Jennifer Writes about Her Son's Journey with Ewing's Sarcoma

The Bike

It sat in the garage, unused for years. This shiny, silver, blue and black bike, still with training wheels attached, beckoned a rider.

We gave this bike to our then four-year-old son, before he became ill, very ill. He rode the bike in nice, outdoor weather, up and down our sidewalks, very excited to be gaining speed. As our son is tall, this was a pretty big bike for a four-year-old, far outstretching his previous tricycle. He proudly wore his bike helmet that matched his bike, with a big grin on his face. He felt very grown up on this big silver bike, shunning the small orange trike that used to cruise around the neighborhood.

Several months prior to Kevin’s fifth birthday, he began experiencing leg and hip pain, fever, decrease in appetite, and lethargy. We took him to the pediatrician, who immediately had him admitted to our local hospital and an orthopedist took over Kevin’s case. They diagnosed him with osteomyelitis, a bone infection. Kevin was on intravenous antibiotics for a month and had to limit his activities, including running, jumping, and riding his beloved bike. It is very difficult to strip these activities from a young boy. Kevin didn’t usually walk -- he jumped, ran, and moved quickly to get from point A to point B. After having his line removed for the antibiotics, Kevin was granted his freedom again. However, pain took that freedom away. It wasn’t until several months later, two months after his fifth birthday, that we learned Kevin did NOT in fact have a bone infection. Instead, Kevin had bone cancer, a rare form of cancer called Ewing’s Sarcoma. Kevin had a massive tumor in and around his right pelvic bone that had literally blown holes through that bone, opening it up from the inside out. It was truly incredible that Kevin still had the ability to walk. Even though the pain was at times very intense, Kevin would push through the pain, and continue to forge ahead. The surgeon was fearful that Kevin would cause further damage to his bone, as well as spread the cancer, if Kevin were too active. Restrictions were reinstated. Kevin was still allowed to walk, but no running, jumping, or any other activity that may jolt that very damaged bone. The shiny silver bike was idle, stored safely in the garage.

Discussions with the oncologists were painful, to say the least. Our beautiful, active, 5-year-old boy would have to undergo a year of very harsh chemotherapy. Also, he would have to have some form of local control – either surgery or radiation. At first we were told our options were not good, not at all good for a young child. The only apparent surgical option was a hemipelvectomy, which required removal of the entire right pelvic bone and right leg, as well. No prosthesis could be attached without a base. With the pelvic bone gone, there would be no base. In fact, Kevin would lose the ability to even sit up straight, as even the "sit bone" on the right side would be removed. Our other option, radiation, came with significant risks and eventual disability, as well. Kevin’s entire right pelvic bone would have to be radiated, as the tumor was all-encompassing. Radiating that bone would then leave Kevin with a 5-year-old sized pelvic bone for the rest of his life, as radiation retards bone growth. It also lowered Kevin’s survival chances. What kind of decision would we have to make for our son? Have him awake from surgery with a major part of his body gone, or let him deal with chronic pain and eventual disability from the radiation? As I would pass the bike in the garage, it brought me great sadness. Kevin may never be able to ride the bike again, let alone walk, all in attempts to save his life from this awful disease.

Thankfully, our prayers were answered and we never had to make this decision, as Kevin’s tumor responded quite well to the induction phase of chemotherapy. The tumor had shrunk enough for a not so radical surgery to be performed. The surgeon was able to remove enough of the pelvic bone to remove the cancer, but also leave enough of the bone there to attach a bone graft and allow Kevin a more "normal" life. The bike remained in the garage and was no longer such a symbol of sadness.

Surgical recovery was long and arduous. Kevin was to be non-weight bearing for a period of 6 months. He was still undergoing chemotherapy during this period, and not much healing was expected. Kevin’s bones thought otherwise and healed before the six-month time frame. At five months post-surgery, Kevin could begin to walk again. Long-term physical therapy began. A need to have a lift designed became apparent, as the growth of the right pelvic bone was far outstripped by growth on the left side, particularly after Kevin’s chemotherapy protocol had been completed. It appeared that Kevin had a leg-length discrepancy due to the pelvic surgery, even though his legs were the same length. Also, as many of the muscles surrounding Kevin’s pelvic bone were partially resected that the need for strengthening the remaining muscles was tremendous. The bike was still in the garage, collecting a thick layer of dust by now.

