I had a pretty good pregnancy, was diagnosed with Placenta Previa very early on but it "corrected itself"??? and really wasnt a problem for too long.
Jameson was born with billateral club feet and looked perfect other than that. By the time he was taken for his first bath and the ankle bracelet was put on, my family immediately noticed some skin breakdown, the bracelet caused his first blister, he was diagnosed his second day of life with Epidermolysis Bullosa.
The first night, Jameson got to sleep with me, he slept on my chest and I noticed he wasnt feeding properly from his bottle, seemed to have a problem latching on and sucking, when I informed the nurse, she was extremely rude and said "Well he NEEDS to eat and he is FINE" Jameson also had Tracheo Laryngo Molacia, which meant everytime he DID suck from the bottle, you could hear a LOUD squeak, of course at first we didnt know what this was either....
Laryngomalacia
From Wikipedia, the free encyclopedia
Laryngomalacia (literally, "soft
larynx") is a very common condition of infancy, in which the soft, immature
cartilage of the upper larynx collapses inward during inhalation, causing airway obstruction. It can also be seen in older patients, especially those with neuromuscular conditions resulting in weakness of the muscles of the throat. However, the infantile form is much more common.
In infantile laryngomalacia, the supraglottic
larynx (the part above the
vocal cords) is tightly curled, with a short band holding the cartilage shield in the front (the
epiglottis) tightly to the mobile cartilage in the back of the larynx (the arytenoids). These bands are known as the aryepiglottic folds; they create the movements that opens and closes the vocal cords for phonation. The shortened aryepiglottic folds cause the epiglottis to be furled on itself. This is the well known "omega shaped" epiglottis in laryngomalacia.
Laryngomalacia results in partial airway obstruction, most commonly causing a characteristic high-pitched squeaking noise on inhalation (inspiratory
stridor). Some infants have feeding difficulties related to this problem. Rarely, children will have significant life threatening airway obstruction. The vast majority, however, will only have stridor without other more serious symptoms.
The conventional wisdom about laryngomalacia is that the noise is more pronounced when the patient is on his or her back (with gravity making the epiglottis fall backwards). This, however, is a more common finding in older patients rather than in infants.
Causes:
Although laryngomalacia is not associated with a specific gene, there is evidence that some cases may be inherited.
[1][2]Prognosis:
Laryngomalacia becomes symptomatic after the first few weeks of life, and may get louder over the first year, as the child moves air more vigorously. It generally resolves spontaneously by the second year of life.
Treatment:
In rare cases, surgery is necessary.
[3][4][5] Most commonly, this involves cutting the aryepiglottic folds to let the supraglottic airway spring open. Treatment of
gastroesophageal reflux disease can also help in the treatment of laryngomalacia, since gastric contents can cause the back part of the larynx to swell and collapse even further into the airway. In severe cases, a temporary
tracheotomy may be necessary.
On day two the Pediatrician came in to visit us, I informed her that he got a "sore" on his ankle from the hospital band and that he WAS NOT eating properly, she looked him over, told me she thought he may have EB and wanted him transfered to St. Davids Hospital in Austin.
Having had a c-section I still had two days left in Georgetown Hospital for myself, but refused to stay while they were transferring Jameson to Austin, so I signed myself out, and followed the ambulance to St.Davids.......
The skin biopsy they took off of Jameson's foot caused the next few blisters, at this point, he was in the NICU, laying on sheepskin, and we were unable to touch him for the first day, they wanted to keep him monitored for signs of infection, and he still was not feeding properly which was a huge concern, they had him on a feeding tube, antiobiotics, and constant pain meds via IV. The staff of course was not familiar with EB and did the best they could, they told us he couldnt wear clothes with snaps, buttons, zippers, etc and were concerned about the possibilty of him not being able to wear diapers. Jameson stayed in the NICU for 5 days, which were the worst 5 days of my life, we constantly worried about infection, more skin breakdown, and how in the world we would be able to treat his clubfeet with the HORRIBLE skin condition. We found out later that Jameson is mild, although we werent aware of this until he was home and started noticing the skin breakdown and blisters were occuring less often, and werent as severe as in the hospital. Almost as if coming home was the best thing for him, at least thats what I like to think.
When Jameson came home, they linned his carseat with sheepskin, told me that anytime he was laying down he needed to have the sheepskin under him, EVEN when we were holding him, this however lasted for maybe two days and then I made the decision to try to hold him and lay him in his bassinet without it. Jamesons skin was amazingly well, and he looked better every single day.
Now, we had to face the scariest part for me, Jamies clubfeet. The first orthopedic surgeon we went to was a jerk, to put it nicely. He was not eager to start treatment out of fear of the skin and told me, "well give me a call in 3 months and remind me off all this again and we will go from there." I left the office and cried and cried, needless to say we didnt use him as Jameson's doctor.
Dr. Williams, was referred to us by my husband's uncle who is also an orthopedic surgeon (not pediatric however) and he has been a blessing! He immediately got us in touch with THE BEST PHYSICAL THERAPIST, and she began excersizing Jamies legs, and wrapping his feet in a corrected position, well at least as "corrected as they could get with just ace bandages" she is our ANGEL and has helped me with many obstacles along the way. Once we were certain that he could withstand the bandages, we moved up to Ponsetti casting, this was the probably the sixth worst day of my life, although his skin appeared fine, he was miserable, he cried for at least 10 hours straight, I was CERTAIN he was in pain, the doctors assured me he was just "protesting" I wound up spending the night at my mothers and we stayed up all night crying and trying to get Jameson as comfortable as possible. The next morning I put Jamie in the tub and soaked the casts off, I KNEW there was going to be massive skin breakdown and blisters and I couldnt handle not being able to see his skin, As we pulled the casts off layer by layer, I realized by the grace of GOD his skin looked perfect! After 6 weeks of ponsetti casting, and six casts being kicked off by "baby houdini" the next step was his first surgery, double heal cord lengthening, surgery went pretty well, although once he came out he had almost completely lost all of his vocabulary. He healed well, with no skin breakdown and he remained in hard plaster casts for 6 weeeks. After the six weeks he went into boots and bars, not fun. Jamie consistenly would kick the boots and bars off and it was a constant battle to keep them on, needless to say the boots and bars were unsuccessful and we went back into plaster casts after two more surgeries, Jameson is currently in casts! We get the casts off March 31st, and go into AFO braces during the day and back to boots and bars for bedtime for the next 3 years. We are optimistic and hope the boots and bars will stay on this time and be effective. Here are some pics of our journey so far:
I thank God daily that Jameson was BLESSED to be so mild, my heart breaks for the children who are majorly affected by this devastating disease.
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