Fiona Midori King

Fiona Midori King

Hope 4 Fiona

We Will Meet Again

 

 

Paxton wrote: 

"Our words are failing us this day. This sweet light in our lives... We can only simply say... Our angel Fiona is in heaven. She went peacefully surrounded by love early this morning (Dec. 23). She left her body and immediately was in the presence of Jesus. No more GM-1. We miss her. We love her and we are more grateful than ever to know all the beauty she brought into our lives... Hug your beloved ones. Lie next to them and kiss their cheeks and hands. Ask them about their important things and lock it all up in your heart. There will come a day when it will not be as easy to come by and you will need those tender, caged moments to carry you through. Love fiercely, without end. We loved her more than she could ever imagined ❤ We will see you in heaven our sweet angel girl ❤ With deep sadness, deep love and joyous remembrance, Dee & Paxton"

 

 

 

 

Happy Father's Day

 

Fiona has never said a single word to Paxton, nor is she likely to on this earth.  He will never teach her how to ski, go to a father/daughter dance with her, or walk her down the aisle to give her away.  But the beautiful bond these two share - not even GM-1 can mar that!  When we welcomed Fiona into this world, Paxton had no way of knowing he would be raising a terminally ill child, had no way of anticipating the many challenges that would test him and force him to face life head on.  Yet from the very beginning he took on this vital role of fatherhood with strength and unconditional love.  It wasn’t the fatherhood he’d expected.  He held me up when I couldn’t stand on my own, and told me that as long as we stuck together, nothing else mattered.  Paxton has done the bravest thing by making life carry on; it's going to work each day and pretending to bosses and co-workers like everything is normal just so the pay check keeps coming in and the medical insurance doesn’t lapse. Its saying “Great” when co-workers ask “how’s it going” instead of, “terrible, my little girl is at home with a lung infection and I want to be at her side in case she doesn’t survive the day.”  Sometimes its retaining his sanity so he can be become my rock. Sometimes it’s just listening when no one else understands.  He is Fiona’s biggest advocate, the researcher of GM-1, the record keeper of all her medical files, the communicator with all her specialists, the fighter of our insurance rejection claims, the keeper of spreadsheets of seizure activity, weight, ketone level, blood glucose, current prescription medications, unpaid medical bills, and the pusher of wheelchairs.  He’s the hug giver, the hand holder, the rock and strength of our family, and the beautiful part of Fiona’s life and mine. For the past 4 years I’ve watched Paxton treat Fiona as the priceless gift she is. He is a gentle, loving, kind, intelligent  man who is not afraid to love our family openly and fiercely.  I want to wish the biggest HAPPY FATHER’S DAY ever to my amazing husband.  He has been such an amazing father to Fiona throughout the years even though his job at work and his job as a father have been much harder than the typical dad experiences.  Special needs dads are in a category all their own and in my humble opinion, they don't always get the credit they deserve.  To all the different types of Fathers out there. YOU ALL ARE NOTHING SHORT OF AMAZING!  Happy day to all of you; I hope you are able to spend it with the ones you love the most! 

 

 

 

Let's Find A CURE!!!

 

Home School

 

This morning Fiona had her special needs school at our house. She did a great job today with various activities and had so many expressions on her face. I know she enjoys the interactions.  I also learned that she didn't enjoy getting slime all over her feet as was one of the interactive activities.  Although Fiona does not attend a “traditional school,” she is still receiving educational experiences within the safety & sanitation of our home & I’m grateful to share these experiences with her.

 

Fiona has taught us a great deal about what truly matters in life.  She has also revealed to us that anything & everything is possible.  If you are a parent raising a child with a health condition, whether it is cancer, autism, a rare disease, deafness, learning disabilities- the most important advice I can offer is to educate yourself about what it is that your child is facing and to seek the best possible treatments. I thank God that we came across amazing families that helped us along this journey, doctors that really care for Fiona,  and all the resources we need to provide Fiona with the best treatments and necessary programs available. 

 

Oddly enough, this story of a family planning a trip to Italy helped me a lot when Fiona was diagnosed. It was a family that dreamt for years about traveling to Italy. They bought books, learned the language and planned every detail of their trip. When the day of the trip finally arrived, they felt really prepared! When they landed, the flight attendant's announcement welcomed them to Holland. "Holland???" said the family. "What? We're not in Italy? But we have everything set for Italy!" Needless to say, to land in Holland was not what they expected or prepared for and their journey turned into something different--with sights, people and places unlike what they had anticipated & hoped for but still an amazing and true learning experience nonetheless.

 

My family's journey since Fiona’s arrival has been much like this family's trip to Holland: unpredictable and new but oh so very fulfilling and blessed with wonderful surprises.

 

 

My Hands Are Full

 

 

When you are a parent of a child with a rare disease, there’s much to consider & remember when going about your days.  When needing to leave the house, its not quite as simple as asking your child to hustle along into the car with a bag of snacks & activities to preoccupy them while you attempt to cross a few errands off your to-do list.  I don’t have to worry about making sure my toddler is holding my hand when we are in parking lots and I don’t have to teach her not to talk to strangers.  What I do have to think about, however, is all the support equipment I need to have ready at a moment’s notice.  I have to think about the germs to which I am exposing my daughter’s lungs. Although I don’t draw the attention of others due to my toddler’s temper tantrum at the gas station or her spirited performance of her favorite song in the middle of a grocery aisle, we do draw a different type of attention.  A type of attention that only families who share the similar life experience of having a member with many medical needs could understand.  It is what my life entails & I’ve learned to better understand it & proudly live it each day.

 

I used to be nervous at the beginning when we took Fiona out of the house. Some of the concerns that ran through my mind included: What if Fiona has a seizure? What if she stops breathing? What if people notice that our daughter is very sick? My anxiety is not because I am in any way embarrassed of Fiona, but as a private individual, the idea of drawing attention to myself or to my family made me slightly apprehensive.
Our lives are remarkably different from that of our friends and families.  Whenever we leave the house (even if just for an hour) our car is packed with an oxygen tank, suctioning machine, extra feeding tubes, emergency seizure medication, and syringes of food.  Longer excursions require more equipment. Trips also have to be planned well in advance to coordinate with oxygen delivery and therapists’ visits; there’s no such thing for me as doing something last minute. Our lives lack their former simplicity and security but I assure you that there are still just as many moments of happiness & joy. 
 

When we are in public places, adults usually glance down at Fiona then quickly redirect their gaze, sometimes smiling uncomfortably. Children, curious by nature, always notice that Fiona is different from them and will stop what they’re doing to stare at her until their parents notice and swiftly whisk them away. In the beginning, the reactions of others used to unsettle me. Their reactions used to emphasize to me what I already knew: that our family no longer fits the mold, the false illusion of what’s ordinary.  It’s understandable to be curious about noticing something in your vision that isn’t seen often, but when those opportunities occur please notice with love & kindness.
 

Fiona had a doctor’s appointment today and as I walked through the hospital clinic I elicited a comment.  "Boy, you sure have your hands full!"  What I want to say in response is "Oh boy, and you've lost your manners!" But instead I just smile.
In fact, I have realized just how much truth there is in these strangers’ bold comments.  My hands are absolutely full!
 

My hands are the part of me that:
- brush Fiona’s hair off her face when I give her kisses
- hold and comfort her every second I have
- mix and administrate myriad of daily medications 
- feed her and bathe her
- move her arms and legs so they don't stiffen
- flip the pages to all her favorite books as I read to her
- play instruments for her to hear 
- assist her in doing arts and crafts
- wipe her tears when she gets shots
- rub her hands and feet when they get cold
- stabilize her tummy when it shakes from clonus
- suction her mouth and nose of fluids  
- provide oxygen when needed
- reposition her so she’s comfortable
- perform nebulizer and cystic fibrosis VEST treatments to clear her lungs
- capture many beautiful photos of her.
 

So you are darn right my hands are full- full of love & care & happiness for Fiona and my family.
I hope it is my hands that hold her when she leaves my arms to rest in Jesus’ arms. My hands are not burdened at all. My hands are blessed, and I am so thankful that I was chosen to be Fiona’s mama. 

 

 

Parenthood

 

 

Last night Fiona was restless.  I looked at the clock; it read 1:45 am.   I kept saying out loud "please sleep," but it didn't work.  I scooped her from the bed, placed her in my arms and then laid her right next to me.  I snuggled her in the blanket and she became calm.  We just laid there together looking into the dim light in the room.  The clock now said 3:30 am and she finally fell asleep in my arms. Maybe, just maybe... she just needed her mama.  I felt her littleness in my arms, the way her body tucked so perfectly right into my body.  I could feel her smooth cheek against mine and hear her soft breaths.  I could feel her trying to reach her tiny right hand on my chest as if she needed to be reassured one more time that I was really there.  And then I heard this in my head, "so this is what parenting is all about."  It's about love.  A love that is so deep, you can actually feel the huge space it takes up in your chest.  A love that is so overwhelming, it stops you in your tracks when you try to wrap your head around it.  A love that has the propensity to instantly swoop your mind off into a magical, timeless dream filled with sweet & precious memories of the past, memory upon memory.  A love so powerful, unimaginable & indescribable that it could only possibly be understood after actually becoming a parent.  I started thinking that I may not have these moments with Fiona when she's not with us anymore.  Waking up tired in the morning no longer mattered because this moment with her was everything.  And when it comes down to it -- all the time, the limited, bounded, finite time we have with Fiona consists of these collections of moments that we are able to share together now,  in the present moment.

A New Road

 

I am taking the very first steps onto a new road but remain on the same journey. Today we met with the new therapists and teachers who will help us transition into the special needs school system. Fiona will have completed the ‘Birth to Three Program’ the day before her third birthday and needs to continue with her existing therapies in the special needs school system. We discussed the various therapies she needs and also determined that everything will be done in our home, just like before. We can't risk exposing Fiona to the other kids with her immune system being so weak. It was a productive meeting and we accomplished much.

When I was pregnant with Fiona, I had visions of her going to school and then she would come home and tell me all about her day. We wanted to give Fiona every opportunity in the world to flourish and be a productive member of society. Like most parents, we wanted our child to be comfortable and happy.  We wanted her to have a positive self-image that would promote positive self-esteem.  We understood that a child must have a strong foundation from the beginning and felt that it was our responsibility to build this foundation for her.

It's been a very different path than I imagined for my little girl. I know it's not the end of the world. I just have to release my very personal, unrevealed dreams that I had hidden in the corners of my heart for my little girl...
 

 

Time, Patience and Love

 

 

 

As I look back around the time that Fiona was first given her diagnosis of having GM-1, I am able to share a few lessons I’ve learned through my experience.   I recall having done a tremendous amount of research online about metabolic storage diseases in order to prepare myself for the diagnosis.  Even though I tried to prepare myself, the diagnosis was still a kick in the gut and I found that really no amount of research or reading could truly prepare me for this experience these past nearly 3 years. There was that small part of me that thought the doctor would say Fiona was “normal” and that there was no need to worry, but…that just wasn’t the case, that is not what was in store for Fiona, or Paxton and me as her parents.


On the day Fiona was diagnosed, I cried and held on to Paxton. I went into our room and cried some more.  I buried my head in a pillow and screamed as loud as I could.  I was heartbroken, angry, confused.  I had read somewhere that after a diagnosis, there is a series of emotions that a parent goes through, I felt them all – and some emotions are better left unsaid.


