CFRD diabetes

Basic Concepts
Pancreas – Located in the upper central abdomen (behind the stomach) and has two main function groups:
1. Excretion of “digestive juices” (enzymes) responsible for the breakdown of proteins and fats in digested food.
2. Hormone secretion – mainly insulin and glucagon, secreted by groups of cells inside the pancreas called “Langerhans”.

Insulin & nbsp; – secreted in response to an increase in blood sugar after eating carbohydrates. Insulin is a “key” for glucose to enter the body’s cells, thus allowing the body’s cells to use sugar as an energy source. Insulin also encourages the formation of protein and adipose tissue.

Diabetes that occurs in CF patients. It is a special form of diabetes that is different from both juvenile diabetes and “type 2” diabetes that usually occurs in older people.

CFRD can be divided into 4 levels:
1. & nbsp; Normal sugar tolerance & nbsp; – Normal sugar values ​​both fasting and after drinking Concentrated sugar solution (fasting sugar values ​​are less than 110 mg percent, and sugar values ​​after loading sugar are less than 140 mg percent).
2. & nbsp; Sugar tolerance disorder & nbsp; – “Pre-diabetes” condition in which the sugar levels after sugar loading are slightly increased (in the range of 200-140 mg percent).
3. & nbsp; CFRD without fasting hyperglicemia & nbsp; – Diabetes “Non-fasting” – a condition in which fasting sugar values ​​are normal, but after loading values ​​are significantly increased (i.e., greater than 200 mg percent).
4. & nbsp; CFRD with fasting hyperglicemia & nbsp; – “fasting” diabetes – the sugar values ​​are increased both during fasting and after loading sugar (fasting is greater than 126 mg percent, and after loading greater than 200 mg percent).

How does diabetes develop in CF patients?
The genetic defect in the CFTR (chlorine channel) gene affects salt content and viscosity of healthy secretions, gastrointestinal tract, exocrine pancreas, and reproductive system. The viscous secretions cause obstructive damage to these organs. As a result, the pancreatic tissue is replaced by fat and scar tissue, and destruction of the pancreatic islets by Langerhans occurs with impaired insulin secretion. Insulin is available. Insulin resistance can occur especially during infection and during glucocorticoid therapy (cortisol, prednisone). Prolonged exposure to high blood sugar levels (ie untreated or unbalanced diabetes) can also cause insulin resistance. Diabetes is not in CF patients. Of “all or nothing,” but there is a continuous and two-way transition between a normal condition and diabetes. In other words, a condition in which a CF patient whose blood sugar levels are normal is normal will develop insulin resistance during hospitalization (due to infection or prednisone treatment). In this type of condition, the body uses insulin less efficiently, leading to intolerance to sugar and even diabetes. After recovery, there will be an improvement in insulin resistance, and then the sugar values ​​will also return to normal values.

What is the prevalence of CFRD?
The average age of CFRD onset is between 18 and 21 years.

From the concentration of reports coming from large centers the prevalence is divided as follows:
Children (up to age 10) – up to 10%.
Adolescents (19-10) – 25% -12.
Adults (over the age of 20) – 50% -30.
About two-thirds of diabetics have normal fasting sugar values ​​(ie we will only detect diabetes if we perform a sugar loading test or check our blood sugar levels after a high-carbohydrate meal).

Symptoms and Treatment of CFRD

Signs of CFRD
CFRD usually appears slowly and gradually and the existence of the disease can be missed for a long time. Suspicious signs of CFRD are:
1. Multiple drinking and urination.
2. Weight loss or difficulty gaining weight despite good nutritional care.
3. Disorder of height growth or sexual maturation.
4. Unexplained worsening of lung function.

Why is it important to treat CFRD?
As with any other diabetes, CFRD can cause complications due to vascular damage. There is talk of “microvascular” complications resulting from damage to the small blood vessels and manifested in damage to the eyes and kidneys and “macro-vascular” complications resulting from damage to the large blood vessels and can manifest themselves in damage to the blood supply to the heart and brain. These complications appear for many years after the onset of the disease and can be prevented almost completely when Diabetes is treated properly. In addition, CF patients with Diabetes directly affect the course of their disease. Untreated CFRD can manifest in impaired lung function and nutritional status and even affect long-term morbidity and mortality.

CFRD Treatment
The treating staff includes the pulmonologist (pulmonologist), endocrinologist, diabetes nurse, dietitian and support staff (social worker and psychologist).
As with any other type of Diabetes, the treatment is based on the trinity of medication, proper nutrition, and exercise.
The first step in treatment is monitoring blood sugar, i.e., measuring blood sugar in a home device 4-3 times a day, so that the medication can be adjusted to the sugar level. There are now new devices for monitoring blood sugar that use a minimal drop of blood, and the pain or discomfort of using them is minimal.
Another device we use today is the Glucoensor, which continuously monitors blood sugar levels for 72 hours and provides more information On blood sugar levels throughout the day.

CFRD Drug Treatment
The recommended CFRD treatment is insulin injection.
Oral drug therapy (as in common Diabetes in adults) is not recommended according to the guidelines of the Association CF in the US. This is due to the lack of experience with this treatment of CF patients and because understanding the mechanism by which CFRD causes insulin therapy is more accurate. A major study is currently underway in the United States examining the effectiveness of oral drug therapy in CFRD patients in the early stages of Diabetes.

Principles of Insulin Treatment in CFRD
Treatment is based on a combination of long-term insulin whose function is to provide the basic insulin intake (ie, the basic level of insulin present in the body even when we are not eating), and short-term insulin whose function is to prevent The increase in sugar levels after meals.
The long-term types of insulin used in Israel are HUMULIN-N and INSULOTARD, which are injected twice a day in the morning and the evening, and LANTUS, which is usually injected once a day in the evening.
Short-term insulin types used in Israel: ACTRAPID, HUMULIN-R, HUMALOG, and NOVORAPID. Short-term insulin is usually injected before each meal. When the dose of insulin is injected depends on the blood sugar level and the meal’s composition.
Each type of insulin has its advantages and disadvantages, and the appropriate combination for each patient depends on the nature of Diabetes and personal lifestyle. In principle, you can usually “buy” more flexibility in the plan and composition of meals by injecting insulin more often during the day.
Another method of insulin therapy is the use of an insulin pump. Inject the insulin inside through a thin tube into a tiny butterfly inserted under the skin. Increases physiological insulin secretion, allowing maximum flexibility in the plan.

Nutritional treatment of CFRD
Unlike patients with other types of diabetes, CF patients are of paramount importance to ensure caloric supply, and therefore dietary guidelines are different compared to “normal” diabetics.
In principle, All types of food (including foods containing sugars) can be consumed, except for sugary drinks. In the meal itself, it is recommended to combine the various food groups and always combine carbohydrates with proteins and fats. For example, we add cheese to crackers or crackers, and yogurt or nuts are added to the fruit. You can learn a carbohydrate count from your dietitian at the CF clinic or the diabetes clinic.

By: & nbsp; Dr. Dalit Modan & nbsp; – The Unit for Endocrinology and Juvenile Diabetes Hospital for Children – Edmond and Lily Safra in Tel Hashomer.

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