Processing…

We all go through many stages of grief when losing something you love. What if that something is your health and you have absolutely zero control over it. No amount of exercise will make it better;  no amount of supplements natural or manmade can help; no amount of talk or research will help; not even eating healthy can make it better. This is how I am feeling!

Every year I suffer another hit to my health and I think to myself, “If I can just survive this, I can do anything.” But each time I get knocked down, it becomes harder to harder for the emotional energy to get back up.  I know I’m usually all about positivity and I would think – “but I get up and fight even harder the next time.” I am not going to say that. This is a vent blog post about the emotions I am feeling and there physical impact on me.

I would normally vent to myself, my dad, my mom, Jake, or my psychologist , but I started this blog to tell the truth about my journey. Jake and I are getting married on October 28th, less than 39 days away. This is supposed to be the happiest and most exciting time in your life, looking forward to a bright future. Young and free, so optimistic; right?  Yes, but see when I presented with new symptoms and knowing that something is wrong, I went into survival mode. My world stopped again and our wedding had to take a back seat.  How am I supposed to think about our future when I feel so sick?

I’m scared to move forward because I know that as I get older that means my diseases will progress. I wish I can just yell STOP and everything stand still like my world has been. I see the hate in the news and around the world.  I think in times like these, I wish everyone could feel what it’s like to see the world through someone else and maybe there wouldn’t be so much hate, but thats a different topic. I just can’t understand why so many people are using up so much energy for hate and protesting, when I’m sitting here wishing I could just go outside. I haven’t for several months due to multiple wildfires within an hour of our home.

I am dealing with the realization that this could be my new normal.  It a scary thing to come to terms with.  Yes, the fires cause the exacerbation of my lung diseases but we still don’t know if this is permanent.  We just don’t know and thats scary for anyone, let alone someone who is only 24. I have always known that it could be worse and that nothing is guaranteed.  I am just now processing that this may be my new normal right and I don’t know how to come to terms with it.

In the past, it would take months and months and even years of working on accepting and learning to cope with my new normal.  That is no longer the case.   I am scared and the last thing I want to think about is the future. Trying to take it one day at a time is agonizing. I want to enjoy my last month as a single women and live, breathe, and think wedding but thats not me and I can’t do it.

Meeting now with a medical team that specializes in advanced pulmonary hypertension is terrifying.  It is another sign that my disease may be progressing to the next point.  After my heart cath on August 29th, we found out that my pulmonary pressures are higher and now I am realizing that I might not have a lot of years left where I remain stable. This is the reality that we are in and I really don’t know what my health will do. My Physician’s team also now includes the heart / lung transplant physicians. I am nowhere near that but when all else fails, its transplant time.

I was fortunate for a number of years that I only take one medicine for my pulmonary hypertension but I am now moving to combined therapies – which brings a whole new set of issues. The therapies will impact me. This is an awful time for my health to start to decline. I only hope, wish and dream that its not like the past where I was in a fog, and I hope for just one day my pain doesn’t ruin it, and  I can fully enjoy our wedding with the love of my life, Jake.

But I’m Getting Married…..!!!

Health-wise, the last month and a half have been very hard for me.  On July 5th, we had yet another wildfire near home. This fire came over the top of our mountain. That day, I was experiencing a full-blown migraines. It was getting bad, and thankfully my mom just happened to come home early, not knowing that I was suffering badly from the full-blown migraine. As she was rubbing my back, my dad called and told us there was a large wildfire  but didn’t know how close.

We went outside and saw a huge smoke plume behind out house. This fire would once again change my health.  We immediately grabbed hoses and watered the land.  My lungs are still paying the price.

I even put on my Vogmask (medical mask with a filter) the moment we saw the smoke.

My lungs are super sensitive and I know that I’m the “human fire detector,” but because we had had so many fires already and the fact that my migraine was a 20 out of 10 on the pain chart and me throwing up, my body was distracted. Then about a week later, the Detwiler Fire broke out and that 2,500 acre fire quickly turned into 81,650 acres.  Our cars had soot, the smoke covered the Valley, I had no energy and was struggling to breathe. I started to just stay home all day with oxygen and some pain meds because my body was hurting. I thought maybe getting out of the smoke and going to visit my grandparents at the beach would help but my energy remained depleted but I was finally able to breathe. Jake and I stayed there for about four days until coming home. 

While Jake and I were with my grandparents and my sister, firefighters got the upper hand and were able to control the fire but it remained burning for several weeks with terrible air. My health changed for the worse and I felt similar to right before the time I suffered from steroid toxicity. I hope its just the bad weather and the awful air, but I am scared because this is how my “bad” normal was for many years.

Four weeks ago, my health concerns increased, I woke at 3 a.m. for the usual reasons,  I started to feel weird and when I went to sit down, I passed out. When I came to, my chest was hurting and I felt my heart racing. I was sweaty all over but luckily Jake actually woke up and ran to get my dad. He took vitals and it was normal. I decided that I wanted to sleep and I’d text Dr. Sine in the morning. Plus, I knew my Concurrent Care nurse was coming in the morning. Although it was quite scary, I also knew that it wasn’t an emergency.

The next day my nurse came and my oxygen levels were a little low, but not too low; however, my blood pressure was low for me and it continued to stay low. All week my energy was low and I remained at home for the most part.  Because I had all of these weird things happening to me, Dr. Sine ordered the usual tests – echo, EKG, blood work and a PFT.  I am grateful to have a Medi-Port and I can just access it in order to blood and anything else I need through IV and the best part is that I can have it accessed at home.

