504 Plan & Accommodations [Elementary School]

A mother with a newly diagnosed son is prepping him for kindergarten later this year. She asked what some of the accommodations are. There are a number of resources available to assist parents with integrating their child into a new environment, such as starting school. Many parents are unaware of the options available. 

A 504 Plan is a plan developed to ensure that a disabled child is identified under the law and is attending an elementary or secondary educational institution receives the appropriate accommodations that assists in their academic success. 

I also highly recommend that you meet with the teachers before the semester starts, inform them of CF and give them your personal cell phone number in case of emergencies.

http://www.cff.org/LivingWithCF/AtSchool/

A few accommodations that can be in place (keep in mind they vary from patient to patient depending on their progression of CF)

       

Children with CF lose five times more salt when they sweat than children without CF. Therefore, XX must drink fluids directly before, during and after exercise (PE, playground, etc). Salt can also be replaced by eating high-salt foods like pretzels or potato chips.

should be allowed to have a sport bottle of fluids on her desk at all times. Plastic water bottles should be thrown away at the end of each day, even if full.

should always have the top shelf or cubby at the end of the row nearest the door.
desk should be on the outside of the class and upfront.

should be moved away from any child who is sitting next to her or at the same table when congested, coughing, runny nose, stomach ache, not feeling well, etc.

Unlimited access to snacks, restroom and water.

may need to attend school with a peripheral interveneous catheter (PICC line) inserted into her arm for antibiotics. It is critical that this line be cared for during the day by school staff to make sure it is not bumped or damaged. Level of appropriate activity should be discussed as well as an emergency plan.
may need to have support when she must stay at home or be hospitalized due to complications from her illness. She may require that only pertinent work (English/reading & math) be assembled by her teachers in the event of absences. Additionally, if she has to miss school for an extended period of time, homebound services (tutor) may be requested.

Rest in nurses office as needed
Notification of viral outbreaks


Adjust or waive attendance guidelines

    Waive tardy regulations/policy due to time consuming morning therapy.
Access to tissues when needed to blow nose due to chronic sinusitis and/or cough.

Antibacterial waterless soap should be placed in all central school areas including the office, computer lab, library, MPR, lunch line, etc.

 
Those with CF must never be in the same room, use the same computer/ lab desk (even at different times), lunchtime serving utensils (salad bar) or any materials whatsoever used by someone else with CF. Especially, not to use the same bathrooms. They also must stay a minimum of 3-feet away from each other.

 
should not be excluded from any physical activity unless notified by parents. Exercise is very beneficial for children with CF.

 
Full participation in extra-curricular activities and field trips.

 
needs as much time as possible to eat meals at school and snacks should always be accessible in the classroom.  

 
should always be able to go to the front of the lunch line when purchasing a school

So, you're being admitted.

Throughout our lives we are hospitalized more than the average person. Reasons for being admitted can vary from malnutrition, depression, pulmonary exacerbations, GI complications, and so much more. The first admission can be intimidating, but over the course of time we learn the ropes and get into our own little groove of things.

My first admission was the spring of 2002. I was overwhelmed and scared, as was my mother. Little did I know that this week and a half would initiate me into the life of required tune ups.

I've composed a list of things to bring and what to expect.

what to bring (many are optional, but common):

house shoes- you don't want to walk around barefoot...it's an easy way to pick up germs of others. You'll most likely have testing done, and it's more convenient to slip on some shoes than to have to tie/untie each time you're needed somewhere.

pajamas- comfy material, easy to slip on/slip off, nothing too tight or revealing

jeans and t's- this is just my opinion, but I hate wearing my PJs all the time. it seems to enhance the idea of being sick.

extra socks

easy to wear sweaters (zip up work best in my opinion)

hair brush, toothbrush, shampoo, conditioner, lotion, chapstick, make up (if you wear it), blow dryer, hair straightener (if you use one!), body wash, deodorant (most hospitals provide the basics upon admission, but it never seems to be enough or of preferred quality)

paper and pen

list of medications and medical history, emergency contact list, list of allergies

medications- especially enzymes- you'll want to have a few doses with you because many hospitals have to order them from their pharmacy. this usually takes a couple of hours, but can last longer depending on supply and demand.

pillow cases, blankets, your own towels if you'd prefer them.

snacks

if you're admitting your child, be sure to include stuffed animals, toys, books, colors, and other activities to keep them busy and entertained.

what to expect:

Every admission and patient is different. Generally, an IV/PICC is placed, or a port is accessed, nurses and doctors will ask the SAME questions, check vitals, stats, blood work will be done, X-rays, CT scan, and many other basic tests. Viral swabs may be done, as well as a wide variety of other tests.

as far as day to day, it really depends on the timing for the IV medications. Usually you'll be woken up at an irritating hour for blood pokes and IV changes...you'll get a mediocre breakfast including a Boost!  You may/may not have PT which can be walking the halls or a treadmill while your O2 is monitored. a respiratory therapist will come in for treatments, and you can expect a dietitian to visit occasionally.

tips:

make sure everyone knows the list of allergies. you may even want to post it on the door of your room.

make sure EVERYONE who comes into the room washes their hands properly and sanitizes as well. If someone is sick, do not let them enter or visit.

never hesitate to ask for a sleeping aid, sleep is crucial in recovery!

stay informed! you can NEVER ask too many questions. if a nurse/doctor does/says something you don't agree with, you have the right to inform their superior and follow the chain of command to mend the issue.

