Monday, August 31, 2015

Biopsies Done!

The kids survived the day!

Last night, I wasn't sure we were going to make it.  By the time they started round two of the cleanse, they were horribly sick and incredibly weak.  The last dose was at 9pm and they spent until 1:30am vomiting and diarrhea.  It was unbelievable.  I had decided to have the boys sleep in my bed so they would be closer to a bathroom and Shelbie slept on a mattress on the floor.  I laid on the couch, jumping up and down assisting them.  I finally fell asleep at 2am and woke up at 3:10.  Wide awake.

We arrived at 6 am.

The kids were in really good spirits and they wore the craziest outfits just to keep the moment humorous.   The boys shared a room and Shelbie was next door.

We don't really have any results.  The boys' scopes looked pretty amazing and very normal but he took several biopsies in hopes that it would hold the key to Spencer's malabsorption.   It could be that the problem is in the liver, pancreas or small bowel.  None of those areas can be viewed in a scope.  Once the GI doc gets all the reports, Spencer will likely do the pill cam so they can get a look in his small bowel...well, unless the blood work shows something.  All of that should be back tomorrow or Wednesday.

Shelbie, on the other hand, had all sorts of GI problems.  She has pretty significant gastritis which could actually be H-pylori and some other unidentified growths that will be determined by pathology.

Shelbie came back to the room wide awake and incredibly agitated and in pain.  They had to give her three doses of morphine before the pain was controlled.  Tonight, she seems to be doing better.

Spencer has done the worst of the three.  He looks like a wreck.  Pale, weak and just skin and bones.

Bone marrow results will take another couple of weeks.

All in all...things went fairly well. I am so glad it's over.

Here are some highlights in photos from today.

Spencer

Sam...with his goofy 'man bun'

Sam post op
Spencer post op


Shelbie post op


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Sunday, August 30, 2015

Surgery Prep

Today we are preparing for our big day Monday.

At 6:00 am we start a host of procedures.

It will mark our 56, 57,and 58th bone marrow biopsies. You would think that by this point we would be use to this but it never seems to get easier.  It's getting a little bit harder.  It makes sense really.  We have managed to avoid myelodysplasia, leukemia, and other clonal abnormalities that can show up.

Dyskeratosis Congenita is a telomere biology disease.  Telomeres are the ends of the chromosomes that protect it from damage and disease. As they get shorter and shorter, more and more damage happens and that typically results in cancer and organ failure. So, the shoe could drop at any time.  The older they get, the shorter the telomeres.  Just as a point a reference, the kids are all at about the 8th percentile for telomere length...that's pretty short.

But, we are moving ahead with hope and determination.

Heading in to biopsies tomorrow of the bone marrow and colon and upper GI, here are the stats

Shelbie: white count 2, platelets 51, red count 4, liver- enlarged

Spencer: White count 4, platelets 200, liver enzymes elevated

Sam: White count 3, platelets 210, liver enzymes elevated, biliruben- elevated

As far as neutrophils go, they are low on all three kids.

I am not as anxious about the bone marrow biopsies as I am the GI workup and liver studies.  But, what will be, will be.

We are half way through the prep for surgery and the kids are wilting fast.  They had to start this in an already depleted state so this is definitely taking its toll.

Tomorrow at 6 am can't come soon enough.

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Saturday, August 29, 2015

An Outsider

One would think that as the children get older, wiser, more mature, life with a chronic illness gets easier too.

In some ways, it does but in most ways...not so much.

It's been an awful weekend.

Spencer has been planning to go to Colorado this weekend to speak at a baptism.  None of us counted on him getting sick, or having to have IV therapy so he headed out for a 36 hour road trip with his dad to give the talk and come home just in time to get therapy done on Sunday and start his prep for surgery Monday.

Shelbie is beside herself with worry about her counts.  She is also feeling the effects of the transfusion and she too has had a host of GI problems lately.

Sam is not doing much better than the other two...well, physically, he is I guess but he is in the midst of a bad depression.  It's been a very long time since I saw this kid happy.  It's only getting worse and this weekend, we sort of had it out with each other.  He is refusing to take medication and that is bugging me to no end.  I don't understand why he won't take it.  I asked him if I told him I was going to give him $500 if that would make him happy.  He nodded "Yes."  Then I said, "Well, I'm not going to give it to you in one lump sum.  I am going to put in my hand a $20 bill every day until I have given you $500.  All you have to do for the $20 is take it out of my hand.  Would you do that much for $500?"
Again, he nodded 'Yes.'
It's the same thing with taking medication.  You have to take one tiny pill once a day and in return, you find a little joy once again in life.   Unless you love moping around, feeling sad, anxious, angry, scared...why wouldn't you take it?  I don't get it.

