In November of 2007, the world as my family knew it would never be the same. My 21-month-old son Maddox had been unwell for some time, experiencing lethargy and a distended abdomen and a mother’s instinct told me something wasn’t right. Maddox was eventually hospitalized for three weeks and after some long, hard days and nights with invasive test after test, he was finally diagnosed with a rare genetic kidney and liver disease called Autosomal Recessive Polycystic Kidney Disease (ARPKD).

As ARPKD is a genetic disorder, I was told I needed to have my 3 year old daughter, Livia, tested too.  My living nightmare continued when it was also confirmed she too had ARPKD. We came to learn that they were lucky to have survived their births since this merciless disease has a 50% mortality rate upon delivery.

ARPKD affects 1 in 20,000 children and both of my precious children were now part of that statistic. As we learnt more about this disease, we began to grieve the normal life that my children would no longer live and we moved on with our ‘new normal’, uncertain of what the future held for them both.

Just when we thought Livia and Maddox had endured so much, we came to learn a few years after their initial ARPKD diagnosis, that they both also had yet another rare genetic condition called Caroli’s Syndrome in which bile ducts in the liver are wider than normal and can cause life threatening infections.

In addition, Maddox was also diagnosed with Congenital Hepatic Fibrosis of the liver, Esophageal varices due to Portal Hypertension, Splenomegaly (severely enlarged spleen) and Thrombocytopenia (low platelet count) all conditions that go hand in hand with his ARPKD diagnosis.

Over the years the kids have both been through so much. Through all the countless blood draws, procedures, imaging, testing, treatments, ER visits, doctor appointments and extended hospitalizations they have handled it all with immense bravery and grace while trying to live their lives just like regular kids. But the reality is they are not like other regular kids. Currently Maddox is on over a dozen medications each and every day to keep him healthy and alive, along with several epogen injections each week. Livia is on numerous medications to stabilize her blood pressure and keep important levels affected by her kidney disease stable.

As of now, Maddox is awaiting a new kidney and liver, and Livia is awaiting a new kidney. For Livia we do not yet know if she could start to show signs of more severe liver involvement and that could mean she will be waiting for a new liver just like her younger brother.

In spite of the adversities these kids have faced and endured in their relatively short lives, they have continued living their lives as fully as they can with family and friends who love them dearly.  In 2012, they became a big brother and sister to their little sister Gracie. She has brought them so much joy and of course so much younger sibling annoyances!  When I talk to people about my children they are always in awe of how resilient and brave they are. They have faced more in their short lives than most experience in their entirety.

Maddox is hilarious, kind, witty and always knows how to lift anyone’s mood. I admire that about him. He is now 17 and a sophomore in High School. You can find him most of the time hanging with his friends, watching sports with his dad and keeping up with his schoolwork. Livia is kind, smart, disciplined, deeply caring and very passionate about what she finds important in life. She is extremely creative and talented, loving art and music. She is now 19, a freshman in college and studying to become a Labor and Delivery nurse. You can find her most days driving back and forth to classes, diligently studying, and chatting with her friends and family. Both Livia and Maddox are affected on a daily basis by their medical conditions. They work hard to keep up with responsibilities while fighting severe fatigue and often ill due to their suppressed immune systems. They push on and are masters at adapting but the mental and emotional toll does not escape them. We are all so proud of the people they have become, and anyone would be lucky to call them their friend.

A new chapter awaits Maddox and Livia after their transplants. They will be on immunosuppressant medication for the rest of their lives so that their bodies don’t reject their new kidneys and liver. They will continue to endure doctor visits, blood work, imaging, amongst other tests to make sure their transplanted liver and kidneys continue to function properly. All of this and much more to keep their new organs strong for years to come.

As they move into their adult years, with these transplants behind us, the fact remains that we must expect additional transplants to follow. This reality will require continuous support as they meet these challenges. My hope for my children is that the financial burden of this challenge is minimal, allowing them to maintain a clear focus on their health and their true joys of life. This is why our family has partnered with COTA to raise funds in honor of Livia and Maddox which will assist with a lifetime of transplant related expenses.

The Children’s Organ Transplant Association (COTA) helps children and young adults who need a life-saving transplant by providing fundraising assistance and family support. COTA is the nation’s only fundraising organization solely dedicated to raising life-saving dollars in honor of transplant-needy children and young adults. 100% of each contribution made to COTA in honor of our patients helps meet transplant-related expenses. COTA’s services are free to our families, and gifts to COTA are tax deductible to the fullest extent of the law.

The stark reality of their health issues means Livia and Maddox will be financially responsible for many, many medical treatments and procedures for many, many years to come. Maddox and Livia are humble, selfless people who would not ask for anything, but as their mother I ask that you please consider donating to COTA for Team Ventura to help lessen the worry and burden for them.

My family thanks you from the bottom of our hearts.