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Re: Introductions - Keely

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Sorry Im so late on the introduction thing, its been a bit hectic

around here lately!

Im Keely, in 23 years old, my husband Dave and I have 2 boys,

whos 28 months, and Tyler who is 9 weeks. I have had a

bizarre medical history that was never explained, including

scoliosis, Paroxysmal Atrial Tachycardia, Heart Conduction Issues,

Pericarditis, cough varient asthma, kidney 'issues' (they bleed,

ALOT... somehow my doctors didnt see this as a major problem...),

after B was born i developed SEVERE orthostatic Hypotension, the

worst my cardiologist had ever seen... during a tilt table test,

they stood me up for 4 and a half minutes before my blood pressure

was 0, and my heart rate was over 160. I take beta blockers and a

steroid to keep my blood pressure up. In '03 i was seen at Mayo and

told that my problems were autoimmune, and i didnt have to worry

about progression. I stabalised that fall thankfully.

Chapter 2. lol!

was very healthy for the first year of his life... he got

one cold, and that was it... he was a happy normal infant. at 12

months he got his MMR shot, and that began a very long difficult

road for us. He had the normal reaction, slight rash, low fever,

nothing major.

but it never went away. by January he was running VERY high temps

(104-106) and the rash was everywhere... I took him to the ER, they

told us to keep treating with motrin and tylenol. I took him to the

doc, they said it was a virus, and i was being paranoid. On January

15th (off and on fevers all along) he started falling (he had been

walking at 8 months, and was very stable by 1), within 4 days he

could no longer stand. I thought maybe he had hurt his leg, so i

took him to the doc, who sent us to the hospital when she heard a

heart murmer and noticed his feet were scrunched up and turned in.

In hospital he had some blood tests and xrays, as they were pretty

sure it was just an injury. His CK enzymes were high, so we wound

up admitted and told he may have Muscular Dystrophy. All I knew

about MD was that kids died of it, and I was terrified.

over 4 days he had a bone scan, CT scan, MRI, EEG, nerve conduction

studies, x rays, and a spinal tap. All were normal (the MRI was

only of his spine). we were sent home and told it was viral and

should pass, but to follow up with our neurologist.

In May I realised he was way behind other kids his age in walking

and balance, and he got sick constantly, and still wasnt saying any

words. I took him to the neurologist and told her something was

wrong. She said he seemed healthy and she didnt think anythign

would show up, but she would order a brain MRI just to cover our

bases. She said at worst it was a bit of damage from the virus he

had in January, but that it was unlikely we were dealing with

anything really serious. This was the same week I found out I was

expecting again. the MRI wasnt normal. he had 'patchy signal

prolongation' in the deep white matter, and the basal ganglia. this

was the first time we ever heard the word mito. she ordered more

blood work.

his lactic acid was elevated, and we got referred to genetics. In

October '04 he got his muscle biopsy, results were in Jan '05 as a

Complex 1 Deficiency. Hes been off and on the cocktail since, we

are trying to very slowly introduce it now,a s when we started it

all at once, he didnt tolerate it well. so far so good though!

Tyler was born Feb 18th '05, and is very healthy so far! his

initial CK enzymes were elevated, so that gave us a scare, but his

repeat came back normal!

We see the genetics doc in May when we will have more bloodwork on

both boys to see where were at... were hoping Tyler dodged the

bullet!

So far is very mildly affected... he has Apraxia of speech,

meanign he doesnt talk but actually understands far more than most 2

year olds. he has a very wide Sign Language vocabulary, and is

very 'talkative' with anyone who can understand! :) He has tremors

in his upper body that have actually been getting worse over the

past month, but are not yet bad enough to interfere with too much.

He wears AFO's as his ankles get quite weak and he falls alot,

especially if hes tiered or sick. his Blood sugar drops if he

doesnt eat constantly, but recovers well with apple juice. so far

its all been stuff we can control, and were praying hard it stays

that way!

Keely

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