Wednesday, September 30, 2009

The Internet Can Be Your Friend

I have never been more grateful for my scientific training. I started gathering my medical records and for one weekend, my husband sat at one computer and I at the other, papers everywhere. It was like a scene from Lorenzo’s Oil where Augusto and Michaela Odone were desperately trying to find a cure for their son, Lorenzo, who was suffering terribly from ALD, Adrenoleukodystropy. Doctors had no answers. They were their son’s only hope at life. We were in a similar search for our lives.

We had it easier than the Odones in 1984. We had two computers and Google, the web search engine. I began with my blood work, using Google to help sort what each test meant, both in purpose and also in results. To my shock, of the ten Lupus tests that had been run four different times over the past couple years, I had never had a positive test! My positive ANA was very high, usually indicative of Lupus. My IGM levels had been elevated on several occasions and it warned on the blood work that lupus and chronic infections of Lyme and Syphilis could be responsible. I ruled out Syphillis.

We looked at more tests. On each of four spinal taps, my protein levels were mildly elevated and climbing, with a few white blood cells in each, consistent with Lyme disease. My general blood work indicated a pattern of elevated white blood cells inconsistent with Lupus. It had been explained by inflammation, but certainly a chronic bacterial infection could produce the same results. Elevated amylase levels, unexplained by the Lupus diagnosis, could be explained by Lyme disease.

I knew there was a controversy concerning Lyme disease in the U.S. and turned to Canada and the CanLyme website for information, hoping to find an unbiased resource. I was startled to see of the 75 symptoms common to Lyme, I had experienced 65! It suggested that if you had more than 20, you should be evaluated for the disease by someone who understood the disease. Many of the symptoms listed, I had experienced during my trips to the ER just weeks earlier when nearly a dozen neurologists had examined me, yet Lyme disease had never been mentioned. Why?

In their defense, one could say my Lyme test had been negative. Unfortunately, a false negative result occurs in 40% of late-stage Lyme cases, which is why a Lyme diagnosis in late-stage Lyme should also be based on clinical symptoms. Though a negative ELISA seemed to satisfy my doctors, it didn't put my mind at ease, due to my overwhelming number of symptoms and especially when I read that Prednisone, a drug I had been on for years, was the last drug that should be given to a Lyme patient because it compromises the immune system. I had been placed on two powerful immunosuppressants, Prednisone and CellCept. If it turned out my disease was indeed Lyme disease, the misdiagnosis had not only robbed me of precious treatment time, but had weakened my own body's ability to fight the disease. Our search continued.

Tuesday, September 29, 2009

Sometimes the Horse is the Zebra II

Now, two people who had battled Lyme were telling me my symptoms matched. My hair stylist was fighting Lyme disease and seemed to know my new symptoms before I walked through the door. Because our symptoms were virtually identical, she wondered if I might have Lyme disease in addition to Lupus while I wondered if her doctor had missed Lupus? Early in our friendship, she gave me a Lyme test kit for Igenix, which my rheumatologist discarded in the trash. At the time, I did some research on my own finding the two diseases were very similar and took comfort in knowing that I was working with one of the top rheumatologists in the country. As my symptoms continued to worsen and my disease pattern mimicked hers, I remember saying later in our friendship either she had Lupus or I had Lyme, because we certainly had the same disease. Now a second person was saying the same thing! The time had come for more research.

Doctors were so insistent on looking for the horse when hearing hoof beats, but what if, just maybe, the hooves belonged to a zebra?

Monday, September 28, 2009

Sometimes the Horse is the Zebra

I had too many doctors, too many egos, with no one willing to be accountable. I was trapped in the middle, passed from one specialist to another, but with no one looking at the big picture. Throw in all of the side effects no one liked to talk about and I was certainly caught in a nightmare with one end in sight. In the back of my mind, I kept thinking how much bad luck could one family have? Statistically, it didn’t make sense. If each event was a separate random event, the odds were astronomical. Could there be another explanation?

A few days after arriving home, I was getting out of the shower and noticed several long red streaks traveling down my spine. I called my daughter and asked if she noticed anything?

