‘This is going to kill me’: Rockton man with rare genetic disorder in need of 2 live donors – Rockford Register Star

Doctors tell Joe Beard his only shot at survival is a two-organ, bone marrow transplant

ROCKTON This time of year almost everyone has a wish list.

Joe Beardof Rockton has a short but weighty list of needs.

The 40-year-old husband and father of twoneedsa new kidney, a new liver and a bone marrow transplant.

Without them, his chances of living long enoughto see his daughters graduate from high school get slimmer and slimmer each day.

In 2018, Beard was diagnosed witha rare genetic disorder of the immune system called STAT3 gain of function.

An article that appeared in theAmerican Journal of Respiratory and Critical Care Medicine the same year that Beard was diagnosed reported only 28 known cases.

"I have an autoimmune disease that's just running wild, attacking my organs, my body, everything," Beard said.

"Eventually, this is going to kill me. … I don't know if it is six months from now or two years from now, but it's not long."

Seated in the living room of his Rockton home with his wife, Hayley, and daughters Elsie, 6, and Molly, 4, Beard said it was six years ago when the disease first manifested.

"I wasn't feeling well," he said. "I started coughing and losing weight."

Over a three- to four-year period, Beard lost 50 pounds, a lot of weight for anyone of any stature, but even more so for someone who is5-foot-8 andinitially weighed 175 pounds.

At the outset, doctors suspected the fitness buffhad a lung infection and prescribed treatment for such, but the illness persisted.

Eventually, the couple sought care from an immunologist at the University of Wisconsin Hospital in Madison.

"On a whim, she decided to test for genetic diseases," Beard said, "andlow and behold one came back positive for STAT3 gain of function."

STAT3 GOF is a rare genetic disorder of the immune system. The disease is named after the gene, STAT3, and the effect caused by mutations in STAT3 gain of function, meaning the genes protein becomes overactive.

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The Beards were referred to the National Institute of Health in Bethesda, Maryland, where the couple visited on a monthly basisfor nearly two years.

"They put me on these experimental drugs to try to control the inflammation," Beard said, "and it seemed to help, but my kidney function continued to decline."

Beard started dialysis about 2 1/2 years ago, about the same time his liver began to fail.

"So now, I am in end-stage renal disease, ESRD, and liver failure," he said."I essentially need a liver and kidney transplant," two vital organs that by themselves won't be enough to save Beard's life because, according to doctors,Beard's immune system will still continue to attack his body.

"So, they said, 'Really what we need to do is give you a new immune system, too,'" Beard said. "So that's where the bone marrow transplant comes in."

A double-organ transplant followed by a bone marrow transplant is a rare procedure, andmultiple hospitals declined to perform the surgeriesbefore theUniversity of Pittsburg Medical Center, one the country'sleading hospitals in performing organ transplants, accepted the case.

Beard was evaluated in January andinformed in March that he was not a candidate for a traditional cadaver-donor transplant because he neededtwo organs and lives more than 500 miles away from the hospital.

"But then they said, 'You could do living donors. If you can find someone to donate half their liver and then another person to donate a kidney and some bone marrow, we'll do it.'"

The Beards'excitement over the news was tempered by their insurance provider. Theywere told the combinations of procedures was "experimental" and would not be covered.

After seven months of filing appeals, submitting letters from doctors and threats of lawsuits, adoctor independent of the Beards and their insurance provider reviewed the case and determined thedouble-organ and bone marrow transplants wasBeard's best option.

"Based on that and per our current laws, they (the insurance provider) had to change their opinion to match the external reviewer," Beard said. "So, we got approved."

Hayley explained not one, but two donors are needed, one to provide the liver, the other to provide a kidney and bone marrow.

Kidney donation is the most common type of living-donor transplant. Individuals can donate one of their two kidneys, and the remaining kidney is able to perform the necessary functions.

As for liver donors, only a portion of the liver is removed and in a matter of months, the donor's liverwill regenerate and perform as normal.

"They'll take up 60 percent of your liver and within three months it will grow back up to 98 percent," Hayley said. "They just said it won't look the same."

Beard said his first choice for donors were his three siblings, all of whom were tested and were found to have the same genetic disorder, which was passed down to them from their father.

Of the four, Beard is the sickest.

"My full sister has no health problems whatsoever, and my other two half siblings have minor health problems, and my dad's been fine."

For the time being, Beard receives weekly infusions of donor antibodies and four days a week he undergoes home dialysis.

He described what is atbest a love-hate relationship with hisdialysis machine, a wonderful piece of technologythat removes waste from his blood and keeps him alive and its a ball and chain that zaps him of his energy and has to go with him when traveling.

"I feel like a bad father sometimes," he said, "because I can't play with my girls."

One of the joys of fatherhoodis teaching achild learn to ridea bike, an undertaking that Beard has had to relinquishto Hayley.

And yet, the dialysis machine has createda special bond betweenBeard and his girls.

Elsie is tasked with entering her father's blood pressure numbers from the dialysis machineinto an app on the family's iPad. Molly pushesthe save button before the information is forwarded to the DaVita Roxbury Dialysis Center wherea dialysis nurse remotely monitor's Beard's health.

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Because of his compromised immune system, Beard doesn't stray far away from home other than to go to work at Taylor Co., a Rockton-based manufacturer of commercial food service equipment.

Beard,an electrical engineer, called the company's management team "gracious" and "very accommodating" as they haveprovided him with his own office and allowhim to work from home as his health dictates.

"People ask me, 'How can you still work? I don't know. I guess I'm just trying to keep things as normal as possible and to provide for, you know, the kids and my wife.

"I told my boss, 'This is a good release for me because it makes me forget about things. For a moment, I'm normal.'"

The Beards said it is their faith that holds them up each day and are blessedto have the support of family and friends who pitch in on a moments notice to care for their daughters when needed.

Still, the family is concerned about two looming deadlines.

The first is Jan. 1 when Taylor Co. changes its insurance provider. TheBeards fear they may have to endure another lengthy and contentious processto convince the new insurer to cover thesurgeries.

The other deadline is more murkyand more daunting.

"They want to do the transplants as soon as possible because, you know, I am declining every day. And there's going to come a point when I'll become too ill to receive a transplant. So, they want to do this while I'm still healthy and have enough energy to get through the surgeries."

Chris Green: cgreen@rrstar.com; @chrisfgreen

Register to be a potential donor atlivingdonorreg.upmc.com. TypeJoseph Beard in the Donation to spot.

To help the family with travel and expenses, donations can be sent to:

Roscoe United Methodist Church

C/O Joseph Beard Transplant Fund

10816 Main St.,

Roscoe, IL 61073

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'This is going to kill me': Rockton man with rare genetic disorder in need of 2 live donors - Rockford Register Star