The day my husband Ed got a really bad bug. So you really are sick, what now?

Ed is a working guy and was an on-the-road serviceman for 30 years, first with Southland Corporation (7–11), then 25 years with a chemical company where he designed, installed and maintained chemical pumping systems. In his mid-forties he was laid off. This was at the end of the housing bubble as the economy was going into recession. He got his contractor’s license and became a self-employed handyman. With his wide skill set he soon had a regular clientele.

When you work for someone else, health insurance is usually available. Ed had worked for big corporations and that was part of his benefits package. Now he had to buy a policy for his family. An annual insurance policy is a necessity. What do you get for that policy that you pay for every single month?

Ed’s Story

Throughout his life Ed had a variety of minor illnesses that were annoying, but incidental, each minor illness lasting a few days or a week longer than everyone else who had it. As a child, he had more than the average earaches, and as an adult he caught most of the colds that the kids brought home. All summer long he used sunscreen on his nose to keep from getting an infection on his skin.

In his forties he had ongoing indigestion which grew slowly in intensity and proved to be a lactose intolerance. Changing to lactaid milk fixed that, although packaged foods and dining out require vigilance to spot milk as an ingredient.

All of these were nuisances with doctors treating issues as needed. Generally speaking, Ed chalked it all up to getting older.

The Health Crisis

Month Zero (July 2015)

At the age of 56 Ed, and the other three people in our household, caught an intestinal bug that ran a 48-hours course. Except Ed. His symptoms of diarrhea and fatigue continued and he began to lose weight. Fast. For Ed, diarrhea meant “all the food I ate an hour or more ago just passed through me chewed, pretty much the way I swallowed it.” Nothing was changed by passing through his digestive tract. He ate copious amounts of calories and lost weight at an alarming rate. Ed dropped from about 185 lbs. to the mid 150s — low 160s in 4 to 6 weeks, by which time I had him to a new gastroenterologist.

Status: Ed had no energy. He sat in his Lazy Boy day after day sleeping, carving wood, and eating chicken noodle soup.

Months 2–4 (August -October 2015)

We had the good fortune of meeting a local physician who was a new member of an established practice. She took the time to listen to the history of Ed’s experience and review any recent testing that we brought with us. Comprehensive blood, stool, and urine tests were ordered. The blood test pointed to a problem in the immune system, which created the need to rule out HIV regardless of virtually zero chance of its being possible.

Blood testing found low levels of immunoglobulin G. An HIV test was ordered, which caused major anxiety over a long weekend as to how he could have contracted it. Results came back negative.

The doctor also found bacteria in his stool and treated it with antibiotics.

Status: Ed continued to have diarrhea and to lose weight.

Months 4–6 (October — December 2015)

So after checking things off the list and having both an upper and lower endoscopy, Ed’s doctor determined that “your case is above my pay grade” and recommended an immunologist at Johns Hopkins as the next step. The most likely candidate for Ed’s condition was something called Common Variable Immune Deficiency or CVID. Here is how they test for it:

• Have vaccinations for flu and pneumonia.
• After about 5 weeks take a blood sample.
• Test for antibodies Ed’s body produced in response to the vaccines. If there are none, the diagnosis is CVID.

Ed’s blood work showed no antibodies for flu or pneumonia. They also tested for tetanus antibodies.

Ed’s immunologist told him that “tetanus is the best vaccine we have made. It will stay in your system for 30 years even if you miss revaccination dates.” Hands-on-guy Ed has always kept his tetanus shots up-to-date. Ed had no tetanus titers.

So what is CVID? A genetic disease, not inherited from your parents, but caused by a gene that you were born without, or turns off at some point. There are many subsets of immune deficiencies. While a whole lot of causes are not known, the result — diagnosable CVID — is.

There are several immunoglobulins (IGs) differentiated by letter, abbreviated IG — A, G, M, etc. The most common issue is with IGG. Normal levels of IGG are 700–1500 parts. Ed’s were less than 120. In immune deficient patients, IGG is replaced by using donated plasma. The average dose comes from many people, effectively giving the recipient a “crowd-sourced” batch of immunity in each treatment. CVID is often under-diagnosed. When it and other immune deficiencies are more accurately identified, the need for plasma donations will increase because there are no synthetic substitutions.

