Fighting a Rare Disease

By Mari Radtke
Kenzi Villarreal is a familiar face at Primghar’s ProGo Convenience Store. She has worked there for ten years and managed the store for seven of them. Kenzi speaks very highly of her employer, saying, “They are a great employer. They’ve been with me through the journey of my health and family.” Kenzi’s first health need came when she was just 22 years old. She suffered a massive horseshoe-shaped blood clot in her chest known as a “killer clot.” It was blocking the entrance to both of her lungs. That cause happened in 2011 and caused her to have her first open heart surgery. That surgery lasted 9 1 / 2 hours. “I was kept alive by a machine. My body was cooled to stop all blood flow. Then it was warmed and blood circulated. Then I was cooled. I was on life support this whole time.” She was 22 with 2 young children. The major risks from this surgery includes neuro damage or even death.
Six months later she was diagnosed with Chronic Thromboembolic pulmonary hypertension. CTEPH for short, is a rare disease suffered by a very small percentage of people who have endured lung blood clots. The condition causes high blood pressure in the small blood vessels of lungs or pulmonary hypertension. Blood clots in the lungs cause the high blood pressure according to Cleveland Clinic. The blood clots cause scar tissue in the small blood vessels creating a host of symptoms including shortness of breath, dizziness and fainting to name just a few. “I’ll be on blood thinners forever,” Kenzi stated. There are some new drugs coming onto the market to aid treating CTEPH, lessening the pressures on the heart and lungs, but currently there is no cure. Six years ago this disease was mostly unknown and often misdiagnosed. She and a few others were flown to New Jersey to meet with officials from drugmaker Bayer Company. They wanted first hand information to help them understand the effects of both the disease and the medications. “It was cool to meet the people who make the drug. They didn’t know 5 or 6 years ago how it would work. The life expectancy now is unknown. Then it was 5 years.”

There is an angioplasty treatment for some. It consists of 4 balloon insertions to open blocked or narrowed blood flow pathways. “I’ve had two angioplasties. The left upper lobe of my left lung is completely blocked. A balloon is not strong enough to break the blockage.” These treatments are usually four treatments.
Kenzi is facing her second open heart surgery in August. A new CTEPH treatment facility has opened in Chicago. San Diego has the only other hospital for CTEPH surgery in the U. S. She is leaving for Chicago in two weeks for an initial round of tests. If the pressures in her lungs are too high they will not operate. She will have a series of scans and lung function tests. She will meet the surgeon and his entire team. If all goes well her surgery will be in the middle or end of August. Her ten year old daughter will stay with her mother who lives in Primghar. Kenzi’s teenage daughters will be with their aunt who lives a block away.
Kenzi expects a similar experience to the first open heart surgery. It will last 9-12 hours. This time they will try to remove all old remnants of the disease that is reachable, clots, scar tissue any other blockages. She is really hoping they can unblock the left lobe ñ hoping, she emphasized. If that works the lung will be opened and reduce pressure on her lungs and heart. It will increase her lung capacity and improve the heart’s ability to distribute blood throughout her body. One side effect of the surgery is an enlarged heart. It happened the first time. It does return to normal size. The heart has a single muscle that cannot be replaced and is necessary for proper functioning of a heart. It cannot be regained. It must be protected.
Kenzi expects her stay in Chicago to be 10-14 days after surgery, depending on her recovery. “I’m hoping for that. I’m hoping I am as strong as I was at 22,” said Kenzi. She knows she will have severe lifting restrictions. She is looking to return to light duty work after 30 days, but her medical team wants 12 weeks. “I believe you do what you can do for the best recovery, not sit on a couch.” she said.

Blood thinners and heart troubles are known to cause swelling in the legs. That swelling weakens skin. That has been an experience Kenzi really never wants to experience again. She got a burn on her leg. That led to cellulitis. “That was a pain I wish on nobody!” She was out of work for 6 weeks. The swelling slowed the healing. It was a viscious circle. It was the worst fight with cellulitis she has had, but not the only. She says her bucket list includes a fun adventure, “How many illnesses can I defeat.” She has a very strong will. Kenzi says stubborn.
A gofundme page is set up for Kenzi to help defray some costs, and there is an account at Savings Bank Primghar for anyone able to assist the family. We’ll do our best to keep you updated.

