Wednesday, July 06, 2005

Kasson five-year-old gets her wish from Wishes & More Foundation

By Ginger Kundert

	Five-year-old Brynn Kundert is pictured by a Kasson fire truck. Kasson volunteer firemen helped make her wish come true at the Kundert family’s home in Kasson.

When you get to know five-year-old Brynn Kundert of Kasson, some of the things you’ll learn about her are that she loves Oreo cookies, fried chicken, mashed potatoes & gravy. One of her favorite books is The Happy Hedgehog, and she likes to sit outside at night watching the stars. She has beautiful hazel eyes and an infectious laugh. And, she can be a bit on the bossy side when it comes to her two-year-old sister, Jadyn, yet very compassionate with her nine-month-old sister, Camryn. She is a blessing to everyone who knows her and a symbol of determination. From looking at her, she looks like a normal five-year-old girl. What most people don’t know is that Brynn has a potentially life threatening Mitochondrial disorder called Complex 1 Deficiency in addition to Hydrocephalus & Chiari Malformation. She’s already gone through more than some of us will face in a lifetime.

When Brynn was 10 1/2 months old, she was diagnosed with Hydrocephalus. She had surgery to have a shunt placed. Six weeks later she went through another surgery for a double hernia.

Hydrocephalus is a condition in which excess fluid builds up in your brain causing abnormal enlargement of the cavities in the brain (ventricles) that contain CSF. The fluid that accumulates is cerebrospinal fluid (CSF), a fluid that normally surrounds your brain and spinal cord. Too much CSF in the ventricles can put too much pressure on the brain, potentially damaging the brain.

In 2004, Brynn was hospitalized at St. Mary’s Hospital in Rochester for five weeks from early February until mid March. It was a long road with many ups & downs. As Brynn’s condition continued to deteriorate to a semi-comatose state, she underwent surgery to revise her shunt. When doctors realized this was not the ‘fix’, she continued to go through a regimen of tests in an attempt to determine what was happening to her. Brynn lost all abilities, including her eyesight, for approximately 2-1/2 weeks. She was only able to chew, swallow and drink through a straw. Amazingly, she started to regain her abilities one day when she sat independently for a minute or two and also giggled. Within a few days she was taking some steps and starting to talk once again. Her sight eventually returned. What a wonderful feeling that was for all! One doctor called her a miracle. Shortly after Brynn was discharged, the test results came back & this is when she was diagnosed with a Mitochondrial disorder. This is a genetic disorder, which affects how the cells in the body produce energy. To date, a cure has not been found. The treatment is frequent feedings and a ‘metabolic cocktail’ that consists of nine different vitamin co-factors that Brynn takes three times a day - 17 doses daily. This is medication she will have to take for the rest of her life. Brynn has come a long way with taking her medication, from crushing & mixing them, accompanied by a few tears to taking them independently & being very proud of herself for doing so.

Mitochondrial diseases result from failures of the mitochondria, specialized compartments present in every cell of the body except red blood cells. Mitochondria are responsible for creating more than 90% of the energy needed by the body to sustain life and support growth. When they fail, less and less energy is generated within the cell. Cell injury and even cell death follow. If this process is repeated throughout the body, whole systems begin to fail, and the life of the person in whom this is happening is severely compromised. The disease primarily affects children, but adult onset is becoming more and more common.

We have learned & continue to learn what we need to do to keep Brynn healthy & hopefully try to prevent a severe episode of decompensation from occurring again. Brynn has made a great recovery from where she was a year and a half ago. She was in St. Mary’s Hospital on the pediatric floor for 3-1/2 weeks and then in the Rehabilitation Unit for a week and a half. She went through outpatient therapy for an additional two weeks upon returning home. This past fall, she attended pre-school in Kasson. Brynn continues to have a few challenges from time to time, but overall is doing well. Through additional testing, it was also noted that Brynn has a Chiari Malformation.

Chiari malformation is a rare abnormality at the base of the brain that results in brain tissue extending into the spinal canal. In Chiari-I malformation, brain tissue protrudes below the opening of the base of the skull. It may also include displacement of the lower part of the brain.

Since Complex 1 deficiency is a genetic disorder, Brynn’s younger sisters were tested as well. Jadyn also has the same genetic disorder as Brynn, though not as severe, and baby Camryn has tested negative for the disorder.

Brynn was granted a wish by Wishes & More, a wish-granting organization. She asked for a play set for her backyard. With donations of time, effort & supplies, volunteers from Wishes & More, the Kasson Volunteer Fire Department, Steve Morris Concrete, Erdman’s County Market, family and friends made this wish of Brynn’s come true. The firefighters from the Kasson Volunteer Fire Department, volunteers from Wishes and More, family and friends were there to assist in the assembly of the play set.

www.wishesnmore.org

Wishes & More™ enhances the life of a child fighting a terminal or life-threatening condition by providing extraordinary experiences... and more...

www.umdf.org

United Mitochondrial Disease Foundation.