Dakota Jean Bihn was born on May 30, 1999. She was a beautiful baby, weighing in at exactly 8 pounds. Everything about her seemed perfect. During Dakota’s first year, she developed as a normal baby should. She sat up, rolled over, ate, and walked, all when she should. Other than a little reflux now and then, she was a perfectly normal baby.
At around 2 years of age, I noticed that when she tried to talk she stuttered a bit. I was a little concerned, and brought it up with her pediatrician who told me that this sometimes happened as children began to speak, and not to worry. My husband’s brother stuttered as a child so it was definitely on my mind a lot.
Dakota’s toddler years continued on, she was a normal, healthy, happy, sweet little girl. At her three year old check up she continued to stutter. The pediatrician recommended we take her to the school system to be evaluated. Around this time I was also potty training her. She proved to have a difficult time with this milestone. Though she seemed to try hard, accidents occurred frequently.
Eventually we did have her speech evaluated at the schools, and they proceeded to tell me that though yes, she had a severe stuttering problem, it was not enough at her age to be seen by the school speech therapist. They gave us some work to do with her at home, and told us to come back to be evaluated again in a year. In the fall of her third year she began going to preschool a couple times a week.
At different times throughout the year it became very apparent to me that she was just a little behind developmentally then her peers. She also startled very easily-very afraid of balloons (for fear of the popping), and seemed to sometimes not realize someone was near her, suddenly startling when they spoke to her. Despite this, she seemed very happy, and kids and adults alike seemed to love her and be charmed by her.
I knew in my heart something wasn’t quite right
By the time Dakota turned 4, I really started to see that she was behind her peers developmentally. While I tried to brush it off, thinking kids develop at their own pace I knew in my heart something wasn’t quite right. A year had passed since her first speech evaluation, so I called the schools again for another speech eval, and asked them to test her development as well. I knew her fine motor skills were not where they should be, and was worried about her in general. This time, the school psychologist and speech teacher told me that yes, she was behind. They wanted her to switch from her current preschool to the school’s intervention preschool program as soon as possible. It was almost Christmas break at this point so we decided to switch after the holiday.
The new preschool seemed to be the answer. They addressed her speech, fine motor, and gross motor problems, working on whatever they could to help and encourage her. Though we saw tiny improvements, we (my husband and I, the pediatrician, and her teachers) agreed at the end of the school year that she was still behind, and that we should wait another year to begin kindergarten. Her pediatrician also gave us the name of a neurologist so that we could be sure that her problems were not more than just developmental. Our next door neighbor is a radiologist, so we consulted with him as we knew an MRI was in Dakota’s future, and opted to see a neurologist at his hospital, Cleveland’s Metro Hospital.
The neurologist, Dr. Rizkallah, was sweet and kind. He scheduled an MRI, hearing and vision screenings, and also a genetic consult. We found no problems with any of these appointments. Results were normal in all these areas. The geneticist found nothing remarkable about our genetic history. When we met again with the neurologist, he said he agreed there was something not right with Dakota, but they had done all they could at this hospital, and recommended we see a neurologist at the Cleveland Clinic. There was a specialist there that he felt she should see, but we first had to go through a pediatric neurologist there.
Off we went to meet with Dr. Friedman. He ran blood tests. We waited. He scheduled another MRI. We waited. He scheduled an EEG. We waited. After each test we were continually told that the results were unremarkable. Let’s wait three months and come back. Like idiots we continued on this path for nearly a year.
She was unable to do things she once knew how to do
In this year, we not only knew Dakota was greatly lagging behind her peers developmentally and physically, but started to realize she was unable to do things she once knew how to do. She couldn’t jump from the curb on the sidewalk. She began to communicate less. Letters and numbers that she once knew she struggled with. Concepts she learned and supposedly mastered in speech, she was suddenly dumbfounded by. Clearly she was regressing.
At this point she had had a urine test and something came back not quite right so we were sent to a Nephrologist. This doctor was also sweet and kind and told us nothing critical was wrong with Dakota’s kidneys, and the test results meant nothing significant, but she could sense our frustration and almost disappointment at again having no real answers. She recommended a doctor that she felt could help us, a Dr. Marvin Natowicz. Well this was exactly the person that Dr. Rizkallah wanted us to see that Dr. Friedman would not send us to. The Nephrologist told us to go home and make an appointment right away and she would call him personally about Dakota’s case. Finally we were getting somewhere.
At this point Dakota had started Kindergarten. Though kindergarten is only a half day in our school system, we had decided that she would go to kindergarten in the morning and a special education classroom in the afternoon. I was a very involved mom, going to the school to help as much as possible, and I must say it pained me to see how behind Dakota was from the other students in her "normal" kindergarten classroom. Though she always seemed happy, she also often looked lost to me. This look saddened me every time I came to help. Luckily she was supported by two wonderful teachers and an incredible aid.
Never in a million years did we think it would be so serious
We finally met with Dr. Natowicz. He was a sweet man who had a gentle way about him. He asked us very thorough, thought provoking questions about Dakota and about our families. He ordered lab work and suggested another eye exam. The eye exam came back normal, however after weeks of waiting for the results of the lab work, we finally got a call from the doctor. He had the results of the blood work and wanted to see us again. He suggested we come without Dakota. We knew the news must be serious. Never in a million years did we think it would be so serious.
