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We are most pleased to present this detailed, touching presentation from Cheryl Hendrickson, Founder of the Beckwith Wiedemann Syndrome Foundation (BWSF), in detailing the struggles by her most-impressive and courageous son, Cody, with Beckwith Wiedemann Syndrome. One can only marvel at their strength and the wonderful caregiving by Cheryl and her husband, Steve.

Due to complications during my pregnancy, I was followed quite closely with several tests, amniocentesis & hospitalizations. During my 7th month of pregnancy, it was learned that my baby, a son, would be born with a rare disorder called "Beckwith". I was told it was an overgrowth syndrome and that he would be "mild to moderately retarded but would be given glucose immediately at birth and he would have a 95% chance of being ‘normal’."

At each ultrasound, his size contradicted my conception date and at first it was thought he would be born in the middle of March, 1993, then the middle of Feb. then early Feb. and Cody decided on Jan. 23rd, believing now to be 3.5 weeks early, to join the world at a whooping 11pounds! Cody was put in the NIC unit amongst other "premies" yet when asked why Cody was in there, folks would cock an eyebrow to my response of "Oh, he's a premie"! Cody was so large that he couldn't fit in the isolate and had to be put on a table!

As this was an "overgrowth" syndrome, not only was he large in size and length, he also had a condition of Macroglossia in which the tongue is enlarged and had Diastasis recti [separation of the rectus abdominis muscle into right and left halves] caused by enlarged abdominal organs.

Expecting to be hypoglycemic, we delivered at the University of Washington Medical center as they were better prepared to handle the birth. Cody's glucose was "2" and he was immediately whisked away and through IV's, was administered glucose. Unfortunately, the amount he needed was so much that it would destroy his little veins and surrounding tissue, so they inserted a "PAL" line through the inner fold of his elbow and threaded to his heart. For the next month and a half, this line administered the medicine he needed to maintain a "normal" glucose level yet each time his body seem to be adjusting and the medicine reduced, his sugars would drop and more medicine was needed to balance out. As he wasn't having success with the reduced meds, they offered us a choice of surgery or steroids and wanting neither, we asked for any other option.

They told us of a doctor at Children's Hospital, Dr. McEvoy, that had success with severe Hypogycemic/Hyperinsulinism and we decided to meet with him. Dr. McEvoy told us about using a "growth inhibitor" called Somatostatin. He told us we would need to give this through injection 3x's a day and check his glucose at first...every 3 hours. If we agreed to this, and after teaching us to administer these shots, they would discharge Cody. We did and on March 11, 1993, we took our little ;) guy home. Steve gave Cody the injections every 8 hours to the minute and I checked his sugars. If Steve missed the 8 hour mark, Cody's sugars instantly started to drop. This continued for the next 8 months until his body started rejecting the medicines and surgery would now be a life saving plan as his sugars were drastically starting to drop to the low 30's and continuing. At 10 months of age, Cody underwent a near total, 95% removal of his pancreas. We were told that his pancreas was enlarged to that of an average 9-year old. During this surgery, as BWS has a predisposition to cancer, we asked the surgery to "check around" to make sure everything was okay while he was "in there". In his exploration, he found a small, pin-head sized growth on the liver and its origin was unknown so it was sent to Dr. J. Bruce Beckwith (namesake of our syndrome and also who discovered SIDS) at Loma Linda University. I spoke with Dr. Beckwith and he told me the growth was that of the Hepatoblastoma, an associated malignancy of BWS, and that he had never seen one so small and said we had a wonderful surgeon for finding this. As this type of malignancy is a risk until the age of 4years, we sat on pins and needles as we tested Cody every 3 months (recommended protocol at that time) until the age of 4 and thankfully, nothing ever came of this. :)

As Cody grew, we noticed he had hemihypertrophy, where one side of his body was larger than the other. He left side was considerably larger yet his left leg was always shorter and he required a lift in his shoe as the difference would cause great discomfort in his legs, knees and back and even as early as preschool, he would often not play with the other children but rather sit and watch as his legs hurt him too much with movement. His leg discrepancy increased in time and soon he needed to have a lift on the outside of his shoe as it would be too much to be inside. This eased his pain yet he still couldn't do distances and even a trip to the mall would require a wheelchair rental. Jumping ahead to December 2006, Cody underwent a wonderful surgery in which he only had two months left in the "window of opportunity" to be successful. He had the growth plate stopped above his right knee at Shriners in Portland, OR. By March of 2007, Cody began a growth spurt and grew 4 inches over the next three months. Now at 5'9", his legs are "even" and he no longer wears a lift nor complains about his leg pains! :)

As BWS has a risk of tumors with Wilm's tumor of the Kidneys and Hepatoblastoma of the Liver being the two most commonly found, Cody had to be screened every 3months by complete abdominal ultrasounds (until the age of 8) and the Alpha Fetoprotein (AFP) blood test (until the age of 4) to monitor for any growths. Although he never experienced any malignancies, we were told that most children who underwent a "near total" pancreactomy end up going diabetic early in their teens due to the lack of insulin.