She said, “You mean those bright red streaks on your back?”

After the neurologist had questioned my ability to perceive changes, I could have kissed her. I may have! We got a camera and began taking pictures. We recorded the streaks on my back, my hands and the dark veins under my eyes that varied with my prednisone levels.

Then the phone rang, the call that would save my life. My neighbor was checking in on me to see how I was doing? When I described the week’s events, she quietly said, “The only time I ever had a headache like that was when I had manifestations of Lyme disease, I was sweating profusely and I remember my eyes were bloodshot. I’ll be right down.”

In her arms, she carried a brown box filled with files she had gathered over the years regarding Lyme disease. A newspaper article of a young man from Ann Arbor who told a similar story took my breath away because there was a photo of his hands. I gasped, “ Those are my hands!”

Sunday, September 27, 2009

Be Wary of Too Many Specialists

With the involvement of each new organ system, I gained another doctor: rheumatologists, neurologists, dermatologist, opthamologist, optometrist, extra trips to the dentist, vascular surgeon, gastroenterologist, internist, hematologist, orthopedic surgeons, ENT. Liv had an endocrinologist, pediatric opthamologist, pediatric orthopedic surgeon, pediatrician, pulmonologist, a bone geneticist and an ENT. It was not unusual for Olivia and I to have two or three appointments a week, between the two of us. The car was on autopilot: Petoskey, Traverse City, Grand Rapids, Detroit. The appointments alone would have made working impossible.
At a point where eating became nearly impossible, too, I called my internist who was out of the office for the day. A partner agreed to see me. After spending time discussing my case and examining me, he asked if he could be frank?

“You have too many cooks in your kitchen.”

I agreed.

“And each one is prescribing medications. You are getting limited benefits and all of the side-effects.”

I knew he was right. My case was fast becoming a hopeless mess of symptoms and side effects. I had just returned from a disheartening trip to Ann Arbor.

With my immune system repressed, I had been hit hard by a respiratory infection. Doctors added strong antibiotics, but I felt terrible. My energy level crashed. I was hypoxic around the lips and had strange tingly sensations up and down my arms. The night of my birthday, I was struck by an excruciating pain in the back of my head unlike any I had ever experienced. Within moments, I had the sensation of something breaking away and I felt a hot liquid sensation move down my spine followed by pain in one leg. We called Ann Arbor. Fearing vasculitis of the brain, the decision was made for my husband to drive me to University Hospital’s emergency room in the middle of the night. The doctors in Ann Arbor feared if I went to Petoskey, the transfer would take days of precious time and the risk warranted the trip. We dropped our girls off at my parents and I said good-bye, not knowing if it would be the last time I saw any of them. I couldn’t lift my head off the seat.

During the week that followed, I was in University Hospital’s ER three times. Over a dozen neurologists, of varying degrees of education, examined me. At one point, we were surprised when the doctor on call told us the rheumatologists weren’t sure I had Lupus. My husband and I were speechless. Lupus had been confirmed by their very own hospital in January, 2006. I had countless tests, an MRI and CAT scans. At one point, doctors felt I was severely dehydrated due to positional changes in my blood pressure. This didn’t make sense, as I was recovering from a spinal tap and had been drinking non-stop. The pain in the back of my head was excruciating and unlike any I had experienced with a migraine. Even my skull was tender. It hurt to lie on a pillow. If upright, I was in danger of passing out. My eyes were bloodshot and I was sweating profusely. The strange tingly sensations continued along my arms. I developed a spinal headache from the spinal tap that showed mildly elevated protein levels and a few wbc.

At the end of the week, I visited U of M’s top neurologist and rheumatologist. The rheumatologist was convinced the MRI of my brain did not show Lupus involvement in the brain and felt my symptoms were more neurological in nature. When one of the residents exclaimed, “But why is her ANA sky-high and climbing?!” No answer. The same intern asked about my CellCept levels being three times the normal amount? Again, no answer, only a stern look. I appreciated her attempt to find an answer. She had the makings of an excellent physician.