Nothing I read prepared me for the years ahead.  Not a single book or online research prepared me for the hardships of parenting a terminally ill child or the heartache I’d feel when seeing other kids Fiona’s age on the playground down the street playing happily.  None of it prepared me for the revolving door of therapists in and out of my home or the daily care that is demanded of me.  Nothing I read prepared me for the emotional toll it takes to parent a terminally ill child nor the advocating for my child’s rights and needs.  

 

On the other hand, those books and online research also never prepared me for the excitement I’d feel when Fiona sat for the first time or for the joy I’d feel watching Fiona giggle and laugh when her father would talk to her or for the tears I cried the first time she babbled endlessly while watching Sesame Street.  In the last three years since Fiona's diagnosis, there have been so many firsts and I’ve treasured all those happy memories.  They are the memories that keep me going on the really hard days.
That’s the thing about raising a child terminally ill - in time you can find peace with it & embrace it, knowing that there is no cure for it.  But to prepare for & accept it is altogether something else.   I’ve realized that acceptance is not a one shot deal, it’s an ongoing process.  There will always be something new to accept.


I spent a lot of time preparing for parenthood and even more time preparing for special needs parenting, searching for answers to all of my questions.  Over the years, I’ve realized that you can’t prepare for it – it’s not a test you can study for. You will never find the answers searching online or in a book.  The real answers come with time, patience and love – and Fiona taught me that.



 

 

 

The Strength of an Egg

 

 

 

Fiona’s condition is stable and she’s comfortable as of now.  She is getting bigger and growing, and her seizures have been under control-some days she's seizure free.  We keep her on oxygen as needed to make sure that she stays comfortable when we see that she is struggling to breathe or when her oxygen saturation drops below 90%.  Fiona had her Synagis (RSV shot) this morning and a "weigh in." She has successfully gained a pound since her last shot 4 weeks ago and now weighs 24.7 pounds. Our house, for the most part, has been quiet. 

 

This article was passed on to me by another mom of a terminally ill child. I can really relate to it and feel it’s a good description of what I endure and feel as I face the day to day challenges that come from caring for Fiona.

 

The Strength of an Egg

by J. Freitag

Parents of children with a terminal illness are often referred to or viewed as having strength “like a rock.”  Although flattering, it isn’t quite true.  It is more like the strength of an egg.  An egg, you ask? Yes!  If you’ll think about it, you’ll see my point.  An egg has a polished, smooth outer appearance, with no cracks or weak spots visible.  It seems almost inconceivable that the inside might not be so smooth or solid. Most children, at some point are shown the famous egg trick. An egg set at just the right angle can withstand enormous amounts of pressure and cannot be cracked or broken. Yet the same egg, tapped gently at an ever slightly different angle, will break. The contents, once so neatly concealed, will come spilling out. The no longer perfect shell will be crushed. It looks so fragile that it seems inconceivable that it ever held any strength.  A rock, on the other hand, is solid all the way through.  To break it is almost impossible.  If you succeed, you will find that there is nothing inside but more rock.  It takes a lot more than pure hardness to hold the hand of hope.  Parents of [medically fragile] children are not solid all the way through.  We hurt, we fear, we cry, we hope.  It takes a very careful balancing act to keep the shell from being shattered.  “Balancing an egg” while running a household, going for doctors’ visits and hospital stays, keeping the family together, and holding on to the constantly unraveling ties of your sanity can be very tricky indeed!  Occasionally, the angle will be off and the shell will break, shattering hope and all the neatly secured appearances of a truly fragile existence.  Unlike Humpty Dumpty, though, parents of medically fragile kids will pick themselves up and put themselves back together again. 

 

 

Our Love

 

 

Last night Paxton and I wanted to see what Fiona would look like if she could stand up.  I held her up and had her feet on the floor.  Paxton held her hands while we both told her how much we love her.  We pictured her running around the house, playing with her toys, and standing right under us asking for cookies.  She brings more joy to my heart than it could hold, yet at the same time I also feel more heartache and pain than it can hold too.  I know Paxton feels the same. I could see the deepened expression settling in his face and hear the pain in his softening voice.  I can read the words lodged in his heart, the ones he doesn't let go.  I know what he wants, what he wishes for and how much he loves.  I think about what this journey has brought into our lives and what it ultimately will take away.


These are all things we never in our wildest dreams thought we would have to deal with, but in our reality it has become what we have had to learn.  You do that for your kids.  We have settled into our not so normal life, which for us has now become normal: various therapy routines,  doctors’ appointments, sleepless nights.  We spend more time with doctors, therapists, and nurses than we do a lot of our friends.  Being a parent of a child with a rare disease has a tendency to take over your social life and what results is a “not so normal to most people but very normal to you” type of schedule and routine full of health-related activities that are necessary for the continued survival of your child.  There are many things that most parents couldn’t imagine having to deal with that Paxton and I couldn’t imagine living without.  Feeding tubes, oximeters, nebulizers, suction machines, oxygen distillation machines,  doctor’s appointment after doctor’s appointment, time-consuming research and reading-it’s a lot, but it’s our life, it’s what we must do.  Not many parents have to worry about limited time with their child, but we have to accept that GM-1 will take our Fiona away from us. It’s an unbearable pain for a parent to have to live with such a prediction for their child; it’s a pain in which our hearts are broken by love, by wishes, by hope for a different outcome.

 

 

Update

 

 

Its been so long since my last update on here.  Fiona will turn 34 months in April!  It’s hard to believe that we’ve been on this journey for almost 3 years.  There’s not an hour that goes by that I’m not thanking God that our sweet Fiona is still here with us to love and care for.  She had a 2 month eye infection which had her lids swollen shut.  I was so concerned that we would not see her pretty eyes open up again.  I had come across a blog in the past of another boy with GM-1 whose eyes were shut at the end of life.  Paxton kept reassuring me that she would eventually open them when the infections were gone.  She also caught a cold and a few days later it reached her lungs.  Gangliosidosis patients always pass on from pneumonia so we aggressively treated Fiona.  She had been on oxygen for several days, breathed through a nebulizer every 4 hours, was continuously connected to a heart and oxygen monitor, and we frequently suctioned her little nose and mouth to prevent fluids from entering her lungs.  We set up our guest-room as a hospital room for Fiona with all her equipment.  Even typing this makes my heart hurt. Once again we were reminded of how precious life is.

 

Fiona is still on the ketogentic diet and 2 different seizure medications to help control her seizures, but she still continues to have mild seizures on a daily basis.  Her seizures usually begin with no foreseeable triggers.  Her face will start to twitch, her hands will clinch, and sometimes she will make little sounds.  All of her medications have to be crushed and mixed with water because she can’t have the sugary liquid medications a normal child her age would receive from the pharmacy.  Everything has to be weighed out and exactly to the milli-gram.  Tending to all these details has become our priority, our life, our normalcy.

 

As the holiday approached, I felt a lot of heartache and sadness for my little girl.  I can remember her first Christmas, when she was just a happy normal little baby.  Now almost 3 years later, she should be getting excited about Santa coming and opening presents like other kids.  We should be watching the excitement in her eyes and be able to hear it in her voice.  It breaks my heart that our sweet innocent Fiona doesn’t get to experience all of that, but she is a constant reminder of what’s important in life!  We have experienced so much love and joy with Fiona.  The smallest things are such a blessing, whether it’s a sneeze, a little movement of her hands, or her extremely seldom seen, sweet smile.  We have so much to be thankful for every single second of every single day.  Though there is heartache each and every day-there is still joy!

 

 

Angels Among Us

 

Our small community, consisting of families who have a terminally ill member, have lost four children recently, and these losses hit close to home for me.  Five angels, Kendra, Allie, Brielle, William, and Lexi lost their battle with GM-1 and Tay-Sachs (GM-2).  These children and their families survived surgeries, hospitalizations and the everyday challenges that often come with raising a terminally ill child. These parents were managing their kids’ health; their kids were stable.  Kendra, Allie, Brielle, William and Lexi's parents were seeking refuge in that stability because that meant success - that is, until the day their children were no longer stable.  The children's physical vulnerabilities restricted their ability to continue the fight and just like that, they are gone.

 

I find myself guilty of getting caught up in the day to day motions and frustrations connected to raising a child with an ultra-rare disease.  What I would define as a “normal” day for me is far from normal for many.  Many parents will never in their lifetime watch their child experience one seizure and I may see several a day for consecutive days.  It’s a foreign concept to me to ever want your child to be quiet because they talk all the time and you’re tired of listening.  I would love to listen to Fiona speak and hold conversations with her.  I would love to see her flipping pages in a book or scribbling with crayons and markers on a picture, but that just isn’t how it is for me, and that’s ok.  My experience with parenting is completely different from most, but I attempt to stay focused on positive thoughts that Fiona is doing well, all considering, and we are managing and caring for her the best we can.  

 

It is discouraging and heartbreaking when I hear of yet another child being taken away by GM-1 or Tay Sachs when a cold develops into a lung infection or the child just couldn't fight the disease any more.  Its then that the reality of my family’s situation comes crashing through.  It’s especially at those moments that I must ground myself again to what is really important.

 

I have learned an important lesson through this experience.  It’s something that I think all moms should know or at least just think about sometimes.  Its that all the other stuff-just really doesn’t matter.  Because life can be so fleeting, I am reminded to slow down and take the special and necessary time to look into Fiona’s eyes and linger a little longer as I hug her or snuggle next to her in bed.  Yes, there will always be house chores and life gets messy and busy, but I don’t know how much longer I have with my Fiona.  So I’m soaking all of it in and feeling as joyful and content as I can with what I have been given.

 

 

 

 

I Love Someone Rare

 

Wishes, Hopes and Dreams

 

 

 

I was in the kitchen baking cookies while Fiona was near me and it was just the two of us. Just for a second I could have sworn that I heard little footsteps- the sound a toddler’s foot-steps would make. I imagined Fiona being able to help me make cookies. I was baking and laughing as she splashed her hands in the flour, smiled at me, looked into my eyes and said I love you mommy. In that moment, I was flooded with occasions that I will never have with my little girl. All these flashes of non-existing events washed over me and I was drawn down into remembering what it is Fiona could never and will never be able to do. And then, as though all the color was sucked out of my world, I return back to the reality, my reality. Because that is what it is-we have lost our Fiona. The beautiful little girl sleeping in front of me in her wheel chair is really just a shell of who she was, one whose body is failing as GM1 slowly takes her away from me.  I still experience her smell, her feel, her soft heartbeat and shallow breaths, but that is all we get now.  I am thankful to still hold her and cuddle her in my arms and tell her how much I love her. 

We don't even get a glimpse of our Fiona's personality. Nothing. I keep hoping and praying that maybe this time will be a breakthrough, and I will get some kind of reaction....even a little one. But nothing. It is as if we only get to keep her body with us, not her smile or personality. 

Then I feel so incredibly guilty for feeling this way. This is my little girl, this is my baby daughter, my miracle. She is still here with us. But at the same time, she isn't really here anymore. I miss her, and I would do anything for just something- a smile, a giggle, just something to let me know my little girl is still in there. 

I'm going to crawl into bed with Fiona and snuggle with her. Maybe, maybe by some miracle, tonight I will get a glimpse of our little girl. 

 

 

Love Is Pain

 

 

Yesterday was a super emotional day for me. By bedtime, I was so incredibly spent and emotionally drained.  I can honestly say that hasn't happened in a while, at least not to that extent and it was all due to GM1. Fiona had so many seizures that I had to give her the rescue medication.  Her serious seizures are frightening for me, especially when she is gasping for air or stops breathing because I don't know if she'll recover from it. To hear such sounds coming from her mouth, whether it is a reaction from pain, sadness, or even involuntary, just scares me. The doctors tell us she's not in pain, but during her seizures my little girl’s eyes look so frightened and it makes me wonder.  To have to watch my baby go through such experiences literally leaves me feeling as if there’s a knife jammed in my heart.

