Meet Kayla, a blonde haired, blue-eyed little girl with a gigantic smile, a tender heart and a disorder so rare that she is the only child exactly like her in the entire world with her particular form of the disorder. This rare nerve disorder is called Hereditary Sensory Autonomic Neuropathy (HSAN). Her body does not signal her brain to regulate her body temperature and she cannot feel pain unless it is extremely intense.
Most recently, Kayla was diagnosed with another rare problem completely unrelated to her HSAN. In March 2006, after over a year of severe headaches, doctors found a malformation in the lower portion of Kayla’s brain. As a result, her brain was growing into her spinal column and to avoid long-term damage and paralysis in September of 2006, Kayla had brain decompression surgery.
She will never be able to play sports, run at recess, or participate in most other normal childhood activities. But, she is surrounded by a loving family and supportive network of friends who always remind her of the things she CAN do. This is her story…
Remember the joys of childhood? Walking around without shoes and socks with the grass tickling your feet? The sun shining on your face as you swing at the park feeling as if you could soar? Running the bases in a little league game with a dream in your heart to one day become a champion? Imagine if you were a child watching this through a window because it was too dangerous for you step outside in the middle of a warm summer day….
THE FIRST SIGNS
Dr. Carlos Anaya was a wonderful pediatrician who had a strong desire to discover the root of Kayla’s sensitivity to heat. At the age of 14 months, Kayla went through a heat test at the local hospital. They placed her in a room that was 85 degrees and after about five minutes she was bright red but she wasn’t sweating, an atypical response to heat. That night, when the Woodhouse family arrived home from the hospital, Jim Robinson, a man from their church, brought them copies of Internet research he had done. Ironically, Jim was a former submariner and part of an organization called CAUSS – a group of retired submariners who donated to organizations that helped children who had to live in a submarine type environment. Jim was familiar with the HED Foundation (later the Sarah Moody Foundation) – a resource for parents of children with Hypo-Hydrotic Ectodermal Displasia; a disorder in which children are born without sweat glands. Sarah Moody was the founder of the HED Foundation who as part of their mission provide funds for special “cool vests” for children who need them to survive in warm environments. Unbeknownst to the Woodhouses, Jim had already put Kayla’s name in for a vest. Two weeks after his request, and a year before her correct diagnosis, Kayla received her first vest and a phone call from Sarah Moody. The vest offered Kayla freedom. It protected her from overheating when she was inside and provided her the opportunity to play outside for short periods of time. Although her doctor was not convinced it was HED, he suggested the family live as though Kayla had the disorder and he scheduled them for an appointment with a specialist in the field. They had to wait three months for an appointment. Dr. Anaya had requested that the specialist do a skin biopsy and a sweat test to confirm the HED. In the three months they waited, they radically changed the way they lived their life, now aware of the reality that Kayla could not get hot at all. She could not be in an environment higher then 75 degrees without fatal consequences. She always had to be in air conditioning. Running errands was no longer easy. Someone would have to baby-sit Kayla. Or, if the store was cool enough, she could join the family on the shopping trip but after shopping someone would have to stay in the store with her while her mom drove around the parking lot to get the car cool enough for a safe trip back home. The family learned to live in a 65-degree house to keep the temperature cool enough so Kayla’s body did not overheat.
At this time people began to step up to support and encourage the family. One couple donated funds for the Woodhouses to purchase outdoor play equipment so that Kayla could have it in her yard and go out at night when it was below 80 degrees with her vest on and she could play for about fifteen or twenty minutes before getting too hot. This was an additional blessing to her big brother Josh who had been restricted to inside living because he was too young to go out on his own.
Kayla as a toddler watching the outside world from inside her home.
