Kira

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Kira was born at 00:31 on June 6, 2003. She weighed a healthy 7lbs 9oz and was 21.75 inches long. Everyone who saw her commented on her full head of black hair and fair complexion. Big brother Lucas, 2 years old, wasn’t too interested in the new addition to the family, but was always gentle and loving when he held or played with his sister. Paul and I (Jen) were very proud parents.

Kira’s first 5 months of life were marked by endless crying. Paul and I tried to chalk it up to colic, but I think in our hearts we knew something was not right. At 3 months, we had expected Kira’s ‘colic’ to lessen and when it hadn’t we took her to our GP. He examined Kira, noted her poor muscle strength, and witnessed her irritability, and referred us immediately to a pediatrician who we met with that afternoon. He told us he thought Kira had a problem with her brain.

Over the next months, Kira had numerous tests. She had routine genetic screens and other blood work, VEP (showing she was blind), ABR (showing she had a hearing impairment), CT Scans (normal), EEG (very abnormal brain waves) and MRIs (delayed mylenation). On Halloween 2003, Kira was hospitalized for infantile spasms (seizures) and we began treating them with different anti-seizure drugs. One was a steroid, that had a number of side effects, most concerning to us was a suppressed immune system. This meant Kira would be more susceptible to colds and infection. At the time, Paul and I were most worried that another cold or infection would make Kira even more irritable. We had no idea that each cold or infection could speed the progression of Kira’s disease.

On November 28 2003, we took Kira to the Emergency Room because she was having difficulty breathing. The Emergency Room doctor believed it was early onset of croup and sent us home. The following day we returned to hospital by ambulance because Kira was in distress. We were admitted to the hospital for observation, and once Kira was on a monitor they realized her heart was beating at 47 (regular infant heart beat at rest should not go below 80). We were rushed to ICU where Kira stayed for the next 12 days. She was no longer able to suck or swallow and a tube was placed through her nose into her intestine to feed her. Her respirations were very low, stopping at times, and her heart continued to beat slowly. It appeared that her body was shutting down. A second MRI was conducted and showed a very significant change from the ‘normal’ one 6 weeks earlier. Kira’s brain stem had deteriorated significantly. The brain stem controls breathing, heartbeat, and all major organs, and Kira’s had been severely damaged.

This is the basis for her Leigh’s Disease diagnosis. Leigh’s Disease falls into the category of Mitochondrial diseases. There is still much progress to be made in the awareness and research of Mitochondrial Disease. Doctors do know that Mitochondrial Diseases result from the body’s inability to produce energy at the cell level. The organs that require the most energy, are most severely impacted and damaged. This disease is degenerative, or ongoing, and is accelerated whenever there are additional demands on the body for energy production, such as a cold or infection.

Although doctors did not want to speculate on life expectancy, they felt Kira had weeks, not months to live. We began on the Ketogenic diet and on a Mitochondrial ‘cocktail’ of vitamins. There was a chance that these would help with Kira’s metabolism and we were willing to try anything that held any hope of extending and improving Kira’s quality of life.

In the New Year 2004, we decided to begin treating Kira’s comfort, not her disease. The vitamins and diet were not improving her symptoms, so we took her off them without incident. After almost 2 months in hospital, we decided to bring Kira home and try to meet her needs where we would all be most comfortable. We established a new family routine. We had a contingent of health care workers to assist us. Community Support Workers and LPNs came in during the night to care and watch over Kira. Our nanny, May, assisted in caring for Lucas and Kira during the weekdays. Our palliative care doctors and RN came for regular visits. We enjoyed having our family together and at home.

The doctors felt that Kira did not have much time left, but she continued to fight. In May 2004, we decided that Kira would undergo surgery to place a feeding tube directly into her stomach. This was a fairly straight forward surgery for other kids, but for Kira we had delayed it as long as possible. Kira did not like having the feeding tube taped to her face, and every now and then she was able to get her fingers underneath and pull it out. This meant a trip to see the radiologist at the ER and possibly an admission to the hospital. The surgery was a success and Kira handled it very well. We are so glad we had it done, as it made everyone’s life much easier.

In June we celebrated Kira’s 1st Birthday and it was a fantastic party! It was a day that many of us did not believe would happen. What better way to celebrate than with a fundraiser. The 1st Annual Kira’s Day raised over $50,000 for the Alberta Children’s Hospital.

In July we decided to repeat Kira’s MRI and the only thing it confirmed was that our precious little girl was still a mystery. The lesions on her brain, (the basis of her diagnosis) had resolved. After further consultation and research, the doctors found evidence that these lesions on the upper brain stem could sometimes resolve, and felt that Leigh’s Disease was still the best diagnosis.

Although we had a number of appointments with specialists throughout the following months, it was at this point that we accepted that Kira’s health issues would remain a mystery. The appointments were more routine and we did not feel they would produce any answers.

In the fall 2004, the doctors warned us that the winter season would be difficult for Kira, and they were right. Kira battled numerous colds and viruses, and each one took its toll on her immunity and strength. It seemed that we moved from one crisis to the next in October and November.

We had some great family time over Christmas. Kira had a cold before Christmas but seemed to recover on Christmas Eve and we were able celebrate the holiday with everyone in good health and spirits. We were so pleased to be able to include Kira in our New Years Eve pary with some close friends. As the holiday came to a close and Paul was going to work for the first time in almost 3 weeks, Kira had other plans. On January 4, 2005, we were awaken early in the morning by our night time nurse who believed Kira had taken her last breath. As we stood over Kira, I picked her up and Kira began breathing again. Kira continued to struggle breathing and by early afternoon our girl took her last breath with her mother and father holding her. Kira was determined to choose her time and place and gave us a wonderful Christmas with her as her last gift.