No one is sure exactly when 12-year-old Emma started feeling ill. She was not much of a complainer, so when her mother Karen went to her room and found her already asleep nearly an hour before bedtime, she simply smiled and thought how lucky she was to have a daughter like Emma.
The next morning when Emma did not come down for breakfast at her usual time, her mother didn’t think too much of it. Yesterday had been a busy day, driving all the way from Emma’s grandparents home farm in Maryland to western Massachusetts after a week of sun and beach and chores. Emma loved the chores; she loved the animals, what few there were now as her grandparents were getting on in years. But Emma still came every July for a month. That month spent among the fields and streams, and along the shore of Chesapeake Bay, would hardly hold her for a year. It’s not that she didn’t like her home, tucked into the hills of Stockbridge, or her school or her friends.
But there was just something about the farm: the smell of salt air, of homemade cookies on Saturday mornings. The cry of the gulls along the shore, the purring of the barn cat’s kittens, the breaking of the waves on the rocks when the wind was strong. Yes, she loved the farm, and truth be told, she would move there if she were given the chance.
Some part of her was glad to be home. Things were familiar here; comfortable. Her mother and father were here, and soon her baby brother would be too. The baby, to be named Jonathan, would be born around Thanksgiving, and Emma secretly hoped it would be before that. It was only the first of August.
By lunchtime Emma still had not come down, and Karen decided to check on her. Emma was awake and sitting up in her bed. When her mother asked if she were feeling all right, Emma remarked that her hand itched. She pulled her hand out from beneath the covers, revealing a slightly swollen and very red palm and fingers. “Does it hurt,” her mother asked. “A bit” came the reply, “but mostly it just itches.”
After Emma had eaten a late breakfast, she sat down on the floor to watch television. She usually played outside but said today she felt “tired, and my hand is staring to hurt,” so Karen said it would be fine to stretch her two hours of television time. Just for today. Emma surprised her parents when she went to bed after eating her supper and complained of being tired. Usually, she was willing to do anything to stay up for “just a few more minutes.”
By the next morning, Emma seemed her old self. She was up early, and after breakfast, she was out the door to her friend Mary’s house. Karen asked Emma how her hand felt, and was told it was better.
It was during the second week of school that Emma began to have pain in her hand again. The redness was gone, but the swelling seemed to be worse this time. In speaking with the school nurse, Karen learned that Emma “must have hit it while playing.” Unsatisfied with that explanation, Karen called the family doctor, Mark Valentine. A visit was scheduled for the next morning, and Emma, who usually loved school, asked to stay home until the appointment. Karen saw little point in forcing Emma to go to school, only to pick her up after a few hours.
Dr. Valentine had been the family doctor since Emma was four years old. A friendly family practice doctor with a quick smile and a warm demeanor, he was a favorite of many of the local families. When the nurse called Emma’s name, she hesitated, looking up at her mother and asking “I won’t have to have a shot will I?” Karen smiled and replied that she did not think so.
Dr. Valentine asked Emma about her hand. He could see it was swollen, but could find no bruising or discoloration. Karen said that it had been swollen some and red over the summer, but that it had gone away. A check of her blood pressure, a look in her ears and eyes revealed nothing out of the ordinary. Looking through Emma’s chart, he could find no evidence that Emma had done anything but bump her hand. Did she remember hitting anything? Did someone grab her hand or had she bumped it? “No” was her reply.
After she had left the doctors office, Karen thought that perhaps she should have told him about her headaches, or about how Emma was tired when she first came back from Maryland. But it was probably nothing. Emma was an active girl, and probably had bumped or banged her hand while playing. The school nurse was probably right.
After a few days, the swelling went down, and Emma no longer complained about her hand hurting.
It was just after Christmas, when she asked to hold Jonathan, now just five weeks old, that Karen noticed the redness had returned to Emma’s hand. Emma said it didn’t hurt “that much” and that she could still hold the baby. Karen asked Emma to squeeze her hand and noticed the girl had difficulty squeezing with any pressure. The next morning Karen made another appointment with Dr. Valentine.
This time, the doctor was immediately concerned. The chances of this being a new injury were unlikely. A check of Emma’s temperature revealed 100.8. Not high, but higher than normal. Checking the records, he noticed that the nurse had not checked Emma’s temperature during her visit last fall. Leafing through the chart, he noticed Emma’s temperature had always run slightly lower than normal at 98.2. This was nearly 3 degrees about Emma’s average, indicating a possible infection.
Examining Emma’s hand, Dr. Valentine noticed a small, hard lump. When he pushed on the lump it did not seem to move, and when asked, Emma remarked it did not hurt very much. He also noticed that, when he compared both of her hands, her right hand was noticeably swollen. He produced a Jamar Dynamometer and asked Emma to squeeze as hard as she could, first with the left hand, then with the right. Her left hand was able to generate 21 pounds of force. This was above average, but then Emma was very athletic and very strong for her age. With her right hand she was only able to generate 11.6 pounds of force. Dr. Valentine explained to Karen that most persons have some difference between strength depending upon dominant hand. Emma’s grip strength difference was significant. When Karen asked what this meant, Dr. Valentine said he did not know. There were several reasons for such a result, only one of which was muscle atrophy. It was more likely that the swelling in Emma’s hand was causing a great deal of discomfort.