Finally...after hours of poking and prodding him, lecturing, asking questions...he yelled, "You have no idea what it's like to be me!  You will never know what it feels like to live with this disease.  NEVER!!  So, why should I talk to you about it?  You don't get it!"

I was not happy.   However, it sounds like a similar yelling fest I have had with Shelbie in the past and actually again on Friday night.   For the first time, I felt like an outsider.  I felt like all the rest of the people who stand outside of our experience and misjudge us.

I wanted to yell back at them all...'You have no idea what it's like to be me! Watching my only kids deteriorate!  What do you think that feels like?'  I bit my tongue for the simple reason that he is right.  They are all right.  I finally understand that.  After all these years, my experience is not better or worse, just different.

Sometimes, these moments happen.  I hate them! I hate it when we all start coming apart at once. I hate the fact that we are each growing into our own stories.  It takes a great deal of energy to regroup and live in the moment of what is.

In so many ways, the kids are way more mature than any other young adults their age.  They have to be yet at the same time, they are very immature emotionally.  I think some of the traumatic moments we have had to face has changed them.  Shelbie spent no less than 20 minutes on Friday telling me what a lousy mom I am to not have found better doctors for them.  If I had worked harder, been more careful, we would have doctors who know what they are doing and doctors who care.  She wouldn't be dealing with low blood counts again if I had just done my job better.

Sometimes, you just feel like an outsider looking in.  It's weird when it's your own children who cut you out from the secret handshake and member's only clubhouse of Dyskeratosis Congenita.

I'm feeling a little beat up and pretty much exhausted.  On a promising note...Sam and I came to some understanding after I dragged him up to the nursing home this afternoon to push old people around in their wheelchairs.  Amazing how quickly one's perspective on the difficulties of life changes when you make a difference for someone else.

Well...on we go.  As usual.  Big day tomorrow...hospital, liquid diet starts at midnight and bowel prep to follow x3.

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Friday, August 28, 2015

Funerals for the living

Today was my grandma's funeral.  Sadly, we weren't able to make the trip back to attend.  In many ways, it made today extra hard.


As I thought about what we were missing all day long, I got to thinking...

We are raised to believe that funerals are to pay respect to the one who passed away.  When you think about it, why would they really care about the kind of funeral they have?  We think we care what kind of funeral we will have, the color of coffin, the flowers in a spray and all the other details we deliberate over.

I think, that funerals are for the living.  Funerals are for those left behind.  It's a time to reflect, remember, process and prepare your mind to move on with one less loved one. Funerals are important.  I feel strange not being able to be at my grandma's funeral to enjoy all those fond memories spoken of and say one last goodbye.

I couldn't just let this day pass without doing something special in honor of my grandma.  Something to set this day apart for all the rest.  So, after we got through with the hospital today, I ran up to the nursing home where a friend of mine is.

His name is Reed.  I have been a housekeeper to him and his wife for the past three years.  He has always been so kind to me.  When he was in better health, he raised chickens and always sent me off each week with a dozen eggs.  He made plum jam for me and shared his tips on growing the biggest tomatoes.   He has been on hospice for a couple of months and since his wife is not in good health either, they decided to put him in the nursing home for 5 days to give his wife a respite.

Reed was not happy.  In fact, he is really sad.

I haven't been able to visit my grandma much since she has lived in a nursing home.  I often wished I lived closer because I would have spent everyday visiting her so she didn't have to be alone.  I thought that visiting Reed was the best way to make this day special and remember my grandma.

My visit with Reed was moving.  He is so sad.  He broke down because he feels like such a burden.  He is lonely and sad.  My heart broke for him.  He cried and cried.  It was the saddest thing to just hold him while he wept in loneliness.  I hugged him while he wrung out his heart in my hands.

When he calmed down some, I packed him up in the wheelchair and took him out to the courtyard with two Maple donuts, his favorite treat!

He sat in the warm sun and savored those donuts and soaked up the sun.  He seemed much calmer and relaxed.  After a few minutes, he nodded off and I enjoyed a few moments in quiet while I contemplated on all the great times I had with my grandma.

It is so sad to think that a wonderful and amazing generation has quietly slipped away.  She saw so many things in this world in her near century of living.  She was talented and set out to accomplish all the things she ever wanted in life.  She had a love for learning, her grand kids and of course music.
My grandma, me- at the piano and Shelbie  with my dad singing some of my grandma's favorite songs. 


She will be greatly missed.  In my sadness, I thought of the great reunion she is enjoying on the other side of this life.  

