States of Healthcare

Interview with Walt Jones, Rare Disease Patient

New York from Manhattan to Upstate 

According to Walt Jones, attending college in New York City was a growing up experience and 1980 was a great time to get a job. That was the year he graduated Columbia University with his undergraduate engineering degree and twenty-two job offers. The Knolls Atomic Power Laboratory (KAPL) near Schenectady, New York stood out. Quite smartly, he notes, on the interview day after lunch the team drove him around the peaceful suburban streets of the area. Not very subtly, they told him that soon he would be able to buy a house in one of the surrounding neighborhoods if he wished. The Schenectady area had good schools, was an equidistant three-hour drive to New York City or Boston, and provided access to lots of recreational activities in the nearby Adirondacks and Helderberg Mountains. Walt was already familiar with the benefits of life in the suburbs, having grown up in one outside of Seattle, Washington. KAPL did not so much plant the seeds of all the benefits life in Schenectady could provide, as show him the budding tree.  

Schenectady, New York, both a city of about 70,000 and county of 160,000 located outside of Albany (population: 100,800) has long held engineering as a core facet of its identity. Most notably, Thomas Edison created the company that became General Electric (GE) here in 1886. The local GE campus once boomed, especially post World War II, but now is mostly a historical artifact of the region. 

KAPL was founded in 1946 as a wartime joint endeavor between GE and the government to research and develop electricity from nuclear energy. Coincidentally, a young President Jimmy Carter trained as a naval reactor engineer at KAPL in 1953 while in the Navy, before resigning the same year to take over his family’s peanut business. Today, KAPL exists distinct from GE as a defense contractor supporting the research and design of US Navy nuclear-powered ships. 

During Walt’s forty-year career at KAPL and helped along by the master’s degree in heat transfer and fluid flow from Rensselaer Polytechnic Institute, control systems design emerged as his ultimate focus area. In layman’s terms, control system design is all about applying logic to interface design, making sure there is a clear flow between the operator and functional applications of a machine. While this sounds simple enough, I would argue the phrase “it’s not rocket science” could be supplanted by “it’s not nuclear submarine reactor science” with equal gravitas. 

His job brought Walt to Schenectady, but his life there was defined by raising a family there with his wife Karen, an upper Midwest native and Sarah Lawrence alumna. The couple recently celebrated forty-two years of marriage and have two adult children. The family has also hosted several international high school students from Germany, Hungary, and Thailand as part of a cultural exchange program, who post-departure remain in touch with their beloved American family. Throughout all seasons of their family’s growth, New York state enabled connection and enrichment at every turn.

“New York is bigger than most people realize – it has everything from wilderness in the North to as urban as urban gets in New York City,” Walt describes. “New York State is a place that puts very few limits on you as long as you can afford it.”

Neurology and Ophthalmology: An Unlikely Pairing

Interwoven with Walt’s life in Schenectady are a set of healthy behaviors. He runs fifteen miles a week for the express purpose of aerobic fitness and loves to cook homemade vegetarian meals, a hobby bolstered by skills imparted by the local Culinary Institute of America (yes, you can technically say he is CIA-trained). Walt has abundant and supportive community. Despite a shortage of general practitioners in the area, he regularly engages primary care. These healthy behaviors have not eliminated a need for intensive healthcare usage. 

“I was immortal until I was forty-nine, and then that came away rather quickly,” Walt summarizes.

This was the age when he experienced an onslaught of symptoms from what he would later learn was Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), a rare autoimmune condition where the body attacks the myelin sheaths that cover nerve cells in the peripheral nervous system surrounding the brain and spinal cord. According to Johns Hopkins Medicine, CIDP is often considered the chronic version of Guillain-Barre syndrome, and if left untreated, about a third of patients require a wheelchair. There is no consensus on the disease’s cause.

“It got to the point where I had trouble walking and was falling down,” he recalls.

Walt was referred to an Albany-based neurologist, who unbeknownst to him was not familiar with his condition. The neurologist initially misdiagnosed Walt until someone in his practice intervened, noting that Walt was much younger than the typical patient of the wrong condition and that he would be crippled within a year if the diagnosis was accurate. After undergoing additional diagnostic tests, including a cerebral spinal fluid sample, Walt was diagnosed with CIDP. However, a correct diagnosis did not equate correct treatment. 

