Baby Emma is two years old. To anybody she seems to be an ordinary toddler… happy, playful and active. What you can’t see is that she battles with a condition known as Hydrocephalus every day. Hydrocephalus is a condition in which the ventricles in the brain cannot properly drain cerebrospinal fluid from the head. The result is the swelling of the ventricles, which in turn places pressure on the brain. Some of the effects of this condition include headache, nausea, vomiting, sleepiness, and irritability. Brain stress is a direct result.
Because of problems with her mother’s health, Emma was born prematurely. Her early birth, at just 28 weeks of gestation, actually turned out to be a blessing. Unbeknownst to doctors, Emma was suffering from a grade 3 bleed in her brain which could have resulted in dire consequences. As it turned out, this bleed was the primary factor in Emma developing Hydrocephalus.
Emma spent the first 74 days of her life in the Neo-natal Intensive Care Unit (NICU) at All Children’s Hospital in Saint Petersburg, FL. At just four weeks old, she had her first surgery; a temporary shunt that the Neurosurgeons thought would allow her ventricles to open on their own. Unfortunately, her ventricles never opened and a more permanent shunt had to be inserted between Emma’s skull and skin. At 8 weeks old Emma underwent her second surgery.
After that Emma seemed to be in good health. Weeks later Emma was able to go home and appeared healthy for the next 6 months. She had the usual developmental delays associated prematurity, such as taking longer than average to sit up or to crawl. While difficult, she could catch up with therapy. Then on May 1st, 2009 Emma began showing signs of her first shunt malfunction such as sleepiness and nausea. At the hospital a Computerized Tomography (CT) Scan revealed that her shunt had tissue growth that was impairing the flow of fluid. Another surgery was necessary and had to be done right away, as her brain was already showing signs of stress. For the third time this sweet baby had to endure life saving surgery. And yet when she was wheeled out of surgery, she was sitting up on the stretcher smiling.
Once again, Emma’s recovery seemed to go well. Doctors at her post-op appointment said she was doing great. Shortly later, on June 23rd 2009, Emma again began to show signs of malfunction. This time her ventricles were even larger, indicating a severe blockage and chance of worse damage. She was rushed into her fourth surgery. The shunt malfunction was resolved and she spent a couple of days in the hospital and was sent home. A month later, Emma celebrated her first Birthday with big Sesame Street party featuring her favorite muppet, Cookie Monster.
A week after turning one Emma was back at All Children’s Hospital with yet another shunt malfunction. Surgery number five was successful and after another week in the hospital Emma went home again. Recovery was followed by Halloween, she was a Princess Fairy. She celebrated Thanksgiving with the whole family like any other baby.
A week after Thanksgiving Emma once again started to show signs of shunt malfunction and her family prepared to endure the cycle of surgery and recovery once again. Emma underwent surgery number six immediately, but this time the initial surgery was unsuccessful. Surgery seven was successfully completed on December 5th, 2009. For Emma and her family, once again things again went back to “normal.” Emma’s family celebrated a heartfelt Christmas, acknowledging God’s role in Emma’s life. After all there could have been no other explanation on how this little baby could endure so much and still enjoy every child’s glee at opening presents on Christmas morning.
Emma started walking on January 1st, 2010. She “graduated” from her speech and physical therapy, and really began to thrive. She was growing and gaining weight and beginning to flourish. Sadly, on February 2nd, 2010 Emma again went to the emergency room for another shunt malfunction. She is older now and is beginning to understand what a trip to the hospital means. For surgery number eight she went into surgery screaming and reaching for her mom. She no longer comes out of surgery smiling but weary.
On March 5, 2010 Emma experienced yet another shunt malfunction, this time resulting in two separate surgeries on the same day. Number nine was done in the early morning with even the doctors unsure as to the outcome and uncomfortable doing the surgery. With so few options, they proceeded. Less than 24 hours later they were forced to perform surgery number ten, another shunt replacement. This time they were also forced to place a second shunt into Emma’s tiny head. While this increased effectiveness, it also increased chances of failure.
