Hazel Update from Jackie Nelson (mom): Today we had an appointment with Childrens Cardiology just to double check her pressures with an echo, and make sure there wasn't more then just a cold going on, hoping we would be told the echo looks better because of the new drug Bosenten that she is on. As I drove in to Children's today, the 15 year anniversary of my brother's death, I looked up at the big tall glass hospital and couldn't help but feel so much emotion. I lost my brother in this hospital 15 years ago, and now, they are taking care of our sick baby girl. We are so lucky to have them out our back door and I'm thankful for them, but I don't think I'll ever have a warm fuzzy feeling coming here. Hazel had some more very unfortunate news. Her lung pressures are 150/50 and a normal babies are 25/10. She is in a very bad place and is being admitted to the pediatric intensive care unit. She be undergoing surgery tomorrow to have a central line placed to begin IV therapy, that will follow her for the rest of her life. She is also not growing like they want her to, but has gained some weight since her last appointment. Our hearts are crushed, but I guess we're use to getting them stomped on. Her cardiologist, Dr. Fletcher, is going to treat this disease called pulmonary hypertension aggressively. He has reached out to colleagues in Denver, Houston, and St. Louis and has mentioned lung transplantation. But with transplantation, you trade one illness for another. Survival rate in lung transplantation in the 1st 6 years is only 50%. It's not a cure. I've never felt some numb. Hazel will not be at her benefit this weekend, but she wants to thank everyone who is coming and has donated and made this all possible for her. We are so fortunate to have you all in our lives. With everything so hectic I have not been able to get written thank you's to everyone, and my list of people to thank keeps getting longer. I apologize for the not getting them sent out but know that our appreciation to you all is overflowing. Hazel's condition is rare, and it's a trail and error to try and help her. She will never recover from this, but we hope we can slow the progression and she can sustain a life with quality. I hope my brother is watching over her and can talk God into a miracle. Please pray for her. Love and thanks to all. #heartofhazel
Hazel is out of surgery and our hearts are crushed.
She indeed has pulmonary arterial hypertension and the heart cath confirmed the
fears from her previous echos. Hazel's heart cath
showed the mean pressures in her lungs are 3 times what they should be. This
causes her right ventricle in her heart to work overtime, and the muscle to
continue to enlarge. She did not respond to increasing her dosage of
sildenafil, and showed almost no change when testing her with oxygen and nitric
oxide. The next step is to start a medication called bosentan, which helps to
decrease pulmonary resistance. This medication is very hard to get approved
from insurance companies due to it being very expensive. We hope to have it in
7 to 10 days but Dr. Fletcher, Hazel's cardiologist said it could take longer.
She will have to undergo testing of her liver monthly to monitor effects that
this drug can cause. If this does not work, the next step will be IV therapies.
Dr. Fletcher is a very straight forward doctor. He told us we are not at a good
point, so he wants to be very aggressive with her therapies. She also has
malacia of the trachea which is where one side of her trachea is not as open as
it should be. Because of this they want to do a sleep study to make sure she is
getting enough oxygen while sleeping. Babies with heart defects are 8 and
1,000, and of those babies, 1% have transposition of the great vessels, and of
those babies, .02% have pulmonary arterial hypertension. This is so rare and
and so few babies have it. They do not know the cause if this, but classify
this as World Health Organization, Group I, caused from her congenital heart
My goal for HSCT is to stop my MS right where it is so I can go back to my young, adventurous lifestyle.
“The dog will be another set of eyes and paws as we monitor Miri’s activities and keep her emerging from her limited world.”