Two weeks ago, while I was having my IVIG infusion, I accidentally spilt water on my Medi-Port site and had to get up to have my nurse change the dressing.  I got up and was completely normal.  We walked to the kitchen until I was about to sit down. I got extremely dizzy and my heart felt as if I was having a bad case of tachycardia.  I felt like I was going to pass out and couldn’t catch my breath. I set my head on my nurses chest and that is all I remember. When I regained consciousness, I was completely confused and had no idea what happened.  After I woke up, I became extremely sweaty and nauseous. It wasn’t until the next morning thatI learned that I didn’t exactly pass out, but had a seizure. This was a complete shock to me because I used to have migraine type seizures called, confusional migraines, but I was able to remember everything and respond while having them.  I even had about a 30 second warning that I was about to “seize.”  This time, I had know post-ictal effects such as inability to walk, except I was unable to remember seizing.

My mom called both my Stanford ACHD doctor and UCLA neurologist and obtained appointments the next week. We obtained the tests ordered by Dr. Sine and went to Stanford to see my cardiologist on Thursday. While there, we also mentioned that we think I had another episode because when I woke up, I had seizure symptoms.   Due to uncertain results and multiple symptoms, we followed up with Stanford Pulmonology on Friday. Signs are possibly pointing to exacerbation of my pulmonary hypertension and the discussion of a catheterization was had.

On Sunday, we drove down to UCLA and on Monday we saw my neurologist. She basically said the same thing.  She was confident that it was not neurological because I would be having more neurologic symptoms and I would not have recovered so fast. In my heart, I’ve thought it was my PH because of how I’ve been feeling and my symptoms fit.

Today, We got word that I will receive a cardiac catheterization procedure on September 15th at Lucile Packard Children’s Hospital to identify any changes from my last one and to determine if any interventions are appropriate.

Historically, I often pick up health changes before medical tests show anything.  I am very sensitive to changes in my body that subtle changes aren’t so subtle to me.   Regardless, something is definitely wrong. I have been getting worse and worse each week. I can barely get out of bed or have enough energy to do anything else. It could be simply that the wildfires triggered something in my lungs, or worse.  However, I am more comfortable knowing I will be having the procedure which is the gold standard to determine real changes in my primary conditions.

We have to figure this out because I refuse to be a sick bride and my wedding is only two months away. I know that its the worse time to get sick again, but at least I have a little time before our wedding. I just want to get better. It’s crazy that my health declined so quickly after such a good run.

My biggest fear is that I will be a sick bride and I can’t bare it. I will continue to update you with news when I have it. Thank you for your love and support.

 

Not Just CHD

Last month, I had the opportunity to tell my story on the on local public radio (KVPR) of transitioning from pediatric to adult care. The story was picked up nationally on NPR – All Things Considered.   I want readers to understand that complex health is not about one disease, not just congenital heart disease (CHD). I have two other life-threatening diseases that are secondary to my heart condition. That means that I need more than a cardiologists specializing in Adult Congenital Heart Disease (ACHD).   For that, I go to four different hospitals; Stanford, Lucile Packard Children’s, Valley Children’s Hospital and UCLA Medical Center.

With only CHD, I wouldn’t need four hospitals. Science and technology are allowing us to live longer; however, we need more specialists that understand our underlying conditions. Often they are super-specialists – very rare and no single system has all of them.   As heart kids, we are used to going to appointments, for me it was every three months with an echo and EKG.  As a heart kid or a parent, you’re so worried about the present, not the future.  Once you realize there will be a future, a whole set of different issues arise.

I was born with pulmonary atresia, which affected my lungs, but my lungs did not become a disabling problem until my late teens.  Although, I saw a pulmonologist regularly just like the cardiologist. We only started thinking about what will happen and who would treat me as I transitioned when I started puberty. In 1993, I was the youngest to survive with my rare subtype of type of defects and the first stage of my surgeries.  I was 9 months old.  Today, they can do most surgeries within days of being born and in some cases they can do surgeries while still in utero, which shows how much science and technology has advanced. If science and technology has advanced so much, then is it so difficult congenital patients to transition?  The fact is we didn’t live and now we are.  The adult medical world is now faced with its first large bolus of us.

There are about 1.1 million pediatric congenital heart patients and 1.3 million adult CHD patients; many of whom have complex diseases and secondary illnesses. In the United States, there are about 2000 pediatric cardiologists and only about 200 boarded adult congenital Cardiologists.  This alone demonstrates a huge gap in care. As we get older, we are faced with the same issues as our peers – adult health issues: if we can or should get pregnant, secondary issues, high blood pressure, obesity, diabetes, general conditions of aging, etc.  I know that seeing a gynecologist was must, but my cardiologist was clueless on who are where to go. Most adult physicians have very little knowledge about childhood illnesses and vice versa. That is the problem and that is why at Stanford and UCLA I see both adult and pediatric physicians.

My transition remains difficult. When I was a teenager, I started to get sicker and we didn’t know why.  As a teenager, I fell into a deep depression that I call the realization depression that I think anyone who grew up with complex disease suffers. Mine really hit when I turned 15 and lasted for two years. Something clicked one day and made me realize that this is reality for me and I will never be healthy.  I never thought that I would be a sick adult. I realized my parents can’t fix this and there are responsibilities that my parents handled, that I will have for the rest of my life.

When I was diagnosed with CVID at 18, I was fed up with the pediatric world. CVID is usually diagnosed when you’re older and rarely when you’re still a child.  Because of that, it was considered an adult illness. The same goes for my lung disease. Yes, I always had problems with my lungs, but when they found out that I had secondary pulmonary hypertension and COPD because of my physiology, they sent me to an adult pulmonologist. This makes coordinating care with all of my physicians – both pediatric and adult and across multiple hospitals near impossible.  It also did not work.