VISIT TO A SPECIALIST
After waiting three months they made the three-hour drive to see the dermatologist who was supposed to be an expert in the field. He entered their room followed by an entourage of about ten other attending physicians, asked questions of Kim and then would leave the room and come back in. He kept telling them Kayla “looked perfectly normal” and refused to do the skin biopsy and sweat test. His greater concern was her eczema – a dry skin condition she’d had most of her life. It was clear he would do nothing related to her possible HED, he wouldn’t even touch her, because he felt she appeared “normal.” As a “specialist” in the condition of HED, he had never seen the cool vest nor heard of the HED Foundation. This was a discouraging visit and the Woodhouses were crushed as they were sent home with a diagnosis of eczema; something they already knew much about. Joshua had had infantile eczema and so they didn’t feel like the eczema was life threatening or related to her heat disorder. Upon arriving home they talked with Dr. Anaya and shared the news of their long-awaited visit. He was angry the specialist had not done the tests and wrote a letter to him sharing this. The specialist called later to apologize to the Woodhouse family and advised them to go to a “larger” city where other doctors may be better able to help.
That summer, when Kayla was about 18 months old and Joshua was 3 ½ they were in Joshua’s room playing while Kim was cooking in the kitchen across the hall. Kim hears Josh begin screaming and when she ran into the room, Kayla was not crying, holding onto a Little Tykes plastic hammer, covered in blood with a small hole in her head. Screaming in the corner, it took a while to get Josh calmed down enough to find out what had happened. He explained that Kayla had taken his blocks away and when she took them he got mad and hammered a plastic block into her head. In response Kayla got mad and yanked the hammer out of his hand and started chasing him and hitting him with it, unaware of the injury to her head. They later shared this with the pediatrician as it was evident Josh felt pain and Kayla did not. About two or three weeks following this incident, they were eating dinner at a friend’s whose house was fully tiled with ceramic tiles. Kayla kept purposefully diving from the top of the couch onto the floor, watching to see if her dad would catch her. Her dad scolded her as she continued to climb, he would frantically try to catch her, and she repeatedly thought it was a game, having no fear of being hurt. Occasionally, a part of her body would hit the hard floor and she would get up and do it again. She had no fear of falling and hitting her head on the floor because she could not feel the pain. In addition, from the time she started walking and running they would have to constantly remind her not to run hard into walls because she seemed unaware that she was hurting herself.
Just before Christmas there was an event at church for the youth group that Kim and Jeremy planned to attend. While they were waiting for their baby-sitter to arrive they noticed Kayla, a typical toddler, was chewing on something and had a little green piece sticking out of her mouth. When Kim pulled it out she realized it was a light bulb from the Christmas tree. They found the spot on the string of lights on the tree where she had pulled it off from the tree by her mouth. She had chewed it so thoroughly that she had crushed the glass down to powder so it didn’t hurt her stomach when she swallowed it. A trip to the hospital proved she was okay. The mystery of this was that the tongue is typically extremely sensitive to pain. She had blood in her mouth from the glass cutting her so they could not understand why she couldn’t have felt pain as she chewed the glass.
BIRMINGHAM CHILDRENS HOSPITAL
In September 1998 they took the specialist’s advice and searched for a hospital in a larger city – leading them to Birmingham Children’s Hospital in
They knew how to live as they waited.
After Christmas of 1998, Kayla started really suffering, as her eczema got worse. She was scratching so badly that she was digging deep holes into her skin. Her parents tried to treat it at home for several weeks. At night, to try to conquer the scratching she did in her sleep, they would tie her sleeper shut at the top of the zipper so she couldn’t get it undone. In addition, they duct-taped socks to her hands so she couldn’t scratch so deeply. They realized the eczema really bothered her. What they would later realize when Kayla was diagnosed is that she was able to feel the itch but not the intensity of her scratching so she was mutilating herself. In the morning, she would wake up with her socks on her hands and sleeper still tied shut but her back was still bleeding. In desperation, her parents called Dr. Anaya. He determined, after seeing her back that she would require a burn treatment to get her back to heal. He called in a nurse to assist with the painful treatment knowing they'd need her to help Kim hold Kayla down throughout the procedure. With a nurse at one end and Kim at the other they worked to clean her back. To the shock of the doctor, Kayla did not flinch. As soon as the treatment was over he pulled Kim out of the office and shared that Kayla should have felt pain and that that experience made him realize that a neurologist needed to be contacted immediately; no more waiting. He scheduled an appointment with a pediatric neurologist that entailed another three-month wait. In April of 1999, they went to see Dr. Terence Edgar, Pediatric Neurologist at Little Rock Children’s hospital.
