In the Beginning..
We are The Laws. George, Richelle, and Cory (Corleen). Our adventure with Lissencephaly started 12/4/93.Cory was born at 11:51pm 12/3/93 at 6 lb 8 oz. The OB did a c-section because after many hours of induced labor my wife still had a ways to go, and also the monitors showed that during contractions, Cory’s heart rate was dropping ( or was it increasing). They thought the cord maybe wrapped around her neck. Little did we know.The doctors came and told us within an hour of Cory’s birth that she was having seizures and that they had move he to the “special care nursery.” We were introduced into the world of seizures the next morning when we went to see Cory. I know that the movements that the termed seizures I saw in the delivery room, and many times my wife and I had thought “Gee, this is an awful active baby” We could feel rhythmic kicks for the few months before birth, so we are pretty sure the seizures began sooner. From day 1 Cory has always had uncontrollable seizures. Even DR Dobyns was shocked by the amount of seizures she had, but that comes later … They initially tried phenobarb for the seizures, saying that many times birth can be traumatic and there maybe swelling on the brain causing seizures. They expected the CAT scan and MRI to show water or bleeding. The CAT scan was done first, and we were told “there might be a migrational disorder, where the neurons in the brain did not complete the development cycle. We weren’t prepared for the bombshell the MRI showed. The doctors came to us and said “The MRI confirmed the CAT scan” I was hot, we were given the impression from the CAT scan that nothing was really wrong, and then told that she has Lissencephaly, that the medical books said she may live 2 years … everything that most other parents hear.
My wife and I tried to explain it. I actually tried to blame her, that it had something to do with her smoking or taking some cold medicine or something. Then we began to expect that we must have both done something very bad in a past life and we were being punished. Neither my wife and I, although we were not married at the time, were religious. And at this point let me say, I don’t want to offend anyone reading this. Instead of putting faith in God, we asked why? We both read the Erma Bombeck poem about how God decides which families are given normal kids and which are given “special” kids. To be honest, that just didn’t cut it. We asked ourselves, how could a loving God do this to a child. We could understand someone being stricken with a disabling illness after a life of sin, but Cory was an innocent child. WHY? HOW? WHY US? But, I am getting off the line of the story.
Like many of the kids Cory had a poor gag-reflex. They tried the n-g-tubes and finally decided that the best thing would be to put in a g-button. We were ready to spend Christmas day at the hospital while the surgeon did the operation, but Christmas eve they decided to put it off until the 26th. During the few weeks in between here they tried dilantin and prednizone and then ACTH. ACTH really threw Cory for a loop. She dropped down to 5# 10oz and there was not much seizure control. They tried the combination of klonopin and depekene elixir and she did better, gaining a little weight back.
February 3rd 1994 was Cory’s homecoming. We had just moved into a new apartment, a little 2 bedroom house. Cory came home with nursing care, to try and help my wife and I keep our sanity. During this time the question arose if Cory would be better off in an institution. Honestly, we never thought of this. Regardless of her condition, she was still our daughter, and all we wanted to do is take her home and love her. Getting used to nurses was difficult. It was hard to sleep at night with a “stranger” in the house. We sure had some real “winners” over the course of the next few years. We also set up schooling for Cory, having a PT and teacher come into the house once a week.
We went through what I am sure all our fellow parents go through. The weekly load of meds. The midnight trips to the ER for level checks. Like all the kids, any sort of infection really gets Cory. Another part of Cory’s condition which makes her different from 99% of the other Lissencephaly kids is that she has what is termed “ambiguous genitalia” What they think happened is that along with the brain development going astray, the sex development also “goofed”. All children start out female genetically. During the course of development there is a hormonal signal given when it is decided if the child will be male or female. Well, with Cory, the signal was given to switch, but did not finish the change, leaving her anatomy in between. This has always caused Cory to be real prone to Urinary Tract Infections. Once minute we could be at home and the next, Cory starts puking her meds and everything else up. Cory also had a hard time maintaining a “normal” body temp. Well, as I am sure we all know, no meds mean more seizures, more seizures means the temp goes up, fever means more seizures … you get the picture. The doctors at the ER, SUNY HSC here in Syracuse all got to know Cory pretty well, sometimes a little too well. They usually had to admit her because to get her meds into her, they had to be given IV. Welcome to Camp Crouse. Crouse Hospital, the best peds hospital in Syracuse. You may ask “why one ER and a different hospital”, I will explain later. After about 6 UTI’s we tried something with no proven medical use, cranberry powder tablets. Let me tell you, they seemed to do the trick. We didn’t seem to have a UTI for several months. We ran out one time and figures, well, is this really doing anything? and didn’t get any more. Sure enough, a few months later she got an UTI. Boy did my wife and I feel terrible!