CVID treatment is a weekly or monthly dose of immunoglobulins for life. There is no cure. Ed started treatment exactly five months after his initial crisis.

To give a sense of how awkward CVID can be, several months after diagnosis Ed got a cut on his head and went to the emergency room for cleaning, stitches, etc. It is normal to get a tetanus shot for such a cut. Ed told the doctor that a tetanus shot would be useless because of CVID. This was a medical condition she had never encountered, logical but a bit foreign to routine ER experience. The hospital in fact had tetanus immunoglobulins on hand for immune deficient patients. Who knew?

So why the weight loss? Since CVID is an immune deficiency condition, other conditions occur. Because the weight loss and diarrhea are part of the digestive system, Ed’s condition was labeled “enteropathy” which means digestive problem. The actual cause of weight loss had yet to be determined and since we were referred to Johns Hopkins for the primary condition and it is a top-rated medical institution, we also went to their gastroenterology department.

During the five weeks we waited to see how his body responded to the vaccines above, Ed’s gastroenterologist at Johns Hopkins began steroids and other therapies in an effort to curtail symptoms.

About one month into the immunoglobulin treatment and two months of steroids, Ed was still losing weight. At this point he was down to 131 lbs. on his six-foot frame. So he was checked in to Johns Hopkins hospital for further investigation.

Status: Ed continued to have diarrhea and to lose weight.

Month’s 7–8 (January — February 2016)

Ed was put on IV nutrition via a peripherally inserted central catheter (PICC line) — Total Parenteral Nutrition (TPN) and IV fluids. A PICC line is a hose that enters your arm near your elbow and travels up your arm across your chest through a vein and stops close to your heart.

He had upper and lower endoscopies. These produced tissue samples that showed a condition called villus atrophy. The small intestine has “villi” which are like little fingers that massage food, helping to break it down. They also slowly absorb nutrition into the body as food passes through the system. Ed’s were limp and lifeless. Since Ed’s condition was similar to Crohn’s disease that was the model on which the doctors were basing decisions. Therapy for Crohn’s was continued. In Ed’s case this included Remicade.

Status: Ed continued to have diarrhea and to lose weight until month 8 when the TPN is begun. His weight is then stabilized.

Month’s 9–12 (March — June 2016)

Prior to the first Johns Hopkins stay we had been advised to apply for a clinical trial at the National Institutes of Health (NIH) for CVID with Enteropathy. After receiving the diagnosis of villus atrophy his condition was even more specific to that trial. Ed applied for the trial.

• CVID is about 1 in 250,000.
• CVID with Enteropathy (in this case specifically Villus Atrophy) is about 1 in 500,000.

We had a meeting with the doctor in charge of the clinical trial. Ed was accepted. We decided that Ed would finish the course of treatment he had started at Johns Hopkins. If it did not improve his health then he would start the NIH clinical trial.

Working with Johns Hopkins, his body weight slowly went up to about 150 lbs. and he was feeling better. Although his stools were still very loose and his diet was still discernible in them, they were becoming very regular and only once daily. All the drugs that the Johns Hopkins gastroenterologist prescribed were being taken and so TPN was slowly tapered off. After nearly three months on TPN it was discontinued; after about a week the PICC line was removed. Within a week he started dropping weight. The TPN had provided nutrition that his digestive system could not absorb naturally. Meds were changed, with little effect on the recurrence of weight loss.

With the renewed weight loss Ed went back into Johns Hopkins for more testing (specifically to look for bugs in his stool of which they found none), a new PICC line and more TPN. The treatment was not proving effective for Ed’s enteropathy and the testing was showing no new information.

In early July, one year almost to the day after the initial crisis, Ed emailed his doctor at the NIH, saying “Johns Hopkins struck out, you’re up to bat.” He sent the email at 7 pm on a Saturday and received a phone call within 20 minutes. We found out later that he was one of less than a dozen people to qualify for this trial, which would study the efficacy of the drug ustekinumab.