When they sat down with Dr. Edgar he asked them to start at the very beginning, sharing everything that had happened in Kayla’s life. When they began to talk, Kim mentioned that her husband was a pastor and Dr. Edgar responded by sharing that he was also a Christian and asked if they would like to pray. To know that they shared their faith in common and had a doctor willing to pray with them was a great encouragement to the Woodhouses in the midst of the long journey they had walked. Following their time of prayer, Kim continued with their story and explained the doctors they had seen, the tests they had done, the results they had received; most expressing the same opinion, “she looks perfectly normal”. When she finished, Dr. Edgar looked at them and said: “I can tell you exactly what it is right now and then I will prove it to you.”
He believed Kayla had a very rare nerve disorder called Hereditary Sensory Autonomic Neuropathy; a disorder so rare that only twenty children in the entire world are known to have it. As Kayla sat in Kim’s lap Dr. Edgar proceeded to touch her and tickle her and she would feel and respond to the slightest touch. However, the harder he touched her and pinched her it was clear she could feel it but could not discern its intensity. Immediately, this explained her episodes with the eczema – she would scratch because it irritated her but she could not regulate the intensity of her scratch. Because she could not feel how much pressure she was applying with the scratch, she would dig holes in her skin. Then he broke a tongue depressor in half. He was trying to show them the intensity factor (pain factor) and he would poke her and scratch her with it and she would just look at him like “don’t do that any more” but not because it was painful. At one point Jeremy got a little protective as the doctor pinched the inside of her leg and twisted the skin to get her to react. In response she just turned away and looked at the doctor with no reaction. He explained that the post-ganglionic sympathetic nerve is missing fibers so the nerve does not signal the brain anything intensity related. She can feel things but she can’t tell the difference between a soft touch and a hard pat. So, she cannot tell that something is painful. She is unable to determine that she is too hot or too cold. As a result, she does not sweat. This also gave the Woodhouses answers in regards to her eczema. The nerves do not send signals to the brain that the skin is too dry so her brain cannot send messages to the body to lubricate the skin – resulting in extremely dry skin. At the end of the appointment Dr. Edgar set-up a nerve conduction study to be done the following month. In this study they sedated Kayla and put pins throughout her body to see the inside of her nerves and get various types of readings. They discovered that it takes Kayla 20 to 30 times the intensity for her to feel something. Dr. Edgar encouraged them that this was good news. Some children with HSAN cannot feel anything unless it is 100 times the intensity, resulting in devastating consequences.
There was a lot to digest on their three hour drive home as they realized the diagnosis was a permanent, life-changing event. Everything was suddenly final at that point when they realized the diagnosis had been confirmed. And, they knew that not only would she live like a child who could not sweat but also a lot of things would have to change because of the “no pain” factor. Jeremy was thankful for a correct diagnosis after such a long wait. Kim was also thankful they finally had answers, but it was both devastating and overwhelming to realize her child would have to live with all of this forever. It couldn’t be fixed.
She tried to imagine what it would be like for Kayla to spend the rest of her life inside.
REALITY OF THE DIAGNOSIS
They soon realized that the “temporary” lifestyle they had been living for the last year now needed to become permanent. They were in the midst of the severe hot months and they began what they explain to be their “confinement.”
The next two years of their life consisted of doing everything inside the house. Heavy drapes were kept pulled so that the heat from the sun wouldn’t warm up the house. The thermostat was kept at 60-65 degrees so the temperature in the house would stay cool while Kayla played. Both children were home-schooled beginning in preschool. Kim chose to do the more “intense” lessons in the summer when the temperatures were hottest so during the “winter” they had the time to play outside. Although most of the time, even in the winter they would have to wait until later in the day and Kayla would have to wear her vest.