 

I'm learning every day about GM1.  I absorb and process as much as I can. You see GM1 is so very complicated and smart; its  like its very own evil entity. Its goal is to rob, destroy and obliterate everything it can. I can't help but marvel at Fiona's particular rare disease.  GM1 has perfectly infiltrated Fiona’s body. The disease starts slowly and accumulates lipids in the brain then at a rapid and frantic pace the lipids intrude all of Fiona’s cells and destroy them.  So you fight back and at first the medications help, but somehow you can't get rid of the lipids and they continue to thrive in the brain and everywhere else. GM1 causes you to lose the ability to swallow, lose function of your arms and legs- never being able to walk or talk.  You take daily medication to prevent seizing, daily therapy and nebulizer treatments to prevent pneumonia, and then you lose your sight and hearing, you just lose all of your abilities.  There is no cure and a short-lived life expectancy of only 2 years.  Finally, you lose the ability to breathe.  Pneumonia sets in.  Life ends.

 

GM1 is an ugly and disgusting monster.

 

I wrote this post so people can see what some days look like in my home. This is the reality. This is the face of GM1, and its heart wrenching, horrible, and awful.

 

 

 

Surviving

 

 

Everyone keeps asking me how I’m holding up.  Honestly I don’t know how to answer that because I don’t actually think that I am holding up.  I’m just going through the necessary motions as a mother who is caring for her child with a rare, debilitating disease.  I have been closing myself off and shutting down because I feel that if I talk to my family and friends about my true and honest experience with all this and actually share with them what I’m really feeling inside, I will fall apart and never get back up.  I may not have the strength or ability to put back the many broken pieces that are left of me.  I know that my isolation is probably hurting my family, my friends and everyone else that is close to me, but all I can focus on is Fiona.  I have this steel rod of determination in my back right now to give Fiona all the things she needs. I don't ever want to look back with any regret and say we should have cared for her differently. I want to look back at her life and smile and say wow look at all the things we did in the time she was here.  She may not have been here very long, but she sure taught us how to live while she was here.  This beautiful soul was given to me to love and cherish and right now that's what I’m concentrating on doing - loving her and caring for her to the best of my ability.  So if I tune you all out and don’t call you, don’t take it personally.  If you find it unhealthy for me to hold it all in as I’m doing and wonder why I’m not crying when you think I should, just know that this is how I’m choosing to survive right now- one step, one breath at a time, with the grace of God.  I do want to thank everyone for being there and for loving and supporting us, for reaching out and lifting us up.

 

 

Return Home

 

 

Fiona is now at home.  She still has a sore throat from the breathing tube that was put in while she was sedated. It’s quite a relief to be home; we are all much more comfortable at home. I stayed with her the entire time at the hospital except for a couple hours when Paxton and I went to dinner at Ruth's Chris.  Paxton and I needed a satisfying dinner after Fiona had 7 hours of surgery and we found out that her CFS pressure was 50 cm(which is very high) and that she may need a shunt put in (brain surgery). A normal CFS pressure is 12-15 with figures less than 20 acceptable. The neurosurgeon was ready to operate on her Thursday but we decided to wait because it was not an emergency and we wanted to consider all of our options.  Wewant to give Fiona the most comfortable life that is possible - shunt or no shunt, and its tough to put her through brain surgery if she’s only got a few months left with us.  I think sometimes people look to doctors like they are gods because of how far along medical science has come and its ability to heal people.  Some people implicitly trust whatever the doctor says or don't feel like they can question a physician. Truth is our voice is all we have in this type of situation. Paxton and I are the only advocates for Fiona. This journey has forced Paxton and I  how to use our voice in a powerful way to protect our child. We asked so many questions about putting a shunt in and the pros and cons of it.  I think the doctors were quite surprised with how much we knew and the questions we asked.  Lesson learned:  Just because you didn't go to medical school or nursing school, it doesn't mean you can't ask questions, it doesn't mean you can't question some of the care you receive, and most importantly, it is actually your right as a patient to receive and demand the best of care. Our goal is to provide our little girl with a comfortable life and to do what's best to care for her. 

 

 

University of Minnesota Amplatz Children’s Hospital

 

Our family could really use some prayers this week. Fiona will be up at the University of Minnesota Amplatz Children’s Hospital for a week of testing and hospitalization for seizure management. We are seeking much guidance and action from her doctors right now, and we have some big decisions to make about the course of Fiona’s future treatment as we try to make the best choices for our little girl's health.

 

 

Birthday Girl

 

Today you turned two years old. It’s been two years since you came into this world, changing your mommy and daddy’s lives forever.  You have given us two years of the most unconditional love we’ve ever experienced.  You have introduced us to a world of physicians, health care providers, therapists, and other families that we would have never been privileged to know.  You have stretched us in the areas of knowledge, patience, perseverance, faith and grace.  You have forced us to slow down and celebrate the tiny milestones and daily activities that we might have just breezed over otherwise.  God has used you to show us the value of every life which has given us such a tenderness and understanding of just how fearfully and wonderful each child is.  What a gift it is to get to love you and be your mommy. Happy Birthday my sweet girl!
 
Love Love Love You!
 
 

Increased Seizure Activity

 

 
Most days, we get along just fine living our life as normally as we can.  I would define our “normal life” to mean daily caring for and tending to every one of Fiona’s recurring and new needs.  My definition of “normal” is ever-changing as it means doing whatever I need to do to provide comfort to Fiona, so as Fiona’s conditions, symptoms and needs alter so does our normalcy.
 
T oday, however, was really much harder than most other days.  Fiona had nine seizures and by the 5thseizure, I fell apart.  Fiona’s seizure activity up until this point has been pretty minimal with 2 seizures being the most she would have a day. Some days she would have none.  Today, more than any other day, I really really hate  GM-1.  I hate when I let it get me down.  We have come so far, yet sometimes I feel like we are still in square one with this disease.  Today is one of those (what Oprah would call) ugly cry days.  I still find myself asking "why us?" at times.  I really try to avoid feeling and thinking that way, knowing nothing good comes from it, but it’s hard sometimes to look into the eyes and face of my beautiful little girl and not think why? Why her? Why my baby girl?  There are also many moments when Paxton and I are thankful for the life God planned for her and for the amazing gift He gave to US when He chose to place her in our care.  
 
It’s a very defeated feeling to experience as a parent that you are unable to provide your child comfort or relief at a moment when they need it most.  Paxton has tried to comfort me through these rough times of her increased seizure activity, but nothing could possibly help me feel better.  From a mother’s perspective, nothing is more agonizing than watching your child suffer....When Fiona cries my natural instinct is to get to her immediately and soothe her but during her seizures, I prove to be ineffective with my motherly duty...... some days my love is just not enough to bring the comfort to Fiona that she needs; and that is the worst feeling to experience as her mother.
 
 

Mother's Day 2013

 

 

I wanted to take a few minutes this morning and wish all of the moms out there a very Happy Mothers’ Day!  Being a mother has been the most exhausting, yet rewarding and gratifying responsibility in my life. While it’s only 10 a.m. on Sunday morning, we’ve already had an eventful Mother’s Day as Fiona suffered from a seizure and has been crying quite a bit.  It would have been nice to not have to start Mother’s Day in such a manner and to not have to watch my child experience such a condition, but I’m always grateful for what I have in Fiona.  I also received an amazing Mother’s Day card and lots of snuggling - all moments that I will treasure forever.

 

My mother was able to come and visit with me for 5 days last week, and I asked my mom, “How did you deal with 5 kids and how did you enjoy me with all the tough times?”  For those of you who are unaware of my family history, my parents escaped a refugee camp from Laos and came to the United States with 4 children under the age of 6 in 1980 as they were escaping from communist control.  Their parenting experience also presented challenges as they came to a new country without knowing any English and an entire family of 6 to raise and care for-they needed to learn and adjust quickly to a very different part of the world.  My mother’s response to my question was “I chose to enjoy every moment and the happy times.”  

 

With a terminally ill child, it’s nearly impossible to enjoy days, weeks or months at a time as there is no break from fear or anxiety of what may or could happen today.   Yet it’s the moments of snuggling next to Fiona or just holding her until my arms fall asleep that keeps me going.  I feel blessed for the days that Fiona does not suffer from a seizure.  It is for these types of happy moments that I choose to focus on and remember, just as my mother did with her children through challenging times.  With my experience as a mother to Fiona, I have also grown an even greater appreciation for my own mother who made difficult times seem easy.

 

These simple, yet powerful words that my mom shared with me have helped me during hard times.  It’s inevitable that for me there will be tough times (and lots of them), but I choose to remember and soak up every moment with Fiona that puts a smile on my face.  Fiona is my lifelong bundle of joy.

 

While I’m getting ready to spend the day with my Fiona and Paxton, I wish you all a wonderful and amazing Mother’s Day, but most of all, I’d like to encourage all of you mothers to treasure the happy moments and be grateful for all that you have in your children. 

 

After all, they are what really count!

 

 

My Sweet Angel

 

 

 

I love the way her face looks when she is sleeping- so sweet, so angelic.  During the night I spend hours just lying next to her, caressing her face and body, combing my fingers through her silky hair and telling her how much her mommy and daddy love her.  

 

I find myself in a very challenging position.  As crucial as it is to relish in the moments I have with Fiona as she is alive and still fighting for her life, I also find myself needing to prepare for what is predicted to come.  I tell myself I am not ready to let her go.  I cradle her sweet face at night and try to absorb every detail of her embodiment.  Even now, knowing what her condition is, I find myself so sad at times because it may not always be like this, and I fear that idea of things changing for the worse.  Honestly, I still cry over the fact that she even has GM1 and petrified at what GM1 will do to her.  I don’t want to lose her.  The thought becomes more unbearable with each passing day because with each day my heart becomes more dependent on her.  The more time I spend with her, the more my love for her grows.  Just the mere thought of life without her brings me such gut wrenching pain.  I know that she can’t stay like this forever, but I’m just not ready for her to go, not soon, not ever.

 

But life goes on. It just does. Even though I have my moments of breaking down (usually when I am alone), I don’t let them consume my life … at this point.  I do live with a constant knot in my stomach wondering about such questions like When will it happen? What will she look like? How will we live on afterwards?  Even though our family still lives our daily lives as normally as we can, those sorts of questions are always on the back of my mind.  It’s extremely difficult not to wonder about the answers to these questions, but all I can do is my best to cope with such a painful situation.

 

I think of just how amazing Fiona is and how much beauty and happiness she has brought into my life.  She has brought me closer to God and strengthened my faith. Fiona has opened my eyes to such wonderful feelings and new insights into life.  Thousands of times over, she has warmed my heart and made me smile.

She is the missing piece I didn’t realize I needed to complete our family. Fiona gives me a feeling that’s so inexplicably good and warm.  It’s undying. It’s powerful. It’s love. Pure, love!

 

 

 

Funeral Planning

 

 

 

There can’t be a much worse thing to do than to plan a funeral for your child.   As I sat with the funeral director, I thought how unnatural it is to be looking at pictures of caskets so small.  The funeral director and I talked about touring different cemeteries to pick the perfect spot where Fiona would spend forever. My lack of sleep and appetite caused waves of nausea, and I found myself facing my reality and wishing I need not have to do this. It would have been much easier to find some sort of template for planning a funeral or have my sisters plan the funeral on our behalf, but nothing about the experience was easy. I cried for 3 days straight after meeting with the funeral director.  As days went by, I discovered that I wanted to plan everything and make it as beautiful as possible. I wanted to celebrate Fionas life. No matter how short her life will be, we have witnessed that it was been a life of value and meaning.