With no answers, I was sent to my neurological appointment, where I was told my headache was ruled a migraine variant and my symptoms were more unexplained autoimmune than neurological; unexplained autoimmune, complicated by the stress of caring for Olivia complicated by depression from my own chronic condition. I tried to explain I was not fighting depression and this was unlike any migraine I had ever experienced. The doctor did not feel it could be vasculitis of the brain because the MRI showed virtually no change from the year before. I shared my husband’s concern that we were running out of time to figure this out. He continued to smile and simply reiterated. When I showed him my hands, which had lost most of their muscle tone in a short period of time, he responded that was how I perceived my hands, though an emergency room doctor had expressed concern about earlier muscle wasting months before. I knew we were running out of time, too, and suddenly I realized these doctors were not going to figure it out. It hit like a ton of bricks. My prednisone levels had been raised to 60mg and my body was reeling from the shock. After holding my emotions together over countless appointments, I started to cry, seemingly adding fuel to the depression argument. I left the office reeling with anger, sorrow, and true fear. I hardly spoke as my Dad took me home, my thoughts spinning.

Saturday, September 26, 2009

Chocolate Chip Pumpkin Cookies


1 cup canned or fresh cooked/mashed pumpkin
1 cup organic sugar
1/2 cup cold expeller pressed olive oil
1 egg
2 cups organic whole wheat pastry flour
2 teaspoons baking powder
2 teaspoons cinnamon
1/2 teaspoon sea salt with trace minerals
1 teaspoon of milk
1 tablespoon vanilla
2 cups organic dark chocolate chips

Combine pumpkin, sugar, vegetable oil, and egg and stir. Dissolve baking powder in the milk and add to the mixture. Stir in flour and dry ingredients. Mix. Stir in vanilla then chocolate chips.

Bake in 350°F oven for 12 minutes or until done.

If you make from fresh pumpkin, be sure to roast the pumpkin seeds when baking the cookies. We like to soak them in a bit of Tamari and roast for about 20 minutes. They aid in inflammation.

Enjoy! : )

Thursday, September 24, 2009

Bad Things Can Happen to Good People III

I taught the day after Christmas break. After 17.5 years of teaching, it would turn out to be my last day. I went to my rheumatologist’s appointment after school and learned if I wanted to be there for my own children, I would need to take a leave from teaching. He felt it was time to seek the advice of more specialists. In early January, I had an appointment with the rheumatologists at the University of Michigan hospital in Ann Arbor. I went straight from my appointment to admissions, where over the course of the following week countless tests were run and rerun. By the end of the week, the following conclusions were drawn. I had elevated lactic acid levels and the doctors believed I was suffering from ischemia of the bowel. They also felt I had celiac artery compression syndrome which would require surgery, but my prednisone levels had been increased while in the hospital and the surgery would need to wait until they were lowered. Finally, they confirmed the lupus diagnosis.

When I returned to see one of U of M’s top vascular specialists six weeks later, he felt the celiac artery alone would not be causing my digestive problems and strongly suspected small vessel vasculitis. Surgery was no longer recommended and I began taking CellCept, an experimental drug first designed for chemotherapy patients. It was a strong anti-inflammatory and immunosupressant. It was also brutal on the stomach, a challenging drug for someone who was already having a difficult time eating.

I started with a half pill, 250mg and over the next several weeks gradually increased to 1500 mg twice a day. Because you could not eat two hours before and up to an hour after, my eating schedule became very regimented. I suddenly had a greater understanding for my grandmother.
I found that oatmeal helped ease the painful side-affects of some of my medications and those I was to take with food, I took with oatmeal. I found I could tolerate pumpkin cookies made from organic ingredients. Later, friends sent a pumpkin bread recipe that was also delicious. When nearly all food became extremely difficult to eat, these foods, along with SlimFast supplementation kept me going.

SlimFast? Why would someone 5’ 9”, who went from a size 12 to a size 4 drink SlimFast? I did get some strange looks in the grocery store, but the answer was simple. I couldn’t tolerate the taste of Ensure or Boost. I mixed SlimFast into Shetler's milk and could tolerate the taste. I supplemented twice a day.