In the fall of 2013, my health seriously deteriorated resulting in multiple ICU admissions less than a month apart and doctors “hot-potatoing me.” It became critical on January 1, 2014, when I was admitted to the PICU at Valley Children’s Hospital, stabilized and then transferred by plane to Stanford. These admissions were after 2-3 admission at UCLA for pulmonary issues – I couldn’t breath.  At Valley Children’s they determined my pulmonary pressures exceeded my systemic pressure and I was suffering from steroid toxicity.  At Stanford my condition continued to deteriorate and I was being evaluated for an emergent heart-lung transplant.

The entire time I was sick at UCLA, my pulmonologist did not contact my adult congenital cardiologist and I was seen by an adult cardiologist. The communication should have been non stop between my adult congenital cardiologist and my pulmonologist.  There was a real disconnect between the coordination of my pediatric care and now with adult care.  As written above, it almost killed me.

That hospital stay in 2014, I almost didn’t walk away alive. I was in the CVICU at Stanford for a month and saw doctor after doctor and even at another major teaching hospital, they could not figure out or agree on how to treat my diseases. I still have yet to receive a diagnosis for the 3rd lung condition I have.  I am also having trouble coordinating with GI and Immunology for my CVID and chronic C.diff.

Even with my dad being a both a health care attorney / and a senior executive at a children’s healthcare system, a brother-in-law who is a pediatric cardiac intensivist, I still have trouble coordinating my care and timely receiving treatment.

Today, more than ever, we need system for adults with chronic diseases of pediatric origin.  We will not survive if we can’t find medical care that has knowledge of our primary conditions.

On July 16, 2017 I will be exactly 16-years since my 4th open heart surgery. Surprisingly, my heart has been the most stable disease.  Thank you Drs. Prochazka, Rompf and Hanley.

 

Compassionate Care Conference 2017

I know my posts have been sparse on my blog for things in my life other than lessons directed at my health or health events.  This update is not directly related to my health, but about an exciting event to me.  This blog I want to explain the reason I started this blog.

I started this blog because I have a story different from most of my age peers. I never understood why I felt the need to cover up exactly how I feel or that my stories that might be too much for others to handle. I always felt that I needed to prove something to everyone else that I was “normal.” It wasn’t until the prednisone overdose that I became comfortable to lay it all out there.  During my journey, I have met many others like me.  Like them, I never in my life thought I’d be a sick adult. The biggest thing I thought was that I would only have congenital heart defect/disease. If I only had CHD, I would be ruling the world. Yes, I have a very serious heart defect and will always have issues due to it, but the combination makes CHD look like cake.

I have met hundreds with CHD, but only a handful with serious secondary diseases like mine and I finally have a place to go to be with others like me. We don’t just have one life-threatening defect or disease, but many. I can not tell you how much I used to spend crying and worrying about my CHD.  I wish I could go back to just CHD. Last year when I started to tell my story, Dr. Sine asked me if I would be interested in doing a panel at the 8th annual Palliative Care Summit, and the Coalition for Compassionate Care of California; AKA CCCC. I thought, why not? The conference and the people I met there once again changed my life. I finally belonged somewhere. Those who were at the Summit understood me and I met the sweetest little girl there that has multiple life threatening diseases. There I was given my voice back, the one I lost many years ago.

In March, the 9th Annual Summit was held in Sacramento.  I got to see my long lost twin whom I met many months ago.  He lives in Philadelphia, is a young adult, and is a survivor of brain cancer. Charlie doesn’t have multiple illnesses like Marla Jan and I, but we got to do a question and answer panel and we made a killer team.

Charlie and I have very different diseases and are at different stages of life, but we had three things in common. 1) We both attend Arizona State University; 2) We have a special power; and 3) We are young adults getting our life back.  I know I will get sick again, and possibly get a new diagnosis, and I will have to rebuild my new normal once again.  But while on the dais, telling our stories and sharing views on doctors, nurses, social workers, and parents, I was Rachael again.  I survived to tell my story, I felt like there is nothing in this world that could stop me.

Marla Jan is a beautiful vivacious women and my hero – she is the ultimate fighter. She has my heart disease, and multiple auto-immune disease.  She has also survived breast and is now doing Chemo to treat her lupus. When we met, I can’t tell you how much I felt as if I was looking in the mirror. Her strength is unparalleled and her courage would challenge Navy Seals. She gave me different perspective.  Despite all her challenges, she is also a pediatric critical care nurse and  a full-time patient like me. I talked to her about the pediatric nurses and doctors fighting us when we go inpatient because they are terrified to treat young adults with pediatric diseases.

Marla Jan, Charlie and me have gone or are going through the transition from pediatrics to the adult world all agreed that there is no way to do it without serious consequences, in each of our cases, almost fatal ones.  When first entering adult medicine, we each got lost and have had to find our way back to hybrid care. None of us think it is sustainable.

What I took away from this conference is that we do have a community for us, its small, but we have a voice from very caring people. After the panel, we sat around with a team involved with the CCCC and we talked about the right and wrong way of dealing with our issues. The truth is that we are still learning how to handle everything that comes with our diseases.

The professionals were very intrigued with my dad’s view on it as he is a senior executive at a children’s health system.  He discussed the challenges and the unfortunate economic realities of young adult and transitional care.  Now that we have this CCCC community, its easier to put our heads together and work towards solutions.  We all knowthere is no easy way to transition and that we cannot afford medical mistakes because of fragile health.

I am so blessed to have had this opportunity and so excited to continue to speak for us -1%er’s.  I wanted the audience understand that we each unique; you will never see two cases the same.  Do not treat us like just like another heart, transplant, cancer, or immune patient.. Don’t tell me you had a case like me or Marla Jan or Charlie because you haven’t.  Our surgeries, complications, medications, scar tissue and damage has made us unique. Treat us like you have never saw this disease in your life – if you listen to our stories first, you will.

