by her mother, Lori
“But surgery will cure her, right? She just needs an operation to make her feel better!” I can still here myself saying these words as my husband and I sit in front of the Cardiologist at Primary Children’s Hospital.
“No Mrs. Heaton, Madison’s condition is incurable. The only treatment is a lung transplant, which I don’t recommend. Take her home and make her life as pleasant as possible, for whatever time she has left.” My ears hear these words but my mind, my heart; my soul does not accept them! This is the beginning of Madison’s story. My name is Lori Heaton, I’m Maddie’s mother. It was 1997; Maddie was 4 years old when we were told she had a large Atrial Septal Defect and pulmonary hypertension (extremely high pressures in the lungs that causes damage to the heart.) This was following a respiratory arrest. Looking back she had other symptoms such as vomiting, blue lips when she was cold and being small for her age. Symptoms that could be explained away, certainly not symptoms of an incurable, life threatening disease!
We would make her life as pleasant as possible, but we would not take her home to die! We found out about a drug called Flolan. It was a new medication, not yet approved by the FDA. It offered patients with pulmonary hypertension an alternative to lung transplantation or eventual death, it offered hope! Two days later, my husband Randy and I found ourselves flying to Denver Colorado. This is where we met Dr. Dunbar Ivy. He told us that although Madison’s condition was incurable, she could live a fairly normal life. It would require committing Maddie to a life on an IV. How could we do this to our beautiful little daughter! But how could we not! Dr. Ivy told us that Flolan offers patients an 80% survival rate at five years. (Ever since Maddie’s diagnosis, it seems that I’m thinking of her future in terms of statistics!)
Madison started on Flolan in September of 1997. Although there was not a significant drop in her pulmonary pressures, her symptoms improved dramatically. Randy and I learned to mix her medicine and care for her central line. In other words, we had to change our lives and learn to live with a child with a serious illness on a continuous IV. I had been working part time as an RN when Maddie became ill, so I quit my job. My daughter had now become my full time job! Being a nurse made all these changes a little less overwhelming. I commend all those parents and spouses who have no previous medical experience, who have learned to care for a loved one on Flolan.
It seemed for awhile that Madison was able to live a fairly normal life, despite the restrictions imposed by her disease and her IV line. But then in 1999, when Maddie was 6 years old, she started having problems with low platelets. We were constantly battling uncontrollable nosebleeds that would send us to the hospital for blood transfusions. At first the problem was thought to be the Flolan or an infection. But the problem persisted, in fact it got worse. She was being transfused almost weekly! Finally after two months of tests and second guessing, a bone marrow biopsy was done.
Again we found ourselves sitting in front of the doctor. He had the same look that the cardiologist had on his face two years before when we were told of Madison’s pulmonary hypertension. This time the doctor told us that Madison had ALL (Acute Lymphocytic Leukemia.) All the shock and disbelief we felt two years ago came flooding back, but this time it felt worse. How could this happen! This was so unfair! Isn’t one life threatening disease enough for one child! Unlike pulmonary hypertension, Leukemia is one of the mort curable childhood malignancies with a cure rate of over 75% with chemotherapy. (Again, another statistic for her future!)
Madison has lived with pulmonary hypertension and IV Flolan for 8 years. Despite numerous IV line infections, Maddie has done well on Flolan. It has given her back a life that otherwise would have been taken. Maddie underwent two years of chemotherapy. She had to endure spinal taps, bone marrow biopsies, blood transfusions, nausea, hair loss and chemical weakness. She is now four years in remission! Before I close this story, I would like to thank all the doctors and nurses who have cared for Maddie through her journey. A very special thanks to Dr. Dunbar Ivy and his caring staff who have seen us through everything from true emergencies, to the everyday concerns that come with the Flolan territory. Thank you for loving her almost as much as we do! Madison is the hero of this story and in true hero fashion; she has been strong, brave and happy. I am always amazed that her small body can contain such a powerful spirit. She is a happy child who has touched and continues to touch many lives. Madison, we love you and are grateful you are our angel here on earth!
In September of 2004, Madison switched from IV Flolan to IV Remodulin and continues to amaze us all!