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.

A Little Advice for the Medical Professionals

IMG_2147On May 13, 2016, I was asked to do a question and answer session at 2016 Summit for “The Coalition for Compassionate Care of California.”  That time has come and past.  This is what happened.  When I woke up that Friday morning, I had absolutely no idea what to expect, except the fact that I was meeting with a representative at the crack of dawn!  For me, waking up…oh lets say…7:30am is like waking up in the middle of the night. I sleep a lot and have trouble falling asleep due to my meds and pain. But when that alarm went off, I knew that this was the start of something that I’ve only dreamt of.  BTW, they also thought it would be important to have my dad up there with me.IMG_4451

My dad, Devon, the representative from CCCC, and I were seated for breakfast and I was told that we would have two others joining the panel. I was a little apprehensive when I learned that the others were a complex patient who was only 11 years-old and her mother. As a 23 year-old with multiple complex diseases and experience, I didn’t think I would connect with my new best friend, Anna — the aforementioned 11 year-old.  I am here to tell you that she is absolutely the most inspiring medically complex patient I have ever met. I found someone, YES, someone a lot younger, with a deep understanding of the issues and frustrations facing patients with complex diseases.  But the point isn’t about age, it’s about our stories!  As to Devon, I love her and look forward to advocating with her, Anna and other like us in the near future.

IMG_2534Anna and I immediately bonded with our endless amounts of “hospital fails” and how we felt we both never really fit in with kids that have just one of our diseases.  I went to a camp with children with mainly CHD, and she likewise, went to a camp for one of her diseases (Muscular Dystrophy).  We shared that we never really felt as if we were understood because we are not just one disease and even our diseases are NOT textbook diseases!  Needless to say, but I will, we also bonded talking about what we like and don’t like from our health care providers.  Number one complaint – they do not listen to us.  Number one compliment, the much better care we get when they listen to us.  Thank you Drs. Sine and Ishiyama.

Our voices need to be heard! That is why Anna and I fight everyday.  Yes, I know IMG_1986we are complex and take up a lot of their time, often underpaid or not paid, in trying to solve our medical puzzles.  You see, Anna and I have had most of the same experiences, the difference is that I am a lot older than her and my voice still isn’t being heard. How do you expect us to trust you when we have heard it all before?  Mostly, we have been disappointed, and thus its really difficult to trust medical personnel. We both have suffered many medical mistakes because OUR VOICES WEREN’T HEARD.  We both carry the scars and impact of these mistakes and have suffered many long weeks over a number of years in various ER’s, acute care floors and PICU’s due to them.

Medical people, we know our bodies and when something isn’t right.   Anna ended up getting a central line infection at camp because the camp doctor didn’t listen to her.  When she needed a dressing change, he disregarded her instructions and did it his way.  She kept telling him he was doing it wrong and telling him how to do it right but he wouldn’t listen! So who had to pay?  The next day ended up with a 106 temperature,  a central line infection and a fast track visit to her children’s hospital.

logo-new@2xWe have to live with our diseases and all that goes with them every day. Unfortunately, they don’t go away and we cannot take a break from them. This is our life and we might not have the advanced degrees YET, but we have something really important to add to the care team.  It our knowledge of our diseases, their impact on us and the whole range of weird symptoms and interactions that go with them.  Know that everything you say and do affects us physically and mentally. Complex patients like us are new, we are surviving and thriving in greater numbers.  If you want improve our quality of life which can range from dismal to FML, then Listen! My one piece of advice to anyone who works with life-threatening medically complex patients, is to visualize what we must have gone through medically, psychologically and socially to survive.  Give us the respect and dignity we deserve to have made it this far.  Know that we are terrified of more mistake, their impact on our lives and little control we have zero over our health and its future impact on us.

I just found out one of my best friends from camp with Pulmonary Hypertension IMG_2535and related complex conditions, like me, only has one more option and that is a heart and lung transplant.  Her name is Kathleen and we have gone through crazy times.  Kathleen is waiting for a hospital room to be transferred so she can start that long process.  She wrote on Facebook that she intends to climb mountains after the surgery and knowing her, I believe it.  The scariest realization for me is we are last two girls from our complex group who are really the -1% –  my name for us medically fragile complex types.  We grew up together and were there for each other when several of our friends passed!  This is of course, when one or the other of us wasn’t hospitalized.

