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.