I had the occasion to speak with a friend yesterday with whom I haven’t spoken in over a year. We did the obligatory “how’s it going?” conversation, and of course, that led to the hardest question of all: “How’s Rhiannon?”
For readers who do not know the story of my stepdaughter, she was born in 1985 with polycystic kidney disease, which is, according to Wikipedia, “the most common genetic, life threatening disease affecting an estimated 12.5 million people worldwide.” Her enlarged kidneys at birth led to a number of secondary issues, including underdeveloped lungs and intestines; at the time of her birth her doctors had no plan of care for her but she beat the odds and managed to survive (after 10 weeks in hospital, much of which was spent in intensive care). For the first three years of her life she progressed, slowly, under the oppressive label of “developmentally delayed,” but as she passed her third birthday she was speaking and even on the verge of walking.
Then she had her stroke.
My wife found her in bed, lying face down and having seizures, one awful Thursday morning in August 1988. Twelve hours later the doctors finally got the seizures to stop, but she remained suspended in a coma for a week. When she finally regained consciousness, she had lost her ability to speak and experienced paralysis on the right (her dominant) side, a condition which landed her in a wheelchair, where she remains to this day. After a grueling regimen of speech, physical and occupational therapy, she has become beautifully articulate, although in the rather trippy scrambled-reality sort of way that I’ve seen with other serious stroke survivors. She cannot write without assistance, nor can she do pretty much any other basic life skill. She needs assistance getting in and out of bed, on and off the toilet, getting dressed, cleaning up, and pretty much any other ordinary task. A doctor once told us she had the self-care abilities of an 18-month old.
It breaks my heart that she is aware enough to recognize just how limited her life is. For years, her stated deepest wish was to walk. But the motor skills necessary to make that happen, even with a walker or some other support, always seemed to elude her, and when therapists (or, worse still, her mother and I) tried gently to push her, she responded with consuming anger and frustration — no doubt not unrelated to the stroke, but still functioning to close off that particular dream. We shed tears when her physical therapist recommended terminating therapy — of course, the therapists have overwhelming case loads, and the cruel nature of triage means that they needed to focus their efforts on those clients who were actually making progress. We fought this for a while, but eventually came to realize that, barring a miracle of Cecil B. DeMille-ian proportions, she was not destined to walk on this side of eternity.
Ten years ago or so a doctor suggested to us that her life expectancy was probably somewhere in her 30s. For the longest time, Fran and I seemed to interpret that in a “glass half empty/glass half full” sort of way. I saw it as the gift of years, whereas Fran saw it more as an impending doom. I suppose that may be the difference in our personalities, or the difference between a biological parent and a step-parent. But as Rhiannon moved out of her teens and into her young adult years, Fran always seemed to hold Rhiannon’s mortality more prominently in her awareness, not in a morbid sort of way but in a more matter-of-fact, this-is-what-we’re-dealing-with sort of way.
Various health issues emerged over the years. We learned that Rhiannon also suffered from liver disease, apparently not uncommon with individuals suffering from PKD like she does. Secondary to the liver disease have been problems with her blood circulation, resulting in an enlarged spleen and, during a harrowing period about eight years ago, several instances of esophageal varices — enlarged veins in her esophagus, that ruptured and led to horrible episodes of her vomiting blood. This condition required a series (12, if I recall correctly) of endoscopies in which her physician literally repaired the wall of her esophagus, using ligatures to choke off the varices. Because of all Rhiannon’s medical issues, this usually meant an overnight stay at the hospital.
She has suffered from debilitating headaches, extreme mood swings (probably related to the stroke), acid reflux, and a creeping but relentless loss of energy, resulting in not only fatigue, but an increasing inability to, as we say, “do her life.” After she graduated from public school (the day before her 22nd birthday), we had her placed in a day program, so she could remain living with us. At first she embraced this new chapter of her life, making friends at the program and participating every day. But as her fatigue grew, she began to fight with us in the mornings, angry at being pressed to get up. A series of in-home nursing assistants have come and gone, providing assistance to Rhiannon both in the morning and afternoon; the morning shift proved particularly difficult, since we never knew in advance if Rhiannon would be her usual sweet and gregarious self, or if the fatigue and the fury would emerge and she would spend the morning screaming, crying, and hitting anyone who dared get close to her.
Last year, at the insistence of her liver specialist, we had her evaluated for a possible transplant. This led to a round of meetings with doctors, nurses, nutritionists, and finally the surgeon who would likely perform the transplant, were it to happen. Along with the meetings came lots of detailed information about the many drugs and other medical concerns that accompany the transplant experience. It all seemed overwhelming, but of course, by this point everything about Rhiannon’s condition seems overwhelming. At the end of all the interviews, the surgeon gently told us that he was concerned that a transplant would actually increase, rather than decrease, her risk of mortality within the first twelve months. So she was turned down. Although in our hearts we knew this was for the best, still it felt like just another door being slammed shut in our faces.
The fatigue and the angry outbursts continued, and so this summer, when Fran was out of work (she’s a teacher’s assistant), we allowed Rhiannon to stay home and rest whenever she wanted. What emerged was a pattern of sleeping in as much as wanting to go out. So we’ve arranged our schedules, and the schedule of her nursing assistant, to allow her to stay home — and sleep most of the day — two or three days each work week. So she still has social interaction away from mom and dad two or three days each week, and we hope that can continue as long as possible — for her sake as well as ours.
I don’t fully understand all the markers for end stage liver disease or the other ways in which the doctors measure just how sick my little girl is. A while back they started monitoring her blood and giving her transfusions whenever her counts fell too low. Apparently her spleen “eats” her blood because the liver and kidneys are just too sick to process it all fast enough. But the risk there is that if her blood cell count gets too low — turning her blood into basically a watered down soup — she runs the risk of heart failure or some other catastrophic event. So now I affectionately call Rhiannon my little vampire, needing a transfusion every two to six months.
Over the summer Fran and I talked to her liver specialist. He said those words that point to the gravity of her condition: “we will do all we can to keep her as comfortable as we can for as long as we can.” We asked him to give us a sense of what we can expect in the months or years to come. Of course, knowing that anything (including miracles) is possible, the doctor hemmed and hawed, but finally said “She probably will not see her 30th birthday.” Considering that she just turned 25, I said “So we have three to four years.” “More like two to three,” he replied. Barring a miracle or some other unforeseen twist, over the course of the next two to three years we will probably see continued decline in liver and kidney functioning, continued need for regular blood transfusions, and at some point will need to make some decisions regarding dialysis. Beyond that? Hospice. And beyond that, there will come a day when we will be asked to give her back to God.
So, back to my chat yesterday with an old friend. How do I compress all this into a brief small-talk kind of reply, without sounding maudlin or hysterical or depressive? To just say “she’s hanging in there” works in some situations, but when it’s a good enough friend that I want to be more honest than that, I end up giving the person probably a lot more information than they need or want. But that’s the reality of the situation. I usually can talk about it without crying, just as I’ve written all this, this morning, without shedding a tear. Because somehow, it’s easy to talk about “her condition” in a sort of detached way. But if I start thinking about her smile, her playfulness, her enthusiasm for seeing me when I come home from work each night, how she and I have called each other “Shrek” and “Donkey” ever since the first movie of that particular franchise came out — then it becomes harder to keep the tears at bay.
And then I take a deep breath, and carry on.