On a beautiful, warm April Sunday afternoon, a miracle happened. The bike’s dust was cleared, and our beautiful six-and-a-half-year-old boy was back on the bike. The seat needed to be raised and another helmet was necessary, as Kevin grew so much since his last bike ride. He began pedaling and the grins returned. He appeared a bit awkward, as his right leg leans inward as he pushes the pedals, but he did it. He rode. All afternoon, with several "rest breaks", Kevin rode that shiny silver bike. Neighbors drove by, smiling and cheering, knowing what it took to get Kevin back on that bicycle. He was tired that night, but so happy, so proud of himself for being back on that bike. The shiny silver bike now sits on our front porch, waiting for its owner to return from school and ride it once again. We hope it never collects dust, ever again!

So, what does it feel like to have a child diagnosed with cancer?

That was a question, I thought several years ago, that had an unimaginable answer. In 2003, our very active 4-year-old boy began limping, having some pain in his right leg and hip, was feverish, and acting lethargic and unwell. The pediatrician immediately suspected a bone infection, osteomyelitis, and had our son promptly admitted to our local hospital and referred to an orthopedic specialist. After 4 days in the hospital on IV antibiotics, we returned home with home health care and continued his IV antibiotics there. I literally was shaking after our return home, and commented to my husband, "how do the parents whose kids have cancer do it?" I knew a bone infection could be serious, but its treatment only lasts a month, then it was back to normal life. Or would it be?

Five months after our son's treatment for osteomyelitis, subsequent misdiagnoses, and requests for referrals to other doctors, Kevin's pain was becoming increasingly unbearable. We finally headed to Texas Children's Hospital in Houston to see a pediatric rheumatologist. At that point, the doctors believed Kevin had developed a rheumatic/rheumatoid condition. It was there where we finally learned that there was no infection, there was no rheumatic condition, but there was, in fact, a very large mass that literally engulfed and burst through Kevin's right pelvic bone. The radiologist and physician thought it could be a malignant tumor. What? Had we heard correctly? From reading about juvenile rheumatoid arthritis, we thought we'd be looking at long-term aspirin therapy. But, cancer? No, that couldn't be it. The pediatric rheumatologist was explaining to us that it was probably Ewing's Sarcoma, and that treatment usually involves some sort of amputation, but that you couldn't amputate a pelvis. What? She also was explaining that since it had been 5 ½ months, the cancer may have already spread across his abdominal wall and possibly into his lungs. What again?

The senses shut down. We were numb. Kevin had fallen asleep. David and I were lost in a fog. We had enough sense to ask the doctor whether we should stay in Houston for treatment. Would we be all right closer to home? Where should we go? What should we do?

The doctor answered those questions quite well. She said we should go back home and be with our other two children. There is a fine institution in Dallas, Children's Medical Center, affiliated with a major medical school, UT Southwestern Medical School – a necessity for childhood cancer treatment. Kevin needed to be treated by a multi-disciplinary team. The rheumatologist called Kevin's pediatrician while we were still in the office. She told the pediatrician what tests were necessary to confirm Kevin's diagnosis, and sent us on our way. She gave us her card, and sadly told us that she wished this were rheumatoid arthritis, because she'd be able to help us then. She told us to pray for an insidious infection, which was an unlikely alternate diagnosis. What again? Pray for an insidious infection?

The drive back to Dallas was another numbing experience. We needed to call the grandparents, but did not know what to tell them. It was all too much to comprehend. We couldn't comfortably talk about what lay ahead with Kevin sitting right behind us in his booster seat. We received a call from the pediatrician in Plano, who let us know that Kevin's MRI had been scheduled, then we would head straight to Children's for further testing. Our heads pounded. It was still too much to take in.

