My name is Jennifer and on May 16, 2002, I gave birth to a little girl named Clover Dawn and we were sent home with a clean bill of health. Shortly after we arrived home, Clover Dawn stopped nursing and never opened her eyes or cried. She was admitted to our local hospital and they ran every test they could think of and she still continued to get worse. She was life flown to the University of Utah at five days old and they diagnosed her with a rare genetic disorder called NKH (Non ketotic Hyperglycinemia) or glycine encephalopathy.

NKH is an inborn error of metabolism characterized by the accumulation of large amounts of the amino acid glycine in the blood, urine and, particularly, the cerebrospinal fluid (CSF). The metabolic block occurs in the conversion of glycine into smaller molecules. Mutations in the AMT and GLDC genes cause glycine encephalopathy.

I was told there was nothing they could do for her. We tried the experimental medicine the doctors said might help and it did a little bit.  At 10 days old, she opened her eyes, pulled out her ventilator and cried.  It was a miracle.  Two days later, they let us take her home and told us to continue the medications and she should be fine.

About 2 weeks later, we noticed the seizures returning and her regressing with feedings.  She then began to start having episodes where she would stop breathing. She was readmitted to our local hospital, where they talked back and forth with the University of Utah and determined that there was nothing they could do but make her comfortable and let her pass.

I was completely devastated. We took her home on oxygen and pain meds and she passed away in peace 28 days after coming into the world on June 12, 2002.

On July 26, 2003, I gave birth to my second daughter Danika.  Shortly after birth, she too was diagnosed with NKH just like her sister.  She had the experimental meds in her body for one hour and she passed away, never leaving the hospital and never opening her eyes or crying except for a few minutes after being born.  Danika lived three days and passed away on July 28, 2003.

15 thoughts on “Baby Sisters Born One Year Apart Both Lose Battle To Fatal Disease Called Non Ketotic Hyperglycinemia”

  1. Jennifer says:

    These are my two beautiful angels. I miss them so much!

  2. sherry says:

    these are my granddauhhters
    watching my daughter bury her two girls was devastaing. and losing them both a year apart was the biggest challege in my life.

  3. Charita says:

    My condolences to you and your family. My best friend lost her daughter at the age of 5 to NKH. It is a devastating illness and more should be done to prevent this from happening.

  4. juan says:

    I am now dealing with Nkh disease my daughter was born 3\1\14 and its destroying my family and i don’t know what to do doctors told me that theirs nothing they can do and It will destroy me and my wife if we lose are baby girl please any advice you can tell me would help me decide next step

  5. Kfa7 says:

    I am sorry for you iam from jordan.i also gave birth two babies with (nkh) both of them died and i want to know how can i avoid this case in next pregnancy. i have one fine daughter now

  6. EKJ says:

    Hello, I have a son that is 2 he has Nkh, are you in touch with other family’s on Facebook, Nkh network, and Nkh uk, or hope for Nkh, you will get a lot of help from the family’s on these sites..

  7. EKJ says:

    Hello my son has Nkh he is 2 yrs old, are you on the Facebook sites, Nkh network, or Nkh uk, you will find a lot of help and support their.

  8. zainab says:

    Please contact me Kfa7.Im from Jordan too and I have baby with Nkh. alastalzainab@yahoo.com

  9. CBT says:

    I two have 2 daughters with NKH. Mine are 21 yrs old and 16 yrs old. I have a son who is a carrier. Mine were diagnosed at Primary Children’s Hospital in 2000. By then my oldest was 6 and the other 1.

    Jennifer, I’m sorry you lost your girls but reading how it presented sounds just like my story with my younger daughter. There has been quite a bit of research done since I had my girl’s. They now know which genes carry it and the gene mix determines the severity. There are 4 different genes. They can also test during pregnancy for it. There is so much more to learn if you’re interested by getting in contact with the families on Facebook.

  10. pineapp says:

    i’m so sorry about that, it’s so sad, this is really a rare disease, just hope that nobody else has it

  11. Julie says:

    My daughters best friend gave birth to her 3rd child on Feb 3, 2016 and the baby girl has been diagnosed with this disease. This sounds horrible and it’s hard to sit and watch this happen.
    Any thoughts or words of encouragement?

  12. So sorry for you losses, and the loss of all the children this awful monster disease steals from us!
    My daughter lost her baby son, lakota Eli smith. You can google non ketotic hyperglycinemia- nkh lakota smith, to read about Lakota,and see our angel. He lived 1 year,1 week, and 1 day. He will forever be in our hearts! We hate NKH! May 2 nkh awareness!

  13. Curtis Chamberlain says:

    My baby girl just passed away December 24th 2017 how often too the same parents none of us have ever had this NKH before and not even in our family background

  14. Carolyn S. Desmond says:

    Hello. I’ve often wondered if science had ever made any real advances with NKH. Our daughter, Jessica, was diagnosed with this condition at 5 days of age. She was the 3rd diagnosed case in the World on 7-20-76. She was born on 7-15-76, 42 years ago today. She died at 18 months of age. When she was allowed to come home at 7 and 1/2 weeks of age there was absolutely no one to help us, regarding a support group. We were a Military Family. We had one set of Friends. However, their daughter had been born just 3 weeks earlier, plus they also had an active 2 year-old Son. They were terrified to attempt to watch our daughter, even for an hour. I wasn’t about to leave her side anyway, however, staying on top of feedings and meds every 3 hours was quite difficult until she received a gastrostomy tube. We chose to not have any more biological children due to the odds of a repeat scenario in which yet another child could suffer the same Fate. We adopted our Son when he was 11 days old. I so wish that there were more that could be done to remedy this condition. We were told that once the damage was done, absolutely nothing could be done to repair it. We’d tried to have a Child for three years. We’d been Married for 5 years. My pregnancy was perfect. I knew the second that I heard her faint cry that something was wrong. During her 18 months of Life, I heard her cry 2 times; once at birth, and 1 time when she was around 1, then it was a strange kind of scream. What a hard and Heart-breaking situation this is for any parent to find themselves in. You feel helpless. “There’s nothing that can be done except to try to make her comfortable”, is something a person expects to hear regarding an elderly relative, but not your Infant. I’m so sorry that there are still parents out there of whom’s children are struggling with this rare condition. I know exactly what you’re going through. I’ll never forget it, anymore than I’ll forget our sweet Daughter. I’d never really thought much about the term “Bittersweet” until our Jessica was born. However, the whole experience made me a lot stronger person in the End. I realize that some of your dear children are still alive. Please, just love them and cherish every moment. I never accepted what the Geneticist told me, that Jessica wasn’t aware of anything. I believe that she could distinguish between our touch and someone else’s. Well, I’ll close for now. God bless you all. Carolyn Sue Desmond

  15. I had two daughters, Megan and Maci, who were born with this. My oldest born 29 years ago today lived a little over 5 years and my youngest who would be 23 the end of this month only lived 3 days. My oldest passed away in November of 94 and my youngest in August of 95. No words can express the devastation I felt. It’s been many years since I lost my daughters but still to this day it breaks my heart. I also have a daughter that is perfectly healthy and had it not been for her I don’t think I would have survived it. I am send prayers up for anyone who has had to deal with the death of a child.

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