The SUNY Experience
One of the hospitalizations Crouse was full, and they admitted her to SUNY. BIG MISTAKE! (We should have listened to the Curtis’s) It was the middle of the summer, and the room she was in had the AC cranked. It was COLD in the room. Well, Cory became hypothermic (?) her body temp dropped to 92 F , her heart rate got real sluggish and she stopped breathing and had to be bagged twice. We got that phone call at 4am, talk about a race to the hospital. Well, here they had Cory in this 65 degree room, without nay blankets, of course she is going to get cold. They would wait until she got cold, than blast her with a aqua k-pad, heat lamps and bundle her up. Well, her temp would go through the roof, and they would turn the lights and k-pad off, and she would get cold again. This went on for a couple days. We finally said “DUH, that isn’t working! Try keeping her bundled and she will be fine. ” Like they say, parents know best! She has never been admitted to SUNY since.
Conference 1994 – Chicago
We attended the conference in 1994. It was not an easy task! We had to drive 90 miles to Rochester, because it was where our flight left from. We got the same plane out as Jim, Mary and Kristin Curtis, also from Syracuse. I had never flown, so it was quite an experience. We had been able to get grant money to help finance the trip because we could not have afforded it ourselves. We met several other families and it gave us a real welcome feeling. We also met DR Dobyns. He held Cory for 5 minutes and could not believe the seizures. He was the first one to put a time frame on Cory’s life, something no one had done before. It was estimated that due to her seizures, she would live to be at most 5, but no one could say for sure. It was quite an experience, the first time my wife and I did 100% of Cory’s care without a nurse. Talk about an exhausting weekend.
Our experiences with nursing care has really been something. We have several nurses that have really become like family. But there always has to be certain ones that seem to know it all or have done it all and have no clue. Soon after returning from Chicago for the conference, we had a nurse who felt she knew it all. She went as far as to call Cory’s doctors and tell them that Cory’s meds were not strong enough and need to be up’ed. At this time Cory was begin given valium as a PRN. Valium did not touch her seizures. Being on klopopin at the same time, the doctor was not surprised that if she was seizing on klonopin, valium didn’t do much. My wife received a visit for an investigator from Child Protective services. Some one has complained anonymously that Cory was being given too much valium. Its hard to give it when we didn’t even bother refilling the prescription when it ran out and there was none in the house. A few days later we were are the neurologists office when she received a call from the investigator. She was told the complaint was that she was not being given enough valium. She vouched for us and said that the valium didn’t work so it was not begin given and that Cory was a difficult child and we were doing good jobs with her. Now, we were never told the same nurse made the call, but it was obvious enough that we had her pulled from the case ASAP.
Summer of 1995
We had another harrowing experience with nursing care. As I was leaving to take my wife to work at about 3:30pm, we told the nurse to give Cory her 4pm dose of chloral hydrate early to try and calm her down. I made the hour and a half round trip, dropping my wife off at work and returning home. I walked in and there was no one home. Talk about panic. I thought the nurse stole my kid! Or someone came in a did something. We lived in a bad section of town and I had to walk the nurses to their cars at night. I called out nursing agency, and my wife’s agency ( she is a home health aide). All I was finally told was “Cory’s has been overdosed, go to the hospital” Mario Andretti would of sure been proud. I got to the hospital and about 10 minutes later my wife arrived, her case has given her a ride 45 minutes to the ER. We walked in and people started asking us questions. How did we know, we just got there. We came to find out that the nurse had misread the med sheet, giving 10cc of chloral hydrate instead of 1 cc. A big difference for a baby who was 12 or 13 pounds. The nurse had been so hysterical that after she called 911 and the ambulance arrived, she had taken herself to another hospital and signed in for psych observation.
