Moriarty

IVA

Moriarty was born to what we thought to be healthy, despite her dad asking about a weird smell that I dismissed as him being crazy because to me she smelled like a typical baby. Two days in the hospital and we were getting ready to go home when her dad again mentioned something he thought was weird to the nurse, holding our discharge paperwork. Moriarty had been in a billiblanket pretty much 12 hours after she was born so we did see much of her other than her face, but while changing her diaper he noticed she was breathing weird. The nurse being absolutely amazing decided it would be best to put off discharging her for the slight difference in breathing and have her checked out at the nursery. Within a few minutes she came back to let us know that Mo would be staying a few more days and they would let us know what was going on with her. I did end up using the last day my insurance would pay for to stay at the hospital with her, only to be told the next morning that they had run every test they could think of and nothing was coming back positive. I’ve never been so fearful of losing something so perfect, I sat in the nursery with her as long as the nurses would let me. I was not allowed to touch her or sit to close as they weren’t feeding her until they knew something and every time she knew I was close would start screaming, causing a chain reaction in the nursery and disrupting all the other babies. On day 4 I finally left the hospital to take a shower and get non hospital food, I was gone for 10 minutes when the doctor called and I thought I lost my baby. It was much better news than anticipated, but not very insightful. He said that a doctor called from Phoenix Children’s Hospital and told him Moriarty needed to be transported as soon as possible and I was needed to sign the release, though the birthing hospital doctor did not get a reason as to why she was being requested at another hospital. I was terrified to watch my baby go in an ambulance to a hospital 45 minutes away, but knew it was necessary to keep her alive.

Once at PCH everything was different, the most amazing doctor waited for us to get to the NICU and very calmly told us our daughter has Isovaleric acid CoA dehydrogenase deficiency. To which my sleep deprived brain took as an insult, but was quickly corrected. I had never been so relieved to hear someone explain such, what at the time seemed, a complicated thing my child would be living with. The doctor explained that she would be fine and she would be starting a special formula as soon as the mixing people could get it ready. Finally after four days of being terrified and not being able to touch my daughter I got to hold and feed her. It was later explained that Mo had pretty much gone straight into a metabolic crisis and when the Doctor initially called to have her transported he was not sure what state he was getting her in. After about 3 ½ weeks we got the good news that she was perfectly fine and we shouldn’t worry about any adverse effects from her initial crisis. Completely terrified that we would screw something up at home, we took our little Mo to the clinic and called pretty much any time she so much as hiccupped. After about three months and getting into the routine of formulas, medicines and figuring out her personality, we realized it would be much easier to care for her than we thought. We didn’t have anything out of the ordinary of keeping up with Mo until her sister went to school and brought home a stomach virus, which landed Mo in the hospital when she was about a year old.

Now at 2 ½ she has only had two other hospitalizations, one at 2 for not having her formula for a week and one on January 2nd of 2019 for a weird random vomiting episode in the middle of the night. So far Moriarty has been a normal child, aside from being extremely smart. She potty trained herself before she was 2 and is currently working on her alphabet after mastering 4 colors. Mo does have a hatred of meat, she has tried most kinds and did have little stint where she liked the Bar S bun length hot dogs, but that passed pretty quickly. She now has a new Metabolic specialist after seeing the same one for the last two years. She seems to like him a bit more and only cried once during her first check with him. Currently she has 14 grams of protein from food followed with her 2mls of Levocarnitine, the generic Carnitor three times a day with 350 milligrams of Glycine twice a day and does amazing with it. We had a very long three month fight when trying to switch from IVA Anamix Early Years to the Next and even tried Isovactin to no avail. She went about a month with no formula at all and one day all she wanted was her Early Years milk. She very happily drinks about 16 ounces a day and calls it her milk while shunning regular milk.
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Aside from her very strong will power and attitude she is a very happy girl and shown us that caring for her is no different from caring for our older daughter who is unaffected. The hardest thing to deal with is when Mo does get sick and needs the extra attention our older daughter feels left out and lashes out after she knows her sister is okay. Mo’s sister did have a hard time when she was born and felt like she was unloved because of the time I spent at the hospital and is still showing those signs of feeling unloved. She is 7 and only somewhat understands why Mo needs more attention but fully understands why she cannot have protein. She is a big help when other people are around and try to give things to Mo and acts as a body guard against all people/food that is being brought to Mo and they have a great bond, just need to work on balancing our time after giving the extra attention to Moriarty. I did not know until recently how different our experience with the diagnosis of IVA was from others, I thought it was normal for any OA kid to be diagnosed in the hospital until we spoke with the new Doctor. I feel like dealing with a crisis before the diagnosis is the biggest challenge we could have faced and it will make anything else we have to go through that much easier when it comes to IVA related illnesses with Mo.

Kaitlyn
Phoenix, Arizona
kaitlyn.winn23@yahoo.com

​From the Spring, 2019 OAA Newsletter