Heart Failure to Transplant: Trip to Cedars-Sinai

The following was posted to my CaringBridge journal on Dec 10 and Dec 13, 2014:

Adam and I will be driving to southern California later today, taking Elliott with us. All the weather reports I've heard talk about how big and powerful the storm system and that is supposed to hit today will be. Please pray for safe and smooth travel for us! We have to be at Cedars-Sinai at 7 Friday morning, and then plan to drive back home late that day.

—

So Adam didn't end up getting off work early as he had hoped and we didn't get on the road until quite late.  The good news is that we stayed ahead of the storm, though the wind whistled loudly at our van windows as we went over the Grapevine.  We didn't arrive at my parents' house until after midnight. 

My auntie Christine drove my dad, Adam and me to Cedars-Sinai.  As my designated caregivers for after the surgery, at least one of them had to attend the appointment and it was a wonderful blessing to have them all there for support and company.  We left my parents' house at 5:15 and arrived at the Advanced Health Sciences parking garage a little after 6:30.  Traffic wasn't bad considering the pouring rain. 

The elevator up to the 6th floor from the plaza/lobby level didn't begin running until 7:00.  I used the time to pump. 

At 7:00, after we checked in, we were shown to an examination room with three extra chairs.  That is where we spent the rest of the morning, and all the members of the transplant team came to examine me and speak with us one at a time.

First a nurse took my vital signs.  Next, the transplant surgeon came to review my case.  He checked me for signs of fluid retention and listened to my heart sounds, then said he agreed a transplant is needed and timing is right to get me listed now.  Then, the social worker came in to discuss my support and care plans before and after transplant, and my ability to comply with the demanding schedule of follow-up visits, dietary restrictions and multiple medication regimen.  The psychiatrist came in next to perform a quick assessment of my cognitive abilities and attitude.  A dietitian went over the food choices that will give me best results both now and after transplant. 

One of the cardiologists on the team, Dr. Kittleson visited the room next.  She was very intense, energetic and direct.  She said almost word for word what Dr. Weisshaar said when we first discussed transplant.  Am I sick enough to need a transplant? Yes, based on the VO2max, Cardiac Index and general clinical picture of my symptoms and limitations.  Am I well enough, supported enough, responsible enough and emotionally resilient enough to thrive after a heart transplant?  Yes, based on my youth and otherwise healthy body, great family and community support, good attitude and faith. 

Dr. Kittleson said that my case will be presented to the whole transplant team next Friday for discussion and I will be listed at that time as a Status 2 candidate.

The final visit was from the financial coordinator.  I'm so grateful that my coverage with Kaiser is excellent, and the transplant surgery and follow-up care will only cost us our hospital visit, office visit and pharmacy co-pays.

The next part of the appointment was a "Lunch and Learn" with other candidates for heart transplant and their families.  Two nurses went over the process of getting on the list, how the waiting list works, what to expect in terms of follow-up care and recovery, and "bridge-to-transplant" options such as IV drugs and mechanical cardiac support. 

Lunch was from Corner Bakery and they had a gluten-free sandwich for me!  Yum yum!

Because I had already completed all the testing and evaluations at Kaiser Santa Clara, I was done for the day, but the other candidates had more testing to complete after lunch. 

We drove back to my parents' house to rest before the drive back up to Visalia. 

I'm very tired and doing my best to take it easy today.

This appointment solidified in my mind that I do in fact need the transplant, it is the right time to be listed, and gave me an even better picture and hope for life after transplant. Everything is falling into place. 

And now we wait....

Putting on the Armor of God

This summer I've been participating in Priscilla Shirer's Armor of God study.  On page 99, Priscilla poses the question:

How did the enemy take advantage of an upsetting, unexpected, chaotic event or circumstance to gain access to your life?

Well, I had a heart transplant 17 months ago.

It all happened quickly.  April 2014 I gave birth to my third baby and a week later was admitted to the hospital with congestive heart failure due to a genetic condition that I had been told wasn't very serious and most likely wouldn't be a problem.  Six months later, I learned that I needed a heart transplant to save my life.  Our family had to temporarily move four hours south to live with my parents a few months later as I waited for and recovered from the transplant.  Then we moved back and have been struggling to find the "new normal" since then.

How's that for an upsetting, unexpected and chaotic event or circumstance?

Those days of heart failure were hard to be sure, but at the same time, it was a beautiful time in my life.  I felt loved, supported, at peace and so very close to God.  It was pretty easy to be heavenly minded when the physical body was declining daily and rapidly.

My post transplant spiritual life has been rocky by comparison,

In the months after my transplant, I was recovering physical wellness, regaining energy, regaining strength almost daily.  I was on an emotional high, so happy and grateful to be alive.  I felt so very much better and could hardly believe there was any limit to how much better I could feel.  It seemed within reason that I was going to be "normal" very soon.

Hello, pride.

God had felt so near.  But slowly, He felt farther away.  Who moved?  That would be me.