During the 24-hour period that had Kevin being sedated for an MRI, blood work, a CT scan, x-rays, and other testing, we had never met so many doctors. First, there was the orthopedic surgery group who said they wouldn't even touch this – they did not handle bone tumors. An orthopedic oncology surgeon would be best at performing the biopsy without disrupting the surrounding tissues. Next, was an infectious disease specialist. She came in and said she did not believe, at all, that this was an infection. Our hearts sank. Finally, the pediatric oncology group came in. They did think, most likely, that this was a malignant tumor. They believed that the tumor was "inoperable" and that Kevin would require a year of treatment – chemotherapy and radiation. A year of treatment, OK, then after that everything would be fine.

Sleep becomes a distant memory when you hear the words … "inoperable", "needs to be performed by an orthopedic oncology surgeon" … and these words are being uttered about your child. After the biopsy, we were told by the surgeon that this was a high-grade malignancy, but he didn't know what kind of cancer, yet. We would have to wait about a week for the pathology report to be completed. In the meantime, we were to sign more consents, allowing an oncologist to perform a bone marrow biopsy and allowing another surgeon to place a port-a-cath, a central line through which Kevin could receive his chemotherapy, blood transfusions, etc. More fog, more confusion, many tears flowing. It was obvious to the grandparents when we emerged from the consultation room what we had been told. Phone calls needed to be made; arrangements needed to be made. How could we tell our daughters, Michelle, a 12-year-old, and Laura, an 8-year-old, that their little brother had cancer? How could we tell anybody?

We told the girls, without hesitancy, that their brother had cancer. Michelle wept, and Laura sobbed. We asked one of the senior pediatric oncologists to help us explain all of this to both of the girls. She was wonderful and reassured the girls that they did nothing to cause their brother's cancer, nor would they "catch" cancer themselves. She asked if they fought with their brother. When they finally admitted "yes", she wanted to know if they would continue to fight with him, now that he has cancer. Laura responded with "Oh, no." Then the doctor said, "Oh yes, you will. You should continue to treat Kevin as you always have – after all, you don't want your little brother becoming a spoiled brat, do you?!" They laughed, we were relieved, and I will never forget that conversation.

This experience shakes you to the core. What I had always believed, what had been a rock in my life when things like jobs and relationships weren't going as well as I'd hoped, has been, "well, at least I have three healthy children." Those words no longer rang true.

After the biopsy, we accompanied our son on his stretcher down a hallway through a set of double doors, under a sign proclaiming, "The Center for Cancer and Blood Disorders." What was this surreal world we were now about to become a part of? How does this happen to a 5-year-old child? Why couldn't this have been one of us? What did we do to our little boy that made him so vulnerable? Was it something during pregnancy? Should I have taken those hormones to sustain my pregnancy with Kevin? Was Kevin exposed to something toxic or power lines or something else that caused this? WHY?

You soon learn that there are no easy answers to childhood cancer. Very strange things become routine. In that Center for Cancer and Blood Disorders, we saw bald children, constantly tethered to IV poles. Some looked radiant and happy, while others looked ill and miserable. Our son would become one of these children.

People have asked, "how do you stay so strong?" I don't. I do what I have to do each day to help our family get through this crisis. I could have curled up in a ball, lying in a corner, but how would that help Kevin? How would that help our girls? So, we went on, many times on automatic pilot, with adrenalin being the force that got us through.

After the type of cancer was confirmed, Ewing's Sarcoma, we met with the oncologist who specializes in bone tumors. He explained the course of treatment, the five chemotherapy drugs that would enter our son's system over a year's time. He would be admitted to the hospital for each round of chemotherapy. Also, quite often, he would be admitted in between rounds of chemo because of fever and low white blood cell counts and would require intravenous antibiotics to control potentially life-threatening infections. The "local control" treatment decision would have to be made – surgery or radiation – and neither were good options in Kevin's case. We were told of certain disability. We learned the potential side effects of the chemotherapy and the odds of Kevin becoming a victim to each one. Surely, Kevin won't have any of those side effects. What were we going to have to do to our child to give him a chance of surviving this cancer? What else could we do?