I am proud to announce that I was asked to partner up with the CCCC to develop an engaged patient advisory group to reinvent the conversation around advanced care planning and participatory medicine where healthcare providers see patients as valued full partners in their care!  A pretty complex statement for LISTENING.

This is just the beginning for me and other -1 percenters like Kathleen and Anna!  These young ladies have made a huge impact in my life.  Kathleen is a rock and one of the toughest young adults I have ever met. Although Anna and I just met, she is incredible and I can’t tell her how much hope she has given me. She’s going though what I have and she is only 11! To tell you the truth, I don’t think I could have done what I am doing now at her age but we know that giving up isn’t an option. So we are now standing up and making medicine listen to us! We never asked for our diseases, but we can’t do anything except teach what we have learned throughout our lives so we can make a difference to the next patient and the next and future generations of patients.

IMG_2061I want to just give a HUGE shout out to my family, Aunt Carrie, who videotaped the whole thing, my boyfriend Jake who drove 10 hours that day, my cousin Maddie and her husband and my grandparents, who came to the conference to support us!  Alexis, my middle sister, was put on the spot and knocked it out of the park.  I know it was uncomfortable and scary but she had the whole room in tears.  Thank you all for coming. I am so happy you got to witness the moment I realized that I have a bigger purpose in my life than just going to school, getting my degrees, and working in my degree field. I have something better right now, it is sharing my experiences and this is my mission and the reason I am still here today!

Finally, Devon and CCCC, I am ready, bring it on, let’s advocate, change attitudes and make a difference.  Kathleen, go climb a rock.

But you don’t look sick . . .

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The world’s okayest sisters

The happiest place on earth is not as happy as happy as it used to be.  When I was little, Disneyland was very understanding of my needs.  So many people began abusing the policies that others began to react with people with disabilities with hostility. Disneyland changed their policy and now, not so happy or understanding.

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This is even more so when you have an invisible disability.  I can walk by myself for limited periods of time and I have no outward sign of disability except when my poparkingdisrt catheter shows or I happen to be using oxygen at the time or sporting my overdose steroid look.  Several times people insulted me and questioned me about why I was provided special services.  My older sister Jocelyn turned on them and in her sarcastic schoolteacher voice gave them a lecture about invisible disabilities.  Its too bad they weren’t the ones Alexis and my dad hit in IMG_0957the heels with my wheelchair as we ran through Disneyland.

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Jocelyn and Alexis

Despite Jocelyn’s intervention, this has happened to me and continues to happen to me at Disneyland, other events and even when using a disabled person’s parking placard.  It doesn’t help that when they finally see some outward sign of a disability with my oxygen backpack, the catheter or me struggling to get somewhere, they look guilty and walk away — the hurt and the pain has already been earned.  Sometimes I even pretend to have a limp or look like Frankenstein’s Igor so that people see an outward sign and I am not given that look.

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Who is the sick one here?

Now, instead of embarrassing myself, if they question me at all, I choose to educate them.  Usually, I begin with “when you are born with only a partial heart, one working lung, and immune issues, the parking passing pass helps.”  “But you don’t look sick . . ,” they say.   I respond (in my head), ” and you don’t look stupid,” as its the more popular response from those of us in the You don’t look sick club!

I now realize that instead of me being shamed about something I had no control over, I decided to spread the word and teach others about invisible diseases. IMG_3327 Surprisingly, the more open I am with others, the more people I realize have similar problems and feelings about their invisible diseases. I am beginning my lecture series on May 12 in Newport Beach to discuss issues dealing with complex diseases and the medical professions response to us.
Finally, just because we don’t look sick, it doesn’t mean we aren’t.  We would give anything to park in the farthest part of the parking lot and not live with the Russian roulette of our daily health problems.

 

 

Idiots on a Plane: or Big Diagnosis #2 – CVID (Common Variable Immune Deficiency)

Pre-CVID Diagnosis
Pre-CVID Diagnosis

How many people are forced to stay at home during winter/cold and flu season? Well, that’s my life since I was diagnosed with CVID (Common Variable Immunodeficiency) when I was 18 years.