Old struggles I thought were resolved cropped back up.  From my high school and college days, God had slowly and painstakingly brought me out of perfectionism, legalism, and the bad habit of measuring myself by my accomplishments or comparing myself to others.  In the past 17 months, I let those snares entangle me again.  I struggled mightily to meet my own expectations and the perceived expectations of others, and despaired when I failed.

Why couldn't I keep up? Maybe I was just lazy.... Maybe I needed to push myself harder.  Maybe I just needed better systems and strategies..  I pushed myself and berated myself for not being able to do and accomplish as much as I thought I should be able to do.

All along, the real and biggest problem was that my focus had moved off of Christ and onto myself, to my struggles and circumstances.  I felt irritable, short-tempered, selfish, restless, unbalanced, uncentered. I was half in denial about the limitations living with a transplant places on me, while also resenting those limitations at the same time.

The enemy had used his usual tactics of deception and hitting at my weak points to pull me down to be sure.


I missed the first three weeks of the Armor of God study due to serving at VBS and needing to get my blood drawn to verify my medicine levels.  Last week I went back through the first few weeks of lessons to catch up.

God's word does not return void and it came at just the right time to rescue me from the pit I had fallen back into.  It opened my eyes to the reality of the enemy and the way he works, but more importantly, reminded me about my spiritual identity and spiritual resources.

With a refreshed and renewed understanding of who I am in Christ, I've been able to better come to grips with the truth of my physical reality as well.

I'm accepting the fact that living with a transplant is like living with a chronic illness or condition.  This new level of acceptance sparked curiosity, and I set out to find out as much as I can about the whats and whys of limits.

Here's what I learned:

Although my new heart works so much better than my old thick, stiff, failing one did, it has specific limitations because of the nerve connections that were severed when the heart was transplanted.

The vagus nerve is the most efficient way for the body to adjust heart rate and blood flow as needed to accommodate for changes in activity level, body position, and external temperature.  But God designed a back-up plan: hormones from the adrenal gland.

But there is a catch: The hormonal pathways my body now uses to adjust my heart rate and blood flow is much slower to turn on and shut off than the nearly instant adjustments the nervous system can make.

As a result, I "feel the burn" sooner when exercising or doing heavy housework, and have more soreness afterward that lingers longer than it otherwise might .  Getting up from a seated or squatting position quickly causes momentary low blood pressure and dizziness, which can be bothersome when picking things up off the floor for example.  If I let myself get too cold, it seems to take forever for me to feel warm again,  More bothersome to me is how much lower my stamina is and longer my recovery time is when the weather is hot.


That is part of the terrain I am traversing.  Other features of my terrain are room for improvement in communication with my wonderful husband who has to work longer hours than I'd like, three active and intense children including a daughter with behaviors on the autism spectrum (we are in the midst of evaluations for a diagnosis) ... those are other features of my terrain.

God is with me, and has not left me helpless or defenseless in the face of these circumstances.  He has blessed me with every spiritual blessing in the heavenlies, but I have to activate them. Richard A. Burr came up with a clever acronym: Prayer Releases All Your Eternal Resources.

Asking for what I need with gratitude relieves anxiety and promotes peace.  I can cast my cares on Him because he cares for me.        

God has been faithful and good through it all.  Here are a few ways he has provided and blessed me:

In my search for truth, I was led to a website for Christians with chronic illness: Rest Ministries.  There I found beautiful words of vulnerability and dependence on God in the midst of suffering.  The prayer cards they offer for download there have been helpful to me as well.

A sibling rivalry e-course by Connected Families we started last month is equipping our family with practical ways to use the shoes of peace in our family relationships.

These months have not been wasted.  Some of what seemed like backward movement may have been necessary to clear the way for new growth in the future.  Certainly, the process of re-evaluating who I am has led to a better certainty and understanding of my gifts and callings.  Creative communication and teaching along with hospitality, which I share with my husband, round out my top three.

This blog is one way to live out my calling.  Opening my home to host La Leche League meetings starting next month is another.  And last but certainly not least, my husband and I will be exercising our shared gift of hospitality by welcoming an exchange student into our family for the coming school year.

I'll be back soon to share more about my journey and what I'm learning from God's graceful discipline.

Heart Failure to Transplant: What to Expect ...

This post went up on my CaringBridge journal on Feb 9, 2015:

I can't help but compare the journey to transplant to the journey of pregnancy and birth. 

Both involve a process of (somewhat) uncertain length, that will be a unique experience for each person who goes through it, but yet for all who go through it there are many experiences that they will share in common. 

The exact moment when pregnancy shifts to labor to birth is unpredictable and (mostly) out of the mother's control.  The exact moment when a heart is found, the process of prepping for surgery, and exact time and date of transplant is likewise unpredictable and out of my control. 

The birth of a baby can be prepared for but becoming a parent changes the mother's and father's and siblings' lives in unexpected, dramatic and irreversible ways, and transplant similarly signals the start of a new way of life. 

Pregnancy and birth change the mothers' body; transplant and immunosuppression does as well.  

Childbirth classes and books on pregnancy, birth and parenting abound, and when I was pregnant the first time I found them interesting and helpful.  Similarly, I've read through all the manuals Kaiser and Cedars-Sinai have provided me covering the facts of life before and after transplant. 