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Thursday, August 27, 2015

From Bad to Worse

It's so crazy how we can go from status quo to a total disaster in 48 hours!

Seriously...

Trying not to panic...just yet.

So...last night, I ended up having to take Spencer into the ER.  He has not been able to keep any food in him for over a week.  When I say no food...I seriously mean, no food.  He is plagued with diarrhea, nausea and, well...I will spare you the bodily details.   By Wednesday night he could hardly stand on his own.  Even Gatorade made him sick.

At the ER, he received lots of fluids and orders to get fluids every day until Monday.  I think it is helping, at least he has a little more energy.  He is losing so much fluid and nutrients and since his gut is so sensitive and cramping, even drinking water is nearly impossible.  So...IV fluids are really necessary.


Today, Shelbie had her transfusion and Spencer had his IV therapy. Transfusion was going well.

 Since all three kids are scheduled for surgery on Monday, the surgeon dropped by to get a history on Shelbie since she couldn't leave the hospital to get to his office. He had asked for her CBC.  The nurse blurted out some numbers- 51 for platelets was a number she had tried to recall.  Without even skipping a beat, I said to the surgeon, "No, she meant 150, not 50."  We continued our conversation and I didn't think another thing about it.

As we left, much later than usual, I asked the nurse for the CBC.  She handed it to me and I was stunned.  Pancytopenia!!  All of Shelbie's blood lines are suffering.  Pretty darn low.  We haven't seen pancytopenia in a long, long time.  Most of all, I was disheartened at her low platelets at 51.

Unless she is working on getting a virus or something, this could quite possibly mean her ITP is back.  If her ITP is back...we are looking at more chemotherapy!  Last time it was 9 months!! Ugh

To say that Shelbie was upset would be, not only obvious, but well understated also.  I think it's safe to say she is already planning her funeral.

I couldn't believe it.  I like to think that maybe it was a calculation error but the fact that all three blood lines have dropped, is only wishful thinking for a virus.  As the night wears on, it's all becoming even more unbelievable that we are in this very lousy place...again!   I can't help but be grateful that we didn't go to Canada for my Grandma's funeral.  Leaving the country with counts like this wouldn't have been the smartest thing to do. Not to mention with a very sick Spencer.

Tomorrow, we are back at the hospital.   I sure hope we make it through the next few days.  I'm pretty concerned.  All day Sunday will be spent in bowel prep.  Surgery Monday will be tag team style and start at 6 am.  Hopefully by early afternoon, we will be home.

I have yet to report on our GI visit.  It was disheartening and worrisome... hence the colonoscopies and endoscopies.  Maybe I will get to that report next.

I know this for sure...my full time job is really getting in the way of my medical life!
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Wednesday, August 26, 2015

It's never simple

I don't even know where to start.  The past 36 hours have been among the most intense.

We headed to Boise Monday night for GI at St. Lukes all day Tuesday.  Our GI doc completely cleared out his day so all he had to focus on was us!  It was amazing and I was so grateful to him.  I will write more tomorrow.

It took so long to get everything done medically.

When we were finally through, we ran to the mall to get some back to school clothes for Sam.  He started school today.  Shelbie and I went one direction in H & M and the boys went another to find what they needed.

Shelbie and I were in one of the tight aisles...with Shelbie in a wheelchair because she has been having more heart problems and breathing issues so it was crammed.  There was a girl about Shelbie's age in the same aisle.  She was pretty intent on looking through the rack so I started to back out of the aisle.  Just as I did...this girl started contorting herself and speaking gibberish!  As I tried to figure out if she was talking to us or what, she went down...Hard!!  She fell against a rack of clothes and banged her head on the bar before hitting the back of her head on the metal bar at the bottom of the rack!

I didn't notice that her mom had been on the other side and as I ran to the girl, so did her mom.  We pulled her out from below the rack and she was clearly having a major seizure.  It was terrifying!!!

Her mom started to panic and I reassured her that I had dealt with many seizures before and she was going to be okay.  I hurried to find her pulse and kept my hand on it to monitor her heart rate.  She was breathing...sort of but still managing shallow breaths.  Her muscles in her legs and arms were seizing violently so we were trying to keep her still and from banging her head around.

At one point, she turned the darkest purple and her breathing stopped but her heart rate remained constant.  I wasn't sure if I should start CPR since she was pumping oxygenated blood so I was hoping that would be enough to keep her safe until the seizing stopped.

The other issue that I worried about was all her piercings.  She had several in her nose, septum and lips.  I was worried that pinching her nose would put more piercings in places she didn't want.  It would have been challenging to get a good seal around her mouth because of the jewelry.

