How it all began...

Alex began to limp at the beginning of August 2005. He was twenty months old. We brought him to our clinic the first week of August. The clinic was unsure of what was causing his limp. He appeared to be healthy and there was no known trauma that could have caused the limp. The clinic took x-rays of his right hip and they also took a blood sample. Nothing was determined to be the cause of his limp during his visit. We were told that it could be a virus in his hip and that it should go away on its own. We were told that if it did not go away in two to three days to come back to the clinic. The limp persisted. We brought Alex back a week later in August. The second appointment brought more x-rays and still no answers. We were again told it was probably a virus in his hip (or maybe even a fracture) and that the limp would probably go away. We were told a second time to come back in a week if it did not. Well it did not go away, we brought Alex back in for his third visit and third set of x-rays in August. Alex was seemingly "normal" in all other aspects. At this visit it was decided to go forward with a bone scan, though the doctor still seemed confident it was just a virus in his hip or a possible fracture. The bone scan was scheduled for the next week at Minneapolis Children's Hospital. A few days later we received a call from Alex's pediatrician. We were told that the radiologist at our clinic found demineralization on his right hip bone (ischium) when he compared the x-rays from the last three visits. The doctor had spoken with an orthopedic specialist and Alex was now scheduled for an MRI instead of the bone scan. The demineralization could have been caused by a couple of different things. Again, overall he was a seemingly very healthy 20 month old boy. No one said it could be anything serious. The MRI The MRI was scheduled for September 8, 2005. This would prove to be the longest day of our lives. The night before the MRI I received a call from Gillette's Children's Hospital to inform me that the orthopedic specialist was not able to fit us in on the day of Alex's MRI. This upset me, as it was another delay in getting answers on Alex's limp. It had been originally set up for us to go to Gillette's Children's Hospital following his MRI at Minneapolis Children's Hospital. I was very disappointed that we were not able to be seen that day. I called Alex's clinic the morning of the MRI to ask for someone to help us get seen by an orthopedic specialist that day. Everything else went as planned in the morning. To our surprise, Alex did not seem to mind that he was not able to eat or drink anything. We were able to be with Alex through the entire process up to the actual MRI. It was hard to watch Alex be put to sleep in his Dad's arms. I thought that was the worst part of the day and was happy (at the time) that it had passed. It was during the MRI that I received a call in the waiting room from Alex's clinic. I walked to the phone happy that they were responding to my earlier message and that I was indeed going to be seeing an orthopedic specialist that day. To say the least, I was wrong. I was receiving a call from the clinic to inform me that they had found something unexpected in Alex's MRI. I was told to take a deep breathe and listen carefully. The doctor then said that they found what they thought were lesions in and on Alex's right hip bone and that we no longer were in need of seeing an orthopedic specialist. The clinic had already made arrangements for us to go to the fourth floor of Minneapolis Children's Hospital to see a Pediatric Oncologist. I did not know how to react. I could not bring myself to say those words out loud to Tom or to anyone else at that moment. I could not stop crying long enough to speak, I had thought the worst of the day was over when I kissed Alex on his head when he was put to sleep. I was wrong. Nothing can compare a parent for those words, nothing. After the MRI was complete a lot of people took a lot of time to speak to us. I do not remember much of those conversations. Alex was then brought to x-ray for a full set of x-rays, from top to bottom. We were then brought up to the forth floor to meet Dr. Richards. He explained that the lesions covered Alex's entire right hip bone and that they had found more lesions on his left shoulder. He also explained to us that there was a chance it was Langerhans Cell Histiocytosis (I had to have him write it down). He was concerned because it was not in usual or expected places and they were larger than expected. The next step was a biopsy. The doctor and the radiologist were very confident that whatever Alex had he would need some sort of chemotherapy, so it was decided to give Alex's the port-cath at the same time as the biopsy. This would reduce the amount of trauma we would need to put Alex though at this time. The biopsy and port-cath insertion went well. We again watched Alex be put to sleep and sent him off to surgery at Minneapolis Children's Hospital. It was a week later that we were scheduled to come in for Alex's first treatment and the same day he was officially diagnosed with single system mulitfocal Langerhans Cell Histiocytosis September 15, 2005. LANGERHANS CELL HISTIOCYTOSIS (from Histio.org website) What is Langerhans cell