To counter inflammation and further compromise my immune system, I stayed on higher dosages of prednisone, taking up knitting to combat the side-effects. I tested negative for SPRUE on multiple occasions and was not allergic to the common foods. This didn’t surprise me. It wasn’t that I couldn’t eat flour. I just couldn’t eat highly processed flour and it needed to be organic. I was especially sensitive to any fruit that was not organic, even bananas and watermelon. The more processed the food, the more difficult time I had eating it. I also had trouble with anything acidic. I had become very sensitive to additives and preservatives. As for meats, I could tolerate fish. In the early days, I was often doubled over in pain within minutes of eating. Some foods seemed to pass very quickly. Eating out was nearly impossible. Traveling, extremely difficult. I began to pack my own food wherever I went. It was too difficult to find food on the road. There would come a day when my body couldn't tolerate SlimFast and I was forced to turn to baby food to keep going, much to my daughters' horror. I am happy to say baby food is no longer in my diet.

Tomorrow, the pumpkin recipes...

Bad Things Can Happen to Good People II

At my first visit with the rheumatologist, I learned I had what is known as true Raynaud’s syndrome, identified by abnormal looping blood vessels at the base of my nails. This explained the discoloration and numbness that would appear when my hands and feet were exposed to the cold. My cheeks were pink and the doctor believed I had a faint malar rash. My joints were very loose and I was considered to have hypermobility syndrome. Pin pricks with a needle suggested some loss of sensation in my feet. An EMG was ordered of my legs and more blood work followed.

I tested positive for mixed connective tissue disease. I also tested weakly positive for antiphospholipid syndrome, suggestive of a potential clotting disorder, a possible explanation for the placental abruption in my third pregnancy. I began Plaquenil, which helped with my stiffness. I visited a hematologist to evaluate if an aspirin a day was adequate treatment for the antiphospholipid syndrome and he felt it was. At the same time, he noted a red lacy rash on my legs.

By August, the diagnosis of Lupus was added to mixed connective tissue disease.
My neurologist felt the daily headaches were migraines, though they were always in both temples, occurred daily, began with the other unusual symptoms, and were not nearly as severe as migraines I had experienced in my teenage years and also when I was pregnant with my first child. The loss of color vision was attributed to migraine auras.

By November, I was experiencing loss of feeling in parts of my face that would last up to two hours at a time. When walking across the gym at Mikayla’s school, I suddenly felt as though I had been stabbed with a knife in my abdomen. Appendicitis was ruled out and a series of tests run. Amylase levels were elevated. I had been experiencing early satiation after just a few bites of food since early in the summer, but suddenly, eating was a truly painful experience. The pain in the abdomen would start minutes after I began eating and would last for hours. A colonoscopy suggested potential vasculitis. I had a cardiac ultrasound that suggested possible clots in the heart.

Wednesday, September 23, 2009

Bad Things Can Happen to Good People

I learned this lesson early in life. As a three-sport athlete, in volleyball, basketball and track, I dreamed of competing at the collegiate level. Those dreams were destroyed along with my anterior cruciate ligament when I was clipped in the right knee while shooting a lay-up at basketball camp the summer of my junior year in high school. I worked hard at rehabilitation, but each comeback was marked by another injury. Finally, during the spring of my junior year, I landed in the office of orthopedic pioneer, Dr. Lanny Johnson, faced with the decision of continuing to compete or walking for the rest of my life. I chose walking, along with an ACL reconstruction using graphite fibers to replace my ligament.

Bad things can happen to good people, a life lesson for me. As I struggled to right my world at the age of 17, the president of student council/quarterback of the football team joined me at lunch to tell me how sorry he was. His kind words touched me. Little did I know, a few years later, I would return the favor after he suffered a similar injury, ending his collegiate career. I rebuilt my life, helping coach my senior year. The experience would shape the direction of my college education and my future career as a science teacher and coach.