At this point, while I'm contemplating what to do, her mom just freaked!!!  She was sure that she was dead.  I just remained calm and said, "She's not dead.  I promise. Do you know CPR?"  I asked.  I was hoping that she did because I'm sure this girl didn't want my germs anymore than I wanted hers.  If her mom knew CPR, I would have had her start rescue breathing.  She didn't.  Instead she yelled out for CPR help.  Shelbie had been trying to call 911 but she had no cell service in the store and she was trying to find a sales clerk to call. The she called out for CPR help too.

The entire store was in an upheaval.  The scary thing was, that the boys saw Shelbie crying and calling for help but they didn't see me.  They immediately thought I was dead.  It was just an intense and scary moment for the all of us.  This girls seriously looked dead, especially when the seizing stopped and she was just still and not breathing.

Eventually, she took a breath and resuscitation wasn't needed.  It was several minutes before this girl regained consciousness.   She was 24 years old and had just returned from Argentina where she had been living for two years.  Her mom knew very little of her latest health history like if she was pregnant, had any illness or disease.  I'm not going to lie...I'm kind of relieved I didn't have to do mouth to mouth but I would have.  I know I would have given it my best effort in order to save her.

It was a hard thing to do.  It was hard to see Shelbie so terrified because this girl appeared to everyone to be dead.   It brought back so many horrible memories of watching my own kids have seizures.

We still haven't processed the whole event.  Shelbie didn't sleep at all last night.  She is truly traumatized.

As we were driving home, I got a call that my grandma had passed away.  I couldn't believe it!!  It doesn't matter how old a person is, death is just not fun.  It's downright hard!

Needless to say, everything combined yesterday made for a very tough day.  Today, I just feel numb and tired and distant.   I'm pretty much exhausted and can't seem to focus.

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Saturday, August 22, 2015

Fidgeting and Fretting

Today has been pretty monumental.

First, the back story.

I have mentioned here a time or two how stuck we have been in getting medical care.  I see all the other DC families with lung, heart and bone marrow problems being proactive and getting all sorts of care and treatment and then we sit here, just twiddling our thumbs hoping that it will all just disappear.

Losing our Hematologist in Seattle seemed to precipitate many other changes.  We have outgrown all of our doctors simply because the kids got older.  Not only that, we haven't been happy with a couple of our doctors.  With the DC diagnosis, they seem out of their league.

With Spencer coming home, we need to get all the DC genetics done and get the same baseline tests that Sam and Shelbie had but now we don't have decent doctors.  I've been praying to find some direction; to figure out where to take them for medical care.

We had options but certainly didn't feel like any of the options were a good fit.

For the very first time, I didn't fret about this or fidget and try to force a plan.  I simply just prayed until something happened.  I don't know what I was expecting...I guess that's just it, I had no expectations.  I couldn't see how this situation could be resolved so I just tried to remain open and hopeful that the way would be made clear when the time was right.

Today, I had to make a trip to Wyoming to work on a hospital project I've been working on the past year.  As we walked through the surgical suites under construction, the CEO asked me how my kids were doing.  I briefly explained that we were assembling a new team of adult medicine docs and it was a huge and hard undertaking.  Out of the blue, he started talking about a cardiologist that works two days a month at the hospital.  I thought absolutely nothing of that.

When our meeting was over, he offered to walk me out to the front doors.  As we left his office, he said, "If you have a minute, I can introduce you to the cardiologist's nurse.  Maybe she could help you in some way."

As we turned the corner from his office, a lady was right there in a hall.  She was coming to see the CEO.  It was the nurse coordinator for the cardiologist he was telling me about!  We were introduced and I followed her back to her office.

We visited for a few minutes and I gave her some background on my kids.  We even set up an appointment to see the cardiologist.  Soon, a man came out of the office.  It was the cardiologist!

He began asking questions about the kids and a history of what we have gone through.  We must have spent 45 minutes talking and laughing!  He was an awesome doctor!! I loved his personality, and sense of humor.

Half way through our visit, he did mention he had a partner who specialized in AV Malformations of the heart and lungs.  His practice is almost exclusively congenital heart defects and AVM's.   He is young and on fire when it comes to researching AVM's.   This cardiologist suggested we see his partner instead of him.  He felt it would be a better fit, plus, he is set to retire soon.

We finished our conversation with instructions from this doctor on what we needed to do next.  He said that 90% of the time, in AVM's of the lungs and heart, the liver has AMV's as well.  He said that liver screenings would need to take place, before we see the new cardiologist.