Salted

In an earlier post, we reviewed what causes Cystic Fibrosis.

Cystic Fibrosis Transmembrane Conductance Regulator- transports chloride ions across the epithelial cells. Mutations such as deletion, duplication and a few other types can cause Cystic Fibrosis. The movement of water in and out of the cells is either limited or blocked, as a result of this the mucus in the body is thicker and stickier than a normal persons.


In the late 1990's a commercial was aired warning parents that if they kissed their child and it tasted salty you should have them tested for CF. But why do people with CF secrete more  salt in their sweat? How does the chloride levels in your sweat during a sweat test determine that you are positive for CF?

 

image taken from: http://cmr.asm.org

the arrows in the above diagram show paths taken by chloride ions during secretion. In both normal and CF sweat glands in the dermis, chloride is present in sercretions at a concentration that classifies them as isotonic (same solute concentration).  The top portion of the sweat gland is normal, chloride is absorbed out of the sweat in a CFTR dependent manner as the sweat travels to the skin's surface from the gland. as a result, the chloride concentration in normal sweat is hypotonic. In the CF sweat gland, chloride absorption is hindered by defective CFTR functions. Because of this defect sweat that reaches the skins surfaces has higher than normal concentrations.

Stay salty,

B.

Genetic Differences amongst the CFTR mutation

You may have noticed that some people with CF are sicker than others, or they have different symptoms from one person to the next. I am the prime example of this. I have more health issues related to my digestive system than I do my respiratory system.

One reasoning for this is that some mutations are associated with pancreatic insufficiency, meaning the pancreas does not produce enough enzymes, thus needing the patient to take enzyme supplements. In some mutations, the pancreas functions properly, and this patient will not need the supplements.

Environmental factors also come into play. Was/is the patient exposed to cigarette smoke, pollution, or other air quality alterations?

Not all CF patients comply with their treatments, or even get the same medical treatment. Some were diagnosed at a later age, others were diagnosed early on which allowed for the opportunity to become healthier.


Remember that everyone is different. Don't let yourself get down because you're sicker than your friends, and don't have "survivors remorse," which is something that I suffered from previously. I used to feel so guilty for being healthier than some of my friends. In the end, we're all fighting this battle together. Keep it up.

-B.

Exercise

There are many benefits to exercising. Here are just a few:

- exercise can increase the levels of relaxing brain chemicals like serotonin, dopamine and norepinephrine.
- slows down the aging process on a cellular level
- reduces anxiety and depression (because of the chemicals released)
- helps build and maintain healthy muscles, bones and joints.
- reduces the risk of developing diabetes
- improves learning: increases the level of brain chemicals, known as growth factors, and builds new connections between brain cells.
- builds self esteem and improves body image
- creates a euphoric feeling- the adrenaline of exercising is also known as a "runner's high"
- the rate of lung functions decreasing is slower in those who exercise, as oppose to those who do not.


Just because you have CF doesn't mean you can't work out. Before doing anything too intense, refer to your doctor for approval. If you are experiencing an exacerbation, infection, or pulmonary hypertension, you should hold off until your functions are back up to exercise. There are a number of exercises you can do.

Yoga- Your CF clinic can provide you with a Yoga  for CF patients DVD. If you cannot get one, or don't know how to, email me and I can give you a copy. It has really great stretches that improve lung functions, posture, and the amount of air your lungs can intake. It doesn't require too much energy, but always leaves me feeling refreshed!

Walking- Get up and walk! Whether it's to the end of your street or around the block, walking is a great way to start exercising! And you'll get some fresh air and sunshine while you're at it.

Jogging- Start off jogging in place to make sure you can breathe properly, and once you're comfortable doing so, start moving around!

Weight lifting- create muscle and tone up, start with minimum weight and after a few weeks steadily increase. You'll be impressed by the muscle you'll build.

Biking- biking is always a great option!


Make sure you monitor your heart rate while exercising, stay hydrated, and if at any point you feel light headed STOP! Start off slowly and build your way up, you won't wake up a triathlete overnight, so don't even try it. Don't push yourself too hard or you'll end up in the hospital, trust me!