When Emma was seated outside the small laboratory waiting for her name to be called, she fidgeted in her seat. She had been to this room before, and she knew they would take some of her blood. Although she did not want to admit being afraid, she was scared of needles and scared that she would keep bleeding after they took the needle out. She explained to her mother that “one of her friends at school had told her that a girl she knew had to have blood taken, and they couldn’t make it stop coming out, and she died.” Karen did her best to alleviate Emma’s fear, but in the end, could only stand next to the chair and hold her hand as they inserted the needle.
On the ride home, Emma kept an eye on the Band-Aid on her arm, just in case the blood started to run out. By the time they reached home, she seemed to have forgotten about it. The following Monday the nurse called from Dr. Valentine’s office. The blood test revealed no infection, and the medical advice was to alternate between heat and ice. Karen thought this was ridiculous, but did as requested. It had no effect.
A few days later Emma came to her parent’s room in tears. She was holding her hand and said that it “hurts so bad” she could not get to sleep. Karen applied a warm cloth to Emma’s hand and noticed the swelling has gotten worse. Now even her fingers were swollen, and she could hardly bend them without tears. As Karen pulled up Emma’s pajama sleeve, she noticed a red line, just under the skin, that went from the lump on Emma’s palm and went halfway up her forearm. Karen woke her husband, and they immediately drove to the hospital emergency room.
The emergency room doctor listened to the history from Karen, and upon examining Emma’s hand, immediately ordered an ultrasound. When Karen asked what this meant, the physician said that he believed Emma might heave something in the tissue of her hand that was causing the problems. The ultrasound showed nothing other than tissue, and an order was written for magnetic resonance imaging. A technician was on call, and the MRI was done that morning. On review, no foreign body was found. However, the lump was shown to be a cyst, and significant edema had spread to her fingers, and now her lymphatic system was bringing the infection to her arm.
As Karen and her husband listened, the doctor spoke of taking a biopsy of the lump, to properly diagnose the cause.
As Emma and her parents waited for a surgeon to perform a tissue biopsy, Emma noticed a man getting a cup of coffee and holding a teddy bear. Turning, coffee in hand, he noticed the small girl sitting with her parents. Walking over, he asked Emma if she liked his bear? When she nodded her head, he handed the stuffed animal to her, and she smiled. As he was about to walk away, he heard his name being paged. Looking around, he saw the nurse who had requested him and walked over to her desk. He was told there was an odd case involving a little girl, and his presence has been requested by the attending.
Emma and her parents were brought back into the examination room by the nurse only to meet the man with who had given Emma the teddy bear. She smiled at him. He smiled. “The bear seems happier with you than he did with me” he replied. He introduced himself as Doctor Burton. He told Emma and her parents that he was the hospital’s infectious disease epidemiologist and that he understood there was a little girl who needed to talk to him. At hearing this, Emma sat up in her chair. “Really,” she asked, to which he nodded. “Really.”
Looking at the intake notes from the previous evening, Dr. Burton asked when Emma had stared to have trouble with her hand? She replied that she couldn’t remember exactly, but she thinks it was around Thanksgiving. Looking at Karen, he asked if she agreed. Karen replied that she thinks it was the week after her summer vacation. Dr. Burton seemed to take an interest in this. “Where did you go on your vacation?” he asked. Karen replied that Emma spends the month of July with her grandparents in Maryland. Dr. Burton turned to Emma and asked, “What do you do when you are there?” Emma remarked that she played in the fields and helped her grandparents with the farm chores. He asked if there were any animals on the farm?
“Not many” Emma replied. Karen explained that her parents were retired, and while they still kept chickens, they had only one cow and one pig. By now physician had entered the room and was listening to the conversation. Dr. Burton looked towards the physician and “I don’t think it’s zoonotic.” It would have moved much faster. This is possibly the result of something that happened several months ago. The physician nodded in agreement. “What else do you do besides chores?” Dr. Burton asked. Emma replied that she played in the field, and walked along the shore. Do you go swimming he asked? “I never go swimming alone, she replied. But I like to watch the birds and pick flowers.” Dr. Burton asked what kind of flowers she liked to pick, to which Emma responded, “the beach roses that grow there.”
The epidemiologist sat back in his chair for a moment. What had he recently read about a similar case in a European Medical Journal? It seems there was an article about a French gardener who had gotten a severe infection from a rose thorn. As he tried to recount the article, it seemed to him the symptoms were similar. As he recounted in his mind, he came to see that the symptoms were nearly identical. “I think we may have a case of Sporotrichosis,” he remarked. “How sure are you of this?” asked Karen. “Pretty certain” came the reply. It all fits. A bacteriological infection would have hit much faster. A benign tumor would not have caused the fever or the inflammation. Looking at the physician, he asked, “Can you think of a more likely diagnosis?” The physician thought for a moment, then shook his head no.
The treatment was an anti-fungal medication called Itraconazole, and treatment would require taking the medication for several weeks. As Emma had not experienced any ulceration of the tissues, treatment would extend just one month beyond the cessation of symptoms to avoid a reoccurrence.
Emma began the medication after a needle biopsy had been performed. Ten days later the diagnosis was confirmed by fungal culture.
Sporotrichosis, or Rose Gardener’s Disease is extremely rare infection caused by the fungus Sporothrix. This fungus lives throughout the world in soil and on plant matter such as sphagnum moss, rose bushes, and hay.