However, in the past few days I've been seeking out and enjoying reading others' personal accounts of their heart transplant journey. 

As I read details of their days leading up to transplant, I identify with many details: the fatigue, shortness of breath, uncertainty, difficulty taking it all in and feeling like it's all going so fast. 

Yet other aspects of other patients' stories make me realize anew just how blessed and protected by my Creator, Redeemer and Sustainer I have been.  Many patients get much sicker than I am now before a heart is finally found, many do not have much of a support network, many have other physical and mental health issues that complicate their recovery, many have problems with their insurance coverage, many experience delays and denials of their claims for disability. 

As I read the stories, I find the moments just before surgery and in the first few days of recovery most compelling because that is what I am most uncertain about.  It seems like there are a wide range of experiences, some have complications and pain, others sail right through.  I find it comforting in a way to know in advance some of the possible outcomes, so that if one of them happens to me I will know it's within the range of "normal"! 

In one of the stories, a woman talked of a moment a year or so after her transplant when she had a mental shift from "transplant patient" to "someone who happened to have had a transplant" and felt a new sense of freedom to enjoy life.  Thinking of what I have read of what to expect in terms of post-transplant care, that shift probably corresponds in part to the less frequent checkups and lighter medication load that happens around that time as things stabilize.  I can expect that I will feel like a patient for a while... but looking forward to the day when it's mostly behind me and the medication regimen and caring for myself as a immunosuppressed person will be assimilated as just my normal state of being, background details as I live out my vocations to love God and love others through my role as wife, mother, community volunteer and whatever else I may find to do in the future.

Heart Failure to Transplant: The Initial Consultation

The following was posted on my CaringBridge journal on Oct. 14, 2014:

The trip to Santa Clara was a little rough on Mr. Elliott.  He slept in 45 minute to an hour bursts, and then cried no matter what we did for about 45 minutes, and it just kept alternating like that.  In all I think we stopped four times on the way up and twice on the way back (thankfully he slept soundly for the second half of the trip home). 

I was so happy to have my parents with me yesterday.  It was fun to spend time together on the road and in the waiting rooms, and it was wonderful to have them there to process the results of the appointment.

The meeting with Dr. Weisshaar was so helpful.  I really love that she tells it like it is, very directly and frankly without leaving things vague or implied.  She has a great understanding of my condition and heart failure in general and is very skilled at explaining things clearly.

Our appointment together was quite lengthy.  First I gave a summary of what I knew about my condition.  Then she asked me a lot of questions about my medical history and current symptoms, and did a physical examination to check my blood flow, fluid levels and pulses at various points of my body.  Next she gave a synopsis of what hypertrophic cardiomyopathy is and how it can lead to death. 

1) Through sudden cardiac death from fatal arrhythmias.  I'm not at high risk for this.

2) Through heart failure as the muscle thickens and stiffens and loses the ability to pump blood. 

She explained how they assess the risk of death from heart failure.  The best prognostic tool they have right now is the VO2max and VE/VCO2 slope, which I explained in the previous post.  My numbers there are quite bad, which is why she recommends seeking transplantation sooner than later to give me the best chance to live, and a renewed quality of life.

She explained that waiting for a heart transplant is like waiting to board a plane with Southwest.  There are different lines for each of the blood types, and then within that line there are three risk categories: 1A = imminent death within a few days to weeks without a transplant, 1B = on IV medication or hospitalized with symptoms, and 2 is for everyone else who is sick enough to need a transplant but not sick enough to be a 1A or 1B.  The O line is the longest because it's the most common blood type. 

Each time a heart becomes available it goes down the priority list until a match is found.  The first person on the list may need a bigger or smaller heart than what is offered, or have antibodies against the heart that is offered, and so they'll keep checking down the list until the right match is found.  There are about 4100 people on the heart transplant list right now, and only about half of them will get a heart this year, mostly 1As and 1Bs, but rarely some 2s.

She talked to us about screening first degree relatives.  I'm in the midst of having genetic counseling and testing done.  If they can identify a gene related to my cardiomyopathy, then they will test my siblings and kids for that gene.  Those without the gene are free and clear, those with the gene will require echocardiograms every two years through puberty and five years thereafter to screen for evidence of disease.  If they don't identify which gene it is, then all first degree relatives will need to be screened on that schedule, because given my paternal grandfather and aunt's medical history with heart failure, it's clearly genetic even if we can't pinpoint the gene.

She explained some of the transplant workup process and some of the recovery process.  After I get on the transplant list I need to be seen once per month, but some of those can be video appointments as long as I go to Santa Clara at least once every three months.

I did some research into the IV drugs that I will likely be given during my likely upcoming hospital stay, which is scheduled for Thanksgiving week.  There are three choices of inotropes that may be given.  I called Infant Risk to find out their compatibility with breastfeeding.  Two of them are L2 - Possibly Compatible with Breastfeeding.  One of those has a risk of reducing milk supply.  The third drug is L4 - Possibly Hazardous to the Baby.  This means it should only be used in a lactating mother in a life or death situation, and it's best if breastfeeding is interrupted while the drug is administered and be resumed after the drug passes from the blood, in this case 8 hours after the last dose. 

