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Heather Joy's Page Welcome to Heather Joy's Web Page. It has been provided to keep people updated about Heather.
Journal
Tuesday, March 22, 2005 6:47 PM CST
At the age of 4, Heather is alive and well. She has been through a lot, but some people in this life seemed to be ordained by God to have those extra challenges. I believe that those are the people most blessed by God. It seems sad to chronicle one’s life by surgeries and illnesses, but I will also try to tell of achievements and accomplishments at the same time.
2001
My pregnancy and delivery were normal. She was delivered very quickly and was bruised over most of her body. We spent a few days in the hospital the first week for Jaundice. There was no concern yet as most of my kids had jaundice at birth.
We didn’t notice any problems but gradually she started missing milestones. At her four month check the pediatrician decided that it was time to see the specialists. By the time we were able to get in, she was already 5-1/2 months old.
Heather’s first two tests came on the same day. She was diagnosed first with bilateral cataracts and hydranencephaly on that very auspicious day. The eye doctor and the neurologist were from two different prestigious Children’s Hospitals, but because of long waiting lists, we decided to divide and conquer. On this day, Heather had nursed as usual and was actually fairly quiet. She normally cried a lot. From these appointments came disappointments. The cataract surgery was canceled by the two doctors, without the input of the parents. This was something that I warned the new doctors never to do to us lest they be replaced like the first two. Heather’s seizures started at about nine months old. She had just gotten over an illness and the chickenpox. We got a second (and third) opinion in neurology. Heather was hooked up with an EEG, and we were hooked up with Early Intervention. She was put on liquid Phenobarb for the seizures, but we quickly got that changed to tablets because she hated the liquid and would spit it out. We also got a second opinion in ophthalmology because we saw Heather trying to see the dark stripes on a white background and thought that if the cataracts were removed she had a chance of seeing. We were happy that Heather’s new eye doctor was very concerned for Heather and was willing to do the eye surgeries. Those were her very first surgeries at 11 (left eye) and 12 months (right eye) old. During the first year, she had a great vision teacher who helped her to “learn” to see. Her physical therapist helped her to remain strong and flexible. Her occupational therapist helped her learn to chew and swallow properly.
2002
At fifteen months we took her off of Enfamil infant formula because she really wasn’t gaining weight. We put her on Pediasure and she seemed to have constant “gunkiness”. We took her to a GI who wanted us to give her all soy products instead of cows milk and to give her a formula (Peptamin Jr.) that was pre-digested so it would be more readily absorbed. At this point I wasn’t ready to change all of her food. We were still feeding her orally and she was getting really good at chewing and swallowing solids. Purees were always good too. We did notice that she was sometimes coughing with thin liquids, so we had a swallow study done. The doctors and nurses were very impressed with her ability to eat “properly” and cleared her for everything but thin liquids. We just had to add baby cereal, applesauce or thickeners to her drinks. They asked us to come back in a few months to test her again. The second test did not go as well as it fell into a bout of illnesses that made it hard for her to breathe.
2003
That winter when she was about 22 months old, she had a continuous string of sinus infections. We would use augmentin to clear them up, but 3 days after coming off the augmentin she would start all over again. She had seven in a row and the pediatrician was very brain tired. She was out of ideas as other anti-biotics didn’t have any effect and she didn’t know what was happening. We were referred to an ENT (Ear, Nose, Throat Specialist) and the Tonsils and Adenoids turned out to be huge. She snored when she was awake, but was quiet when asleep. The ENT lost her reports two different times and never called back to let me know what was going on. We finally changed ENTs. At this point we also changed Pediatricians. The new doctor referred us to a new ENT who agreed to take the tonsils and adenoids out, even though she was younger than what they liked to allow. The infections stopped immediately. She had tubes placed in her ears at the same time, although both ears were ok at the time of the surgery. We spent one night in the hospital with this surgery. By the next morning, Heather was eating Blueberry Buckle Baby Food again.
