How is she doing?

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?”

Carl and Rhiannon, July 2004, World War II Memorial, Washington DC

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.

2014 Update: to learn more about Rhiannon’s life and the illness that would eventually claim her life, click here.

  • Ellen N. Duell

    Blessings for Rhiannon, for you and for your wife! May there be beauties, joys, and much love for you all in these next months and years. The love will continue to be shared after Rhiannon passes from her body–I can attest to this, in no measureable way, because my husband, one of my sons and two of my daughters now live in their “celestial” bodies. (I Corinthians, 15:35-end of chapter).

  • http://discombobula.blogspot.com Sue

    Oh, ((Carl)),

    What a beautifully written ode to ((Rhiannon)). I just love that photo. I also love what Ellen wrote above here.

    I went walking this afternoon, grumpy and stroppy and feeling hampered, and began listing the things I was grateful for. One of those was for health, after an almost-three-week flu. I felt the blood pumping into my body, the joy that comes from exercise, and now reading this it makes me feel more grateful for small mercies and also railing at the unfairness that your little girl does not get to do that. It’s just really not fair, is it?

  • Simon Whitney

    Carl
    We will all be praying for all of you.
    Such love turns words to straw.
    Simon

  • http://ponderingpagan.wordpress.com TLH

    You are nothing short of incredible.

    God be with you, today and every day.

    You are in my prayers.

  • Kevin Murphy

    Carl, thank you for the searing honesty and courage to publish this. Your family’s journey into the triumphs and tragedies posed by Rhianon’s conditions are wonderful lessons in the power of love, perseverance and joy that I can only stand in awe of. Your family knows of struggle and commitment that few of us will ever experience. Thank you for the glimpse into your journey together.

  • http://www.lfhgroup.com Leslie Hershberger

    Much gratitude Carl…thank you for sharing your encounters with pain and the grace that holds all of us.

  • Frank

    I love your story, so much dedication and Love. I was trying to remember something about the more pain and suffering we have to endure the more peace and joy we will get in it’s place. Your family are in our prayers.

  • suzanne kurtz

    Carl,
    I have tried to imagine myself in your place, in Rhiannon’s place, in Fran’s place, and wonder what it is that Spirit pours into you three that allows you to just keep drawing breath, in faith.
    All blessings, all three of you remain in a humble prayer.
    Suzanne

  • http://wwwspiritusabbey.com Fr Chuck Cooper

    Carl,

    I’ve tried to write something but words just sputter.

    Love, prayer, silence, awe, and love again are all that come to mind.

    Bless you my brother and your whole family,

    Chuck Cooper

  • Nolan ‘Gus’ Galloway

    What an amazing story of God’s grace manifested through you and your wife toward Rhiannon – thank you. I am in awe of the power of God’s love for you which enables you to function this skillfully. My life’s story includes the death of two of my adult children – one to suicide another to a brain birth defect – and in that darkness I saw and experienced grace so I know how realistically you are dependent on the amazing love of God. Your story has fed me today.

  • http://www.discovercedarhill.org Kat Stratton

    Carl,

    The community of Cedar Hill will hold you all in prayer and meditation. Thank you for sharing from the depths of your humanness. Peace.

  • Al Jordan

    Carl,

    The Peace of the Lord be with you and with Fran and may God’s love envelop Rhiannon in the days and months ahead.

  • Lainey Grace

    Dear Carl
    What wonderful love you and Fran have shown Rhiannon for all of her life. The last time I saw here she had just moved into her teenage years. I have thought of you all so much. I think of the prayer; Christ has no body now but ours, no hands, no feet on earth but ours, Ours are the hands with which he shares commpassion in our world. Ours are the feet with which he walks to do good.” You and Fran have certainly lived this prayer.

    With the interest in devices for people with stroke and or head injury – are any of the computer assisted devices applicible for her?
    The 3 of you are an amazing couple. I hold you in my heart an will also ask the Franciscans in my group and our church prayer chain to pray as well

    God Bless and give you all hearts of peace,

    Pace e Bene,
    Lainey Grace

  • http://www.metanoia-mrc.blogspot.com/ Robin

    It now seems completely clear to me why you have embraced and delved into and sought to share the experience of mysticism.

    Prayer surround all of you.

  • Brenda

    God bless and strengthen your daughter and family while going through your personal trials of life. Our perspective is limited in this lifetime and sometimes we wonder where God is during these difficult times, but remember he is there. God is aware of your personal situation. Thank you for sharing your personal heartfelt story which I am sure will touch many hearts. God works in many wondrous ways…Strenghten yourself with faith in God…

  • http://heartofflame.blogspot.com Yewtree

    I was also very moved by this post and all of the responses above. You and Fran and Rhiannon will all be in my thoughts too. You are all very brave and loving people.

    Huge hugs.

  • episcopalifem

    (((((Carl)))))) (((((((Fran))))))) (((((Rhiannon))))))

    What everyone else has said, and some more.

  • chaidrinkingfool

    Thank you for being willing to share this.

    I’ve heard about Rhiannon, but being new to this blog, haven’t seen a picture of her before. This is a neat picture: I like it a lot.

  • Pingback: Concerning Spiritual Noise, (Lack of) Inner Silence, and Singlemindedness | Anamchara • The Website of Unknowing


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