My breastfeeding relationship with my boys is very important to me.  I'm OK with Zachary being done at this point although I will miss having that tool to comfort and connect with him, but I'm really not ready to wean Elliott.  I'm going to do everything I can to avoid weaning for as long as possible.  I will pump milk for when we are separated, try to minimize our separation, ask that the L2 drugs be tried first, and other strategies.

After Dr. Weisshaar and I finished talking, then she sent in a case worker and a nurse and one of the other cardiologists to meet me.  By the time we were done and headed to the lab for the first round of blood work, it was already 3:45.  The first step of the workup process was to give about 12 vials of blood, some of which had to be sent to Stanford for analysis.  We had to wait quite some time while they figured out if they could get it to Stanford in time.  I was relieved that my blood flowed well and quickly and I didn't feel woozy at all. 

Once the blood work was done we went down to the lobby for a final potty and nursing break before hitting the road.  After I washed my hands in the restroom I noticed a woman in a wheelchair behind me, trying to make the automatic paper towel dispenser work.  She seemed to have cerebral palsy or something similar that made it difficult for her to control her movements, so I offered to help and waved my hand in front of the red light and pulled the towel off for her.  She asked me if I would pick up her purse which slid off her lap and fallen to the floor below the sink area.  We were the only ones in the restroom, so I offered to hold the door open for her as well.  I felt so glad to be able to help her, but when I came out of the restroom my parents told me they had been worried that I passed out in there!

Heart Failure to Transplant: Still Waiting

The following was posted to my CaringBridge journal on Feb 16, 2015:

This morning when Adam started a new bag of medicine, we discovered that the pump had quit working almost 25 hours before.  I'm not sure why I wasn't aware of it at the time, but I went a full day without the dopamine.  Did that affect me?

I felt good most of the morning yesterday.  In the afternoon, Adam and I took the kids to the OC Great Park to ride on the carousel.  That went well but took a lot more out of me than I was expecting it to.  As the evening went on I felt increasingly fatigued and heavy and weak.  This morning, Zachary woke up early but I couldn't seem to fully awaken to get up with him.  Instead I spent the next few hours in and out of dreams that all had a common theme of me having a hard time keeping my eyes open.  When Adam finally had me get out of bed at 9:15 so he could switch my medicine, I felt more wiped out than I have in a long time.  Every time I moved at all, every one of my muscles would shake and twitch as if I were a weightlifter who had just finished an enormous number of repetitions. 

Coram Infusion Services who supplies my medicine called shortly after I woke up to schedule my next delivery and I told them what happened with the pump.  They will be sending a replacement by courier this afternoon. 

Next I paged the on-call heart transplant cardiologist at Kaiser Santa Clara to give them an update about the pump failure and resultant fatigue.  Dr. Weisshaar called me back and says it sounds like the dopamine is doing something for me after all... it's kept me from having these severe payback days.  She seemed to think that now that it's restarted I may find the recovery period shorter than I would have without it.  I'm feeling less shaky but still VERY tired.

Whether or not it shortens the payback period, I'm glad to know the dopamine is actually doing something for me, even if it is just keeping my condition more stable while I wait.

How much longer will I be waiting?  It's impossible to predict or know for sure.  The call that they have a heart for me truly could come at any time.  Every time I speak with a member of my care team, they say, "Hopefully the next time I talk to you, you will already have your new heart."  But on Thursday the Cedars-Sinai transplant coordinator, Jenna, told the Kaiser transplant coordinator, Lena, that she is confident that I'll have a new heart no later than the end of March.  So it could be tonight, or it could be 5 weeks from now.  

The end of March sounds far away now, but in the scheme of things it really isn't that much time (only 35 days or so) and will go by quickly if indeed I do have to wait that long. 

I'm anxious for things to move forward, but am truly powerless.  All I can do is trust God's timing and learn to be content and enjoy my present circumstances. 

My title has a double meaning.   The first is plain: I'm still waiting in the sense that the heart transplant has not happened yet and I have no choice but to continue to wait.  The second is more of a goal than a reality: the Spirit is reminding me of the need to be still while I wait, to allow Him to still my anxious thoughts and replace them with trust and assurance and praise and surrender.  Yet as it is written, "the spirit is willing but the flesh is weak."  It's an ongoing struggle, and I am in need of your prayers.

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Heart Failure to Transplant: Test Results

The following was published on my CaringBridge journal on Oct 14, 2014:

Bottom line:  I need a heart transplant sooner than later to save my life. 

My VO2 max before I had to stop the test due to dizziness was 9, which is 31% of what would be expected for someone my age, and the ratio between the amount of breaths I took per minute and the amount of carbon dioxide exhaled (VE/VCO2 slope) was 46.  For reference, someone with a VO2 max below 12, or below 50% of age expected performance, and/or VE/VCO2 slope greater than 35 have shown in studies to have 66-75% risk of death within one year.

