Haley's Story
Haley was born December 8th, 1996. The pregnancy and delivery was uncomplicated. In fact, Erika even slept through 90+% of the delivery. Haley was the dream we asked for. Things were going great. Haley was developing fine and we didn't think anything could possibly go wrong.
On February 8th, we took Haley to the doctors for her check up and her two-month shots. She had a mild cough, but hadn't had a fever so we never thought anything of it. Our doctor decided to hook her up to a pulse ox and she discovered that she was only had a pulse ox of 84-87%. She sent us directly to the hospital where she spent 4 days to have breathing treatments and antibiotics for an ear infection. We finished the antibiotics at home and continued with breathing treatments. She did great. After the first antibiotic she developed another ear infection and we were on this roller coaster until May. We were told that the time of year that she was born made it hard for her to get over colds. We spent long nights up with Haley thinking that the medications were keeping her from eating. We also thought that she was continually crying because she was always hungry.
We finally had enough and said no more. Our doctor sent us to Denver Children's Hospital for a GI Test. They discovered that she had severe reflux. However, during the test it did not show that she was aspirating. She appeared to be able to stop herself from aspirating. But then the question, why does she have the wheeze, kept coming up. Our doctor decided to have a Bronchoscopy done. It showed that she had weakened airways but very mild and that she definitely aspirates. They suspected that her airway weakened while sedated (or asleep) and then she wasn't able to stop aspiration. They put Haley on propulsid and Tagamet to stop the reflux. It seemed to worsen everything. She would scream in pain uncontrollably and wouldn't stop. We called the doctor and told her that we were taking her off these drugs. That's when they started talking to us about doing stomach wrap to stop the reflux. Our Pulmonologist asked us about Haley's development. That was the beginning of our discovery of Krabbe's.
We
had explained that Haley had stopped doing some of the things that she had once
done. She no longer was playing with her toys; she no longer was trying to hold
up her head, and was starting to become less active. It all had started at about
4 - 4 1/2 months of age. Our pediatrician wouldn't let us do anymore until we
saw a neurologist. On June 19th, Erika took Haley back to Children's
and within 5 min. of seeing the doctor he was able tell that Haley had a severe
disorder. They immediately had me paged out of work and sat us both down and
explained that Haley had a terminal disease. They would not be able to limit
down the number of different diseases without doing a MRI. We did the MRI on the
following Monday and that same day they were able to tell us that Haley had one
of the Leukodystrophies. He gave us a list of about 4-6 different
Leukodystrophies and we took them home and studied all of them while we waited
for an answer from the blood tests.
Meanwhile, Haley was loosing weight slowly. With the combination of the severe reflux and the fact that Haley was loosing weight slowly, we added the fact, that at six months of age, Haley was still getting up every hour to an hour and a half. We decided to go ahead with a G-Tube and the stomach wrap. We were skeptical but we knew that Haley truly was in very good health and if we do it we would only use the G-Tube during the night and we would feed her orally throughout the day. The surgery was scheduled for mid July. The surgery went great. They went straight for a button that really pleased us and the scar that was left was remarkably clean. We were pleased.
While we were waiting for Haley to have her first meal, post surgery, she had developed a severe rash. Most likely, the rash was caused by some medication she had while in surgery or immediately afterwards. The rash lasted weeks and nothing we did helped. The rash had to run its course. The rash was not only red but bright red. It would travel across her body until it effected every part of her body. The hard part was that along with this rash came severe, painful swelling. Once again, nothing would relieve it. The rash finally subsided in about two to three weeks.
All
of that was the easy part compared to what we really lost. Before the surgery,
Haley was very responsive. She would not only be very aware of her environment
but she would laugh and smile frequently. She loved us to play with her and had
far more tolerance to times being left alone. After the surgery, she no longer
smiled, she rarely paid any attention to her environment, and now she won't eat
at all orally. At first we thought that maybe when all of the medications wore
off that she'd come back. Then we thought that when the rash went away that
she'd come back. But now we realize that none of that was true. We are still
trying to get her to do all of the above, however it gets frustrating when there
are no results. Haley has improved very slowly. She does give a rare half smile
but you can see it in her eyes. She is slooooowly starting to be more tolerant
of being left alone.
