Kenzie's Court

Makenzie, our beautiful niece, is a fighter. She was 10 months old when she got sick and started to lose strength in her legs. Doctors had no explanation and struggled to find answers. When she was 14 months old, she had a second sudden episode and was admitted to the ICU. Eventually after several days in Seattle Children’s Hospital, doctors discovered she had a rare genetic disorder. Rare translates to known cases being in the hundreds worldwide, and no specific name. Referred to as LPIN1, the deficiency in the gene causes her body to go stiff and more or less attack it’s self. As her parents became more educated about LPIN1 as her caregiving team, they found the diagnosis to be devastating. As the body responds to an episode, the blood increases creatine kinase (CK) levels. “Rhabdomyolysis, caused when muscle tissue breaks down realeasing harmful proteins into the bloodstream. It’s measured by checking a patient’s creatine kinase level. A typical CK reading is around 150”. Today Makenzie is in the midst of her 6th episode defying a deficiency and doctors alike, as she encountered CK levels of 2.8 million. The onset of the latest episode came quick. Within hours of waking up she was in an ambulance to Seattle Children’s Hospital, arriving with a CK of 11,000 and potassium levels exceeding the testable range. Within minutes she went into cardiac arrest. Receiving CPR and several defibrillator shocks to her heart, doctors performed surgery to maintain blood flow to her heart. Family rushed from California and Alaska fearing during their flights, she did not make it. She was not yet ready and fought for her life. That evening her heart started to function on its own. Although still unable to pump her own blood, there was a silver lining to a dark day. She had 7 machines maintaining her life, and the extent of damage was unknown, with brain damage seeming likely. The onset of this episode was April 29th.

Today, Makenzie is still in ICU making slow, steady progress as some life support systems are turned off as her body is regaining strength. Her mom has not left her side and the expenses are growing. Her father makes the 2 1/2 hour drive to their home to check on her brother, who is staying with family, and job sites he currently oversees. Extended family has traveled to Seattle for support and delivers meals as long-term treatment and life style plans are being made. This latest episode is forcing some major changes to Makenzie’s life. The distance from her home is too great for the care she needs, and the risk of closed mountain passes will be mitigated with moving. In the short-term that translates to staying with family.  Hopefully a new home in the near future, where her father can have a reasonable commute and care centers are close. Despite all the stress of being in ICU and a future which is not yet charted, Makenzie has a personality that shines through and loving parents who somehow maintain a positive environment with humor and love. Makenzie is a princess and her room has often been called her court. Kenzie’s Court, a little hearted joke that helps ease tension when she is not in the mood for treatment that is extremely uncomfortable. As we all continue to pray for healing and strength, we stared this page on behalf of the family to ask for financial support in the added expenses. We are forever grateful for the prayers you have given and for the opportunity to see God work through Makenzie's life, thank you.

With gratitude,
Nick and Rena