By now we were part of the International Hydranencephaly Support Group that my Mom had found on the web at hydranencephaly.com. I was learning many useful things. One of the things the group talked extensively about was feeding tubes and nissen fundoplications. Heather was not gaining weight and was now taking 3 hours to eat a meal. I asked the doctor about the feeding tube. The period of illnesses was scary. If it happened again would she be able to eat or drink? She was unable to gain weight with the food she got. She was irritable because she was hungry. Would she be able to eat by mouth if she had a feeding tube? Would her younger siblings pull it out? We had many concerns, but put them aside as we saw her need for better nutrition. We requested that the fundo be done at the same time because we saw that many of the kids on the group who didn’t have them done together needed to go back later to have a fundo done for reflux. In order to do the fundo, the doctor needed a medical reason (for insurance to cover it.) She had a swallow study done and it was determined that Heather did have reflux. We hadn’t even known about this potentially deadly problem. She was immediately put on Zantac and the G-Tube and Fundo were done using laparoscopic surgery. Heather was in the hospital for about 5 days and came home with a Ross Patrol pump and a new way to eat. We had trouble getting the site to heal. She also would do retching with her feedings. She had nasty new stool problems...blowouts that smelled very bad. About one month after this surgery we had the tube replaced with a Mic-key button. It was nice an flat and nothing to snag on her equipment.
About this time I was able to meet with some members of the support group when we met in Orlando for the World Congress and Exposition on Disabilities. I met many parents who were in the same boat...they taught me many valuable things. I was taught how to keep Heather’s feeding tube site clean and dry to aid in it’s healing. I was also shown the right was to use a pump. I had been doing bolus feeds up to this point. I was also shown that with the right textures and consistencies, foods other than formula could be put into the tube. I learned syringe magic and feeding tube wonders. Heather was spoiled beyond belief by the love and care and I actually considered letting some of these people who were much better at this “stuff” take her home to care for her. By the end of the week, I felt much more confident that we at home could do the necessary work to give Heather a good life. I felt renewed in spirit to do the big job ahead. The sessions and exposition also gave me things to think about. I saw Heather participate in play as I had never seen her do before. Some of the sessions were designed to involve the child as well as the parents. She amazed me. She was always a hard worker in her therapies and now I got to see her having some fun as well. When we got home, I designed a scooter board for Heather using a cushioned seat from an old kitchen chair and 4 castors. She pushed herself around with her feet while lying on the board belly down. The neurologist introduced Heather to Lamictal for seizures and slowly weaned her from the Phenobarb. This was an attempt to keep her from being so sleepy when school started. At this point, she was still fairly loose in the arms, but her legs were beginning to tighten up. Orthopedics said her x-rays had looked good so far, but one hip was now starting to come out and she was no longer comfortable in the Microshell positioning seat she had been in, or in the wheelchair.
2004
The Winter of 2003-04 was hard. This was to be Heather’s last few months of in-home therapy. She would be turning 3 and would hopefully be attending The Overbrook School for the Blind in January on her birthday. Unfortunately, she just kept getting sicker and sicker. She started not taking her food as well. We had to keep cutting back her formula. She switched all of her therapists at age 3 as she moved out of early intervention and into the early childhood program of the intermediate unit. With all of her illnesses and the length of time it took to get her transitioned we decided to wait until the fall 2004 to start school. We started her on Baclofen to see if it would help her with her growing spasticity. She did have some relief, but started to have more seizures again. We gave her Botox injections and weaned her from the Baclofen. By March I was going nuts trying to figure out why she was so sick. We took on a Diagnostic Doctor to try to help us solve the mystery. I had individually spoken with her specialists to see if they thought her problems might stem from their area of study. The Orthopedic Surgeon assured me that the pain was not from her hip (although he was late for the appointment which didn’t allow enough time to do an x-ray to see where the hip was). The neurologist thought that it couldn’t be the medicine or seizures. The GI (tummy doctor) felt that it wasn’t her feeds or tube (which still hadn’t fully healed!). The Diagnostic Doctor talked to all three of the specialists after listening to Heather’s story. The neurologist increased her Lamictal and added Clonazepam. The GI doctor switched her reflux med to Prilosec (Omeperazole) and we were given an appointment with CP clinic. In March she started throwing up. One of the benefits of the fundo is that, generally, you don’t throw up past the fundo. She was ill enough that we were now worried that maybe she had “blown” the fundo, or opened it up. All scans showed that the fundo was intact, and yet she continued to be sick. We decreased the amount of food and that helped for a bit, but she was still losing ground. She had some good days, but most were unhappy and uncomfortable for her.