We found CJ's web page in mid-June but never contacted them until July. We didn't know if Haley had Krabbe's or not so we waited until we got confirmation. They have been exactly what we've been looking for. Not only have they been supportive, friendly, and easy to get along with, but also they have also been informative and have enabled us to contact more people like ourselves.
When
we decided to call Chuck & Jennifer it was a coincidence that they had
written to us that morning and had mentioned a possible transplant surgery. We
had asked about it and she gave us the information that she had. It led us to
Peter and Michelle.
Peter & Michelle were in North Carolina to see to what extent Krabbe's had progressed in their son Joseph. We found out that Duke University had recently performed two Cord Blood Transplants. We called the Robinson's (Peter & Michelle) the next night. They seemed just as excited to hear from us as we were to hear from them. They gave us the scoop on the hospital and the progress that Duke has made with the only two Cord Blood Transplants on Krabbe's patients. Within the next two weeks we had talked with doctors in North Carolina and in Colorado to see if we could get this surgery done for Haley. Within the same two weeks we were able to see the transplant surgeon here in Colorado, have Haley evaluated physically, and have a MRI done. With the proper knowledge we got a lot done.
We chose not to proceed with the transplant because the MRI revealed a significant deterioration since her June MRI. Throughout the time we were pursuing the transplant we were always questioning if we would or should do this for Haley. The MRI just made the decision easier. For more information regarding the transplant and a more detailed process for the transplant please refer to the Cord Blood Transplant site.
Life started to get back to normal. We took a well needed break and left for Florida. On our way we stopped in South Carolina to visit with friends. It was very relaxing and well needed. Believe it or not we drove close to 70 hours total and really enjoyed the trip. Someone was definitely looking out for us.
With the help of "A Special Wish Foundation" Haley's first birthday was perfect. Their gift also enabled us to have one of the most memorable Christmases ever. Haley was such a big girl in her new bed and with all of her new toys.
New Year's had definitely brought new Life. Haley definitely noticed that the stress level had dropped around our house and had become more responsive to her environment. We had originally decided that we were going to put life on hold until everything finalizes with Haley. However, we managed to change our minds and we felt it wouldn't have been fair to Haley. We decided to move and we also decided to try and get pregnant again. These were probably two of our best decisions.
We moved to South Carolina in April, 1998 and Haley found out that she was going to have a little brother. Since our "big" move, Haley has proven once more that she can handle stressful situations. Haley had started Occupational, Physical and Speech therapy, and was responding very well to them all. She was doing a lot of things I never would have dreamed of. The concept of "unconditional love" was really working out to be the most wonderful thing in the world.
When
we moved to South Carolina, we had requested assistance to help pay for all of
the therapies and medications not covered by our medical insurance. Haley's life
here in South Carolina began to mean more than we would ever know. By having
Haley evaluated by the state she has opened the door for other children with
Leukodystrophies. Other children with Leukodystrophies will automatically be
eligible for aid.
After we got here, we were contacted by a family that was waiting for results on their child about Krabbe's. They felt after reading about Haley that they knew what the results were. Because of Haley, we have been able to not only meet this new family but three others. Within a very short period of time we were able to get enough information to this family to get them to Duke University to have their beautiful daughter, Julia, evaluated for a Cord Blood Transplant. The other families, by reading about Haley and Joseph, were also able to get to Duke for Cord Blood Transplant evaluations. Of these four new families, three were eligible for transplant.
Summer was going great. Erika and Haley made a trip back to Denver for a visit and Brytannia and Dad went to Florida for a short vacation. We all came back into our new house and things were going well. Sometime in August we had started to notice that Haley was having brief periods of cyanosis (blueness). We suspected apnea (Short periods without breathing). We called her doctors and requested a pulse oximeter so that we could monitor how she was doing. Our doctor felt that maybe we should try an apnea monitor if we truly felt it was apnea.