My cardiologist recommended that based on my O positive blood type that I begin the workup process right away to determine if the functions of my other organs and immune system will allow for a heart transplant.  The workup process involves lots of blood work, meetings with a social worker and psychologist, and a test called a right heart catheterization to measure the blood pressure in my lungs.  I likely have to be hospitalized for about a week after that test because they are going to attempt to lower the blood pressure in my lungs by trying different combinations and levels of IV medication.  If they find something that works, then I'll be on IV medication until an matching heart is identified.

The cardiologist said if I'm on IV medication that puts me in a higher priority class on the transplant list, and it's realistic for a matching heart to become available within the year. 

The recovery process will be quite long.... 6-8 weeks before I can drive or lift more than 5 pounds, 3-6 months before I can resume my mothering duties at the level I'm at now, 6 months to a year before I am up to my new improved full capacity.  It will involve moving temporarily to within 20 minutes of whatever hospital does the transplant for the first month of recovery. 

It's a lot to take in.  I really don't like the idea of being cut open or having to hope that someone dies so I can have their heart; on the other hand, I want to be around for my kids and Adam as long as possible.

Heart Failure to Transplant: Take this Cup

Later on Oct 16, 2014 my emotions grew darker:

God my God
I cry out
Your beloved needs you now

...

On Monday I was given a large packet of informational flyers, leaflets and booklets. I started reading the easy stuff, diet tips, lists of appropriate foods, sodium content of fast food, other recommendations for self care and healthful living.

Next I read the overviews of cardiomyopathy and heart failure.

This morning there were only two booklets left to read, neither of which I really wanted to look at:

1. Advanced care directives
2. Your care after a heart transplant



I decided to make myself read the post transplant care information.

I found a long detailed accounting if the multitude of drugs I may have to be on for the rest of my life and their many possible side effects, such as damaging my liver and kidneys, the always popular nausea and vomiting, causing high cholesterol, high blood pressure, excessive body and facial hair growth but regular hair loss, and acne.

I found a detailed description of the right heart catheterization test I'll have in November and which they will also use to perform frequent heart biopsies to check for rejection.

I found a discussion of rejection, how it it could happen at any time, the risk lessens but never goes away over time, and most transplant patients have some degree of rejection in their first year. With early detection it can be reversed before the heart is damaged enough to require a second transplant so its important to be alert to symptoms. But sometimes there are no symptoms, and sometimes the symptoms are the kind of mild "just don't feel right" things anyone could get when fighting off an infection.


I found discussion of how my new heart won't have any nerves and so may not speed up and slow down appropriately.

Its not as simple as new heart, cured. Those unpleasantries I just listed will always be part of my life.

And if I had a say, I don't want them to be part of my life!



Is the cure worse than the disease?

But, then again, I want to live!

Really, I want to find out this is all a bad dream or a big embarrassing mistake.

I want a miracle.



In some ways, I'm less afraid of death than of the suffering ahead.

Jesus prayed for the Father to take the cup of suffering and death away, and then said yet not as I will, but thy will be done.

That is also my prayer. I want to live for the sake of my family and because there is so much in this life that is good.

I will submit to the suffering ahead if God wills it, if He ordains my journey here is not done.

And in the midst of all the waiting, anxiety, failing strength and suffering ahead, in bad days and on good days,

I will lift my eyes to the maker
of the mountains I can't climb
I will lift my eyes to the calmer
of the oceans raging wild
I will lift my eyes to the healer
of the hurt I hold inside...

Heart Failure to Heart Transplant: Hurry Up and Wait

I posted the following to my CaringBridge journal Nov 8, 2014:

Dr. Nishime came to see me this morning.

She examined me, discussed treatment options and for now we decided to discontinue the Lasix to see if maybe it is pulling too MUCH fluid out of me.

She said that people with diastolic heart failure like I have don't tolerate extremes well. We do best when our blood pressure, fluid level, and heart rate are all in a nice targeted zone.

I look good, so the fact that I'm feeling so bad needs investigating. The leading theories right now are:

1) slight dehydration/low blood volume from too much diuretic (testing by stopping the diuretic and upping my liquid intake today)

2) pulmonary hypertension (right heart cath test needed to confirm or rule out)

3) low heart flows because my heart muscle isn't relaxing enough (again right heart cath will tell us more).

So I need a right heart catheterization. They don't usually do them on the weekends except for emergencies. The cath lab is schedule is booked tight on Monday because the nurses will be going on strike on Tuesday and Wednesday and they are not allowed to do any procedures on those days unless they are an emergency.

Dr. Nishime was going to see if they can make an exception and have the right heart cath done for me tomorrow or possibly get me squeezed in on Monday. If not I will have to wait until Thursday to have one.

So today is a bit of a wait and see day.

Dr. Nishime is coordinating with the lead heart transplant nurse to figure out what I am still missing from my workup and then see if we can get any of it done today.

