missmollyb.com - Information on Hurler Syndrome

Road to Recovery

After reading about the disease, we decided a transplant would be the only route for us to take on Molly's behalf. Even with all the complications associated with it, we felt they outweighed the option of having her struggle to live to her 10th birthday, if that. We researched a lot, spoke to other families with children who went through transplants for Hurlers, and on December 26, 2000, got screened to be possible bone marrow donors.

The blood tests revealed that neither John, me, nor her 3-year-old brother,TJ, were compatible matches for Molly. So, we contacted Fairview Hospital in Minnesota to get Molly's name entered into the database of the National Bone Marrow Registry. Soon after, we went there for a visit with the bone marrow transplant team and got a tour of the hospital, as the computer searched for a match for Molly. A week later, we flew to Duke University Medical Center in North Carolina. We decided to stay there and have Molly undergo an unrelated umbilicol cord blood transplant.

The pictures below briefly tell Molly's transplant story.

(Chronologically from left to right.)

John, Jenny, and Molly (9 months) on our way to NC from Chicago: January, 2001.	Duke University Medical Center: Durham, NC	Duke Children's Hospital (clinic)
February 16, 2001: Molly's pre-transplant work-up included 2 weeks of tests, getting a shunt placed, and getting central lines placed. The lines are very helpful for obtaining blood draws and giving IV medications.	After the pre-transplant work-up, Molly tests positive for RSV, which delays our start to the process. She needed about a month to recover fully and be 100% for her admittance on March 13, 2001. Pictured here is her door, greeting all who walked by or entered her room.The precaution posters indicate to medical personnel that she has a history of RSV, and they had to wear gowns, gloves and masks to enter the room.	Prior to transplant, Molly received 10 days of chemotherapy to kill her existing bone marrow. It's effect comes a few weeks later, so for now, she is happy and playful!
Day 0: Transplant Day: March 23, 2001. Grow, cells, grow!	During the 2 weeks post tranpslant, the effects of chemo start... Chemo kills all the dividing cells in her body: her bone marrow, the lining of her gi tract, and, yes, her hair.Here, Nurse Michelle helps mom "buzz" the last of the locks, which were otherwise falling out in clumps in her crib. Bye, bye bows!	Molly is very lethargic, battling continuous vomitting, diarreah, and fevers. Daily labs keep us updated on the status of her blood counts and organ activity
Day 11: Molly tests positive for RSV with a white count of 0. She must go into a tent to inhale a drug for 8 hours a day, which will help halt the virus from growing. She also gets inhalant treatments 4 times a day.	Day 13: Our good friend Kathy visits us at the hospital. Despite needing a bit of oxygen to help her infected lungs, Molly GROWS NEW CELLS! The miracle begins...	April 19, 2001: Molly's 1st birthday. Even with efforts to make her a star for the day, poor Molly is so wiped out, she can't really celebrate.
Dad and big brother TJ come for a quick visit. Germs are our biggest fear, so "little" visitors can't stay long.	The wearin' of the yellows... Due to past bout with Molly's RSV, we must garb up in robes, masks, and gowns everytime we leave our room, so as not to bring any possible infection to other patients on the ward.	Molly with her wonderful doctor. Dr. Joanne Kurtzberg is the director of the Pediatric Bone Marrow and Stem Cell Transplant Unit at Duke. We love Dr. K!
After transplant, Molly is put on a number of drugs.She is on numerous antibiotics to protect against infection, and GCFS, which promotes the new white cell growth. Steroids and FK506 help her body accept the new marrow, and fight graft vs. host disease, but also puff her up and promote hair growth. (look at those fuzzy arms!)	Day +85: Molly's confetti parade! She is discharged from the hospital. For 1 year post-transpanst, she must wear a mask in public to avoid possible infections. We go back to our apartment, with daily clinic appointments set up.	Life at the apartment is busy for mom, who has become an "almost RN"! Molly gets daily IV infusions, blood draws, dressing changes, etc... but we've been taught how to do it all. Molly likes being back at the apartment, but she is basically still in isolation.
Molly at clinic. She receives IV medications, blood transfusions, and check-ups by doctors daily for awhile.	Molly works hard with therapists (Occupational, Speech and Physical) at the apartment to regain her strength and build stamina after her long hospital stay.	5 months post-transplant, Molly's shunt got infected, and had to be removed. This was taken just before Molly went in for the emergency surgery. However, she came out of it feeling 100% better!
September 29th: 6 months post-transplant, Molly gets the doctor's blessing to go home to Chicago!	This is Molly at age 18 months, finally "celebrating" the birthday she wasn't able to at one year.	This is Molly at age 22 months (11 months post-transplant.) She is off almost all medications, and has 8 therapy appointments a week to help her learn to walk, talk, and feed herself. Her alpha l-iduronidase enzyme level is at 59.2 (from 0.1 pre-transplant), and she is 100% engrafted. We are so blessed.