At the time, I had no idea what that meant.  Looking back, I was happy to know that there was an explanation why I was repeatedly ill, well beyond anyone else I knew.  I was also relieved to know that it wasn’t all in my head.   At the time, I was just accepted into Fresno State and my beloved best friend, Oakley, a golden retriever was diagnosed with cancer. My life was full of changes both good and bad.

CVIDI didn’t have the best high school experience because I had a very different life than any of my classmates.  I spent the first two years fairly engaged, I even made the Freshmen and JV cheer squads.  My next two years; however, were mostly home hospital instruction.  All through high school I was dealing with what it means to live with my life-long disease, now diseases. My diseases are not curable, but treatable to an extent.  These treatments are palliative, meaning they are meant to improve quality of life and control pain.  Thus, from 15-18, and even today, I am still dealing with the consequences.

Post-CVID diagnosis with Alexis
Post-CVID diagnosis with Alexis

Early in high school, I went through “The depression.” The depression is what most people with serious chronic disease realize their disease is not going away and it will likely get worse.  That time happened just before I turned 16.  It was one of the worst periods I have ever had.  As I was getting help with my depression and dealing with that, I went to my gynecologist because I had a cyst that keep bursting.  At 17, she accidentally informed me that I would never be able to carry my own children. Although, I have heard before that this was a possibility, it was the first time it was definitively stated.  With this knowledge, I was so ready to start fresh and begin my adult life.

ASUThings didn’t last long for me  at Fresno State. I was able to get a single room dorm to try and stay as healthy as I could, but even I didn’t have a full understanding  what is really means to have CVID.  My doctor obviously did because he would not sign off on my health physical without requiring a single dormitory.   At the time, I feared that my college experience would yet be another high school experience and it scared me. I wanted to prove everyone wrong and that included me.  After just one semester at Fresno State, I had to leave.  This will be topic of another blog.  It has now been almost 5 years and I still haven’t graduated college. After Fresno State, I found myself getting sicker but not willing to give up on college so I applied and was accepted into Arizona State University. ASU provides extensive online courses and this seemed and seems like the ideal solution for higher education.

With my CVID, I get sick so easily.  I can actually tell you exactly when I will get sick.  One example was from the last trip I took.  My family and I went to South Carolina for Christmas and New Years. Of course, I often get sick on planes due to other.  But this time I felt good about the trip since I was on antibiotics for yet another recurrent C-DIFF.  I had a mask, wipes and antibacterial spray.  Things were looking pretty good.

On the plane
On the plane

That was until the leg from Charleston to Dallas.  I want to tell this story because its important!!!!!!! When you are sick and contagious, DO NOT GO OUT IN PUBLIC! Or, at least control it with a mask and basic hygiene.   There was a young child, about 8+ years of age, sitting next to his dad coughing and wiping his nose without covering his mouth or sanitizing his hands.  The cough sounded very croupy – now I know what it was – parainfluenza 3. Why hadn’t this child and his parents learned the importance of covering his mouth and not wiping boogers everywhere and more importantly why didn’t his father intervene?  I turned to my dad and said “mark my words, in 3-4 days and I will be sick!” and crap, I was all too right.

At the hospital with Jake
At the hospital with Jake

About 3 days after we returned, I became congested, starting desaturating, and my concurrent care nurse called my dad.  He listened to my one good lung and it wasn’t so good that day.  I was struggling even on home oxygen.  He told my dad, I needed to get to the hospital and get respiratory support.  I went to the ER, was admitted to the PICU and received high flow oxygen and supportive support.  My blood test indicated I had Parainfluenza 3, the virus that causes croup – just like that kid.  This time, I promptly recovered, except for the CLABSI that I picked up along the way, but as above, that is a story for another day.

Practical-popupThe point here, is that there are many ill people; many with compromised immune systems.  If you are ill or a family member is ill, recognize you have a duty and an obligation to contain the illness.  If it means masking up, not traveling or seeking treatment, do so.  For your selfish actions can place others at risk or serious illness or death.

In my case, they did – thus don’t be that selfish Idiot on a Plane.