Meds & the Ketogenic Diet
We tried all the meds available. Tegretol, Dilantin (again), felbatol, neurontin, valium, chloral hydrate … No real luck in seizure control over maybe 50%. Last ditch effort was the Ketogenic Diet. We spent months planning a vacation for 1996. One where we could get away from the city, but still be able to get nurses to be able to take Cory with us. Here in NY a lot of things are dependent on the counties. We made reservations for go camping the week after labor day in a cabin at Green Lakes state park. We moved into the cabin on Monday, had a neurologists appointment on Tuesday, and it was decided that Cory should be started on the diet, only thing was .. she had to be hospitalized to do it. Cory’s vacation was moved from the cabin to the hospital, a place she always seems to like to visit twice a year. We started a liquid tube fed version of the diet and after a 3 day hospitalization turned into 2 weeks, Cory came back home. During the last few days that my wife and I were camping alone we played more games of cards and backgammon than should be legal, we were ready to KILL each other. The first 2 weeks of the diet were GREAT. Cory was like a new child. She was awake, alert and the best seizure control that we ever had. That lasted for about 2 weeks. Her gastroenterologist was not happy with the calories that the diet was providing and was adamant about changing the formula for the mix. We mixed pediasure, water and MCT oil. The GI Doc changed everything, and threw the seizure control out the window. We spent a year and a half trying to regain what we had for those 2 weeks and finally gave up the diet in Feb. 1997.
Bah Humbug 1996
The holidays not been good to us. 10/96 my wife’s grand father died, followed by my father on 11/30/96. To make things worse, Cory developed her first pneumonia and went into the hospital in early December. Now, my father had just died of pneumonia which complicated lung cancer and also, we had always read that the kids with the g-buttons usually passed away because of pneumonia. It was not a good time. Not to mention that we made 1 ER visit with O2 stats in the mid to upper 80’s. The doctors did not even look for pneumonia, looked for a UTI because that is what they were used to. This was soon after we stopped the cranberry tabs mentioned earlier. They found a UTI, prescribed antibiotics, and sent us home, never questioning the O2 stats. We were back 3 or 4 days later, she was throwing up, running a fever (very unlike her) and synoptic (turning blue). Still, no questions about the O2 stats or the turning blue. They decided to admit her and we had them take her to Crouse. The next morning the attending physicians felt that her respirations sounded labored so they did a chest X-ray. Low and behold, they found pneumonia. That hospitalization seemed like it would never end. It got to be 12/20 and we finally said enough, send her home. So we brought her home without nursing for the first few days, but at least she would not be in the hospital for Christmas.
Christmas Eve I blew our car up, blowing the head gasket and almost seizing the engine. I wrecked a car I bought in the mean time on 1/20/97 and yes, I thank God that I walked away. Someone had to be looking over me because I went off the road doing 45 mph, hit a mail box, a parked car, and head on with a tree. Car was totaled, I split the tree in half, no seat belt, and I walked away with some bumps and bruises and a ticket for not wearing my seat belt. My uncle then died in March and a great aunt was killed in a car accident in June.
Cory returned home, having been in the hospital again for 2 weeks and coming home 7/1/97. This time they tried 2 oral antibiotics before going in the hospital, 3 IV antibiotics (at the same time) for 10 days in the hospital, and then an oral antibiotic to come home on. In the end, she had at least as much pneumonia as when she went in the hospital. We are now on a “comfort care” basis because the doctors do not know what else to do. They have tested for reflux, no reflux. They feel that neurologically Cory has deteriorated to a point where her gag reflex ( as little as was there) is almost gone and she is aspirating her secretions. She has also started to develop contractures on her right hand. We were always told that the longer the seizures go uncontrolled, the more damage they will do to the brain, the more damage to the brain, the more seizures are caused. It seems that we are now to a point where Cory is either sleeping and seizing or awake and seizing, there is not time where she is really alert or aware any more.