My Normal; it’s Not Your Normal

What is your definition of normal? Would you like mine? Well, mine is not going directly to college and getting a BS, BA, MS, MA, MS, PH.D, or any other degree. What about working a full time job? Well, that used to be my new normal, In fact its still my sisters normal, but for me, it’s far from my normal.

Don’t get me wrong, I grew up in a household with two college graduates, One went to Law school. I was taught as far as I remember that you needed to get a College education to make it in this world. This was constantly reinforced through high school. Both my older sisters graduated. One obtained her teaching credential after high school.  The other received her BS in Business at UCI. My path was different.  I was accepted to Fresno State University following graduation from high school.  I accepted; but my normal had other plans.

I know I’ve written this before, but I never thought I’d be a sicker adult than I was a child.  I was dead wrong. I was only able to be on campus at Fresno State one semester before I was forced to resign. I than applied and successfully was enrolled with Arizona State University online. Unfortunately, both times, my normal forced different plans.  My normal was a series of stacked medical issues, hospitalizations, medicines and all the related issues.  It has now been five years and I only have 3 semesters.  At this rate, my normal will be graduation in 2100 or so.

ASU was amazing, extremely helpful and eager to help me to be successful. Unfortunately, my health decided to once again interfere with the normal life. When I became sick in January 2013, I had no idea that it would literally turn my life upside down. Yes, I was overdosed with steroids, suffered steroid toxicity and severe Cushings syndrone.  I also developed secondary diseases, some temporary and some permanent damage from that, but I never thought it would change my path forever.

It made me realize that the cookie cutter path that many “healthy” people travel  will never be the path that I would follow.  I call it my “NEW NORMAl.” Trust me, I would love to work full time and provide for my future family.  Physically, I can’t.  I was raised to be self sufficient and contribute to society.  I will always try to do so – if my new normal lets me. I grew up being taught that you need to contribute to society; work, volunteer, be a good person . . .

This morning I woke up at 3 a.m. while receiving my every 3-week IVIG infusion.  C.Diff. was back, severe nausea and the other nastier side effect.  Once again, my new normal struck.  This week began incredible, I started to see a personal trainer and had my 3rd appointment with him.  Things were looking up, I felt stronger, encouraged and maybe was doing something regular normal.  But my new normal had other plans. The truth is that being a chronically sick adult is hard; it upsets the best laid plans over and over and over again.

Sick adults are treated very different than sick children; many think we are a burden on society.  We didn’t ask for it,  we didn’t cause it, it just is. I wish I was like my sisters and other family members. Sometimes I feel like they got the great end of the stick – the one with the candy on it.  I got the other end, just a stick and it kept hitting me over and over and over again. I wish that my health wouldn’t be this unpredictable.  Its hard to explain to someone who doesn’t know what its like, but thankfully my parents get it.

When I was 18, I was super sick. I would sleep 20 hours or more a day was even too weak to walk to the bathroom. We went from doctor to doctor, but no one had any answers to why I became so much sicker. This also took a toll on my family. I know everyone was on edge and about to snap and snapped. It even got to the point that my sisters were convinced after seeing the stress this was doing to everyone, that I was faking it. That went on for about a month, I was angry, sad and most of all – hurt. No one knows how much it actually sucks unless you walk a second in my shoes, my normal, not theirs.   This search led to great news, not, a new diagnosis of a rare immunodeficiency disease and in fact I was about a month away from death if I wasn’t treated.

This is the thing, no one can truly believe that someone can be this sick, this look and over and over again that we must be faking it.  I’m sick of the dirty looks and people yelling at me when I use my handicap plaque because I don’t fit the mold of what they think a disabled person should look like. I am sick of getting asked, “so when will you finish school or get a job.” My answer is simple, when I am able too.  I wake up fighting for my life everyday and if if the most I did today was go to the pharmacy, pick up my meds, and breathe – I would consider that a win. Some weeks like this, I did 2 personal training sessions and thought, wow, I’m normal, and not my normal.  My new normal reminded me last night that I’m not.

This is my life. I got dealt a crappy hand and I am doing my best to live in a world not made for us. I am blessed to work with a nonprofit that helps medically fragile children and their families with various type of financial assistance.  I speak on compassionate and palliative care.  I blog, sometimes, with the hope that my experiences help others.  I might not be back in school yet, but I will. My goal is to continue my education one day when my health will allow me too.   In the meantime I will continue to be an advocate for those whose normal is not your normal.

 

New or Next Chapter

A New Chapter – Tillium Kids, Inc. (www.trilliumkids.org)

Hello friends, I am very excited to share that I was asked to be on the board of Trillium Kids, Inc., a non-profit that provides support for families with medically fragile children. My official title is Creative Marketing Designer. I will be responsible for marketing on social media and fundraising. I would like to thank my Concurrent Care physician, Dr. David Sine, M.D., for this opportunity.

This is what I think is my purpose, I am supposed to help raise awareness for us “-1%ers.” I say us “-1%ers” because we are the rarest of rare patients, we aren’t just affected by 1 severe or rare disease, we are affect by multiple. I have been fighting rare diseases since birth and the fight continues today. I am extremely lucky to have my parents who were always by my side while sick. I was never left alone. I remember waking up after my 4th open-heart surgery and although my parents weren’t there, my grandparents were right next to me looking over me.

I grew up in a household with both parents working. When I got sick, I realized that I might not be able to work because my health makes me inconsistently available. Most children and teenagers don’t think much about the future. Due to my diseases, it was always first and foremost; it required me to grow up in many ways faster. Each decision required thoughts on medications, nearest medical care, elevation, risk factors and timing.