Having met with the nurse coordinator as well, we already that much ahead in the scheduling procedure and she knows all about our situation already.

It was so clear, as we stood there talking that this had been completely and totally orchestrated by God.  It was the most amazing feeling to see things work out so simple and easily, without any fretting and worry on my part!

Part 2 coming soon...


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Thursday, August 20, 2015

Better than this?

Do you ever sit in the middle of a day and think..."Wow, it doesn't get any better than this."

It's hard to imagine that in a world with so many opportunities to be scared, tired, worried, fearful and in pain, there would be moments that you wanted to freeze in time, when you could actually stand apart from the surface and wonder if life could really get any better.

This morning, Spencer and I spent 2 1/2 hours at the temple doing some sealing work with some friends.  I thought it would only be about an hour which would have worked out fine because I had to clean a house, but it took much longer.  I just couldn't stand the thought of leaving such a peaceful, happy place where families were being joined for eternity.

We left though and I got to my house to clean a full hour late.  When I arrived, the house was in chaos.  The elderly man, who is on Hospice, had become disoriented in the night and flooded the bathroom.  His dear sweet wife didn't hear because she sleeps upstairs and had closed the hall door to keep the dogs out from bothering her ailing husband.

I immediately got to work sopping up water on the bathroom carpet.  She was saying that she wanted a gate for the dogs so she could hear her husband and not have to close the door to keep the dogs out.  I had one at home that was on its way to the thrift shop.  I called Sam to see if he could run the gate up.   It's a really nice gate that also has a 'door' in it so you don't have to climb over it.

As I moved through the house cleaning up messes, the wife said, "I need you to hurry and get done because I can't keep the tears in much longer and I don't want to cry while your here."

I put down my stuff and gave her a hug, telling her I wouldn't let her cry alone.  Just as she was unloading on my shoulder, all of my kids pulled up in the driveway!  It was like a little miracle.

They came in with the gate.

Spencer got to work installing it.  Sam and Shelbie went down to the basement apartment to check on water damage from the flood and I started folding an overflowing basket of laundry.  Then Shelbie sat and visited with the wife and Sam finished helping Spencer.  Spencer gave her a blessing and then the kids went and got her some lunch.

It was such an incredible feeling to see all the kids working to serve this sweet couple.  I knew they had a long list of things they needed to do but they didn't complain.  Shelbie even offered to sit with her husband so she could get out of the house for a couple of hours.

It wasn't anything huge or monumental but it was so needed.  By the time I finished cleaning and the kids had brought lunch, she was smiling again and feeling so much better about their bleak situation.

It sure doesn't take much to make a person feel loved.   In fact, a little bit of love goes such a long way, not only for the person receiving the love but the people giving the love too!

Seeing my kids so happy and jumping in to help, melted my heart.  Everyone was so happy the rest of the day!  I couldn't imagine that life has ever been hard...for a minute at least.  I couldn't imagine that life could ever be anything but this kind of happiness.

It doesn't get much better than this.


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Tuesday, August 18, 2015

Out of the blue

Yesterday, I received in the mail, quite by surprise...

This book.

Diagnosis and management guidelines for Dyskeratosis Congenita.

I didn't order it.  I didn't even know such a book existed.

It is no less than 438 pages of information regarding the disease my kids have.  There is not another book of it's kind.  It was written by 40 of the top researchers and providers of care for DC kids/patients.  It is complete with references to studies and articles.

It's pretty amazing.  I am seriously amazed.   In awe.  Overwhelmed in fact!   This is worth it's weight in gold!  I can't even imagine the work it took to get this compiled.  It takes a lot for me to be moved like this.

It was out of the blue.

The other thing that came quite by surprise was a phone call from our GI doc in Boise.  Just a few minutes ago, his nurse called and said that Dr. T has cleared out an entire day for us to bring the kids next week!  Next week!  He's going to see my kids!  I'm so relieved.  This will be the last time but I love this doctor and I'm so glad he has taken such great lengths to care for us.

His nurse literally said, "He has cleared out the entire day so you can choose any time you want to come and see him and he will spend whatever time it takes."   That has never happened to me before...ever!

We haven't seen him in three years but this is why I love him.

When Sam was just 15 months old, he was all sorts of sick and failing to thrive.  We had already gone the run around with Shelbie and Spencer at Primary Children's GI and Toronto for Sick Children, who, at the time was the end all and be all of SDS research.   They had messed us up which is a long story in and of itself.

By the time Sam arrived, we had gone two years without being treated for pancreatic insufficiency.  Our family doctor knew we needed to find out what was wrong and he mentioned this brand new doctor in Boise who might be able to help us.  It was Dr. Thompson at St. Lukes.

