Nika

Propionic Acidemia

Nika’s story is not one of tribulation but one of triumph. She arrived 2 weeks early, on a Thursday morning, weighing 5.8lbs. There were no complications except she had a low glucose level that was quickly remedied. We marveled at how beautiful and tiny she was. Compared to the other babies, she was by far the quietest.
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There were no major incidents that Thursday or Friday except that as a first-time mom, I had trouble nursing. I believed that Nika was not getting enough milk as she would go through periods of loud shrieking and hand shaking. I was told that this was normal and that newborns did not need much milk. Her shrieking and “cluster feeding” however only intensified the guilt I felt about my lack of milk. I insisted that she be given formula. I also refused that she be given the Hepatitis B vaccine as I had been reading about the pros and cons of vaccinations and was still unsure of my stance.

We were due to go home on Saturday morning. As Nika was born with low glucose, the nurse checked it once again before letting us go. Her level had declined again. The Nurse urged me to nurse her so that her levels would go back up. With what milk, I wanted to know with frustration. When they returned, they found that her values were even lower. I was sent home and she was kept overnight to get her glucose to normal levels. I remember feeling angry at myself that I did not notice that something was seriously wrong.

Nika’s father and I returned Sunday morning to check her progress. We were told that she had improved. I am not sure what else we may have been told at that time but I remember thinking that she was getting better so she will be home soon. Unfortunately, while sitting outside we received a call that Nika was going to be transferred to Mount Sinai Hospital and that she was suspected of having a metabolic disorder. We were scared since we were unsure of how serious this could be. I just kept saying to myself that God would not have given her to me to take her away so soon.

Nika spent two weeks at Mount Sinai Hospital. We were first told that it may be weeks before Nika was ready to go home as the doctors were unsure of what metabolic disorder she had at the time. A few days later, we learned that they believed she had Propionic Acidemia and were starting a course of treatment. We were also told not to research the disorder on our own because PA patient’s varied and someone else’s story may not be Nika’s. We decided to put our faith in God and the doctors, one of which surprisingly was the daughter of a doctor my grandmother worked with. Nika’s levels improved daily. It was nerve racking keeping up with all the numbers when all we really wanted to know was whether she was better or not. The biggest battle during this period was that she not be sent home with a feeding tube as the doctors were discussing. I knew that she had no problem breastfeeding except for my lack of milk so I strongly told the doctors to give her time. At the end of the two weeks, she was taking her goal feeds by bottle and was ready to go home. We were giving emergency protocols, diet plans and numerous other information to care for our special angel.

Nika’s first year was incident free except for a few instances of bad acid reflux that resulted in vomiting. She also had a cold that first winter that lasted a few weeks which also involved frequent incidents of vomiting. One of the biggest challenges during this time was keeping up with the recommended daily protein intake. Nika was not and still is not a large eater. I was so frustrated with reaching the protein goal on a strictly vegetarian diet that I began including small quantities of meat products in her meals. There are also times when we feel pressured to try to get Nika to eat more or to take certain vitamins especially when we hear from the doctors that they would like her to gain more weight. However, we have decided to stick to what we know works. We understand that Nika’s condition makes her more vulnerable but sometimes we feel that the doctors are expecting too much. Both Nika’s father and I were on the smaller side as children. It is expected that Nika would be slender also. We know she is not starving and the fact that she has been healthy so far attests to that.

As Nika approached her first birthday having experienced no metabolic crisis, the doctors started to talk to us about liver transplants. The transplant would help her body to process more of the propionic acid and therefore lessening the amount of ammonia in her body which held in the 80’s since her discharge. My family and I talked about it, we talked to other PA families and I read what I could find online. Because of the crisis at birth, the doctors were convinced that her version was one of the more serious ones. We knew that she could go years without a crisis or maybe her entire life, but we had to answer the question of whether we were willing to take that risk. What if we waited and she went through a crisis that left her brain damaged? We decided to put her on the donor list.

One year to the day that Nika came home from the hospital and a few days before my BAR exam, we received the call that there was a match. As much as we thought we were ready, I remember thinking that this call could not come at a more ill-prepared time. I told the coordinator that I would call him back. I called family members and my bar mentor to get advice. In the end, we decided to accept the transplant. The surgery was the next morning and lasted about 6 hours. I spent those 6 hours in church asking for prayer. All went well and Nika spent another 2 weeks recuperating. While in ICU, there was a day or two of uncertainty as the medical staff could not figure out why Nika was having a hard time getting her oxygen level to remain at 100% on the ventilator. In the end, after hearing that these machines are not necessarily made for young patients, we decided to take her off and see how it goes. It worked! Nika was breathing on her own. Two weeks later, Nika was again home bound, although we did have the customary battle with the doctors that she was not going home with a feeding tube.

About 1 month after the transplant Nika did have a biopsy as her liver numbers kept fluctuating but the biopsy was normal. The doctors decided it may just be her liver settling in. Since then her numbers have been amazing.
Nika has been slow to develop in some areas. She did not walk until she was 21 months and is slowly learning to speak sentences. It has been a tough year in terms of decision making with the pressures of COVID-19 in terms of minimizing Nika’s exposure while ensuring she develops as normally as possible. We did decide to enroll her in a private day care center in September. She is learning from her peers and we continue to see her progress daily. Nika has also recently qualified for speech and physical therapy through Early Intervention. We pray that these services help her go even further.

Through it all, Nika has been a warrior and an inspiration. She continues to be a blessing to those she meets with her infectious smile. We pray every day that God continues to keep her in his grace.

We would like to say a special thanks to our Family and Friends who have been supporting us in every step of this journey. Also, to the Doctors and Staff at Mount Sinai Genetics Clinic and to the Liver Transplant Center. They have become our second family. Lastly, we cannot forget our OA community for being there whenever we have questions and for the continued love and support.

Danika and Jemmel ​
Queens, New York
danikac06@gmail.com

From the Fall 2020 OAA Newsletter