I knew wanted to work as it gives one purposes; the frustrating part was my inability to do so on a regular basis. Also, having rare diseases is expensive. I knew I couldn’t be permanently dependent on my parents. However, after getting sick 3 years ago, It became clear that I will not have a job with normal hours. I decided to stop school (well my health forced me too) but I was incredibly disappointed. Another dream lost.

After Jake and I met, I freaked out because I wanted to marry him but I knew I wanted to give something to the relationship. I needed to feel like I was doing something for myself. I started to blog and speech to tell my story. Now I have expanded this to Trillium Kids, Inc. and the Compassionate Care Coalition. I will soon begin lecturing medical professionals on the proper behavioral management of the chronically ill. I think I have found my purpose. Thank you Dr. Sine, Jake and my family for believing in me.

Please following on Facebook (Trillium Kids, Inc.) and Instagram (Trillium Kids)

tilliumkids.org – Donate Today

Everything REALLY Does Happen For A Reason!

Everything Really Does Happen for a Reason.

I am sorry I have not done a blog post in months; things have been a little crazy, but extremely exciting.

I’m so happy to announce that Jake asked me to be his wife.  It was a complete and utter surprise that I would ever be asked. Most don’t know the story of how Jake and I met, so I am now going to tell all!

Three years ago I got sicker than ever before when they used heavy and multiple steroids to “help my lungs.” That decision was never mine and I will be carrying around that mistake the rest of my life. I was fighting for my life and just got out of a very toxic relationship that was 3-years on and off before I was admitted to the ICU at Valley Childrens. There I was very drugged up and barely remember anything except for looking over to the couch from my hospital bed and always seeing my dad. My dad spent every single night with me and that’s when I realized that I am okay to be single because no one can measure up to my dad. I also couldn’t imagine finding anyone willing to chose this life.

Being told constantly by the person I was in the toxic relationship with, that I wouldn’t find anyone who would be willing to be with someone as sick as me, I started to believe that he was right. It wasn’t until I became so sick and had to think about the possibility that, yes, I might not make it out of the hospital, that I had to create a new outlook on my life and my own worth.

I vowed that I would no longer settle, I couldn’t. I realized that I had to change my mind set from: “I couldn’t live without him” to, “I need to live without him to save myself.”  It also helped that my dad is the strongest most loving man I have ever known in my entire life. He has been by my side since day one and never cared that he might lose me, he loves unconditionally and has never left when things get hard. For the next 3 years, I kept to myself and fighting for my life every single day.  I was content with the realization that I might be single forever and as much as I wanted to find love.  I was not willing to sacrifice or settle for anything less, so thats when I realized, I will be okay being by myself.

It was last year in December when my friend told me to go on Tinder. . .   If you know my sisters who are extremely overbearing, it was a short matter of time till they told my parents. Now many people hear this and think. . .  YOU ARE 23 and can do whatever you want.  However, my family has been the only consistent thing in my life and I know I wouldn’t be alive today without them so their opinion is very important to me.

I came home one night and my dad and I were in the lounge talking and he looked at me and said, “Rachael get off tinder.” I was shocked and I told him that meeting people when your sick and you rarely go out is hard and I would just like to meet people and just date. Get back out there is what I meant.  I was completely shocked when he suggested I try match.com. I told him that I doubt I will ever find the one, but getting out is what I need. That night, that was it, and I was on Match. Being me, I put on my profile stuff about my health because its not fair to hold back the most important aspect of your life and I am proud of what I overcame.   I got some hits, but I kept seeing a profile ad I couldn’t take my eyes off, so I messaged him. My first Match date was set 3 days later and I was off Match thereafter.

December 12, 2015, was Jacob and my first date. He is from Tulare, which is about an hour away from Fresno. The night of our first date I got in a huge argument with my mom because I wasn’t dressed enough for the restaurant he made reservations.  I didn’t know how nice it was.  I wanted to be comfortable and I was so nervous  after my mom’s comment.  I was abut to cancel until my mom reminded me that he drove all this way to meet me and take me to one of the nicest restaurants in Fresno. I am always a little late and that night was no exception.

I drove to the restaurant with puffy eyes from crying and when I walked up to him everything negative I was feeling about myself disappeared. We ended up talking and barely eating because we couldn’t stop talking and getting to know each other and I didn’t want the date to end and I knew he loved Christmas lights as much I do.  So I suggested to him lets go get hot cocoa and go look at lights. It turns out that we think much alike. He was already planning on that exact thing after dinner.

I think that Jake and I realized how similar we both were and when I went to hold his hand it was so sweaty that I pulled away but the best part was that I found that I fell hard for him.

After he kissed me three times and that was it! I knew that I would be seeing a lot more of him. I came home to my dad and talked to him as if he was a girlfriend and told him all about the date. It felt different and then I got a text from Jake saying thank you and if I would like to go with him to a house in Tulare that decorated their house Grinch themed. I ended up not feeling well the next night but he could care less and we rescheduled for the next night. I was never expecting to meet his family so soon because I made a promise to myself that I would not introduce him to mine until we got to know each other.  Thus, it was huge that he wanted his parents to meet me that night. Unfortunately his dad, Ronald, had the flu and already Jake already aware that I couldn’t be around anyone sick, made other plans.  I got to meet his mom, Kathy.  That night we watched Investigate Discovery, I knew that this family was perfect. I realized that I found the one. I will actually be loved for the first time and I didn’t have to be alone to be happy.

It was very hard to let Jake in my “medical world.”   This was not because I doubted him, but  I didn’t want him to have to deal with it. We ended up celebrating our first month together in the ICU because I got sick with a central line infection and emergent surgery. He was by my side every second even though he had work full time and school; also, he would have to drive an 1hr and 30mins each way.  I was always told that I would probably not be able to fight off a central line infection if I got one because of my immune system.