The doctors have suggested that the only other thing that can be done to help remove the secretions is surgery to place a trachea and possibly do surgery to close the airway off from the mouth. Another thing I hope does not offend people is our decision on this. Initially, 3 yrs ago Cory was a partial DNR. We felt that we at least wanted 10 minutes of bagging and chest compressions done, but no meds and no intubation. Someone, possibly a nurse asked us about the DNR, and if the bagging and chest compressions were for Cory’s sake, or more for ours. We really thought this over deeply, and decided that when it happens, it is time for it to happen, there is no sense to trying to delay it.
One of the few good things that has come from my uncle’s death was that he left my mother $300,000 When most parents of 3 1/2 yr olds are out making plans for their kids to start school, we borrowed money to go and make funeral arrangements. Its a load off our minds. That was something that always stood out because we knew Cory would pass away and have no savings or insurance or anything. I have finally, after 6 years,a job for what I went to school for, but its a small company and I am not making much. Its just one less stressor.
Being realistic here, Cory does not have much of a life. Don’t get me wrong here. I love Cory and I would not trade her for a “”normal”” child for the world. There were times when we were optimistic about Cory’s progress. It took over a year for us to see our first smile. And the few times that we got to see her awake and aware of her surroundings are something that we will always remember. But those days are past. It has really come down to a point where we watch her suffer every day. She has always had alot of seizures, some small, some large, some that seem to go from one right into the other. She can seize for hours and hours at a time. Many of her seizures are small. A facial grimmace, a glance upward. But to watch her whole body twitching for hours and not be able to do anything is finally taking its toll. We decided against any surgery and even the doctors agreed that it would be too hard on her. Its “time” and we have accepted that. Its just tough waiting now that we know its going to happen soon. As much as we love Cory, its time.
12/7/97 – The Energizer Bunny
We just celebrated Cory’s 4th birthday. Like the Energizer bunny, she keeps going, and going, and going … Its so hard to belive that it has been 4 years. Cory still has pneumonia, and a recent added feature is Pseudo-pneumonis (spelling?) Again it time for the holidays, and again its BAH HUMBUG! We don’t know how most of the other families get by during the holidays. Its hard with us because Cory is our only child. You know those commericals that come on during the holidays of kids coming down christmas morning to see what Santa brought. Or of kids out making snowmen. These things depress us more than anything just because it it something that Cory will not ever do.
Our Angel flies away
On the evening of March 3rd, Cory spiked a 106 degree temp. I was home alone with her at the time. She was just hanging out in her diaper watching TV with her pop. I picked her up and immediatelly knew she was very HOT. Altough her front and legs were cool, her back was burning up. I gave her a cool bath and brought her temp down to about 99 degrees. The night nurse arrived an hour later and by then she was back up to 101 and stayed between 101 and 102 the rest of the night. The nurse woke me about 6:30am to tell me that she had been warm all night and had vomitted a few times. To me this was a sign to pack the bags, off to the ER. My wife got home at 7:30 after working 11pm-7am and by 8 we set off for the ER. We got to the ER and of course, Cory no longer had a fever. Blood work showed she had an elevated white blood cell count, signalling an infection somewhere. They took more blood, did a chest x-ray, a urine culture/analysis. They decided to admit her which we agreed was the best thing. We ended up leaving the hospital at 3:30PM, both my wife and I were exhausted. We received a call that they thought there might be a brain infection and they needed us to sign consent forms to do a spinal tap. Never hearing of a brain infection before, we rushed back to the hospital and then it was explained that the reason for doing the spinal tap was just the last step in a “Full Work Up”. Of course, the spinal tap showed nothing out of the ordinary. They tried several antibiotics ovre the course of the next week and finally called in a consult from Infecteous Diseases. The specialist suggested another antibiotic to treate the pneumonia and pseudo-pneumonis and it was started immediately. After 24 hours her fever broke and her oxygen stats were improved. She even looked like she was breathing much easier. During this whole hospitalization, she was usually sleeping which is uncommon for her. She is usally seizing away like there is no tomorrow. The only concern was that her potassium level kept dropping. They had given her some supplemental potassium via IV, but were puzzled because the level still dropped.