It become clear they had to have foresight and really think outside the box, realizing the extenuating circumstances such as a house fire or a car breaking down on a hot summer day could have serious consequences for Kayla. A faulty lint trap in their dryer caught on fire in their home one hot summer day, igniting all of the clothes in the dryer and it filling the house with smoke. Kim called 911 and they instructed her to get out of the house for their safety and she told them she couldn’t because of the heat index of 115 degrees that day. She could not get to her car because she would have to go by the dryer and none of the neighbors were home. As a result, Kim and her children sat at the opposite end of the house with wet towels over their faces while they waited for police to come with an air-conditioned car so they could safely leave their house in the midst of the fire. Had Kayla gone outside, she could have overheated and died in just a couple of minutes in those temperatures.
FOURTH BIRTHDAY TRIP TO THE ALLERGIST
They had been waiting for Kayla to turn four to see a pediatric allergist, as they were getting very concerned about her eczema. Knowing it would always be a part of her life because of her disorder, they wanted to see if there was something they could do to alleviate the discomfort. Allergy testing on her back determined she was severely allergic to dust mites and many other environmental allergens. They also determined she was allergic to lots of common foods including yeast, eggs and nuts. However, doctors suggested they not radically change her diet concerned that if they took everything out of her diet and she was exposed to a trace of it later she could have a severe reaction. So, changing her diet became their last resort to help the eczema. Kim and Jeremy were given lots of information to read and were told they would have to pull up the carpet, buy all leather furniture, and cover their mattresses because they could not control the humidity enough in the South to kill off dust mites. Or, the doctor joked, they could move to
Out of the blue a few weeks later a friend called to share that he was putting Jeremy’s name in for a ministry opportunity in
They lived in
Kayla, Josh and Kim riding bikes in
A NEW CONCERN
In January of 2005, Kayla overheated and then later developed a serious headache. Because of her tolerance for pain, she had never had a headache that she could feel before so they knew it must be bad. In addition, they noticed her temperature stayed elevated and they couldn’t bring it down. Her normal body temperature is 95.9. Anything over 99 is considered to be very serious. Her temperature was over 100 for six weeks. Doctors could not figure out what was causing the fever. A spinal tap showed she did not have meningitis. Blood work was done numerous times and a CT scan could not give them clues at to what caused the headaches. They were labeling the headaches as migraines by this point because of their severity. During this time the vibrant, bright little girl that everybody had once known began to shut done, her eyes were glassy and she lay curled up in a ball on her oversized teddy bear, not moving. Adults who saw her this way who had known her before would cry because it was difficult seeing the pain and drastic change in her personality. They knew she was in more pain then probably any one could imagine. Doctors wanted them to keep a log of her headaches, temperatures and medications during this time, yet they still could not see consistency in cause, severity, length, etc. to try to determine a cause. Dr. Steve Baker in
MOVE TO COLORADO
The doctors in
ANOTHER HILL TO CLIMB
Following their move to
Doctor Morgan informed Kayla’s parents that she had a rare condition called “Chiari Malformation of the Brain” with a cyst (called a “syrinx”) below it. They explained that Kayla’s cerebellum tonsils – a part of her brain- hang down into her spinal cord because there is not enough room in the skull for her brain. As a result the brain is pushed into the cavity at the top of the spinal column, putting pressure on the spinal cord and a cyst has formed there. They met with a neurosurgeon the following week. He was very concerned and expected to be operating on Kayla within the next few weeks but first wanted to do another MRI of the full spinal cord. The surgery is called a “brain decompression.” It entails an incision at the back of the head, drilling out a portion of the skull, opening the dura membrane and then putting in a dura graft – which is basically a patch to make the membrane bigger. By taking out a portion of the skull it gives the brain more room. The MRI of the spinal cord was later in the week and the following Monday when they met with the neurosurgeon he felt confident it was fine to wait on the surgery and watch Kayla for six months because the spinal cord was straight and they didn’t find any more syrinxes. As long as Kayla did not become symptomatic (difficulty swallowing, gagging, severe headaches in back of the head, severe balance issues, etc.), the surgery was postponed until Fall 2006.