Jake comforted me and he began to knock my walls down. I didn’t understand why he would want this in his life. He stayed, he learned and even when I stayed at his house once over night due to the fog, I forgot to do my infusion to help fight my CLABSI and he woke me up quietly and did my infusion for me. I was already deep in love with this man, but everyday my love for him grew stronger and stronger.

Jake supports me through everything and doesn’t care that I am this sick except for how it makes me feel and also that I am not able to have biological kids. He has become my rock and my dad has never trusted anyone to take care of me the way my dad does with Jake.  That is why when Jake asked my dad for my hand in marriage my dad shed a few tears and said yes, even though we had only been dating for 9 1/2 months. I trust my dad and respect him more than anyone and he will randomly look at me and say how much he loves Jake and how comfortable he is that I will be with him forever.

We have a love I never thought I deserved because of my health. Jacob has made me love myself and love my journey. He has completely changed my entire outlook on life and my opinion on being alone. He has made me stronger and happier than I ever had been and that has made my family and I closer and have a much better relationship. He told me that he would never stay at our house or work at the hospital and less than 6 months later he is working at the hospital and was welcomed in. He found his calling and absolutely loves being in the medical world. They say that you will marry someone like your dad and I know that I am. He has the biggest heart and wants to help and understand my health.

I never thought I would meet anyone, nor picture a “dream” wedding like most do.  I was lost, My sister got married a week before I got engaged so my mom is helping me. When I tell you that I literally never imagined anyone who would love me despite my health, I mean it! I am so lucky to have found my best friend. I realized that I am not less worthy because I have diseases that I never could escape from.

Our relationship has given me a new prospective on chronically sick patients. We have had to fight to survive and know that not everyone is as lucky as us. My life is  more than my disease and just because my health is unpredictable doesn’t mean I cant have love. I think and now know, it will just take a strong person to come in my life and stay. I needed someone who never gives up.  I am so happy and look forward to what our future holds.  Finally, I couldn’t imagine settling for someone who gives up when life gets hard and I know Jake won’t.

The Ugly Side of Living with Severe Medical Issues – A Good Day At the End

img_9364These last few weeks have made me realize the ugly side of severe illnesses. Generally, it is extremely hard to keep up with people who are “healthy.” Early this month, I had a full blown migraine that came out of nowhere. I usually have some sign that a migraine is coming on, but this time nothing.  I ended up having to leave my psychologists office early and as soon as I got to my car – it hit with a vengeance.  Luckily, I went shopping earlier and had a plastic bag or I would of had to dispose of the car.  You can imagine why.

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Jocelyn and Alexis

These health episodes love to act up when I am finally living and doing normal things. It always seems like it punishes me when I attempt to have a “normal life”.   My sister, Jocelyn, had her bachelorette weekend in Southern California; I was so frustrated because I wear out so much faster than everyone else. My sisters have had to sacrifice a lot growing up.  So, when I get sick when it’s supposed to be about them, I get so mad. I know its outside my control but it is also so frustrating.

This blog is about my life journey and how to deal with being a young adult with complex diseases. It is truly an unpaved road and we haven’t been able to figure out how to make it easier for anyone involved – so I am going to be honest. My sisters and I have had a rocky relationship for a long time and although we know that this is something none of us could control or desire, my health is the big reason why we don’t have the relationship we wish we could. When my health flares up, it stops not just my life but my sisters, my parents and now Jake. It has been hard for them to understand my relationship with my parents because its completely different from theirs.

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Unswollen / New Med Swollen

There is no surprise that the last three to five years hasn’t been normal or easy for us. My health has taken many turns and with that comes a lot of pain and changes. I recently asked my middle sister, Alexis, who is only 13 months apart why she gets frustrated at me so easily. Her response was that it wasn’t me, it was the situation and how I act when my parents are around. She isn’t wrong about how my relationships with my parents.  But, she also doesn’t understand that going through this everyday for years with my parents changed our relationship.

My parents weren’t just my parents, they have become my caregivers, my psychologists, my best friends, my links to life! It had to be this way in order to have survived.  This  because what people forget is the mental torture of being so close to death over and over again. Alexis understood because I explained to her that it was the same for my mom and dad. We are not a perfect family; however, if you know us, we are a very strong and loving one

The statistics state that most parents who have a “sick child” are very unlikely to img_9435stay together because of the constant stress and continual challenges. I am very proud to say that my parents just celebrated their 34th wedding anniversary. It also marked another milestone:  they were able to go on vacation for a week out of the country. Since my health started going downhill, they have not been able to do normal “married” things for a long time – like vacations. They had to put them aside in order to take care of me. I have big guilt that my health affects the people I love the most. I would do anything to not let it affect them, but we are a family.

Me and Kathleen

It was very overwhelming when they went to Cancun and left Jacob and I to watch over the house and animals while I was fighting C diff for the 11th time. Our dog Bella has massive anxiety and isn’t allowed to be with other animals because she behaves poorly and we have to separate them.

Regardless, I am happy to announce that they were finally able to focus on their marriage and still have the strongest relationship of any couple I know. Their love is unbreakable and shows us how families are supposed to be.

img_9329P.S. My sister, Jocelyn, was married last Saturday evening. Although, it was at a high elevation for me and a nonstop weekend, I was still “healthy” enough to attend and witness her marry her best friend. It  took every ounce of me not to drop on her wedding day. Yes, this is our reality, and of course I am frustrated when my health doesn’t corporate.  In order to survive, I have to constantly alter my life.

I missed the cutting of the cake and most of the reception because I needed to get an oxygen break or I wouldn’t of lasted. Many frustrations come to my mind with my health during her day, including medicine that made my weight blow up. I know is superficial and I am grateful I was even there, but for once I just wanted that day to be perfect for her, so I pasted a smile on my face.  Instead of focusing on what my body was doing without being able to change it, I focused on her.

