What Can You Expect When Raising a Child with ARPKD/CHF?

March 2, 2013 / admin / 2 Comments / ARPKD/CHF Alliance

When my OB/GYN told me that my unborn daughter’s kidneys were too echogenic, I didn’t have any idea what in the world she was talking about, let alone the impact it would have on our lives in years to come. When my husband and I stood over our newborn daughter’s bassinet in the Neonatal Intensive Care Unit, numb from the shock of her clubfeet, her distended abdomen, and the doctors’ casual use of words like dialysis, we couldn’t grasp the significance of these challenges that our daughter faced. I remember repeating to myself, “I have a special needs child,” in an attempt to just absorb and process the enormous challenge that had been handed to me at only 23 years of age. In those first few days we had one burning question: What can we expect in the future? But no doctor could answer that.

When you have a baby, you have some ideas in your mind of roughly what it will be like to raise that child. You have had younger siblings, or babysat, or watched people around you raise children. In my case, I had spent all of my junior high and high school years babysitting. I had worked as a part-time nanny through college, and a full-time nanny for several families after I was married at age 21. I felt as prepared as anyone to raise a child. But nothing in my experiences had prepared me to raise a child with a rare, life-threatening, degenerative kidney and liver disorder, and no one could offer me a hand-book to guide me through the jungle of tests, appointments, questions, and emotions that lay before me.

Our nephrologist tried to calm me as I stood in tears over Naomi’s bassinet. “Remember, it’s a baby, not a kidney,” he counseled. I understood what he was trying to communicate: that my daughter was a whole human being, not just diseased kidneys; that I should enjoy her dark brown eyes and her long piano fingers and her sweet, sleepy smiles. But the nephrologist didn’t understand what I was trying to communicate to him: that those diseased kidneys with all their frightening unknowns cast a shadow of fear over those dark brown eyes, those fingers, those smiles. I didn’t know how to enjoy a child whose life was so fragile, so tenuous, so unpredictable.

We lived through that day, and then we lived through the next. We finally took her home and began to get to know Naomi. For a few days we were so caught up in the normal challenges of first-time parenting that we almost forgot about her kidneys. Then came a routine blood draw, followed by the terrifying call from the nephrologist, and the rush to the emergency room because Naomi’s potassium level was life-threateningly high. I stood in a corner of the curtained-off ER room with hot tears squeezing out of my eyes as nurses pinned my screaming two-week-old daughter to the gurney and stuck her with needles again and again in an attempt to draw more blood and start an IV line, and I thought, “Is this what I have to expect? Is this what my daughter’s life will be like—constant fear and worry, one painful day after another?

There was no older, more experienced mother who had walked this road before me to put her arms around me and tell me that it would be OK. I knew no one who could encourage me that I could be a good mother to Naomi, and that Naomi would grow into a beautiful child who loved life, even with all its pain and challenges. Eight years later, I would like to take this opportunity to be that mother for someone else who may be starting out on the road of raising a child with ARPKD/CHF–to tell you what I’ve learned on this journey and speak the words of encouragement to you that no one was able to speak to me.

Of course, I must say first, that my story is not your story, that every child with ARPKD/CHF is different and that their clinical courses and your personal experiences may be vastly different than my own. Unfortunately, no one can tell you exactly what to expect from this disease. Some babies do die in infancy; some require immediate and drastic intervention such as dialysis and kidney transplantation. Some children have more hospital stays, more life-threatening episodes, and more suffering than my family has faced, and I do not pretend to know what it is like to walk in their shoes. I am only speaking from my experience.

I am now raising two daughters with a milder form of ARPKD/CHF, Naomi who is eight years old, and Emma who is five. If you are new to the journey of raising a child with ARPKD/CHF, I would like to offer to you some ideas of what you can expect.

