Failure to Thrive (FTT)

The first few years in a child's life are critical period during which the most dramatic changes in growth and mental development take place. When a baby or toddler doesn't gain weight and grow at the predicted rate, a doctor may say that the child is failing to thrive. In many cases, failure to thrive and its accompanying symptoms can be the key to effectively diagnosing cystic fibrosis as early as possible.

Failure to Thrive (FTT) is detected when a child falls below the 80th percentile for height and weight at a given age. Failure to thrive is a somewhat ambiguous term, is not a formal diagnosis. However, it serves as a starting point for exploring the underlying cause of the condition.

Symptoms or behaviors that may indicate failure to thrive include:

-Frequent diarrhea or vomiting

-Irritability and colic

-Unfocused eyes, inability to concentrate

-Developmental benchmarks such as sitting up, talking, etc. are not reached when expected

-Refusing food

-Repeated infections

In many cases, failure to thrive is connected to a baby's inability to eat or utilize calories and nutrients properly. The causes can be either physical, as in the case of a child with a cleft palate who cannot suckle for breast or bottle feeding. Failure to thrive may also be the result of a chronic condition like cystic fibrosis, in which the pancreas cannot secrete the enzymes required to help the body absorb food. Food allergies, celiac disease, and inability to tolerate milk protein are just a few of the underlying causes of failure to thrive in infants.

A child who is failing to thrive may also be the product of a poor socio-economic environment. Living in poverty without access to health care, or an inability to buy the appropriate foods for an infant, can cause failure to thrive. Sadly, failure to thrive is sometimes caused by child abuse.

Doctors rely on growth charts to determine whether an infant is growing normally. Specifically, a baby's birth weight should double by the age of 4 months, and should grow almost 10 inches in the first year. Although birth weight may drop initially, when an infant does not recover that lost weight in a suitable amount of time (2 weeks), the doctor will look into the reasons as to why. Even babies who are born prematurely are expected to progress according to an adjusted growth chart.

Correctly diagnosing the cause of FTT as early as possible is essential to prescribing the most appropriate and effective treatment plan.

What is a Pulmonary Exacerbation?

Pulmonary exacerbation [pull-moh-NARE-ee eks-a-sur-BAY-shun] is defined simply as a temporary worsening of the lung function due to an infection or inflammation. Although no formal definition exists, an exacerbation is generally characterized by the following symptoms:

1. Shortness of breath
2. Fatigue
3. Increased cough
4. More productive cough
5. Drop in FEV1 or other markers of the pulmonary function tests

There is really no way to categorize what constitutes an exacerbation for everyone with CF. Generally, if something is happening that feels out of the ordinary with regard to breathlessness or lung health, then it might be an exacerbation. In adults with cystic fibrosis, P. Aeruginosa is most often the cause of a pulmonary exacerbation, and is diagnosed with a sputum culture.

Exacerbations or "Flare Ups" associated with lung disease in cystic fibrosis are the body's way of asking for help. A doctor may diagnose an exacerbation by listening to the lungs for any unusual crackles or wheezes, and looking at comparative pulmonary function tests.

Some people with cystic fibrosis experience exacerbations at rather predictable times in the year. For some, seasonal allergies can bring about a flare. For others, it's humidity. For still others, an exacerbation may be caused by an upper respiratory infection or pneumonia.

Treating an Exacerbation

Cystic fibrosis specialists usually like to treat an exacerbation initially with a short course of prednisone. CF patients who have an asthma in addition to CF most often benefit from these corticosteroids. Bronchodilators are also useful to open up tightened airways.

In moderate exacerbations where the FEV1 drops more than a few mL from the normal baseline PFTs, oral antibiotics or inhaled antibiotics such as TOBI, Tazicef or Colistin might be prescribed. The more serious exacerbations that occur with fungal infections or bacterial infections may require a "tune-up" in the hospital where the patient can benefit not only from medication, but additional chest physiotherapy, a monitored diet and, when possible, rest.

Positive Information

It's important to remember that an exacerbation is not necessarily the precursor to declining health. People who experience predictable flare-ups at certain times of the year benefit from doing preventive tune-ups to help them enjoy longer periods of good health with relatively few symptoms. Also, just because a cystic fibrosis patient experiences an exacerbation, it doesn't mean that they have been colonized or infected with a new type of bacteria. It could simply be that an existing bug is growing too fast for the body to fight without antibiotic intervention.

