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Isle woman struggles with rare illness


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POSTED: Thursday, March 26, 2009

When his wife was diagnosed with calciphylaxis, Darren Araki said, he found out it is a rare disease.

“;Doctors, a lot of them, haven't even seen a case,”; he said. “;A lot of them have heard about it, but all are saying it's difficult to treat.”;

“;It's pretty unusual,”; said Dr. James Wong, a Straub Clinic & Hospital blood vessel surgeon who diagnosed Araki's wife, Janell, about 1 1/2 months ago. “;It's kind of out of my league, but I am trying to help them. ... What they need is beyond surgical care.”;

Calciphylaxis was known previously as calcific uremic arteriopathy, Wong said. It is a kidney-related disease in which calcium deposits build up in small blood vessels in the skin, causing painful skin ulcers, serious infections and organ failure.

Janell Araki, 38, owns Precision Sound, a Honolulu company that provides sound systems for concerts. The Kaneohe couple has a 14-year-old daughter, Jayna.

Araki, 39, said his wife has had type 1 diabetes since age 13. “;After so many years, it takes a toll on the body and kidneys.”; Her kidneys failed Thanksgiving week, and she was put on dialysis three times a week, he said. “;It happened pretty fast.”;

She got a cold and could not shake it, he said. “;She had water in her lungs, and they did tests and found her kidneys weren't working properly. Fluid was building around the heart, and she had difficulty breathing.”;

She was anemic and collapsed at home with low blood sugar and low blood pressure, Araki said, explaining she spent the last week of 2008 in Castle Medical Center's intensive care unit.

She was adjusting to a kidney dialysis schedule and getting better after leaving the hospital on New Year's Eve, then a water blister appeared on her leg and turned gangrenous, he said.

She was hospitalized for about a week at Straub after Wong did a biopsy and discovered she had calciphylaxis.

“;It is not a very friendly disease,”; he said. “;Many patients will expire from this.”;

Wong said it is usually seen in patients with kidney failure on dialysis but also occurs occasionally in patients with autoimmune disorders such as rheumatoid arthritis, though that is less common. It affects anywhere from 1 to 4 percent of patients on dialysis, he said.

The disease was first reported about 20 or 30 years ago, he said. Some papers have reported the incidence at 1 to 2 percent, but “;it's actually increasing,”; Wong said. “;It is not well established, and there is no standardized or proven treatment program.”;

Some doctors recommend cleaning out the ulcers, and others recommend against that, he said.

“;Surgically, you can't fix the problem because you can't fix blood vessels when they're calcified. They shut down.”;

A nephrologist, infectious disease specialist and internist are working with Wong in a multifaceted approach.

She still has a big wound on her leg that has not healed and two more on the same leg, Araki said. “;They just pop up for some reason. Any type of trauma, scratching or wound, can turn into that.”;

She is supervised at home to keep her from getting trauma to her legs or any part of her body, he said. “;Every day is an uphill battle for her,”; said Araki, who is continuing his wife's business. “;She's a trouper. She wants to go back to work.”;

She is usually always working at concerts because of her business, saying the only group she would want to see is Journey, he said. So she was given a night out yesterday to see the group in concert at the Blaisdell Arena.

Wong said a couple of drugs are used to help decrease the amount of calcium in the body for calciphylaxis patients. Hyperbaric oxygen is another option but is not proven and could run $25,000 for a course of treatments, he said.

He said he wants to put Janell on sodium thiosulfate, given intravenously by a nurse, but it costs about $800 a month. The injections helped one patient who was on it for a year, he said.

Araki said the prescription allowance on their HMAA plan ran out in three months because of all the medications. “;I've got to pay on my own. Insurance companies don't even want to hear my story.”;

The Arakis also received a letter recently from the insurance company saying they want to re-evaluate her eligibility, he said. “;It's another stress we have to deal with.”;

He said, “;Doctors are going out of their way (to help), but their hands are tied. They can't get medication.

“;You never think it's going to happen, and when it does, your whole life changes. Things I thought were very important to me are now on the back burner. I want to get my wife better. Every day is a battle.”;

 

Isle performers slated for benefit show

TV personality Tiny Tadani and other entertainers are organizing a fundraiser for Janell Araki from 2 to 10 p.m. May 3 at Waterfront (formerly Kapono's) at Aloha Tower Marketplace.

Groups performing will include Kapena, Na Leo, Kupaoa, Maunalua, Weldon Kekauoha and Vaihi and others. The American Diabetes Association and Straub Clinic & Hospital also will participate.

“;It is becoming really a humanitarian effort,”; said Janell's husband, Darren. “;We wanted to be able to tell people what she is going through ... to bring awareness to diabetes, kidney failure and this disease.”;

Tickets are $10. They can be purchased at the door or by contacting Darren Araki at 479-0606. Donations also can be made by sending checks to Friends and Family of Janell Araki, P.O. Box 6477, Kaneohe 96744; attention: Janell Araki.