The year of treatment had its many ups and downs. It is truly a nasty roller coaster ride for which we never bought a ticket. The doctors, nurses and staff were wonderful throughout. I have no idea how they do what they do, but they are expert at explaining things, extremely compassionate, and very concerned about these children. I am still in awe of them – doing what they do, and somehow managing smiles on their faces, while children still lose their battles.

Guilt becomes a constant feeling. I'm spending so much time with Kevin, but so little time with the Michelle and Laura. My husband and I rarely see each other – one is at the hospital with Kevin and the other at home with our daughters. It has to be that way. There has to be some sense of normalcy and routine for the girls. They must go to school and continue with their activities, even though "normal life" has virtually halted for their younger sibling.

Some of all of this is a blessing. Hugs are so much sweeter, time with the children is so much more precious, and simple things become triumphs. Our son had major pelvic surgery in January, 2004, to remove the diseased bone, tumor, and surrounding tissue. He was to be non-weight bearing on his right leg for a period of 6 months, still going through chemotherapy. Walking once again was a HUGE triumph. The physical therapist was working with Kevin in the hallways of the oncology clinic. Staff members happily watched, and a few had tears in their eyes. At one point, it had been believed that Kevin may never walk again, but here he was — using crutches, tethered to his IV pole, and walking! It was glorious!

We also learned of the kindness and generosity of others. People, both familiar and unfamiliar to us, helped our family in innumerable ways. We had meals delivered to our home, our house was cleaned once a week, laundry was done, the dog was walked, and the lawn was mowed. Our girls were being cared for, on many occasions, by good friends and neighbors. We had others raising money for us to help with our ever-increasing medical expenses. We were honored and humbled by everyone's kindness. That was a beautiful blessing pushing past all the misery.

So, how does it feel? The emotions are raw and ever-changing. A milestone is passed and another hurdle lies ahead. Will we ever be comfortable again? Will we ever truly believe our son is cured?

Kevin has been cancer-free since his treatment ended in September, 2004. He has, however, been diagnosed with several of those "side effects" we thought would never happen to our son. Most are rare, some were not even mentioned at first, but all are a reality now. Kevin has many orthopedic challenges that lie ahead. His right pelvic bone is not growing, so there will be more surgery in his future. Kevin has intermittent pain, walks/runs with a limp, and has a hard time keeping up with his peers in simple activities, such as a game of tag. He has experienced partial, permanent hearing loss, and wears a hearing aid at school. Just weeks after Kevin's cancer treatment was completed, he was diagnosed with cardiomyopathy ("heart muscle disease") brought on by one of the chemotherapy drugs. He is followed by a cardiologist, takes heart medication, and may require a heart transplant at some point. The hurdles keep presenting themselves, but Kevin crosses them and goes on. He keeps us going. Thank God for that.

We are grateful, so grateful that our son is still with us. We are thankful for all the blessings bestowed upon us. We are hopeful that we will never hear the word "relapse." We are hopeful, too, that a cure will be found for childhood cancer … a real cure, with no strings attached, no potential long-term side effects, and nothing to worry about in the future.

Unexpected Effects

So many times, we’ve been told that we should be grateful that our son’s chemotherapy protocol has come to an end. We are. However, everyone unfamiliar with cancer assumes that once treatment ends, the patient is fully recovered and can resume a perfectly normal life. We wish.

When our 5-year-old son Kevin was diagnosed in September of 2003 with Ewing’s sarcoma of his right pelvic bone, our world was rocked. Somehow, someway, we all made it through a year of grueling chemotherapy, pelvic resection and bone graft surgery, a staph infection, numerous blood transfusions, five months of non-weight bearing on his right side, etc. We were also under the assumption that once treatment was finished, we would be able to relax, breathe easier, and enjoy life. We wish...for Kevin’s life is far from carefree.

We knew surgical recovery would be long. Once again, we are grateful that our son’s wound and bone graft healed while he was on chemotherapy...highly unusual we have been told. Kevin walks with a limp, has sporadic pain, and is undergoing physical therapy in attempts of strengthening the muscles that had been partially resected around his pelvic bone. He becomes very discouraged when trying to play a simple game of tag with his friends … he just cannot keep up. While we are thrilled that Kevin can walk, we are saddened by his limitations. This once very athletic little boy tends to sit on the sidelines, at times looking very defeated.

