Bay Biosciences provides high quality, clinical grade bio-samples, cryogenically preserved biopsy tissue, FFPE blocks with matched sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid specimens from patients diagnosed with Cystic Fibrosis (CF) disease.
The sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid samples are processed from patient’s peripheral whole-blood using customized collection and processing protocols. The Cystic Fibrosis bio-specimens are collected from unique patients diagnosed with Cystic Fibrosis (CF) disorder and are provided to a valued pharmaceutical customer for research, development and drug discovery.
Cystic Fibrosis (CF) Disease Overview
Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the lungs, digestive system and other organs in the body. Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery. But in patients with Cystic Fibrosis, a defective gene causes the secretions to become sticky and thick. Instead of acting as lubricants, the secretions plug up tubes, ducts and passage ways, especially in the lungs and pancreas.
Although cystic fibrosis is progressive and requires daily care, patients with Cystic Fibrosis are usually able to attend school and work. They often have a better quality of life than patients with Cystic Fibrosis had in previous decades. Improvements in screening and treatments mean that patients with Cystic Fibrosis now may live into their mid- to late 30s or 40s, and some are living into their 50s.
Cystic Fibrosis Symptoms
In the United States because of newborn screening, cystic fibrosis can be diagnosed within the first month of life, before symptoms develop. But Cystic Fibrosis patients born before newborn screening became available may not be diagnosed until the signs and symptoms of Cystic Fibrosis disease show up.
Cystic fibrosis signs and symptoms vary, depending on the severity of the disease. Even in the same person, symptoms may worsen or improve as time passes. Some patients may not experience symptoms until their teenage years or adulthood. Patients who are not diagnosed until adulthood usually have milder disease and are more likely to have atypical symptoms, such as recurring bouts of an inflamed pancreas (pancreatitis), infertility and recurring pneumonia.
Patients with cystic fibrosis have a higher than normal level of salt in their sweat. Parents often can taste the salt when they kiss their children. Most of the other signs and symptoms of Cystic Fibrosis affect the respiratory system and digestive system.
Cystic Fibrosis (CF) Respiratory Signs and Symptoms
The thick and sticky mucus associated with Cystic Fibrosis (CF) disease clogs the tubes that carry air in and out of the lungs. This can cause signs and symptoms such as:
- A persistent cough that produces thick mucus (sputum)
- Wheezing
- Exercise Intolerance
- Repeated Lung Infections
- Inflamed Nasal passages or a stuffy nose
- Recurrent Sinusitis
Cystic Fibrosis associated Digestive Signs and Symptoms
The thick mucus can also block tubes that carry digestive enzymes from the pancreas to the small intestine. Without these digestive enzymes, the intestines aren’t able to completely absorb the nutrients in the food you eat. The result is often:
- Foul-smelling, greasy Stools
- Poor weight gain and growth
- Intestinal blockage, particularly in newborns (meconium ileus)
- Chronic or severe constipation, which may include frequent straining while trying to pass stool, eventually causing part of the rectum to protrude outside the anus (rectal prolapse).
Cystic Fibrosis Disease Causes
In Cystic Fibrosis (CF) disease, a defect (mutations) in a gene, the cystic fibrosis transmembrane conductance regulator (CFTR) gene changes a protein that regulates the movement of salt in and out of cells. The result is thick, sticky mucus in the respiratory, digestive and reproductive systems, as well as increased salt in sweat.
Many different defects can occur in the gene. The type of gene mutation is associated with the severity of the condition. Children need to inherit one copy of the gene from each parent in order to have the disease. If children inherit only one copy, they won’t develop cystic fibrosis. However, they will be carriers and could pass the gene to their own children.
Cystic Fibrosis (CF) Disease Risk Factors
Because Cystic Fibrosis disease is an inherited disorder, it runs in families, so family history is a risk factor. Although Cystic Fibrosis disease occurs in all races, it’s most common in white people of Northern European ancestry.
Cystic Fibrosis (CF) Disease Complications
Complications of Cystic Fibrosis disease can affect the respiratory, digestive and reproductive systems, as well as other organs.
Cystic Fibrosis related Respiratory System Complications
- Damaged Airways (Bronchiectasis): Cystic Fibrosis (CF) disease is one of the leading causes of bronchiectasis, a chronic lung condition with abnormal widening and scarring of the airways (bronchial tubes). This makes it harder to move air in and out of the lungs and clear mucus from the bronchial tubes.
- Chronic Infections: Thick mucus in the lungs and sinuses provides an ideal breeding ground for bacteria and fungi. Patients with Cystic Fibrosis (CF) disease may often have sinus infections, bronchitis or pneumonia. Infections with bacteria that is resistant to antibiotics and difficult to treat is common.
- Growths in the Nose (Nasal Polyps): Because the lining inside the nose is inflamed and swollen, it can develop soft, fleshy growths (Polyps).
- Coughing up Blood (Hemoptysis): Bronchiectasis can occur next to blood vessels in the lungs. The combination of airway damage and infection can result in coughing up blood. Often this is only a small amount of blood, but it can also be life-threatening.
- Pneumothorax: In this condition, air leaks into the space that separates the lungs from the chest wall, and part or all of a lung collapses. This is more common in adults with Cystic Fibrosis (CF) disease. Pneumothorax can cause sudden chest pain and breathlessness. Patients often feel a bubbling sensation in the chest.
- Respiratory Failure: Over time, Cystic Fibrosis (CF) disease can damage lung tissue so badly that it no longer works. Lung function usually worsens gradually, and it eventually can become life-threatening. Respiratory failure in patients with CF is the most common cause of death.