On our first visit, he recognized some serious problems with one of the kids whom shall remain nameless because it's a TMI kind of story.  This child of mine had to have an enema while on our first visit.  After a horrendous amount of time, the child unloaded in the bathroom at the hospital.  I got Dr. Thompson to let him know we were done and he came into the bathroom, pulled on some gloves and began to fish around in the toilet bowl, literally sifting through the remains from an enema!

To say I was shocked would be a blatant understatement.  I stood there in awe!  I said, "Why are you doing this?  Don't you have people to do this for you?  What in the world made you want to do this specialty?"

He looked up at me as he crouched down beside the toilet..."It's fascinating!"  Just then, his pager and hospital ID started to fall from his shirt pocket, headed straight to the toilet.  He caught it with his free hand!  Then he looked at me and said, "There's never a dull moment!"

In that moment I thought, "This guy is a keeper!"  He's truly been a team player and listened with empathy to the kids and to me.

I am pretty sure it's all been orchestrated by God himself.  I have been so stuck lately, not really knowing what to do so I have done nothing.  I just kept praying that I would be led to the next best thing and I would figure out what to do about our doctor dilemma.  It has all just fallen into place.

So, Oncology and Gastroenterology all next week and bone marrow biopsies will happen if not next week, then for sure the following week.

These are some major appointments and all that is left is cardiology, pulmonology and ophthalmology and a sneaking suspicion that all three will be going in for baseline colonoscopy testing and one more appointment for Sam to see the Urologist.  It's a lot left to do but we can manage.  We've had a nice little break from the fray.   Time to get things taken care of.

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Sunday, August 16, 2015

Life Goes On

I heard this all familiar saying today...Life goes on.

It is a statement of truth.  Life goes on no matter what you happen to be going through, have gone through or will go through.

I remember so clearly, the day we walked out of the Oncology office just over a year ago, after getting blood drawn on Shelbie and Sam to send off for confirmation of Dyskeratosis Congenita.   The nurse had inadvertently handed me the wrong paperwork and I saw the diagnosis from the research lab. Something that legally, I was not suppose to see.

As I pushed through the front doors, the mid-day sun magnified a glaring reality on the world around me.  I was facing what felt like the worst news we had ever received and my world was cloudy yet everyone around me, seemed to be enjoying their world in the sun; even the people coming out from a chemotherapy treatment seemed more at ease with life than I was in that moment.

I remember looking down the street to line of kids waiting for ice cream at the new food truck.  Driving home, the park was filled with families having fun, playing, laughing.  The radio station still cranked out the tunes that made everyone tap a toe or move to the beat.

'How can these people be going on with life?' was the thought that percolated through my numb mind.   My life had seemingly stopped.  It was a moment that would forever define my life. This oncology visit became a dog eared page in the book of life that I would somehow keep going back to and re-reading the fine print it left in my mind...like I did today.

Over a year later, life continues it's rhythm; albeit syncopated and paused at times, but nevertheless a rhythm we have adjusted to.  Are adjusting to.

Over a year later, I can see that that distant day in the past was not the worst day ever.  It wasn't the best day either.  It was a day that left us in pieces.  Little bits of what we were once familiar with seemed even more fractured. Somehow, life goes on in those little bits and pieces that fell away that day.

Funny...how that happens.  Funny how life goes on even when you can't see any possible way for it to do so.

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Saturday, August 15, 2015

Missing Pieces

I've been trying to get appointments for the kids set up but it's not easy.

Having to start over with all new doctors has been daunting.  I don't even know where to start looking for new ones.

I've been really bothered with the whole hematology situation. The more time I spend in the Dyskeratosis group and reading about the disease, the more confused I am.  We are one of the very few families who has not been to transplant yet.  In fact, one family is starting transplant very soon and their child actually has better counts than any of my kids!  I am continually told that it's better to transplant while they are healthy.  Once the lung problems start, it's too late to transplant.

We have never been told any of this. I feel like we don't have anyone looking at the big picture. Now without our top Hematologist and GI doc, we are even more lost.

I'm hoping I figure something out soon.  We could go to Seattle and meet with the new doctor Dr. Shimamura suggested we start seeing but honestly, the thought of going there and driving all that way fills me with dread.  However, she works closely with Dr. Shimamura and has been groomed to take over her transitioning patients so it makes sense.  We won't have to re-invent the wheel.  Still...that drive!

As far as GI...I have no clue where to go.  We need someone pretty fast.  Spencer keeps asking when he will start feeling better.  Without a plan...he isn't going to start feeling any better.