The apnea monitor came was OK for the first weekend. It only went off a few times, but then started going off all the time. Haley managed to fill a month's worth of memory in about four days. One thing we noticed is that it was telling us that she was having apnea but we were watching her breath. Obviously we had no confidence in this machine and so we called and asked for a pulse oximeter once again. They sent us another apnea monitor and once again Haley managed to fill its' memory 60% in about 6 hours so we just disconnected it and gave up.
About a week later, we got frustrated and we demanded to have a pulse oximeter. It was here the next day. It was September 2. When we hooked it we were able to watch exactly what Haley was doing and we discovered that she wasn't actually having apnea but rather cheyne stokes respirations which is where her breathing goes from really fast and deep to really shallow and slow. It's a cycle that just continues. We determined that this was probably just a progression of this disease and that our time with Haley was most likely even more limited.
Two days later, it had gotten extremely worse. Our nurse had come to the house and between all of us we knew that Haley would not make it through the weekend. Her pulse ox would continue to rise and fall. Friday was her worst day where she looked the worst and acted the worst. Her pulse ox had gotten as low as 24% and here heart rate stayed in the 180's and as high as 200+. Saturday came and it was a little better but not much and her pulse ox went as low as 18% but her heart rate stayed in the mid-150's. Sunday came and things appeared to be much better. She was more responsive to us and starting to act normal and look normal. But we did see one period were her pulse ox dropped as low as 12%. Over the next few days she just getting better and better. In fact, they were pretty great. We were of course afraid to admit that she was doing better because that last weekend was so scary.
On Wednesday, September 9th, we had noticed what we thought, Haley had caught a cold. So we called the doctor and had her put on an anti-biotic. She did seem very comfortable. I went to work Wednesday night knowing that she was in the best hands with Erika. Erika was woken by the pulse ox machine around 3:30 going off and beeping non stop. She got up to find Haley's saturation level staying in the 60's. Erika also noticed that she had spiked a fever. Erika gave her Tylenol and tried sucking her out. She had no success. She called me at work and I was on my way home by 4:30.
When I got home Haley was maintaining in the low 60's and her heart rate was over 200. I suctioned her out and started an albuterol nebulizer. Her pulse ox went up to the mid-80 and her breathing seemed to be eased. I looked at Erika and told her that she probably wouldn't go today but she probably had no more than two days. I thought it would be a good idea if we start taking turns with her while the other one got some rest. Erika went off to bed while I spent some time with Haley. While holding her, I got the full impact of how hot she was. I took her temperature and it was 106.3. I rushed her into a bathtub to cool her off and make her comfortable. It worked and her temperature was coming down. I returned to our permanent place on the couch and tried to make her comfortable on my lap. I didn't want her absorb the heat from my body so I was trying to position her on my lap with as little contact as possible. Her pulse ox machine kept going off where the numbers would go blank as if it was a loose connection and I would play with the probe and it would come back. While I was doing all this fidgeting, I noticed that the loose connection light on the pulse ox machine had never gone off this whole time and that the gurgley breathing that she had been doing stopped. I grabbed my stethoscope and listed to her the last time that her pulse ox had gone blank. I knew at that time that Haley was truly comfortable.
I carried Haley in to Erika and handed Haley to her. We shared this moment and cried. We called our home nurse who was more a friend than a nurse and she was on her way. We spent the next two hours at home with Haley saying our goodbye. This was probably one the most memorable and important times we've ever spent with Haley.
We took Haley to the mortuary ourselves and we made all of the arrangements with Haley in our arms. After all was done, I took Haley back so that way Haley wasn't taken from Erika by a stranger.
I gave so many details of Haley's final moments because I wanted to share how important times like these can be. Haley was definitely comfortable. It was a very sad time for our family but we know that Haley is now sharing candy with Joseph in heaven. Even though we are sad and we truly miss Haley, we are happy for her. Her services were held on Monday, September 14th, 1998. The only word I know how to describe her services, is "Perfect". Haley's life was perfect, so it's no wonder why her death be anything else.
In Memory of
Haley "Belle" Justine Sears
December 8, 1996 ~ September 10, 1998
The fullness of our heart is expressed in our eyes, in our touch, in what we write, in what we say, in the way we walk, the way we receive, the way we need. That is the fullness of our heart expressing itself in many different ways.
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