I’m happy I followed through and planned everything because the things that initially seemed hard ended up becoming another part of the long and painful healing process for me.  I find that as I suffer, I must also heal.  I believe it to be true that there is not greater loss than the death of your child and making the final arrangement can be almost as painful as the loss itself, but Paxton and I found a trusted funeral home and director, and we were able to make all the decisions.

Planning a funeral for our child was not something we imagined having to ever do, but I chose to complete this task because I followed my instincts and to my surprise I was able to find comfort in the “planning” of it.  I wanted the event to mainly focus on sweet Fiona’s life and what a wonderful gift she was to us and how much we love her. I wanted to be able to plan this event during a time that I am able to still capture Fiona’s living life and while she is still within my reach.  It may be an extremely difficult concept for anyone else to grasp, but it is the way and order I am choosing to handle this obstacle in my life and I am thankful for having the strength it took to get through it.

 

 

4 Pieces of Advice

 

 

 

Paxton and I learned a lot through this journey. There is no set of directions, no road map, nothing to follow.  All we can do is continue to put one foot in front of the other and do our best. We want to be certain to share with other parents, who have also had their child diagnosed with GM1 or any other rare diseases that you can and will get through it.  Here are some of my suggestions for any parent who is finding themselves in the same position as me.

1. Educate yourself! The more you know about what you are going to face, the better you can face it. We coped with Fiona’s illness by educating ourselves about GM1.  Paxton searched the internet and read as much as he could about the disease, took notes, printed out copies of various literature, kept copies of Fiona’s medical records close by (organized on our kitchen island for easy access when writing letters to our insurance).  We met with all the doctors involved with Fiona’s care and we asked a multitude of questions. We also sought out other parents and not just health care providers for answers. The more we knew about the things Fiona was going to go through, the better we felt about helping to care for her.

2. You are your child’s best advocate. You are going to know what’s best in most cases and you will work harder than anyone to get them what they need. I know Paxton read our entire insurance policy. I think he could probably submit and approve all of our insurance claims himself. Do not hand over your child’s well-being to anyone. That little person is your responsibility. Everyone thinks they know what is “best” for your child, don’t listen to them, you are the mommy and daddy and YOU know what is best for your child. Listen to your gut instinct; it will rarely lead you astray.

3. Your faith will be tested so hold on tightly to it. At the beginning of the journey, I was always worrying, wondering, fearing Fiona’s future, but I decided that I couldn’t live like that anymore. It all comes down to your faith and trust in God. I learned to look to God for strength, guidance, love and peace, rather than an immediate answer to my prayers or a cure for Fiona’s illness.  Understand the parts of the process that you are in control of and manage them well.

4. Ask for help and surround yourself with people who are positive and supportive. Ask for help if you need it, especially in those early days where everything is new and frightening. There are wonderful people out there ready to help- family members, friends, church families, neighbors all you have to do is ask. Paxton and I are so blessed to have so much love and support from people around us.

 

 

Fiona's Daddy

 

 

 

I am amazed at how wonderful Paxton is at being a Daddy. I didn't expect that my feelings towards Paxton would really change much after having Fiona. I already loved him but I didn’t quite know how much more I could really love him until I saw him care for and love Fiona. I think I fell in love with him a thousand times over just watching him be such an amazing Daddy. He was there for the diaper changes, the feedings, the happy times and the sad times. He always wants to be there with us as a family-for each little moment and I am so thankful.

 

Paxton dove into the parenting trenches with me immediately. He was just as determined for Fiona to breastfeed as I was, and he was my rock during labor and delivery. On several occasions, he's changed numerous blowout diapers, soothed her to sleep, comforted her when she cried, and did everything in his power to give her the best care possible.

 

Paxton has taken to being a Daddy like I never expected. He's loving, attentive, fun (that I knew he'd be), caring, selfless, and has developed quite a paternal instinct. Not only does he take care of Fiona when I need a break, but he takes care of me in a way that's best for Fiona. He is my strength and comfort and a shoulder to cry on; he takes over when he sees I'm falling apart. He is my partner in life.

 

Paxton fulfills a role within our family that is so much more than just going to work and providing for us.  Although Paxton and I do not always see eye-to-eye on how to parent,  we are always in agreement as to what our main priority in life is and role as Fiona’s parents.  Sure there are household/family tasks that generally fall on me and those that fall on him; however, the lines are not black and white, and we often find the need to cross over them as necessary. There are things I do better and things he does better, but we both rest assured that the help from one another is always within reach. That's what I feel is a requirement for effective parenting and what a solid partnership entails.

 

It's so amazing when I see who Paxton and I created when we gave birth to Fiona. She would not exist without both of us. A mixture between any 2 other individuals wouldn't have produced this beautiful, little girl with his eyes and my nose.

 

We are blessed to have Fiona as the center of our lives. She has given both Paxton and I a new direction for living and growing both as individuals and as a partnership within our marriage. Fiona takes care of our hearts in a way that nothing else can, and she is the light of our family.

 

 

My Church Family

 

 

 

God has blessed me in so many ways throughout my life. One of them is placing me in First Evangelical Lutheran Church. When Paxton and I moved to La Crosse from Chicago, we were looking for a church to join and we visited several churches in the area. I knew I wanted to join a church that gave me that same feeling that my parent’s church does, which is a very genuinely warm, loving, and caring environment.  The church that my parents raised me in is Peace CRC in South Holland, IL: the church where I was baptized, where I professed my faith as I got older, where I attended catechism & Calvinettes and eventually taught Sunday school, where Paxton and I got married.  Because of my great experience with Peace CRC, I knew what I was looking for when picking out a church in La Crosse. 

 

The first church Pax and I attended was First Lutheran Evangelical, and I kept finding myself coming back to it after visiting the other churches.  I knew what I was searching for within a church body and I found it at First Lutheran. The church isn’t about the physical building, although our church is quite beautiful.  Rather, the church is about the people - the group of men, women, boys and girls, young and old, that makes up the body of Christ. God has put us together each with our own talents and strengths, and I feel as if God placed me here for a reason.  I feel the love of First Lutheran Evangelical when we worship together and have seen and experienced the love of my church in times of need.

 

The congregation has become our church family and has been with us through all the happy (Fiona’s baptism) and sad times, always extending God’s unconditional love to us. When I need prayers for Fiona; all I have to do is place a call and in matters of minutes, my church is in action, praying for Fiona and doing what they do best- showing her God’s love. Being a member of a church family of believers here at First Lutheran Evangelical has been a joy and blessing that I am very thankful for.

 

http://www.peacecrc.org/

 

Daily Medications - March 1, 2013

 

 

 

I have families who have seen Fiona’s website and contacted us because their child was recently diagnosed with GM1. I wanted to list the Medication Therapy Fiona is getting at the University of Minnesota. Her physicians are Dr. Chester Whitley and Dr. Jeanine Utz, who are angels sent from above. 

 

Treatment

3x100mg Mislustat (Zavesca) 

1x600mg Acetylcysteine

2x3ml infant Ibuprofen

 

Seizures

2x1.2 ml Keppra

 

Rescue Seizure Medication

Diazepam As needed

 

Other

1 ml Polyvitamin Drops

1mg Tolteodine Tartrate to help with urination

1ml Sodium Fluoride to help weakened teeth

3x1ml  Zantac (to protect her stomach lining from Ibuprofen)

720 ml of Ketocal 4:1 formula. Boulous feedings every  2  ½ hours

 

 

 

 

Ketogenic Diet - March 1, 2013

 

 

 

 

Fiona started on the Ketogenic diet.  The Ketogenic diet is a high fat, adequate protein, low carbohydrate diet, primarily used to treat difficult-to-control epilepsy in children.
The diet mimics aspects of starvation by forcing the body to burn fat rather than carbohydrates. Normally, the carbohydrates in food are converted into glucose, which is then transported around the body and is particularly important in fueling the brain.  If there are very little carbohydrates in the diet, however, the liver converts fat into fatty acids and ketone bodies.  The body produces ketone bodies in a state known as ketosis.  The ketone bodies pass into the brain and replace glucose as an energy source.
The reasons Dr. Utz wants Fiona on the ketogenic diet are:

 

1) Zavesca interferes with how the body digests and processes carbohydrates. Lactose can interfere with Zavesca, which is why Fiona can’t have dairy. Paxton and I have been very careful about what Fiona’s diet consists of.  Her diet has always been low in carbohydrates and with breast milk there is a very small amount of carbs. KetoCal is a low-carb formula prescribed to epilepsy patients. Fiona's diet now consists of Ketocal mixed with breast milk.

 

2) It may help reduce her seizure activity.  For a period of time, Fiona was having mild seizures that occurred once or twice a week. Kappra has been successful in controlling her seizure activity,

 

3) The ketogenic diet may help increase the amount of Zavesca that reaches the central nervous system, which is the most important reason for putting Fiona on this diet.  Pharmacokinetic studies indicate that only about 25-30% of the Zavesca dose reaches the brain. A study in adult Sandhoff disease mice showed the combination of a ketogenic diet with Zavesca resulted in significant reduction in GM2 brain content and a 3.5-fold higher cortex Zavesca brain content compared to mice on a standard diet with Zavesca.

 

The doctors commented to Pax and me the other day that we were doing a great job caring for Fiona, who looks great considering all that she’s gone through.  At this point, however, I don't feel as if it matters how great of a job my best has done for Fiona when my best just simply isn't enough to heal her. It's really wearing heavy on me to see what GM1 is doing to my little girl.

 

I now find myself at a weird crossroad.   I want Fiona to get better but I have to ask and always consider at what costs it will take for her to feel better and whether or not it’s the best thing for Fiona. The treatment and the diet are not guarantees that it will all pan out smoothly for Fiona, and I have a lot of apprehensions.  What if none of this works and all she will ever know about life is what she has experienced up until now?  There is a tremendous amount of guilt associated with it all.  At the moment, I feel emotionally drained.  I know I need to be stronger... I think that has been a recurring theme in most of my updates, but never in my life have I had so much weighing on me and some days I just feel like holding her forever and never letting her go…

 

 

Exhaustion - March 1, 2013

 

 

 

I am physically and emotionally exhausted.  Parenting can be an exhausting responsibility for any parent, but parenting a terminally ill child takes things to another level of fatigue.  Between doctors’ visits, feedings through her feeding tube, administering medicine, and tending to Fiona’s every need and move (not to mention the time it takes to research and understand this disease) there is just no break for me.  Not only am I taking on the responsibility with so little knowledge going into the process as I’m no doctor or pharmacist, but I’m doing my best to act flawlessly as her mother and nurse as any minor mistake or misunderstanding can possibly result in a disastrous situation for Fiona.   I’ve accepted that my life demands such a constant feeling of emotional and physical fatigue.  Hospital and doctors' visits are not just a few times a year; they may be a few times a week. Imagine a list of meds and administration times longer than your grocery list every day. Instead of calling friends, you are mostly calling doctors. Picture frequently handing your child over for tests and surgeries to complete strangers while you sit and watch the clock move at a snail’s pace, not knowing if your child is going to wake up the same person as when they fell asleep.

 

My days revolve around caring for Fiona all 24 hours, feeding her every 2-1/2 hours, pumping, positioning her to sit a certain way so she's comfortable, advocating for her, and trying to learn everything that I can to ensure Fiona is getting the best help available. And sleep... That is almost non-existent.