By the end of April, on a Wednesday, I took her to the emergency room at Children’s Hospital. I had never done this before, but we were scared. They did an x-ray and determined that she had some stool in her intestines, but it didn’t look like enough to be causing problems. They gave her an enema and sent us home. Thursday seemed better, but on Friday she was very lethargic. I called the pediatricians office and they had me call 9-1-1. With in half a minute the ambulance was out front and the medics were in the house. They laid her down on the floor and were opening up their equipment when she stopped breathing. Her heartbeat quickly stopped to, but not before they had her intubated. She was life-flighted back to Children’s Hospital, this time to the trauma unit. When we caught up to her, she was very limp and cold. Her body temperature had dropped to 86ºF and she was still fairly unresponsive. I held her hand and eventually she started to squeeze my finger. She was coming around! After we moved up to the Critical Care Unit from the trauma room, I received a call from my Mother-in-Law who was babysitting the other kids. The ER nurse at Children’s (where we currently were) had called to say that Heather’s blood work from Wednesday had turned up a Urinary Tract Infection, caused by the constipation, and containing e coli bacteria. We were in the hospital for 9 days. When we left, she was nowhere near as active as she was before the sepsis. She looked and acted as if she had had a stroke, but the neurologists there said no stroke because nothing showed up on either EEG or CT scan. One side of her body was relaxed the other loose. There was a difference between the sides of her face. She was put on Miralax to make sure that the constipation would not be a further issue. Also, the Pediatrician put her on a prophylactic dose of Bactrim in a follow up visit. We didn’t want any more close calls.
We had one more ER visit in the summer. In August she started coughing during her bath. Her aide brought her downstairs and she had blood on her mouth. We thought she may have bitten her lip, but is was soon evident that she was coughing blood. The aide (who was still very nervous from April) said that she wasn’t breathing right, so we called 9-1-1 again and the ambulance drove her to our local Hospital. The ambulance crew reported two instances of apnea before we got to the ER, so she was brought in for observation. She continued to cough and vomit blood. The doctor in the Emergency Room told me he wasn’t worried about the blood. The x-rays showed that her lungs were clear so he believed that the blood was probably a blood vessel in her throat that had ruptured because of the heavy coughing. He said he was going to transfer us to Children’s, however, because of the apnea. We weren’t at Children’s too long. They agreed about the blood, and then sent us home.
It was now Fall 2004 and Heather was set to start school. They are used to children with severe disabilities, but they were scared when she had a couple of short seizures in October in which she turned blue. The school is a lot closer to Children’s Hospital in Philadelphia than I am, so they called an ambulance and within 10 minutes the teacher’s aide and Heather were in the ER. She had no more seizures there, but because of the increased instances in apnea they made sure that we went home with a pulseox machine and a suction machine.
We finally got to the CP clinic. At the clinic I told the director (who happened to be the boss of the unhelpful Orthopedic doctor) what had happened at the last appointment with that Orthopede. He assigned us a new Orthopedic Surgeon. Heather had x-rays taken which showed that her hip was now completely out. Although it seemed to not causing her acute pain, we agreed that it needed to be fixed. On November 2, 2005, Heather had bilateral osteotomies and tendonotomies done. She came through the surgery very well and was in the spica for 5 1/2 weeks.
2005
Heather started back to school after Winter Break was over. A couple weeks later she turned 4. She has, so far, had a fairly healthy winter. We all had the stomach virus on Christmas, but it was short. She has her colds and such, but is nearly recovered from the sepsis of April 2004 and from the hip surgery. She continues to improve in school and can now eat by mouth again. The seizures are calm at the moment. Her next issue that we will tackle is her spasticity. She has been approved as a candidate for a baclofen pump. Later this year she will probably have one implanted. There also remains the possibility that the Orthopede will want to take out the plates and screws that were put in during the original surgery in November.
Equipment
Aphakic Contact lenses, Ear Tubes, Dafo’s by Cascade, Hand Splints and Vest by Benik, Feeding Tube Buttons (Kimberly-Clarke's Mic-Key, the Corflo Cubby and the AMT Mini), Stander by Leis Tech, MicroShell Positioning Seat, Zippie Tilt-in-Space Wheel Chair by Quickie, HeadMaster Neck Brace, Rifton BlueWave Bath Chair, SleepSafe Plus Bed, Ross Patrol Pump, Zevex Portable Pump, Devilbliss Suction Pump, PulseOximeter, Powerlink 3, 2 Jellybean switches (red and yellow), and a BigMac Switch.
Medications
Current: Lamicatal, Omeperazole, Bactrim, Clonazepam
Discontinued: Phenobarb, Zantac, Baclofen.
Family
Dad (Mike)(37), Mom (Karen) (37), William (11), Laura (10), Eric (9), Peter (7), David (5), Heather (4) - herself, Elizabeth (2), Jonathan (1)
Read Journal History
Links: http://hydranencephaly.com A website that provides information for the people who care for a child with hydranencephaly
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