Dr. Nishime also offered to start me on IV dopamine just to see if that will be effective to relieve my symptoms before we do the right heart cath, but use of IV initropes like dopamine will possibly prevent me from participating in the drug trial, so we decided to wait to try them until after the right heart cath is completed and we know for sure if they are needed.

I need to contact the lead investigator of the drug trial Dr Pham and find out if a trial of dopamine that is discontinued would disqualify me from participating or not. I also need to figure out how to get him my medical records ASAP.

Heart Failure to Transplant: Diagnoses

I posted the following on my CaringBridge journal in 2014:

In 2010 I saw my primary care doctor to investigate the irregular heart beats I had been feeling.  At first she wanted to brush it off as just anxiety, but decided to do an EKG just in case.  After reading the results of the EKG she was insistent that I see a cardiologist right away because the pattern of my heartbeats was consistent with left ventricular hypertrophy.  The cardiologist did an echocardiogram and confirmed the diagnosis of Hypertrophic Cardiomyopathy (HCM).

He told me it wasn't a big deal, just mostly annoying because of the palpitations, which I could control by avoiding caffeine and chocolate.  Oh, but in rare cases some HCM patients experienced Sudden Cardiac Death.  Mostly athletes, so just avoid strenuous activity and I should be fine.  Come back once or twice a year to make sure it wasn't getting worse, and that would be it.

Fast forward to 2014, and a few days after giving birth to my third baby, I was diagnosed with pneumonia from a chest x-ray.  Antiobiotics didn't make a difference, and a few days later, I went to the ER with extreme shortness of breath worse when lying down, new swelling in my lower legs, and one instance of coughing up blood.  They performed a chest CT and then admitted me, I thought for severe pneumonia. 

They treated me with IV antibiotics and Lasix, and performed an echocardiogram. As I neared readiness for discharge two days later, a doctor informed me that the primary reason for admission wasn't the pneumonia which was actually not that bad, but the pleural effusion related to heart failure.  I was sent home with Lasix to take and instructions for a low sodium fluid restricted diet until the remaining of my leg swelling dissipated.  Then I threw it away, because I was sure everything would go back to the way it was.  I stopped watching my sodium intake and limiting fluids as well.

I had an echocardiogram a month later and because of the results of that was referred to the Heart Failure and Transplantation clinic in Santa Clara.  My heart now had thickening on the right ventricle as well as the left, and the atria were dilated.  I had a few phone appointments and did some blood work remotely.  The doctor wrote me a new prescription for Lasix, which I kept forgetting to fill.  I understood that I should take the Lasix only if the leg swelling retuned, or if I gained weight rapidly.  I was supposed to weigh myself every day, but sometimes forgot.  Toward the end of the summer, the times I did weigh myself I noted the scale inching up a bit and my waistline getting a little thicker but figured I may have been slightly overeating or something.

Then at the beginning of September, the extreme fatigue and shortness of breath/discomfort when breathing returned.  I felt sure I had pneumonia again.  On the phone with the advice nurse, I was informed that my chart showed I have pulmonary hypertension (PH).  To my recollection, that was the first time I had been given that information, so I double checked with my PCP who confirmed that my most recent echo did show PH.  A chest x-ray ruled out pneumonia, so I was referred back to my local cardiologist, Dr. Z.

The cardiologist in Santa Clara who had been following my case had left Kaiser, but before he left he had mentioned wanting me to have an exercise tolerance test to see if I needed a implantable defibrillator or pacemaker.  I had been waiting for someone to call me to set the appointment and then had almost forgotten about it. 

Dr. Z got me in touch with another cardiologist at the Heart Failure and Transplantation clinic, Dr. Weisshaar.  She put the order in for the Treadmill EKG, and answered all my questions very clearly and directly. 

The PH is secondary to Stage C, Level II or III (depending on if I'm having a good day or bad day) Heart Failure, which is a result of my cardiomyopathy.  Because of my young age, I most likely will need a heart transplant one day, but hopefully not for a long time with proper lifestyle management.

I filled the prescription for Lasix, adopted a strict low sodium diet and began weighing myself faithfully first thing in the morning and last thing before bed.  I lost four pounds of water weight in the first two days and continued to lose a pound a day for the next few days.  My thickened waistline disappeared, and my breathing eased a bit. 

However, I'm still feeling worse now than I did over the summer.  I fatigue and get winded easily, and sometimes get lightheaded.  My resting heart rate is slow, between 57 and 60 beats per minute, and my blood pressure is quite low. 

I had the Treadmill EKG on September 24.  According to Dr. Weisshaar, the results mean I don't need an implantable defibrillator or pacemaker, but because my exercise capacity is so poor, they want to do an VO2/cardiopulmonary stress test to determine why. 

On October 13 I'll be driving to Santa Clara to ride on a stationary bicycle while hooked up to an EKG and breathing into a special tube.  Best case scenario, they discover my poor exercise tolerance is just because I'm deconditioned (fancy talk for just really really out of shape).  Worst case, they determine that I might need a transplant sooner than originally thought.

