It seems so hard to believe that only a year ago our third child, Laura Margaret, was born. She has been through enough to last a lifetime! Laura was born with Krabbe's disease, the same disease that took the life of our first child, Joshua, just 5 short years ago. This is her story - it is one filled with tremendous hope for the future because she has been given a second chance at life - a chance because a mother was willing to donate the cord blood from her newborn baby. That cord blood was a match for Laura, who received it on August 19, 1999 - when she began her "new life". I am so happy to be able to tell her story!!
When we found out I was pregnant in the fall of the 1998, we were quite surprised. We were happy and looked forward to giving our 4 year old daughter, Olivia, a baby brother or sister. Yet we knew this baby had a 25% chance of having the same disease that claimed the life of our first child. Initially, we decided not to have the baby tested while I was still pregnant. Why run the risk when, regardless of the outcome, we would never consider ending the pregnancy. Several months into the pregnancy, my husband was searching the internet and found websites about Krabbe's disease. I remember the day he learned that bone marrow transplants were being performed on Krabbe children with very promising results! I was napping and he came in and woke me up and told me I just had to come and look at what he had found. I turned over and said I would read it later. No, I had to read it now! It is hard to express the disbelief, excitement, awe, and hope that emerged as we read about children being given a bone marrow transplant to REVERSE Krabbe's disease! This was not an option when Joshua was diagnosed. We read many of the articles and decided that we needed to know if our baby was affected so that if indeed the baby had Krabbes, we would be ready for the transplant as soon as possible after birth. But, I reasoned, it could not happen to us a second time!
Several months later, I was again awakened from a nap to find my husband kneeling beside the bed. I knew something was wrong. My father had just had bypass surgery and I thought something had happened to him. No, the doctor just called with the results about the baby. She has it. NOOOOOOOOOOOOOO!!!!!!!!!!!!!!!!!!!!!!! Why?!!!!!!!! How?!!!!!!! NOOOOOOOOOOOOOOOOOOOOOOOOOOO!!! I cried the entire weekend and then got up Monday morning and began making calls so that my baby could live. We initially talked with two doctors who gave us so much hope for little Laura (as she was now named). We then met a doctor here in our hometown of St. Louis who was very excited about helping us. We made the decision to have the transplant done here. How I thank God for the doctors who made it possible for us to actually have options about Laura's treatment! 7 years ago we were sent home with our dying son and told to make him as comfortable as possible. I am so glad that we found out about Laura's diagnosis before she was born. We were able to have everything in place for the transplant and had time to educate ourselves about the procedure. We were prepared (as much as anyone can be prepared for it!!). I think being able to have everything in order brought a sense of stability in a world that was soon to be turned upside-down.
Laura came 5 days past her due date in the wee morning hours on July 31, 1999. She was born 20 minutes after we sped to the hospital (I almost had an unplanned homebirth - my first 2 were planned!). I think God knew I needed an easy labor so that my recovery time would be short (there was a lot to do), but this was ridiculous! She was 7lbs. 11oz., 19in, and looked the picture of health! We treasured every minute of her time home with us before the transplant. She was a calm, peaceful baby - so very different than my son. I found myself hoping that the diagnosis was a mistake. One week after she was born, pre-transplant tests were run and we received confirmation of the diagnosis (Laura's own cord blood was used for this and to confirm her HLA typing). When she was just 9 days she entered the hospital, had a central line placed, and began the chemotherapy that would destroy her own bone marrow. She was as brave and good as a baby could be. I, on the other hand, was a basket full of tears, weeping and sobbing, the post-partum blues in full swing! The 10 days of chemotherapy were very emotionally difficult. Knowing that everything the nurses were giving her was going to make her weaker and weaker made me dread their entry to Laura's room. Yet Laura did amazingly well. She slept much of the time and continued to nurse well. It was hard to believe the chemo was working. But as we began to see her white blood cell count drop, reality set in. She slept more and more. When the chemotherapy was almost finished, the central line stopped giving blood! She had to go into surgery and have another one placed.