 

Welcome to The Road Unpaved – About Me

IMG_5184Hello friends, this about my life journey . . . so far.

My name is Rachael Goldring and this isn’t your average health blog.  Twenty-three years ago I was born and I guess that’s when this story of mine began! Congenital heart disease runs in my family on my moms side.  In fact, I had an uncle who passed away in 1962 with my heart defect.  Five years went by and my Grandma had another baby that was born with a congenital heart defect.  He survived because his heart defect was less severe.  Also, technology had improved enough during that period to really give my uncle a boost.

With the next generation, came me.  I was born with a severe congenital heart defect called Pulmonary Atresia.  I was 9 months old before my surgeon performed the first stage correction of my disease.  He had to as I was getting worse. I was born with several variation of defects which resulted in only one functioning lung.  Now, this isn’t the end our family history. My uncle (see above) and my aunt had a baby who has the same defect as me but he thankfully has a less severe form and two functioning lungs.  He is quite the soccer player.

Okay, back to me.  Of course I was always the “sick” child growing up.  But I really thought that when I grew up I wouldn’t be sick. I know that sounds weird.  I didn’t realize that CHD is never cured but palliated.  No matter, although my CHD was bad, I’ve tried to make sure it didn’t dictate my life.  If I only had CHD, I would be able to live a relatively normal life. What dictates my life now are all the secondary diseases and complications I developed. You already know about my one lung, but I also have a disease called pulmonary hypertension, which results from continuous high blood pressure to the pulmonary vasculature.

When I was 18, I was sicker than I have
ever been. All of my tests were coming out normal which drove me crazy. I was told that it was all in my head and I was fine. We did test after test with many doctors.  Over time, I had the sense that no one believed me and I thought I would die. I slept 20 hours a day and could barely walk to my bathroom which was in my room. Finally, one of my dad’s best friends, who is an anesthesiologist Valley Childrens Hospital in Central California, took my dad aside and said we should test my immune system.

Weird, huh?  As a child, I was sick every six weeks with bronchitis. He then suggested I get my IGG levels checked and BAM – my numbers were extremely low. I remember the morning I went to see this new immunologist and he reviewed my medical history and took one look at me and said, “I am surprised you’ve survived this long!” Not something you want to ever hear from anyone let alone a doctor. He then went on to confirm I wasn’t crazy and had something else wrong with me….I have CVID! CVID is short for common variable immunodeficiency. I now get IVIG infusions for my CVID at least once or twice a month.

After my CVID diagnosis, I thought okay, I am finally in the clear and have my whole future ahead of me. I was accepted into Fresno State University and I even moved into the dorms.  My immunologist required that it be a single. I was okay right? I’m 18, a college student and I even rushed and accepted into the Delta Gamma sorority, I was on top of the world. Of course, my health had a different plan.

I have always had bad migraines, but in December of my Freshman year, I had a severe one that caused “stroke” like symptoms. I suffered severe aphasia, numbing, loss of hearing and fear. I ended up being diagnosed with confusional migraine which mimics stroke symptoms. This was only two weeks before finals and I could barely write the word “the.” I ended up taking a medical leave from Fresno State because I couldn’t get the disability assistance  I needed. Which is what started me thinking of others like me.

How many chronically sick people hear this, “but you look so healthy!” Just because you cannot see my disability, it doesn’t mean I am not.  That’s why its called an invisible disease. Following my first stroke like migraine, my symptoms grew worse and ended up being transferred to UCLA for a lung transplant evaluation. Thank goodness, I don’t need one right now. I transferred all of my care to UCLA, because they pioneered the first Adult Congenital heart disease program on the West Coast. Sadly, things didn’t end there.  I ended up having non-epileptic seizures with postictal symptoms. I ended up in the adult neurotrauma ICU where they had no clue what was going on….So once again I felt crazy. It turned out to be another manifestation of confusional migraine. With these migraines, you have a variety of symptom, such as; hearing noises and debilitating nerve pain. Once we got my migraines under control (Thanks to UCLA neurology), we started to focus on my recurrent lung infections.

Around this time, I applied to and was accepted into Arizona State University.  They allow hybrid programs that include both online and in class options.  I have completed three semester on and off due to illnesses and am currently on leave.  I hope to begin again Fall, 2016.  Back to my medical history.

