Joseph's Journey

(The Circle of Life)

On September 12, 1996 we welcomed with joy into our lives Joseph Miller Robinson. He was a beautiful boy with great big eyes and long eyelashes. He had a wonderful disposition and a million dollar smile.

Shortly after Joseph was born, he began having episodes of high pitched screaming which became more and more frequent. We went from doctor to doctor, all of them telling us that Joseph had colic -- the worst case of colic they've ever seen, 'true' colic, etc. We were given advice like "take him home and let him cry" and "give him some tylenol and take him for a car ride." When Joseph was 7 weeks old, we finally found a sympathetic pediatrician who understood that we were not just hysterical parents. He treated Joseph for gastroesophageal reflux, which seemed to help a little, but something still wasn't right.

At 13 weeks, the pediatrician referred us to a pediatric gastroenterologist at Penn State Children's Hospital in Hershey, PA who thought that Joseph had a rectal shelf that was causing his pain. After over a month of laxatives and suppositories, he continued to get worse, so we took Joseph to the emergency room at Hershey. We had prepared ourselves to hear that our little boy was going to need surgery. Much to our surprise the ER doctors ordered a neurology consultation. Joseph was admitted to the hospital and spent the next 4 days being poked and prodded in every possible way.

On February 21, 1997 the pediatric neurologist called to say that Joseph has Globoid Cell Leukodystrophy or Krabbe's Disease. It is a rare genetic disorder (1 in 150,000) which short circuits the central nervous system. Krabbe's is a cruel degenerative disease that is relentless in it's progression. There is currently no cure and life expectancy is 1 to 2 years. 

We first looked into bone marrow transplant and were told that was not an option. We decided to spend as much time as we could with Joseph while he could still communicate. We both took leaves of absence from our jobs and travelled from Pennsylvania to Illinois to stay with Michele's parents. For three months Michele's parents took care of us and we all took care of Joseph.

At 6 months Joseph's mind was still sharp and he was continuing to advance mentally. We could see his frustration as he began to lose his physical abilities. He was increasingly irritable. If you've never spent time with a Krabbe's baby, you don't know the meaning of irritable. Joseph would scream for hours while we did any and everything to try to distract him. We walked, bounced, danced, and flew him around the room like an airplane for hours and hours. He would sleep for an hour and a half at most and he would wake up screaming again.

In April 1997 we were contacted by Dr. Trent Nichols of the Center for Nutrition and Digestive Disorders in Hanover, PA. He had read a newspaper article about Joseph and wanted to tell us about DMSO. A Krabbe's patient of his had been taking DMSO with positive results. Dr. Nichols referred us to Dr. Stanley Jacob at Oregon Health Sciences University.

The first week in May we travelled to Oregon to meet with Dr. Jacobs and learn about DMSO. DMSO (Dimethyl Sulfoxide) is actually an industrial byproduct that is used by veterinarians quite often. It is also used in bone marrow transplants, organ transplants and to treat interstitial cystitis. It is an antioxidant with very few side effects. The only known side effects are an aftertaste -- which Dr. Jacobs has minized by "deodorizing" it, abdominal cramping, gas and loose stools.

We contacted 2 neurologists and Joseph's pediatrician about beginning DMSO and the overwhelming response was "why not?" We had nothing to lose and everything to gain. And after battling chronic constipation for over 6 months we were thrilled that the DMSO might cause loose stools.

Almost immediately we began seeing improvements in Joseph's behavior. He began sleeping for up to 4 hours at a time and his daily medications seemed to be too much. We began taking away his seizure medications until he was completely off of Klonopin. Joseph was obviously more comfortable and he was taking less medication. He began to regain skills that he had previously lost. His swallowing improved and he began to move his arms again. Joseph had swallowing and physical therapy weekly and both therapists documented his improvement.

At this point we felt that we had slowed the progression of the disease and we wondered if it had bought us enough time to have a bone marrow transplant. We contacted Joseph's pediatrician and had a MRI done just to have a baseline and to see where Joseph was.

Michele began contacting transplant centers all over the country and found 6 that were willing to see Joseph and consider him for transplant. Dr. Joanne Kurtzberg, Director of Pediatric Bone Marrow Transplant at Duke University, has transplanted 2 krabbes babies and they are both alive and doing well. After several telephone conversations with Dr. Kurtzberg we traded in our cars for a minivan and packed up our belongings and went to North Carolina.

Joseph was now 11 months old and soon after arriving in NC he began to go downhill again. We met with a neurologist at Duke who put Joseph on phenobarbital. The phenobarbital took away what was left of Joseph's personailty and left him groggy or sleeping most of the time. We felt we had to use this drug because if Joseph did have the transplant, it could be given in IV form to control seizures and the dosage could be easily adjusted.

We went ahead with pre-transplant testing, but it was becoming obvious that a transplant was not an option for Joseph. When we saw the results of the MRI from August next to the ones from just a month before we were devestated. Joseph's brain was continuing to deteriorate at a rapid pace. Apparently the DMSO had helped to control the inflammation in Joseph's brain and given him a better quailty of life, but it did not slow the progression of the disease. We talked with Dr. Kurtzberg and decided not to attempt a transplant.

The first week in September while we were still in NC, just a few days before his first birthday, Joseph began having difficulty swallowing. He would cry because he was hungry, suck on a bottle or take food in his mouth, but not be able to swallow. Over a period of 3 days we tried thickening liquids and different ways of delivering food and were only minimally successful. Before that week we had been against the idea of a feeding tube, thinking that by the time Joseph needed one he would be in a vegatative state. Joseph was very alert and he was communicating very clearly that he was hungry and he wanted to eat. He was also still irritable and prone to seizures and we were unable to get his medicines into him by mouth. On September 4th a g-tube was placed in his stomach.

