To the untrained eye, Mikaela Sienkiewicz of Burlington, Vermont appears to be a typical little girl with lots of energy and a smile for everyone. What most people don’t realize is that Mikaela has inherited a genetic disorder called Polycystic Kidney Disease.

An Inherited Genetic Disease
Mikaela has the recessive form of PKD. This means that her parents, Michael and Heather, each carry the gene but do not actually have the disease. For carrier parents, there is a one in four chance in each pregnancy that PKD will be transmitted to the baby.

Mikaela’s condition was discovered during her wellness check at age four. Because her liver felt somewhat enlarged, she was sent to a specialist for further tests. Those tests revealed scarring of the liver, anemia and low kidney function. Polycystic Kidney Disease was the official diagnosis.

Because there is currently no cure of PKD, management consisted of constant monitoring of her condition and treatment of symptoms. For several years, Mikaela showed no symptoms and routine blood work and ultrasounds assessed the progression of the disease. Iron supplements helped keep her anemia in check.

An Unexpected Crisis
All that changed one evening in late May 2004. After a ballet rehearsal, Mikaela started to feel sick. Things got worse through the night and by morning she was very pale, vomiting blood and, ultimately, fainted. After being rushed to the Emergency Room, an examination found she was bleeding internally and half her blood volume had been lost. Transfusions began immediately. Mikaela received four units of lifesaving blood.

Because of the severe scarring of the liver, it is difficult for the blood to pass through. Pressure builds as it tries to find another route. Four veins in Mikaela’s esophagus had ruptured, causing the severe bleeding.

Waiting For A New Liver
Now in liver failure, Mikaela has been placed on the waiting list for a new liver. Estimates say a suitable organ might be found within eight months to a year. Preparations are being made for when the family receives that all important call, including an Angel Flight to Children’s Hospital in Pittsburgh where the surgery will be performed.

At that point, Mikaela will once again depend on the kindness of strangers for the blood she will need. “We can’t schedule something like this so we need to depend upon people donating regularly so the blood will be there when she needs it,” said Heather Sienkiewicz.

The kidney disease will not affect the new liver because it will be completely different genetically.

Thankful For Blood Donors
The Sienkiewicz family is very grateful for the blood their daughter has already received and has worked with friends to organize blood drives in her name as a way to give back to the community. According to Heather, “Blood donors literally saved my little girl’s life.”

Mikaela may ultimately need a kidney transplant as well. As long as there are dedicated Red Cross blood donors, however, the Sienkiewicz family will never need to have the additional concern about whether there will be enough blood to perform these lifesaving transplants.

Red Cross blood donors help patients just like Mikaela in cities and towns throughout New England and across the United States. Please….give blood. Together, we can save a life.

Patient Stories