Our daughters learned the lesson the day our youngest was born. Unfortunately, the lesson kept coming. Olivia’s early struggles with vision and rickets, were not her only challenges. She battled one respiratory infection after another. At eighteen months, we nearly lost her to respiratory syncytial virus, RSV. A child at daycare was identified with RSV and within 24 hours Olivia began coughing in a way we had not heard before. She visited the pediatrician the following morning. I was nursing her in the evening when she stopped breathing. We were a half hour away from going to bed. If it had happened later in the night, we might have missed it. I rushed her outside into the cold air while Chris called the ambulance. The shock of the cold air triggered her breathing and the ambulance arrived within minutes. Her oxygen saturation levels were in the seventies on the ride to the hospital as she struggled to breath. Upon examination in the emergency room, the pediatrician told us it was one of the most serious cases of RSV he had seen and she was admitted immediately. By the end of the week, she lay draped over my lap as we "camped" in an oxygen tent, turning blue whenever her oxygen levels dropped and sweating profusely. She had not said a word in days. When the pulmonologist and head nurse both expressed concerns about the need for a transfer, we listened and pushed for the transfer. They accompanied us in the ambulance to Helen DeVos Children's Hospital in Grand Rapids, though both of their shifts had ended. I was grateful for their care and support. Olivia responded well to the change in treatment and within 24 hours we knew she would make it.

The respiratory infections continued, each ending in an ear infection.

With all we were facing, it was not surprising that I was not feeling well. All of the trips to Detroit and Grand Rapids, along with teaching full-time started to take their toll. My dermatologist, noticed a strange rash on my forehead and ordered a biopsy that came back inconclusive for scleroderma. She asked, “How are you feeling?”

Tired was an understandable answer considering everything we had been through. I had never fully recovered from the bed rest and resulting caesarean. Months of stress, managing Olivia’s care had taken their toll. I was also teaching full-time: three science preps/five classes. I graded papers while I ate lunch at school.

Little things, each easily explained, started to pop up. A co-worker noticed I sometimes had difficulty breathing. My toes began to go numb. I started experiencing daily headaches. My body would get very stiff when I would sit, making it difficult to stand. Some days I would experience severe neck or back pain. I woke each morning with a sore throat. I lost my voice easily.

One of the strangest things happened while teaching. As I looked out in the room, it looked like the back of the room was in a fog, the colors muted. We were in the middle of construction in our building and it wasn’t unusual for a hallway to fill with dust or sometimes smoke. This time I couldn’t smell anything. I asked the kids if they noticed it? Some thought so. I called a custodian in and he quietly told me there was no fog in the room. I called Olivia’s opthamologist in Petoskey and went for a check up. I would later learn a loss of color vision occurs when your retinas do not receive adequate blood flow.

About the same time, I jumped over a small pile of snow in our driveway and was startled by a crack that sounded almost like a gunshot. I was even more surprised when I took a step and my ankle felt mushy in my boot. I had torn ligaments in my ankle, but unlike a typical sprained ankle, I had not twisted it. It happened just from the impact of landing. When I met with the orthopedic surgeon, in addition to the torn ligaments, he identified severe Raynaud’s syndrome in both feet that required medical treatment. I called my dermatologist, who asked if I could come right away.

“Kim, which ankle did you injure?”

It was not uncommon for a foot to stay blue for several days after a sprained ankle, but both were blue. “Kim, which ankle did you injure?”

We went through my list of oddities and a patch on my forehead was rechecked. It was time to order additional blood work. When it came back, my antinuclear antibody test, also known as an ANA, was positive and quite high. An elevated ANA often indicates autoimmune conditions. Something was up, something more serious than just being tired. An appointment was arranged with a rheumatologist as soon as was possible.

Tuesday, September 22, 2009

I am One Tough Cookie in my Wonder Wear

When Olivia began vitamin D supplementation we hoped her growth rate would improve. Before beginning supplementation, she was well below the lowest line on the growth curve, in a fraction of one percent. We hoped for a lengthening in her arms and legs that would improve her overall proportions. We also hoped she could make up some lost ground with her overall length.

