Quality of Life Issue | Transplant & Post Transplant FAQs
Pre - Transplant Medical Que FAQs
1. What is Cystinosis?
2. How do you get Cystinosis?
3. How do you know if your child has Cystinosis? (Symptoms)
4. What is a Metabolic Disease?
5. What is a Lysosome?
6. How will Cystinosis affect my child in
terms of:
a. growth
b. eyes
c. muscles
d. kidney
e. nutrition (frequent vomiting)
f. bones
g. central nervous system
7. Is the brain affected? Learning affects?
8. What is Tetany?
9. Are there blood tests to determine before birth? / Carrier status?
10. What treatment is
available for Cystinosis?
a. Hydration
b. Electrolyte
Replacement
c.
Nutritional Supplement (g-tube, central line, j-tube)
d. Cystagon /
Cysteamine eyedrops
e. Kidney Transplant
One Family's Perspective after 12
Years, the Schleuder's in Novi, Michigan, USA:
Below are listed some quality of life issues
we have dealt with over the past 12 years with our son. The "Answers" are based
on "our" individual experience and are by no means meant to imply this is the
only answer or the only issue. Cystinosis has many common facets, but also many
unique ones. For example, many children with Cystinosis experience severe
growth problems or rickets. In our personal experience, that has not been an
issue. So we can't address it. To make this section complete and beneficial to
everyone, input from others describing their unique experience would be
helpful.
DEHYDRATION?
Answer:
Have fluid (water) available at all times in all places for the
child. A filled water bottle should accompany the child everywhere he or she
goes: school, church, playdates, etc. Keep a water bottle at the bedside, (in a
bucket or tray to catch the leaks) at night.
FREQUENT URINATION?
Answer: For older children, know the bathroom location of each
place you go. Be sure the child uses the bathroom before leaving home. For
lengthy car trips plan to stop every 1.5 to 2 hours or have urinal handy,
(works best with boys).
For younger children, change diapers fequently
and have dry clothes ready when traveling. At night, protect the matress with
plastic cover. Short of getting up every 2-3 hours all night long to change
diapers, we did not figure out how to keep the bed dry. We placed towels under
our boy every night, used diapers, diaper doublers and plastic pants, but still
washed night clothes and sheets daily. It became routine.
TOILET TRAINING - WHEN?
Answer: Day time - For us age 3.5 years old. It was not much
more difficult than with our two non-Cystinosis boys. The Cystinosis son just
went more often, about every one and a half to two hours.
Night time -
Took much longer. What worked miraculously for him at age 9 was a urinary alarm
for bedwetters. Within 6 weeks of using this device, he was dry at night. In
retrospect, it would have work much sooner had we known about it. Our son
learned to recognize the sensation of a full bladder and awaken at the
sensation before he urinated. Pre-transplant , he woke up about 2 times during
the night to go to the bathroom in addition to first thing in the morning and
the last thing before bed.
FREQUENT VOMITING?
Answer: Between ange 18 months and 3 years old, our boy vomited
on average 5 times per day, usually at meals. He choked easily and certain
textures of foods bothered him. At times the medications triggered the vomiting
within an hour or so of taking them. Through experience, we learned to have a
container handy by his bed and all the other places this occured: in the car,
at restaurants - every time!, in the family room.
MEDICATIONS?
Answer: At the age of 23 month, our son had a Gastrostomy tube,
(G-tube), inserted for supplemental night time nutrition and extra hydration.
We used this tube to administer all of his liquid medicatioins. It worked
beautifully! He got what he needed without difficulty. This worked until age 6,
when he learned to swallow pills. Fortunately he takes pells much better than
most of us adults. At this time, February 2000, all his med's are in pill form,
which he swallows smoothly 6 times a day. We were introduced to plastic
containers for all the pills, (that cover each part of the day for seven days),
at a Cystinosis Conference. This allows filling the doses once a week which is
much easier, less time consuming and allows easy dispensing with no mix ups as
to what was done and what was not.
SCHOOL AND EDUCATIONAL
ISSUES?
Answer: (Michigan Detroit Sub-burb) Each Fall
before school starts, a letter is written to our son's teachers, (with
knowledge and permission from the Principal and Assistant Principal), outlining
Cystinosis and the special needs our son will require. The letter is factual
and to the point. It includes a request for frequent communication between the
teacher and ourselves. In ALL cases, the school has been accommodating for all
of his needs.
Educationally, our son is followed by the Special
Education Department and qualifies under the classification, POHI, (physically
or otherwise health impaired). Currently, he is keeping up at grade level with
minimal special education involvement.
On a few occassions, talking
directly to our son's classmates explaining why he is "different" has stopped
any teasing issues before they became a problem. We have found the compassion
of nearly all the children to be remarkable.
CYSTAGON AND "BAD
BREATH"?
Answer: The use of "Breath Assure" with the
Cystagon dose, (4 or 5 pills at a time), has helped reduce the bad breath. In
addition, frequent brushing of the teeth and use of mouthwash helps. If our son
is teased or a comment is made about his breath, an explanation as to why is
usually enough to stop the problem.
OVERHEATING?
Answer: For our son this has been a "moderate" problem that usually
occurs after physical activity in hot, humid weather. At it's worst, he suffers
intense headaches and vomiting with it. For the short term, keeping a small ice
filled cooler with cloths to wipe him down has helped. Keeping extra water for
drinking and pouring over his head has also been beneficial. As soon as
possible, we get him out of the heat to an air conditioned room. He usually
lays down for an hour or two with a cool cloth on his head and rests. If he
isn't vomiting , tylenol helps the headache.
EYE PAIN AND EYE DROPS - LIGHT
SENSITIVITY?
Answer: Light sensitivity - eye pain has been
a long term problem,since age 3 years. Sunglasses and baseball caps became the
normal on Sunny days. Playing baseball and soccer in sunglasses were required
exceptions to league rules. Between the age of 5 and 7 years, occasional eye
pain was severe enough to incapacitate our son for short peiods of time. This
would occur one or two times per month. At age 8, the Cysteamine eye drops were
started through a National Institute of Health study. Within a few months of
eye drop use, the improvement for the eyes was miraculous! Though strict
compliance has been difficult, (10 to 12 times per day per eye), light
sensitivity and pain have been greatly reduced. The eye drops are carried in a
pants pocket and are accompanied by a digital watch with hourly alarm chime.
Our son is responsible for administering the drops to himself for himself. This
has been crucial to everyone's sanity. We remind him. He does it.
Transplant & Post Transplant Medical FAQs
1. Will my child need a kidney transplant?
2. At what age might my child need a transplant?
3. What symptoms occur at end stage renal disease?
4. What is Hemodialysis?
5. What is Peritoneal Dialysis?
6. Type of
kidney donors? Advantages & Dis-advantages of each?
a. Living related?
b. Cadaver?
7. Expectations
Post transplant:
a. Fluid Intake
b. Medications
i.
Antirejection (including side effects)
ii.
Hypertensives
c. Nutrition (low
salt / low fat)
d. Monitoring for
signs of rejection
i. Weight gain
ii. Fever
iii. Elevated
blood pressure
iv. Urinary output, pain
e.
Immune Suppression precautions:
i. Live
vaccines vs. killed vaccines
ii. Chicken
Pox
8. Expected life of transplanted organs?