My lung diseases include pulmonary hypertension and COPD (structural, not acquired and no, I’ve never smoked).  But in reality, no one truly knows exactly what my lung disease is as it crosses multiple diagnosis.  Since the doctors don’t have a clear diagnosis or a treatment plan, they decided that every time I get sick, I needed a burst of Prednisone, other steroids and antibiotics.  This is where my next leg of the journey started. I was in and out of UCLA due to my infections and it wasn’t getting better. On January 1, 2014, I went to Valley Childrens because I was having a very hard time breathing. I begged them not to give me any steroids because I had just finished a long course and I felt little relief, why would they magically work now? Well, after consulting with UCLA, they thought they knew my body better than me and this is when my life changed forever! I was rushed to the ICU after i had an echocardiogram and saw that my pressures which are normally 50-60% systemic to 90% plus of systemic. They pumped me hard with steroids and we thought it would be best to be transferred to Stanford because UCLA’s treatments failed.  We were hoping we’d get a different diagnosis and treatment plan…..I gained 60 pounds in 3 weeks. I am 5’1 and my weight ranged between 98-115lbs. my whole life. I had to hold my skin together so it would break and bleed. This was painful. I spent a month in the CVICU at Stanford and it took 8 months to taper completely off the steroids. I had severe Cushing’s Syndrome.

Its taken me 2 years to mostly shed my steroid weight . . . but it continues. I KNOW MY BODY BETTER THAN ANYONE! A month before my 22nd birthday, I noticed a small change and felt that something big with my health is coming . . . I was right, I had a severe gut infection that was finally diagnosed in December.  I  went August to December before treatment. If I hadn’t gone to the hospital when I did, I would have died in a couple days. I have refractory C-diff and still have it after a year and a half. My body isn’t normal and that’s why treating me is so complex.

My pain medicine for this awful infection is IV Benadryl. We don’t know why but it helps. I hate narcotics! I was so sick that I ended up needing a TON of fentanyl while in the ICU’s at Valley Children’s and Packard Children’s.  It took me almost 4 months to fully taper from fentanyl.  Since that time, I’ve refused all narcotics.

This past January (2016), I was admitted to Valley Children’s, again, for difficulty breathing.  This time, only parainfluenza – which I received on a plane from a coughing child whose parents allowed this ill child to travel while actively infected and coughing.  I’ll cover this chronic issue in a later blog.  I was admitted to the PICU for oxygen support and the parainfluenza resolved in four days, a record for me. HOWEVER, on the day of discharge, my admission blood cultures came back and I had a severe life-threatening fungal infection.  Since I have a central line, I had a complication called a CLABSI or central line associated blood stream infection.  This required immediate surgery for removal of my central line, insertion of PICC line and a six week course of anti-fungal IV therapy.

I lost myself at this point. I had NO confidence that I would live and fight this off. I am still here. Now the hard part starts, I suffer from PTSD due to the past four years. I recognize that we, meaning the congenital chronically ill patients need a bigger voice. I feel as if I’m waking up from a four year long coma and now have been thrown back into society. I want to tell my story so maybe others don’t suffer the medical mistakes I have endured. I want to speak out and tell my story and guide physicians, nurses and hospital administrators how to treat and how to talk to us as complex adult patients. I might be 23, but my diseases are not adult diseases. I will forever be affiliated with a children’s hospital due to the origins of my diseases.

EVERYTHING HAPPENS FOR A REASON and I believe that I’ve survived for a reason and my reason is to help other adult complex congenital patients. We are not alone! I am Rachael and I have spent my entire life fighting and if I can help/inspire just one other person, my journey will be worth it. Don’t give up, I have wanted too many times.  But, I know that if I do, then everything would have been for nothing.

My body is calming down (for now) and I am now dealing with PTSD and anxiety. PTSD is now documented in most chronic severe patients (It was documented last month in a study that is a first of its kind for CHD.)  I hope this blog will speak to you, whether you are sick, have a family member who is sick, passed away, or just someone who wants to help others like me.

Thank you for reading, this will be a Blog about my health and struggles. By following me, maybe we can help each other or those with friends or family facing similar trials.