Preliminary blood work through the NIH immediately showed that Ed had a parasite and two bacteria in his system, bugs that a week of sampling at Johns Hopkins had not found. Antibiotics were given and within a week Ed’s symptoms were reduced.

Ed’s experience

After the first Johns Hopkins stay Ed was sent home to continue TPN via the PICC line daily, and IGG subcutaneous infusions, which were weekly injections into each thigh.

About every other day there would be a home care nurse to check on the PICC line, take blood and other vitals from Ed to check blood levels of minerals, vitamins, liver health etc. The nurse would deliver all data and samples to the Johns Hopkins testing facility within hours.

The Johns Hopkins pharmacy would assess the test results immediately so any changes could be made to the custom mix of everything a growing boy needs. His pharmacist kept tweaking his milkshake based on these blood tests. Weekly, or sooner if a change were made, a special delivery from Johns Hopkins would come to our door in refrigerated boxes. They would contain bags of TPN, hoses, wipes, bandages, flushes, and other supplies for the nurses. Once a week the nurse would change the bandage around the line in his arm and inspect for infection.

With me, his loving wife, being “needle adverse,” Ed did all his at-home hookup care himself. This meant nightly removing the bag of milkshake from the refrigerator; filling a syringe from vials of vitamin supplements to inject into the milkshake bag at a special spot; hooking up a metering pump to himself and the bag; and starting the 12-hour timer, then sitting or sleeping until finished. In the morning, 12 hours later, he removed the bag hose from the PICC line in his arm, removed the pump, plugged it in to charge, flushed out the PICC line and secured it to his arm with a gauze bandage. It was imperative to keep the line and everything around it clean, as this was a perfect spot to infect one’s body, bloodstream, heart, and add to his immune issues.

Once a week he would infuse himself with IGG subcutaneously. This meant filling a large syringe with 40 ml IGG from pre-measured vials and attaching it to a hose that split into two separate hoses with a needle at each end. One needle was stuck into each of his thighs. The syringe was inserted into a pump which, once started, pushed the syringe plunger slowly for about 25 minutes. Ed was up for all of it and found the entire experience interesting.

Throughout this time Ed was never hungry, but he would eat. Most every day I would ask what he felt he could eat. I would make supper and deliver to him. Sometimes when a requested meal was delivered he would say “sorry I can’t eat that” as the sight of it made his body rebel.

Status: Ed continued to have diarrhea, his weight is stabilized until he got off TPN and it began to go down again.

Month 13–32 (July 2016 — February 2018)

Ed started in the clinical trial for ustekinumab in December 2016. The trial ran through February 2018, a year and two months later. In the course of working with the NIH Ed has been added to up to seven clinical trials. Most of them are information gathering, and will involve taking blood annually and gathering other testing statistics through his life. These include but are not limited to MRIs, biopsies, CT Scans. Some are observational for known CVID repercussions such as nodular regenerative hyperplasia, a condition of the liver. Because his condition was found at the beginning of physical effects/changes that occur with CVID he is a time capsule of CVID knowledge. The ustekinumab trial produced positive results for Ed and his villi are getting healthier but absorb only one half of the normal rate of fats and protein. He remains on the — rather costly — drug therapy.

Status: At some point in the NIH trial diarrhea eased and food processed a bit more normally. By the end of the trial his weight increased to about 190 lbs. Low absorption rate of fat and protein persist.

Dorothea’s Experience

Throughout this saga my role was caregiver and paperwork/appointment guardian. The caregiver role was constant but low tech and not overwhelming, all things considered. There is a relentlessness to a chronic health problem that goes beyond medical care or general physical back up. There are the daily needs of running a household, paying bills, acquiring money for said bills. But one blessing for our situation was that we have a fairly uncomplicated lifestyle, in that we are both creative and self-sufficient and don’t need to be entertained. We always have projects around and lots of supplies to do them. So tightening our belt for managing regular expenses was a little bit more flexible for us than for many people.