My case is a little tricky to treat with medication because of my slow heart rate and low blood pressure.  Most people in heart failure have rapid heart beats and high blood pressure, so most of the drugs approved to treat heart failure work by slowing the heart rate and lowering blood pressure. 

Heart Failure to Transplant: Estimating Wait Time

The following was posted to my CaringBridge journal on Jan 16, 2015:

The transplant list is kept nationally, but it is not exactly first come first served.  Time on the list is only one of many factors that go into determining who receives the offer when an heart becomes available.

Say that someone experiences a traumatic injury and is declared brain dead, and has stated their preference to be an organ donor.

If this happens in Los Angeles, the transplant coordinator would first look for a matching recipient in the Organ Procurement Organization (OPO) sub-region that LA is part of (I believe it's Region 5).

The transplant coordinator would look in the database of transplant candidates within Region 5 to see who matches the donor in terms of size, blood type and other immune factors.  I think they also consider age of the donor compared to age of the recipient.  They wouldn't give me a 70 year old heart, for example.

If more than one candidate is a good match for the donor, then they look at how sick the candidates are.  This is where the priority statuses come in.  1As get priority, then 1Bs, then Status 2s.  

If there are no 1As and more than one 1B that is a good match for the donor, then it comes down to who has accrued the most time waiting.  I was placed on the list officially as of January 12, so my wait time starts accruing from that date.

If there are no matches for the donor at all in Region 5, then they would offer the organ to matching candidates in neighboring OPOs (there are two covering Northern California and one that covers San Diego).  The process would be repeated for neighboring regions in Oregon and Nevada and so on until a matching recipient is found.

My blood type is O, the most common type, which puts me in a large group of candidates.  But my petite size means I need a small heart.  

Cardiomyopathy like I have is a relatively rare reason for heart failure.  More common is coronary artery disease and heart attack.  Those on the list for those reasons likely tend to be older and bigger than me.  Those who experience traumatic injuries and brain death tend on the whole to be younger (because of more risk-taking behavior in the young) and potentially have hearts on the smaller end of the spectrum. 

So, there are fewer candidates of my size and potentially more donated hearts that match my size, which explains why the Cedars-Sinai team feels that I will likely be getting offers sooner than later.  

Heart Failure to Transplant: Right Heart Catheterization

This post went up on my CaringBridge journal on Nov 26, 2014:

On the way to Santa Clara, I read portions of a book called Christ the Healer.  It talked about God's compassion, willingness and power to heal, which was very encouraging.  I'm fully convinced He will give me a new heart, one way or another.

The night before the test, Adam and I took advantage of the kid-free time and went to see Interstellar.  Wow!  What a great, thought provoking movie!

When I went to sleep the night before the test, and when I woke up in the early morning hours to pump, I thought about how wonderful it would be if God chose to heal me that morning, so that when they looked at my heart during the catheterization, they'd say, "Wait a minute!  Do we have the right patient? Why are we doing this? This heart is completely normal!"

When they took me back for the test, it was chilly in the room and one of the nurses took a blanket from the warmer and placed it over the operating table, and then put a few more warm blankets on top of me.  Another nurse covered my hair with a turban to keep it clean in case of any blood spatter.  I said I felt like I was at a spa, and she said that was the first time she'd heard the cath lab called a spa!  I said I was just trying to make the best of the situation.  Even funnier, when Dr. Parekh came in to begin the procedure, she said, "With that turban on your head you look ready for a day at the spa!"  The nurses started joking about getting me some cucumbers for my eyes, or painting my toenails during the procedure.

Genevieve, the medical student from Stanford, came in to observe the procedure, and Dr. Parekh offered her the chance to inject the lidocaine and insert the catheter to my neck vein.  Unfortunately, she had trouble with getting the catheter in properly, and after three unsuccessful tries, Dr. Parekh had to step in.  Dr. Parekh even had a little trouble and said she felt resistance in my vein almost as if there were scar tissue there, which is very odd because I've never had procedures or injuries of any kind in that part of my body as far as I know.

They took their measurements by moving the catheter around, inflating and deflating a balloon, and flushing the line with saline.  It provoked some palpitations which tickled and made me cough a bit. 

The good news is that the pressures in my heart and lungs are normal.  The pulmonary hypertension I had in May is officially gone!  Praise God for healing!

The other measurements they took were to determine how much blood flows out of my heart with each beat.  There are two ways they can measure or estimate that amount.  One involves calculations based on the saturation of oxygen in my blood at different points, and the other involves flushing the line with saline that is a different temperature than my blood and observing how long it takes for the saline to move all the way through the heart by tracking the temperature change.  I don't recall which method yielded which result, but one method showed a ratio of amount of blood pumped in one minute to my total body area (Cardiac Index or CI) of 1.8, and the other method showed a CI of 2.1. 

For comparison, a normal heart has a CI of 2.6 and above, and CI at 1.8 or below is often associated with lack of blood flow to vital organs, a condition called cardiogenic shock.  My heart is not working well at all. 