Remember to contact your physician in regards to exercising. Everyone's body is different, we have different tolerance levels and lung functions. The information above should not replace a personal trainer's advice or medical professional's advice.


Stay strong,

B.

More than just CF

I have composed a list of other medical conditions that are associated with CF. The list is brief, but each item will be reviewed in its own post at a later date.

CFRD- Cystic Fibrosis Related Diabetes
Osteoporosis
Arthritis
Congential absence of the Vas deferens in males
Malnutrition
Low vitamin levels
Depression

CF & School

I'm not sure how many of the readers here are in school, but here are a few tips that you may/may not know about. If you have some advice for me please feel free to share in the comment section, and thank you in advance!

When  I was in high school, I met with my teachers before the semester started. I was able to inform them of my diagnosis, treatment and accommodations that would be necessary if I was hospitalized. In the state of Texas, we call this a 504 plan and a 504 meeting. Whenever I transitioned to a community college, I was able to have the same information provided to my professors, under the same title of 504 plan. Things were added, and I needed additional documentation. My 504 plan that I have on file with my community college contains a letter from my doctor stating that I was diagnosed with Cystic Fibrosis. It includes a list of medications, emergency contact numbers, allergies, and a plethora of other crucial information. In addition to that, I was able to meet with my social worker at the clinic (the hospital that I attend makes it mandatory to meet with the social worker, even if it is just to chat, but mainly because they want patients to know about the resources available to them. I'm very thankful for this service!) My social worker provided me with a list of recommended accommodations. See below:

1. Frequent restroom breaks
2. An extra set of textbooks and classroom material in case I'm hospitalized or unable to attend class
3. A tutor for hospitalizations and home visits
4. Extended time for assignments and tests (ONLY to be used when hospitalized, or ill at home, additional doctor's note is required.)
5. Extra time between classes for a treatment (at one point I was taking albuterol 5x a day!)
6. Beverages and snacks in class, but put away under the desk or in my bag.
7. Access to employee or nurses restroom
8. Excused tardiness for morning classes
9. An assigned note taker or a recorder provided to me by the school for lectures
10. IV treatments during class
11. Sitting closer to the front of the classroom
12. One on one visits with instructors, advanced access to testing and course materials
13. Excused absences on clinic days
14. Ability to email assignments to instructors due to hospitalization or sickness at home


I think that about covers the accommodations that I have!! I don't always take advantage of them, but when I have been extremely ill they are a great tool. It's always best to have them on file for the unexpected things that occur! Also, when you're visiting a college, swing by their disability services and see what documents they require. Each institution is different! They usually offer scholarships for disabled students, so check that out as well! Before the semester starts I have to get a form from the disability services and get each teacher to sign it stating that they are aware of my special needs, and that they will abide by those rules printed in the agreement!


Best of luck to all you students out there, it isn't easy, but it's worth it!

-B.

Treatment Regimen

Immediately upon my diagnosis of CF, I was given a strict regimen of treatments, a prescription pad full of medications, and a new diet. I was told I would need to be seen by my CF doctor every three months, for the rest of my life. My mom was informed that I wouldn't live very long...the average life expectancy was around 15, if I was lucky.

Let's talk more about my treatment regimen:

First stop:
Nebulizer's and inhaled medications. I was prescribed the following:

• Albuterol 3 times a day, up to 5 times per day, if needed. Albuterol is a bronchodialtor that relaxes the muscles in the airway. Once these muscles are relaxed air flow to the lungs increases. A well known side effect of Albuterol is that it makes me hyper. It increases my heart rate, I get nervous, and then comes a nice little headache.

Over the years I was prescribed additional aerosol treatments:

• Pulmozyme-initially prescribed at 2 times per day, but this was soon changed to 1 time per day. Pulmozyme kind of acts like scissors that cut up the mucus. It thins the mucus and keeps it loose, which means you'll be able to pass it quicker, leaving less build up in your lungs. As a result, it reduces the risk of respiratory track infections
• Tobi- 2 times per day, 28 days on, 28 days off. Tobi was made to target a specific bacteria, psedudomonas aeruginosa. (more about common CF bacteria next post!) The first time I took Tobi, I felt like I could finally breathe! It was a great feeling, obviously!
• Hypertonic Saline- a sterile solution of sodium chloride (NaCl) this is a 7% solution that hydrates the thick mucus and makes it easier to expectorate! It basically does what the mutated CFTR gene doesn't do! 