As she approached the age of two, though her bones showed visible strengthening and her alkaline phosphatase levels were again within normal range, we faced sobering news. Olivia’s growth was leveling off. Our baby, at twenty-one months was 28 inches tall and weighed 18.5 pounds. We made the decision to begin growth hormone therapy on her second birthday.
There were very few times I cried during those first two years. The day we learned Olivia’s growth rate was slowing brought tears. There was no guarantee growth hormone therapy would work. If Olivia’s body was unable to make growth hormone on its own, supplementation could potentially be successful. If Olivia’s body already made growth hormone on its own, but didn’t know how to process it, the additional growth hormone would make no difference.

Olivia has an amazing spirit. We have often said, “The only thing tiny about Olivia is her size.”

Facing the world at a height of less than three feet is a daunting challenge for a spirit of any size. We prayed for the best.

I was very nervous when we received our training to begin injections. I had handled the contact lens without a problem, though I didn’t wear contacts myself. I had weathered the eye surgeries. This was, somehow, different. Fortunately, Chris had previous experience giving injections and took the lead. He was also very encouraging, insistent I learn, too. The Genotropin pen is designed to minimize discomfort and Olivia would later criticize flu vaccinations for not using a similar needle and technique. Fortunately, I mastered the technique and Olivia, after weeks of blood draws was an amazing patient. She would try hard not to wiggle, knowing it would hurt less if she didn’t and we had to promise not to breath. The same promises still hold true.

She once told me just before her shot, “I am one tough cookie in my wonder wear!”

We laughed and laughed and you know what? She is!

I once heard a physician at St. Jude’s say, “We would never wish a serious illness on a child, but those who survive grow up to be really cool adults.”

Olivia will be a real cool adult. Her sister Mikayla will, too.

Monday, September 21, 2009

The Attributes of an Outstanding Physician II

There were others who were instrumental in changing Olivia’s life for the better. We were told repeatedly, that she would experience catch-up growth by the age of two, but as I changed her diapers, I had a nagging feeling something was wrong. On hearing my concerns one day, my older daughter's pediatrician asked if she could take a look?

I undressed Olivia and she turned to me and said if it were her child, she would take her to a pediatric orthopedic surgeon. Later admitting she was unsure if she was seeing a rare form of dwarfism, she was certain something was very wrong. She arranged for the appointment within a month, just shy of her sixth month birthday. She took the time to listen. Olivia’s life would be forever better because of it.

The ortho ordered an x-ray of her femur. It showed a fine white line along the outside edge of her bone. He repeated the x-rays on a few more bones. All showed the same fine white line. There were also hairline fractures. He sent us immediately to the hospital for a full skeletal set of x-rays. Every x-ray showed the same pattern.

At the end of the day, he turned to us and admitted he did not know what was wrong with Olivia, but asked for time to think about it and to make a few phone calls. I knew we had another excellent physician, greatly appreciating his honesty and candor.

A few days later he called, asking us to take Olivia in for blood work.

“What are you thinking?” I asked.

“I think she has rickets.”

“Rickets!!” I exclaimed.

I remembered rickets from my freshman Biology class as the disease that results from a vitamin D deficiency. I was shocked!

When her blood work returned, her alkaline phosphatase levels were sky high. Dr. Forness’s hunch had been correct. She had a severe case of rickets. We were to discourage any standing and begin vitamin D supplementation immediately. She began weekly bloodwork to monitor her condition.

So, how did an infant in 2003 develop a disease hardly reported in the United States for decades?

As one specialist colorfully put it, “We had a train wreck in the making.”

What conditions led to our train wreck? Olivia, because of the severe intrauterine growth retardation caused by the insufficient placenta, ran a 70% risk of developing a metabolic bone disorder. I had been on bed rest through the summer, depleting my own vitamin D supply. Olivia’s skin is a bit darker because of her father’s Filipino heritage, increasing her risk even more. She had spent little time outside and when we did venture out, her car seat was covered with a blanket so we would not accidentally lose the contact lens. Finally, I was breast-feeding and our pediatrician had not seen the need for vitamin supplementation.