- Acute Exacerbations: Patients with Cystic Fibrosis (CF) disease may experience worsening of their respiratory symptoms, such as coughing with more mucus and shortness of breath. This is called an acute exacerbation and requires treatment with antibiotics. Decreased energy and weight loss also are common during exacerbations.
Cystic Fibrosis related Digestive System Complications
- Nutritional Deficiencies: Thick mucus can block the tubes that carry digestive enzymes from the pancreas to the intestines. Without these enzymes, the body can’t absorb protein, fats or fat-soluble vitamins, so patient can’t get enough nutrients. This can result in delayed growth, weight loss or inflammation of the pancreas.
- Diabetes: The pancreas produces insulin, which the body needs to use sugar. Cystic Fibrosis (CF) disease increases the risk of diabetes. About 20% of teenagers and 40% to 50% of adults with Cystic Fibrosis develop diabetes.
- Liver Disease: The tube that carries bile from the liver and gallbladder to the small intestine may become blocked and inflamed. This can lead to liver problems in patients, such as jaundice, fatty liver disease and cirrhosis and sometimes gallstones.
- Intestinal Obstruction- Intestinal blockage can happen to patients with Cystic Fibrosis disease at all ages.
- Intussusception a condition in which a segment of the intestine slides inside an adjacent section of the intestine like a collapsible telescope, also can occur.
- Distal Intestinal Obstruction Syndrome (DIOS): DIOS is partial or complete obstruction where the small intestine meets the large intestine.
Cystic Fibrosis related Reproductive System Complications
- Infertility in Men: Almost all men with Cystic Fibrosis (CF) disease are infertile because the tube that connects the testes and prostate gland (vas deferens) is either blocked with mucus or missing entirely. Certain fertility treatments and surgical procedures sometimes make it possible for men with Cystic Fibrosis (CF) disease to become biological fathers.
- Reduced Fertility in Women: Although women patients with Cystic Fibrosis (CF) disease may be less fertile than other women, it is possible for them to conceive and to have successful pregnancies. Still, pregnancy can worsen the signs and symptoms of Cystic Fibrosis.
Cystic Fibrosis related Other Complications
- Thinning of the Bones (Osteoporosis): Patients with Cystic Fibrosis (CF) disease are at higher risk of developing a dangerous thinning of the bones. They may also experience joint pain, arthritis and muscle pain.
- Electrolyte Imbalances and Dehydration: Patients with Cystic Fibrosis have saltier sweat, the balance of minerals in their blood may be upset. This makes them prone to dehydration, especially with exercise or in hot weather. Signs and symptoms include increased heart rate, fatigue, weakness and low blood pressure.
- Mental Health Problems: Patients suffering from a chronic illness like Cystic Fibrosis (CF) disease, that has no cure may cause fear, depression and anxiety in CF patients.
Detailed clinical data, elevated Immuno-reactive Trypsinogen (IRT) levels , biomarkers, CFTR genetic information, biopsy, pathology annotations associated with the Cystic Fibrosis (CF) patient’s specimens is provided to a valued customer for research, development and drug discovery. The Cystic Fibrosis (CF) disease sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid samples are processed from patients peripheral whole-blood using customized processing protocols provided by the researcher.
Bay Biosciences is a global leader in providing researchers with high quality, clinical grade, fully characterized human tissue samples, bio-specimens and human bio-fluid collections from cancer (tumor) tissue, cancer sera (serum), cancer plasma, cancer PBMC and human tissue samples from most other therapeutic areas and diseases.
Bay Biosciences maintains and manages it’s own bio-repository, human tissue bank (biobank) consisting of thousands of diseased samples (specimens) and from normal healthy donors available in all formats and types. Our biobank procures and stores fully consented, deidentified and institutional review boards (IRB) approved human tissue samples and matched controls.
All our human human tissue collections, human specimens and human bio-fluids are provided with detailed samples associated patient’s clinical data. This critical patient’s clinical data includes information relating to their past and current disease, treatment history, lifestyle choices, biomarkers and genetic information. Patient’s data is extremely valuable for researchers and is used to help identify new effective treatments (drug discovery & development) in oncology, other therapeutic areas and diseases. This clinical information is critical to demonstrate their impact, monitor the safety of medicines, testing & diagnostics, and generate new knowledge about the causes of disease and illness.
Bay Biosciences banks wide variety of human tissue samples and biological samples including cryogenically preserved -80°C, fresh, fresh frozen tissue samples, tumor tissue samples, FFPE’s, tissue slides, with matching human bio-fluids, whole blood and blood derived products such as serum, plasma and PBMC’s.
Bay Biosciences is a global leader in collecting and providing human tissue samples according to the researchers specified requirements and customized, tailor made collection protocols. Please contact us anytime to discuss your special research projects and customized human tissue sample requirements.
Bay Biosciences provides human tissue samples (human specimens) from diseased and normal healthy donors; including peripheral whole-blood, amniotic fluid, bronchoalveolar lavage fluid (BAL), sputum, pleural effusion, cerebrospinal fluid (CSF), serum (sera), plasma, peripheral blood mononuclear cells (PBMC’s), saliva, Buffy coat, urine, stool samples, aqueous humor, vitreous humor, kidney stones, renal calculi, nephrolithiasis, urolithiasis and other bodily fluids from most diseases including cancer. We can also procure most human bio-specimens and can do special collections and requests of human samples that are difficult to find. All our human tissue samples are procured through IRB approved clinical protocols and procedures.
In addition to the standard processing protocols Bay Biosciences can also provide human plasma, serum, PBMC bio-fluid samples using custom processing protocols, you can buy donor specific sample collections in higher volumes and specified sample aliquoting from us. Bay Biosciences also provides human samples from normal healthy donors, volunteers, for controls and clinical research, contact us Now.
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