EPILOG

(Place at end of book. Page 314)

Epilogue

October 2019

I'm sitting with Holly in an office at Penn, waiting for the Endocrinologist to see me.This is my second time seeing her, after being introduced back in the spring. I have seen so many new doctors in the last few years. A Medical Genetics physician, my Pain Specialist, a Cardiologist, along with my normal team, consisting primarily of my Urologist and Family Doc. Most are young. I think how this all started, when the distinguished older gentleman named Dr. Nicholson took care of me.

But that's life. Everything goes in cycles. Even the building I'm in is so different. Sparkling new, with brightness and multitude of windows letting light inside, even on such a cloudy, windy afternoon in autumn as this. This gleaming building  of glass is so alive with hope and  brightness compared to the gray, dark fortress of stone which was the old hospital in Philadelphia which no longer exists. One's mood is uplifted  by the vast openness and vivid colors  of the future, especially compared to depressing, narrow corridors and drab colors of the past.

I must admit that I am nervous today visiting Dr. Mona. I did all she asked to prepare for this appointment: new blood work to satisfy the many different tests she wished to check; another urine collection  (always fun); and completed all paperwork that was due.

So why am I nervous? My blood work in the spring was good.  I had elevated my water intake and was concentrating on raising my calcium too. I added weekly salmon to my diet ( also great for increasing Vitamin D, which she also requested); I was now drinking more milk ( preferably chocolate); I liked most diary products, especially ice cream ( probably too much so, but ice cream is a pleasure in life I just can't ignore)' I couldn't do much cheese or yogurt ( unless yogurt pops...sort of like Popsicles,  count).

I began taking vitamin D supplements, which I actually like. Chewable gummy bears are always good. I feel like eating more every morning and have to stop myself from overdosing on vitamin D.  Funny, gummy bear withdrawal.

Dr. Mona was treating me to strengthen my brittle bones, but she was also focusing on preventing more kidney stones. She asked me to start taking 500mg of some kind of horse pill every morning to increase my citric acid level. When Dr. Rose was my Urologist he suggested the same thing, only spiking my water with a squirt of lemon juice to give it an extra zing of taste.

I always had trouble swallowing pills, especially big ones. I had to crush everything, even going back to my days at Children's Hospital. Otherwise count on major gagging and the pill not going down the pipe. I'm still embarrassed by that: here I am, 62 years old, soon to be 63, spitting out pills like our dogs Bud and Katie., even tiny baby aspirins that should be no problem on the back of my tongue and with a gulp of juice.

However now I have  a legitimate alibi: since I was diagnosed with Barrett's Disease of the Esophagus. Dr. Rock, my Gastroenterologist, recommended that I have all my pills ether crushed or substituted for liquids or capsules, if possible.  I even have a struggle with capsules.

So now I had another reason to go along with personal gagging.

Also, the last time I saw Dr. Mona, she recommended I think about having an infusion done once a year to make my bones stronger. This new procedure hasn't been  approved yet by the FDA so I had some concerns that I wanted  to discuss with her. Plus I had another worry on my mind concerning the event.

She had mentioned having a Bone Scan done down the road. I probably had one done somewhere in my past but I honestly couldn't remember. One procedure and one test seemed to jumble with another test and another procedure. I just can't keep track anymore.

So, I have a lot on my plate as we wait and wait and wait. These are the new treatments and techniques used for OI patients in 2019. There is still the ol' pins and rods surgeries that I had so long ago, designed to keep the weak lower leg bones from curving and from breaking so easily.  They still cast fresh fractures but now there are more funky  and decorative casts to choose from.

Now I'm beyond any kind of surgery, even for my progressive scoliosis in my back. Oh, I could consult with a spine doctor and go through operations to straighten  my back.   Right now everything is rotating to the left. I fear someday I will resemble the Elephant Man, with the trunk of my body all distorted and deformed.