 

I am no different than any other mom. We don't just hate it when our kids are sick- it hurts us. However, any mom called to this journey can and will do it, because there is no choice.  As a parent, you give everything you have to make sure your child is happy and healthy- no matter the cost to you.  You make the adjustments out of love, and you realize that now your life is just different.

 

There are plenty of days I wish for my little girl to be healed, to not suffer, and to be able to just be a kid. That being said, I wouldn't trade her for anything! She has a perfect soul and is an unbelievable gift granted to her daddy and me.  I love her so much.  She is a warrior and my hero.  I am immensely blessed to be Fiona’s mommy!

 

 

Missing Our Bond - March 1, 2013

 

 

 

One of my favorite bonding experiences with Fiona was being able to cuddle for hours and just stare at each other.  Paxton would say that Fiona lived on my shoulder all her life.  She loved being held on my left shoulder and I loved having her there.  If she was fussy, crying, or needed me all I had to do was put her up on that left shoulder of mine and that immediately soothed her.  Paxton and I always felt that for a nonverbal child, she is incredibly expressive.  You would always know what she was feeling because of her eye movements and facial expressions.  It is completely unfortunate, but Fiona is no longer able to be expressive; that is completely gone now- all of it.

 

Fiona’s eyes are now both just staring into heaven. She can still move them to the right or left when we talk to her and up and down; but that’s about all she is capable of doing right now.   I know that she still reacts to light, but I’m mostly concerned about the overall decline and her inability to be expressive entirely.  I’m worried about what it all means in terms of what is really going on? We also wonder if she can still see us. Could she have lost part of her vision or even all of it? We just don’t know with this disease, and to have all these unanswered questions is very difficult and heartbreaking as a parent.   

 

There were times in the past that I could put my face strategically right in front of hers and we would make eye contact, but that’s no longer the case; it just doesn’t happen anymore, and I deeply miss that interaction between us.  Fiona is presently using her eyes similarly to how a blind person uses theirs, but whether or not that is the actual case, we just don’t know.  There are those rare times, however, where I will lay her on her side and let her watch Sesame Street on her iPad.  It’s only a matter of 1 or 2 minutes before I realize that she just isn’t interested in doing so any more.  I remember when she used to love watching Sesame Street and would react to Elmo, Abby, and Grover.  Her mouth would open wide with excitement and sounds would come out.   It’s been about two months now since she has last made eye contact with either one of us.   Now it is almost as if she looks right through Paxton and me, she is just no longer making that connection with us and I miss it, badly. 

 

It’s been extremely difficult for me to no longer make that eye contact with Fiona and to be able to engage in that kind of experience.  I feel like my only way of having two-way communication with my baby girl is now gone.  Although Fiona and I never did have a conversation with words or signs, I feel as if we were still able to communicate to each other through our eye contact.   Losing that bond with her now, not being able to have her really see into my eyes, or hold her on my shoulder, or nurse her and tell her how much I love her and how much I hurt for her feels so unfair. This disease is so damn mean and cruel! 

 

 

 

New Year's Resolution

 

 

 

 

 

 

My new year’s resolution is to write more blogs.  I’m aiming to write once a week but that may be a bit challenging depending on how Fiona is feeling, so at the very minimum I will do my best to write twice a month.

 

I experienced a heart stopping moment when we found out Fiona had GM-1, but nothing could prepare me for watching my daughter experience her first seizure in late October, which caused a decline in her overall health.

 

No training, literature or insight can prepare you for the horrendous, gut-wrenching experience of watching your child suffer through a seizure.  Although I have now experienced seeing Fiona suffer through a multitude of seizures, every time a seizure occurs it still feels like the first time.  There is an overwhelming sense of fear, helplessness, danger and frustration that comes with the inability to stop your child’s suffering while also witnessing the visuals of what is happening within Fiona’s little body.

 

We were aware that seizures were likely to occur with GM-1, so I always had that worry in the back of my mind.  Not knowing when the first one would happen, what it would be like, and how I would react was always a huge concern of mine.  I talked to other mothers concerning this topic, and I thought I had a decent idea of what Fiona’s first seizure would be like.  Little did I know that the reality of it all was nothing I could have imagined or been prepared for.  No matter how much knowledge I had going into the process, I will never forget the first encounter and all the dreadful emotions that came along with it.

The night of the first seizure, Fiona was fussy all night and was in some sort of pain. I held her through the night and continued to nurse her, but my maternal instinct knew something was not right. The next morning she ran a fever, and around 6:00 her lips turned blue. She completely stopped breathing-was flaccid and lifeless.   I was in a panic and screamed out to Paxton who came running into the room where Fiona and I were in.  He yelled at her name in desperation and slapped her cheek.  I heard the panic and fear in Paxton's voice, and felt so incredibly helpless as I watched my sweet baby girl in such a state....finally after about a minute she came back to us and started to breathe again.  Fiona’s entire right side began to twitch, and she twitched the entire time until we made it to the ER.  Our local ER refused to see us, so we had to drive 80 minutes to Rochester MN – to the Mayo Clinic ER.

 

I’ve been extremely concerned about Fiona’s health these days ever since the first seizure.  I experience an ample amount of anxiety worrying about when and where the next seizure will occur.  This week she's had mild seizures which last for 5-10 seconds.  That may seem like a short time frame, but for me it certainly feels much longer.

 

I will admit that now after witnessing Fiona having to experience all these seizures, I’m angry and have grown to hate this disease more than ever.  Although it’s a discomforting and frightful process to have to watch as a mother, I’m not angry about what I endure- I’m angry about what Fiona endures.  I find my heart breaking more and more each day being the person who so intimately shares with Fiona the negative effects of GM1.  I’ve noticed how deteriorating this disease has been to Fiona’s health.  I have no other choice but to fight this fight with Fiona and offer her all the strength and comfort I possibly can.  All I can do is take it step by step and day by day, and  sometimes hour by hour. Regardless of the sadness this disease brings into my life, it will never surpass the amount of joy that my little heroin has given me and will continue to give me for the rest of my days.

 

 

 

 

A Mother's LOVE Sunday - December 30, 2012

 

 

 
I’ve been thinking about what a mother is.  What she does.  What makes a mom a mom?  And then I came across this poem that to me is exactly what a mom is. Simply true and so beautiful.  Exactly what my mom was and still is to me.  And exactly how I hope to be seen by my children.  
 

 

A Mother’s love is something
that no on can explain,
It is made of deep devotion
and of sacrifice and pain,
It is endless and unselfish
and enduring come what may
For nothing can destroy it
or take that love away . . .
It is patient and forgiving
when all others are forsaking,
And it never fails or falters
even though the heart is breaking . . .
It believes beyond believing
when the world around condemns,
And it glows with all the beauty
of the rarest, brightest gems . . .
It is far beyond defining,
it defies all explanation,
And it still remains a secret
like the mysteries of creation . . .
A many splendoured miracle
man cannot understand
And another wondrous evidence
of God’s tender guiding hand.

 

~Helen Steiner Rice

 

 

Thank You Sunday - December 30, 2012

 

 

Paxton and I just really want to say thank you to everyone who has offered to us their love, prayers and support! This year has been a trying one for us. It´s been an extremely difficult time for our family, yet there have been so many gestures of love and support from all of you out there! You have all acted as such wonderful Gifts of God to us. Thank you all for joining us in this journey.

Pax and I are amazed at the number of people who don’t even know us who have reached out to us! We are so grateful to everyone, whether you were a stranger, friend or family member; you have all done things or said things that have been very helpful and meaningful to us, things that I will always treasure.  God is faithful and so good to us in a number of ways. People we have encountered on this journey have been an example of God’s Goodness and Love.

The examples of love that I have received from others has made me want to be a different person…better somehow…in some way. I hope one day Pax and I will have the opportunity to give back all of the kindness and generosity that has been shown to us. 
 
Thank you, thank you, thank you to each and every one of you who stand by our sides and keep us standing through this trying time!!!

Fiona's Update Sunday - December 30, 2012

 

 
 

I know it’s been a while since I’ve written a post. So much has happened with Fiona that I don’t’ know where to begin.

 

Fiona had an awful decline after receiving the MMR, DTAP, flu & varicella vaccines on 9/28/12. Within 24 hours symptoms occurred. We were unable to elicit a smile or giggles from Fiona, her appetite decreased and she was losing weight drastically, her eyes rolled up into her head, and when awake she whimpered for hours. A large red lump appeared at the injection site. She no longer slept, and she began to have difficulty breathing. Many doctors examined her. No answers.

 

Three weeks after the vaccinations, Fiona had a massive seizure. No hospitals in town were willing to see her. We drove to The Mayo Clinic ER and carried our convulsing daughter up to the window. She was admitted immediately, but her condition continued to deteriorate. She was stable, but lost the ability to eat.

 

Paralysis has set-in. Her hemoglobin dropped to 50% of normal. 15 different doctors tried to put an IV in her for 24 hours but couldn’t find a vein. She was rushed into surgery to have a tube implanted into her chest leading to her heart. IV nutrition was started along with a blood transfusion. Several days later we implanted a feeding tube into her gut and cinched her esophagus shut to prevent food from reaching her lungs and a pneumococcal infection. Through all the surgeries Fiona was a fighter! She knew we were at her side, fighting along with her and loving her. I barely left her side at the hospital and snuggled next to her in bed every night. Paxton would hold her hand and she would instantly wrap her fingers around his – that was all she could move, just fingers. Paxton and Fiona have always had this connection since she was born.  After 17 days in the hospital, we came home. She’s been back to the hospital a few times since, but she’s generally stable with some lower grade seizures periodically. Fiona is still paralyzed and can no longer see, smile, giggle, move, or even play.

 

It’s difficult for me to see Fiona in the state that she’s in. It breaks my heart that she has to fight in order to live. I wanted to get angry with God and shake my fist at the unfairness of it all, but I know better. When you have a child that’s dying, you see how unfair life can be; it’s a consequence of living in a fallen world. Because I believe in God, am I to be spared tragedy? No, not even Jesus Christ himself was spared. Paxton and I are left with no answers and must keep moving forward with Fiona’s treatment, to keep fighting and to hold on tighter.

 

I spent so much time worrying and being upset that I couldn’t even enjoy my life, but this is the reality of my life for now.  It’s often a very difficult pill to swallow, but when I give myself a moment to put things into perspective, I am able to regain the strength and motivation necessary to keep fighting for my baby girl.

 

Fiona fights so hard every day that she deserves to wake up to a mom who is happy and smiling. I need to be that mother regardless of how hard it is. I will savor each moment with her like it’s my last, which is how I should have been living in the first place. Paxton and I are committed to making her as happy and as comfortable as possible during this time.

 

 

 

 

Welcome to Holland - by: Emily Perl Kingsley Sunday - December 30, 2012

 

 

The beautiful Emily Perl Kingsley wrote a short explanation in her tremendously touching poem “Welcome to Holland”. This story of a family planning a trip to Italy helped me a lot when I was feeling down. It was a family that dreamt for years about making it to Italy. They bought books, learned the language and planned every detail of their trip. When the day of the trip finally arrived, they felt really prepared! When they landed, the flight attendant’s announcement welcomed them to Holland. “Holland???”  said the family, “what? We’re not in Italy? But we have everything set for Italy!” Needless to say, to land in Holland was not what they expected and their journey turned into something different-with sights, people and places unlike what they had hoped for, but still amazing and a true learning experience. Our family’s journey since Fiona arrived has been much like this family’s trip to Holland: unpredictable and new, but oh so very fulfilling and blessed with wonderful surprises.