The surgery and recovery went very well. We continued to offer Joseph food by mouth and let him suck on things, but we usually had to suction them back out. With the g-tube we were able to give Joseph more food and we began having more problems with gastrophageal reflux. We started Joseph on Prilosec and were very pleased with the results. Prilosec has not been recommended for use by small children due to possible long term side effects. But by this point we were used to the fact that none of our decisions were easy, we always had to choose the lesser of the evils that were offered to us.

September 29 we returned to Pennsylvania with heavy hearts. We knew that a bone marrow transplant would have been the only possible cure and Joseph was coming home to die. While we were in NC Joseph's respirations began to drop as low as 5 per minute when he was sleeping. We assumed that the disease had progressed to the control center in his brain and he didn't have much time left. We began measuring time by holidays. We wondered if Joseph would make it to Halloween.

We were pleasantly surprised when we were able to dress Joseph as a blue M & M for Halloween. We then looked to Thanksgiving, and then Joseph spent his second Christmas with us. Joseph's body, not to mention his will, were incredibly strong. We discussed what we would do when he died, made funeral plans, etc. and then made the decision to live. No more waiting to die, we were going to have some fun.

Joseph loved to look at lights, Christmas lights were the best! So in February we took Joseph to Las Vegas to see lights. We stayed at a place called Circus Circus. It was very colorful and there was an indoor amusemet park. Joseph rode on the carousel and opened his eyes wide to take everything in. There were slot machines everywhere so one time we stopped in the lobby and took a seat. We put Joseph's hand on the button and helped him push it. He watched the wheels spin then we heard "clink, clink, clink. . . " He won $20.00! Before we could try again a security guard informed us that minors were not allowed in the gambling area.

Joseph went from needing oxygen while sleeping to needing it all the time. Taking him out to go to church or a doctors appointment was a major undertaking. We had to travel with a diaper bag, suction machine, and now oxygen. This was also around the time that the hiccups were becoming a serious problem. Joseph would get the hiccups for days at a time. When he had the hiccups he couldn't eat (even through the tube) or sleep. He would become exhaused to the point that it looked like it was possible that he would die from the hiccups.

Some of the things we tried for the hiccups include putting sugar in his mouth, ativan, thorazine (IM), phenerghan suppositories, compazine suppositories and electronic accupuncture. When they were first introduced each of the methods worked for a while, then he seemed to become immune to it and we had to start trying combinations of the above medications. It got to the point that we were threatening Joseph's respiratory status by medicating him for the hiccups. The only thing left was for him to hold his breath, but how do you tell a baby to hold his breath? We took a syringe and put water in it and squirted some in his mouth. He would gag and sputter for a few seconds, then the hiccups would be gone. But like everything else, that stopped working after a while. We tried doing the same thing with juice, knowing it was more dangerous if it got in his lungs, but the flavor seemed to get his attention and he would cough and sputter and get rid of the hiccups. Everyday, sometimes several times a day, we would have to decide if we should take the chance of causing pneumonia by putting juice in his mouth or let him have the hiccups for days.

March 11 we began suctioning out something a little darker in color than we were used to. By the next morning we were suctioning blood and Joseph was passing coffee ground like stools. By the time we got to the pediatrician Joseph was bringing up bright red blood. It was decided that Joseph had either developed a stress ulcer or the lining of his stomach was inflamed and bleeding.

On the way to the hospital we noticed that Joseph was mottled (blue in color) up to his elbows. We thought this was it, time to say goodbye. Joseph was admitted to intensive care at the hospital and given Carafate to coat his stomach. He responded well to the treatment and the bleeding stopped almost immediately. We struggled to keep his body temperature up near normal. We also discovered while in the hospital that Joseph had pneumonia. Joseph was 17 months old and this was his first illness other than Krabbes and an ear infection when he was 7 weeks old. He was one tough little guy.

After over a week in the hospital Joseph went home again. He was having a slow recovery, but he was getting better. It was becoming increasingly difficult to keep his body temperature up, but otherwise he was moving in the right direction.

The night of April 3, 1998 Joseph slept well. When he woke up the next morning he opened his eyes wide. He had a big breakfast (though is tube) and kept it down. Michele read his favorite book "Brown Bear' to him and he looked at the pictures and his eyes always went to the eyes of the animals on the page.

Joseph laid down for a late morning nap with Grandma and Grandpa Miller hovering over him, stroking his head and hands. Peter noticed that Joseph's oxygen levels were low even with oxygen on. When he woke Grandpa and Grandma gave him a bath and dressed him. Grandma rocked with Joseph a little then Michele took over. When Michele picked Joseph up he opened his eyes and looked at her and then he bit her cheek as he always did (he wouldn't do that with anyone else). Peter called the pediatrician who told us to bring Joseph to the emergency room for a chest x-ray to see what's going on. We were not concerned that Joseph would die this time. This had become our routine and Joseph had looked worse in the past and survived. Peter began packing the van while Michele rocked Joseph.

After rocking for about 15 minutes Michele thought that Joseph had stopped breathing, but it was difficult to tell because at times he would breath only 3-5 times a minute. She asked her dad for a stethoscope and when she put it on Joseph's back she knew. Joseph died at 1:30PM April 4, 1998. There was no struggle, no last gasp. One minute he was here, the next he was gone.