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.