The AELAM drive against lymphangioleiomyomatosis

2023-06-09 08:17:05

The AELAM drive against lymphangioleiomyomatosis

So much so that the Spanish Association of Lymfangioleiomyomatosis (AELAM) organized its XIII medical conference on June 3, 2023 in order to update the medical keys of this respiratory pathology at the level of its diagnosis, treatment and prognosis.

This specialized conference was inaugurated by Dr. Julio Ancochea Bermúdez, head of the Pneumology Service of the Hospital Universitario de la Princesa in Madrid and professor of Medicine at the Autonomous University of the Spanish capital.

Precisely, the link between Dr. Ancochea and these women since the beginning of ELAM has impregnated the pulmonologist with a passionate pride that was also reflected in the book “Rare but interesting” with a poem dedicated to all those who suffer from lymphangioleiomyomatosis.

Since then, there are many LAM women and their families who have recognized her medical work over the last four decades, always promoting research and medical care quality, two unequivocal characteristics of the Pneumology team A.

A feeling of belonging that sinks into the heart of the humanist doctor, disrupting his sinus rhythm, after receiving the sincere thanks of each and every one of them… “Very special” women, Julio emphasizes.

Dr. Ancochea’s AELAM charter

“It is with a deep sense of gratitude and appreciation that I address you in this letter.

I want to express my most sincere gratitude for your immense support and dedication to the AELAM association, the Spanish Association of Lymfangioleiomyomatosis.

Their initial commitment and help have undoubtedly been essential for the involvement and awareness of other professionals in this rare disease.

It is thanks to all of this that today we can celebrate the twentieth anniversary of AELAM in this Aelam Conference 2023.

Her humanistic vision of medicine has helped many women affected by Lymphangioleiomyomatosis and their families. Her dedication to understanding and treating this disease has been truly inspiring.

Her commitment to Lam and her desire to help women of childbearing age who suffer from this disease have given us hope and have positively impacted countless lives.

Thanks to your wonderful decision to support AELAM, the association has been a beacon of knowledge, support and resources for those women who fight against Lymphangioleiomyomatosis.

His compassionate approach and ability to connect with people have left an indelible mark on all those who have been fortunate enough to know him.

Without your valuable contribution and support, we would not be here today celebrating this 20th anniversary.
His work during these two decades has been inspiring for many of us.

On behalf of all AELAM members, as well as the women and families affected by Lymphangioleiomyomatosis, we extend our most sincere thanks. His dedication and contribution have been invaluable for AELAM.

We hope that it will continue to be a guide and a reference for us and for other pulmonology professionals in the future.

His legacy is a testament to his commitment and passion for medicine and helping others.

We sincerely thank you for your support and the wonderful video you sent us and your warm words that made more than one of us cry with emotion.

With sincere gratitude and admiration…

Silvia Leida Vallespí, president of the Spanish Association of Lymphangioleiomyomatosis

It should be remembered that in Spain one in every 100,000 women between the ages of sixteen and seventy suffers from LAM; a pathology that is included among the diffuse interstitial lung diseases (EPIDs).

It first manifests with dyspnea generated by lesions in the form of bullae or hidden cysts in the lungs; non-cancerous neoplasms that systematically impair respiratory function until it is reduced to nothing.

The patient begins to drown after undertaking very small efforts, such as walking down the street or through a beautiful garden; abnormal situation at certain ages. The shortness of breath will increase for no apparent reason as the months go by.

Everything points to mutations in the TSC 1 and 2 genes, which is why two types of lymphangiomyomatosis are diagnosed:

• The LAM linked to tuberous sclerosis (TSC 1 mutation, between 30%-40% of cases) is characterized by the formation of hamartomas -congenital malformation- at the level of the central nervous system, skin, eyes, kidney and lung.
• In the sporadic LAM (TSC 2 mutation) lung damage predominates, although renal angiomyolipomas, axial lymphadenopathy, and abdominal lymphangiomas may develop. The nervous system, the skin or the eyes are not altered.

Lymphangioleiomyomatosis is complicated by pneumothorax (69% of cases) and chylothoraxin addition to 20% of cases of hemoptysis (expulsion through the mouth of blood from the pulmonary system), 50% dry cough and another 50% renal angiomyolipomas (benign tumor mass).

LAM evolves towards respiratory failure, one of the causes that delays early diagnostic from two to five years when confused with asthma, emphysema or COPD.

“And I would like to highlight that there are more than 200 EPIDs and lymphangioleiomyomatosis represents only 1% of them. They all have common clinical, radiological, functional and respiratory characteristics”, explains Dr. Julio Ancochea.

Diffuse interstitial lung diseases affect the distal airway epithelium, alveolar walls, capillary endothelium, and connective tissue between the septa and peribronchial and peribronchial tissue.

Therefore, they decrease the transfer of gas exchange (carbon monoxide diffusion) and cause restrictive ventilatory disorders, although in LAM, given its characteristics, an obstructive pattern is observed.

Curing these diseases, as far as possible, as well as deepening research, teaching, innovation and the transfer of this group of lung pathologies to the population as a whole, is the main objective of the specialists involved. .

In addition to the favorite son of A Pobra de Trives from Ourense, they participated in the XIII AELAM medical event, the Dr. Piedad Ussettiformer head of the Lung Transplant Unit of the Puerta de Hierro University Hospital of Majadahonda and the Dr. A.S. Alvaro Casanovapulmonologist at the Hospital del Henares de Coslada, both in Madrid.

At the LAM Clinical Table: present and future, the speakers were the Dr. Maria Molina, pulmonologist at the Bellvitge University Hospital in Barcelona; the Dr. Rosalia Laporta, a pulmonologist at the Puerta de Hierro University Hospital in Majadahonda; and the Dr. Berta SáezLung Transplant Unit of the Vall d’Hebrón University Hospital, also in Barcelona.

Regarding the state of research in lymphangioleiomyomatosis, both in Spain and internationally, comprehensive information was offered on:

progress of LORALAM clinical trialwith Dr. María Molina, a pulmonologist at the Bellvitge University Hospital and a researcher of diffuse interstitial lung diseases at the IDIBEL.

“Lipid metabolism and characteristics of LAM disease”, with the doctoral student Sandra Baigesof the ProCURE ICO-IDIBELL program.

“Why do LAM cells proliferate slowly for years?”, with ProCURE ICO-IDIBELL PhDs, Sandra Baiges, Roderic Espin and Irene García.

“Studies on the relationship of hormones in Lymphangioleiomyomatosis and summary of current international research”, with the intervention of dr. Stephen R. Hammeshead of the Division of Endocrinology and Metabolism at the University of Rochester (New York, USA).

Likewise, the structural keys of the agreement between AELAM, the Catalan Institute of Oncology (ICO) and the Bellvitge Biomedical Research Institute (IDIBELL): Future of the study and priorities, with the participation of the Dr. Miquel Ángel PujanaHead of Breast Cancer Research at IDIBELL.

And to close this self-sacrificing and voluntarist writing, I reproduce some verses by Mario Benedetti that Ancochea recites from memory:

“Don’t give up, please don’t give in,

although the cold burns,

although fear bites,

even if the sun sets and the wind stops,

There is still fire in your soul,

There is still life in your dreams”

Women with LAM do not give up… they are the paradigm of dreams, that is why they are essential… and as Ángel Gabilondo, the current Ombudsman, would say, with them, and with you, I mean better with me. With them, and with you, it’s less difficult.”

Dr. Julio Ancochea Bermúdez, madrigallego doctor and university professor