Illness doesn’t stop Negaunee teenager

NEGAUNEE – In many ways, Angeleah Gravedoni is a typical teenager. The 16-year-old Negaunee High School junior is an excellent student, earning straight A’s; and outside of school, her great passion is racing motocross.

But four years ago, Gravedoni was diagnosed with postural orthostatic tachycardia syndrome – an illness whose many symptoms manifest because the patient’s nervous system doesn’t function properly, causing, among other things, wild fluctuations in blood pressure. It is often extremely debilitating.

Angeleah’s struggle with POTS literally began overnight, changing her life forever.

“She went to bed on Jan. 2, 2010 fine,” said her mother, Rhonda. “And on Jan. 3, physically could not get out of bed. And she was in bed for almost six weeks.”

Angeleah’s symptoms included severe headaches, extreme fatigue, difficulty breathing, dizziness, muscle weakness and “brain fog,” among others. Visits to her pediatrician and multiple area doctors and specialists could not identify what was wrong. Her pediatrician thought the symptoms were psychosomatic, or psychological in origin. And the results of a CAT scan on Angeleah’s brain had an ear, nose and throat specialist thinking she had a fungus growing on her brain; a followup scan showed that that wasn’t the case – but had it returned the same results as the first, the next step would have been to schedule the girl, then only 12 years old, for brain surgery.

“I didn’t know. I honestly thought I was dying,” Angeleah said. “I didn’t know what was going on. I just kind of wondered why it happened to me, and why, overnight, it just happened.”

For the six weeks she was bedridden, Angeleah’s bewildered doctors placed her on a smorgasbord of drugs to try to address her symptoms: she was put on Loratadine, Cetirizine and Fluticasone – all allergy medications; for her pain, she was prescribed opiates like guaifenesin with codeine and dextropropoxyphene (best known as Darvocet); and she went through several antidepressants, to no effect.

Part of the reason it was so difficult to diagnose Angeleah – and why for years it remained difficult to treat her – is how common her symptoms are to a wide variety of different illnesses.

“They were grasping,” Rhonda said of the doctors. “And when you say the symptoms match for so many other things … It was frightening. I’m not saying that doctors are God, but I’ll tell you what, when they’re treating your child, you just hope they’re as close to God as they possibly can be.”

Another factor is how recently POTS became recognized as an illness. Though symptoms and evidence of the disorder can be found dating back as far as the 1940s, it wasn’t named as a disorder until 1993 – meaning many doctors simply haven’t learned about or studied it.

Experts still don’t know what causes the disorder – though there’s evidence of a genetic component, it seems to disproportionately affect teenage girls and women of menstrual age, and even climate has been shown to be a factor, with symptoms becoming the worst during winter months and in colder, wetter climates.

Finally, after six long weeks during which Angeleah showed no improvement and the Gravedonis found no answers, Rhonda and Angeleah went to the Marshfield Clinic in Marshfield, Wis., where Dr. Hema Murali recognized the symptoms of POTS almost immediately.

“I took her to Marshfield on my own, because I knew something was going on with her,” Rhonda said. “And it just so happened that we were fortunate, that (Murali) had trained under the founding father of POTS. So she knew, she took one look at her and she said to me, she said, ‘Mrs. Gravedoni, I need to commend you.’ She said, ‘I’m pretty confident I know what your daughter has. One simple test will tell me.’ And within 30 minutes we knew.”

POTS is just one of many autonomic disorders – illnesses that affect the nervous system’s control of unconscious functions such as heart rate, digestion, breathing, perspiration and pupil dilation – and can be extremely unpredictible. In the case of her daughter and others with POTS, Rhonda said Angeleah’s blood vessels are “elongated and floppy.”

“The blood is sent, but because of the floppiness, the laziness of her vascular cells, it doesn’t stay there,” Rhonda said. “It seeps out and it pools in other areas. So her blood pressure can go from – resting, it’s really high, to standing up and it plummets.”