It turns out that breast milk is almost the perfect food. It has everything a baby needs with the exception of vitamin D. Olivia’s pediatrician sincerely apologized for the oversight, another important quality, and we began treating the deficiency with supplementation. Olivia’s alkaline phosphatase levels slowly came back into the normal range. An endocrinologist oversaw her treatment.

Her pediatrician sincerely apologized.

“I should have caught that. I won’t make the mistake in another child,” she promised.

Her sincerity was so very much appreciated, for isn’t that what most of us hope for? That we learn from our mistakes and in the life of a physician, that another child is spared the same mistake? I consider the courage to admit a mistake and correct it whenever possible, the gold standard in medicine. Ironically, if it happened more, I suspect there would be fewer lawsuits and healthier patients. Truly, what patients want more than anything is to get better.

To say her endocrinologist had a significant effect on Olivia’s life would be an understatement of immense proportions. He is an amazing man and an amazing caregiver. In his personal life, he battled diabetes and Parkinson disease with courage and grace and was an inspiration to his young patients and their parents. Though no longer actively seeing patients, I am sure his colleagues continue to value his wisdom for he is highly intelligent, a fantastic problem solver and leaves no stone unturned. He loved Olivia and the feeling was mutual.

He once told me, “We may never understand all of Olivia’s mysteries, but we should never stop trying.”

This final quality would ultimately save our lives.

Sunday, September 20, 2009

The Attributes of an Outstanding Physician

Olivia’s PHPV took us to Children's Hospital of Michigan in Detroit because our eye doctor told us our doctor was the best. I knew we were working with someone special when my phone rang at lunchtime. I was in the Taco House drive-through getting lunch and was surprised to see the doctor’s number. Our first appointment was a few days away. I excused myself and answered the phone. He was on his lunch break, in a similar drive-through and wanted to introduce himself and gather information on Olivia’s case.

When I would later see just how busy this man was, every day, helping kids to see, I would find that initial phone call even more compassionate and amazing. Upon examination, we learned Olivia’s eye condition was more severe than originally thought, along with two attributes of an outstanding physician, honesty and the ability to know your limitations. A outstanding physician has the confidence to ask for help when needed. Olivia's doctor, already one of the best, turned to one of the top retinal surgeons in the world for help.

The retinal surgeon did an amazing job removing Olivia’s stalk of blood vessels, but left the lens intact. We were puzzled because the defect in the lens blocked 80% of her vision when the pupil was dilated. How would she see? Three weeks later, it puzzled her other doctor, too, for the retinal surgeon's reputation was flawless. After examining Olivia, he asked us if we would mind having lunch at the hospital while he arranged for an afternoon appointment with his superior for another opinion. The lens did indeed need to be removed and Olivia was quickly scheduled for a second surgery.

This doctor had the confidence to ask for help while questioning the earlier decision of a highly regarded colleague. When facing a medical crisis, it is critical to remember that even the best can make a mistake. Doctors are human. The patient came before a reputation and Olivia had a better chance of seeing because of it.

Over the coming months and years, Olivia returned for several exams under anesthesia. Each time, a Children’s Hospital nurse would wrap her in a warm blanket when we would arrive early in the morning and Olivia would often fell asleep before anesthesia. I will never forget one exam under anesthesia in particular, the morning her doctor asked for the honors to carry Olivia to anesthesia, asleep in his arms. We are forever grateful for his courage, compassion and integrity and give thanks to both doctors for Olivia's miraculous gift of sight.

Friday, September 18, 2009

When the Alligator Opens II

She was a fighter from the beginning. Just fourteen inches long, Olivia surprised all by perfect Apgar scores. She was a 35-week baby trapped in a 30-week body. An ultrasound showed brain sparing, the body’s amazing ability to preserve its most important part during starvation. Nearly a third of the placenta was dead tissue and scar tissue, causing severe intrauterine growth retardation and shortened limbs, yet miraculously, Olivia’s brain was of normal size. She would spend the following weeks growing under the protective watch of the Neonatal Intensive Care team with the normal ups and downs of the life of a preemie. Her spunkiness served her well.