I am often asked to describe the experience of raising a child with a disability and/or special need- to try to help people who have not shared that unique experience to understand it and to imagine how it would feel. Just as in Emily Kingsley’s poem, where a family planned for and made all the preparations for a trip to Italy yet landed in a very different location where all of their prior research wasn’t as applicable as anticipated, so is the story of what it has been like parenting Fiona.

It's like this......
When you're going to have a baby, it's like planning a fabulous vacation trip to Italy for example. You buy a bunch of guide books and make your wonderful plans to see the Coliseum, Michelangelo’s David, gondolas in Venice. You may learn some handy phrases in Italian.  It's all very exciting, you feel extremely prepared after having done all the research necessary and creating an agenda that covers all that you want to experience in that new country.

I feel like my experience with mothering Fiona has been similar to the family’s experience in Kingsley’s poem.  After months of eager anticipation, you expect one thing and have been given an experience that is entirely different from what you anticipated for months.  The experience that you dreamed of for such a long time was simply entirely different than what you could have imagined and prepared for. 

Although that family was still able to be on a vacation, it wasn’t at all what they signed up for.  After all, the family did not land in a horrible, disgusting, filthy place, full of pestilence, famine and disease….they simply landed in a place very different from what they anticipated and expected but still very beautiful with so much to offer.

So what’s next?  How do you act when you’re given such a change in plans?  Well, you keep moving forward- you must go out and buy new guide books and learn yet another new language.  You will find that you have experienced new things and have met a whole new group of people that you would have never met otherwise.  You focus on the positive aspects of your surprising trip and do the best you can with what you have been given. 

So now what? You find yourself in a different place than you expected and you make note of the differences, sure Holland happens to be slower-paced and less flashy than Italy, and maybe the pizzas and pastas don’t quite have that authentic Italian taste you were anticipating, but so what?  After you've been there for a while and are able to catch your breath and take a good look around- you begin to notice what Holland has to offer.  Holland has windmills and is filled with tulips; Holland even has Rembrandts.  Although Holland has its differences from Italy, it’s still a beautiful vacation none the less.  

In the meantime, everyone you know is busy coming and going from Italy... and they're all boasting and bragging about what a wonderful time they had there, as they have every right to.  But then you realize that although that was the destination you were planning for, that was not the destination you landed at, and for the rest of your life you will be the person saying "Yes, that's where I was supposed to go. That's what I had planned."  You had a dream to go to a specific place yet simply landed somewhere different.  

The pain of that truth, that my experience with parenting a special needs child has been very different than the experience that parents with perfectly healthy children have.  The pain that I suffer from knowing that Fiona does not have the health and capabilities of a healthy child her age will never, ever, ever, ever go away...because the loss of that dream for any parent that is going through this similar experience is a very,  very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things… about Holland.
 
 
 
 
 

From A Daddy's Heart

 

 

Paxton King-

 

Had some choking this evening and what looks like some pus and bubbling coming around the outside of the G-Tube port but the doc's say the tube port looks like its healing well with no infection. Seems odd to me that a white thick substance that bubbles a bit when she sneezes is normal, but that's what they tell me (if you can tell me otherwise, please do) She's been a little fussy as we put 85 mL into her over 20 minutes every 2 1/2 hours + med's (I think there's still about 10/day). I held her tonight for 2 hours and spent a lot of time looking into her eyes, but could never coax a smile from her. It just seemed she was content to be held by daddy (and hold my hand too). But no IV, no blood pressure meds now for 4 days, and we're talking about pulling the PICC line tomorrow and we've been discussing - just discussing - GOING HOME!!! She is not the same little girl from 30 days ago when she giggled and laughed and ate her meals from a spoon held by her mom. She can only move her eyes, eye lids, & her lips. We see the occasional cough, yawn and sneeze and periodically some choking although there is very little in her throat upon which to cough. I so miss my more animated daughter who would smile when she heard my voice & saw my face... But a daughter at home is FAR better than a doctor in a hospital. Today was my 4th weekend straight in a hospital, and our 17th straight day comes tomorrow as inpatients at Mayo Clinic. I am thoroughly drained. It’s like working 16 hour days for 4 weeks straight, then night you find a bed for 6 or 7 hours and grab a shower & coffee and do it again. And if I have to eat another slice of cafeteria pizza... Ugh! I SO need a break, as does Dee who rarely leaves Fiona Midori’s side. I just hope when this day comes to bring her home and we carry her into the house and place her in her favorite spot and tell her we love her that maybe, for a brief moment, we will see some happiness and a smile on that little face. When our minister came to pray over her, her eyes just lit up and she looked around the room. I still wonder what she saw? But we hope so very much that she wants to come back home for a little bit longer to be loved a bit longer by mommy and daddy before GM-1 extinguishes her light and she is granted her wings to fly high. I looked deep in her eyes tonight and asked her never to forget how much she’s loved and that daddy tried so hard to help her and save her, but just couldn’t. Asked her to remember how special she is and that she will be so badly missed when she leaves us. So… tonight Fiona is stronger than she’s been in weeks. She is quiet, but vitals are good, and food intake is reaching levels from pre-vaccination and pre-seizure (liquids only of course). They prayers have helped. Let’s get the little one back home soon…

 

 

 

Condition Summary 9-18-2012

 

 

 

 

Some detailed analytical results were reviewed during Fiona’s follow-up visit to Minneapolis in mid-September.  Although the analysis of her spinal fluid is not yet complete, results of her eye exam and MRI were available for review & discussion.

 

Fiona’s eyes are grossly unchanged.  Her optic nerve still shows mild nerve atrophy which was no worse than her April check-up.  Her cherry red macular spots appeared identical to April’s exam as well, indicating no further deterioration of her vision.  Auditory testing conducted locally to La Crosse has seemed to indicate no additional loss of hearing as well.  This was all good news and seems to indicate Fiona’s medications have halted the deterioration of her senses.

 

MRI results did show atrophy of Fiona’s brain.  Her brain mass has increased disproportionally to her age which we saw in Dee’s friend’s little boy in LA California, who sadly, passed September 29th.  Our neurologist believes this is due to accumulation of substrate (lipids) throughout the brain which have increased its mass – no way to know for sure though.  On MRI we can clearly see that the brain now lacks much of its fluid gap between the skull and brain surface, meaning the brain is so large it is nearing the skull.  The MRI also showed some lipid buildup in specific locations within the brain as was indicated in April. 

 

The hypointensity within the splenium of the corpus callosum (the bundle of nerves that connect the two hemispheres of the brain) has slightly diminished since April suggesting a slight loss of myelination of Fiona’s nerve cells.  We learned that loss of myelination on nerves is akin to loss of insulation on an electrical wire.  Signals in those wires will travel slower, and at some point, they will not travel, as myelination breaks down.  Myelination of nerves occurs in the first 4 years of life, so it is discouraging to see it breaking down at this age.  GM-1 Gangliosidosis can break down myelination and the doctors are not aware of methods to build it back up, stating, “it is the subject of much research.”  We discussed diseases such as Leukodystrophy and Multiple Sclerosis which chiefly involve destruction of myelination and subsequent paralysis.  Symptoms of Leukodystrophy are loss of body tone, movements, gait, speech, ability to eat, vision, hearing – sounds kind of like GM-1 doesn’t it?

 

Of course we also discussed parental observations of Fiona on a daily basis – a reason for optimism.  What Dee and I have seen is continued increased awareness in Fiona of her surroundings compared to her condition in April and May.  I can elicit a smile from my daughter in under 30 seconds whereas in May, she was completely unresponsive to outside stimuli.  If Fiona is given attention and stimulation for 45 to 60 seconds, she is usually giggling, grinning, and starting to use her voice again.  On 9/16 I saw Fiona lift her head and use her neck muscles when she was upset.  Previously we’d only seen use of those neck muscles when she was happy, so this appears to be an improvement as well.  When these observations were discussed with the treatment team, they said other parents in the program have reported similar improvements.

 

We will add an additional drug aimed at further protecting her nerve cells from damage over the next 4 weeks and will evaluate her again in Minneapolis in December.   As of this morning, Fiona had a great night’s sleep and is wide eyed and giggling this morning – a happy, happy girl in her mother’s arms.

  

I once read that having a child with a terminal disease is like a roller coaster with constant ups and downs….that is a perfect description of what Paxton and I are experiencing and feeling right now.  After receiving the news of Fiona's subtle decrease in myelination, we were saddened.  We will continue fighting this relentless nightmare of a disease, but Paxton and I do feel a bit defeated – there’s seemingly no way to fight myelination loss.  Although we have not “lost” our baby angel, the mere thoughts of it, again, turned our lives upside down.  Receiving this news within her updated reports has really impressed upon us the real facts involved, facts that weaken our hope and hint at potential future defeat; if the loss worsens, Fiona won’t have a very long, nor pleasant future.  Paxton and I admitted to each other that we were tired, and I do not mean a sleepy kind of tired; rather, an emotionally and mentally draining kind of a tired from fighting GM-1.  We are both willing to admit that we are exhausted from the draining effect this disease has had on our well-beings. With that admission came a lot of guilt, but it felt good to say “I’m tired."  The next few days were just as tough as we struggled to stay positive and to be strong.  It appears we have reasons to be encouraged as well as reasons to be discouraged.

 

So much is in God's hands and so little is in ours that I need to let go. My daughter is as happy and healthy as she can be today, and that is all I am going to get right now…..It needs to be enough.

To Fiona with Love - Sunday August 26, 2012

 

 
 
Dear Fiona,
 
Today you are one year old. Exactly one year ago, God gave us a
beautiful little girl-God gave us you. Last year on this very day, on
June 8th at 7:20am you were born, and with you came magic that Daddy
and I never knew existed. I hope you will never forget how much you
were wanted and how happy you made us-just by being you. You have
embroidered our lives with a meaning that cannot be expressed in
words. You are an absolute miracle of love.
 
Since the day we saw your little heart flutter inside of me, you have
brought great joy to our lives. You have taught me so much about love,
life and motherhood. You have completely changed everything about me-
the way I view the world, the way I understand life, my ability to
love deeper and stronger without bounds. You have even caused my love
for daddy to deepen in new ways and to new heights. You have taught me
to trust God, even when it seems impossible. You have taught me that
five minutes of snuggling with you are more important than anything
else. You have taught me to fight for what I love with all that I
have. You have given me strength that I never knew existed, faith that
doesn’t falter, and courage that allows me to face the most difficult
of times.
 
I appreciate the smallest milestones that you reach, and I celebrate
how far you have come every day. Your smile and laughter make me look
forward to each and every day. I am a better person today than I was a
year ago, all because of you.
 
You bring so much joy into our lives.
You are so precious and so loved!
 
Happy First Birthday!
 
 

Photos by Kim Anthony - Wednesday August 22, 2012

 

 

 

 

Below is a letter that Paxton wrote to Kim Anthony, a professional

photographer, in which he thanked her for the photos she took of us at

our home.  I wanted to post this letter in order to share Fiona’s

father’s perspective on things as well.

 

Paxton is an amazing man, wonderful father, and a thoughtful husband.

We have been through so much together this year and he has always been

my rock. Without him, I am not sure I would survive this journey.

Throughout everything we have come across, he has always been my

strength, comfort, happiness, and love- really my everything for the

past 9 years and I feel blessed to have him right by my side.