March 12th I called the hospital on my way to bed. It was about 12:45am and at first I hesitated to call, being so late. After a second, I thought “Aw, what the heck?” The nurse said that Cory’s heart rate was dropping into the 50’s. This was not a new thing because when she slept, she really really slept. I told the nurse that it was not unual, and if anything else happened to give me a call back. I then called my wife at work and told her breifly what had happened. She said that she would call on her lunch break and check on Cory. Not 2 minutes after I hung up with her, the phone rang again. This was “The call” that we had always dreaded. The doctor said that Cory just stopped breathing and was gone. My wife got home from work and my sister picked us up, neither one of us were in the condition to drive. It was the *longest* 20 minutes of my life. We arrived at the hospital hoping that it was not true, but she was gone. The nurses said that she was very peaceful when she passed on. Although it hurt us terribly, we knew that it was her time to go and did not question it.
Looking back, we feel that Cory’s comfort during this hospitalization was probably her way of telling us that it was her time and she was not suffering anymore. As the doctor on call at the time told us, “You know now that she is running and playing and happy with the other angels in heaven.”
Cory was laid to rest a few hundred feet away from her namesake, my wife’s grandmother. We purchased 3 grave sites, her in the middle with my wife and I to be laid to rest on either side. We are currently shopping for a headstone to fit, we have some ideas, but don’t know exactly what can be done on a head stone.
This has been been awfully hard to finish. What the future holds we do not know. We have been told there is at least a 25 percent chance that future children will also have the same medical problems. This, as you can imagine puts a damper on things. I think I would rather play russian roulette, the odds are better! People tell us we should write a book based on our time with Cory. But who knows?
Update – 2001
Mackenzie Ann Law was born 4/12/2001. We finally decided that it was worth the chance and try for another baby. After all kinds of genetic testings, the time arrived. They decided to induce labor because they thought Mackenzie was getting big and did not want a 10 pound baby. Like Deja-vu, the doctors thought the baby was in trouble, so they decided to do a c-section. I was rushed into the recovery room wondering what the hell was going on. All I could think of is “This is too much like Cory’s birth, what if something goes wrong??” Thankfully, they came and got be a few minutes later when they decided to do an epidural which meant I could be present for the birth.
Update – 2002
First Birthday time. We celebrated Mackenzie’s First birthday on 4/12/2002.
Update – Fall 2007
As I finally get a chance to sit down and redo the whole website, I wanted to add a quick update to the story here. A lot has changed here in 5 years. The Law family relocated in October 2003 to Greenville, SC. Some days it is like living on another planet. Talk about culture shock!! In 2006, we did something we never thought possible, … we bought a house. Mackenzie is 6 and is in 1st grade at Liberty Elementary School. Richelle is currently a full time student at Greenville Technical College, pursuing an AS in Radiology.
Mackenzie never knew her sister, but she speaks of her almost daily. That sure tugs at the heart strings. . We are considering becoming foster parents.
The first update here in a long time, so hard to believe that 12/3/2016 would have been Corleen’s 23rd birthday. I’ve been busy working on some other website updates now that the launch pad website has been recreated here. Recently, with the age of my niece (19) and Mackenzie (15) and the question of them passing Lissencephaly along to their children, we have begun to pursue genetic counseling and testing. We have come to learn that they did eventually find a deletion on the ARX gene on one of my wife’s X chromosomes. This all was news to us but at least provides some hope that we won’t win the lottery this time around with a grand child. The good news is that Mackenzie is not a carrier – so the chance of an affected grand child should be null. 🙂