In one week, Kayla’s parents faced the realization that their daughter had another rare condition - a brain malformation - and she would be having brain surgery. Without the surgery she could face permanent spinal cord injury or paralysis.
Update: Kayla had started falling down the stairs and running into things during the summer. Her heat tolerance was getting worse. Kayla Woodhouse had brain decompression surgery on September 26, 2006. They drilled out a large portion of her skull, part of two vertebrae, opened the dura membraine and put in a dura grapht to make more room for her brain. As of her MRI in March 2007 – her brain is now where it should be, there is plenty of room for it, and miraculously, the syrinx is completely GONE!
Many things have changed for Kayla over the past year. Her heat tolerance is much worse. The house must always be below 62 now – she cannot go outside unless it is less than 55. The sun is dangerous for her – it makes her too hot. She just recovered from a severe double infection in her skin. Think about it: How do you keep a hospital room cool enough?
Kayla will always have her nerve disorder. She will always be confined in hot weather. She will always have severe eczema. Her parents will always be trying to keep her from scratching and mutilating herself, and trying to keep her from hurting herself. Her life will not be “normal” according to the world’s standards. However, her parents desire to offer her as normal a life as possible. They want her to be happy and comfortable. Because of the medical costs and the overwhelming amount of bills the Woodhouses accumulated when they were without insurance for Kayla, and so they could continue to qualify for insurance to cover the costs of her medication and impending surgery, they needed to sell their home quickly and move to a place that was more affordable in the midst of life’s current circumstances.
Unfortunately, their home was for sale almost a year and they have gone through the awful circumstances of foreclosure. Many, many people helped them through this difficult time and they are thankful for their community support.
EVERY DAY LIFE
Life is unique for this family. They spend most of their time inside and Kayla has to be watched and monitored so that she does not overheat or cause damage to herself as a result of her inability to feel pain. Simple things people take for granted could seriously hurt Kayla…
When Kayla was younger her mom was sewing and set her scissors down on the table. Kayla had been observing Kim cut strings off of some clothing, took the scissors and she cut a string off her outfit mimicking her mom as little girls often do. Later, Kim discovered that when Kayla was cutting the string she cut through her clothes and into the skin on her stomach. She never felt a thing.
Cooking with her mom is an activity they do indoors but she has to be closely watched and kept away from the stove and oven because she cannot feel if she is burned.
She applies medicated cream several times a day to her skin and has to be reminded to stop scratching so that she won’t dig holes in her skin. At times Kayla has scratched so hard that she has developed staph infections and her open wounds don’t heal because she continues to scratch.
A working car with air conditioning is a must. If something were to happen to the car on a hot day, it would be very dangerous and they would only have a matter of minutes without air conditioning before it became life-threatening.
They have always had to have some form of a communication device (such as a cell phone) to assure that help is accessible. Kayla wears a special medical bracelet that gives emergency personnel brief information on her disorder with a number to call where they can get information and be told what to watch out for.
They found out when she was a baby that detergents bothered her skin. She always had to have dye-free, fragrance-free detergents to protect her skin from breaking out in an allergic reaction. Whenever they travel to a hotel or stay at someone’s house, they have to wash the sheets in special detergent.
She is severely allergic to dogs and cats. They were noticing that whenever she would be around someone or hug someone that had pets, her skin would have a negative reaction. People close to the family who understood their situation would wash their clothes in the same type of detergent and they would use allergen spray and spray themselves before being around Kayla. Because of her allergies they also have to use a higher quality, heavy- duty filtration vacuum cleaner and special air filters running in their home to keep the air clear of dust mites, pollens, etc. The newest blessing, however, came in the form of a wiggly, wet-nosed birthday present when Kayla and her brother Josh each got a puppy. Doctors suggested that dogs be re-introduced, as outside pets, so that Kayla can build up a tolerance to their dander. It’s working! Her little puppy gets lots of hugs and Kayla’s skin is responding well.
