First Post for Anabelle
03/22/13
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Welcome friends, family and anyone with a kind heart. I am Maemo, Anabelle's grandmother. It has been a long road the past 8 months. We found out around 6 months ago our sweet Anabelle has Zellweger disorder. Children with Zellweger disorder are missing one to three enzymes that aid with absorption of protein and other nutrients. She was having around 10 diarrhea diapers a day before finding a formula she can now tolerate. We are at a normal one to two poopy diapers a day and happy about that. The feeding tube also allows for a slower rate of absorption along with special amino acid-based formula made it possible for her to hit an all-time high of 12 lbs. when weighted by home health nurse this
week. She has cyst in her kidneys, enlarged liver, also liver cyst and atrial valve defect (hole in heart) but no complications from these challenges so far. Today she is shown napping in her swing with black strapping on her wrist and around her thumb to position in a functional way (she has increased flexor tone causing her to fist involuntarily due to disease process). She just got them Tuesday and already her little hands are beginning to open and allow her to have correct alignment of joints, so she can hold things.
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We saw the optometrist last week and so far, her retina looks good. It is common for degeneration of retina leading to blindness with Zellweger. The ENT (Ears, nose and throat specialist) tested her hearing using brain wave reaction and noticed a discrepancy between right and left ear so further test will be done. I can tell she hears by how she turns her head toward my voice. It's just hard to tell how well she hears. We go to the pediatrician every other week. Home health nurse comes once a week, OT and PT alternate weeks. We have tried to minimize ER visits by close communication with our health care team.
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Last week we had a time with her having constipation. I've learned new message techniques that help facilitate the bowels to move and how to avoid dehydration. It was so scary the way it began. She started to have "spitting up episodes." She would start chocking, unable to clear her airway due to decrease of motor control with swallowing and muscle weakness. I would have to quickly turn off the feeding pump, grab the hand-held bulb suctioner and manually clear her throat while positioning her so that she is upright as possible. I later discover that the spitting up was a result from her poor little GI system becoming backed up. It took two days to get over but thank God she finally was able to have a good movement. The pediatrician was also pleased at how well we survived that scare.
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Today has been a good day. A good day is one where Anabelle is not in pain. We were able to take a stroller ride walking to local park and back. She is sensitive to sunlight, so she had her cool shades on. I don't know who enjoys the walks more, Anabelle or Maemo. She is napping and so I get to finish launching this website and blog.
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Thanks so much for all the prayers and sweet messages. I
hope this blog gives you a window into our lives. We feel everyone's love and want to share with you how much you help us through all these challenges. Please know we feel so very blessed to have Anabelle in our lives. She is never a burden. She has taught us each how to love deeper, how to prioritize our lives for what counts the most, and she has drawn our family closer together as we all help to meet her needs.
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We have recently experienced other trials in our Anabelle care team. Great grandmother, known to Anabelle as "GG," had shoulder surgery and will be unable to help for 4 - 6 months. Anabelle's other grandmother had emergency surgery last week with a ruptured appendix and will be unable to help me for the next 2 months, Nikki (Anabelle's mother and my oldest daughter) is still ill and needs our prayers as she tries to cope with her heartache so deep it is affecting her functioning.
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I am living on faith. I pray all day long. I just ask God for strength and to let Anabelle not hurt. Sometimes I live one day at a time, other times it's one hour at a time.