Ehlers-Danlos causing dysphagia leading to many aspiration pneumonias?

[Guest guest]

My son is 3 years old is thought to have Ehlers-Danlos Syndrome, a

connective tissue disorder his 8yr old sister does have. He has the

loose skin and hypermobile joint issues, but the main problem all his

life is pneumonia after pneumonia. We found out he has an abnormal

swallow which includes delayed swallowing, pooling, residual which

leads to aspiration and the many, many pneumonias. He also has reflux

and the doctors were convinced the acid was what was damaging the

throat muscles causing the aspiration, therefore, he had a Nissen

Fundoplicaiton surgery this last May. I have always had a gut feeling

that his lazy swallowing process was due to the decrease in the amount

of collagen in those tissues from the EDS.

Below is a portion of an article I found that might be the answer to

my son's aspiration problem. I f anyone out there knows, especially

you wonderful SLP's, what would a swallow study look like if the

arytenoid muscles, joints, cartilage was affected?? I know the article

says tight vocal cord closure, but would that be observed on a swallow

study or would just the aspiration be identified. I know these are

hard and individual specific questions, but I just feel if this is all

due to the EDS, it will never get better. We did go to Cleveland in

Jan and had Electrical Stimulation Therapy for his swallowing and it

worked perfectly for about a month and then it all became lazy again.

We thought it was because of the reflux and now that we had the

Nissen, when we go back for more treatments in Oct, he should not lose

his perfect swallow this time. My question is though, if the problems

are due to EDS, then without continuous stimulation the muscles and

all will get lazy again causing aspiration.

I know we are probably the only ones in this exact scenario... I don't

know if any other people with EDS and swallowing problems have had

EStim therapy and reflux with a Nissen. Only time will tell after we

get more treatments if it will work or whether he will be tube-fed the

rest of his life.

Thanks for listening! Amy- Chelsea, 11 yrs, asthma and reflux,

Cydney, 8 yrs, Ehlers-Danlos Syndrome, hypotonia, hearing loss,

bleeding tendency, hypermobile joints with dislocations and pain and

Bobby III, 3 yrs, Ehlers-Danlos Syndrome, dysphagia, aspiration,

reflux, Nissen, tubes in ears, BARD g-tube, bronchomalacia, chronic

lung disease, Celiac Disease and many food allergies

Speech and hearing problems associated with Ehlers-Danlos:

¨Articulation problems

Loose joints and a poor sense of proprioception make it difficult for

precise movement of the articulators.

¨Swallowing problems

The arytenoids are joints held together by collagenous muscles,

cartilages, and ligaments. Improper function of these joints creates

a problem with tight vocal fold closure during a swallow, leading to

aspiration. Weak muscles also make it difficult to maintain good neck

posture, another factor involved in swallowing.

¨Hoarseness/Weak Voice

The vocal folds are composed of layers of tissues, easily bruised and

strained due to tissue fragility and weakness. The lungs are also

affected by EDS, leaving some with a limited capacity to produce the

appropriate intensity for speech.

¨Fluency

Although the exact cause of stuttering has yet to be determined, some

researchers believe it is multidimensional in nature, caused by

psychological and physiological components. People who stutter have

been observed to have trouble coordinating their articulators,

initiating laryngeal and oral movements, and using proprioceptive

feedback in oral movements. Due to muscle weakness, these problems

occur in people with Ehlers-Danlos Syndrome, and may therefore result

in errors in fluency.

¨Hearing impairment/Sensitivity

Hypermobility of the joints of the bones in the middle ear (malleus,

incus, and stapes) creates problems with effective sound conduction

across the middle ear. This loss of energy in route to the inner ear

leads to a mild to moderate conductive hearing loss. Because the

inner ear is also responsible for balance, improper function leaves

some affected with bouts of dizziness.