You can expect to feel overwhelmed at first. Most families who discover their child has ARPKD/CHF, have no family history of this disease. You are thrown into an ocean of new terminology, new tests, new medications, and new fears. There are no swimming lessons and at first you will doggy-paddle your way through the days to keep your head above water, but as the days and months pass, learning about this disease with all of its terminology and treatments is like learning the breast-stroke, and the back-stroke, and all the other swimming strokes. You don’t have the option to leave the ocean, but you can learn to thrive in the water, and you will.

You can expect to hear and read and think about the scariest scenarios first. It was so helpful to my husband and I when Dr. Heller from the National Institutes of Health sat down with us and explained that while what we read about ARPKD/CHF on the internet wasn’t necessarily false, it also wasn’t an accurate representation of what most families with ARPKD/CHF will face. He drew a picture of an iceberg in an ocean and told us that most of what we will read on the internet is about the worst case scenarios because those were the cases that got diagnosed before the regular use of ultrasound tests. They are like the tip of the iceberg sticking out of the water. But now, especially with the advent of routine prenatal ultrasound, we are finding that many more people have a milder form of ARPKD/CHF like my daughters do, something that might have gone undiagnosed for years before, and the outcome for this group may be much brighter.

You can expect to feel more worried than the parent of a healthy child. Each symptom raises the questions: Is this serious? Is this another urinary tract infection? Is this the dreaded cholangitis? Is that rash petechiea from low platelets? The first time your daughter spits up something red you will pick up the phone to call the ambulance, fearing she is having a GI bleed, but set it down again as you realize she just gagged and spit up her red multivitamin. You will probably have to fight the urge to call the doctor, or visit the doctor, or run to the ER for each and every minor illness or unusual symptom. I don’t know that there is a cure for this, but educating yourself about the disease and its possible complications will help. If you know the symptoms of a UTI, cholangitis, or a GI bleed, then you are less likely to be overly concerned with the regular childhood illnesses.

You can expect to find comfort and support from others who have walked this path before you. The ARPKD/CHF Alliance and the online Yahoo and Facebook groups for ARPKD/CHF have been an invaluable resource for me to learn about my daughters’ disease, to pose my questions to parents who have been through this before, to connect with others who have gone or are going where we are headed and learn from their experiences.

While it is certainly not guaranteed, it is our experience, and that of many others that you can expect your child to grow and do many of the things that healthy children do. Many children with ARPKD/CHF enjoy years of near “normal” lives. They have friends, attend school, and play sports. They enjoy their lives and have dreams for the future. Yes, they have more regular doctors’ appointments. They have more blood draws and get drug to the ER by slightly paranoid mothers more often than their peers. They may need multiple prescription medications, or require at-home blood pressure monitoring, or wear a spleen-guard when they ride their bike. Their school-age years may include an emergency procedure to stop an upper GI-bleed, or it may include a kidney transplant. But their resilience and desire to live through it all will be an inspiration and blessing to those around them.

Lastly, you can expect to be blessed by your child with ARPKD/CHF. Watching a toddler smile, oblivious to the GI tube taped to her face, or a little child face a blood draw with courage, or a young adult face a kidney transplant with hope helps to shine a new perspective on all of our lives. I am reminded often that life is incredibly fragile, yet also incredibly resilient—that none of us are guaranteed a certain number of days on this planet, and that each day should be lived carefully, deliberately, purposefully. I am reminded more often than most parents how important it is to hug my child, spend time with my child, and tell my child I love her. I am a more careful, more purposeful parent than I would have been if I had continued to take health for granted. I am a more compassionate, more caring person when I meet other parents of special needs children. I am a better person than I was before, after having walked this difficult road. The pain my family has endured has also brought us immeasurable blessing.

If you are new to the journey of parenting a child with ARPKD/CHF let me put my arm around your shoulder and offer you the words of one of my favorite song writers, Rich Mullins:

It seems that love blooms out of season

And much joy can blossom from many tears…

…Knowing morning follows evening

Makes each new day come as a gift

Katherine Eby

–Mother to five children, including Naomi and Emma who are affected by ARPKD/CHF