5. Gener al Discussion
M diastinal fibro sis is the le ast co mmo n, but the mo st se ve re , late co mplicatio n o f Histo plasmo sis.
e
M physicians be lie ve me diastinal fibro sis to be an abno rmal immuno lo g ic re spo nse to
any
antig e ns re le ase d by the so il-base d fung us Histo plasma capsulatum. I sho uld be
t
diffe re ntiate d fro m the many o the r le ss-se ve re me diastinal co mplicatio ns o f Histo plasmo sis,
and fro m o the r cause s o f me diastinal fibro sis, which are te rme d idio pathic me diastinal
fibro sis. I pathic Fibro sing M diastinitis is e ve n le ss co mmo n, but may have multiple
dio e
cause s, no ne o f which are re late d to Histo plasmo sis. A rding ly, the re are two type s o f
cco
Fibro sing M diastinitis; Histo plasmo sis-re late d Fibro sing M diastinitis, and idio pathic
e e
Fibro sing M diastinitis which may have multiple cause s unre late d to Histo plasmo sis.
e
B th type s are rare diso rde rs cause d by pro life ratio ns o f co llag e n, fibro sis tissue and asso ciate d
o
inflammato ry ce lls within the me diastinum (the space be twe e n the lung s).
Po st Histo plasmo sis me diastinal fibro sis is characte rize d by invasive , calcifie d fibro sis ce nte re d
o n lymph no de s, which, by de finitio n, o cclude s majo r ve sse ls o r airways.
Ofte n sympto ms o f Fibro sing M diastinitis do no t de ve lo p until the dise ase has pro g re sse d to a
e
le ve l at which the re is damag e to so me ve sse l o r o rg an. The build up o f the scar tissue can be
slo w g ro wing in so me case s and in o the rs the scar tissue may g ro w at a rapid rate .
6. Sympt oms of I diopat hic FM?
Po ssibly chro nic co ug h, nig ht swe ats, fe ve r, fluid accumulatio n in the lung s and
pe ricardial sac, sho rtne ss o f bre ath, che st pain wo uld be include d.
I pathic FMcan have any o r all o f the sympto ms liste d abo ve . Othe r sympto ms may
dio
include swe lling o f face , ne ck and arms fro m co mpre ssio n o f the supe rio r ve na
cava. So me patie nts with this fo rm o f FMmay also have fibro sis in re mo te
lo catio ns, including the ne ck, the thyro id g land, o r be hind the abdo me n (re tro
pe rito ne um), which may cause sympto ms re late d to dise ase in tho se site s.
Co ug h and sho rtne ss o f bre ath o ccur with many lung dise ase s, so the y are no nspe cific
sympto ms. I I pathic FMco ug h may be re late d to the dire ct co mpre ssio n o n the
n dio
airways, o r to fluid accumulatio n in the lung cause d by co mpre ssio n o f ve sse ls. The
circulato ry syste m can be affe cte d by dire ct co mpre ssio n o f the pulmo nary arte rie s
(which carry blo o d to the lung s) o r the pulmo nary ve ins (which carry blo o d fro m
the lung s). Co mpre ssio n o f the supe rio r ve na cava may cause swe lling o f face o r
arms, and e nlarg e me nt o f the ve ins o n the che st.
7. I diopat hic FM
B cause the re are so fe w patie nts with I pathic FM re liable info rmatio n abo ut re spo nse to
e dio ,
tre atme nt is scarce . The o nly tre atme nt data is fro m re po rts abo ut sho rt te rm tre atme nt o f
individual patie nts. So me re po rts de scribe re spo nse s o f individual patie nts to pre dniso ne o r
tamo xife n, but we ne e d be tte r info rmatio n abo ut the mag nitude o f re spo nse , its duratio n, and
fo r what pe rce nt o f patie nts.
