Not Just CHD

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

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

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

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

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

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

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

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

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

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

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

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

 

Compassionate Care Conference 2017

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

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

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

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

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

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

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

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

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

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