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 . . .

FullSizeRender
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.

IMG_0956

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.

sisterdisney
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.

IMG_3398
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.