At first glance, the children in this collage might look happy and healthy. But what if we were to tell you that most of them won’t live to adulthood?
Terminal illnesses come in a variety of diagnoses to a variety of patients. Rather than being told “you have six months to live”, some mothers are told “your child won’t live past age 10”. You can imagine how heartbreaking this is to a family, especially when their child’s disease is rare and there is no known cure or treatment.
HB 701, Representative Mazur’s Right to Try law, would have made experimental treatment an option for the mothers of these children in the House version and the version recommended by the Senate Health and Human Services Committee. Today, the language of the bill is being deliberated in a committee of conference due to an amendment by the Senate that would require death be “in the near future” for the patient. You can see how this would be harmful to the children with genetic diseases. Their slow deaths by lack of development don’t count as “terminal” in this Senate version despite the fact that these illnesses will kill them.
Athena (baby on far left) is the child of a mother who testified on HB 701 whose husband Jake died of a progressing tongue cancer. He was willing to try an experimental treatment that was promising early on, but the doctor backed out due to liability. By the time Jake was dying, even if he found a doctor willing, he would have been too weak to try anything. Athena was born shortly after he died so she never got to meet him.
Leo is a toddler of a mom who testified on HB 701 as well. He was born with TNPO2 mutation which makes him the second child to ever have it. Leo was born with a small head due to this mutation and cannot develop due to a single misspelled gene called TNPO2. Additionally, some nucleotides of his DNA produce a toxic protein that causes seizures 10 times a day. From what we know of this mutation, the life expectancy for Leo is age 6-9.
Yiannis is 4 years old and has a disease called IRF2BPL. Only a handful of kids in the world have it. This illness causes the child to develop normally as babies, but then around when they’re maybe a year old they start suffering neurological degeneration until they can’t walk and then eventually can’t eat or breathe. There is no specific life expectancy for this disease, but it will be what kills him.
Austin is 5 years old and he has LMNA-related congenital muscular dystrophy, which is a progressive muscle wasting disease. He is cognitively typical but cannot sit, stand, walk, or eat by mouth. He suffers from joint contractures, scoliosis, hyperlordosis, heart arrhythmia and needs full feeding support and breathing support at night. There are risks with sudden cardiac arrest and respiratory illness like RSV or pneumonia. The life expectancy is uncertain, but likely mid-teens.
Lastly, Tristan is also 5 years old and he has the KCNH1 mutation, which is a neurodevelopmental disorder that causes epilepsy and severe intellectual disability. Only a few children have this condition. We do know that he will die from one of his seizures, whether that will be tomorrow or years from now. The life expectancy is also bleak for Tristan, but in a couple rare cases, people can live into their 20s.
What’s key here is that people who are actively dying aren’t always on their deathbed. These children, and their parents, deserve the right to try to live. Not only when their children are 6 months away from death’s door, but also when they’re strong enough to handle it early on. The committee of conference on HB 701 today will decide whether these kids get to try to live a little longer. Please contact them with your support to change the definition of terminal illness back to the original House or Senate HHS version of the bill!
Below are the emails of the committee:
elephantsmarching@msn.com (Rep. Wayne MacDonald)
Erica.Layon@gc.nh.gov
Steven.Kesselring@gc.nh.gov
Jessica.Lamontagne@gc.nh.gov
David.Rochefort@gc.nh.gov
Daryl.Abbas@gc.nh.gov
Suzanne.Prentiss@gc.nh.gov