A nswe r Fro m Do cto r Jame s Lo yd o n de ath,
I my e xpe rie nce the po st Histo -re late d fo rm o f FMappe ars to be far mo re co mmo n, pe rhaps 1 0
n
time s as co mmo n as diffuse pro life rative I pathic FM Ihave se e n abo ut 1 0 0 patie nts with
dio .
po st Histo FMduring the past 20 ye ars, and abo ut 1 0 patie nts with the I pathic diffuse fo rm
dio
in the past 8 ye ars. Patie nts in who m FMaffe cts bo th lung s are at the g re ate st risk, and
appro ximate ly 1 0 -20 % o f patie nts with the po st Histo fo rm have bo th lung s affe cte d. Ikno w
se ve ral patie nts (e le ve n) who die d fro m FMaffe cting bo th lung s, usually be cause o f
re spirato ry failure o r he art failure . B cause the I pathic fo rm is far le ss co mmo n, the re is
e dio
far le ss info rmatio n abo ut the risk o f de ath fro m it. Ihave se e n so me patie nts in who m this
dise ase cause d se rio us o bstructio n, so it has the po te ntial to be life thre ate ning ,
9. Super ior vena cava syndr ome
Supe rio r ve na cava syndro me : The sympto ms that re sult fro m co mpre ssio n o f
the larg e ve in that carrie s blo o d do wn to the he art.
The supe rio r ve na cava is a larg e ve in that transmits blo o d fro m the uppe r
bo dy back to the he art. The supe rio r ve na cava is lo cate d in the middle o f
the che st and is surro unde d by rig id structure s and lymph no de s. The
structure bo rde ring the supe rio r ve na cava include the trache a, ao rta,
thymus, rig ht bro nchus o f the lung and pulmo nary arte ry.
Co mpre ssio n o f the supe rio r ve na cava by dise ase o f any o f the structure s o r
lymph no de s surro unding the supe rio r ve na cava can cause the supe rio r
ve na cava syndro me .
The supe rio r ve na cava syndro me is characte rize d by swe lling o f the face ,
ne ck and/o r arms with visible wide ning (dilatio n) o f the ve ins o f the ne ck.
Patie nts o fte n have a pe rsiste nt co ug h and sho rtne ss o f bre ath. O the rs
sympto ms can be pre se nt including ho arse ne ss, swe lling aro und the e ye s,
fatig ue , che st pain, he adache s, and dizzine ss.
10. Super ior vena cava syndr ome
The cause s o f the supe rio r ve na cava syndro me include cance r (malig nancie s) and
no n-cance r (be nig n) co nditio ns. The co mmo n fo rms o f cance r that can cause the
supe rio r ve na cava syndro me are lung cance r, lympho ma (cance r o f the lymph
no de s), and cance r that has spre ad (me tastasis) to the che st, mo re co mmo nly bre ast
and te sticular cance r. N n-cance r cause s o f the supe rio r ve na cava syndro me
o
include infe ctio ns (such as tube rculo sis, fung us, and syphilis), be nig n tumo rs (such
as te rato mas, thymo ma, de rmo id cyst), ao rtic ane urysm, pe ricarditis, sarco ido sis,
irradiatio n tre atme nt to the che st, air in the che st (pne umo tho rax), and
co mplicatio n o f ce ntral line cathe te rs and co ng e nital he art surg e ry. The diag no sis
o f supe rio r ve na cava syndro me can be made with the typical finding s abo ve . The
diag no sis is suppo rte d by ide ntifying a cause fo r the supe rio r ve na cava syndro me ,
typically re q uiring X-ray imag ing o r CA o r M scanning .
T RI
The tre atme nt o f supe rio r ve na cava syndro me is dire cte d to ward the e xact unde rlying
cause . The re fo re , tre atme nt mig ht include radiatio n tre atme nt, antibio tics,
che mo the rapy, clo t-busting (thro mbo lytic) drug s, blo o d thinne rs (antico ag ulatio n),
and ballo o n ang io plasty, and e ve n surg e ry. The o utlo o k fo r patie nts with the
supe rio r ve na cava syndro me de pe nds o n the unde rlying cause .