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Princess Bride

That day was the day she dreamt of since she was five and she looked every bit the princess.  Yes, I was swollen and it was harder to breathe and I had no energy; however, she had a perfect day.  Our lives are relentlessly difficult because of my health, but we are a family and we will not let my health tear us apart.

 

The Human Fire Detector!

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Air Crane

First and foremost, I am sorry for not doing a blog post for a while. I’ve not felt well; I found out that I have C.diff for the 10th time in 23 months. C.diff is actually more reliable in my than my own flippin periods. My Compassionate Care / primary physician – David Sine, has identified several possible options, since the traditional cures have not stopped the recurrent infections.IMG_9642

I live in the Central California, in the San Joaquin Valley and in the hills outside Clovis. It shouldn’t be any new news that California has had some devastating wildfires due to the extreme drought.  In 2010, my parents moved to a house on the first foothill above Fresno and now wildfires pop up all around us during the fire season. Although it is paradise where we live, it’s not always easy when you have “one” functioning lung.

I never had any special talents except for being funny in crappy situations until a couple years ago when I finally found my very own weird talent.  I can detect a wildfire from miles away before anyone knows it. I know most won’t believe me, it’s still hard to believe it myself, but I have proved that I can in fact detect wildfires. The very first wildfire I ever detected was right after my middle sister, Alexis, graduated from UCI in Irvine, Ca. We were on the “beautiful” drive back to Fresno and while we were driving on the Grapevine, my lung(s) felt a little tighter and just overall weird. I can’t remember how long it was until we saw a huge smoke cloud on the horizon.  I was weirded out and kinda impressed with myself, but I wasn’t sure if it was just a coincidence. We continued on the scenic route to  Fresno and about 15 minutes outside Fresno and even further from our house, I had the same feeling I had on the Grapevine and this time I spoke up and BAM, I was right the second time that day!

Since that day, I have detected multiple fires before anyone else.  With only “one” lung, the wildfire season is extra stressful, not just for me but for the people around me. Recently my boyfriend, Jake, learned just how sensitive I am to any fire. It was only about three-weeks ago we were on our way down to San Clemente, Ca for Alexis’ boyfriends’ twins’ wedding.  While on the Grapevine, again, except the other way, and after passing the only wildfire I knew about, my lung started to burn again. I knew another fire had begun.  Everybody doubted because they couldn’t yet see or smell it.  I kept saying that there is another fire, and less than 10 mins later we rolled up on the first Santa Clarita fire of the season.  It would end up closing part of the I-5 later that day.

IMG_9611Its strange that my weird talent matches a fun fact about me; I am both fascinated and terrified with wildfires. I have always had a big fear of any fires in general but  I overcome my fear is by learning everything I can about them. Now, I jump in the car and go up on the top of our mountain just to see a wildfire; almost three times per week since the beginning of June. I think it all started last year when we had our own wildfire on our property.  This wildfire burned as close as 3 feet from my bedroom window.

That was a crazy night. It all started when my mom yelled to my dad and me that we had a fire on our property. At first, we didn’t really believe her because like me, she’s a little dramatic.  We immediately realized how serious she was. Our water pump to our pressure tank sparked the fire and shorted out.  That means a fire and no water pressure.  And that was what happened.  Fortunately, we built a pool on the property.  Valerie, our former great neighbor, sped to our property and my mom, dad, Valerie and me sprung into action. Now,  imagine four adults with only 5 gallon buckets and a pool.  We (me for a very short while due to the smoke) IMG_9630successfully halted the fire from the propane tank, the house and the roof.  We continued to fight until Cal Fire arrived. Due to my backup oxygen tanks in the garage, I was really concerned about a house fire and potential explosion.  My parents sent me inside, the fire department made me wear a mask and I wasn’t allowed outside to help at all until the house was no longer threatened the house.

CalFire was incredible.  Within no time at all, a spotter plane was circling, two air tankers were dumping retardant, a Cal Fire helicopter landed on our lower property and retrieved water from the Friant-Kern Canal and 15-20 engines and fire crews were deployed.  All in all, only 9 acres were burned and no structures were destroyed.  A watch crew was on the property all night and we had a great result.  It really showed us how much fire preparation is necessary and helpful.  If we had not built and maintained a defensive zone, I am certain the house would have been severely damaged or destroyed.

Now, I think, you will understand how my weird talent combined with my health and real world events, causes me to obsess about wild fires.  I guess being a human fire detector is not worst weird talent one can have; especially when you live in a fire prone area in the middle of a drought. I want to just thank CalFire and our fire departments for their hard work, not just at my house but all year around!

 

 

 

 

Medications: Mixed Blessing for the Medically Complex

CCFB1D80-911B-46E9-8534-25D0EBCA64D3For 23 years (my whole life), I have never been completely off any prescription medications.  There is huge debate surrounding medicine and the pros and cons of medicine therapy; but for someone who never was able to make that decision, the fallout is hard. I have been reading side effect labels since I can remember for physical effects, but now the psychological effects. I have said this and will continue to say it, I feel as if I am waking up from a coma after 3-years of massive amounts of medications to treat my diseases, reversing side effects from other medications, and countering the ill effects of other drugs.

During the most recent period, I felt like I was either IMG_4074crazy, dazed, slow, confused, amnesiac-like, irrational or a combination of all of the above.  I would think I am acting completely crazy in my rational mind and still have no ability to control my emotions.  The smallest thing would set me off and it didn’t matter who it was.  Jake and I weren’t even together for a month when they put me on Vancomycin again. It wasn’t fair to him but we talked and talked about it and he just said I feel like I bug you more now. It’s hard for any relationship, especially a new one, to work, but add medications that absolutely change your whole personality makes it a hundred times more difficult.  He gets it, but its not fair to anyone. Luckily, I have tools that help me think sane even its only momentary. That is my mind and gut telling me that this isn’t me and that I need to keep fighting. It was hard though, my medicine induced depression and anxiety, it made me contemplate death everyday and sometimes all day long, but add PTSD on that and it is almost unbearable.