histiocytosis? Langerhans cell histiocytosis (LCH) is a rare disorder that primarily affects children. The disease was first described in medical literature around the turn of the century. Although physicians have written about the disorder over the years, it has only been recently that it has received much attention. A histiocyte is a form of white blood cell, which is found in every human body. Its job is to help destroy certain foreign materials and fight infection. For some reason, patients with this disease have too many histiocytes (Langerhans cells). These cells accumulate in certain areas and cause problems. The Association works closely with an international group of physicians, known as the Histiocyte Society, who are dedicated to studying the histiocytoses. Through this partnership more has been learned and better treatments have been discovered. Langerhans cell histiocytosis has also been known as: Histiocytosis-X Eosinophilic granuloma Hand-Schuller-Christian syndrome Letterer-Siwe disease There are also a number of other terms which have been used to describe syndromes which are considered to be Langerhans cell histiocytosis (LCH). These include reticuloendotheliosis, Hashimoto-Pritzker disease, self-healing histiocytosis, pure cutaneous histiocytosis, Langerhans cell granulomatosis, type II histiocytosis, and non-lipid reticuloendotheliosis. How many people are affected? It is estimated that one in 200,000 children are affected each year. Seventy-six (76) percent of the cases occur before ten (10) years of age, but the disease is also seen in adults of all ages. What tests are done? A diagnosis of LCH is usually made following a biopsy and microscopic examination of the affected tissue. To determine the extent of the disease and subsequent treatment plan, several other tests may be done. These include blood tests, x-rays of the chest, bones (skeletal survey), and CT scans. Sometimes a biopsy is performed of the liver or bone marrow. How is it treated? Treatment, if any, depends upon the individual patient. In some cases the disease will regress without any treatment at all. In others, limited surgery, small doses of radiation therapy or chemotherapy will be prescribed, depending on the extent of the disease. Treatment is planned after thorough evaluation of the patient to determine the extent of involvement. The goal of an overall treatment plan is to use as little treatment as possible to keep the disease under control, allowing the body to heal by itself. Is it cancer? No. Over the years, cancer treatments have been used in patients with histiocytosis. Consequently, hematologists and oncologists, who treat cancer, also treat children with Langerhans cell histiocytosis. However, the disease is not a cancer. Radiation therapy, if used, is given in much lower doses than that which cancer patients receive. Although histiocytosis is a disease of the immune system, it is not related to AIDS. Will patients recover from the disease? The vast majority of patients will survive the disease. Some may develop lifelong chronic problems,while others remain symptom free. In some cases the disease is fatal. Usually these are very young infants who have a rapid downhill course and do not respond to any known treatment. Physicians will be able to discuss the patient's likelihood of responding and doing well. Whether or not the disease responds to treatment will often depend on the extent of organ involvement. However, it is often difficult to make definite predictions, much to the frustration of the physician and family. What kinds of problems can be encountered? A patient can have very limited involvement in only one part of the body, or involvement in many different sites. Usually the disease is more serious when several sites are affected and the patient is a young infant. Possible sites of involvement include: Skin (rash) Bone (single or multiple lesions) Lung, liver, and spleen (dysfunction) Teeth and gums (loose or lost teeth, swollen gums) Ear (chronic infections or discharge) Eye (vision problems or bulging) Central nervous system (excessive thirst and urination = diabetes insipidus) General symptoms like fever, weakness, and failure to gain weight may be present. All patients do not have all of the above involvements. It is difficult, if not impossible, for a physician to say with certainty how each patient will respond to therapy. This uncertainty at diagnosis is very frustrating for the physician and the family. What causes Langerhans cell histiocytosis? The cause is unknown. It may be triggered by an unusual reaction of the immune system or by something commonly found in the environment. It is not a known infection or a cancer. It is not known to be hereditary. Patients do not catch it from anyone and cannot infect anyone else with the disease. Is help available? The Histiocytosis Association of America is an international group of parents, physicians, and friends in search of a cure. The Association's goals include public and professional education, patient and family support, and stimulation and support of research. Langerhans cell histiocytosis is considered an "orphan disease," which means that it strikes too few people to generate government support of research.

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