LCHM Summer 2017 - 14

FEATURE

In the beginning doctors treated the heartburn but weren't really sure what was going
on with the fingers. She found out in 1999
when a rheumatologist tested for the scleroderma antibody. You would think finding
out what she had would lead to help and a
better quality of life. But that has not been
the case for Jean or for many with systemic
scleroderma. There is a huge chasm between
the scleroderma diagnosis and receiving care
that changes outcomes. There is a lot of trial
and error, often treating an affected body part
rather than the disease. Many scleroderma
patients are frustrated. "There's a lot of guessing,"
Jean says. "We are sent to different specialists
who are afraid to touch us, thinking they will
make it worse. Each time they guess and try
another doctor, it costs us money." She also
says that she has encountered surgeons who
won't operate after another surgeon. If one
surgeon has stopped treating her, the next
one thinks nothing further can be done. It's
often misdiagnosed and treatment is delayed.
Starting treatment early could possibly delay
future complications.

fresh vegetables, fruit, dairy, breads, or pastries.
She has to have water when she eats because
of the ulcers in her esophagus. If she eats the
wrong thing she gets sulfur burps, cramping
and diarrhea. She can suffer for six or seven
hours dealing with the effects of one bad food
choice. There's also a painful quarter-sized
calcium deposit on her hip that is trying to
expel itself, burning as it pushes out. They
don't operate on these because they are likely
to come back in the same spot, which has
prompted Jean to remove them herself with
a razor blade and Neosporin.

to believe the patient knows anything and
then Jean suffers.
Going to the dentist is also a challenge.
Scleroderma patients can't open their mouth
very wide because of the tightening facial
skin. Salivary gland damage causes dry mouth
and speeds up tooth decay, and damage to
connective tissues in the mouth can lead to
loose teeth. A dentist who doesn't understand
can actually tear the patient's mouth. Jean's
dentist uses child-size instruments on her;
finding a dentist who understands scleroderma
was not easy.

Having an IV inserted is an excruciating
experience for Jean. With scleroderma, blood
It's a very frustrating cycle: patients presenting
vessels are constricted and collapse. It takes a with difficult symptoms and doctors who truly
At this point, 20 or so years after the initial team to insert an IV. It's extremely painful but want to help, yet they are in unknown territory.
diagnosis, Jean has had an array of procedures somehow, Jean keeps her arm steady through Physicians treat the most current problem but
including an arterial bypass that left her with the many attempts. Two years ago I took her there isn't much that can be done to quell the
pinkie fingers that don't bend; a sympathectomy to the emergency room because it was time disease. Jean wonders, "Was everything I went
that was supposed to increase blood flow and to deal with the finger that was eventually through necessary, like the arterial bypass that
deaden the nerves out to the extremities; the amputated. She made them aware of her left me with pinkie fingers that I can't bend?
nerves in her spine have been stapled, which disease and the difficulty with her veins. She There was a lot of experimenting, but I can't
was extremely painful and ended up working told them to use ultra sound to find the vein, go back." It's often hit or miss and the patients
for only six months. She's had blood clots in but no one paid attention. For over an hour, suffer and get discouraged. "We get bitter and
her arm three times; a rib removed to insert a two or three different people tried to hit the on edge with the doctors. Maybe we are a little
stent, and the tip of her left index finger has vein to no avail. I watched my friend writhe in moody and not very nice sometimes, but we
been amputated. Looming in front of her is pain, tears streaming down her cheeks as they just want to get some answers and relief," says
the possibility of more fingers being amputated. stuck her again and again. She was holding Jean. Hearing "It's a tricky disease" or "That's
Her right index finger is always covered with onto me with her other arm for stability and how scleroderma is" is endlessly frustrating
a band-aid and seven additional fingers have practically climbed my body with her legs due for patients. It's exhausting and patients often
to be covered with band-aids as well. Her to the pain BUT she kept her arm steady so get discouraged.
morning band-aid ritual takes half an hour they could try to hit a vein. I had never seen
every day. "Thank God for Neosporin," says anything like that. Even with information
Most scleroderma patients hope to be
Jean. "It helps so much with the pain." And about her condition, the providers seemed directed by their physicians to specialists who
then there is the gastroparesis. Scleroderma to minimize it and moved full steam ahead. at least have some knowledge of scleroderma
affects the stomach, too. She has reactions And that was not first time something like that and won't play guessing games when it comes
to what she ingests almost daily. She doesn't happened. When she had the sympathectomy, to treatment. "If you get a doctor with some
always know what is going to trigger an attack. they tried 12 times to get an IV in and finally knowledge of the disease, what a difference it
One day she can eat something and the next had to have someone from the Med Evac crew makes with the treatment and results," Jean
it causes her terrible heartburn, which leads do it. This happens a lot with scleroderma says. And, of course, there are the insurance
to hours in the bathroom. Everything she eats patients - Emergency Room providers have companies, a whole different challenge. About
must be bland, mashed or liquefied. No spices, little knowledge about the disease, don't seem eight years ago, a doctor suggested Viagra to
14 Lehigh County Health & Medicine | SUMMER 2017

Table of Contents for the Digital Edition of LCHM Summer 2017