As you can guess Ed couldn’t work much and hadn’t for several months now. We were VERY lucky to have benefactors that saved us from bankruptcy. A dear family member said “Ed, I’m paying you to get well” and meant it. Members of our congregation put on a benefit concert for him as well.
With TPN he started to put on a few pounds, and get a little energy to do some small home improvement jobs. Because of the PICC line in his right arm he was restricted to lifting 10 lbs. maximum with that arm. He did not like that one bit! Right at this time he had the good fortune to meet a person who needed part-time work and became Ed’s helper two days a week. He got Ed through a really tough work period, able to drive when Ed was too tired, hand off tools, and generally help in every way. He is with Ed to this day and has become a family friend.

So why tell the tale?

We were very fortunate with resources and Ed’s overall attitude was “I’m not afraid of dying. If this illness and the studies on my body bring new information to medical care, that’s fine with me.”

My experience from 1983, when I was first self-employed, to the present day has given me a pretty complete education in what I now call:

Our Health PAPER WORK Care System

The system we have was created to fill the needs of people and corporations who are not the health care receivers (of course some of those people will need a doctor at some point). There is a fascinating history of the health insurance industry that I hope to write about in a separate article. For Ed’s story, here are a few details of what we, as consumers, must accept to receive health services in the USA.

1) Health insurance policies are stunningly expensive. Between the start of Ed’s illness and three years later the cost of a monthly premium for our household directly from Blue Cross Blue Shield went from about $1000.00 to over $2700.00. The loss of income resulted in qualifying for the Maryland Health Exchange but there were a couple of really tough years before then. The deductible/out-of-pocket for the family is still well over $10,000.00.

2) To receive the benefits of health care while insured, you must follow all the rules and requirements presented to you by two entities — the insurance company and the health service provider of the moment (i.e. any doctor you are seeing). You must manage the fiduciary relationship you have with your insurer, the one you have with your doctor and — without any decision-making power — the one between the insurer and the doctor, effectively meaning that one third of your job is to satisfy an agreement you did not make!

3) A structured appointment schedule must suffice for any critical thinking that a doctor, PA, RN or other medical professional can give to you. It is this last point that shows the cracks in the wall of medical economics. If your doctor does not have time to assess your medical records and symptoms as they occur how can they possibly do a good job?

The Affordable Care Act calls for records to be kept electronically. With the increasing use of digitized medical data it becomes more possible for doctors to compare records, and for computer applications to run diagnostics to look for patterns in health histories. Data sharing through one access point, rather than multiple portals, would benefit all of us.

Billing for that first year of investigation for Ed’s symptoms was about $175,000.00, including but not limited to:

• Medical visits and testing
• Initial gastroenterology doctor.
• Johns Hopkins doctor visits, two one-week or longer in-patient stays.
  Medications — Prescriptions at our local pharmacy

The therapy that Ed must be on is weekly IGGs for the rest of his life and on ustekinumab for the foreseeable future.

• IGGs, about $50,000.00 per year.
• Ustekinumab is one $20,000.00 shot every 8 weeks. That’s $120,000.00 — $140,000.00 per year depending on the exact delivery dates.

These are the “billed” amounts and thankfully the amount paid by the insurance company and out-of-pocket is much lower. But the question is — what is the cost, what is the actual value of medicine and medical providers? The work done by the NIH is funded by us — our tax dollars. It is a remarkable resource. Much of the research becomes available to pharmaceutical companies. What impact does that have on the real cost of medicine? With profits being a part of the equation, will we ever know?

For this episode in our lives Ed and I have been extremely blessed and grateful that we had resources — family and many friends. Some brought food, some laughter, some made financial contributions. In many ways we had built a life that could absorb a hit. But many, many people cannot, and it is unclear if we could withstand it twice.

Ed and I both believe that there are some things government is simply better at handling. Health care finance for a large population is one of those things.

Medicare for All.

Thank you for reading my story

In my day job, I restore family photographs. Each family story shows the importance of learning history and sharing stories. On Medium, I write about our human nature, my experiences, and episodes in our collective story. Part of my story is that I am a founding board member of Kitsune, Inc., 501c3 non-profit. We work on creative solutions for independent living for adults with developmental disabilities, but without an intellectual disability — our fellow citizens who fall through the cracks in our social safety net.
Your participation supports this work too. Please Follow me here on Medium, and see my work at ChandlerDesignsLimited.com.