 

Paxton wrote:

"Kim, as I think back over my past 38 years, so many special

individuals have passed into and out of my life and during those

moments when we were together, some of those individuals touched me

very, very deeply. Some of those individuals changed me forever and

taught me lessons about life and love, even though, now, they are long

gone. Never did I imagine that in such a short time, my only child

would be the most important of these individuals who would touch me so

briefly and teach me such deep lessons, never uttering a word. Never

did I dream that my daughter would pass before me for such a fleeting,

short time and be one of those individuals who passed into and out of

my life. Parents are not supposed to outlive their children.

 

You have now passed into my family’s life and you have given myself and

Dee a gift that will help the memory of Fiona live for many more years. You

captured special moments in our home, of our special daughter, that in

a brief time, will be forever gone; but yet, the memories aren’t gone,

because Dee and I will be able to cherish the time you devoted to help

the special memories of Fiona endure. We will be able to recall a few

special moments that you captured.

 

I still do not know exactly why Fiona was put into our lives, but I search

for that answer and maintain a journal of what she has unknowingly taught

me. You’re right, she does change your outlook... Suddenly you realize how

important the simple things are... how reaching out to help that

little one bring her thumb up to her lips just brings her utter

elation! And you’re right, she is a blessing, as is every day we are

able to share with her. And in the same way you’ve thought about her,

she consumes us – we think about her all day long. She is my first

thought as soon as I wake, and my last thought as I fall asleep.

 

Your boy spoke of how he hopes she’ll get better and his comment brings to

mind an image I often see in my mind that is similar to the photo you

took of the 3 of us with the meadow in the background. I dream of my

young girl happily running through a similar field of grass, arms

outstretched as she runs towards me... I have that image etched in my

mind but will never see it on this earth... Fiona will affirm what is

important in life – family, kindness, thoughtfulness, faith, hope and

love. I know my faith has been challenged and I know at some point,

all of my hopes for Fiona will finally be dashed… But we will always

have memories of her love – and be reminded of it through your photos.

Thank you, most sincerely, for what you gave to my family."

Fiona’s Progress - Wednesday August 22, 2012

 

 

 

Fiona is better in some respects, and worse in others. Her sense of
hearing is pretty badly damaged. She can hear loud noises, so we turn
up the stereo pretty loud for her and she'll smile.  We have learned
that one of her favorite songs is the Miami Vice theme song.  Fiona
was losing weight, but we added some corn oil to her diet and she is
now gaining weight. We will have her brain checked later this month
and will then compare the images to the test results from April to see
if the lipid buildup has changed or stayed the same. She has pretty
much lost use of her legs - we just see them twitch now. She can only
move her legs while they are under water (like while she is bathing).
She can lift her arms to chest height, but then they fall back to her
sides. She cannot grab objects any more or hold anything; she used to
be able to pick stuff up and put it in her mouth. 
 
Fiona is much more  cognizant of her surroundings and of Paxton and me. 
She looks at us  more than ever and makes a lot of eye contact again and
is able to  show emotion, which she was not able to do 2 or 3 months ago. 
I can  talk to her from across the room and she’ll smile. I can even get her
laughing on queue now.  In short, regarding Fiona’s progress in
appears that parts of her brain have gotten worse and other parts have
improved.  Although we are always grateful for her improvements and
progress and saddened at any regressions, one fact remains, we are
joyful that Fiona is here with us today!
 
So that’s where we are.  We head up to the University of MN at the end
of August for 3 days of testing. Hopefully then we’ll have a good idea
if the medications are working.  I still cry through any major medical
procedure; I just can’t get used to seeing her little body go through
all these tests. You would think that after all this time I would be
used to it.  A part of me dreads the upcoming tests. I just so
desperately want to hear good news and am terrified of all the
possibilities…please keep Fiona in your prayers.
 
 
 

My Experience With Grieving - Wednesday August 22, 2012

 

 

 

Even if you are not the mother of a terminally ill child, I am sure
you would agree that minor grief sometimes plays a part in the
emotional journey of parenting. Mothering a special needs child may
cause one to experience a very rare type of grief, this is true for
me.
 

I have learned that the 5 stages of grief are denial, anger,
bargaining, depression, and acceptance. Imagine all of these stages
of grief being thrown at you all at once and all the time. This is
what I feel grieving has been for me. I do not feel as if I have
experienced “stages” of grieving; rather, I feel as if I am
continuously experiencing a non-stop cycle of grieving without any
type of order or end. My soul grieves deeply for my baby and I can’t
imagine it will ever stop.
 

A few weeks ago, I saw an adorable little girl who looked about
Fiona’s age. The little girl was dancing alongside her mother so
happily, so effortlessly, so freely- she was so healthy. I was envious
of the two and had quickly found myself with tears running down my
cheeks. I mourn for the fact that Fiona does not have the health of
an average child. Fiona will never know the freedom that comes with
being a toddler or child who can dance and move freely.
 

I don’t want anyone to know the sadness in my soul; I do not want to
expose my weakness at a time when I’m demanded to be strong. But I
can only be so strong before the depths of grief become so raw and
intense that I lose control and breakdown. Such is the case when I
begin to think about all that Fiona has gone through and will continue
to go through during her fight with GM-1.
 

I am trying to see the beauty in this journey. In spite of any and
all of my sorrow, I am trying to be joyful for having this journey. I
am able to recognize, though it takes a tremendous amount of sought
after optimism, that today I’m happy because Fiona is with us and
doing well in this moment.

 

 

The Special Mom, by Erma Bombeck - Wednesday August 22, 2012

 

The Special Mom, by Erma Bombeck

I want to share my favorite Erma Bombeck writing about "special" Moms
in honor of Mother's Day.
I must note that I disagree with Erma on one part of this piece- that
we (mothers of children with special needs or terminally ill children) are not saints; I believe that
we are just doing what any parent would do. For the most part, Erma
has perfectly described all that God has shown me in my very special
daughter. Happy Mother's Day!

Most women become a mother by accident, some by choice, a few by
social pressures and a couple by habit. This year, nearly 100,000
women will become mothers of handicapped children. Did you ever wonder
how these mothers of handicapped children are chosen? Somehow I
visualize God hovering over Earth selecting His instruments for
propagation with great care and deliberation. As He observes, He
instructs His angels to make notes in a giant ledger.
“Armstrong ,Beth: son: patron saint…give her Gerard. He’s used to profanity”
Finally he passes a name to an angel and smiles. “Give her a blind child.”
The angel is curious. “Why this one, God? She’s so happy.”
“Exactly.” says God. “Could I give a child with a handicap to a mother
who does not know laughter? That would be cruel.”
“But has she patience?” asks the angel.
“I don’t want her to have too much patience, or she will drown in a
sea of self-pity and despair. Once the shock and resentment wear off,
she’ll handle it.”
“But Lord, I don’t think she even believes in you.”
God smiles. “No matter. I can fix that. This one is perfect. She has
just enough selfishness.”
The angel gasps. “Selfishness? Is that a virtue?”
God nods. “If she can’t separate herself from the child occasionally,
she’ll never survive. Yes, here is a woman whom I will bless with the
child less than perfect. She doesn’t realize it yet, but she is to be
envied.
She will never take for granted a spoken word. She will never consider
a step ordinary. When her child says ‘Momma’ for the first time, she
will be present at a miracle and know it! When she describes a tree or
a sunset to her blind child, she will see it as few people ever see my
creations.
“I will permit her to see clearly the things I see – ignorance,
cruelty, prejudice –and allow her to rise above them. She will never
be alone. I will be at her side every minute of every day of her life,
because she is doing my work assuredly as she is here by my side.”
“And what about her patron saint?” asks the angel, pen poised in midair.
God smiles. “A mirror will suffice.”

Quick Treatment Update - Sunday June, 10 2012

 

 

Quick Treatment Update: It’s been about a month and a half now since we started the new treatment, and I thought I’d give a quick update. We have noticed many marked improvements and no drastic declines. Fiona does not appear to have lost any more skills; in fact, we have noticed that she has gained some skills, which she had lost 45 to 75 days ago. Her motor skills, neck strength, eye contact and interaction with her environment have all improved. Fiona has also smiled at both of us; we have not seen a smile on her face in 2 months. Paxton was elated when she smiled at him 4 days in a row while he was talking to her.

 

I give credit to Paxton for being the aggressor when it came to medicating Fiona. From the beginning, we worked our way to full dosage of 100 mg Zavesca, 3 times a day, within 3 weeks, when the doctors told us it would take 8 months and that Fiona would experience harsh side effects from a strong dose too early.  I still can recall what Paxton said to me regarding why we have to be aggressive. He said that we just don’t have the luxury of time and that every day that passes, GM1 was taking our daughter from us; permanent, irreversible damage was occurring each day.  In the end, there were no harsh side effects for Fiona whatsoever.  This is a worry we now live with every day- the amount of time we have with our firstborn.  If you live life always worrying however, you sometimes forget to appreciate all that you really do have. We have Fiona today, and I will focus all my time, energy and love upon her.

 

We are living differently these days because there are certain facts that we know about GM1. For one, there is no cure. The second is that the disease destroys nerve cells in the brain and spinal cord. The most significant fact that is an impossible pill to swallow is that Fiona may not survive to see her second birthday. All of these facts help us to take joy in the simplest moments with Fiona, whether we get a snuggle, a smile, or a quick laugh-it's in these simple moments that we find the most shared happiness as a family!  The smiles and laughs have come with much less frequency now, but its out hope that we see them come more often in the future again.

 

We still have a long and uncertain road ahead of us, but we are trusting that God is in control and He loves us and our little girl.

 

 

NTSAD 2012 Family Conference - Sunday June 10, 2012

 

At the end of April, we attended the National Tay-Sachs and Allied Diseases (NTSAD) Annual Family Conference in Orlando, FL. I don’t think there are words that can truly describe my experience; “overwhelming” doesn’t begin to explain what Pax and I went through. I experienced a wide range of emotions during that trip as I felt a combination of emotional highs and lows. There were moments I felt hope & comfort and then moments I felt saddened & defeated.  Overall, we learned a lot & I was glad for the opportunity.

 
When Paxton and I were in our darkest hours after Fiona was diagnosed, we searched for answers to this disease. Painfully, we came to know the heartbreaking truth of the matter-there simply weren’t any answers available and no cure. NTSAD supported and connected us with other families that were in the same situation we are in, and although we were sad, we didn’t feel alone anymore. We are very grateful for NTSAD and the support this organization has given us.
 
The conference included updates on recent research findings from scientists working towards a cure, support group sessions, and practical information and tips on how to raise a child with a rare, debilitating, neurologic, genetic disease.
 
We learned a lot from some of the other families and were touched by their stories. The families were all so willing to share their stories with us, and they were able to provide us with honest glimpses into their everyday lives. Our main source of information and comfort has been other parents that we met through the NTSAD. These parents were able to answer the day to day questions we had that our medical team could not answer.  When Pax asked the physicians at Mayo Clinic Rochester about their experience with GM-1, one physician had never seen a patient and another had treated one patient over 20 years ago.  By networking with other parents, we could talk to individuals with years of experience.  Although every case is unique in its rate of development and decline, the insights from other families were both overwhelming and invaluable.  We learned about the best types of juvenile wheel chairs for paralyzed children, that at some point we’d need a live-in nurse, that we’d need to implant a feeding tube, that our child would develop severe pneumonia… It gave me some comfort knowing that there were other families that I could draw strength from.  It also helped to lessen my feeling of isolation.  I learned that there are two types of feeding tubes, one that is placed through the nose that goes down the esophagus and into the stomach. The second type is inserted through the abdomen and connects directly to the stomach. There was even a way to implant a tube directly into the intestine which is important because the GM-1 children can develop stomach and esophageal reflux and aspirate food into the lungs, causing pneumonia.
 