But unless my life is in jeopardy I don't want anything extraordinary  done. Everything is quality of line now. Surgery is great for younger people with Osteogenisus Imperfecti. Whatever it takes to live a long, pain-free and unbreakable future. For yours truly, these  new treatments- like the proposed infusion- are my options to make life more stable.

Life really does go in cycles! I think about how it was when I was an infant, then as a little kid, all the way to my teenage years. Broken bones and more broken bones. Emergency Room visits and hospital stays were my life. And when I didn't fracture I was healing until the next break occurred.

Then in my middle-aged years, from early 20s to 50 or so, everything solidified. I no longer snapped a bone by merely turning over in my sleep or sneezing too hard. Now it took a real fall, and those were few and far between, thank God.

But now here we are, entering the autumn of my life, and I'm beginning to sustain more fractures with each passing season. Only now, they don't happen with such fury, so devastatingly fast, the crunching of my bones and that all-too-familiar  knifing pain. Now they sneak up on me in the disguise of stress fractures.  I mistaken them for arthritis sometimes, an achy throbbing in my old bones. They often happen in the spring and fall, when the weather changes or during a particular rainy time of the year. I can "feel it in my bones," literally.

Yet many times it isn't arthritis at all ( which I have plenty of anyway), but a stress fracture, often in the thigh and femur area, where there is additional stress from sitting and transferring. These go away  in a few weeks but they frequently don't treat them with casting, especially when the cracks occur in the ribs or the back.

Treatment is generally heating pads, pain meds and pain patches ( although patches are expensive and often not covered by insurance).

So it's a matter of waiting it out with many sleepless nights and painful mobility, which wears one down and makes one feel even crappier.

If any of these new procedures can help, why not try? Plus participating in certain experimental treatment may not only benefit me but those to come in the future. If I can help lessen fractures for those after me, why not ?

As we continued to wait, my eyes scanned the small waiting room. It was painted in ugly  army green and gray, surprising since the outer part  and lobby area of the medical building was so cheerful. The usual medical equipment hung on the wall or sat on the cabinet: purple latex gloves, different instruments, like blood pressure cuffs, jars of gauze and tongue depressors.  A computer was on, hooked up  to a small  desk. Pretty standard stuff.

Holly nudged me and pointed to a yellow blob  near the sink. "Remember that?" she asked.

How could I forget? I couldn't believe when Dr. Mona revealed what it was, to satisfy our curiosity: a five pound piece of fat.  It looked like the famous Blob in the 1950s horror classic of the same name, which oozed around my hometown of Phoenixville, especially the historic Colonial Theater. Only this hunk of blob was yellow.

"Hey, until she gets here, like tomorrow, let's play touch football with the fat," I joked. Holly laughed as Dr, Mona herself whisked into the room.

"So how's it going?" she asked me as we shook hands. I had forgotten how pretty she is- tall and thin, dark eyes and long dark hair pulled back.

She brought my recent blood work up on the screen and seemed pleased. Same with my urine output. I passed that exam, drinking extra water on collection night to make sure I met the quota.

She continued to scan the screen, reviewing my electronic records as she asked, "So tell me what happened regarding the pills?"

"Oh, I couldn't take those. I would have to crush them and you said  'no crushing allowed'."

"That's right," she said. "No crushing. So why didn't you take them?"

"Well, Dr. Rock said I needed to crush all my pills since  she found I have Barrett's. Plus I have this little pocket in my upper esophagus that sometimes catches food and pills." I left out the part how I was a real wuss and couldn't swallow pills of any kind.

"They were big," she acknowledged. "That's okay." I felt vindicated and my manly pride was still intact.

" So how's your calcium intake going?"

"Great," I said, perking up."I'm eating salmon every week.."

"Hun, she said 'calcium'," Holly gently interjected.

"Oh, of course." I had to get the fact that I was eating fish every week in there somehow.

"Well, eggs, milk with cereal twice a week. Chocolate milk, of course. Lots of ice cream. I mean, ice cream."

"Cheese?" she inquired.

