Presley’s Story

JA HONOREE 2020 WALK TO CURE ARTHRITIS

In April of 2018, Presley began to show signs of illness. Her family assumed it was a virus or common cold, as she ran a low-grade fever for several days.

 After a few trips to her physician and some antibiotics, she was not showing signs of improvement. The fevers continued, as well as signs of pain which were mistaken for a UTI or viral infection. Presley turned 2 on May 3rd of 2018 and was not her usual outgoing self at her birthday party on May 5th. The once active and outgoing little lady chose instead to be cuddled and held during most of her party. At this time, her parents, Hope and Quentin, decided to get a second opinion. 

She traveled to Lincoln where a blood draw was completed, and labs ran once a week for 3 weeks. Upon receiving some concerning lab results at the end of May, her pediatrician asked Hope to take Presley to Children's Hospital immediately. 

Presley left Lincoln right away to go to Children's Hospital in Omaha.  More tests were completed, along with blood draws and questions from several teams of physicians at Children's. Around 10pm that evening, it was determined that Presley had a septic hip. That meant she had an infection (caused by unknown circumstances but normal in children of her age) in her hip that was causing fluid to build up around the joint. 

Hope and Quentin were asked for permission to complete a quick surgical procedure to remove the fluid and flush the hip right away. Consent was given and the procedure completed. Shortly after midnight, the surgeon came to the waiting room to explain that the infection had been found in the other hip and the same procedure was to be completed while she was under anesthesia. At 2:30am, the procedure was completed, and her parents were able to be reunited with her back in her room in the early hours of the morning.

She had shunts in both hips to drain the fluid, but she was very happy when she woke up, eating again and enjoying the children's play area at the hospital. After 3 days, Presley was released to go home. However, the symptoms returned after a few short weeks. Hope and Quentin continued to take Presley to multiple doctor appointments, demanding answers and working with the team at Children's Hospital. Hope, and Quentin were able to get a consult with a rheumatologist at Children's. It was then that it was determined that there was a possibility that Presley had juvenile idiopathic arthritis. Though the diagnosis was very difficult for them to hear, it was amazing to finally have an answer for what had been causing their baby girl to be ill for many months. 

Hope and Quentin took Presley to Kansas City for an additional consult and second opinion with the rheumatologist there. The same diagnosis was provided, and it was determined that Presley would begin a round of medications to alleviate her symptoms as well as build up her compromised immune system. 

So, what is Juvenile Idiopathic Arthritis? 

Nearly 300,000 children – from infants to teenagers – in the U.S. have some form of arthritis. Juvenile idiopathic arthritis (JIA) is the most common type of arthritis in children. The term idiopathic means “of unknown origin.” JIA was previously called juvenile rheumatoid arthritis, or JRA. JIA is not contagious.

There are six JIA subtypes. Systemic JIA is considered an auto-inflammatory disease. The other types are considered autoimmune diseases. A healthy immune system fights invader, such as viruses and bacteria. In people with autoimmune or inflammatory diseases, the immune system does not work properly. It becomes overactive even when there is no infection to fight, or it mistakenly attacks healthy cells and tissues.

Systemic JIA causes inflammation in one or more joints and is often accompanied by a high spiking fever (103°F or higher) that lasts at least 2 weeks and a skin rash. Other possible signs include inflammation of the heart or lungs or their outer linings; anemia; or enlarged lymph nodes, liver, or spleen. About 10 percent of children with JIA will have the systemic form

Presley has the rarest form of this disease. Yet, she continues to not give in to her pain and symptoms by staying her typically active and fun-loving self. Presley has had a few setbacks in recent weeks. Because JIA weakens her immune system, she is more susceptible to illness and has had the stomach flu and a few viral infections. Also, when weaned off her steroids (which assist with building up her immune system), the JIA symptoms have returned and resulted in flare ups. This means that Presley begins to have discomfort, pain, swelling and fevers. It can get to the point that Presley struggles to walk or cannot kneel/sit on the floor to play. She attends physical therapy to assist in building her muscle around the joints and alleviating any stiffness near her joints. She also has been taking pain medication, steroids, and monthly injections to assist with alleviating the symptoms of JIA. 

Unfortunately, the medications have not been as effective as hoped, and she is now switching to a daily injection with the hope that it will put her disease into remission. Presley is TERRIFIED of needles and the poor girl has been poked more times than many adults. Her parents have diligently worked to provide support,  prizes and rewards for each blood draw, but now she will also endure daily injections that are known to burn and causes "flu like symptoms" for a few days after the injection. 

Hope and Quentin participate in the Walk to Cure Arthritis to hopefully support finding a cure for Presley and all others suffering from this terrible disease.