12. I diopat hic pulmonar y f ibr osis
I pathic pulmo nary fibro sis is scarring o r thicke ning o f the lung s witho ut a kno wn cause .
dio
Cause s, incide nce , and risk facto rs
N o ne kno ws what cause s idio pathic pulmo nary fibro sis o r why so me pe o ple g e t it. I cause s the
o t
lung s to be co me scarre d and stiffe ne d. This stiffe ning may make it incre asing ly difficult to
bre athe . I so me pe o ple the dise ase g e ts wo rse q uickly (o ve r mo nths to a fe w ye ars), but o the r
n
pe o ple have little wo rse ning o f the dise ase o ve r time .
The co nditio n is be lie ve d to be due to an inflammato ry re spo nse to an unkno wn substance o r
injury. " I pathic" me ans no cause can be fo und. The dise ase o ccurs mo st o fte n in pe o ple
dio
be twe e n 50 and 7 0 ye ars o ld.
Sympto ms
Che st pain (o ccasio nally)
Co ug h (usually dry)
De cre ase d to le rance fo r activity
Sho rtne ss o f bre ath during activity (this sympto m lasts fo r mo nths o r ye ars,
and o ve r time may also o ccur whe n at re st)
N kno wn cure e xists fo r idio pathic pulmo nary fibro sis. Unfo rtunate ly, no me dicatio n has be e n
o
sho wn to impro ve the o utco me o f patie nts with this co nditio n.
13.
14. Lumbo-sacr al or Lumber
Lumbo -sacral dise ase is a te rm use d to de scribe co mpre ssio n o f the ne rve ro o ts and spinal co rd as
the y pass thro ug h the lumbo -sacral po rtio n o f the lo we r spine , which is the lo we r back ne ar
the hips. The lumbar spine is made up o f five ve rte bral bo die s in the lo we r back. N rve s
e
co ming o ff the spinal co rd trave l tho ug h the spinal canal and e xit the canal thro ug h small
o pe ning s o n the side s o f the ve rte brae calle d fo ramina (sing ular = fo rame n). The se ne rve s
transmit se nsatio ns fro m the butto cks and lo we r e xtre mitie s thro ug h the spinal co rd to the
brain and transmit mo to r sig nals fro m the brain to the lo we r e xtre mitie s to pro duce mo ve me nt
o f the le g s, to e s, and jo ints o f the lo we r e xtre mitie s.
Lumbar ste no sis (spinal ste no sis) is a co nditio n whe re by e ithe r the spinal canal (ce ntral ste no sis)
o r o ne o r mo re o f the ve rte bral fo ramina (fo raminal ste no sis) be co me s narro we d. I the
f
narro wing is substantial, it cause s co mpre ssio n o f the ne rve s, which cause s the painful
sympto ms o f lumbar spinal ste no sis, including lo w back pain, butto ck pain, and le g pain and
numbne ss that is made wo rse with walking and re lie ve d by re sting .
The o utlo o k fo r patie nts with lumbar spinal ste no sis varie s and de pe nds o n the se ve rity and
duratio n o f sympto ms at the time o f initiatio n o f tre atme nt. Ultimate ly, the o utlo o k de pe nds o n
an individual's re spo nse to tre atme nt. The re spo nse to tre atme nt is also de pe nde nt o n the
se ve rity and cause o f the lumbar spinal ste no sis as we ll as the unde rlying me dical co nditio n o f
the patie nt.
15. With God all things are possible, I will win the battle one
day at a time. I REFUSE to be a VICTIM to
I diopat hic Fibr osing Mediast init is
I diopat hic pulmonar y f ibr osis,
Lumbo-sacr al or Lumber ,
Super ior vena cava syndr ome
ONE PERSON, ONE VOICES, CAN MAKE A
DIFFERENCES AND I CHOOSE TO BE THAT
PERSON!
FOR MORE INFO ON FIBROSING MEDIASTINITIS
PLEASE VISIT MY WEBSITE AT,
http://fibrosingmediastinitis.webs.com/
Please take the time to read and learn, help save a life
maybe even yours!
Awareness is Powerful!