An Eye to the Future

Cystic fibrosis researchers are still studying the link between exacerbations, their severity, and antibiotic therapy. Clinical trials for certain types of inhaled antibiotics are ongoing. The hope is that preventing exacerbations rather than just treating them will greatly improve the quality of life and mean survival age of people with CF.

Nasal Irrigation

Cystic fibrosis patients are prone to nasal congestion, which can inflame the upper respiratory tract and lead to sinus infections. One of the ways to help keep the sticky mucus from staying trapped in the sinuses is to gently flush them with a saline solution. Such rinses are effective in reducing the inflammation and clearing the symptoms of allergies or rhinitis.

Commercially available sinus rinses and saline sprays are available in different concentrations of salinity. Some kits come with the salt water already mixed, while others require you to add a salt packet to warm water that you put into specifically designed container such as a squeeze bottle.

To make your own saline rinse, you can follow this easy recipe:

Isotonic saline

½ teaspoon of sea salt or kosher salt

1 cup of warm water (or room temperature will do)

½ teaspoon of baking soda

Combine all ingredients in a cup or bowl. Using a bulb syringe, draw the salt water into the bulb.

Proper administration of a saline flush is necessary in order to reap its benefits. A thorough flushing can be a messy process, so it’s best to lean over the bathroom sink or shower when rinsing with it. Start by tilting your head down toward the bowl of the sink. Leave your mouth open. With the filled bulb syringe (or squirt bottle that came in the kit) squeeze firmly into one nostril. The water should flow out the other nostril. If your sinuses are quite congested, some water may flow down the back of your throat and out your mouth. This is normal. Continue rinsing with the other nostril.

Follow up the procedure by gargling with water. This will keep the salt from irritating your throat and causing a cough reflex.

A more aggressive way to flush congested sinuses is to run the saline mixture through a Water-Pik. Don’t use the attachment as is; cut it back about an inch so that the pressure of the water coming out is reduced. This method is quite effective for dislodging stubborn mucus that lingers following a sinus infection. Water-Pik now makes a nasal flush attachment tip that makes flushing your sinuses even easier.

Hypertonic saline is useful in stimulating the linings of the nasal passages to produce some moisture of their own. This helps give the mucus a more slippery surface to slide across, making it easier to clear out any congestion. Hypertonic saline is a saltier concoction and therefore may sting sensitive sinuses. It’s best to slowly increase the salinity (saltiness) of the rinse solution by adding gradually more salt.

CF Patient Preferred Sinus Rinse Products

• Breathe Ease
• Ocean
• Oasis
• Salt-Air

How is an Antibiotic Desensitization Done?

When a sputum sample is taken, the laboratory technicians will let the sample grow in a number of different types of environments to see what grows from the sample. Once bacteria is cultured, it is treated in the lab with different types of antibiotics in order to determine whether that particular bug is sensitive or resistant to specific types of antibiotics. If a culture shows sensitivity to a drug, that is the drug prescribed. But what happens when a person has an allergy to drug that the doctor wants to use to treat the infection? Suppose a culture is sensitive to a broad-spectrum antibiotic such as Cipro or Septra, but the patient experiences pulmonary edema or a severe allergic reaction called anaphylaxis when exposed to the drug?

When there are few or no alternative medications with which to treat the infection, a doctor may recommend a procedure called antibiotic desensitization. This process takes place under extremely careful supervision in the intensive care unit (ICU) of the hospital. A patient is started on a very small dose of the antibiotic and watched carefully for any signs of allergic response. When the patient is observed to tolerate that dose, the pharmacist will prescribe a slightly higher concentration of the drug. Little by little, stronger concentrations of are delivered to the body until the full recommended treatment dose is achieved.

At this point, if the patient has exhibited no severe allergic reactions to the antibiotic, he or she is said to be successfully desensitized. From that point forward, that antibiotics should always be used first to treat infection so that the patient does not redevelop sensitivity to it.

Related Links

One medical protocol for desensitization to Bactrim/Septra (TMP/SMX)

Antibiotic desensitization in adults with cystic fibrosis

Successful Meropenem Desensitization in a Patient with Cystic Fibrosis

Desensitization Useful for Allergy to Life-Saving Drug

Antibiotic allergy in cystic fibrosis

Tune-Ups Explained

Preventive care is a cornerstone of effective treatment for cystic fibrosis. A “tune-up” is a term used in the cystic fibrosis community to refer to a preventive course of intravenous antibiotics and respiratory therapy. Depending on the doctor’s preference and the specific case of the individual with CF, a “tune up” can last from 2 to 4 weeks. Usually a tune up is started in the hospital, where the CF specialist and team can keep a close eye on things with regard to dosages of antibiotics, and any changes in pulmonary function.