In hot months they are limited as to where they can go. Restaurants, Malls, Target, Wal- Mart, and King Soopers are off limits because the air is not set low enough to keep Kayla’s body temperature at a safe level. In
They choose home schooling because Kayla needs to be watched closely to make sure that she doesn’t hurt herself and assure she stays cool. Early on, they realized they couldn’t expect a classroom to set room temperatures in the 60’s for Kayla; and what if there were a substitute teacher one day who didn’t understand fully? Kayla could be sent out to recess, overheat and possibly die. Even the church they attend has air conditioning in the sanctuary but not in any of the other rooms so there is a lot that she cannot be a part of.
Kids can be cruel. Although Kayla has only faced a couple of negative comments in her life, they were painful to her and had lasting effects. At the grocery store when she was about four, another little girl noticed Kayla’s “cool vest” and said: “Look at how weird that little girl looks,” proceeded to ask her if she was wearing a bulletproof vest and if she was worried someone would shoot her. In another instance a little girl saw the eczema on her wrist. It was pretty bad at that time and the little girl said: “Ewww, what is wrong with you?” For a year after that comment Kayla insisted on wearing gloves and long sleeves because she didn’t want anyone to see her eczema. As she has grown, she is learning how to deal with the comments. And, she realizes that God has made her very special. Her parents remind her that she is uniquely created and this shouldn’t be a negative, but a positive – saying: “Wow! Look at what God has done through you…”
WANT TO MAKE A DIFFERENCE?
You can make a difference in Kayla’s life. Perhaps you or someone you know could help with the following:
1.A financial donation of any amount would be gratefully received by Kayla’s family to help with significant medical and housing expenses. The Gift of Pain Foundation has offered to receive your 100% tax-deductible donation on behalf of the Kayla Woodhouse family. Please write your check to the Gift of Pain Foundation and make sure to put “Kayla Woodhouse” or “The Woodhouse Family” in the memo section of your check. A Gift Of Pain Inc.
23080 162nd Street
Elk River, MN 55330
Several years ago, when Kayla was four or five years old, a family friend suggested that Kayla would be the perfect Olympic swimmer. Swimming is the one activity Kayla can do because it keeps her body temperature down so she can move and exert energy and her body does not overheat. Kayla loves to swim! It’s an activity that she sees as something she can be good at and her dream is to become an Olympic athlete. Generous people have donated a couple of months of a membership to the local YMCA so Kayla can swim. Her ultimate dream is to one day have her very own swimming pool so she can play outside like other kids and do her favorite thing… swim!!
2.This very special family needs a home. They are looking for an affordable place with space in the
The Woodhouse Family: Jeremy, Kim, Joshua, Kayla
The fluid inside the vest was developed by NASA for those doing space walks. Sarah Moody was the one who pushed to have them designed and developed for kids with HED (Hypo-Hydrotic Ectodermal Displasia; a disorder in which children are born without sweat glands.) The vests used to be 65 degrees and now are about 57-58 degrees. They are now provided through the Heat Relief Depot and used to range in cost from $500 to thousands of dollars. Thankfully, they are much more affordable now.
In the past parents would pack their kids in ice to keep their body temperatures down. The ice would burn their skin. The vest provides these children a life and a way to have a sense of normalcy. For Kayla, it allowed her to see and experience the outside world. During cool months she has spent time at Disney World, air-conditioned Children’s Museums, air-conditioned aquariums, the Seattle Space Needle, and she rode a carousel…twice!
The Sarah Moody Foundation had taken care of all of the equipment and expenses associated with Kayla’s vests. They need to be replaced as they grow. She is now on her sixth vest and sadly, the Sarah Moody Foundation has shut down. Gift of Pain is now helping the Woodhouse family and others like them. www.gift-of-pain.org
Kayla Woodhouse (right) in her vest with good friend Erin