A Beloved Old Friend Stares Death In The Face

The cold, cruel world paid a visit this week when a lifelong close friend of mine sent me this devastating note below telling me that she has been diagnosed with a rare and fatal disease. She was diagnosed two years ago, but kept it from many of her friends. But she summoned up the courage to disclose it to me. I'm shocked, and heartbroken. Her terrible news just stopped me in my tracks. But I'm proud of my friend's courage, and inspired by her will to live. And I'm sharing her letter with you. Because she wants me to. And because it's important. She asked that I please use an alias for her. She prefers not to use her real name. Because if she is able to get off disability, she fears she will not be able to find work, as she is well known in her community. That's of course the very least I can do for my beloved old friend.
                                                                             --- Jamie Reno

Dear Jamie,

I’ve always said that life can change in a blink of an eye. Mine did, in January 2015, when I was diagnosed with idiopathic pulmonary arterial hypertension (PAH).

A very rare disease, PAH affects 1 in 150,000, or 7 to 8 people in 1 million. Putting it in perspective, that would mean that in New York City, approximately 56 to 64 people are afflicted with PAH.  

It's a chronic disease in which the arteries in your lungs become narrowed, making it harder for blood to flow from your lungs and thus raising the pressure in those arteries.  

This causes the right chamber in your heart to work harder to pump the blood, eventually causing the right side of your heart to weaken and fail.

My disease is fatal. I know how I will die: from a massive heart attack. As I write this, the right side of my heart is three times its normal size. If I don’t die of a heart attack, I will slowly suffocate, gasping for every breath.

Before I found out that I was sick, I decided to leave my corporate job and establish my own company. I had a full medical physical while I still had health insurance with my company. 

I saw a heart doctor, lung doctor, ob/gyn, eye doctor, and my primary care doctor. I did have some shortness of breath, but all the doctors assured me that if I lost a few pounds, I would feel better. 

Great! I had the green light to start my own company. I felt like I had the knowledge in my field to be successful, and grow a company from the ground up. I quit my corporate job of over 25 years, opened my own business, and hired an employee. 

Still, I was short of breath. I had COBRA insurance, and started seeing heart and lung specialists. When I didn’t feel I had gotten a good answer or report, I saw another specialist. And then another.   

I just knew something was off.  Why could I not walk a few feet without getting short of breath? Each specialist thought that I should either lose some weight and/or that I had asthma.  

I started using inhalers, but they did not seem to help. And the shortness of breath was getting worse.

My work is very physical, and can require me to be on my feet sometimes as much as 12 hours a day.  How could I keep my company going, if I can’t walk more than a few feet without having to stop to catch my breath? 

In December of 2014, I noticed that my feet were very swollen. I mean so swollen that I could barely put shoes on. I called a heart doctor, and when he saw my feet, I could see in his face that he was alarmed. He said we need to do a right heart catheterization. Now. 

I prepared for the procedure, and afterwards, he came into my room and said, “Katie, I think you have pulmonary hypertension." I thought, "So, okay, fix it."  He said, “Katie, this is bad. Really bad. I need you to see a specialist.”

He set up an appointment for me with a specialist at a local university hospital. I didn’t know it at the time, but I was assigned one of the finest pulmonary hypertension doctors in the country. I found out that day, that I had this disease, and I began to process exactly how serious it is. There is no cure.  

Because mine is idiopathic, the doctors are not sure how I developed this disease. Since it took so long to diagnose, which is not unusual, my case is Class III, severe.  

Meaning that my heart is in severely bad shape, and that my ability to breathe has become labored with doing normal activities such as bathing, dressing, fixing my hair, laundry, grocery shopping, household chores.  

When you've moved into Class IV, you have trouble breathing while just lying in bed. This disease is often misdiagnosed as asthma. Only a pulmonary specialist can diagnose it, and only after having a right heart catheterization.

I asked my doctor, “How long do I have?”  

He wouldn't answer, but did tell me, “If you don’t take any medication, you will die within 6 months.” 

Six months. I was that sick. I am that sick.

"Great, so now I’m sick," I said to myself. "I’ve only just started a new company a little over a year ago. And I took all my money out of my 401K to put into my company. Oh, and my COBRA is about to run out in 4 months. What the hell am I going to do?"

My doctor started me on two drugs to help slow the progression of the disease, and to help open the arteries of my lungs. There is no cure – there is no possibility of getting better. But they can hope to slow the progression of the disease.  

One drug is called Adcirca and costs $3,418 per month, the other is Opsumit and costs $7,839 per month. That’s $11,257 each month, or $135,084 per year. 

My doctor puts me in touch with a foundation in Virginia which helps people with the top 20 rare diseases in the world. Thank God for these people, as they helped me to navigate the choppy waters of insurance and social security disability.  

After many long conversations with this foundation, I decided that I would need to sell my company and go on disability and Obamacare. That would be the only way I would be able to afford my medication.  

It’s simply too expensive to pay for it with private insurance.  Even with private insurance, my deductible was over $10,000, and each month my medication would have cost me a minimum of $800, out of pocket.   

Meeting those types of numbers each month was out of my reach. I don’t think I could have even done it when I was making a high corporate salary. Being sick is very expensive.

Had it not been for Obamacare when my COBRA ran out, I would already be dead. I had a pre-existing condition.  

I would have had to declare to any insurance company that I had PAH. And my medications were very expensive. Not one company would have insured me. 

Although I was “maintaining” my numbers with my disease, I was not improving with my six-minute walk tests. So in June of 2016, my doctor started me on another medication, hoping that perhaps I would be able to walk further without so much shortness of breath.  

This drug, which was just recently approved by the FDA, is called Uptravi. It costs $22,324 per month, or $267,888 per year. Now for just three medications, my costs are more than $400,000 per year. 

I take 41 pills a day to stay alive. Pills for depression, allergies, water weight gain, potassium… the list goes on and on. My medications all together cost nearly half a million dollars per year. If I won the lottery, it would be gone very quickly, just for paying for mediation. Half a million per year to stay alive.

I'm a single, white, college educated, well-read woman in her mid-fifties who made a nice corporate salary that allowed me to purchase my own home and travel whenever I desired. I wanted for nothing.  

I ate out in nice restaurants nearly every day, and enjoyed a nice lifestyle with my friends and family. I thought I would be just as successful opening my own business. I had, after all, more than 30 years of experience in my field, and was well known in my community.  

I had all the components needed to be successful. Except my health. Little did I know, I had been sick for many years. 

I’m also a women who's worked since she was 14 years old.  I worked every weekend during high school, and during the summer I worked over 40 hours per week. 

During my first three years of college I had a part-time job, and took a full-time job my senior year. Now, in my mid-fifties, I’m on disability.  

To look at me, you would have no idea that I take 41 pills a day and am forced to live on disability because I can’t afford the premiums and the deductibles with my illness. 

However, I do not feel guilty for being on disability. I put into the system for 43 years of my life. I paid 25% of my salary for taxes. I paid my dues.

But I thank God for Obamacare. It allowed me to get insurance, when I first discovered I was very, very ill. No insurance company could refuse me, no matter the cost of the drugs. No matter my diagnosis. 

I’m scared to bits thinking of what Trump and the Republicans will do with healthcare. I will be eligible for Medicare in the summer of 2017. 

What will happen to me? 

I would say to everyone who thinks Obamacare didn’t do much – think of me. 

I would say to everyone who asks, “Why is it the responsibility of the government to make sure every American has health care?” I hope they think of my story. 

Because without help from the government, I will die. With no access to drugs, I will die within 6 months.

Thanks for listening, Jamie. 

Your friend always,

Katie