Chemotherapy was then finally finished and after one day of rest, transplant day arrived - August 19, 1999! I looked forward to knowing that everything from here on out was done to make her better and stronger. The week after transplant is called the "dog days". Laura's were relatively mild though she did stop nursing for about 5 days but still took bottles of my expressed milk. In about 7 days her white count starting going up. And if never came back down! It shot up so quickly and she was doing so well that she was sent home on day 13 after the transplant! Though it was exciting for her to break the hospital's record of shortest BMT stay, I feared something just had to go wrong. This seemed just too easy. Little did I know her worst days were still to come. She had been home for about a week when she started having difficulty nursing. She nursed for shorter and shorter periods of time and began vomiting quite often. Back we went to the hospital for the weekend so that she could be monitored. We went back home on Sunday but I had a hunch we would be back. On Tuesday, she was to be seen in clinic. Once there, she proceeded to vomit after every feeding. When her doctor was listening to her heart, she thought she detected a murmur and sent Laura for an echocardiogram (the first of many). When the technician saw her heart he asked if she was on steroids. Yes, like all transplant children to keep graft-vs-host disease under control. Nothing more was said until we got the news that she had major steroid induced heart damage. The cardiologist said that no more steroids under any circumstance were to be given to her - not even to wean her off. She went on a heart medication to try to relax the heart muscles so that hopefully the thickening of the heart walls would gradually return to normal ( I heard the word hopefully - there was a chance that this damage was permanent). Because the steroid was pulled, the only medication to control Laura's GVH (graft-vs-host) was cyclosporine and it could not do the job. Laura's GVH made her so sick that she was in the hospital this time for 6 weeks. She was so weak she could no longer even eat (what she did eat she immediately threw up - she often even threw up bile - her own stomach fluid). She was on TPN (an IV nutritional source) for over 3 weeks. She had severe diarrhea. Her skin was deep red and blistered and any body hair that was left after the chemotherapy was now gone. The blisters on her hands were dark purple and we eventually had to bandage them so that she would not suck on them and hurt herself. She was so weak that she would lay in my arms most of the day and sleep. One thing that helped us through these days was the fact that Laura had so many wonderful nurses that we could trust. They loved and cared for her so much. Many days she had a nurse all to herself so that I could go home at night and have a good night's rest. Often these nurses would sit in Laura's room and rock her and hold her while she was sleeping. We also had the baby monitor on so that when they were outside her room at the desk, they would hear any little peep she made. I remember one morning when I came in, I was overwhelmed at how terrible and weak Laura looked. I broke down and cried so hard (I usually saved this for the ride home at night). The housekeeper, Clara, (who became my older daughter's very good friend) came in and hugged me and prayed with me for Laura. "Laura is going to make it! Don't you worry - she is going to make it!" Clara would tell us everyday. There were days when Laura's breathing was so fast that I thought her body could just not do it anymore. Several days after her GVH flew out of control, the doctors started another treatment that would help her fight off the GVH. It took some weeks but the treatments started working. Gradually her skin began to heal and then her stools returned to normal. She was still very weak but seemed to be on the upswing. Her heart condition was still very serious though. The medication caused her heart rate to go so low (sometimes down in the 50s!) that they decided to change to a more commonly used one. Laura seemed to handle this one much better though we would eventually have to change to a third medication (Verapamil) to see some significant improvement. Echocardiograms became routine. Slowly Laura resumed nursing. Weight gain was a problem though. At 4 months she still only weighed about 8lbs!
In October, Laura finally got to come home with us! It felt so wonderful to have our whole family together again. Our days gradually developed into a routine and Laura continued to do well. At about 5 months, Laura began gaining weight (I had been wondering where all the milk had been going - she nursed very often!). Around this time too she began to get stronger. Her fine motor skills were developing (passing toys from hand to hand, etc.). She seemed to really blossom at about 9 months - she was sitting up and began baby talk (dada, baba, a-da, yaya were her favorites). She put her hands together and clapped and could wave byebye. She began interacting with us a lot more around this time as well. We got word also that her heart was officially back to normal! This was a huge relief!
Often when I look at her I think about Joshua, who didn't get this second chance at life. People would try to comfort me with the verse "God causes all things to work together for good" after Joshua's death. I would get so angry because I believed that nothing that would come of Joshua's death could ever be good enough for me. This past year, on the anniversary of Joshua's death, I heard Laura making her sweet sounds when she woke up in her crib. I thought of the fact that it's because of Joshua's death that we knew to have Laura tested. Laura would not be here today if it weren't for Joshua. This is certainly a very big good that has come. And this brings me to the present. Laura just turned 1 and will soon have her 1 year post-transplant party! She continues to grow stronger. My favorite new ability is calling me MaMa! She is able to hold her own weight on her legs (which are now very chubby) and and scoot about in the walker (though still not forward!). She loves to imitate my silly faces, jump up and down in the johnny-jumpup, and loves to play with her big sister's long hair. She is rarely fussy (though she still gets up several times during the night). Plans are in the works for central line removal this week (we kept it in for the immune booster she gets monthly and for blood draws - I am so glad we did this -no needle sticks at all). She is still on cyclosporine and MgCl2 and will be quitting Bactrim this month. MRI results came back last month normal for her age, she is producing 100% donor cells, and the enzyme level (that was only trace when she was born) has come back normal several times now. So all in all, we have every hope for her future! And the most wonderful part of this story is that it doesn't end here. God willing, I will have many more stories to tell about Laura - when she starts walking, when she says "I love you", and when she learns to read!
Thank you for listening to her story - it is a joy to tell! For current news about Laura, please see www.caringbridge.com/mo/lauranews
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