At the conference, we were able to see all that's involved with raising & traveling with a child with a rare disease. I saw families who needed to carry so much equipment with them for their children-suction machines, oxygen machines, nebulizers, and big boxes of medication. It was quite an eye opener and a difficult glimpse into our future.
 
At 10 months, Fiona is doing reasonably well. Fiona is not on daily seizure medication and is still quite alert and reactive despite the physical limitations of her body. Fiona takes her meals by mouth and not through a feeding tube. I take care of her at home without 24 hour nursing assistance and have had no emergency hospitalizations. Fiona is comfortable and as healthy as she can be, and for that we continue to be grateful!

Early Intervention - Sunday April 8, 2012

 

 

 

 

It’s amazing the things you learn when you have a child diagnosed with a rare disease. I had no idea what the term “early intervention” meant before Fiona was born…and did I learn quickly that it’s an imperative part of her development. Early intervention is programming consisting of therapy sessions, activities and exercises to help address the delays found in individuals with special needs.

 

Our next step was to get Fiona evaluated so that we could begin this early intervention process. Fiona was evaluated and she qualified for the program. We were then paired with a Service Coordinator, who put together a team consisting of physical and occupational therapists, who further developed a plan for Fiona that will lead to the best possible outcomes for her development. 

 

Fiona’s physical therapist’s name is Megan, and she comes to our home once a week to work with Fiona. The rest of the week, I am the one who takes on the responsibility of working with Fiona, doing all the different exercises with Fiona that Megan has shown and taught me. I always do my best to try and make it fun for Fiona because she doesn’t seem to enjoy it very much. I try to incorporate the therapy into as much of our day as possible. I find ways to sneak it in here and there during meal, play or bath times. The amount of time spent on therapy varies from day to day. The first therapy session was terribly difficult to have to watch as Fiona’s mother. Fiona was screaming and hollering at the top of her lungs begging for the exercises to stop and the session to be over. The absolutely only thing that made it bearable to watch, was knowing that Megan was a trained professional who knew better than me what was best for Fiona. In fact, I needed to trust Megan with Fiona the way I need to trust God. At times we may question why God chooses to put us through such pain in our lives, but we must believe and trust that as our Father, he is always doing what is best for us. Isaiah 41:10 says, “So do not fear, for I am with you; do not be dismayed, for I am your God. I will strengthen you and help you; I will uphold you with my righteous right hand." I have found that this is way easier said than done! I have found it extremely difficult at times to truly trust that any good can come out of such a painful experience. Knowing that my first and only baby girl may not see the age of two has been the most difficult experience I have ever gone through thus far in my life, by far…..

 

I think back when I was carrying Fiona in my belly. I recall all the thoughts and wishes I had for what I wanted to be able to provide to my daughter as her mother and what Paxton and I would be able to provide to her as her parents.  I would imagine things such as what my baby would look like, whether the baby would like more like Paxton or me?  I had so many hopes, dreams and wishes for my little girl as she grew up. I wanted her to do well in school and excel at an activity that she found interesting and enjoyable. I wanted her to be passionate about life and respectful and loving towards others. I wanted for my baby the same things that all parents want for their child. But when Fiona was diagnosed with GM1, I realized I wasn’t going to have that normal experience of parenting that most parents get to enjoy.  Never once did I think that I was going to have a baby that was diagnosed with a rare disease that could very quickly take her life!  All I think about all the time is Fiona. I know that God will provide Paxton and I with the strength, courage, and love necessary to raise her the very best that we can, no matter what obstacles come our way, we will get through these tough times as a family and we will do it day by day. Whether Paxton and I have the honor of parenting Fiona for much longer or not, we are proud parents of our little angel and nothing will ever take that away from us, not even this awful disease….

 

Insuring Peace of Mind - Sunday April 1, 2012

 

 

The insurance company appointed one of their staff nurses to support us through our medical crisis. When we read about the support that was to be provided, it really didn’t appear to be beneficial for Fiona. It’s just so difficult to determine who to trust right now. It’s tough to know who is an advocate for Fiona and who is not. I guess ultimately, we, Fiona’s parents, are her only true advocates.

 

The processes and procedures we have to deal with regarding our health insurance plan involve a lot of busy work. Paxton had to deal with many rejected claims. He read our health insurance policy manual and all the complicated coverage explanations of benefits so many times that I actually think he memorized it. He researched the rejected claims, kept a detailed spreadsheet, and composed detailed letters, which eventually helped cover the medical bills. Although there were some tenuous moments when medical benefits weren’t covered, ultimately through multiple appeals, all expenses have been paid – sometimes many months later.

 

I’m so grateful that Paxton’s employer benefits have covered and continue to cover Fiona’s treatments of GM1. Our insurance carrier paid for almost all of our medical bills, which totaled upward to a lot of dollars---and counting. Considering the present healthcare crisis in this country and the many Americans who don’t have any coverage, we are very blessed and fortunate to have good coverage during such a difficult time. Praise God for his blessings in our time of crisis!

Unconditional Love - Sunday March 22, 2012

 

 

 

 

The past 9 months have changed my life in so many ways. I feel things I never thought I could feel, and I love deeper than I ever knew possible. When I think about this love that I have for Fiona, I think of how unconditional it is and that no matter what, above all else, I will always love her.

 

Fiona is my baby and my miracle in life. I find myself paralyzed with so much love for her during the simple moments that Fiona is snuggled into my neck, or smiling up at me. I would do absolutely anything for my baby! I can finally say that I truly understand what unconditional love is. I love my family, I love my mom and sisters deeply, and I love my husband in ways I can’t describe. However, when I look into the eyes of my daughter, I feel something I never knew existed. She makes everything in my life shine a little brighter, and even on my worst days, I go to bed with my heart feeling so incredibly blessed. I feel so lucky to be her mom. I had no idea that I would love and embrace motherhood this much, and I can’t imagine my life without Fiona in it.

 

Whenever I would see parents of special need children before this past year, I would think to myself how very special those parents are, but there’s certainly nothing special about me. The love I have for Fiona comes naturally and is limitless, despite all my fears and heartaches.

 

There are times that I fear the future, but most of the time I look forward to watching Fiona learn and grow daily. I take pride in all that she is and receive so much joy from her. I didn’t think I could love her this much, but I absolutely love her with all that I am! 

New Mom Group - Sunday March 18, 2012

 

I am no longer able to find myself attending the “New Mom and Baby Group” at the hospital anymore. I’ve found that I can’t even bear hearing another mother telling everyone how advanced their baby is or how their child is crawling, sitting and pulling up on furniture. It’s not so much that I’m not happy for them that their child is developing normally, it’s just not my life and it’s not what I’m experiencing as a mother; in fact, it’s far from it. I realize that my child is not normal, that she is unable to develop normally, and I must direct all my energy and love into supporting and helping her to live and to live her life happily, even if it’s a very different life than most. I may not have the “normal” experience as a first time mother, but I’m doing my best with what I’ve been dealt.

 

 I look at Fiona and every day I celebrate all that she is to me. I rejoice when she smiles at me, and I’m filled with joy when she laughs. I learned to stop worrying about the standardized milestones, and instead, I create my own! Instead of looking for what is not happening within Fiona-I strive to notice all that is! 

Words of Encouragement - Sunday March 11, 2012

 

 

Much of what strengthens me and continues to do so is knowing that God was with me all the time. I did not have to go through this journey alone. I realize that not everyone who reads this is spiritual, religious, or a believer in Christ. For me God is the only one who can truly heal, restore, and renew. The scriptures below brought me comfort and encouragement during difficult times.
 
Joshua 1:9
Have I not commanded you? Be strong and courageous. Do not be terrified; do not be discouraged, for the Lord your God will be with you wherever you go.
 
Psalm 139: 13-14
For you created my inmost being; you knit me together in my mother's womb. I praise you because I am fearfully and wonderfully made; your works are wonderful, I know that full well.
 
Proverbs 3: 5-6
Trust in the Lord with all your heart and lean not on your own understanding; and in all your ways acknowledge him and he will make your paths straight.
 
1 Samuel 17:47
All those gathered here will know that it is not by sword or spear that the Lord saves; for the battle is the Lord's, and he will give all of you into our hands.
 
Isaiah 41:10
So do not fear, for I am with you; do not be dismayed, for I am your God. I will strengthen you and help you; I will uphold you with my righteous right hand.
 
Isaiah 41:13
For I am the Lord, your God, who takes hold of your right hand and says to you, Do not fear; and I will help you.
 
Isaiah 42:2 (Thank you Lynn DeJong)
When you pass through the waters, I will be with you; and when you pass through the rivers, they will not sweep over you. When you walk through the fire, you will not be burned; the flames will not set you ablaze.
 
Matthew 6:8
Do not be like them, for your Father knows what you need before you ask him.
 
Matthew 6:33-34
But seek first his kingdom and his righteousness, and all these things will be given to you as well. Therefore do not worry about tomorrow, for tomorrow will worry about itself. Each day has enough trouble of its own.
 
Romans 5:3-4
Not only so, but we also rejoice in our sufferings, because we know that suffering produces perseverance; perseverance, character; and character, hope.
 
Romans 8:26
In the same way, the Spirit helps us in our weakness. We do not know what we ought to pray for, but the Spirit himself intercedes for us with groans that words cannot express.
 
Philippians 4:6-7
Do not be anxious about anything, but in everything, by prayer and petition, with thanksgiving, present your requests to God. And the peace of God, which transcends all understanding, will guard your hearts and minds in Christ Jesus.
 
Hebrews 11:1-6
Now faith is the substance of things hoped for, the evidence of things not seen…But faith we understand that the worlds were framed by the word of God, so that the things which are seen were not made of things which are visible…he who comes to God must believe that He is and that He is a rewarded of those who diligently seek Him.
 
John 14:18
I will not leave you desolate; I will come to you.

Tears - Sunday March 11, 2012

 

 

I don’t know how to control these emotions. Just when I think it’s getting better, something triggers these unmanageable emotions within me.  All it takes is the simple acts of watching Fiona sleep peacefully, seeing her chest slowly rise and lower, feeling her grip onto my finger while she sleeps, kissing her precious little head, listening to her make her wonderful little noises, or feeling her breath while she sleeps on my chest.  
 
It’s often the little things that I enjoy about watching Fiona grow that strike such substantial and overwhelming emotions. It’s at those moments that I experience overpowering feelings of being hurt, sad, mad and frustrated which always ends in unstoppable tears as well. I am constantly asking myself such questions: How long is this going to last? How long before I no longer cry over my daughter’s suffering? How long before my heart doesn’t literally feel like it's tearing into broken pieces that I can’t imagine will ever be whole again?
 
Caring for an ill child can be both physically and emotionally draining. When you are in the middle of it, it feels like’s never going to end! I can’t sleep, and at times I'm completely drained of energy and my own health feels depleted at times. Fiona consumes all my thoughts all the time.
 
I learned as a new parent that when your child is sick, nothing else matters but your child's health, wellbeing, and safety. The most important responsibility I have towards Fiona is to offer all my unconditional love to her and to wholeheartedly help her in her fight for life.  After all, when it's all said and done, whether life is short-lived or long-lived all that really matters is experiencing how to truly love and be loved by someone.  Fiona is surely loved by many, and she knows that.
 
Loving and mothering Fiona has left me forever changed and has taught me that every aspect of life, every thought, every feeling, every moment is an experience and a gift, be it tears or laughter, sadness or happiness. It all counts. It all matters. I feel even more blessed to have the reminder that I must cherish Fiona, enjoy the moment, and invest relentlessly in the most precious relationship of my life.

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