At 6 years of age, Cody started showing signs of diabetes by having a constant, unquenchable thirst and constant, high volume of urine output. We were sent to the endocrinology department at Children's who tested Cody for every form of Diabetes and then tested him for Diabetes Insipidus which was of the Kidneys. Baffled by all the negative results, I contacted Dr. J. Bruce Beckwith and asked him if he had any thoughts. His response was Medullary Sponge Kidney Disease. Dr. Beckwith contacted the endocrinologist and spoke of his suspicions and we were then sent to a Nephrologist and under the IVP [intravenous pyelogram] kidney scan, in September of 1999, Cody was officially diagnosed with MSK [renal cystic disease]. :(

We were told both of his kidneys were affected and while we couldn't cure this disease, #1 it is manageable and #2 if one is to have a kidney disease, this is a better one to have. Cody was put on a Hydrochlrothiazide and has been taking it since he was 6 years old. Now at age 15, he takes 50mg a day and is doing great! In time, his kidneys could have more issues and may require dialysis or transplant but that may be years down the road and a bridge we will cross should we ever get there. I am hopeful his medical regime will keep him going for many years and avoid any treatment. :)

When Cody was in 3rd grade, his hearing became worst. We knew he had high tone hearing loss and I made the mistake of thinking he didn't need to hear the list of things they said he was missing like that of a dripping faucet or the rustling of leaves. My hope was to not to add any more to his "medical life" than he was already going through and let him just be a kid having fun in his youth. As he started getting louder and things needed to be turned up more, we took him in to be fitted for hearing aids. When Cody received them, his little eyes lit up in wonder at all the sounds he never heard, ones that I always took for granted and never thought he would miss them. As we were walking from one office to the other with the audiologist and Cody wearing his new aids, I whispered from behind Cody..."I love you Cody" and he turned to me with his nose scrunched up and said..."MOMMMMM!!!" as if I had embarrassed him!! LOL A tear came down my cheek as this was the first time Cody could hear my whisper! His appt. was on Friday and Cody couldn't wait to go back to class to share his new aids with the kids. He had fancy ones -- he got to choose the color of the mold and the ear piece AND...they came with a remote that he could adjust for quieter or louder rooms and one little button that would turn them off so he couldn't hear things. When the other kids heard that, they eagerly asked if he could even turn off the teacher and he replied..."Yes, but mom said I can't do that"! ;)

On Saturday, it was a beautiful day and all the birds were singing. I went in and got Cody and told him to turn off his aids and come with me. He did and I took him outside and we sat in the chair on the back porch. I asked him what he heard and he said "Caw Caw" as the crows near by were calling out. Then I told him to turn on his aids and his eyes widened and the expression on his face is one I will never forget. I asked him what he heard and he said "BIRDS!! LOTS OF BIRDS!!!" They were all chatting up a storm and singing their beautiful songs and together Cody and I sat there and listened for what seemed eternity! He was full of excitement and I full of bittersweet tears for realizing what I withheld from him for all those years thinking they were sounds he wouldn't miss. :(

When Cody was 10 years old, he underwent a glossectomy or tongue reduction procedure because the enlargement was taking a toll on him and he had to wear a C-Pap constantly [devices and masks commonly used with sleep apnea]. Upon a sleep study, it was noticed that Cody's airway was even further compromised as the tongue would fall backwards into his airway blocking it even more. We went to St. Louis which is the center of excellence for BWS and Dr. Jeffrey Marsh performed his surgery. Dr. Marsh has performed about 200 successful glossectomies on Children with BWS and the only one we trusted for this surgery. Cody had a hard time with recovery because being 10 years old, it was like he lost a friend and for a spell he had a bit of depression. Now though, no one even notices his tongue and he hasn't worn a C-Pap since!

Cody is now 15, in high school and looking at him, you would never know the life he lived when younger or that his kidney disease and his hearing loss, both progressive, remind him everyday that he has a syndrome that few have ever heard of. We don't talk about it like we did when he was younger as he just wants to be "Cody" and while he still goes to his many medical visits that include a Pulmonologist, Endocrinologist, Nephrologist, Audiologist, Oncologist, Orthodontist, Orthopedic, Urologist and the list goes on, he is a happy 9th grader that now is having the "normal" adolescent issues...mainly of girls!

As the lack of information about BWS was on going, I joined the network in 1995 and in 2002, branched off and formed the Beckwith-Wiedemann Children's Foundation. We now assist children worldwide and are bringing awareness to this often unidentified disease that affects 1 in 14,000 children. Cody was and is my driving force!