Since CIDP is an autoimmune condition, immunosuppressant medications are typically prescribed to manage the chronic disease. Because they are leveraged to suppress the body’s immune system, they can have harsh effects on general health and leave patients vulnerable to other illnesses. Among his many medications, Walt found the steroids, a subclass of immunosuppressants, to be particularly unpleasant. 

“If you come to me as an engineer and say I want you to design a bridge I would say I’m not that kind of engineer, here’s my friend that does that though. In my experience, many doctors will take a shot at designing the bridge even if they don’t have the experience or training, and that was my experience with my first neurologist. He had no experience with my disease, granted it’s hard to come by, but he should have sent me some place else,” Walt explains.

Twenty years earlier, Walt first engaged with specialty healthcare at the onset of pigmentary glaucoma. Despite being treated according to the American Medical Association guidelines, he still had poor vision. Being his methodical, engineering self, Walt read scientific papers on the condition shared to him by a nurse friend.

“My friend tossing me a half dozen papers pre-internet, getting me to question more, and to gain some responsibility in learning about my condition, changed my life,” he credits.  

When his initial ophthalmologist could not answer Walt’s clinical questions, the physician referred him to a more experienced colleague who ended up taking Walt on as a patient and helped him manage the condition better. Walt later learned this physician was a leader in the field and a minor celebrity in ophthalmology. When this doctor eventually retired, he transitioned his patients to a former student who also embraced Walt’s scientifically engaged and medically literate patient style.

Walt advises, “It’s important to be able to speak doctor when you need answers.”

It was this replacement ophthalmologist, who raised the alarm about Walt’s unsatisfactory neurological treatment following his CIDP diagnosis.

“He said you look awful, write down every drug you are on. Then he said this is a list of drugs that a neurologist gives when they don’t know what’s going on; you need to get out of town and go to Boston, New York City, the Mayo Clinic, or Stanford to see the big kids. And I did.”

Walt turned to his local library, which employs a medical librarian who helps pull academic papers from diagnostic codes for patrons. He read the papers available about CIDP, including one by a specialized neurologist with Columbia-Presbyterian in Manhattan. Seven months later, he was able to be seen by the author, and within three months, this neurologist had “figured it out.” Although considered a rare disease itself, CIDP is a broader classification of autoimmune conditions, or “a garbage can diagnosis,” as Walt describes it. With additional testing, Walt’s diagnosis was further narrowed down to MAG-antibody induced chronic inflammatory demyelinating polyneuropathy. This neurologist, who Walt references as “the wizard,” also worked with Walt to adjust his medications to better manage the disease. The updated regimen notably excluded steroids. 

“Now I am on the right medications to effectively control my disease. There are not necessarily fewer drugs than before, but they have way fewer side effects. That was part of the original neurologist not knowing what they were doing. It’s quality of care and lifestyle, not number of drugs, that matter. Now I can run, ski, and pretty much have no restrictions beyond getting an infusion every six weeks.”   

After being treated at Columbia-Presbyterian for six months, Walt asked the neurologist expert if he knew anyone in the Albany area skilled in treating CIDP. As Walt anticipated, it is a small physician community, and the physician was able to make a referral to another specialist he knew at Albany Med. Walt has been treated there for the past twelve years and is happy with his quality of care, but notes that his current physician credits the Columbia-Presbyterian neurologist for doing the “heavy lifting” of getting Walt back to a decent place with the disease.  

“My ophthalmologist being a good doctor kicked off that whole train of events. I’ve been told by some clinician friends that the ophthalmologist was my friend.”

Walt’s biggest takeaway from his diagnosis journey is the importance of soliciting clinician recommendations from other healthcare workers that he trusts. Currently he is treated by eight practitioners, three of which he thinks he has built up a relationship to the point that they will give him honest, real recommendations. One of Walt’s main concerns regarding his care is that several of his trusted team of clinicians are nearing retirement. With a provider shortage in the region, he worries about finding the right replacements. 

Still, he notes that New York has many positives when it comes to accessing healthcare.

“If you know how to access them, there are almost infinite medical resources in this state. It takes time, money, and effort to access the resources, but resources being available to you is about as good as it gets in this country.” 

