like a big deal having a person with POTS featured in a major publication??? Thanks for sharing the story.

[Guest tearose]

Thank you for bringing this to our attention ETOLY! And Katherine, I did not have the energy to register..thank you sooo much for posting the article! I will print it and keep it handy. tearose

[MomtoGiuliana]

geneva--it does seem like a big deal to me! I copied this article and sent it to a bunch of friends and family last night as part of my on-going education crusade

You're welcome tearose. I hate all that registration hassle and I figured I'd make it easier for anyone who may not read the Post on-line already.

Katherine

[ETOLY]

My Mom brought me the article yesterday morning and we both thought it was great to see POTS information out there in a major newspaper. And thanks to Dr. Moak at Children's Hospital in Washington D.C. for his awareness of this syndrome.

Etoly

[yogini]

Thanks so much for the article! I also sent it to my family and friends. I noticed that the reporter included his email address at the end of the article. I am going to email him and thank him for writing the article. I also wonder if he would be willing to write more articles about POTS/dysautonomia.

I think awareness of this condition is so important. Someday, when I feel better and have more energy, I would be interested doing things to make the community more aware of POTS through DINET or otherwise.

-Rita

[Ernie]

Thanks Etoly and Katherine for sharing this article. It is encouraging to see that reporters are starting to be interested in our disorder.

Nice initiative Rita to write to the reporter.

Ernie

[Melach]

What a great article! Maybe if the writer gets enough pressure from potsys, she/the newspaper will be motivated enough to write more on the topic. Thank you so much for sharing this!

Ayelet

[briarrose]

I sat down last night and started a letter to the writer but had to put it in drafts. I will finish it but this next 1 1/2 weeks are going to be too busy and I will be limited in what I can do.

I definitely think everyone should send a letter off though.

steph

[Timbo]

Great find! I showed it to some of my family to help them understand what I am going through.

[briarrose]

This was such a great story that I wanted to bump it back through for all of the new people. I was also thinking of Roselover as I was reading it and thought I would send this her way

[DSM3KIDZ]

Thanks for bringing my attention to this story. It's definitely a step in the right direction!!!!!!!!!!!!!!!!!!!!!

I'm going to print this for my family. I'm also happy to hear the girl will be getting some help. I can't even begin to imagine being ill at a young. My heart goes out to her family.

dayna