Oral medication:

• Pancreas enzymes, later changed to Creon supplements with meals, later changed to Creon. 6 with meals 4 with snacks
• Urisodol, also referred to as "liver pills."  It reduces the cholesterol absorption and aids in dissolving gallstones
• ADEK- a multivitamin that includes vita A, D, E, and K. 2 times per day, later changed to SourceCF gel vitamins
• FloNase, later changed to: Nasonex- sinus spray 2x per day
• Beta Carotene 2x per day
• Vitamin D 50,000 units
• Vitamin E 400 IU 
• Advair
• Zantac, later changed to Prevacid
• Albuterol inhaler

New Diet:

• High calorie (anywhere from double to triple the normal calorie intake)- People with CF burn more calories trying to breathe than a normal person, so increasing our calorie intake makes up for the difference
• High protein
• High Iron- eat with caution, you don't want to consume too much iron. Iron helps fight infections, and it helps carry oxygen in your blood from your lungs to every cell in the body
• Salt- because we lose salt in our sweat we should consume more especially when exercising or in hot weather
• Zinc- helps fight infection and is important for growth
• Calcium- people with CF are at risk for osteoporosis, a condition in which the bones are weakened. 

It is extremely important to meet with a dietician and create a diet that caters to your specific needs! Also, remember to take your enzymes before meals as to digest and absorb the nutrients better!

Next post: common bacterias in the lungs of a CF patient

see ya later,

B.

CFRD

Cystic Fibrosis Related Diabetes (CFRD)

What is CFRD, and how is it different than any other form of diabetes?

CFRD is not the same as other types of diabetes. The treatment is much different. There are two different types of diabetes in the non CF population, which are known as type I and type II. Type I is "insulin dependent," and type II is "non insulin dependent." CFRD is a unique combination of both forms of diabetes. The body of some CF'ers with CFRD does not create enough insulin. This happens as a result of scarring in the pancreas. The other type of CFRD is insulin resistant. People with this form have a defect that causes their body to abnormally use the insulin it creates. Some symptoms of CFRD include: increased thirst, high blood sugar levels, weight loss, fatigue, decreased lung functions, and increased urine production. To determine whether or not you have CFRD your doctor may request a oral glucose tolerance test, also known as an OGTT. You will be asked to fast. A sample of blood will be taken prior to the test starting. You will then drink a liquid beverage containing a specific amount of glucose (usually 75 grams). Your blood will be taken in 30-60 minute intervals after you drink the solution. The test takes up to about 3 hours. During the test you may feel nauseous, sweaty, light headed, or short of breath. Be sure to provide your doctor or the person administering the test with a list of medications you've been taking, as certain medications can interfere with your glucose tolerance. Treatment for CFRD includes insulin, changes in your diet, and checking your blood sugar levels on a regular schedule. The insulin allows sugars and proteins to move from the blood into the body's cells. It is then used to build muscle, and for energy.


*The information in this blog is not intended to replace advice from a medical professional. If you suspect that you have CFRD, please contact your primary physican and request an OGTT.*


The link below has wonderful information, resources and tips on how to manage CFRD properly.

http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/Diet/Diabetes/CFRD-Manual-5th%20Edition-05-2012.pdf


The information included in this blog comes from previous experience, and www.cff.org


Stay Healthy,

B.

Introduction

I am 24 and living with Cystic Fibrosis (CF). Before I dive into my personal experiences with CF, I'd like to give you more information about what it is, how you get it, and how you can manage it.

In 1989, after 10 solid years of research, the CF causing gene was located and identified.

CF is a genetic mutation caused by an abnormality in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)  gene, which is located on chromosome number 7. The CFTR gene moves chloride ions across the epithelial cell membranes located in the pancreas, lungs, and a few other organs. The mutation that causes CF can occur due to a number of reasons such as: deletion, duplication and inversion. The problem with the CFTR gene is not solely deletion, duplication or inversion, the problem lies within the folding of the protein. The protein does not fold normally, therefore restricting the movement of water and chloride ions in and out of the cell.The movement of water and chloride across the cells is either blocked or limited, thus causing the mucus in a CF patient to become extremely thick and sticky. This thick, sticky habitat makes a great home for bacteria to grow.

CF is one of the most common lethal genetic diseases. Because it is genetic, it cannot be spread from person to person.  It is inherited through your parent's DNA. DNA, short for deoxyribonucleic acid, is the hereditary information that is present in all humans. Your DNA also contains information that determines your height, hair color, eye color and many other things. In order to be born with CF you must inherit two copies of the defective CF gene, one from each parent. A person with one defective gene is called a carrier. They carry the CF gene, but do not have CF because they only have one mutation. If a man and a woman are both carriers of the CF gene, their offspring will have a 25% chance of inheriting both copies, a 50% chance of inheriting one defective copy, and a 25% chance of not having CF or being a carrier. Approximately 1 in 31 Americans are carriers of the CF gene.

See chart below for most common CF mutations (CF Patient Registry, 2011)

I would like to thank you for taking the time to read my blog. As time passes, I will share more facts about CF, statistics regarding treatments and life expectancy, as well as my personal experiences, trials, tribulations and everything in between.