Paying for Care: Insurance Hurdles

With Walt’s CIDP diagnosis came insurance paperwork. Since his disease is so rare, most of his medications are prescribed to him off-label, meaning that they have not been approved by the FDA for use in patients specifically with CIDP. Because of this, his insurance collects letters from Walt’s medical team that provide clinical context about his disease and justification for the prescriptions. A medical review panel, an insurance-employed team of clinicians, then approves or rejects coverage of the off-label medications individually. The review process used to terrify Walt, but the panels have been reasonable in approving his therapies in line with his neurologist’s recommendations. 

Prior to his recent retirement, Walt utilized employer-sponsored private health insurance. Every few years his workplace changed the insurance package offerings, and he had to meticulously comb through the drug formularies of the available plans to find one that covered all his medications, which can be quite expensive. Each selection year, there was always at least one offered plan that covered his medications. Multiple years only one plan covered his medicines, provoking dread for a hypothetical future with no full coverage formulary in the plan catalogue. Now Walt is insured through Medicare, which covers all his medications albeit with similar panel review requirements. 

Walt’s primary complaint about private health insurance is a lack of transparency. While this is hardly a controversial opinion, Walt’s data-driven approach seems to especially come in conflict with bureaucratic secrecy. In one particular example, when Karen needed a spinal surgery, the couple researched options. They learned that a cutting-edge University of Pennsylvania clinic could complete the surgery with a far narrower and more precise incision than what was offered in Albany. The narrower incision was clearly preferable as it translated to fewer health risks, less pain, and faster healing for what was already going to be an arduous recovery. Walt and Karen collected the procedural billing codes during a clinical consultation and proactively asked their insurer if the more advanced clinic was in-network and what they could expect for out-of-pocket costs. Even with the billing codes and site information, their insurance plan was unable to answer either question.

Ultimately Walt and Karen had to gamble on cost, knowing that the quality of life benefits of a smaller incision were important and they had savings to fund the procedure if necessary. After the procedure, they were lucky to learn that the University of Pennsylvania clinic was in-network through a third-party contract with their insurer and therefore reimbursed to have the same out-of-pocket costs as the Albany site. However, Walt was left feeling like his insurance had behaved in an “underhanded” way by seemingly obfuscating the coverage information as long as possible. 

“It would have been very difficult if we didn’t have the means to take that risk,” he recounts. 

Walt’s Prescription for the System

So after all his experience receiving healthcare, how would Walt fix the system?

Based on his experience with Medicare, Walt supports a single payer healthcare system for the country. Compared to the stress of having to reselect a commercial insurance plan periodically and a perceived lack of transparency, he finds that Medicare with a supplemental private policy works well. He knows that currently it is difficult to access essential care for many New Yorkers who do not have sufficient insurance coverage but identifies this as a nationwide problem versus a New York-specific issue. He sees that the component unique to the state is how some of the most advanced medical research in the world is conducted in New York City’s academic hospitals, creating a “wide spectrum” between those with and without access to the necessary medical care.

To improve healthcare access in New York state, Walt supports efforts to alleviate provider shortages. He encourages increasing pay for advanced practice clinicians (i.e., nurse practitioners, physician’s assistants) to encourage recruitment, especially in primary care. As a patient, he has seen that the lack of general practitioners in the region has resulted in tangible barriers to care. It is difficult to get a primary care appointment and overworked offices are not very responsive, putting it on the patient to chase them when phone calls are not returned. He likes his primary care provider and her willingness to answer his technical medical questions, but she always seems hurried and stressed seemingly due to her patient load. 

“I don’t think she can practice medicine how she wants to practice under the constraints she works in,” he theorizes.

Despite the many hiccups along his medical journey, Walt emphasizes the positives of his experience, especially crediting the clinicians who contributed to his current state of health.  

“I have been very lucky with the healthcare system. I had some people hold my hand at the right time,” he reflects.

Walt’s health is a team effort but, arguably the most important member of his clinical team is Walt himself. He is about as engaged and medically literate as a patient can be, and surely his relevant questions on the mechanics of his care have only encouraged his provider team to provide high quality treatment. Walt’s informed advocacy, a trove of medical resources in New York, a few clinicians that went above and beyond, and a bit of luck have provided a strong foundation of care that enables Walt to lead a high quality, albeit mortal, life.