Nicholas Robert Wendt was named after his grandfathers, Nick Velardi and Robert Wendt, who both passed away before Nicholas was conceived. When you meet Nicholas, the first thing you notice are cornflower blue eyes that sparkle like jewels. Chances are you'll be greeted by a huge, toothy smile. Chubby, chipmunk cheeks and reddish-blond hair that defies gravity complete this adorable picture. Nicholas was born 10 months ago to Joanie and Richie Wendt of Valley Stream, NY. He was also born with SCID.
Nicholas in Durham NC, a few days after his bone
marrow transplant
SCID (Severe Combined Immune Deficiency Syndrome) is a rare genetic disorder of the T-lymphocyte and B-lymphocyte cells that results in a dysfunctional immune system. About 1 in 100,000 children born each year are diagnosed with the disorder (the estimates vary from source to source). Those affected, usually boys, are vulnerable to infections and normal childhood illnesses that other children shrug off. Nicholas was diagnosed in March during a five-week battle against pneumocystis carinii pneumonia (PCP) at Long Island Jewish Hospital.
The only treatment for SCID is a bone marrow transplant before fatal infections can attack. SCID's most famous victim was David Vetter, the Bubble Boy, so called because he lived in the sterile environment of a plastic bubble until his death at age 12 in 1984. Today, thanks to improved transplant procedures and new medications, SCID kids like Nicholas have a shot at a normal life.
Nicholas underwent a bone marrow transplant on April 29 at Duke University Medical Center (DUMC) in Durham NC. His physician is Dr. Rebecca Buckley, who has spent 15 years researching SCID and treating affected children. The DUMC Pediatric Immunology Department performs more SCID transplants than any other medical facility in the country and has the highest success rates (about 78% have survived treatment; the oldest are now teenagers). Nicholas was Dr. Buckley's 80th transplant patient.
After numerous compatibility tests, mom Joanie was selected as the marrow donor. Her marrow was harvested, depleted of mature T-cells, and infused into Nicholas through an IV. Dad Richie was by their sides. Nicholas felt no pain, and played with the doctors throughout the procedure. Joanie, on the other hand, was sore for weeks afterward from the harvest. Unlike other hospitals, DUMC does not subject SCID patients chemotherapy unless absolutely necessary, a decision that undoubtedly saved Nicholas (not to mention Mom and Dad) a lot of wear and tear.
Nicholas and Joanie returned to their
temporary "home away from home" at the Royal Oaks Apartments in Durham two days
after the transplant. About 10 days later, Nicholas developed a 103-degree fever and a
bright red skin rash all over his body. His liver enzyme levels shot up past 500. Richie
rushed to Durham. The diagnosis was Graft-Versus-Host Disease (GVHD), which the doctors
believe was caused by maternal T-cells (those passed to him by Joanie in the womb) still
lurking in his system. The maternal T-cells attacked the new cells introduced by the
transplant. Nicholas was readmitted to the Medical Center, where he stayed for a week
before being discharged on May 17. The episode was upsetting to the family, but was not
necessarily a bad sign. GVHD is common in transplant patients who haven't undergone
chemotherapy. Nicholas' case was relatively mild and, God willing, may never recur. 
Nicholas, after Aunt Vivian tried to fix his "Skeezix" hair
Today, about a month after his transplant, Nicholas is once again filled with smiles and laughter. The skin rash is almost gone (except for some dryness around the mouth) and his liver enzyme levels are back to normal. Although he doesn't seem to care for his bottle anymore, he's eating like a truck driver. He's on a variety of medications including antibiotics, anti-antibiotics and antivirals, and receives intravenous immunoglobin (IVIG) monthly. Nicholas' first function test (to determine if he is engrafting T-cells) took place on May 22. Another is scheduled three weeks later. Depending on the results, there is a remote chance that we may see him back on Long Island in time for his 1st Birthday on July 21st. Despite a constant influx of family members to Durham-- Grandmas Vivian and Peggy, Grandpa Eddie, Aunts Christine, Vivian and Georgianna, to name a few -- Joanie is eager to bring Nicholas home before he turns into a "Tar Heel".
Due to his long hospitalizations, Nicholas faces some developmental challenges, but Dr. Buckley is confident that he will catch up physically as soon as he gains back his strength. There's certainly nothing wrong with his brain. Recently, Joanie bought him an Electronic Discovery Desk. One of the games requires dropping shapes into their corresponding holes. Joanie thought it would take him a while to figure it out. It took him only SECONDS. And while visiting Duke for his IVIG, Nicholas grabbed the stethoscope from the doctor and placed it on his own chest. The kid likes to take charge.
Joanie and Nicholas spend a lot of time at the beautiful Duke Gardens. Joanie loves to go the movies, too (without Nicholas). She saw "Liar, Liar" a week or so ago and was spooked to find herself the only person in the theater. She plans to see "The Lost World" as soon as it opens. Joanie also looks forward to a 4-day visit by her best friend Anna (expecting her first child), who is scheduled to arrive the last week of May after Grandma Viv returns home to Island Park.
Additional tests to determine what form of SCID Nicholas has are now being performed by Dr. Jennifer Puck at the National Human Genome Research Institute in Washington, D.C. The most common form of the disorder, X-Linked SCID, is generally inherited from the mother. However, as Nicholas is the first manifestation of the disorder in our family, he may be a spontaneous mutation. The test results will be of special interest to Aunt Vivian and Uncle Matt McGuire, who hope to enlarge their family in the near future. (Chances are they have nothing to worry about. The McGuire's 2-year old son, Liam, while congenitally nutty, is not affected by SCID.)
The Nicholas Wendt Fund, established to help with the short-term expenses of maintaining two household and to provide a financial buffer for the future, continues to grow thanks to the generosity of our many friends and neighbors. The Nicholas Wendt Raffle was held on Memorial Day.
We are also grateful for the information and
emotional support provided by the SCID Mail Group. The group, comprised of more than 20
families of children affected by SCID and other serious immune system disorders, is the
brainchild of Barb Ballard, who also maintains the SCID Home Page.
Related Internet Links 
SCID Home Page (the only Internet resource guide to SCID)
"Treatment Aids 'Bubble' Illness" (article about Dr. Buckley's research)
DukeNews: "Longer Lives for 'Bubble' Boys" (article about Dr. Buckley's research)
DukeNews: Radio Interview with Dr. Buckley (hear Dr. Buckley speak!)
"Repairing Faulty Immune Systems in the Womb" (article about in-utero transplants for SCID)
"Bursting the Bubble" (article about David Vetter, the "Bubble Boy")
Durham Convention & Visitors Bureau (information for those planning to visit Nicholas)