The low CI explains all of my symptoms.  In fact, with a CI that low, a clinician might expect me to be doing worse than I am.  However, my blood work continues to show that all my other body systems are functioning normally... no kidney failure, no liver failure.  I credit my Jehovah Rapha for protecting my organs and allowing me to live a full of life as possible while I'm waiting for the new heart He will give me.

Dr. Parekh said there is nothing more they can do for me medically, except to get me on the transplant list at Stanford, Cedars-Sinai or both.

That's fine with me because "nothing more we can do" is exactly the situation where God has the most opportunity to show his power and glory. 

Heart Failure to Transplant: Acceptance Feels So Good

This post was first published to my CaringBridge journal on Dec 21 and Dec 29, 2014:

Got the call Friday morning that the selection committee at Cedars-Sinai had discussed my case and decided to accept me into their transplant program.

For some reason that particular way of phrasing it tickles me.

It feels good to be accepted.

Acceptance is the first step on the road to healing.

Apparently there were some final checks to complete before they submitted my name to be listed with UNOS, the national organ sharing network, so I don't think I'm officially on the list quite yet.

Cedars-Sinai has an excellent transplant program. They do more heart transplants per year than any other transplant center. They are pioneering new techniques and protocols for transplant surgery to maximize the functionality of the new heart and shorten recovery time, and also to improve post operative care and medication regimens to minimize the chance of rejection.

I still pray for a miraculous healing so I can keep my own heart, but Cedars-Sinai is a wonderful provision and answer to prayer too.

—

Cedars Sinai called Kaiser and said I have a few more tests to do: 24 hour urinalysis, Pap smear and more blood work. They said no rush, it can wait until after the holidays.

I have a video appointment on January 5 with the heart failure team of Kaiser Santa Clara.

Right now I have a yucky sneezy head cold. :(

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Heart Failure to Transplant: Social Worker Appointment—Logistics

I posted the following to my CaringBridge journal, describing events from Nov 25, 2014:

We left Tulare on Monday at 7:00 or so.  There was an accident blocking 152 and traffic came to a dead stop near Santa Nella so we rerouted up to the 5 and took 580 into Santa Clara.  My first appointment was at 11, but we arrived at Kaiser Santa Clara at 11:30.  We had called ahead to let them know of the delay and they worked with us to juggle the appointments. 

First we met with Janet the social worker.  She went through the detailed logistics involved in the transplant process and asked us questions about our options and plans. 

Background Info:
There are 250 transplant centers in the US grouped into different Organ Procurement Organization (OPO) regions.  When organs become available they are first offered to centers within the original OPO region.  Each transplant center maintains their own list of patients eligible for transplant.  It is possible to be listed at more than one center, but there is no benefit to be listed at multiple centers within the same OPO region.  Most of the patients who work with Kaiser in Northern California get on Stanford's list.  But some of their patients can be put on the list at Cedars-Sinai, which is in a different OPO region, instead of or in addition to Stanford to either increase chances of finding a matching organ or ease the burden of care on the patient's family/support network.  I'm not sure if the decision is completely up to me, but we let them know we preferred Cedars-Sinai because of my family support in Southern California. 

Here is some compare and contrast of what is involved in pre and post transplant logistics at each location:

Getting On the List:
STANFORD: Present my case to transplant team next Friday, they will decide that afternoon whether or not they will accept me as a Priority 2 transplant candidate. 

CEDARS-SINAI: Present my case to transplant team via email/fax/mail at some point in the next few weeks, but they wouldn't make a decision about whether to accept me on to their list until I can be examined in person.

Pre-Transplant Travel Restrictions:
STANFORD: I have to stay within 4 hours of Stanford at all times once accepted onto the waiting list, and report to Kaiser Santa Clara for monthly clinic visits and lab work.  This means I won't be able to visit my family in Southern California until after the transplant.

CEDARS-SINAI: I have to stay within 4 hours of Cedars-Sinai at all times once accepted onto the waiting list, and report to them for monthly clinic visits and lab work. Not sure what this means in terms of being able to drive to Selma or Fresno for other activities or appointments...

Post-Transplant Travel Restrictions:
STANFORD: Once transplanted, I would stay in the hospital for 10 days to 3 weeks, and need to stay within 20 minutes of Kaiser Santa Clara for daily/near-daily follow up care the first 30 days after release.  Kaiser would reimburse me for lodging up to $100/night. 

CEDARS-SINAI: Once transplanted, I would stay in the hospital for 10 days to 3 weeks, and need to stay within 20 minutes of Cedars-Sinai for daily/near-daily follow up care the first 30 days after release, and in the area for a total of three months.  Kaiser would pay in advance for lodging up to $100/night, and in addition provide a per-diem stipend.

Post-Transplant Care:
At either center, I would need a primary companion to provide care/supervision 24 hours a day during the first 30 days after release from the hospital, with a secondary person available for respite care.  We also need to arrange care for Charlotte, Zachary and Elliott.  I have a large extended family in Southern California that could step into these roles more easily if we are down there near Cedars-Sinai, which could also potentially minimize the separation for the kids by allowing for more frequent visits.  By contrast, I know very few people in the Bay Area, and Adam's extended family is all here in the Central Valley.