:"Sorry, no cheese. I was never a fan. I make up for it in ice cream," I insisted.

I was amazed to find out that Dr. Mona had actually ordered that I do eat salmon, but canned salmon, plus the bones. Holly and I looked at each other, perplexed. Bones? We don't let our dogs eat bones. Yet  she wants bones to be added  to my diet?

Luckily  she let that one go. Overall Dr. Mona was happy. Next came the meaty part of the visit.

"Have you had any more fractures since I saw you last?" she wondered.

"No," I answered honestly. "Just the usual aching from seasonal arthritis. Why?"

"Well, your  recent blood work shows your phosphorous levels are high. They were high last time, and even higher now. We may have missed a recent  fracture."

Was that the reason why my legs hurt so much this spring, and even now, into fall? Have I been going along with fractures and didn't even know it? Maybe I couldn't tell the difference anymore between arthritis and stress fractures? That possibility made me feel cold.

"You're probably right," I mumbled, staring at the floor.



"So, I think now we can move on with scheduling the infusion I talked to you about last time. But I've heard you have concerns?" she began."I pretty much know what they may be from my other patients."

"I do have concerns," I admitted. "Can you please review the whole thing again?"

Dr. Mona came closer on her stool and looked me  straight in the eyes. She once again explained how it was an experimental procedure and how it was used for people with osteoarthritis and more common maladies. It had been tried on OI patients with success. In fact 50% of the cases reported less fractures during a long period of time after treatment. It was given by IV once a year, a 30-minute dose of Reclast (Zoledronic Acid) Some side effects were flu-like symptoms, jaw swelling or pain, and in  even rarer cases, stress fractures in the femur areas of the legs.

That puzzled me. Something that is supposed to make bones stronger may actually cause fractures?

After she explained everything about the infusion treatment, she asked if there were anymore concerns.

I hesitated. This was important, so I needed to be open about my fears. Over the past few years I  became even more fearful of being lifted.  As I became quiet, Holly, my angel, spoke for me.

"Greg is afraid to be lifted onto a table.," she softly said.

"Why are you afraid, Greg?" Dr. Mona asked, her voice lowering.

"Oh, I've had some bad experiences  being lifted," I said, my voice cracking. It was true. Being lifted wasn't my favorite thing in the world. My bones ached from age and wear. Depending on who was doing the lifting depended how gently it was done- or not. No matter how much I tried to explain before the lift, often lifters would grab at the wrong area of my legs. Usually it was one person behind me, under my arms, and another person in front, holding onto my legs. I'm still very light of weight, maybe 100 pounds, so all it takes is 1-2-3 and up we go. But during the lifting anything can happen, from cracks to full=blown fractures, as I close my eyes tightly, my heart pounding, trying to relax, fearing the next fracture, not wanting to experience that feeling again."Try not to think about it. Try not to think about it," I kept saying to myself during the lift..

My eyes starting misting  with tears. I bit my lip, trying not to cry. I looked at Dr. Mona and whispered, "Too many fractures."

"I know," she said. I could see her sink as she stared at me.

"Can it be done in my chair?" I asked, hoping she would say yes, fearful she would say no.

"Of course," she replied. I sighed in relief. "Actually it's better if you stay in the chair. We often do the intravenous infusion in the chair. No problem."

Suddenly I saw Dr. Mona in a new light. She really understood and she cared.

So it was decided to go ahead with the infusion. Since she knew how fearful I was about being lifted, she asked me to only consider the bone scan for now. "It may not be needed if your phosphorous levels are back down.  Don't worry about it, okay? We will work around things."

She smiled and I felt so much better.

It was time to go. So many exciting things were on the calender soon. A concert, a fall Apple Festival, an Eagles football game, and my birthday.

"Let's go home," Holly said as we gathered the paperwork.

"Good idea," I agreed. "You know, between the flu shot earlier this week and today, I'm gonna need some extra TLC," I said, laughing, as we headed for check-out and then for home.