IMG_7584My dad is my security blanket and I know that sounds weird but he and I just get it.  I never have to say a word and he just knows I’m fighting inside and I am about to break. He and I fight, but my rational side has never been mad at him. He knows that he is the only person that can calm me down and really feel how I feel. He always tells me how proud he is of me or that I’m his hero, but in reality, he is my hero. I can’t tell you how many times I’ve had a complete break down and extreme anxiety and I see him and I am okay. Both my parents are my advocates, but my mom has a different outlook and way of dealing with my health.  She is a sibling of two CHD warriors, my Uncle Colby and Uncle Randy and relates with my sisters. But my dad and I are going through this together for the first time. He has the most compassion of anyone I have ever met. He tells me all the time how unfair it is and that he doesn’t know how I can still be funny because this sucks! but I realize that when I’m making humor about my situation that is the real me shining though. The mental torture is the worst.  Its scary thinking and feeling like you will never be yourself again and its a dark place to be in.  I get embarrassed because I don’t remember many things that have happened and my only response is, “was I on drugs?” The answer is yes, followed by a sad face.  I think in some respect I’m lucky I don’t remember everything because it would be too painful to contemplate.

No matter what medicine you take, you will be impacted.  Just because it doesn’t have a certain warning, doesn’t mean it won’t have effects other than its curative purpose. When you mix 9 plus medications, drug interactions are unavoidable. I’ve had my entire personality turn into someone I didn’t recognize and that wasn’t just for a couple weeks or months, its been for several years now. I can now say I am feeling and acting like the real me for the very first time in years. Getting off medications is hard and that is because my body has been on them for years.  Although I do not have a drug addiction and I want more than anything to get off many, I do not have a choice. The world doesn’t have a magic pill for me or any patient with IMG_4072serious congenital anomalies.  All our surgeries and all our medications are considered palliative, not curative.  (i.e. to improve quality of life, reduce negative impacts, and maximize physiologic function with what we have).  Most people think because I’m on an X amounts of medication that I’m okay and will be okay and that’s just not the reality. Most days, I can barely brush my teeth when I wake up because I feel like I got hit by a truck or have a migraine, or some exotic combo.

If you knew me 5-6 years ago, you know I was a happy IMG_2187optimistic person who was able to at least try to keep up with others.  Now fast-forward to today and you haven’t seen that person, I haven’t recognized me in years and years. I didn’t even realize that I had a complete personality change until recently. I still have low energy and my baseline isn’t even half of what it used to be, but my mood is restoring and it’s a very sad reality.  I call it medication-induced depression and it’s a real thing. Some days I would just lay in bed hating my life, wanting it to end, I felt like life wasn’t worth it. I’ve asked myself. “Is it worth all this suffering to just survive?” Luckily, my thought processes at these times opposed by my “real” personality.  The real me would kind of shine through during these darkest times and I would know that if I just kept holding on and fighting to just survive, that it will be worth it.

Yes, this surviving and fighting thing was and is the
hardest thing I have ever done. It’s true, mind over matter, but how do you do that when its been 5 years of hell and your life is completely out of your control? Not having any control over your health is the worst feeling.  I didn’t even have the luxury to do the things over many years that damage your own body; I was born already damaged, the reality is that it sucks for everyone involved.  You, your family and your friends. This is truly a road that is unpaved. We need to recognize that the mental state has a direct relationship to what you’re going through and have gone through. How can anyone be okay after the hell that I was put through? The answer is  you have to continually change your reality, reinvent yourself and create new dreams and futures.  I’ve given up the Olympics, traveling to the moon and climbing Mount Everest.

My amazing adult congenital heart disease cardiologist saw me this week. I was happy and energetic about what’s been going on and what I feel and want and I was telling her that I’m looking into plastic surgery.  It is my hope to fix at least some of the terrible damage to my body caused by Cushing’s disease bannerfrom steroid toxicity.  She did not know me prior to that mistake, so she’s never seen my upbeat, happy, clear minded, optimistic…. personality and I know that she was probably thinking that this was an extreme emotion and wasn’t real. She shot meFullSizeRender-2 down and said that she wants to see me more medically stable and work towards my future before she will consider giving me the green light for any procedure. She also stated that I need to accept that this is my body and be happy because I am beautiful and not discount what I’ve gone through.

IMG_4071The funny thing is that I do feel beautiful and I haven’t discounted this whole ordeal, but quite the opposite, I have been working every single day to fight the emotional carnage from the last years. I have PTSD and I know the reality is that even if I get any plastic surgery, its not going to cure even a small part of the lasting side effects that I will carry around the rest of my life. I’m not naïve, I am terrified that in a second, my entire life will be turned upside down again because of my health. That isn’t me worrying too much or having an episode of anxiety, it’s my reality that medication has both saved me and failed me in spectacular ways.  My physician’s often admit they are not certain what negative impacts some medications will have but I need the beneficial effects or else.   Else is not a good choice.  Unfortunately, this is my everyday life.  A recent text reads like this “. . . with the combo of all the effects, we are in uncharted territory . . .   I guess that sounds like the physician version of a road unpaved.

I don’t get to choose the times that are convenient forFullSizeRender me to deal with all of this, it is a never ending battle for me and my family.  I know that with every crisis, new complications will arise and I will have to work non-stop fighting them and hoping the cure is less onerous the disease.