Angeleah was put on a regimen of medicines that help her to function more or less normally. A small dose of a steroid helps her retain salt, thereby increasing blood pressure and volume; a vasoconstrictor called Midodrine also increases blood pressure, allowing more blood to return to her upper body; the anti-depressant sertraline (commonly sold as Zoloft) helps both to regulate aspects of her nervous system and to treat the depression that accompanies the disorder (though doctors don’t know whether depression is a symptom of the disorder in its own right, or an effect of the drastic lifestyle change); a whole spectrum of natural vitamins and minerals help keep her as healthy as possible; and strict orders to drink between 90 and 120 ounces of water each day ensure that Angeleah’s blood volumes stay high.

Though there was relief in finally understanding what was happening, Angeleah’s struggles were far from over. Because while POTS is a very manageable illness, there was still no specialist in Marquette County with enough knowledge of POTS to treat it. So when her symptoms were at their worst, and Angeleah was nauseous or struck with a headache or struggling to breathe, for about the first three years of her illness, the Gravedonis had no recourse but to take their daughter to the emergency room. This was problematic, Rhonda said, because the ER physicians continued not to know how to help Angeleah; consequently, they deferred to the Gravedonis themselves for instructions on how to treat her.

In order to help local doctors give her daughter the care she needed, Rhonda began compiling all the information on POTS that she could find, as well as Angeleah’s entire personal history with the disorder. The resulting binder, some eight inches thick, would come to be called Rhonda’s “bible” by Dr. Jennifer Atkins, a pediatric specialist in Marquette who met with the Gravedonis last year and agreed to familiarize herself enough with the girl’s condition to act as a liaison between the family in Negaunee and Angeleah’s team of doctors in Marshfield.

It wasn’t just Angeleah’s physical health that suffered. So did her ability to attend school and maintain a social life. To this day, Angeleah says the limitations of her condition prevent her from having friends and participating in the social activities of a normal teen.

“When I got diagnosed, my life completely changed,” she said. “I lost all my friends. I can’t really do anything anymore, even racing – it’s a struggle. So it really changed my whole life.”

Angeleah’s illness even forced her to be homeschooled for a year, though she has since returned to attending NHS at least part of the day, taking her other classes online. But Angeleah was only able to come to this arrangement after Rhonda, during a brief battle with the school – which also had never heard of POTS, saying that the illness didn’t qualify Angeleah as a disabled student – met with a special education advocate and consulted federal law to have her daughter’s disorder recognized by the school as a valid disability and consequently provide her with the necessary accomodations.

One thing that Angeleah hasn’t given up is racing motocross, despite her parents’ continuing anxiety over the hobby, and repeated episodes where she lost consciousness while racing and on several occasions was even thrown from her bike.

“If I race and it’s really hot, I can do a lap or two and then I’ll be going over jumps and my head will be bobbing,” she said. “And hit one wrong bump and I’m over the (handle)bars and can’t move. My dad (has) had to carry me off the track.”

“I need people to understand that we don’t want her to do that, but as parents, that’s her love,” Rhonda said. “She loves it, she’s passionate about it, it’s the only thing she does. I hate that she does it … it’s very dangerous. Very, very dangerous.”

Rhonda hopes that by shining a light on her daughter’s disorder, she can spare others diagnosed with the illness from enduring the same struggles for proper medical assistance, special health accomodations, understanding and empathy that Angeleah has continued to go through for the past four years.

“I’m just hoping that getting the word out there,” Rhonda said. “I know what we and what she has gone through – half the battle is just trying to get an answer. Once you know what you’re dealing with, you deal with it.”

She said Angeleah’s resilience in going through so much at such a young age speaks to the strength of her character.

“She’s an amazing young lady,” Rhonda said. “How she endures and carries herself as she does, she amazes me. She really does.”

Zach Jay can be reached at 906-486-4401.