This is only the tip of the iceberg.  Yesterday was overwhelming as Spencer continued to talk about all the things that are ailing him.  New symptoms...All DC related.

Ahh...we will move ahead and I'm sure things will fall into place soon.

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Friday, August 14, 2015

My Birthday Girl

Another birthday for my girl!

Shelbie turned 23 yesterday!

I look at her and I don't even know how we arrived here so fast!  In  many ways, I remember so clearly each stage of her life.  It's a cool thing to look back and see how each moment was a defining one.  For good or for bad...it made her the wonderful young woman she is today.

Shelbie likes people to think she is aloof and maybe even jaded but in reality, she is the most tender hearted person I know.  She is sweet and has an intense desire to not miss out on anything in life.  She has talent galore but doesn't always see the gifts that she offers the world around her as anything special.  I guess in a way, that keeps her humble.

As I think about Shelbie, there is so much to love but the thing I love the most about her, is the way she lives her life.  She is genuine and tenacious.  Okay...two things!

She doesn't stand for fake people, selfish people or people too consumed with 'life' to care about others.  She is who she is and she doesn't apologize for that but at the same time, she is herself in a humble, caring way.

Out of all the people I know, Shelbie has had more reasons to throw in the towel than anyone but she doesn't.  She may wobble a bit at times, but she always gets back up, brushes herself off and marches on. She gives the people who use her and hurt her more chances than they deserve...ever!  I love that about her.  She struggles through something until she understands it.  She laughs at hard times, she cries...she is real.

So...with another year behind us..

Happy Birthday...My DEAR!

Shelbie loves the white resin deer with antlers and requested a 'deer' cake.  the deer on the front of the cake is even edible!  It's sugar paper. 

Present opening!


Playing games


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Wednesday, August 12, 2015

Back at it

I never realized that missing a week on my blog is like missing a part of myself.

I think we have bobbed back up to the surface of our somewhat stormy life and we are ready to find our new normal for a few weeks.

Spencer is doing so much better the last day or so.  Monday, almost felt like he was never gone.  The three kids together are having a great time.  Last night, as we all sat down for dinner and everyone was laughing and joking around, my heart was so full to see all the ways I have been blessed as a mother.  I might even say this stage of life is my favorite...

Not the part where they all leave me...but the part where they are all mature and can enjoy each other as best friends instead of siblings who had their moments of bickering and teasing.  It's amazing to see that they have all turned out pretty good so far.  They have learned to make the best of things and the most of a day.

Monday, I had a few minutes to get focused on what we need to do to get Spencer feeling better.  He still gets sick every day with nausea and pain.  Yesterday, he spent the better part of the day with pain around his heart and his sternum.   We have a few appointments scheduled but they are way out on the calendar.  I found out yesterday that our GI can no longer see the kids since he only sees patients up to age 19.  I begged and pleaded with the nurse to let us see him one more time but I'm not sure that is going to happen.

So...I'm on the hunt to replace the GI and figure out what to do about Oncology.  For now, we will see our local doc and do the upcoming biopsies at our local hospital but I feel like in some ways, we have outgrown that clinic.

Before Spencer got home, we had finally fallen into our snug little world of denial and living with DC had become a little easier.  Having to bring Spencer up to speed on things and get him scheduled to start this process all over again, has been really hard.  Harder than I thought it would be.

But such is life...




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Thursday, August 6, 2015

A Scary Story

When I die and meet God, I am going to ask him one thing...

I would love for him to explain why he gave me all the rare, never before seen problems in the world?  We don't get your run of the mill trials...

Tuesday, when we were contemplating a trip to Salt Lake, I had this uneasy feeling about my car.  For one thing, the front tires are bald and second of all, there have been some odd problems with my tail lights.  They go out all the time.

After a little research months ago, I found out that this is a problem with Hyundais and when I called, they didn't have a way to fix it.  The weird thing is, the brake light problem is associated to a problem with the cruise control and affects the way that works.  I know this because I had both brake lights burn out and my cruise control stopped working.

Tuesday, I had this feeling that this whole issue was going to be a problem.  I ignored that but decided to at least have my tires rotated so better tires were on the front.  With the tire problem sort of solved, I pushed the other worries to the back of my mind and we took off.

About 2 1/2 hours into the trip, we were getting into some congested traffic.  I switched lanes to pass a string of slowing moving trucks.  I pushed the button to set my cruise and it didn't stop accelerating!  In a second the pedal was completely to the floor of the car.  I couldn't get it to stop.  I turned the cruise off but that didn't help.  We were accelerating fast and got past 90 mph.  There were cars in front of me and cars to the side of me and I was going faster than all of them!  I sort of panicked but more because I was shocked that the very thing I had worried about happening, was actually happening!

