Kelli and Len McHugh have been busy this summer planning their third Walk for PKD, to be held Sunday, Sept.17 in St. Joseph, MI. The McHughs are excited to participate in something that will hopefully benefit their daughter, Jenna, and others with PKD.

The McHughs had never even heard of polycystic kidney disease or thought of holding such a major fund raiser before Jenna was born. But everything changed after she was diagnosed with the recessive form of PKD (ARPKD). Now Len and Kelli want to do everything possible to help find a treatment and cure for PKD and give Jenna the best possible opportunity in life.

During Kelli’s pregnancy, doctors monitored her closely for toxoplasmosis and other possible complications. Although she had multiple ultrasounds, they never revealed anything unusual, and nobody seemed worried about the baby’s health.

Jenna’s birth was an exciting, special event for the family (Jenna is their first child), and she initially appeared to be a typical, healthy baby. But, soon after her birth, Kelli’s intuition caused her to suspect that something wasn’t right.

Although Jenna was a bit tiny, that wasn’t the reason. Kelli is a nurse and, while she couldn’t point to anything specific, told Jenna’s pediatrician that she thought something was wrong.

Although the doctor was attentive to Kelli’s concerns, it was only when Jenna was 4 months old that he began to notice that her kidneys, spleen and liver were enlarged.

Jenna was sent to a hospital in Kalamazoo for weeklong, intensive testing, including a bone-marrow biopsy to rule out cancer. Doctors also suspected a possible third-world disease. The tests finally led to the diagnosis of ARPKD and congenital hepatic fibrosis.

Jenna, who will turn 5 on October 22, is currently fine, with normal kidney function and stable blood pressure, but she has encountered many medical bumps along the road. The disease has affected her liver more than her kidneys so far, and she has a lump on her liver that is being monitored. Jenna’s portal vein is totally occluded, causing circulation problems. She has enlarged kidneys and varices (bulging blood vessels) on her esophagus that could possibly rupture and lead to an emergency. Jenna’s blood pressure and blood levels are frequently monitored, and she takes blood pressure medication as prevention. Her large organs have also caused occasional back pain.

Her future can’t be predicted, and it is all more than any little girl normally has to handle.

Despite everything, Jenna is a trouper and a wonderful child. She likes to draw, loves music and enjoys playing with dolls. Jenna understands that she has a "kidney disease in the liver" and that she has a lot of doctor visits and tests. She knows that she takes medicine to help her liver get better.

On a daily basis, though, her life is not terribly affected. Jenna’s activities are a bit restricted no roughhousing allowed and she knows that she can’t eat a lot of chocolate and must stay away from other foods, especially those with caffeine. However, none of this seems to bother her.

The effect on Kelli and Len has been more profound. During Jenna’s testing and after the diagnosis, "we didn’t know if Jenna was going to live or die," Kelli said.

The McHughs felt anger and guilt and wondered if it would be fair to have more children. It has also been tough to deal with the questions people ask about Jenna’s condition.

Because she is a nurse, Kelli has sometimes felt like her own worst enemy having a much better understanding of her daughter’s illness. During the worst of times, she talked to her doctor and was prescribed anti-anxiety medication to cope and handle the stress.

"This is something you normally don’t share with the world, but I want everyone to know that it’s important to take care of yourself," Kelli said. "If I can’t handle my emotions and be strong for myself, how can I help and be supportive to my daughter? Jenna is smart, and I want her to have hope and a positive attitude. If she can accept what PKD is, hopefully, in the future, if it gets bumpy, she will be better able to cope."

Kelli and Len feel much better these days. Soon after Jenna’s diagnosis, they attended their first Annual Conference on PKD and met other families in similar situations. They were very inspired after meeting a man at the conference with ARPKD. He had survived into adulthood, which was so encouraging. After the conference, they felt less lonely and isolated and began talking to others about the disease. Kelli did have one miscarriage after Jenna was born. Then Caitlyn, their second child, was born in the summer of 2005 and, to date, has shown no signs of having PKD.

"I don’t know what we would do if we didn’t have our family, friends, faith, co-workers and church to support us," Kelli said.

Len feels very fortunate that Jenna is doing so well, especially knowing that other families have faced much worse. Kelli has seen Len as her backbone always optimistic that Jenna will be okay.

Kelli and Len encourage other parents to look forward, hope for the best, take care of themselves and enjoy life as much as possible. They have included Jenna in planning for the Walk for PKD and look forward to a great event.

As Jenna says, " It is a lot of fun and helping my liver."

To register or learn more about the Walk for PKD, visit www.pkdcure.org. To contact Len and Kelli McHugh, email kmpkdwalk@comcast.net