On July 23, 2010 Emma celebrated her 2nd birthday with friends and family. Just like any other child, Emma laughed played and even dressed up as her favorite Sesame Street Character Cookie Monster. All in attendance remarked on how well she seemed to be doing. Early the next morning, Emma’s mother found her lying in her crib dazed and covered in her own vomit, sure signs of a severe malfunction. Emma’s family rushed her to All Children’s hospital and were given the now familiar diagnosis and treatment: A shunt malfunction requiring surgery. Surgery 11 was performed shortly thereafter.
On Sunday July 25, 2010 Emma was still not acting normal. An MRI showed that the shunt was not working, requiring surgery 12. During the twelfth surgery, the doctors noticed blood in the ventricles, a bad sign. Because of this, she was given an external shunt which drained the fluid into a bag attached to the bed. She was forced to wear splints on her arms to prevent her from grabbing at the tube that now protruded from her head. A vigil was kept to ensure that the bleeding did not worsen. Eventually, the cerebrospinal fluid lost the red tint that comes with blood in the ventricles. Emma underwent surgery 13 to replace reinsert her shunt and tubing.
Later that year, Emma enjoyed another great Halloween and Thanksgiving full of great times with Family and Friends. On November 30, 2010, Emma again started showing the same signs of shunt malfunction followed by surgery 14. She went home for just a few days and was back with another malfunction on December 5th, 2010. Another surgery (15) was done and again she was sent home. She had a great time at the annual family Christmas hay ride, playing with all the kids. On December 19th of 2010, she again was found in her bed unable to sit up and covered in vomit. This time at the hospital, doctors found not only a malfunction of the shunt, but also an infection in her brain. Emergency surgery (16) had to be conducted in order to remove one of her shunts and insert another external ventricular drain in order to drain the infection. This was followed with a regimen of very strong antibiotics. She spent Christmas in the hospital with this dangerous infection slowly draining into a bag attached to the side of the bed.
A couple of days later, doctors inserted a new shunt back as a part of surgery 17, this time hoping for better results. She went home on New Year’s day for a total of one and a half days. On the 3rd of January she returned to the ER showing the usual malfunction signs. Surgery 18 was performed in a matter of hours. She was sent to a room on the usual Neurology floor, however, after a few hours Emma’s neurological status began to decline. At 2am on the 4th, doctors called in the On-Call Operating room staff and emergency surgery 19 was performed early that morning. Again she was sent home with a “clean” bill of health. This time, she made it three days. She was quickly brought back to the ER with those all too familiar signs. Doctors again had to give the devastating news of another shunt malfunction.
She was rushed into surgery number 20. She not only had to have a new shunt but also a device known as a “Power-Port”. The “Power-Port” is for the easy insertion of intravenous (IV) lines . Because of her medical history, the IV team has very few places and a very difficult time inserting an IV, while causing much pain and stress for Little Emma. The port is inserted under the skin in her upper chest. With this new port they simply stick the IV into the port under her skin and its done. She was finally sent home after a few days. As the family always says: “Maybe this will be her Lucky Number.” She is currently doing very well…She’s really quite a Miracle!
The truly sad thing about this whole story is that Hydrocephalus has been overlooked and ignored. The federal government allots less than 1 million dollars per year on research to improve treatment and attempt to cure this condition. The only known treatment is neurosurgical, and has barely changed since it was developed in the early fifties. Over 40,000 surgeries are performed each year in the United States, averaging 1 surgery every 15 minutes. Hydrocephalus affects people of all ages from infants to the elderly. One to two in every 1,000 babies is born with Hydrocephalus.
Hydrocephalus is also the most common reason for brain surgery in children, and yet there is still very little funding for research into a cure for this chronic condition. Even after treatment, many people face repeated surgeries resulting in diminished quality of life as in the case of little Emma. These are the sad facts that most people in the US are ignorant of and will remain so without greater exposure.