Some CF specialists and CF centers maintain a practice of preventive “tune ups” every six months.

The practice of regularly scheduling a tune-up can be especially beneficial for young adults with cystic fibrosis who may not always be the most compliant with their medications. By giving them a boost of antibiotics and bronchotherapy before they exhaust themselves and deplete their health reserves, they will be in a better state health-wise to continue as many normal activities as possible with their peers.

Similarly, many college students with CF have found that by scheduling a tune-up during their semester breaks, they are able to regain some of the ground they lost due to stress during the school term. Checking yourself into the hospital for a tune-up while classes are not in session is a great way to keep from missing important class notes and lectures.

The most difficult part of a preventive tune-up is that the patient may not feel as sick and worn out as during other times requiring IV therapy. This makes the resting and recuperating part of a tune-up a challenge, because the temptation is there to remain active and continue in all normal activities. The powerful combination of antibiotics may actually make a person feel worse for a time, but a rebound is almost always sure to follow. Even though a person may feel “fine” during a tune-up, it’s important that they rest, stay well hydrated, and use the time to replenish the body.

The hope and goal is to prevent repeated hospitalizations due to pulmonary exacerbations, pneumonia, or other worsening of symptoms.

What To Expect when Having a PICC Line Placed

PICC stands for “peripherally inserted central catheter.” It is a long tube inserted into the arm and used for intravenous antibiotic therapy.. The tubing that goes into the patients arm is threaded into a very large vein, which connects it to the right side of the heart. By delivering medication so close to the heart, it gets pumped through the body more efficiently.

PICC lines are often used in CF patients because they can last much longer than traditional IV needles. When properly cared for, they can last weeks, or even months. The flexible tubing of the PICC line enables a patient to continue with most normal activities.

Inserting a PICC line is a longer and more difficult process than starting a regular IV. It must be done by a specially trained radiologist, nurse, or other medical professional. The most important part of placing a PICC line is to verify that the tip rests in the correct position near the heart.

The process of inserting a PICC line is as follows:

1. The length of the PICC to be inserted is determined by measuring the distance from the crook of the elbow to the heart.

2. A trained medical professional preps the site (usually the inside elbow of the left arm) with disinfectant.

3. The patient may receive local anesthesia by injection or application of a cream at the site of insertion

4. A small needle is inserted into a large vein in the inside elbow of the left arm.

5. A wire is placed through the needle, then the needle is removed. A special tube called an “introducer sheath” is placed over the wire. This makes sure that the puncture in the arm is large enough for the PICC line.

5. The PICC line and introducer sheath are fed into the appropriate vein over the wire. Threading stops when the total length inserted matches the length that was measured at the beginning of the procedure.

6. The introducer sheath and wire are removed.

7. The line is capped with a cannula or septum that will be the site of infusion. To verify that the line is in the vein, it is flushed via syringe with 3-5cc of normal saline. The syringe is then used to check for blood return.

8. A chest x-ray is done to confirm that the line is placed in the correct position.

9. The line is flushed with heparin, to prevent clotting in the line.

FAQ about PICC lines:

1. Can I shower/bathe with it?

Yes, but it needs to be protected as much as possible to keep moisture out of the dressing.

2. Can I do normal activities with it?

A PICC line should be handled with care. No vigorous activities (such as sports or heavy lifting) should be done within the first 72 hours after receiving a PICC line.

3. How do I care for the PICC line?

The Cystic Fibrosis Foundation has a PDF available with information about how to care for a PICC line

4. Does it hurt?

Initially, there is a some discomfort from the procedure of having the catheter inserted. Within a day or two, it will feel better. Most often the discomfort is caused by the dressing irritating the skin around the site.

5. How long can a PICC line last?

CF patients who have intravenous therapy courses lasting 10-30 days are well suited by a PICC line. Although PICCs cannot be used for extremely long terms (like a portacath) they can last up to 6 months.

6. How is the PICC line removed?

The catheter is removed by simply taking off the dressing, then pulling the catheter out. Pressure is then applied to the site to reduce bleeding. A bandage can be used to cover it while it heals.

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For more information please visit the following links:

CFF.org PICC Info

PICC line Wikipedia