I was trying to slam on the brakes and nothing was working.  Spencer reached over the pulled the gear into neutral, shut the car off with the key and started pulling up on the emergency brake.  I found a small opening in the right lane and flew through that and onto the shoulder til I could coast to a stop. It was pretty darn scary!!  Like, really scary!

Even with the car stopped, the gas pedal was still completely down and touching the floor of the car.  Brings new meaning to 'pedal to the metal'.

Today, I called the dealership and they think they have a way to fix it and yes...it has in fact been recalled!   The service guy said he had heard that this could happen but hadn't actually heard of it happening!

Trust us to have the wild and crazy stuff happen.

Ahh....at least we are all still alive!

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Wednesday, August 5, 2015

Settling In

Spencer's been home a week!  It's been quite the busy week.

All in all, he is starting to settle in better than the first few days.  I was pretty surprised at how hard it was to have him home.  He felt awkward to be 'in the world' again and none of us really knew how to act.  I think we were all trying to pretend none of us were living in the world either; we tried to shelter him from our usual, hectic life.  He was really homesick for Colorado too.  It was almost as if he was sad.  His face, his actions, everything just seemed sad.

I wanted to fix it.  Then I thought maybe it was me making him sad.  It was just hard.  Nothing at all like I had expected.

I wasn't sure how we were going to tell him about all the things that happened over the past two years, especially about his 'new' disease, Dyskeratosis Congenita.  Thankfully, it didn't end up being as big a deal as it was in my head.

On Thursday morning, his dad came over and we were planning the day.  Somehow, we all migrated to Spencer's bedroom.  I think he was showing us his lack of clothes.  I had bought him some new clothes but he has lost a lot of weight!  I bought him the size he wore when he left but we had to exchange them for two sizes smaller!  His smaller frame led to a conversation on how he lost the weight.  Well, he's been sick almost the entire two years.  Not only was keeping stuff from him but he was keeping stuff from me!

He talked about how run down he feels.  How his asthma is giving him problems.  How is gut and pancreas seem to causing him all sorts of problems.  He even mentioned that there have been a few times he has passed out for no reason...just standing in his apartment doing nothing and he goes down.

When he was done, I talked about DC and all the things we have found to be problems in Shelbie and Sam.  With each symptom and problem, he just said, "Ya, that happens to me too!"  It all made sense to him.  He already knew he was sick, he didn't need me explaining some rare disease to him, he has been living it.

I'm kind of glad things happened the way they did.  I'm not sure how he is feeling about things.  It will probably become more real when I start getting appointments scheduled and we get a better picture about what is happening.

Tuesday, we made a quick trip to Salt Lake to go back to school shopping for Spencer and Sam.  While we were there, an old companion of Spencer's asked him to stay a few days and help him lay some tile.  So, we left Spencer behind, said goodbye...AGAIN and came home.

Being with is friend has been really good for him I think.  It is good to be with someone who has already gone through the transitioning phase.  I talked to Spencer on the phone tonight and already, he sounds happier.

It's been great to have him home.  We have had some really awesome talks about life.  We have gone to the temple and enjoyed being together as a family!  

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Sunday, August 2, 2015

Welcome Home Spencer!

It's been a whirlwind of a week!

My missionary returned home safely on Wednesday night.  It was an amazing experience.  I have to admit, I was pretty emotional all day.  In two years, I have never shed a tear over Spencer being gone and Wednesday, my thoughts were filled with tender memories of Spencer's life and everything we have been through.  I figured I deserved to cry now!

Remember how I said I had hired a photographer?  The best decision of my life!  I'm so glad I did.  She was amazing.  It was so nice to not have tell people to stand still, smile, pose...she just snapped away, all candid shots.  She posted this awesome one of us walking out of the airport!  I love it!!  I adore it!! I could look at all day long!

It's a picture of joy!  Complete happiness and the whole point to life...Family.  I even love that my ex husband and his little girl are in the picture.  We have worked really hard to continue to have a good relationship for the sake of our kids and each other's happiness.  Contention is just not worth it.  We are all happy and get along great!  It's the best kind of divorce to have.



Just like old times, these three can't take a serious picture!  Oh well...It was good to see them back in their groove! 


We went for dinner after the airport and took a minute for a couple of pictures outside. 


Below is a sweet video my Wasband's nephew made.  I didn't know he was doing this but it was such a wonderful surprise!  I love it.  I love that this moment was captured. 


I will post more pictures when I get them back from the photographer but I wanted to check in before anymore time passed.
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