In the spring of 2004, we began noticing that Kevin was always turning the volume up on the television, and sometimes did not hear us when we called him. We were never sure if he was just tuning us out...after all, we had spent a great deal of time together during Kevin’s treatment, and maybe, he was just tired of us. He would also call for us from his bed – this was during the "non-weight bearing period." I would always answer with "I’m coming," but he seemed panicked by the time I arrived. Apparently, he never heard me. I brought this to the attention of Kevin’s oncologist, and he ordered an audiogram. We learned that Kevin has suffered permanent, sensorineural hearing loss associated with the massive doses of intravenous antibiotics he had been put on during times of fever and neutropenia. He has been tested again, and his hearing loss was slightly worse – in the mild to moderate category, still not too bad. Recommendations from the ENT specialists are that Kevin should continue to have his hearing tested every 3 months. They have already recommended preferential seating for Kevin in the classroom, and have said that Kevin may eventually require hearing aids. The assumption is that Kevin’s hearing may very well worsen over time. While this is certainly not as devastating as a cancer diagnosis, it is yet another obstacle our once healthy son has to overcome. As with everything with Kevin, we are told that this side effect is "highly unusual," since the blood levels of the antibiotics he received were monitored very carefully.

It has also been revealed, on regular scans, that Kevin has several nodules on his thyroid gland. We do not know the long-term implications of these nodules. Recommendations from the endocrinologist are that Kevin have his thyroid function tested regularly, and have an ultrasound of Kevin’s thyroid gland performed every 6—12 months. Kevin will be having another ultrasound in the coming weeks. The presence of these nodules probably has nothing to do with Kevin’s treatment, nor his cancer, but it is something else that will need to be watched.

Our most recent, and serious, scare has come from a diagnosis of cardiomyopathy. Kevin’s clear scans in October, 2004, came along with something else "to discuss." Kevin had already had a poor echocardiogram after just two treatments of doxorubicin. That drug was suspended for a while, but as Kevin’s oncologist let us know, doxorubicin is the drug of choice against Ewing’s sarcoma. We were all hoping that Kevin’s tumor would be 100% necrotic when he had his surgery. Unfortunately, it was not, so the need for doxorubicin was still present. Kevin received three more rounds of doxorubicin, and his echocardiograms had returned to normal. However, his first echocardiogram following the completion of chemotherapy revealed cardiomyopathy—a weakening disease of the heart muscle, and most specifically, the left ventricle in Kevin’s case. The very day we learned of Kevin’s heart condition, he had started to experience shortness of breath (and this was before we had seen the doctors that day). Kevin has been referred to a pediatric cardiologist, as he now becomes a long-term cardiac patient. The cardiologist will most likely put Kevin on heart medication at some point, which will work for a while, we were told. Presently the medications available only work for a limited period of time, and no one knows how long they will work for Kevin...after that, a heart transplant may become necessary. Almost unthinkable, given everything our son has already gone through. Once again, this is considered to be a "highly unusual side effect" of chemotherapy treatment. Only about 5% of those on doxorubicin show signs of heart problems – most are revealed many years following treatment, and only a handful of the heart problems ever become severe enough to warrant a heart transplant.

Another common assumption from those unfamiliar with cancer is that once treatment is completed, the patient is cured of their cancer. God knows, we hope this to be true. Because of Kevin’s intermittent pain and increasing limp, he had another bone scan, earlier than originally planned. The scan revealed a few "hot spots" on his pelvic bone—very scary in the world of bone cancer. The good news is that Kevin’s oncologist and orthopedic oncology surgeon both believe that those spots are due to inflammation, as Kevin is so much more active than they ever thought was possible. They will continue to watch, however, and closely monitor Kevin’s complaints of pain. Statistics-wise, we can hardly rest easy. Chances of relapse are far too great for us to feel relief. I hate being in this place, and wish I could resume being an easy-going mom, but it’s just not that simple. However, in spite of all of these "unusual" long-term side effects and our ongoing fears, our funny, adorable, little boy is still with us, and we will be forever grateful for that.