On September 1, 2005 we received horrible news about Dakota. She was diagnosed with a rare disease called Juvenile Onset Tay Sachs. This disease is very rare and terminal. We could not believe this news. Our hearts were breaking as the doctor explained what life with this disease would be. Dakota would eventually loose her sight, her ability to walk, she would have severe seizures, and eventually loose her ability to swallow. He explained this all to us with tears in his eyes. He gave us the name of the National Tay Sachs and Allied Disease organization, and we had to go have our own blood drawn at the lab to help determine our carrier status. I can barely remember driving home that day. It was the worst day of my life. We had to stop at the school to pick up Dakota and her sister, Bailey. Ken thought enough to call ahead to have the school get the girls ready and waiting for us. It was clear to everyone at the school that afternoon that something was terribly wrong.
We had to call our family and tell them this horrible news. We had to call our friends and the people at the school and explain what we ourselves could not comprehend. We had to talk to our oldest daughter and explain to her why all the adults around her were so sad. How do you explain this to an 8 year old? Our happy life as we knew it had suddenly ended. We knew nothing would ever quite be the same again.
We did all that we could to educate ourselves
After recovering from the initial shock of the diagnosis - and this took weeks - we did all that we could to educate ourselves on how best to help Dakota. We joined the National Tay Sachs Foundation and learned that there was another family in nearby Amherst who had a 4 year old daughter, Lexi Markowich, who was also recently diagnosed with the same disease. Since there are only about 100 known cases of this disease, this was pretty amazing. The other little girl and her family had learned about cord blood transplants being done on patients born with Tay Sachs at Duke University Medical Center. We eventually met up with Lexi's family and also looked into going to Duke to find out more about the transplants.
We went to Duke to see Dr. Joann Kurtzberg and we were very impressed with her and the doctors we met with. They were caring, professional and organized and were the first group of medical people who could really tell us what we needed to hear about the disease. The news wasn't good, and the decision didn't come easily but after running many tests on Dakota, and being told she could be a candidate we decided to pursue doing the transplant. Unfortunately things didn't go too smoothly with our insurance company, but we eventually won that battle and were able to move forward. We were able to enjoy a week at Disney courtesy of the Make A Wish foundation, making such wonderful memories with our family.
On January 3rd, 2006, Dakota and our family embarked on our new journey. We began the process of a cord blood transplant. Weeks of testing, and Dakota had central lines placed and entered Duke Hospital on Jan 17, 2006. She began a 10 day course of chemotherapy to kill her existing blood, so that she could be transplanted with new blood, rich in the enzyme, Hex-A which is the key element missing in Tay Sach’s children. Dakota received this transplant on January 27th, 2006, the day she started a new life. We knew this was a journey of hope and possibilities, but also one with no guarantees.
After the 10 days of chemotherapy, came the waiting game. We waited as the new blood cells set up shop and began to make new white blood cells. During this wait Dakota lost all of her hair. She had many episodes where she vomited blood and suffered from severe diarrhea. She went through a period where severe blisters began to surface on her skin. The blisters were on her butt, shoulders, elbows, hands and back. They were so severe she looked like she had been burned. The scary part was that the doctors had no explanation for these blisters. She was seen by the head of dermatology, and infection specialists. She had two skin biopsies. Over time, the blisters did heal, but the doctors never really knew what caused them and only guessed that they were an after affect of the chemo.
Every day we would try to get Dakota up to walk in the halls of the transplant unit. This proved to be more difficult than we expected. Dakota had many days where she was so weak she could barely walk a few yards. Some days we were happy to just get her standing in her room for 15 minutes at a time. Dakota loved to do art and music therapy when she was able to and this too allowed her to get up and out of her hospital room. The transplant process was slow. After nearly 2 ½ months in the hospital, Dakota was released in March and we spent another 5 months living near Duke while Dakota was recovering. During these 5 months she went to the transplant outpatient clinic several times a week for blood transfusions and check ups. She also received physical, occupational, and eating therapy.
In August Dakota was finally strong enough to return home. She was and is not the same girl who walked into the hospital. She no longer can walk without assistance. Her speech comes less freely then it used to, but she still talks and is talking a little better than she did a year ago. Her hands tremble some when doing fine motor work such as turning pages in a book or coloring. Though it is sometimes hard for us to see these changes, the essence of Dakota is still there. She is still a sweet, loving little girl, who is devoted to her friends and family. She can give kisses and sing “You are my Sunshine” with you. She loves Belle, Minnie Mouse, listening to music and her sister. Her smile can light up a room, and turn your gray day blue again. Her laughter can be contagious. Her strength and determination amazes all that know and get to know her.
While we were at Duke, The Cleveland Plain Dealer did a series of articles about Dakota and Lexi Markowich (www.cleveland.com/taysachs/).
When the article came out, we hated to admit that we were disappointed about how the article portrayed our families. We felt in the end it had a negative tone, that we pursued a hopeless, cruel treatment for our children. It focused on all the negative aspects of the transplant process rather than focusing on the hope for a cure for this horrible disease. It did not focus on the love our families had for our daughters or the support our community showed us. It did not show the determination our daughters showed us, their beautiful spirits, the support we gave each other as families. Though the article gave some good information explaining the disease, we ended up feeling frustrated with how it portrayed our daughters and our families.
We know there are no guarantees with the transplant we chose to have for Dakota. Our greatest hope was that it might stop the progression of the disease, or at least slow it down enough until a real cure or a better treatment could be found. Medical science has come so far in even the last 10 years and we know research is close to solving the Tay Sachs puzzle. We hope and pray that enough money can be raised to continue the research so the cure can come soon.
Dakota continues to fight this disease on a daily basis. There is not a day that goes by that we don’t see her smile, or hear her laugh. Her days and weeks consist of various therapies-physical, occupational, speech and swim therapy. We are surrounded by friends, family, teachers and therapists that see how joyful Dakota can be, and who know how hard she works at any task given her.
Julie Bihn, July 2007
For more information, please visit www.caringbridge.org/visit/dakotabihn