When she was a month old, a pediatric opthamologist discovered a rare birth defect. Olivia had a condition known as primary hyperplastic persistent vitreous (P.H.P.V.), a rare eye condition found in infants. Olivia's was particularly severe and unlikely related to her prematurity. P.H.P.V. involved a stalk of blood vessels that, during the early formation of the eye, provides a blood supply from the retina to the newly forming lens. In a normal eye, the blood vessels degenerate to the optic nerve and the lens clears. Olivia’s stalk of blood vessels never degenerated. If you looked at the pupil of her right eye, it looked like she had a tiny white daisy in the center. When we took her photo, she had “red eye” in just one eye, her right, every time.

She faced a quick flurry of appointments and surgeries at Children’s Hospital of Michigan and Beaumont. Timing was critical. The earlier the repair, the better the prognosis for vision because the brain must learn to see. The first surgery removed the stalk of blood vessels. Three weeks later, a second surgery removed the lens in the same eye. We were fortunate to have some of the best doctors in the world fighting for some semblance of vision in her eye in a race against the clock.

The night of her first surgery was the longest of my life. She was strapped face down to a board for the first twenty four hours after surgery. She couldn't nurse for comfort. To make matters worse, at one point in the night, there was a fire in the kitchen of the hospital and the fire alarms blared for what seemed like hours. I finally learned the trick of bundling her tiny four pound body, resting my hand on her back for comfort, and the two of us captured a few hours of much needed sleep.

It was also the surgery where I learned firsthand that you must stay close to your child in the hospital. Olivia's I.V. had been placed in backwards and she caught it on the board she was strapped to. A stream of blood pooled below her. My father ran for help and the nurses came running. I shudder to think what might have happened had we not been there, as tiny as she was.

When Olivia received her first contact lens at just shy of three months, she rode in her car seat, flipping her hand over and over, staring at the details of her hand. We knew we had made the right decision, preserving eyesight in her spare eye. It would never equal the other, but at least if something happened to her good eye, she would still be able to see. The doctors referred to it as her spare eye.

After a dozen trips to Detroit, Olivia’s eye was healing and her vision greatly improved by the contact lens. We had much to celebrate. I was understandably exhausted. The stress of the pregnancy had been hard on my body combined with bed rest meant I had underwent a C-section at the physically weakest time in my life. With no time to recover physically, I had moved straight into the rigors of the NICU: long hours, the daily stressors, the physical demands of nursing a preemie, slow healing and excessive bleeding wore me down even more. As the months passed, I was tenuously balancing a teaching career with an infant who required much attention, another who had just started school and one who was in high school. Yes, I was tired, exhausted even, but understandably so and I kept putting one foot in front of the other, moving forward.

When the Alligator Opens

It was 2004. We were just checking in to the Renucci Hospitality House at Helen DeVos Children's Hospital in Grand Rapids, Michigan. Our daughter, Olivia, then two, was there for another round of appointments with her specialists. After a warm welcome and quick hello to the fish in the aquarium, we headed to our room. "When the alligator opens, you go inside!" she instructed.

The elevator opened and in we went, facing the unknown head-on.

"How do you do it?"

I've been asked this question more times than I could count. No one asks for a situation like ours. You just take it one step at a time, one day at a time. Always making the best of the situation at hand.

Olivia had faced the alligator since birth, actually before birth. A partial abruption of my placenta left her literally starving in utero, the placenta crippled in its ability to provide desperately needed nutrients to her growing body. She was my third child. Her siblings, both preemies, brought warnings of her eminent premature birth. Despite bed rest, I experienced frequent contractions and remained small, warning bells of an impending problem. Yet the complication amazingly went undiagnosed until days before her birth, by Caesarean section, during her thirty-fourth week. She weighed just 2 pounds 9 ounces. A true miracle at the beginning of her journey and ours.